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Contents Vol. 6, No. 7 (July 2015)

Cover Image 431 Single coronary artery arising from the right coronary sinus with mid-left anterior Magnetic resonance imaging scan of the brain descending artery segment courses through showing a vermian hemangioblastoma associated the ventricular myocardium: A rare entity with 2 small right cerebellar hemangioblastomas. Andrea Romagnoli, Irene Coco, Dominique De Vivo, Eros Calabria, Giovanni Simonetti 436 Cannabis consumption before surgery may Case Series be associated with increased tolerance of anesthetic drugs: A case report 391 Anomalous origins of a coronary artery from the Georg Richtig, Götz Bosse, Friederike Arlt, pulmonary artery: A series of three case reports Christian von Heymann Cover Figure: undetected into adulthood 440 Primary hydatid cyst of gallbladder: A case Katie L. Mastoris, Ataul Qureshi, Navin K. report Subrayappa, Matthew W. Martinez, James Wu Pronoy Kabiraj, Shib Shankar Kuiri, Utpal De Case Report 444 Occult hip fracture diagnosed by MRI scan after inconclusive X-ray and CT scan 396 An unusual association of von Hippel–Lindau Bogdan Deleanu, Radu Prejbeanu, Florin disease and posterior nutcracker syndrome: Birsasteanu, Dinu Vermesan, Liviu-Ionut Management of neurological and urological Micle, Eleftherios Tsiridis, Vlad Predescu aspects in a young male Intramedullary spinal cord abscess by Nadhir Karmani, Faouzi Mallat, Wissem Hmida, 448 Nocardia: A case report Khaled Ben Ahmed, Oussama Karmani, Khaled Alshabani, Joseph Adrian L. Sidiya Oueld Chavey, Amel Ben Abdallah, Buensalido, Milagros P. Reyes, Ayman O. Faouzi Mosbah, Hedi Krifa Soubani 403 1,25-Dihydroxyvitamin D-mediated hyper- calcemia in a patient with malignancy without Clinical Images lymphoma Katie Mastoris, Sarah Park, Vasudev Magaji 454 Perianal aggressive angiomyxoma in a 407 A case report of an uncommon large size of male patient prostatic cyst Zainab Taha ALHumoud, Najla Aldaoud, Jingjin Yang, Xingkai Liu, Yong Zhang Hussain Abrar, Amro Salem Bowen’s disease involving the dorsal and 411 Coincident retroperitoneal and sigmoid colon 457 liposarcoma: A rare occurrence volar aspects of left thumb: An unusual Ayvaz Ulaş Urgancı, Erkan Oymacı, Enver site diagnostic entity Vardar, Ebru Akıncılar, Ömer Engin Shagufta Rather, Peerzada Sajad, Iffat Hassan 416 Scrub typhus vasculitis causing pan-digital gangrene Suja Lakshmanan, Krishnamoorthy Editorial Seetharaman, Ramakrishnan S.R., Sathiyan Sivanandam, Koushik A.K. 460 Management of Inflammatory breast cancer: current concepts 422 Adult nephroblastoma Awad Ali M. Alawad All Articles: N. Naqos, Z. Bouchbika, A. Taleb, N. Benchkroune, H. Jouhadi, N. Tawfiq, S. Sahraoui, A. Benider 427 Eruptive Collagenoma in a mongol girl: A rare association Balwinder Kaur Brar, Mahajan B. B., Nidhi Kamra

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CASECase REPORTSeries Peer Reviewed OPEN| OPEN ACCESS ACCESS Anomalous origins of a coronary artery from the pulmonary artery: A series of three case reports undetected into adulthood

Katie L. Mastoris, Ataul Qureshi, Navin K. Subrayappa, Matthew W. Martinez, James Wu

Abstract strong evidence based recommendations for management of adult patients. With advances in Introduction: An anomalous origin of the diagnostic modalities and potentially an increase coronary artery from the pulmonary artery in reported ACAPA cases, more data will become (ACAPA) is a rare congenital anomaly resulting available to helpguide the decision of surgical in sudden death in 90% of infants during their verses medical management. first year of life. Diagnosis in living adults is particularly unusual, especially for left coronary Keywords: Anomalies, Cardiac surgery, Chest artery arising from the pulmonary artery pain, Coronary vessel, Pulmonary artery (ALCAPA) given the large perfusion distribution that the left system provides to the myocardium. How to cite this article Surgery to correct and restore a bi-coronary arterial circulatory system has historically been Mastoris KL, Qureshi A, Subrayappa NK, Martinez the standard of treatment given the lethality of MW, Wu J. Anomalous origins of a coronary artery the diagnosis. However, advances in coronary from the pulmonary artery: A series of three case imaging have led to an increased incidence of reports undetected into adulthood. Int J Case Rep diagnosis in adulthood and challenges the true Images 2015;6(7):391–395. association between sudden death and ACAPA. This makes the decision for surgical correction doi:10.5348/ijcri-201505-CS-10056 more complex. Case Series: Herein, we presented three cases of coronary anomalies arising from the pulmonary artery that remained undetected until adulthood, only one of which was managed surgically. Conclusion: Currently, the prevalence INTRODUCTION of ACAPA in adults is low. Hence, there is no Anomalous origins of the left or right coronary artery from the pulmonary artery (ALCAPA or ARCAPA) 1 2 2 Katie L. Mastoris , Ataul Qureshi , Navin K. Subrayappa , are rare congenital anomalies. Sudden death from Matthew W. Martinez2, James Wu3 myocardial infarction and congestive heart failure occurs 1 Affiliations: D. O, Department of Medicine, Lehigh Valley in 90% of infants with this anomaly within the first year Health Network, Allentown PA; 2MD, Division of Cardiology, of life [1, 2]. For the few that survive into adulthood Department of Medicine, Lehigh Valley Health Network, Allentown PA; 3MD, Division of Cardiothoracic Surgery, by way of collateral systems, ALCAPA/ARCAPA may Department of Surgery, Lehigh Valley Health Network, be an important cause of sudden cardiac death. The Allentown PA. pathophysiology between the two diseases is similar Corresponding Author: Matthew W. Martinez, MD, Address: [2]. However, ARCAPA in adults has been reported 1250 S Cedar Crest Blvd, Suite 300, Allentown PA 18103; more commonly than ALCAPA [3]. This distinction is Ph: 610-314-4161; Fax: 610-402-3112; Email: matthew_w. likely due to the large perfusion distribution that the left martinez@lvhn. org system provides to the myocardium. Regardless, both diseases have remained rare diagnoses in adults [3, 4]. Received: 22 January 2015 We present three cases of coronary anomalies arising Accepted: 25 February 2015 from the pulmonary artery that remained undetected Published: 01 July 2015 until adulthood, which may alter the current correlation

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):391–395. Mastoris et al. 392 www.ijcasereportsandimages.com between coronary anomalies, sudden cardiac death and Case 3: A 63-year-old female with an extensive the immediate need for surgical correction. cardiac history including childhood myocardial infarctions, congestive heart failure, and a presumed diagnosis of endocardial fibroelastosis presented with CASE SERIES left shoulder pain radiating to her upper back. She underwent a nuclear stress test, which was abnormal, Case 1: A 48-year-old Caribbean male was admitted to and an echocardiogram, which showed decreased left the hospital after experiencing chest pain and progressive ventricular systolic function with heavy calcification shortness of breath over a three-month period. Initial ECG of the endocardium in the apical lateral, anterolateral illustrated left ventricular hypertrophy with ST-T wave and inferolateral segments. The patient had subsequent changes compatible with repolarization abnormality. cardiac CTA, which showed the presence of ALCAPA This patient had an abnormal adenosine myocardial with a scarred anterior and anterolateral wall resulting perfusion imaging study demonstrating a moderate in hypokinesis (Figure 4). Moderate central mitral perfusion abnormality in the inferobasal wall, which regurgitation was also identified. Although surgical prompted a cardiac catheterization. Catheterization intervention was initially recommended, the patient verified a dilated left coronary system in the absence of declined at the time of diagnosis. She is currently being the right coronary artery ostium. Subsequent imaging managed in collaboration with an adult congenital heart with a cardiac MRI confirmed ARCAPA (Figure 1) disease specialist who also recommended closure of the along with a hyperdynamic left ventricle and severe left anomalous left coronary artery through catheterization ventricular hypertrophy. The right ventricle was within to prevent the steal phenomenon. The patient however normal limits for contractility and no ischemic scars were remains satisfied with her overall level of functioning and noted. Three months later, the patient underwent surgical symptoms and has declined interventions. She continues correction to successfully restore a bi-coronary arterial to be monitored for cardiac arrhythmias. circulatory system (Figure 2). Intraoperative findings were consistent with ARCAPA however significant right ventricular dysfunction and distension, particularly at DISCUSSION the intersection of the pulmonary artery, was now noted. The right coronary artery measured 8 mm in diameter An anomalous origin of the coronary artery from the with a great deal of collaterals surrounding the heart on pulmonary artery (ACAPA) presenting into adulthood the epicardial surface. The surgery consisted of dissecting remains a scarce diagnosis as over 90% of patients die the right coronary artery from the pulmonary artery and within their first year of life [1, 2]. During fetal life, the re-implanting onto the aorta. The patient was discharged pulmonary arterial pressure equals the systemic pressure home after a normal postoperative course. His anginal allowing for antegrade flow into the anomalous coronary chest pain completely resolved however some residual artery and perfusion of the myocardium. After birth, shortness of breath continued for the first few months postoperatively. Case 2: A 49-year-old female, suffering from recurrent bilateral breast cancer and awaiting bilateral mastectomies, experienced right-sided chest pain that radiated to her axilla and right arm. Nuclear stress test demonstrated normal diaphragmatic attenuation artifact with no obvious signs of ischemia. Given the magnitude of the surgery planned, a cardiac catheterization was performed and displayed a very large, right coronary artery and a left main artery, which could not be cannulated. A cardiac CTA identified an ALCAPA and a diffuse ectatic coronary vessel with extensive collateral vessels throughout the myocardium and communicate between the right coronary artery and the left coronary artery (Figure 3). Echocardiogram evaluated normal regional wall motion of both the left and right ventricles with normal systolic function. Given the extensive collateral vasculature it was felt that the patient was not at an increased risk for surgery. She underwent the planned bilateral mastectomies successfully without any cardiac complications. Currently, the patient is being managed by a cardiologist; no surgical correction is planned at this Figure 1: Cardiac magnetic resonance imaging (MRI) in the time. sagittal plane illustrating the ARCAPA.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):391–395. Mastoris et al. 393 www.ijcasereportsandimages.com

Figure 2: Intraoperative visualization of severely dilated coronary artery vessels in ARCAPA patient prior to successful surgical restoration of bi-coronary artery system.

Figure 4: Cardiac CTA in the axial plain illustrating the enlarged coronary artery arising from the pulmonary artery trunk.

artery compared to the left [3, 4]. ARCAPA’s overall incidence is estimated to be 0. 002% compared to 0. 008% for ALCAPA [3]. With advances in technology, the modality of diagnosis has changed over time from autopsy to surgery to catheterization and now angiography [5, 6]. These modalities have led to an increased number of reported adult cases of ACAPA. Many have postulated that the actual prevalence in adult population may be higher than reported and therefore the true association between sudden death and ALCAPA/ARCAPA is lower than described [2]. Historically, due to its association with myocardial infarct, heart failure, ventricular arrhythmias and sudden Figure 3: Contrast-enhanced coronary computed tomography cardiac death, the standard treatment has been surgical volume rendered image of the entire heart demonstrating the severely dilated coronary artery vessels in our patient with correction [2, 7]. Limited data is available regarding the ALCAPA. most effective surgical strategy, however, establishment of a dual coronary artery system with coronary transfer is preferred even if interposition graft is required [8]. Traditionally, plans for surgical correction before the however, the pressure in the pulmonary artery decreases onset of left ventricular function was favored. Owing to and retrograde flow into the anomalous coronary artery the possible lower association between sudden death and ensues. This is known as coronary steal phenomenon and ACAPA in adults, the decision for a challenging surgical results in myocardial ischemia, infarction and sudden intervention becomes more complex. Recent literature cardiac death [2]. has suggested that if mild to moderate chronic ischemia The proposed mechanism of both ARCAPA and and limited necrosis is present, survival without surgical ALCAPA survival into adulthood involves significant correction is possible [2]. There is even less evidence collateral circulation to the myocardium originally based recommendations for the medical management supplied by the anomalous vessel. ARCAPA has more of ALCAPA and ARCAPA. Theoretically, optimizing risk commonly been reported in adults and thought to be factors for acute coronary syndrome and preserving less fatal in infancy than ALCAPA due to the smaller collateral flow should delay the onset of the coronary perfusion distribution supplied by the right coronary steal phenomenon.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):391–395. Mastoris et al. 394 www.ijcasereportsandimages.com

Data remains limited on adult manifestations of Copyright ALCAPA and ARCAPA and its true association between © 2015 Katie L. Mastoris et al. This article is distributed sudden cardiac death, progression to severe cardiac under the terms of Creative Commons Attribution pathology, and treatment modalities. Our cases represent License which permits unrestricted use, distribution three symptomatic adult manifestations of ACAPA; only and reproduction in any medium provided the original one of which underwent recommend surgical correction author(s) and original publisher are properly credited. to restore a bi-coronary arterial circulatory system. Please see the copyright policy on the journal website for The latter two preferred monitoring and referral to an more information. adult congenital specialist for further guidance and management. REFERENCES

CONCLUSION 1. Selzman CH, Zimmerman MA, Campbell DN. ALCAPA in an adult with preserved left Currently, there are no strong evidence based ventricular function. J Card Surg 2003 Jan- recommendations for management of adult patient’s Feb;18(1):25–8. with anomalous origin of the coronary artery from 2. Peña E, Nguyen ET, Merchant N, Dennie G. the pulmonary artery (ACAPA) and strong collateral ALCAPA syndrome: not just a pediatric disease. circulation due to the low prevalence of the anomaly. Radiographics 2009 Mar-Apr;29(2):553–65. Our hope is that with advances in diagnostic modalities 3. Williams IA, Gersony WM, Hellenbrand WE. and an increase in reported ACAPA cases, more data will Anomalous right coronary artery arising from become available to support the decision of surgical or the pulmonary artery: a report of 7 cases and medical management. a review of the literature. Am Heart J 2006 Nov;152(5):1004.e9–17. ********* 4. Modi H, Ariyachaipanich A, Dia M. Anomalous origin of right coronary artery from pulmonary Acknowledgements artery and severe mitral regurgitation due to Anil Gupta M. D- the referring provider from Pocono myxomatous mitral valve disease: a case report Medical Center, Elizabeth Adams D. O. – ADCH specialist and literature review. J Invasive Cardiol 2010 at Hershey Apr;22(4):E49–55. 5. Hundley WG, Bluemke DA, Finn PJ, et al. Author Contributions ACCF/ACR/AHA/NASCI/SCMR 2010 expert Katie L. Mastoris – Substantial contributions to consensus document on cardiovascular magnetic conception and design, Acquisition of data, Analysis resonance: a report of the American College of and interpretation of data, Drafting the article, Revising Cardiology Foundation Task Force on Expert it critically for important intellectual content, Final Consensus Documents. J Am Coll Cardiol 2010 approval of the version to be published Jun 8;55(23):2614–62. Ataul Qureshi – Analysis and interpretation of data, 6. Su JT, Krishnamurthy R, Chung T, Vick GW Revising it critically for important intellectual content, 3rd, Kovalchin JP. Anomalous right coronary Final approval of the version to be published artery from the pulmonary artery: noninvasive Navin K. Subrayappa – Analysis and interpretation of diagnosis and serial evaluation. J Cardiovasc data, Revising it critically for important intellectual Magn Reson 2007;9(1):57–61. content, Final approval of the version to be published 7. Wilson CL, Dlabal PW, Holeyfield RW, Akins Matthew W. Martinez – Analysis and interpretation CW, Knauf DG. Anomalous origin of left of data, Revising it critically for important intellectual coronary artery from pulmonary artery. Case content, Final approval of the version to be published report and review of literature concerning teen- James Wu – Analysis and interpretation of data, Revising agers and adults. J Thorac Cardiovasc Surg 1977 it critically for important intellectual content, Final Jun;73(6):887–93. approval of the version to be published 8. Rajbanshi BG, Burkhart HM, Schaff HV, Daly RC, Phillips SD, Dearani JA. Surgical strategies Guarantor for anomalous origin of coronary artery from The corresponding author is the guarantor of submission. pulmonary artery in adults. J Thorac Cardiovasc Surg 2014 Jul;148(1):220–4. Conflict of Interest Authors declare no conflict of interest.

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CASECase REPORTReport Peer Reviewed OPEN| OPEN ACCESS ACCESS An unusual association of von Hippel–Lindau disease and posterior nutcracker syndrome: Management of neurological and urological aspects in a young male

Nadhir Karmani, Faouzi Mallat, Wissem Hmida, Khaled Ben Ahmed, Oussama Karmani, Sidiya Oueld Chavey, Amel Ben Abdallah, Faouzi Mosbah, Hedi Krifa

Abstract Conclusion: The complex presentation of the VHL disease must not makesome other serious Introduction: Von Hippel–Lindau (VHL) disease problems easily missed or neglected like renal is a complex and systemic entity that can be carcinoma or nutcracker syndrome as seen in discovered by neurological complications. Its our case. association with other serious malformative disease like nutcracker syndrome (NCS) was not Keywords: Hemangioblastoma, Nutcracker syn- described, and its complex presentation must not drome, Renal carcinomas, Total removal, Von make some other serious problem easily missed Hippel–Lindau (VHL) disease or neglected. Case Report: A 27-year-old male patient with 3 intramedullary hemangioblastoma How to cite this article of six months of insidious evolution complicated by medullary compression. The lesions were Karmani N, Mallat F, Hmida W, Ahmed KB, Karmani completely removed with excellent neurological O, Chavey SO, Abdallah AB, Mosbah F, Krifa H. An postoperatory outcome. Thinking of VHL unusual association of von Hippel–Lindau disease disease, our investigations were expanded and posterior nutcracker syndrome: Management of and revealed multiple vermian and cerebellar neurological and urological aspects in a young male. hemangioblastomas, renal masses, and multiple Int J Case Rep Images 2015;6(7):396–402. pancreatic cysts. In addition, an incidentally found of posterior NCS during the abdominal doi:10.5348/ijcri-201565-CR-10526 computed tomography (CT) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal INTRODUCTION carcinomas) and for the missed symptomatic posterior nutcracker syndrome were discussed. Hemangioblastoma is a rare tumour of the central nervous system characterised by a high vascularization. It 1 2 2 Nadhir Karmani , Faouzi Mallat , Wissem Hmida , Khaled accounts for 1.5–2.5% of spinal cord tumors. In the case Ben Ahmed2, Oussama Karmani3, Sidiya Oueld Chavey3, of von Hippel–Lindau (VHL) disease, hemangioblastoma Amel Ben Abdallah3, Faouzi Mosbah2, Hedi Krifa1 occurs in 20–30% of patients and is often multiple [1, 2]. Affiliations: 1Neurosurgery Department, SahloulHospital, Surgical experience is often limited because of the rarity Sousse, Tunisia; 2Urology Department, SahloulHospital, Sousse, Tunisia; 3Radiology Department, SahloulHospital, of this tumor. Microsurgical removal is recommended Sousse, Tunisia.Email ID: [email protected] in symptomatic patients or in cases with tumor growth Corresponding Author: Dr. NadhirKarmani, Department of during follow-up [3, 4]. Neurosurgery, Hospital of Sahloul, Sousse, Tunisia; Tel: The association of VHL disease with other serious 0021697885092; Email: [email protected] malformative disease like nutcracker syndrome was not described. Herein, we present the case of young male having complicated presentation VHL disease with an Received: 14 September 2014 Accepted: 16 February 2015 incidentally radiological found of a missed symptomatic Published: 01 July 2015 posterior nutcracker syndrome.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):396–402. Karmani et al. 397 www.ijcasereportsandimages.com

CASE REPORT Based on this clinical presentation and these radiological and biologic finding, a diagnosis of posterior A 27-year-old male was hospitalized at the nutcracker syndrome was confirmed. neurosurgery department because of signs of medullary In summary, we have a young patient with both serious compression with incomplete tetraplegia especially in the presentation of VHL disease with multiple neurological right lower limb, appearing insidiously for six months locations of hemangioblastoma complicated by that did not ameliorate. medullary compression due to spinal hemangioblastoma The patient denied any bladder or bowel dysfunction and right renal carcinomas. Our patient had also missed or problems with handwriting, or fine motor skills. symptomatic posterior nutcracker syndrome. An MRI of the spine showed multiple intramedullary masses. The symptomatic lesions was situated in the terminal cone; the largest mass measured 48×13×1O mm in size. The mass appeared as a cystic lesion with a tissural mural nodule, with hypointense T1 inhomogeneous signal, with increased gadolinium uptake in T1, with perilesional oedema. Just above this mass, there are two other small cystic lesions, up to 1/3 medium of the vertebral body T6; one of the nodules had a subdural extra axial component (Figures 1 and 2). Based on the characteristics on the MRI, a diagnosis of multiple hemangioblastoma was favored; and thinking of von Hippel–Lindau (VHL) disease, our investigations were expanded: The patient underwent an MRI of the complete neuro-axis including brain and spinal cord showed a vermian hemangioblastoma associated with 2 small right cerebellar hemangioblastomas (Figure 3). The abdominal CT scan revealed 2 small renal masses, less than 2 cm in size; with multiple pancreatic cysts (Figure 4); without liver lesions, or adrenal masses. An ophthalmologic examination demonstrated none of the stigmatisms Figure 1: Magnetic resonance imaging scan of the spine showing associated with VHL. Physical examination did not show multiple intramedullary masses. The symptomatic lesions was any café au lait spots. situated in the terminal cone (red arrow); the largest mass At this first step and on neurological level, a diagnosis measured 48×13×10 mm in size. The mass appeared as a of medullary compression as a complication of multiple cystic lesion with a tissural mural nodule, with hypointense T1 intramedullary hemangioblastoma in a young male with inhomogeneous signal, with increased gadolinium uptake in T1. VHL disease was done. At the second step, and as an incidentally finding, the abdominal computed tomography (CT) scan done for VHL disease investigations, revealed a vascular compression: left renal vein passing posterior to the aorta, entrapped between the aorta and the vertebral column. Left gonadic vein, drained to the second left renal vein, was dilated with multiple pelvic venous collaterals. These aspects were indicative of posterior NCS (Figures 5). Interviewing the patient after discovering the vascular compression (posterior NCS), he complained of intermittent and various symptoms: urinary (intermittent macroscopic hematuria, intermittent left flank pain and chronic pelvic pain aggravated by physical activity) and systemic signs dominated by chronic fatigue and persistent headache. All these symptoms lasted for his childhood; without definitive diagnosis, despite a long investigational history of several imaging examinations and laboratory tests. In addition, high degree of left varicocele was noted at physical examination; laboratory tests revealed anemia at 10.0 mg/dl, microscopic hematuria, and 24 hour urine Figure 2: Magnetic resonance imaging scan of the spine (axial) showing the three symptomatic intramedullary masses was collection analysis showed elevated proteinuria. situated in the terminal cone.

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Figure 3: Magnetic resonance imaging scan of the brain showing a vermian hemangioblastoma associated with 2 small right cerebellar hemangioblastomas.

Figure 4: The abdominal computed tomography scan revealing two small renal masses (yellow arrow), less than 2 cm in size; with multiple pancreatic cysts (red arrow).

Figure 6: Intraoperative view of a intramedullary hemangioblastoma in the spinal cord (A) Before the duralinsision, (B) After the duralinsision, and (C) After complete resection.

mobilization and kinetotherapy was done to prevent deep venous thrombosis. Preoperative deficits were gradually ameliorated after the operation. Figure 5: Computed tomography showing compression of the The MRI scan of the cervical spine performed 8 days left renal vein (red arrow) between abdominal aorta (yellow after the operation confirmed the total ablation of the 3 arrow) and vertebral column with dilatation of the spermatic tumors and the patient was discharged from the hospital vein (blue arrow). one week later. With 11 months of follow-up, the patient is doing well, and was neurologically cured. Last MRI of the Prior to surgical intervention, the patient was complete neuro-axis including brain and spinal cord counseled on the risks entailed in the removal of a spinal showed same aspects of the vermian and the cerebellar cord tumor. hemangioblastoma, without recurrence or appearance After urinary catheter insertion and administration of of spinal masses.The patient remained neurologically a steroid-type anti-inflammatory (dexamethasone) and asymptomatic and had returned to his usual life. cephalosporin antibiotics, surgical exploration was made On the urinary and vascular level, our attitudes were: through a prone position, and a large median incision. Concerning the renal masses: tumorectomie was The dissection in the subdural space and especially technically impossible; and right nephrectomy was done. the dissection of the tumor were performed using an Macroscopic examination revealed multiple small tissular operating microscope. masses (more than 3) and histopathological examination The inspection of the medullary surface showed 3 confirmed the diagnosis of clear renal cell carcinoma. exteriorised masses red, bloody and very well vascularized Concerning the posterior nutcracker syndrome, (Figure 6). our attitude was conservative, only the varicocele was After careful and complete dissection, the tumors treated and medical treatment of anemia was done. were totally ablated (Figure 6), and their vascular pedicle Transposition of the left renal vein was proposed in case of being coagulated and sectioned at the end. severe complication. At the last follow-up, the varicocele Final histopathological examination confirmed the improved significantly, but intermittent hematuria diagnosis of hemangioblastoma. persisted. Laboratory tests revealed hemoglobin at Postoperative course was uneventful; There were 12.0 mg/dl, with persistent microscopic hematuria and no postoperative complications; the drainage tube was proteinuria. The last abdominal CT-scan showed no removed 48 hours after the operation and precocious recurrence of renal mass in the left kidney.

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DISCUSSION hematoma, meningocele or cerebrospinal fluid fistula and arachnoiditis. Postlaminectomy cervical kyphosis Hemangioblastoma are rare tumors of the central is more common with children and it can be prevented nervous system characterised by a high vascularization. by performing laminoplasty or laminotomy [6]. The they account for 1.5–2.5% of spinal cord tumors [1]. They postoperative course of our present case was not eventful. appear spontaneously in most cases; and only 30% of The follow-up was based on both clinical and MRI them were associated with von Hippel–Lindau disease examinations to detect tumor recurrence every six months and are often multiple [2, 3]. after the operation, or in case of multiple localization to Von Hippel–Lindau disease is a rare autosomal evaluate tumor progress. The neurological follow-up was dominant genetic syndrome, characterised by the growth evaluated according to the classification of McCormick of retinal angiomas, pheochromocytoma, polycythemia, [8]. brain and spinal cord hemangioblastoma, renal and In our case and on the neurosurgical level, we are pancreatic carcinomas [5]. In our case, hemangioblastoma faced to two serious problems: of the brain and spinal cord, multiple renal carcinomas The first Problem was the three intramedullary and pancreatic cysts were found. hemangioblastoma complicated by medullary More than the half of spinal hemangioblastomas are compression: Our attitude was an urgent total ablation. accompanied by a syrinx [5]. In our case syrinx was not The second problem is non-complicated small vermian noted. and cerebellar hemangioblastoma, that our attitude was The initial symptoms of spinal hemangioblastoma are conservative, with strict and regular follow-up. radicular pain, sensory or motor deficit slowly progressing At the last follow-up, the patient remained according to the level of medulla involvement as seen in neurologically stable. our case that was complicated by medullary compression. Our patient had in addition to the neurological MRI has become the diagnostic tool of choice and presentation, urological presentation of the VHL disease is recommended by several authors in the diagnosis of as right multiple carcinomas and had also symptomatic hemangioblastoma; it is also helpful in preoperative nutcracker syndrome. planning and the differential diagnosis of spinal cord Posterior nutcracker syndrome results from neoplasms and vascular lesions [6, 7]. compression of the left renal vein between the aorta On unenhanced T1-weighted images and vertebra, with subsequent development of venous hemangioblastoma appears as an isointense. On varicosities of the renal pelvis, ureter, and the gonadal T2-weighted images, it appears hyperintense with vein [9, 10]. The true prevalence of nutcracker syndrome a homogeneous intense enhancement. Larger especially the posterior form remains unknown and it hemangioblastoma can appear as a hypointense or mixed might be underdiagnosed and easily delayed [11], as seen hypo-isointense on T1, heterogeneous on T2, and shows in our patient despite several imaging and laboratory an intense heterogeneous enhancement [6]. In addition, investigations. cystic components of the hemangioblastoma can be The computed tomography angiography (CTA) is the associated with a non-enhancing rim of the cyst. procedure of choice of the diagnosis with high sensitivity During surgery and because of the risk of massive and specificity [10, 11]. bleeding, intramedullary hemangioblastoma surgery The management of the NCS is controversial and the pose severe problems [3]. invasive procedures should be considered only when Indications for treatment and timing of surgery of symptoms are severe or persistent, including severe hemangioblastoma are still unclear because of the rarity of pain, severe hematuria, renal insufficiency, and failure to these tumors and the relatively limited surgical experience. respond to conservative treatment [9, 10]. Most of the recently published series recommend Follow-up, conservative approach and surgical microsurgical removal in symptomatic patients or in cases therapy are the different treatment options proposed with tumor growth during follow-up [3, 4]. for this syndrome. The available surgical procedure of Using the aforementioned principles of microsurgical the PNCS is the transposition of the LRV. Other options removal of spinal hemangioblastoma, microsurgical were described including intra-vascular or extravascular removal is feasible with a low procedure-related stents and renal auto transplantation. The indications morbidity and can be recommended, especially in VHL for laparoscopic surgery continue to augment for the patients who were symptomatic or with tumor growth treatment of the nutcracker syndrome [12, 13]. during follow-up. In this case, we are faced to several urological Occlusion of the feeding arteries, shrinking of the problems: tumor by bipolar coagulation with low power and its en The first we have multiple right renal tumors < 2 bloc removal are the key points for a safe microsurgical cm provided to be clear cell carcinomas in the context treatment. It is essential that the ablation must be done of a serious and complicated form of the VHL disease;: en bloc. conservative attitude with strict and regular follow-up The most common complications that arise with of right renal parenchyma and the left kidney was our such lesions are: intramedullary hematoma, epidural proposed procedure of choice but because the lack of

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):396–402. Karmani et al. 400 www.ijcasereportsandimages.com the ablative techniques in our country (radiofrequency Copyright and cryoablation) and the intraoperative difficulties of © 2015 Nadhir Karmani et al. This article is distributed multiple tumorectomie, a nephrectomy was made. under the terms of Creative Commons Attribution The second problem was in the left kidney: posterior License which permits unrestricted use, distribution nutcracker syndrome, our attitude was conservative, and reproduction in any medium provided the original only the varicocele was treated and medical treatment of author(s) and original publisher are properly credited. anemia was done; transposition of the LRV was proposed Please see the copyright policy on the journal website for in case of severe complications. more information.

CONCLUSION REFERENCES

Von Hippel–Lindau (VHL) disease and nutcracker 1. Isu T, Abe H, Iwasaki Y, Akino M, et al. Diagnosis and syndrome are rare and severe entities. There association surgical treatment of spinal hemangioblastoma. No was not described. The complex presentation of the VHL Shinkei Geka 1991 Feb;19(2):149–55. disease must not make some other serious problems easily 2. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas missed or neglected like renal carcinoma or nutcracker of the central nervous system. A 10-year study with syndrome as seen in our case. special reference to von Hippel-Lindau syndrome. J Neurosurg 1989 Jan;70(1):24–30. ********* 3. Malis LI. Atraumatic bloodless removal of intramedullary hemangioblastomas of the spinal Author Contributions cord. J Neurosurg 2002 Jul;97(1 Suppl):1–6. Nadhir Karmani – Substantial contributions to 4. Samii M, Klekamp J. Surgical results of 100 conception and design, Acquisition of data, Analysis intramedullary tumors in relation to accompanying and interpretation of data, Drafting the article, Revising syringomyelia. Neurosurgery 1994 Nov;35(5):865– 73. it critically for important intellectual content, Final 5. Rengachary SS, Blount JP. Hemangioblastomas. In: approval of the version to be published Wilkins RH, Rengachary SS (eds) Neurosurgery. Faouzi Mallat – Analysis and interpretation of data, McGraw-Hill, New York; 1996. pp. 1205–19. Revising it critically for important intellectual content, 6. Chu BC, Terae S, Hida K, Furukawa M, Abe S, Final approval of the version to be published Miyasaka K. MR findings in spinal hemangioblastoma: Wissem Hmida – Analysis and interpretation of data, correlation with symptoms and with angiographic Revising it critically for important intellectual content, and surgical findings. AJNR Am J Neuroradiol 2001 Final approval of the version to be published Jan;22(1):206–17. Khaled Ben Ahmed – Analysis and interpretation of data, 7. Seifert V, Trost HA, Stolke D. Microsurgery of spinal angioblastoma. Neurochirurgia (Stuttg) 1990 Revising it critically for important intellectual content, Jul;33(4):100–5. Final approval of the version to be published 8. McCormick PC, Torres R, Post KD, Stein BM. Oussama Karmani – Analysis and interpretation of data, Intramedullary ependymoma of the spinal cord. J Revising it critically for important intellectual content, Neurosurg 1990 Apr;72(4):523–32. Final approval of the version to be published 9. Kurklinsky AK, Rooke TW. Nutcracker phenomenon Sidiya Oueld Chavey – Analysis and interpretation of and nutcracker syndrome. Mayo Clin Proc 2010 data, Revising it critically for important intellectual Jun;85(6):552–9. content, Final approval of the version to be published 10. Wendel RG, Crawford ED, Hehman KN. The Amel Ben Abdallah – Analysis and interpretation of data, “nutcracker” phenomenon: an unusual cause for renal varicosities with hematuria. J Urol 1980 Revising it critically for important intellectual content, May;123(5):761–3. Final approval of the version to be published 11. Mallat F, Hmida W, Jaidane M, Mama N, Mosbah F. Faouzi Mosbah – Analysis and interpretation of data, Nutcracker syndrome complicated by left renal vein Revising it critically for important intellectual content, thrombosis. Case Rep Urol 2013;2013:168057. Final approval of the version to be published 12. Xu D, Liu Y, Gao Y, et al. Management of renal Hedi Krifa – Analysis and interpretation of data, Revising nutcracker syndrome by retroperitoneal laparoscopic it critically for important intellectual content, Final nephrectomy with ex vivo autograft repair and approval of the version to be published autotransplantation: a case report and review of the literature. J Med Case Rep 2009 Oct 27;3:82. 13. Zhang Q, Zhang Y, Lou S, Liu F, Ye Z, Zhang Guarantor D. Laparoscopic extravascular renal vein stent The corresponding author is the guarantor of submission. placement for nutcracker syndrome. J Endourol 2010 Oct;24(10):1631–5. Conflict of Interest Authors declare no conflict of interest.

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About the Authors

Article citation: Karmani N, Mallat F, Hmida W, Ahmed KB, Karmani O, Chavey SO, Abdallah AB, Mosbah F, Krifa H. An unusual association of von Hippel Lindau disease and posterior nutcracker syndrome: Management of neurological and urological aspects in a young male. Int J Case Rep Images 2015;6(7):396–402.

Nadhir Karmani is Resident at Department of Neurosurgery Sahloul Sousse Tunisia, Faculty of Medecine Sousse Tunisia, and Department of Neurosurgery Angers France, Faculty of Medecine Angers France. He earned undergraduate degree neurosurgery doctor from Faculty of Medecine Sousse Tunisia and Postgraduate Degree Form Department of Neurosurgery Angers, Faculty of Medecine Angers France.

Faouzi Mallat is working in Urology Department, Sahloul Hospital, Sousse, Tunisia

Wissem Hmida is working in Urology Department, Sahloul Hospital, Sousse, Tunisia

Khaled Ben Ahmed is working in Urology Department, Sahloul Hospital, Sousse, Tunisia

Oussama Karmani is working in Radiology Department, Sahloul Hospital, Sousse, Tunisia

Sidiya Oueld Chavey is working in Radiology Department, Sahloul Hospital, Sousse, Tunisia

Amel Ben Abdallah is working in Radiology Department, SahloulHospital, Sousse, Tunisia

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):396–402. Karmani et al. 402 www.ijcasereportsandimages.com

Faouzi Mosbah is working in Urology Department, Sahloul Hospital, Sousse, Tunisia

Hedi Krifa is working in Neurosurgery Department, Sahloul Hospital, Sousse, Tunisia

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS 1,25-Dihydroxyvitamin D-mediated hypercalcemia in a patient with malignancy without lymphoma

Katie Mastoris, Sarah Park, Vasudev Magaji

Abstract How to cite this article

Introduction: Hypercalcemia resulting from Mastoris K, Park S, Magaji V. 1,25-Dihydroxyvitamin calcium homeostasis imbalance is seen in D-mediated hypercalcemia in a patient with one-third of malignancies. Solid malignancies malignancy without lymphoma. Int J Case Rep cause hypercalcemia from high parathyroid Images 2015;6(7):403–406. hormone-related peptide (PTHrP) and, infrequently, from osteolytic cytokines. Excessive 1, 25-dihydroxyvitamin D, seen mostly doi:10.5348/ijcri-201566-CR-10527 in lymphoma, causes hypercalcemia by 1-alpha- hydroxylation of 25-hydroxyvitamin D. This mechanism has also been reported in a few cases of dysgerminoma, myofibroblastic tumors, and gastrointestinal stromal tumors. Case Report: We INTRODUCTION present an unusual case of 1,25-dihydroxyvitamin Hypercalcemia is one of the most common endocrine D mediated hypercalcemia in a 62-year-old male and electrolyte derangements seen in malignancies. with colonic adenocarcinoma and mesothelioma. Paraneoplastic mechanisms leading to hypercalcemia Conclusion: This case emphasizes that elevated include tumor production of parathyroid hormone- 1,25-dihydroxyvitamin D may be a hypercalcemia related peptide (PTHrP), osteolytic bone lesions, etiology in non-lymphoma malignancies and ectopic parathyroid hormone (PTH) production, or should to be considered when workup for elevated 1,25 dihydroxyvitamin D [1,25(OH) D] levels elevated PTHrP and osteolytic hypercalcemia is 2 through activation of an extrarenal 1-alpha-hydroxylase. negative. Hypercalcemia from elevated 1,25(OH)2D is commonly seen in patients with lymphoma [1]. We report a case of Keywords: Adenocarcinoma, 1-alpha-hydroxy- 1,25(OH) D mediated hypercalcemia in a patient with 2 lase, 1,25-dihydroxyvitamin D, Hypercalcemia, 2 primary malignancies: adenocarcinoma of the rectum Mesothelioma and epitheloid mesothelioma.

Katie Mastoris1, Sarah Park1, Vasudev Magaji2 CASE REPORT Affiliations: 1D.O, Department of Medicine, Lehigh Valley Health Network, Allentown PA; 2MD, Division of Endocrinology, A 62-year-old male presented to his primary Department of Medicine, Lehigh Valley Health Network, care physician’s office with one-month duration of Allentown PA. difficulty moving bowels, bloating, and abdominal pain. Corresponding Author: Vasudev Magaji, MD, Division of Abdominal computed tomography (CT) scan revealed Endocrinology, Department of Medicine, Lehigh Valley Health rectal thickening concerning for malignancy with Network, Allentown PA 18103; Ph: 610-402-6790; Fax: 610- metastatic lymphadenopathy and a right upper quadrant 402-6979; Email: [email protected] 20 cm mass centered in the left lobe of the liver. Biopsy of sigmoid colon showed invasive moderately differentiated Received: 22 January 2015 adenocarcinoma (Figure 1). Pre-colonoscopy laboratory Accepted: 20 March 2015 evaluation at this visit demonstrated an elevated calcium Published: 01 July 2015 level (Table 1). Five days following colonoscopy, the

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):403–406. Mastoris et al. 404 www.ijcasereportsandimages.com patient presented to the emergency department with right lower extremity cellulitis leading to hospitalization and septicemia. Due to concern for liver abscess, the liver mass was biopsied. Pathology showed high- grade epithelioid mesothelioma (Figure 2). During the hospitalization, his calcium levels rose (Table 1) and workup for the etiology of hypercalcemia began (Table 2). There was no evidence of osteolytic lesions on imaging, serum and urine electrophoresis were negative, and thyroid-stimulating hormone was within normal limits. There was also no evidence of granulomatous disease or evidence of granulomatous infection on imaging studies. Bisphosphonate administration brought the patient’s calcium level to within normal range (Table 1). His hospital course continued due to septicemia and renal

Figure 2: Liver core biopsy showing high grade malignant neoplasm consistent with epitheloid mesothelioma. Immunoperoxidase stain for inhibin, CAM 5.2, CK7, calretinin, WT1 were positive. This malignant neoplasm is histologically distinct from the patient’s rectal adenocarcinoma and represents a synchronous secondary primary neoplasm.

failure requiring dialysis. He was eventually transferred intensive care unit because of hypotension and septic shock from Escherichia coli bacteremia secondary to right leg cellulitis. Despite being on 3 vasopressors, antibiotics, and continuous renal replacement therapy, his condition deteriorated and patient died on comfort measures.

DISCUSSION Figure 1: Sigmoid biopsy in formalin showing invasive moderately differentiated adenocarcinoma. Depth of Hypercalcemia resulting from calcium homeostasis involvement could not be determined. imbalance is seen in one-third of malignancies and

Table 1: Patient’s laboratory values at noted intervals, compared with normal range values. Normal Presentation Hospitalization Administration of Range July 10 October 22 October 31 bisphosphonate Serum calcium, mg/dL 8.2-10.4 9.8 10.5 11.9 7.0

Corrected calcium, mg/dL 8.2-10.4 - - 12.4 7.0

Serum albumin, g/dL 3.5-5.0 4.6 4.2 3.4 1.9

Ionized calcium, mg/dL 4.6-5.4 - - 6.45 - Serum phosphorus, mg/dL 2.5-4.5 - - - 8.1 eGFR, mL/min/1.73m2 >60 96 46 36 14 Serum BUN, mg/dl 10-26 19 29 79 89 Serum creatinine, mg/dL 0.7-1.5 0.79 1.59 1.95 4.31 Abbreviations: eGFR, estimated glomerular filtration rate; BUN, blood urea nitrogen

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):403–406. Mastoris et al. 405 www.ijcasereportsandimages.com

Table 2: Workup for etiology of hypercalcemia transiently responded to tyrosine kinase inhibitor and bisphosphonates [5]. Normal Patient Patient range levels levels post Our case demonstrates the rare occurrence of pamidronate 1,25(OH)2D-mediated hypercalcemia in a patient with mesothelioma and colon cancer. Moreover, it shows Vitamin D, 25 hydroxy 30–100 46 34 total, two different malignancies possibly associated with this ng/mL mechanism. There has only been one prior reported case of pleural mesothelioma associated with hypercalcemia Vitamin D, 18–64 95 77 and 1,25(OH) D elevation [4]. Interestingly, there is no 1,25-dihydroxy, 2 pg/mL adenomatous colon cancer case report of 1,25(OH)2D- mediated hypercalcemia despite ex vivo upregulation Intact PTH, 14–72 12 - of mRNA encoding for 1-alpha-hydroxylase being pg/mL described in colon cancer [6, 7]. Due to our patient’s PTHrP <2 1.1 - underlying septicemia and deterioration, we were unable pmol/L to explore which malignancy caused the elevated levels of Abbreviations: PTH, parathyroid hormone; PTHrP, 1,25(OH) D. parathyroid hormone-related peptide 2 commonly occurs through increased synthesis of CONCLUSION parathyroid hormone-related peptide (PTHrP) or metastatic osteolytic bone lesions. Our patient’s elevated We recommend considering further investigation in patients with hypercalcemia when workup for elevated calcium level occurred through excessive 1,25(OH)2D levels as evident by normal PTHrP, lack of osteolytic PTHrP and osteolytic hypercalcemia is negative, as lesions, low PTH and elevated 1,25(OH) D. Elevated elevated 1,25(OH)2D could be a hypercalcemia etiology in 2 non-lymphoma malignancies. 1,25(OH)2D results in hypercalcemia through increased calcium absorption in the gastrointestinal tract and by promoting osteoclast differentiation and increased ********* activity. Author Contributions Elevated 1,25(OH)2D mediated hypercalcemia has been established in granulomatous diseases and Katie Mastoris – Substantial contributions to lymphomas. This mechanism occurs through elevated conception and design, Acquisition of data, Analysis extra renal hydroxylation of 25-hydroxyvitamin D. and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final Normally, regulation of 1,25(OH)2D levels occurs in the proximal convoluted tubules of the kidney through approval of the version to be published hydroxylation of 25-hydroxyvitamin D by the 1-alpha- Sarah Park – Substantial contribution to design, hydroxylase enzyme. Regulation of this enzyme by the acquisition of data, Analysis and interpretation of data, parathyroid hormone (PTH) and calcitriol results in Drafting the article, Final approval of the version to be normal calcium levels. Extra-renal 1-alpha-hydroxylase, published however, is insensitive to normal feedback mechanisms, Vasudev Magaji – Substantial contribution to design, which leads to uncontrolled hypercalcemia. Extra-renal acquisition of data, Analysis and interpretation of data, 1-alpha-hydroxylase has been established in tumor Revising the article critically for important intellectual adjacent tissue macrophages in lymphoma patients content, Final approval of the version to be published [1] with very few cases reporting extra-renal 1-alpha Guarantor hydroxylase or elevation of 1,25(OH)2D as the cause of hypercalcemia in other malignancies. The corresponding author is the guarantor of submission. Among these neoplasms, dysgerminomas of the ovary leading to hypercalcemia have been reported [2]. Conflict of Interest Resection of the dysgerminoma resulted in normalization Authors declare no conflict of interest. of serum calcium and 1,25(OH)2D. Similarly, one patient with pleural mesothelioma had increased Copyright © 2015 Katie Mastoris et al. This article is distributed calcium levels due to elevated 1,25(OH)2D which again normalized after resection [3]. A case of inflammatory under the terms of Creative Commons Attribution myofibroblastic tumor on surgical pathology associated License which permits unrestricted use, distribution and reproduction in any medium provided the original with hypercalcemia and 1,25(OH)2D elevation [4] showed abundant messenger RNA (mRNA) encoding author(s) and original publisher are properly credited. for 1-alpha-hydroxylase in the tumoral tissue. While Please see the copyright policy on the journal website for in a case of gastrointestinal stromal tumor with more information.

1,25(OH)2D-associated hypercalcemia, the patient

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REFERENCES D and hypercalcaemia in a patient with inflammatory myofibroblastic tumour. J Clin Pathol 2003 1. Hewison M, Kantorovich V, Liker HR, et al. Vitamin Apr;56(4):310–2. D-mediated hypercalcemia in lymphoma: evidence for 5. Jasti P, Lakhani VT, Woodworth A, Dahir KM. hormone production by tumor-adjacent macrophages. Hypercalcemia secondary to gastrointestinal stromal J Bone Miner Res 2003 Mar;18(3):579–82. tumors: parathyroid hormone-related protein 2. Evans KN, Taylor H, Zehnder D, et al. Increased independent mechanism? Endocr Pract 2013 Nov- expression of 25-hydroxyvitamin D-1alpha- Dec;19(6):e158–62. hydroxylase in dysgerminomas: a novel form of 6. Ogunkolade BW, Boucher BJ, Fairclough PD, et humoral hypercalcemia of malignancy. Am J Pathol al. Expression of 25-hydroxyvitamin D-1-alpha- 2004 Sep;165(3):807–13. hydroxylase mRNA in individuals with colorectal 3. Lee JM, Pou K, Sadow PM, et al. Vitamin cancer. Lancet 2002 May 25;359(9320):1831–2. D-mediated hypercalcemia and Cushing syndrome 7. Tangpricha V, Flanagan JN, Whitlatch LW, et al. as manifestation of malignant pleural mesothelioma. 25-hydroxyvitamin D-1alpha-hydroxylase in normal Endocr Pract 2008 Nov;14(8):1011–6. and malignant colon tissue. Lancet 2001 May 4. Ogose A, Kawashima H, Morita O, Hotta T, Umezu 26;357(9269):1673–4. H, Endo N. Increase in serum 1,25-dihydroxyvitamin

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS A case report of an uncommon large size of prostatic cyst

Jingjin Yang, Xingkai Liu, Yong Zhang

Abstract Keywords: Prostatic cyst, Prostatic hyperplasia, Perianal incision Introduction: Prostatic cyst is an uncommon disease, especially large one, which need to How to cite this article be differentiated from prostatic neoplastic masses, prostatic non-neoplastic masses and Yang J, Liu X, Zhang Y. A case report of an uncommon periprostatic neoplastic masses. Case Report: A large size of prostatic cyst. Int J Case Rep Images 63-year-old male who had dysuria for three years 2015;6(7):407–410. and could not urinate for three days. Initially, he was diagnosed as prostatic hyperplasia by color doi:10.5348/ijcri-201567-CR-10528 Doppler ultrasound. After further examinations with prostate-specific antigen (PSA), computed tomography (CT) scan, magnetic resonance imaging (MRI) scan, cystoscopy and transrectal ultrasound-guided aspiration, he was diagnosed INTRODUCTION as prostatic cyst. The size of prostatic cyst was 9.2x4.9 cm. Two weeks after diagnosis patient Prostatic cyst is a rare disease with uncertain origin received an open surgery through perianal [1–4], which needs to be differentiated from prostatic incision to remove all content in the cyst and neoplastic masses, prostatic non neoplastic masses, clean this large prostatic cyst. Patient is still seen periprostatic neoplastic masses, periprostatic neoplastic for follow-up visits every two months to further masses and cyst of seminal vesicle. Most of prostatic cysts assess the treatment. Conclusion: Ultrasound, are small and located laterally without spermatozoa [5– CT scan and MRI scan together help to get 8]. To make a correct diagnosis it is needed to perform final diagnosis of prostatic cyst. The surgery of clinical test, ultrasound, computed tomography (CT), prostate cyst excision we performed improved magnetic resonance imaging (MRI) and tissue biopsy. All outcome although we still need to follow our these procedures can help to understand non-neoplasia patient for longer time. or neoplasia, location, size, margins and its contents. Prostatic cysts include utricular and mullerian duct cysts, Jingjin Yang1, Xingkai Liu2, Yong Zhang2 diverticula of the ampulla, cysts of the ejaculatory duct, Affiliations: 1(MD, Chief of Department),Department of retention cyst of the prostate, hemorrhagic prostatic cyst Urology, The 463th Hospital of PLA, 46 Xiao He Yan Road, and cyst associated with prostatitis [6, 8, 9]. Shenyang, Liaoning, China; 2(MD, a doctor), Department of We noted that only very few cases have been reported Urology, The 463thHospital of PLA, 46 Xiao He Yan Road, worldwide. Herein, we reported an uncommon size Shenyang, Liaoning, China. of prostatic cyst, which finally diagnosed through Corresponding Author: Dr. Jingjin Yang, Department of all procedures of rectal examination, clinical test, Urology, The 463th Hospital of PLA, 46 Xiao He Yan Road, ultrasound, CT, MRI and transrectal ultrasound-guided Shenyang, Liaoning 110042, China; Tel: 86-3322445090; aspiration of prostatic cyst, and successfully performed Email: [email protected] an open surgery.

Received: 27 February 2015 Accepted: 22 April 2015 Published: 01 July 2015

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CASE REPORT

A 63-year-old male without urination for three days was admitted to the hospital. The patient had suffered from dysuria, frequent urination, a delay in starting urination, a weak of slow urinary stream, a feeling of incomplete bladder emptying and getting up frequently at night to urinate (4–6 times/night) for three years. He was diagnosed as prostatic hyperplasia by abdominal color Doppler ultrasound without any treatment at his local hospital before coming to our hospital. All his general physical examinations were normal except rectal examination. His rectal examination showed that his prostate gland was grade II prostate enlargement, the central sulcus swallowed, both sides symmetry increase, firm and smooth without nodules, absence of tenderness, feeling a cystic lesion (~4 cm) above dentate line without clear boundary and pain, and no blood on the glove. Color Doppler ultrasound showed Figure 1: Images of computer tomography (CT) scan and 4.2 x 4.9 x 3.9 cm of enlarged prostate with clear outline, magnetic resonance imaging (MRI) scan. (A, B) computed rough internal echo and multiple bright echos. tomography (CT) scan which showing that a 4.5 x 4.2 cm of To further confirm the diagnosis, the patient received even hypodense shadow is behind prostate gland and pushes PSA test, CT scan, MRI scan and transrectal ultrasound- prostate gland to front left. The prostate gland is enlarged with guided aspiration of prostatic cyst. Patient’s PSA test was smooth boundary. (C, D) MRI scan showing there is irregularly negative. CT scan demonstrated that there was a 4.5 x a long T1 and T2 signals (9.2 x 4.9 cm) near the right peripheral 4.2 cm of even hypodense shadow behind prostate gland leaves and penis cavernous part which extended to perineum and rectum. resulting in pushing prostate gland to front left. Patient’s prostate gland was enlarged with smooth boundary (Figure 1A–B). MRI scan showed there were irregularly a long T1 and T2 signals (9.2 x 4.9 cm) near the right cyst, an uncommon large size of the prostatic cyst. The peripheral leaves and penis cavernous part which result from transrectal ultrasound-guided aspiration extended to perineum and rectum (Figure 1C–D). Figure of prostatic cyst further confirmed that the patient was 1 showing that surrounding tissues were compressed and suffered from the prostatic cyst, not prostate cancer and displaced. We performed transrectal ultrasound-guided prostatic hyperplasia. aspiration of prostatic cyst and collected 15 ml of viscous In general, treatments of prostatic cysts include milky fluid, which contained many epithelial cells and transurethral resection, endoscopic marsupialization, no sperm and no cancer cells. Bacterial cultures were endoscopic urethrotomy and incision, transrectal negative. ultrasound-guided drainage, and open surgery [4, 11]. Two week after diagnosing, we performed an open It suggests that invasive procedures are best avoided surgery through perianal incision to get rid of all content [2]. After patient was diagnosed, we had difficult time in patient’s prostatic cyst, clean this cyst and try to to decide which treatment would be benefit to the remove all cyst lining although this surgery was not very patient because we did not have good experience about easy. Currently, we follow-up this patient after every two the uncommon large size of prostatic cyst. Finally, we months to monitor patient’s condition and further assess performed an open surgery through perianal incision to our surgery treatment. remove all content in the cyst, clean this large prostatic cyst and try to remove all cyst lining. We hope that the bladder and pelvic-cavity-pressure will push the prostatic DISCUSSION cyst and eventually close dead space of the prostatic cyst. After surgery, all infravesical obstructive symptoms were Patient was diagnosed as prostatic hyperplasia in disappeared. Patient is still seen for follow-up visits every the local hospital. After admitting to our hospital, we two months in order to monitor patient’s condition and repeated abdominal color Doppler ultrasound plus CT further evaluate the effect of our treatment. scan. The results indicated that the patient could have the prostatic cyst. The negative PSA indicated that the patient did not have prostate cancer. A previous study CONCLUSION suggests that MR imaging is useful in demonstrating the liquid content of prostatic [10]. Further MRI scan indeed Ultrasound, computed tomography (CT) scan and showed clear image of prostatic cyst, and was consistent magnetic resonance imaging (MRI) scan together help with CT scan. The patient was diagnosed as prostatic

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):407–410. Yang et al. 409 www.ijcasereportsandimages.com to get final diagnosis of prostatic cyst. The surgery of 5. Magri J. Cysts of the prostatic gland. Br J Urol 1960 prostate cyst excision we performed improves outcome Sep;32:295–96. although we still need to follow our patient for longer 6. Rieser C, Griffin Tl. Cysts of the Prostate. J Urol 1964 time. Mar;91:282–6. 7. Fischelovitch J, Meiraz D, Lazebnik J. Cysts of the prostrate. Br J Urol 1975 Dec;47(6):687–9. ********* 8. Kirkali Z, Yigitbasi O, Diren B, Hekimoglu B, Ersoy H. Cysts of the prostate, seminal vesicles and Acknowledgements diverticulum of the ejaculatory ducts. Eur Urol We would like to thank Dr. Changyu Zheng (NIDCR, 1991;20(1):77–80. NIH, USA) for manuscript editing assistance. 9. Nayyar R, Wadhwa P, Dogra PN. Midline intraprostatic cyst: An unusual cause of lower urinary Author Contributions tract symptoms. Indian J Urol 2008 Jan;24(1):109– Jingjin Yang – Substantial contributions to conception 11. 10. Gevenois PA, Van Sinoy ML, Sintzoff SA Jr, Cysts and design, Acquisition of data, Revising it critically for of the prostate and seminal vesicles: MR imaging important intellectual content, Final approval of the findings in 11 cases. AJR Am J Roentgenol 1990 version to be published Nov;155(5):1021–4. Xingkai Liu – Substantial contributions to conception and 11. Jarow JP. Diagnosis and management of ejaculatory design, Acquisition of data, Analysis and interpretation duct obstruction. Tech Urol 1996 Summer;2(2):79–85. of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Yong Zhang – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Guarantor The corresponding author is the guarantor of submission.

Conflict of Interest Authors declare no conflict of interest.

Copyright © 2015 Jingjin Yang et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.

REFERENCES

1. Chou YH, Jang MY, Huang CH. Ultrasound detection of prostate cyst. J Med Ultrasound 1996 Jan;4 (1):43– 45. 2. Singh CH, Rastogi MV. Prostate cysts. Med J Armed Forced India 2000;56:359–60. 3. Zhang HH, Qi F, Wang J, Chen MF, Li Z, Zu Xb. Midline prostatic cysts presenting with chronic prostatitis or secondary infertility and minimally invasive treatment: Endoscopic laparoscopic approach? Surg Sci 2011 Jul;2:285–89. 4. Dogan B, Canda AE, Akbulut Z, Atmaca AF, Duran E, Balbay MD. Prostatic cyst causing severe infravesical obstruction in a young patient. Urol J 2011 Fall;8(4):330–2.

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Coincident retroperitoneal and sigmoid colon liposarcoma: A rare occurrence

Ayvaz Ulaş Urgancı, Erkan Oymacı, Enver Vardar, Ebru Akıncılar, Ömer Engin

Abstract doi:10.5348/ijcri-201568-CR-10529

Introduction: Retroperitoneal liposarcomas are rare malignancies. There are a limited number of liposarcoma cases in gastrointestinal system in literature. There is no known etiological INTRODUCTION factor in the pathogenesis of liposarcomas yet. The treatment is total resection. Case Report: A Soft tissue sarcomas are less than 1% among all types 59-year-old male patient detected synchronous of cancer [1]. One-third of malignant tumors originating liposarcoma both in retroperitoneum and in from retroperitoneum are sarcomas and liposarcomas colon and treated with en block resection. We are the most frequently observed retroperitoneal sarcoma discussed our case with review of literature. types [2]. Fifteen percent of adult soft tissue sarcomas Conclusion: We think that even though it could are located in retroperitoneum. Forty-one percent of not be detected in preoperative examinations, retroperitoneal sarcomas are liposarcomas and malignant this rare togetherness should be considered tumors with mesenchymal origin [2]. Moreover, intraoperatively. liposarcomas with colon origin are quite rare and there are limited numbers of cases reported in literature [3]. Keywords: Colon liposarcoma, Gastric liposar- There is no known etiological factor in the pathogenesis coma, Retroperitoneal liposarcoma of liposarcomas yet. There are five types of liposarcomas: well differentiated, myxoid, round cell, pleomorphic, and How to cite this article undifferentiated, out of these types pleomorphic and undifferentiated types have poorer prognosis due to high Urganci AU, Oymaci E, Vardar E, Akincilar E, recurrence and metastasis rates [1, 4]. Engin Ö. Coincident retroperitoneal and sigmoid Retroperitoneal liposarcomas usually grows slowly colon liposarcoma: A rare occurrence. Int J Case Rep without manifesting any symptoms, and they form a Images 2015;6(7):411–415. gross abdominal mass [1, 2]. Their treatments consist of en block R0 (absence of residual tumor) resection. Adjuvant treatment modalities are controversial [2, 5]. 1 2 3 Ayvaz Ulaş Urgancı , Erkan Oymacı , Enver Vardar , Ebru The complication risks of these tumors may be reduced 1 1 Akıncılar , Ömer Engin by multidisciplinary approach, careful diagnosis and Affiliations: 1MD, Department of General Surgery, İzmir treatment approach; and survival rates and life qualities 2 Buca Goverrment Hospital, Turkey; MD, Department of of the patients may be improved. In this study, we aimed Gastroenterology Surgery, İzmir Bozyaka Training and to present a case of rare retroperitoneal sarcoma and Reserch Hospital, Turkey; 3As. Prof. and Clinic Chief, synchronous colon liposarcoma. Department of Pathology, İzmir Bozyaka Training and Reserch Hospital, Turkey. Corresponding Author: Ayvaz Ulaş Urgancı, 121 Street No:7/13 Evka3 Bornova İzmir, Turkey; Tel: 0 5056491127; CASE REPORT Email: [email protected] A 59-year-old male patient admitted after he realized a swelling in his abdomen with asthenia, fatigue and Received: 25 January 2015 abdominal pain complaints for the last few months. In Accepted: 25 February 2015 the physical examination, vital findings were normal. Published: 01 July 2015 In palpation, a mass was detected filling lower left

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):411–415. Urgancı et al. 412 www.ijcasereportsandimages.com quadrant, and extending to the right of midline and umbilicus. In laboratory examinations, white blood cell (WBC):15.400/mm3, Albumin:2.1 mg/dl and HCV RNA (+). Carcinoembryonic antigen (CEA), CA 15-3, CA 19- 9, CA 125 were found to be within normal ranges. In magnetic resonance imaging (MRI) scan, a mass lesion with the size of 25x19 cm, filling the lower half of abdomen, having cystic necrotic areas internally. The mass was well circumscribed and pushed the intestinal segments and mesenteric tissues towards periphery (Figure 1). No finding indicating metastatic disease was found. The patient was taken for operation after preoperative preparation. An approximately 30x30 cm mass originating from left retroperitoneum, pushing sigmoid colon and intestinal segments to the upper right abdominal quadrant was detected in the operation. The mass had no organ invasion but left testicular vessels and left ductus deferens could not be prepared. The mass was excised totally (Figure 2). Afterwards in the exploration, a second lesion, approximately 20x20 cm in mass, that was originating from sigmoid colon wall was Figure 1: Magnetic resonance imaging scan of abdomen. detected (Figure 2). It was excised with Wedge resection. Both excised masses were reported to be pleomorphic liposarcoma in the pathological examination of the piece (Figure 3–5). CD-34 was detected to be (+), CD- 68 was (-) in pleomorphic cells, S-100(-), actin was (-), desmin was (-) in the mass originating from sigmoid colon. Actin was found to be (-), S-100 was (-), and CD- 68 was (-) in pleomorphic cells in retroperitoneal mass. Surgical margins of the resected pieces were reported to be negative. Postoperative period of the patient was problem-free. The patient was mobilized on the 1st postoperative day. Bowel movements were started on the 2nd postoperative day, and oral food was given to the patient on the 3rd postoperative day. Abdominal drains were removed in 6th and 7th postoperative days. The patient was discharged on the 9th postoperative day. The patient was discussed at tumor committee. Figure 2: Retroperitoneal and sigmoid colon liposarcomas. Follow-up without any additional treatment was decided. No findings indicating metastasis or recurrence were detected in the three months follow-up of the patient.

DISCUSSION

Most frequent intra-abdominal mesenchymal tumors are liposarcomas. They usually manifest with non- specific abdominal pain and abdominal mass [2]. The patients may usually have mild abdominal symptoms, weight loss and usually they are generally diagnosed in late period. In our case, the present symptoms were recognized in later period and the patient was admitted with a big mass. Rarely, neurological findings due to mass pressure, may accompany [6]. Metastasis may be detected in approximately 11% of the patients and metastases are frequently occur in lungs and liver [6]. Despite admitting Figure 3: Normocellular and focal oedematous areas several in later period, no distant metastases were detected in the hyperchromatic nuclei were seen in low magnification (H&E patient. stain, x200).

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cell and pleomorphic based on their cytogenic and morphologic anomalies [1, 4]. Well differentiated and myxoid liposarcomas are seen much more frequently among primary tumors, and their prognoses are better and metastasis rates are lower than other types. On the other hand, dedifferentiated liposarcomas are seen more frequently in recurrent tumors. The rates of dedifferentiated or pleomorphic subtypes, are higher in recurrent tumors than primary ones [2]. Very frequent local recurrence (66%) and organ invasion were the leading mortality causes of the disease [2, 5, 9]. In wide series, five-year disease-free survival after en-block resection was given as 18% [10]. Recommend treatment is re-operation in recurrent cases. De-bulking and radiotherapy may be recommended in cases for which R0 resection cannot be performed [2, 9]. Figure 4: Rare lipoblast-like cells were seen in high magnification A second mass was detected in intra-operative (arrow-head) (H&E stain, x400). observation in our case despite it could not be recognized in preoperative examinations. Liposarcomas are rarely seen in gastrointestinal system. While there are liposarcoma cases detected as multiple foci in retroperitoneum in literature, we did not encounter any case who had synchronous liposarcoma in gastrointestinal system and retroperitoneum. Recurrence is an important criterion for survival in these patients, and the probability of synchronous tumor must also be considered.

CONCLUSION

In conclusion, while retroperitoneal liposarcomas are rarely seen as single tumors, synchronous colon liposarcoma occurring together is very rare in the literature. We think that even though it could not be detected in preoperative examinations, this rare Figure 5: Pleomorphic liposarcomatous areas were stained togetherness should be considered intraoperatively. negative contrary to the positivity of peripheral nerve in left side of the image (S100, x100). *********

Author Contributions Abdominal computed tomography (CT) scan and Ayvaz Ulaş Urgancı – Substantial contributions to MRI scan may show the association of the mass with conception and design, Acquisition of data, Analysis adjacent organs and vascular structures, and may and interpretation of data, Drafting the article, Revising provide information about histological type. There are it critically for important intellectual content, Final publications recommending abdominal CT scan or MRI approval of the version to be published scan as ideal method [6–8]. In our case, the structure Erkan Oymacı – Substantial contributions to of mass and its association with surrounding tissues conception and design, Acquisition of data, Analysis and adjacent organs were assessed in detail before the and interpretation of data, Drafting the article, Revising operation with MRI. it critically for important intellectual content, Final In our day, the only treatment for retroperitoneal approval of the version to be published liposarcomas is en bloc R0 resection [2, 5, 6, 9]. There Enver Vardar – Analysis andinterpretation of data, is not enough evidence regarding radiotherapy and Drafting the article, Revising it critically for important chemotherapy modalities [2, 5, 6, 9]. The most important intellectual content, Final approval of the version to be prognostic factors are R0 resection and tumor histology published [3, 9]. Multifocality is a rare but important prognostic Ebru Akıncılar – Acquisition of data, Drafting the article, parameter [6]. Final approval of the version to be published Liposarcomas are assessed in five sub-groups such Ömer Engin – Acquisition of data, Drafting the article, as well differentiated, dedifferentiated, myxoid, round Final approval of the version to be published

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Guarantor pregnancy. Obstet Gynecol Sci 2014 May;57(3):236– The corresponding author is the guarantor of submission. 9. 5. Bursics A, Besznyák I, Mersich T, et al. Surgical Conflict of Interest treatment of retroperitoneal sarcomas. Magy Onkol 2014 Mar;58(1):47–51. Authors declare no conflict of interest. 6. Theodosopoulos T, Dellaportas D, Psychogiou V, Yiallourou A, Polymeneas G, Gkiokas G. Multifocal Copyright retroperitoneal sarcoma. Case Rep Surg 2013 May 2. © 2015 Ayvaz Ulaş Urgancı et al. This article is 7. Song T, Shen J, Liang BL, Mai WW, Li Y, Guo HC. distributed under the terms of Creative Commons Retroperitoneal liposarcoma: MR characteristics and Attribution License which permits unrestricted use, pathological correlative analysis. Abdom Imaging distribution and reproduction in any medium provided 2007 Sep-Oct;32(5):668–74. the original author(s) and original publisher are properly 8. Hekimoglu K. Giant retroperitoneal liposarcomas: Diagnostic approach with multidetector computed credited. Please see the copyright policy on the journal tomography and magnetic resonance imaging. JBR- website for more information. BTR 2013 Nov-Dec;96(6):375–7. 9. Joshi RM, Gangurde GK, Talathi NP, et al. Large retroperitoneal liposarcoma - a series of five cases. REFERENCES Indian J Surg 2013 Jun;75(Suppl 1):64–8. 10. Anaya DA, Lahat G, Liu J, et al. Multifocality 1. Shahaji C, Amit P, Prashant P, Sachin T. Giant in retroperitoneal sarcoma: A prognostic factor retroperitoneal liposarcoma: A case report. Case Rep critical to surgical decision-making. Ann Surg 2009 Oncol Med 2012;2012:869409. Jan;249(1):137–42. 2. Lu W, Lau J, Xu MD, et al. Recurrent abdominal liposarcoma: Analysis of 19 cases and prognostic factors. World J Gastroenterol 2013 Jul 7;19(25):4045–52. 3. Türkoglu MA, Elpek GÖ, Dogru V, Calis H, Uçar A, Arici C. An unusual case of primary colonic dedifferentiated liposarcoma. Int J Surg Case Rep 2014;5(1):8–11. 4. Oh SE, Kim HJ, Choi SJ, Oh SY, Roh CR, Kim JH. A case of huge retroperitoneal liposarcoma in

About the Authors

Article citation: Urganci AU, Oymaci E, Vardar E, Akincilar E, Engin Ö. Coincident retroperitoneal and sigmoid colon liposarcoma: A rare occurrence. Int J Case Rep Images 2015;6(7):411–415.

Ayvaz Ulaş Urgancı is working in General surgery Department at Buca Seyfi Demirsoy Goverment Hospital in İzmir-Turkey. He earned undergraduate degree from İzmir Ege University Hospital Turkey and postgraduate degree (general surgery) from İzmir Atatürk Research and Training Hospital-Turkey. He has published 15 research papers in national and international academic journals. His research interests include gastrointestinal surgery, laparoscopic surgery, colorectal surgery. E-mail: [email protected]

Erkan Oymacı is working in Gastrointestinal Surgery at Bozyaka Research and Training Hospital in İzmir-Turkey. He earned the undergraduate degree from İzmir Ege University Hospital Turkey and postgraduate degree in Gastrointestinal Surgery from TYIH Research and Trainin Hospital Ankara- Turkey. He has published 40 research papers in national and international academic journals. His research interests include gastrointestinal surgery, laparoscopic surgery, colorectal surgery. E-mail: [email protected]

Enver Vardar is working in Patolhogy Derpartment at Bozyaka Research and Training Hospital in İzmir-Turkey. He has published 40 research papers in national and international academic journals. His research interests include gastrointestinal pathology.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):411–415. Urgancı et al. 415 www.ijcasereportsandimages.com

Ebru Akıncılar is working in General surgery Department at Buca Seyfi Demirsoy Goverment Hospital in İzmir-Turkey. She earned undergraduate degree from 9 September University Hospital İzmir-Turkey and postgraduate degree form general surgery from İzmir Atatürk Research and Training Hospital-Turkey She has published 15 research papers in national and international academic journals. Her research interests include Colorectal Surgery, Breast Surgery.

Ömer Engin is working in General surgery Department at Buca Seyfi Demirsoy Goverment Hospital in İzmir-Turkey. He has published 40 research papers in national and international academic journals. His research interests include Gastrointestinal Surgery, Colorectal Surgery.

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Scrub typhus vasculitis causing pan-digital gangrene

Suja Lakshmanan, Krishnamoorthy Seetharaman, Ramakrishnan S.R., Sathiyan Sivanandam, Koushik A.K.

Abstract typhus as evidenced by the presence of eschar and positive serology. Patient recovered with Introduction: Scrub typhus is a mite- ventilatory support and antibiotics though her borne infectious disease caused by Orientia digits could not be salvaged which was managed tsutsugamushi. Farmers account for conservatively. Conclusion: While going through approximately two-thirds of all reported cases. literature we observed that there were no case Incidence rates are highest in people aged 40 reports of gangrene caused by scrub typhus. Pan- to 60 years. The severity of infection can range digital gangrene is an unusual complication of from mild symptoms and signs to multi-organ scrub typhus. Hence scrub typhus has to be ruled failure. The chief target of the organism is out in patients presenting with this complication. the vascular endothelium of various organs. There is inflammation in and around the blood Keywords: Pan-digital gangrene, Scrub typhus, vessels that causes endothelial damage resulting Vasculitis in vascular leakage and organ dysfunction. Case Report: A 62-year-old female, farmer by How to cite this article occupation who presented to us with features of acute respiratory distress syndrome (ARDS) and Suja L, Krishnamoorthy S, Sathiyan S, Sivanandam meningitis, subsequently developed gangrene S, Koushik AK. Scrub typhus vasculitis causing pan of digits of all four limbs. We investigated the digital gangrene Int J Case Rep Images 2015;6(7):416– patient for infectious and non-infectious causes 421. of vasculitis leading onto gangrene. We attributed the cause for pan-digital gangrene as scrub doi:10.5348/ijcri-201569-CR-10530

Suja Lakshmanan1, Krishnamoorthy Seetharaman1, Ramakrishnan S.R.2, Sathiyan Sivanandam3, Koushik A.K.4 Affiliations: 1MD Gen Med,Assistant Professor, Department of Medicine, Sri Ramachandra Medical College and Research INTRODUCTION Institute, Chennai, Tamil Nadu, India; 2MD Gen Med, Professor, Department of Medicine, Sri Ramachandra Medical College 3 Orientia tsutsugamushi is an obligate intracellular and Research Institute, Chennai, Tamil Nadu, India; DM gram-negative coccobacillus which is the etiologic agent Nephrology, Assistant Professor, Department of Nephrology, Sri RamachandraMedical College and Research Institute, for scrub typhus. There are three strains of Orientia Chennai, Tamil Nadu, India; 4MD Gen Med, Post Graduate, tsutsugamushi namely Karp, Gilliam, and Kato strains. Department of Medicine, Sri Ramachandra Medical College Infection with one strain does not confer immunity from and Research Institute, Chennai, TamilNadu, India. infection with another strain. Scrub typhus is endemic in Corresponding Author: Suja Lakshmanan, 40/23 Kamdar India, China, Korea, Pakistan, Taiwan, Japan, Thailand, Nagar 1st Street, Mahalingapuram, Nungambakkam, Malaysia, and Northern Australia. The vector for scrub Chennai, Tamil Nadu 600034, India; Ph: +91-9940135101; typhus are larval trombiculid mites, also called chiggers Email: suja. lakshmanan@gmail. com [1]. After an incubation period of 7–10 days, the disease Received: 31 December 2014 may start with prodromal symptoms like headache, Accepted: 28 January 2015 anorexia, malaise with fever [2]. Rash and characteristic Published: 01 July 2015 eschar may develop subsequently. The infection can have

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):416–421. Lakshmanan et al. 417 www.ijcasereportsandimages.com varying presentation. Some have mild symptoms, while Table 1: Laboratory investigations of the patient. others can develop multiorgan failure. Approximately, Hemoglobin 9. 6 g/dl 4% of patients hospitalized have fatal infection [3]. Elderly patients are more likely to have severe illness MCV 87. 9 fl and complications. Some patients develop a localized MCH 29. 7 pg necrotic skin lesion (eschar) at the site of their infecting Total Count 13900 cells/mm3 chigger bite. Differential Count P70 L28 E1 M1 Generalized lymphadenopathy, acute kidney injury Peripheral Smear Toxic neutrophilic granules with and respiratory complaints are often present. Rarely, acute shift to left respiratory distress syndrome may occur. Involvement of blood vessels in the central nervous system may produce Platelet counts 1. 50 lakhs / mm3 meningitis or encephalitis. Herein, we present a patient Serum creatinine 1. 5 mg/dl who developed severe respiratory failure due to acute Serum sodium 141 meq/l respiratory distress syndrome requiring ventilatory Total bilirubin 0. 98 mg/dl support and pan-digital gangrene due to scrub typhus. ALT 10 U/L AST 42 U/L CASE REPORT Alkaline phosphatase 42 U/L Serum albumin 2 g/dl A 62-year-old female, farmer by occupation presented Electrocardiogram. Normal to the emergency department with history of high grade fever and chills for three days. She was managed with Abbreviations: antipyretics in outside hospital with no benefit. She MCV mean corpuscular volume developed breathlessness at rest for 1 day. Hence she was MCH mean corpuscular hemoglobin referred to our institution for further management. On ALT alanine transaminase examination, she was febrile (101°F) and tachypneic with AST aspartate amino transferase a respiratory rate of 40 per minute. Her pulse rate was 106 per minute and her blood pressure was 110/80 mmHg. Table 2: Etiological workup for fever, ARDS and altered She had an eschar in her right loin. Her respiratory sensorium. examination revealed bilateral extensive crepitations. QBC – MP NEGATIVE Her jugular venous pressure was normal and she was clinically euvolemic. We proceeded with the following MALARIAL ANTIGEN Negative investigations as given in Table 1. RT-PCR FOR H1N1: throat swab Negative Arterial blood gas revealed type 2 respiratory failure SEROLOGY FOR SCRUB TYPHUS Positive (pH 7.04, pCO2 7.5 mmHg, PO2 57 mmHg). In view of progressive breathlessness despite supportive measures IGM ANTILEPTOSIRAL ANTIBODY Negative she was intubated and connected to a ventilator. Chest IGM DENGUE ANTIBODY Negative X-ray showed features of consolidation (Figure 1) which was subsequently confirmed by CT scan of thorax (Figure HIV Negative 2). Anti HbsAg Negative The patient developed altered sensorium subsequently. CT scan of brain was normal. Cerebrospinal Anti HCV Negative fluid (CSF) analysis showed sugar of 72 mg/dl, protein 67 mg/dl, 140 WBC/hpf, neutrophils 65% and lymphocytes Blood culture No Growth 35%. CSF culture, gram staining, HSV serology done were Urine culture No Growth negative. Etiologic workup for fever, ARDS, and altered sensorium were done (Table 2). CSF culture No Growth Patient was initially managed with broad spectrum NON BAL culture No Growth antibiotics – imipenem plus cilastatin and linezolid. After serology report of scrub typhus being positive she Echocardiogram No evidence of IE , EF 64% was started on doxycycline 100 mg BD and rifampicin 600 mg OD. On day-3 of admission patient developed Abbreviations: blackish discoloration of the terminal digits of upper QBC-MP quantitative buffy coat-malaria parasite and lower limbs which was progressive in nature and RT-PCR real time polymerase chain reaction ultimately developed into dry gangrene (Figures 3 and HIV Human Immunodeficiency Virus 4) over 4–5 days. However, all her peripheral pulses Anti HBsAg anti hepatitis B surface antigen ANTI HCV anti hepatitis C virus were felt normally. Vasculitis workup like ANA, dsDNA, Non-BAL non-bronchoalveolar lavage antiphospholipid antibody, C-ANCA, P-ANCA, Anti

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):416–421. Lakshmanan et al. 418 www.ijcasereportsandimages.com centromere antibody were negative. Vascular surgery opinion was sought and the patient was started on LMWX and aspirin. Biopsy of the lesion was not attempted as the patient’s relatives did not give consent. After eight days of doxycycline therapy patient showed gradual improvement in clinical condition and was weaned off from ventilator subsequently. Patient developed severe pain in her upper and lower limb digits around the gangrenous areas. She received several analgesics including tramadol, amitriptyline, paracetamol, NSAIDS with partial relief of pain. She was started on cilostazol, aspirin, pentoxifylline by vascular surgeon after cessation of LMWH. She eventually developed auto amputation of her right lower limb second and third toes followed by second toe of left lower limb and little finger of her right hand over the next 2–3 months.

Figure 3: Dry gangrene affecting all digits of both hands.

Figure 1: Chest X-ray anterior-posterior view showing bilateral ground glass opacities.

Figure 4: Gangrene involving digits of all four limbs.

DISCUSSION

Scrub typhus infections may present as mild illness or can lead onto fatal complications like ARDS, meningoencephalitis, acute kidney injury, myocarditis, hepatic dysfunction and multiorgan involvement. Figure 2: Computed tomography scan of thorax plain: Patchy Complications usually occur after the first week of illness. consolidation with air bronchogram, ground glass opacities Due to non specific clinical features of scrub typhus it is seen to involve bilateral lung fields suggestive interstitial is grossly under diagnosed, though it is an growing and pneumonia.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):416–421. Lakshmanan et al. 419 www.ijcasereportsandimages.com emerging disease. Since scrub typhus can lead to life While looking into the infective causes as in our threatening illness, initiation of treatment should be case, the diagnosis of scrub typhus should be made by early and prompt based on clinical suspicion, which later excluding other viral infectious diseases like dengue, should be confirmed by serology. infectious mononucleosis, HIV and bacterial diseases like O. tsutsugamushi proliferates in the endothelium typhoid, leptospirosis, meningococcal disease causing of small blood vessels that results in cytokine release. ARDS and vasculitis. These infections were excluded This cytokine causes endothelial damage leading with appropriate investigations. As serology for scrub to leakage of fluid and aggregation of platelets, and typhus done by ELISA was positive, we thought about the proliferation of polymorph and monocytes leading to association of scrub typhus as the cause of vasculitis. focal microinfarction. This eventually leads to venous The clinical features of this 62-year-old female thrombosis and peripheral gangrene. Various organs like who presented with fever, breathlessness and altered skin, kidneys, skeletal muscles, brain lungs, and cardiac sensorium who responded to treatment with doxycycline muscles are predominantly affected by this process [4, 5]. are consistent with scrub typhus. This is confirmed by the O. tsutsugamushi infection spreads to multiple organs presence of eschar and positive scrub typhus serology. through blood stream and lymphatics. It predominately We eliminated all other differentials for scrub typhus targets the macrophages present in the spleen and liver with multi-organ involvement. The patient subsequently [6]. The Rickettsia attacks the endothelial cells causing developed pan digital gangrene. We excluded other inflammation by a mechanism called oxidative stress infective and non infective diseases causing vasculitis. leading onto local and systemic vasculitis [7]. Despite Hence we conclude that the patient developed pan-digital the fact that disseminated endothelial infection occurs in gangrene secondary to scrub typhus vasculitis. To our scrub typhus, the real incidence of vasculitis leading to knowledge this is the first reported case of pan-digital vaso-occlusion is quite rare which is the highlight of our gangrene in scrub typhus infection. case. Systemic vasculitis causing gangrene can occur due to both infectious and non infectious cause. Among CONCLUSION the infectious cause viruses are the leading organisms that lead to systemic vasculitis. Hepatitis B virus is Although there are so many conditions causing associated with polyarteritis nodosa. Hepatitis C virus vasculitis, it is essential to differentiate infective from related mixed cryoglobulinemia causes vasculitis in only non infective causes as the treatment is entirely different a minority of people who acquired the infection. There . From the case that we have presented here, we learn that are a few other viruses that cause vasculitis like Human pan digital gangrene may be an unusual complication of immunodeficiency virus (HIV), parvo virus B19, varicella- scrub typhus and as many cases have not been reported , zoster virus, human T-cell lymphotropic virus (HTLV)-1 this has to be ruled out in cases of digital gangrene. and cytomegalovirus. Bacteria, fungi or parasites also cause vasculitis ********* which could be by direct invasion of blood vessels or by septic embolization. Syphilitic aortitis or cerebrovascular Author Contributions disease and rickettsial diseases are other, more specific, Suja Lakshmanan – Conception and design, Acquisition bacteria-induced vasculitides. of data, Analysis and interpretation of data,Drafting the The importance of differentiating infectious and non- article, Critical revision of the article and Final approval infectious cause of vasculitis is necessary because the of the version to be published treatment strategies are different. Bacterial, parasitic Krishnamoorthy Seetharaman – Conception and design, and fungal infections are treated with appropriate Acquisition of data, Analysis and interpretation of data, antimicrobial agents. Combination of antiviral drugs and Drafting the article, Critical revision of the article and plasma exchanges are effectively tried for HBV infections Final approval of the version to be published causing polyarteritis nodosa and HIV related vasculitis. Ramakrishnan S.R. – Analysis and interpretation of data, HCV related cryoglobulinemic vasculitis is treated with Critical revision of the article and final approval of the antiviral drugs combined with low dose steroids [8]. version to be published The development of digital gangrene is a sign of Sathiyan Sivanandam – Conception and design, Analysis systemic diseases. Smoking, hypertension, diabetes, and interpretation of data, Drafting the article, and Final obesity and hyperlipidemia are commonly associated approval of the version to be published with digital gangrene. Homocysteinemia, vasculitides like Koushik A.K. – Acquisition of data, Critical revision of the primary systemic vasculitis and medium vessel vasculitis article and Final approval of the version to be published such as polyarteritis nodosa commonly presents with digital gangrene. Rheumatoid arthritis and systemic Guarantor lupus erythematosus cause premature atherosclerosis and The corresponding author is the guarantor of submission. gangrene. Thromboangiitis obliterans (TAO) or Buerger’s disease can cause claudication and digital gangrene [9].

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Conflict of Interest 3. Sonthayanon P, Chierakul W, Wuthiekanun V, et al. Authors declare no conflict of interest. Rapid diagnosis of scrub typhus in rural Thailand using polymerase chain reaction. Am J Trop Med Hyg Copyright 2006 Dec;75(6):1099–102. 4. Cowan G. Rickttsial diseases: The typhus group © 2015 Suja Lakshmanan et al. This article is distributed of fevers--a review. Postgrad Med J 2000 under the terms of Creative Commons Attribution May;76(895):269–72. License which permits unrestricted use, distribution 5. Mahajan SK. Scrub typhus. J Assoc Physicians India and reproduction in any medium provided the original 2005 Nov;53:954-8. author(s) and original publisher are properly credited. 6. Moron CG, Popov VL, Feng HM, Wear D, Walker Please see the copyright policy on the journal website for DH. Identification of the target cells of Orientia more information. tsutsugamushi in human cases of scrub typhus. Mod Pathol 2001 Aug;14(8):752–9. 7. Valbuena G, Walker DH. Changes in the adherens junctions of human endothelial cells infected with REFERENCES spotted fever group Rickettsiae. Virchows Arch 2005 Apr;446(4):379–82. 1. Hornick RB. Rickettsial Diseases. In: Bennett 8. Pagnoux C, Cohen P, Guillevin L. Vasculitides JC, Plum F, editors. Goldman: Cecil Textbook of secondary to infections. Clin Exp Rheumatol 2006 Medicine. 21st ed. Philadelphia, USA. WB Saunders Mar-Apr;24(2 Suppl 41):S71–81. Company 2000. pp. 1911–2. 9. Thachil EJ, Prasad IR, Narsimulu G. Vasculopathies 2. Sonthayanon P, Chierakul W, Wuthiekanun V, et of Peripheral Arterial Disease: Uncommon Etiologies. al. Association of high Orientia tsutsugamushi DNA J Assoc Physicians India 2011 Apr;59:215. loads with disease of greater severity in adults with scrub typhus. J Clin Microbiol 2009 Feb;47(2):430– 4.

About the Authors

Article citation: Suja L, Krishnamoorthy S, Ramakrishnan SR, Sathiyan S, Koushik AK. Scrub typhus vasculitis causing pan digital gangrene Int J Case Rep Images 2015;6(7):416–421.

L. Suja is Assistant Professor in Sri Ramachandra Medical College and Research Institute, Chennai, India. She is interested in the field of autoimmune disorders. E-mail: [email protected]

Krishnamoorthy Seetharaman is Assistant Professor in Sri Ramachandra Medical College and Research institute, Chennai, India. He earned his undergraduate degree from Calicut Medical College, Calicut University, Calicut Kerala, India and postgraduate degree from Stanley Medical College, Tamil Nadu Dr MGR Medical University, Chennai, Tamil Nadu, India. He has published one research paper in a national journal. His interest include detailed evaluation of rare and interesting cases, comprehensive management of each patient and delivering lectures in various forum. E-mail: [email protected]

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):416–421. Lakshmanan et al. 421 www.ijcasereportsandimages.com

Ramakrishnan S.R. is Professor in Sri Ramachandra Medical College and Research institute, Chennai, India. He earned undergraduate and postgraduate degree from Stanley Medical College, Tamil Nadu Dr MGR Medical University, Chennai, Tamil Nadu, India. His research interest is on diabetes mellitus. E-mail: [email protected]

Sathiyan Sivanandam is Assistant Professor in Sri Ramachandra Medical College and Research Institute, Chennai, India. He earned undergraduate degree from PSG medical college, Coimbatore, Tamil Nadu, India and post graduate degree from Madurai Medical College, Tamil Nadu Dr MGR Medical University, Madurai, Tamil Nadu, India. He did his super specialization in nephrology at Madras medical college, Tamil Nadu Dr MGR Medical University, Chennai, Tamil Nadu, India. He is interested in renal transplantation and clinical nephrology E-mail: [email protected]

Koushik A.K. is a postgraduate in Sri Ramachandra Medical College and Research institute, Chennai, India. He has published a couple of case reports in the national journals. E-mail: [email protected]

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Adult nephroblastoma

N. Naqos, Z. Bouchbika, A. Taleb, N. Benchkroune, H. Jouhadi, N. Tawfiq, S. Sahraoui, A. Benider

Abstract How to cite this article

Introduction: Nephroblastoma is rare in adults. Naqos N, Bouchbika Z, Taleb A, Benchkroune It accounts only 0.5% of all renal tumors and it N. Jouhadi H, Tawfiq N, Sahraoui S, Benider A. is often diagnosed in advanced stages. Histology, Adult nephroblastoma. Int J Case Rep Images grading and staging are similar to pediatric 2015;6(7):422–426. nephroblastoma (PN) but its prognosis in the same clinical stage is more pejorative. However some reports use the current pediatric protocols doi:10.5348/ijcri-201570-CR-10531 to treat adult nephroblastoma (AN) taking into consideration tumor stage and grade. Others recommend using advanced disease regimens for all stages and grades and no standardized treatment is defined yet. Case Report: A 29- INTRODUCTION year-old female who was diagnosed with stage IV nephroblastoma. She underwent Wilms tumor (WT) or nephroblastoma, is one of the radical nephrectomy and received multi-agent most frequent malignant renal tumor in children [1], it chemotherapy according to the International is extremely rare in adults, its incidence is 8 per million Society of Pediatric Oncology (SlOP) protocol No. in children [2], whereas its incidence is lower than 0.2 9301, a very important reduction of metastasis per million per year in adults, There is no difference size occurred after three cycles. Conclusion: between histological features of nephroblastoma in Adult nephroblastoma is rare, its prognosis is children and in adults, Treatment guidelines for pediatric poorer than that of children when the disease nephroblastoma are well established but are less clear for is compared stage for stage, but the outcome adults [1–4], in view of the rare occurrence of this tumor for adult patients having this tumor is steadily in adults it is extremely difficult to carry out randomized improving if it is early detected. clinical trials and therapeutic guidelines have not been defined yet. Keywords: Adult, Nephroblastoma, Pediatrics, Current therapies are based on trials and studies Wilms tumor for children conducted by the Société Internationaled’ Oncologie Pédiatrique (SIOP) and the Children’s Oncology Group (COG) [3]. The prognosis for adult was reported to be worse than that for children [1, 2] because N. Naqos, Z. Bouchbika, A. Taleb, N. Benchkroune, H. Jouhadi, N. Tawfiq, S. Sahraoui, A. Benider Adult nephroblastoma has more aggressive behavior and shows worse therapeutic response [2]. In children the Affiliations: 1Mohamed VI Center of Cancer Treatment, Ibn Rochd University Hospital, Casablanca Morocco. overall survival is 90% for stage I and 70% for stage IV, Corresponding Author: Naqos Nora, Hospital Lyon Sud, 165 this decrease with 76 % for stage I and with 11% for stage Chemin Du Grand Revoyet 69310 Pierre Benith Lyon; Email: IV in adult [4]. [email protected] We report a new case of adult nephroblastoma (AN) in which we discuss the diagnosis, treatment and outcome of this rare tumor for which the therapeutic strategies are Received: 02 February 2015 more developed for pediatric groups and on which there Accepted: 25 March 2015 Published: 01 July 2015 are no large studies.

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CASE REPORT

A 29-year-old female was admitted to our department after 5 months history of left loin pain. Physical examination revealed hypertension and a palpable left loin mass. Biologically, the blood analysis were normal: hemoglobin: 12 g/dl, neutrophil: 3.4 g/l, leucocyte: 4.5 g/l, platelets: 340 g/l, creatinine: 58 mmol/l. Computed tomography (CT) scan of the abdomen showed a large left renal tumor with extension into renal vein and para- aortic lymphadenopathy with liver metastases, CT thorax confirmed multiple bilateral pulmonary lesions consistent with metastatic disease. A radical, transabdominal nephrectomy was performed. The operative specimen weighed 1540 g with a size of 13.5x13.5x8 cm surrounded by peritoneal grease with irregular outer surface. The kidney Figure 1: The operative specimen showing the kidney 19x17x15 measures 19/17/15 cm and presents a white yellowish cm with a white yellowish upper pole tumor of 13.5x13.5x8 cm upper pole tumor of 13.5x13.5x8 cm encapsulated. It has encapsulated. a soft consistency with necrotic bleeding site estimated at 20%. The next of renal parenchyma measures 5x6.5x3 cm (Figure 1). Histology confirmed a diffuse blastmental and epithelial Wilms tumour, it shows tumor proliferation with dual component: epithelial and mesenchymatous; the epithelial component makes glandular and trabecular structure bored with cylindrical basophile cells with big size, with hyperchromic nucleus and atypical mitosis. The epithelial structure is furrowed with fusiform cells big nucleus size, all are developed in a stroma surrounded by a fibrous tick capsule respecting the adjacent parenchyma. No image of vascular embolism was seen, the renal hilum was unaffected as well as the ureteral cuts and the adrenal (Figure 2). The lymph node dissection was negative 0N/6N. There were no postoperative complications. Chemotherapy according to the SIOP 93-01 protocol was started as soon as possible. After 2 cycles of chemotherapy combining actinomycine D at 15 mg per kg and per injection/3 weeks , adriamycin with the dose of 50 mg/m² each 3 weeks and Vincristine 1.5 mg/m² d1- d8 , a very good clinical response was seen and a marked reduction in metastases size occurred (Figures 3 and 4). After six cycles of chemotherapy a complete remission of Figure 2: Tumor proliferation with blastema, glomerular and liver metastases was obtained with a very good reduction tubular formations, and mesenchyme (Gx250). of lung metastases, unfortunately two months later the patient died after a sudden crisis headache.

indicative of advanced disease [5, 6]. The most common DISCUSSION metastatic sites are the lungs, liver and the bones [3]. The classic histology of nephroblastoma is that of a triphasic Nephroblastoma is rare in adults and could have a embryonal neoplasm containing varying amounts of more aggressive clinical behaviour than that in children, blastema, stroma and epithelial cells forming abortive it is frequently diagnosed at more advanced stages, stage tubular or glomerular structures. For diagnosis to be made III disease represents 50% of AN. not all the classic features are necessary, as some Wilms Clinically, the tumor is often discovered as an tumors may contain blastemal or epithelial features only incidental abdominal mass either by routine palpation or [7]. According to all available studies and due to the very on a CT-scan or ultrasound done for other reasons. More small number of reported cases, randomized trials cannot commonly however, flank pain and/or hematuria are the be undertaken and results of pediatric trials should first indications of the condition. Systemic symptoms be considered. And Until recently, no standardized such as weight loss, malaise, weakness, and fever are treatment for AN was found in literature [3, 8]. There

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Figure 4: Computed tomography abdominal scan showing the liver metastases before (A) and after (B) 3 cycles of chemotherapy.

In 1982, the NWTSG reported 31 patients with AN Figure 3: Computed tomography pulmonary scan showing the pulmonary metastases before (A) and after (B) 3 cycles of who were treated between 1968 and 1979 [8, 6]. 51.7% chemotherapy. had stage III and stage IV disease. The three-year survival rate was 24%, compared with 74% for patients with pediatric nephroblastoma (PN). These results indicated were only a few reports of small series of patients with a need for new therapeutic approaches for patients with AN who were treated with different protocols involving AN. In 1990, Arrigo et al. [8, 11] from the NWTSG studied chemotherapy, surgery, and radiotherapy [8]. The results 27 adult patients who were treated between 1979 and of these early studies are somewhat contradictory. 1987. Their results demonstrated a survival rate of 67% Fortunately, the more current guidelines proposed by the at three year. Moreover, the national Wilms Tumor Study National Wilms Tumor Study Group (NWTSG) and The (NWTS) recommends a multidisciplinary treatment: for International Society of Pediatric Oncology (SIOP) have stages III and IV, a large nephrectomy associated with helped to direct physicians [8]. a triple Chemotherapy (actinomycine D, vincristine and National Wilms Tumor Study Group (NWTSG) doxorubicin) during 15 months then a radiation of tumor realises nephrectomy at the time of diagnosis for all bed and a double chemotherapy without radiation for resectable primary tumors. Surgery is then followed both stage I and II [12]. by chemotherapy and radiation therapy to sites of For our patient we followed the NWTS’s metastatic or residual disease after surgery [9]. In recommendations, after surgery a combination of contrast, The International Society of Pediatric Oncology vincristine, dactinomycin and doxorubicin was used, (SIOP) recommends administering several weeks of after three cycles a remarkable reduction of about 80% of chemotherapy before nephrectomy [10]. lever and lung metastases was obtained.

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In the study of the International Society of Pediatric of data, Drafting the article, Revising it critically for Oncology [3], 30 AN were treated: 66% of them were important intellectual content, Final approval of the localized whereas 33% were metastatic at the time of version to be published diagnosis. Unlike protocols for children, the majority of Z. Bouchbika – Analysis and interpretation of data, adult in SIOP study underwent primary surgery; whereas Revising it critically for important intellectual content, preoperative chemotherapy was administered to only 4 Final approval of the version to be published out of 30 patients. The event-free survival was 57% at 4 A. Taleb – Analysis and interpretation of data, Revising years and the overall survival was 83% [3]. it critically for important intellectual content, Final Terenziani et al. [13] reported their institutional approval of the version to be published experience regarding AN. Between 1983 and 2001, 17 N. Benchkroune – Analysis and interpretation of data, patients with AN who were older than 16 years were Revising it critically for important intellectual content, treated according to pediatric nephroblastoma (PN) Final approval of the version to be published guidelines. In this series, the overall survival was 62.4% H. Jouhadi – Analysis and interpretation of data, at 5th year. Revising it critically for important intellectual content, Due to the frequency of lung metastases in stage III Final approval of the version to be published and IV, Hentrich et al. justify a systemic pulmonary N. Tawfiq – Analysis and interpretation of data, Revising irradiation of lung metastases they consider that an it critically for important intellectual content, Final irradiation of bed tumor may complete surgery following approval of the version to be published by chemotherapy (Dactinomycin + Vincristine + S. Sahraoui – Analysis and interpretation of data, Doxorubicin). Lately, in relapsed AN patients, high- Revising it critically for important intellectual content, dose chemotherapy followed by autologous steam cell Final approval of the version to be published rescue has been used as the salvage therapy but only a A. Benider – Analysis and interpretation of data, Revising small number of patients have obtained long complete it critically for important intellectual content, Final remission [1, 14]. The use of the new combination approval of the version to be published chemotherapy regimens based on the SIOP-2001 second-line protocol has not improved the outcome in Guarantor patients with recurrent AN. The best approach in the The corresponding author is the guarantor of submission. treatment of AN still needs to be defined. Abu-Ghosh et al. [15] reported an overall response rate of 82% in 11 Conflict of Interest patients treated with ifosfamide-carboplatin etoposide Authors declare no conflict of interest. chemotherapy for poor-risk relapsed nephroblastoma. Italiano [16] reported one case of a short but striking Copyright response to paclitaxel in an adult who had recurrent © 2015 N. Naqos et al. This article is distributed under the massive disease. Our patient presented with advanced terms of Creative Commons Attribution License which disease. His clinical follow-up suggests that treating AN permits unrestricted use, distribution and reproduction in like childhood Wilms tumor remains an effective option. any medium provided the original author(s) and original New modalities of treatment are being tested. One publisher are properly credited. Please see the copyright example is the use of recombinant interferon-a to treat policy on the journal website for more information. a case of recurrent adult nephroblastoma [17]. Whether these new modalities of treatment will prove to be more effective, they await additional data, hopefully from REFERENCES standardized clinical studies on adults. 1. Zdrojowy R, Sawicz-Birkowska K, Apoznanski W, et al. Adult Wilms tumour. Int Urol Nephrol 2011 CONCLUSION Sep;43(3):691–6. 2. Vehbi Ercolak, Ismail Oguz Kara. A case report of Wilms tumor or nephroblastoma is an exceptional adult Wilms’ tumour, ContempOncol (Pozn) 2014;18. tumor in adults. This diagnosis, often histological, is 3. Reinhard H, Aliani S, Ruebe C, Stockle M, Leuschner generally established at a more advanced clinical stage I, Graf N. Wilms’ tumor in adults: results of the than in children and the prognosis, for the same clinical Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) stage, is comparatively poorer, it must be kept in mind Study. J Clin Oncol 2004 Nov 15;22(22):4500–6. that only the early detection of the tumor offers patients 4. Tahri A, Benchekroun N, Karkouri M, et al. the best chance for survival. [Nephroblastoma in adults. Three case reports]. [Article in French]. Ann Urol (Paris) 2001 ********* Sep;35(5):257–61. 5. Dawson NA, Klein MA, Taylor HG. Salvage therapy Author Contributions in metastatic adult Wilms’ tumor. Cancer 1988 Sep N. Naqos – Substantial contributions to conception and 1;62(5):1017-21. design, Acquisition of data, Analysis and interpretation

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6. Fosså S. Rare and unusual tumors of the genitourinary 12. Kalapurakal JA, Nan B, Norkool P, et al. Treatment tract. Curr Opin Oncol 1992 Jun;4(3):463–8. outcomes in adults with favorable histologic type 7. Huser J, Grignon DJ, Ro JY, Ayala AG, Shannon Wilms tumor-an update from the National Wilms RL, Papadopoulos NJ. Adult Wilms’ tumor: a Tumor Study Group. Int J Radiat Oncol Biol Phys clinicopathologic study of 11 cases. Mod Pathol 1990 2004 Dec 1;60(5):1379–84. May;3(3):321–6. 13. Terenziani M, Spreafico F, Collini P, et al. Adult Wilms’ 8. Arifi S, Mellas N, Kaikani W, et al. Adult Wilms’ tumor: A monoinstitutional experience and a review Tumor: Report of 2 Cases and Review of the Literature. of the literature. Cancer 2004 Jul 15;101(2):289–93. UroToday International Journal 2010;3:1944–5784. 14. Petty R, Culligan D, Bissett D. Successful salvage 9. Grundy P, Green D, Coppes M. (2002) Renaltumors. of relapsed adults Wilms’ tumour with high dose In: Pizzo PA, Poplack DG (eds) Principles and practice chemotherapy and autologous peripheral blood of pediatric oncology. Lippincott Williams & Wilkins, stem cell rescue. Clin Oncol (R Coll Radiol) 2002 Philadelphia. Aug;14(4):329. 10. Tournade MF, Com-Nougué C, de Kraker J, et al. 15. Abu-Ghosh AM, Krailo MD, Goldman SC, et al. Optimal duration of preoperative therapy in unilateral Ifosfamide, carboplatin and etoposide in children with and nonmetastatic Wilms’ tumor in children older poor-risk relapsed Wilms’ tumor: a Children’s Cancer than 6 months: results of the Ninth International Group report. Ann Oncol 2002 Mar;13(3):460–9. Society of Pediatric Oncology Wilms’ Tumor Trial and 16. Italiano A, Sirvent N, Michiels JF, Peyrade F, Otto J, Study. J Clin Oncol 2001 Jan 15;19(2):488–500. Thyss A. Tumour response to paclitaxel in an adult 11. Arrigo S, Beckwith JB, Sharples K, D’Angio G, Haase with relapsed nephroblastoma. Lancet Oncol 2005 G. Better survival after combined modality care for Apr;6(4):252–3. adults with Wilms’ tumor. A report from the National 17. Tawil A, Cox JN, Roth AD, Briner J, Droz JP, Remadi S. Wilms’ Tumor Study. Cancer 1990 Sep 1;66(5):827– Wilms’ tumor in the adult--report of a case and review 30. of the literature. Pathol Res Pract 1999;195(2):105– 11.

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CASECase REPORTReport Peer Reviewed OPEN| OPEN ACCESS ACCESS Eruptive Collagenoma in a mongol girl: A rare association

Balwinder Kaur Brar, Mahajan B. B., Nidhi Kamra

Abstract How to cite this article

Introduction: Eruptive collagenoma is a rare Brar BK, Mahajan BB, Kamra N. Eruptive type of connective tissue naevi with predominant Collagenoma in a mongol girl: A rare association. extra-cellular matrix component being collagen. Int J Case Rep Images 2015;6(7):427–430. Lesions are characterized by abrupt history of onset. Case Report: An 18-year-old female with a clinical diagnosis of Down syndrome presented doi:10.5348/ijcri-201571-CR-10532 with abrupt onset of skin colored nodules on upper back and mons pubis. Lesions were asymptomatic. On examination multiple, firm, discrete, non-tender, flesh colored nodules were present on upper 2/3 of back and mons pubis. INTRODUCTION Histopathology aided with Masson’s trichome stain confirmed the diagnosis of collagenoma. Connective tissue nevi are hamartomas characterized Conclusion: The association of collagenoma has by an excess or deficit in the number of cells and their been known with various systemic disorders but biosynthetic products including collagen, elastic fibers, its alliance with Down syndrome and localization and glycosaminoglycans [1]. Connective tissue nevi with over mons pubis has still not been reported. predominantly collagen are referred to as Lipschutz type and with elastic component as Lewandowsky type. Keywords: Eruptive collagenoma, Down syn- Multiple collagenomas are invariably present in several drome, Mons pubis, Flesh colored modules distinct syndromes like Buschke-Ollendorf syndrome, eruptive collagenoma, familial cutaneous collagenoma and tuberous sclerosis. Isolated cerebriform collagenoma of the palm and sole is a well-known entity and has been reported in association with Proteus syndrome. Eruptive Balwinder Kaur Brar1, Mahajan BB2, Nidhi Kamra3 collagenoma is a rare type of acquired collagenoma that was first reported in 1955 by Colomb [2–5]. Herewith a Affiliations: 1Assistant Professor, Department of Dermatology, Guru Gobind Singh Medical College and Hospital, Faridkot, report of eruptive collagenoma in Down syndrome is Punjab, India; 2Professor and Head, Department of reported for its rarity and unusual localization Dermatology, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab, India; 3PG Resident, Department of Dermatology, Guru Gobind Singh Medical College and CASE REPORT Hospital, Faridkot, Punjab, India. Corresponding Author: Dr. Nidhi Kamra, Department An 18-year-old female with history of delayed of Dermatology, Guru Gobind Singh Medical College milestones, low IQ, and typical Mongolian facies (Figure and Hospital, Saadiq Road, Faridkot, Punjab, India; Ph: 1) (typical slant of forehead, and macroglossia) presented 07508405707; Email: [email protected] to our outpatient department with abrupt onset of asymptomatic skin colored nodules within a period of Received: 26 October 2014 one month on upper back. The patient did not report any Accepted: 07 January 2015 previous history of chicken pox/ trauma at the involved Published: 01 July 2015 site. Her family history was unremarkable. A review of

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):427–430. Brar et al. 428 www.ijcasereportsandimages.com the various organ systems (cardiovascular, respiratory, gastrointestinal tract, central nervous system) was within normal limits. Examination revealed multiple, discrete, firm, non-tender, skin colored nodules measuring 0.5 to 2 cm in diameter with no scaling or exudation on the surface. The lesions were present predominantly on upper 2/3 of back and on thorough cutaneous examination similar lesions were present on the mons pubis, which as per patient developed 6 months back (Figures 2 and 3). We kept the differentials of eruptive xanthoma, steatocystoma multiplex and eruptive collagenoma. There was no evidence of hypopigmented macules on wood’s lamp examination or skin lesions suggestive of tuberous sclerosis. There was no significant past medical or surgical history. The hematological and biochemical investigations including complete blood count, renal, liver function tests, urine analysis, lipid profile, electrocardiogram, abdominal ultrasound and chest roentgenogram were within normal limits. Skeletal survey did not demonstrate any evidence of osteopoikilosis. A skin biopsy obtained from the lesion on upper back and mons pubis showed focal acanthotic epidermis and significantly increased density of collagen bundles in the deep reticular dermis (Figure 4). Masson’s trichrome stain confirmed the presence of dense collagen bundles with decreased elastic fibers (Figure 5). Thus a diagnosis of sporadic eruptive collagenoma with Down syndrome was made. Figure 2:Multiple, discrete, skin colored nodules on upper back.

Figure 3: Multiple flesh colored nodules on mons pubis. Figure 1: The patient has typical Mongolia facies expression

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Table 1: Classification of collagenoma (Clinical variants)

Familial cutaneous collagenoma( FCC) Shagreen patch(TS) Eruptive collagenoma Plantar cerebriform collagenoma Linear connective tissue naevus Knuckle pads Other collagenomas

Table 2: Classification of collagenoma (Genetic inheritance pattern).

Inherited collagenoma 1. Familial cutaneous collagenoma 2. Dermatofibrosis lenticularis disseminate 3. Shagreen patch( tuberous sclerosis) Acquired Collagenoma Figure 4: Focal acanthotic epidermis with significantly increased 1. Eruptive collagenoma collagen bundles in dermis. 2. Isolated collagenoma

Collagenomas in FCC are also distributed symmetrically on trunk and proximal extremities, but are more numerous (in hundreds) and are also associated with various cardiac abnormalities like cardiomyopathy and conduction disorders [3, 6]. Shagreen patches (plaques of collagenoma) are present in tuberous sclerosis with other classic cutaneous findings like ash-leaf macules, facial angiofibromas, periungual fibromas (Koenen’s tumor), gingival fibromas and fibrous plaque of forehead. Isolated collagenomas are sporadic and are localized most commonly on palm, sole and labium majus. Cerebriform plantar nevi are considered to be pathognomic of Proteus syndrome (a type of epidermal nevus syndrome) [4, 5, 7]. However, many authors have reported the presence of plantar collagenoma without any co-existent features of Proteus syndrome [8] isolated collagenoma on the scalp has been reported and can manifest as cutis verticis gyrata [9]. Collagenomas have also been reported in alliance with pseudohypoparathyroidism and hypogonadism [10, 11]. Though the pathogenesis of collagenomas is unknown, sporadic collagenomas may be related to trauma, since Figure 5: Masson trichome stain: Increased collagen bundles in they appear most frequently in areas subject to friction, in dermis (blue). Down syndrome too its pathogenesis is unclear. However, elastosis perforans serpiginosa is also well known as a complication of this syndrome, suggesting that Down DISCUSSION syndrome may have various accompanying connective tissue disorders [12]. Collagenoma (collagen nevi) have been classified into distinct groups on the basis of clinical considerations (Table 1). Depending upon classification of the genetic CONCLUSION inheritance pattern, collagenomas are classified as either inherited or sporadic (Table 2) with autosomal dominant Collagenoma may be a marker of internal inheritance common to all inherited subtypes. Eruptive disease like tuberous sclerosis, Down syndrome, collagenoma is characterized by abrupt development of pseudohypoparathyroidism and it may be present in multiple asymptomatic skin colored papules, nodules, isolated or eruptive pattern as in our case. To the best plaques symmetrically on torso and proximal upper of our knowledge, only eight case reports of eruptive extremities but localization to mons pubis as in our case collagenoma could be retrieved by searching on has not been reported. Collagenoma in eruptive type are PUBMED/MEDLINE, with none being reported in Down smaller than those of familial cutaneous collagenoma. syndrome with unique localization to mons pubis.

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********* 2. Zhao C, Ma W, Wang Y, Sun Q. Female with eruptive collagenoma clustered in the left lateral aspect of the Acknowledgements abdomen. J Dermatol 2010 Sep;37(9):843–5. 3. Yahya H, Rafindadi AH. Eruptive collagenoma in a Dr. Asha Kubba, Delhi Dermatology group. Nigerian girl. Int J Dermatol 2006 Nov;45(11):1344– 6. Author Contributions 4. Biesecker L. The challenges of Proteus syndrome: Balwinder Kaur Brar – Substantial contributions to Diagnosis and management. Eur J Hum Genet 2006 conception and design, Acquisition of data, Analysis Nov;14(11):1151–7. and interpretation of data, Drafting the article, Revising 5. Beachkofsky TM, Sapp JC, Biesecker LG, Darling TN. it critically for important intellectual content, Final Progressive overgrowth of the cerebriform connective approval of the version to be published tissue nevus in patients with Proteus syndrome. J Am Mahajan B. B. – Analysis and interpretation of data, Acad Dermatol 2010 Nov;63(5):799–804. 6. Uitto J, Santa-Cruz DJ, Eisen AZ. Familial cutaneous Revising it critically for important intellectual content, collagenoma: Genetic studies on a family. Br J Final approval of the version to be published Dermatol 1979 Aug;101(2):185–95. Nidhi Kamra – Analysis and interpretation of data, 7. Pierard GE, Lapiere CM. Nevi of connective tissue: A Revising it critically for important intellectual content, reprisal of their classification. Am J Dermatopathol Final approval of the version to be published 1985 Aug;7(4):325–33. 8. Nelson AA, Ruben BS. Isolated plantar collagenoma Guarantor not associated with Proteus syndrome. J Am Acad The corresponding author is the guarantor of submission. Dermatol 2008 Mar;58(3):497–9. 9. Laxmisha C, Thappa DM, Jayanthi S. Isolated scalp collagenoma mimicking cutis verticisgyrata. Conflict of Interest Indian J Dermatol Venereol Leprol 2006 Jul- Authors declare no conflict of interest. Aug;72(4):309–11. 10. Kakinuma Y, Endo H, Tsukahara T, Futoeda Copyright T, Saito Y, Shinkai H. Collagenoma with © 2015 Balwinder Kaur Brar et al. This article is pseudohypoparathyroidism. Br J Dermatol 2000 distributed under the terms of Creative Commons Nov;143(5):1122–4. Attribution License which permits unrestricted use, 11. Sacks HN, Crawley IS, Ward JA, Fine RM. Familial distribution and reproduction in any medium provided cardiomyopathy, hypogonadism, and collagenoma. the original author(s) and original publisher are properly Ann Intern Med 1980 Dec;93(6):813–7. 12. Togawa Y, Nohira G, Shinkai H, Utani A. credited. Please see the copyright policy on the journal Collagenoma in Down syndrome. Br J Dermatol 2003 website for more information. Mar;148(3):596–7.

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1. Uitto J, Santa Cruz DJ, Eisen AZ. Connective tissue nevi of the skin. Clinical, genetic, and histopathologic classification of hamartomas of the collagen, elastin, and proteoglycan type. J Am Acad Dermatol 1980 Nov;3(5):441–61.

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Single coronary artery arising from the right coronary sinus with mid-left anterior descending artery segment courses through the ventricular myocardium: A rare entity

Andrea Romagnoli, Irene Coco, Dominique De Vivo, Eros Calabria, Giovanni Simonetti

Abstract bridges cannot be demonstrable angiography, so anatomic and panoramic CT-evaluation give Introduction: Congenital anomalies of the more information especially if associated with coronary artery have an incidence of 1% functional evaluation. Knowledge of physiology, and isolated single coronary artery without normal and variant anatomy, is most important other congenital cardiac anomalies has an in managing congenital and acquired disease, approximate incidence of 0.024–0.066% in and variation in coronary arterial patterning is general population. This rare entity can be frequent. diagnosed incidentally during life, however may lead to symptomatic or asymptomatic myocardial Keywords: Coronary artery anomaly, Cardiac infarction and sudden heart attack, even among anomalies, Heart attack, Isolated single coro- young athletes. The prognosis varies according nary artery, Myocardial bridge, Ventricular my- to the anatomic distribution, associated ocardium atheromatous disease and associated vascular anomalies. Case Report: A 45 years old male How to cite this article with presentation of a single coronary artery from the right coronary sinus with subsequent Romagnoli A, Coco I, De Vivo D, Calabria E, coursing between the aorta and pulmonary Simonetti G. Single coronary artery arising from trunk arteries and mid anterior descending the right coronary sinus with mid-left anterior artery segment course through the myocardium. descending artery segment courses through the Patient presented with non-typical angina ventricular myocardium: A rare entity. Int J Case Rep symptoms, normal resting electrocardiograph, Images 2015;6(7):431–435. myocardial perfusion scintigraphy suspect to reduced left ventricular blood flow but negative doi:10.5348/ijcri-201572-CR-10533 angiographic evaluation. Conclusion: Thin

Andrea Romagnoli, Irene Coco, Dominique De Vivo, Eros Calabria, Giovanni Simonetti INTRODUCTION Affiliations: 1MD, Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, Tor Congenital anomalies of the coronary artery have an Vergata University Hospital Foundation, University of Rome incidence of 1% and isolated single coronary artery without Tor Vergata, Italy. other congenital cardiac anomalies has an approximate Corresponding Author: Dominique De Vivo, MD, Department incidence of 0.024–0.066% in general population. This of Diagnostic Imaging, Molecular Imaging, Interventional rare entity can be diagnosed incidentally during life, Radiology and Radiotherapy, Tor Vergata University Hospital however may lead to symptomatic or asymptomatic Foundation, University of Rome Tor Vergata, Italy; Email: myocardial infarction and sudden heart attack, even [email protected] among young athletes [1]. The prognosis varies according to the anatomic distribution, associated atheromatous Received: 28 February 2015 disease and associated vascular anomalies. Accepted: 22 April 2015 Published: 01 July 2015

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CASE REPORT

A 45-year-old male was admitted in our department with symptoms characterized by intermittent substernal chest discomfort non-provoked by exertion or emotional stress enlarging for over four months. The patient was a smoker of 7–8 packs of cigarettes per week for 10 years. Clinical history was negative for hypertension, diabetes mellitus, hyperlipidemia or family history of premature vascular disease and no risk factors for pulmonary embolism or respiratory distress. Physical examination showed normal blood pressure (BP: 128/88 mmHg), heart rate of 73 beats/min, no signs of vascular disease and normal cardiac examination. Initial laboratory tests showed no particular anomalies with normal blood levels of glucose and cholesterol (LDL: 103 mg/dl; HDL: 35 mg/dl; TG: 198 mg/dl). Resting electrocardiogram (ECG) showed normal sinus rhythm at 82 beats/min without anomalies in stress examination. Several echocardiographic examinations revealed a moderate enlargement of the left atrium; an apical form of left ventricular hypertrophy; a preserved left ventricular (LV) systolic function (LV ejection fraction, 57%); and moderate mitral valve regurgitation, with no abnormality of the mitral valve apparatus or of LV wall motion. Three months ago the patient had undergone coronary angiography evaluation which demonstrated an anomalous vascular anatomy with absence of coronary ostium in left cusp of the aortic valve and a single coronary artery (SCA) with origin by the right coronary cusp with Figures 1: Coronary computed tomography angiography with one and only common trunk (CT) which gives rise to right volume rendering reconstruction (1) and with Curved MPR (2) coronary artery (RCA), left anterior descending (LAD) images demonstrated an anomalous vascular anatomy with and diagonal artery, circumflex branch (CB) and the absence of coronary ostium in left cusp of the aortic valve and a single coronary artery (SCA) with origin by the right coronary proximal left marginal artery (LMA). The RCA gives off cusp with one and only common trunk (CT) which gives rise the posterior descending artery (PDA) and the postero- to right coronary artery (RCA), left anterior descending (LAD) lateral branch (PL). and diagonal artery, circumflex branch (CB) and the proximal Significant flow-limiting stenosis was not detected left marginal artery (LMA). The RCA gives off the posterior with regular caliper representation of the three main descending artery (PDA) and the postero-lateral branch (PL). coronary branches up to their distal segments. Contrast ventriculography confirmed the apical LVH and showed no wall motion abnormality. In our department, the patient underwent CT scanning, which confirmed the anomalous origin and course of coronary vessels showing also an inter-arterial course of the CT between the pulmonary artery and the aorta (Figures 1–2). A 2-cm segment of the proximal- middle tract of the common trunk was embedded within Figures 2: Coronary computed tomography angiography with the inter-ventricular septum, following an intramural volume rendering reconstruction (1) and with Curved MPR (2) course with a relative reduction of tunneled vessel (Figure images demonstrated an anomalous vascular anatomy with 3). absence of coronary ostium in left cusp of the aortic valve and No significant atheromatous alterations have been a single coronary artery (SCA) with origin by the right coronary highlighted, if we except for some parietal irregularities cusp with one and only common trunk (CT) which gives rise to right coronary artery (RCA), left anterior descending (LAD) at LAD. and diagonal artery, circumflex branch (CB) and the proximal Dipyridamole Tl-201 SPECT confirmed a perfusion left marginal artery (LMA). The RCA gives off the posterior abnormalities with reversible perfusion defects in the descending artery (PDA) and the postero-lateral branch (PL). mid anteroseptal wall without an apical abnormality.

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and the type of anomalous artery should be carefully evaluated [6]. Usually, typical angina does not occur with SCA in the absence of coexisting coronary artery disease or aortic stenosis [1]. In our case, the patient’s symptoms could be attributable to the intramyocardial segment of LAD. The myocardial bridge, occurs when the artery coursing within the myocardium, presents compression to the contraction of the heart muscle to systole, which is clinically silent most of the time [6]. A deepened critical analysis of many autopsy samples was first presented by Geiringer et al. in 1951: clinical interest and systematic research were triggered by the observation of myocardial bridging right along with myocardial ischemia [7]. The rate of angiographic bridging is <5%, linked to thin bridges that provide a light compression. Carrying out provocation tests in subjects presenting normal Figure 3: Curved MPR image showing the presence of 2-cm segment of the proximal-middle tract of the computed angiographic coronary arteries may enhance the systolic tomography was embedded within the inter-ventricular septum, myocardial compression and could thereby demonstrate following an intramural course with a relative reduction of myocardial bridges in ≤40% of cases [8]. tunneled vessel. Myocardial bridges are preferably localized in the middle segment of the LAD [7]. The patient was admitted to cardiology center for One of the parallel LAD branches frequently keeps an further evaluation, treatment and periodical follow-up. intramural course [7]. Diagonal and marginal branches may be respectively involved in 18% and 40% of cases [6]. By the angiographic DISCUSSION side, myocardial bridges are almost exclusively spotted in the LAD. They set at 1 to 10 mm depth showing Isolated SCA without other congenital cardiac typical length around ≈10 to 30 mm [7]. Ferreira et al. anomalies is very rare among the different variations of distinguished two types of bridging: (1) superficial bridges anomalous coronary patterns occurring in approximately (75% of cases) crossing the artery in perpendicular 0.024% of the population [1]. way or taking an acute angle towards the apex, and (2) Origin of the LCA in the right coronary sinus has been muscle bundles arising from the right ventricular apical described at a frequency of 0.02% in autopsy series and trabeculae (25% of cases) that transversely, obliquely, from 0.05–0.19% in angiographic series [2]. or helically cross the LAD before they flow in the The LCA either has a common ostium with the RCA, interventricular septum. Arterial segments could also be or arises independently to the ostium of RCA. These set in a deep interventricular gully. The segment proximal cases are classified according to the course of LCA into to the bridge frequently, because of hemodynamic forces, four categories: (1) inter-truncal or inter-atrial, between shows atherosclerotic plaque formation, although the the aorta and pulmonary arteries; (2) anterior, in front tunneled segment is typically spared [9]. Neither low value of the right ventricular outflow; (3) posterior or retro- proximal to the bridge stenosis, nor systolic compression aortic, behind the aorta; (4) inter truncal-septal or of the tunneled segment are allowed by the way to explain trans-septal, through the supraventricular crest and a severe ischemia and its related symptoms. When inter-ventricular septum. The retro-aortic course of the the arterial occlusion was limited to the only systole, LCA is an uncommon entity. This anomaly is serious and phasic coronary blood flow and distal coronary pressure associated with sudden cardiac death and myocardial were observed in a considerable delay, contributing to infarction, if anomalous LCA passes between the aorta a smaller myocardial oxygen consumption and to the and the pulmonary artery [3, 4]. The anterior as well as increase of the coronary sinus lactate concentration. the posterior course have been considered to be clinically Angina, myocardial ischemia, myocardial infarction, insignificant [4]. However, there have been isolated left ventricular dysfunction, myocardial stunning, reports of ischemia or sudden death associated with the paroxysmal AV blockade, as well as exercise-induced retro-aortic course of LMCA or one of its branches [5]. ventricular tachycardia and sudden cardiac death are Although most patients with the anomalous LCA arising reported as sequelae of the myocardial bridging.However, from the right sinus of Valsalva are asymptomatic, the following the prevalence of myocardial bridging, these therapeutic approach must be individualized in each complications are rare. Patients may complain atypical or subject. In asymptomatic subjects, the age of the patient angina-like chest pain with unsure association between

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):431–435. Romagnoli et al. 434 www.ijcasereportsandimages.com the severity of these symptoms and length/depth values Guarantor of the tunneled segment, or the systolic compression The corresponding author is the guarantor of submission. degree [6]. The up-to-date gold standard for diagnosing myocardial bridges remains the coronary angiography Conflict of Interest presenting the typical “milking effect” and a “step down– Authors declare no conflict of interest. step up” phenomenon induced by the systolic compression of the tunneled segment. By the way, these signs give Copyright insufficient information about the functional impact on © 2015 Andrea Romagnoli et al. This article is distributed myocardium. Proximal stenosis and myocardial bridging under the terms of Creative Commons Attribution could only be identified by carrying out a percutaneous License which permits unrestricted use, distribution transluminal coronary angioplasty, because higher and reproduction in any medium provided the original intravascular pressure values and reversed hypokinesis author(s) and original publisher are properly credited. can reveal the myocardial bridging [10]. Please see the copyright policy on the journal website for more information. CONCLUSION REFERENCES Coronary angiography evaluation has demonstrated an anomalous vascular anatomy with absence of 1. Thummar AC, Lanjewar CP, Phadke MS, Sharma RB, coronary ostium in left cusp of the aortic valve. Computed Kerkar PG. Isolated single coronary artery (RII-B tomography (CT) scanning has confirmed the anomalous type) presenting as an inferior wall myocardial origin and course of coronary vessels showing also an infarction: A rare clinical entity. Indian Heart J 2014 inter-arterial course of the CT between the pulmonary Sep-Oct;66(5):553–4. 2. Kimbiris D. Anomalous origin of the left main artery and the aorta. A 2-cm segment of the proximal- coronary artery from the right sinus of Valsalva. Am J middle tract of the CT was embedded within the inter- Cardiol 1985 Mar 1;55(6):765–9. ventricular septum, following an intramural course with 3. Madea B, Dettmeyer R. Sudden death in cases with a relative reduction of tunneled vessel. Thin bridges anomalous origin of the left coronary artery. Forensic can be not demonstrable angiography, so anatomic Sci Int 1998 Sep 28;96(2-3):91–100. and panoramic CT-evaluation give more information 4. Abouzied AM, Amaram S, Neerukonda SK. Anomalous especially if associated with functional evaluation. left coronary artery arising from right sinus of Knowledge of physiology, normal and variant anatomy, valsalva could be a minor congenital anomaly--a case is most important in managing congenital and acquired report and review of the literature. Angiology 1999 Feb;50(2):175–8. disease, and variation in coronary arterial patterning is 5. Basso C, Maron BJ, Corrado D, Thiene G. Clinical frequent. profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden ********* death in young competitive athletes. J Am Coll Cardiol 2000 May;35(6):1493–501. Author Contributions 6. Möhlenkamp S, Hort W, Ge J, Erbel R. Update Andrea Romagnoli – Substantial contributions to on myocardial bridging. Circulation 2002 Nov conception and design, Revising it critically for important 12;106(20):2616–22. 7. GEIRINGER E. The mural coronary. Am Heart J 1951 intellectual content, Final approval of the version to be Mar;41(3):359–68. published 8. Diefenbach C, Erbel R, Treese N, Bollenbach E, Meyer Irene Coco – Acquisition of data, Drafting the article, J. Incidence of myocardial bridges after adrenergic Final approval of the version to be published stimulation and decreasing afterload in patients with Dominique De Vivo – Acquisition of data, Revising angina pectoris, but normal coronary arteries. [Article it critically for important intellectual content, Final in German] Z Kardiol 1994 Nov;83(11):809–15. approval of the version to be published 9. Ferreira AG Jr, Trotter SE, König B Jr, Décourt LV, Fox Eros Calabria – Analysis and interpretation of data, K, Olsen EG. Myocardial bridges: Morphological and Drafting the article, Final approval of the version to be functional aspects. Br Heart J 1991 Nov;66(5):364–7. 10. Tobias SL, Videlefsky SW, Misra VK. Physiological published significance of a proximal coronary artery stenosis on Giovanni Simonetti – Substantial contributions to a distal intramyocardial bridge: coronary flow velocity conception and design, Revising it critically for important patterns pre- and post-angioplasty. Cathet Cardiovasc intellectual content, Final approval of the version to be Diagn 1995 Jun;35(2):127–30. published

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About the Authors

Article citation: Romagnoli A, Coco I, De Vivo D, Calabria E, Simonetti G. Single coronary artery arising from the right coronary sinus with mid-left anterior descending artery segment courses through the ventricular myocardium: A rare entity. Int J Case Rep Images 2015;6(7):431–435.

Andrea Romagnoli is working in Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, Tor Vergata University Hospital Foundation, University of Rome Tor Vergata, Italy. E-mail: [email protected]

Irene Coco is working in Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, Tor Vergata University Hospital Foundation, University of Rome Tor Vergata, Italy. E-mail: [email protected]

Dominique De Vivo is working in Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, Tor Vergata University Hospital Foundation, University of Rome Tor Vergata, Italy. E-mail: [email protected]

Eros Calabria is working in Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, Tor Vergata University Hospital Foundation, University of Rome Tor Vergata, Italy. E-mail: [email protected]

Giovanni Simonetti is working in Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, Tor Vergata University Hospital Foundation, University of Rome Tor Vergata, Italy. E-mail: [email protected]

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Cannabis consumption before surgery may be associated with increased tolerance of anesthetic drugs: A case report

Georg Richtig, Götz Bosse, Friederike Arlt, Christian von Heymann

Abstract of cannabis intake should be taken into routine preoperative assessment and patients should Introduction: Cannabis is the most commonly be closely monitored by experienced anesthesia used illicit drug worldwide in adults. Several staff for adequate anesthetic depth and analgesia. studies suggest an interaction of cannabis intake and anesthetic drugs like propofol and thiopental, Keywords: Anesthesia, Cannabis, Interaction, but the pharmacodynamic interaction between Pharmacodynamics cannabis and commonly used anesthetic drugs is still not well understood. Case Report: A 37-year- How to cite this article old male patient with a history of regular cannabis consumption and cannabis intake the day before Richtig G, Bosse G, Arlt F, von Heymann C. Cannabis surgery was admitted for an arthroscopic consumption before surgery may be associated with shoulder stabilization. Induction of anesthesia increased tolerance of anesthetic drugs: A case report. required large doses of propofol, thiopental and Int J Case Rep Images 2015;6(7):436–439. sevoflurane. Maintenance was achieved with the additional application of nitrous oxide to provide doi:10.5348/ijcri-201573-CR-10534 clinical tolerance of the surgical procedure. Return of consciousness and the postoperative course were uneventful. Conclusion: History

Georg Richtig1, Götz Bosse2, Friederike Arlt3, Christian von INTRODUCTION Heymann4 Affiliations: 1MD, Research Fellow, Clinical Institute of Medical Cannabis is the most consumed illegal drug worldwide and Chemical Laboratory Diagnostics, Medical University and there is a growing number of ongoing programs to of Graz, Graz, Austria; 2MD, Consultant, Department of offer a legal source of cannabis to chronically sick patients Anaesthesiology and Intensive Care Medicine, Campus [1]. Charité Mitte and Campus Virchow – Klinikum, Charité Several studies suggest an interaction of cannabis 3 - University Medicine Berlin, Germany; MD, Registrar, intake and anesthetic drugs like propofol and thiopental Department of Anaesthesiology and Intensive Care Medicine, [2]. However, this interaction and its implications for Campus Charité Mitte and Campus Virchow – Klinikum, Charité - University Medicine Berlin, Germany; 4MD, DEAA, anesthetic practice in patients with chronic cannabis Professor, Department of Anaesthesiology and Intensive intake is not well understood. Care Medicine, Campus Charité Mitte and Campus Virchow Herein, we describe a case of a patient scheduled for – Klinikum, Charité - University Medicine Berlin, Germany. a routine surgical procedure with a history of regular Corresponding Author: Georg Richtig, Department: Clinical cannabis and tobacco consumption, who needed large Institute of Medical and Chemical Laboratory Diagnostics amounts of different anesthetic agents to provide Institution: Medical University of Graz. Auenbruggerplatz 15, sufficient condition for intubation. Graz, Postal Code: A-8036, Austria; Ph: +436647812823, Fax: +4331638512466; Email: [email protected] CASE REPORT Received: 28 March 2015 Accepted: 08 May 2015 A 37-year-old male patient, 93 kg weight, 206 cm Published: 01 July 2015 tall, was scheduled for an arthroscopic stabilization of

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):436–439. Richtig et al. 437 www.ijcasereportsandimages.com his right shoulder due to a traumatic glenoid fracture. added with a concentration of 50% and a flow of 1 L/min. His medical history revealed an appendectomy at the The entire induction period took 15 minutes from age of nine with no anesthetic problems. The patient was the application of fentanyl to the maintenance state with healthy and in good medical conditions. He reported a sevoflurane and nitrous oxide at the end. Only after the daily nicotine usage of 10–15 cigarettes, a cannabis intake application of sevoflurane and nitrous oxide the patient of 1 g per week and an occasional use of alcohol. He had a significant drop in his blood pressure and heart rate. admitted smoking one cannabis cigarette the day before After fractional (4x2 ml) application of akrinor (cafedrine surgery. hydrochlorid 200 mg and theodrenalinhydrochlorid 10 In the operating room non invasive cardiorespiratory mg, Akrinor®, CT Arzneimittel, Berlin, Germany) the monitoring measured a blood pressure of 120/70 mmHg, patient showed a stable blood pressure of around 110/60 a heart rate 80 bpm and an oxygen saturation (SpO2) of mmHg through the whole procedure. For intraoperative 98%. Prior to induction the patient was oxygenated with analgesia a total amount of 0.5 mg of fentanyl was a FiO2 of 1.0 and a flow of 12 L/min for 5 minutes. A administrated. 20 minutes before the end of procedure 2 g regional anesthetic technique like an interscalene block metamizole (Novaminsulfon-ratiopharm®, Ratiopharm, had been declined by the patient. Ulm, Germany) were applied. Since the patient had Over an iv-line on his left backhand (BD Venflon™ a constantly high blood pressure (150/80 mmHg) Pro Safety 18G, Becton Dickinson, Heidelberg, Germany) after extubation and no pain, he also received 60 µg 0.2 mg of fentanyl (Fentanyl®-Janssen, Janssen-Cilag, clonidine (Catapresan®, Boehringer Ingelheim Pharma, Neuss, Germany) were injected and the patient reported Ingelheim, Germany). After 30 minutes of observation in of a dizzy feeling after four minutes. 200 mg of propofol the post-anesthesia care unit, the patient was fully awake, (Braun Propofol-®Lipuro, B. Braun, Melsungen, showed no signs of persisting anesthetic drug effect and Germany) were administered without any anesthetic was discharged to the surgical ward. Post-surgical pain effect as the patient was still speaking with the anesthesia was controlled with 500 mg metamizole every eight staff. Another two doses of propofol of 100 mg each were hours. The patient was discharged two days after surgery. injected, but the patient did not show any reduction of his conscious state. The iv cannula was checked for correct position and no signs of extravasation of the anesthetic DISCUSSION agents were detected. The anesthetic regime was changed. We decided to Herein, we report a case of a 37-year-old male with a administer 500 mg of thiopental (Thiopental Inresa® regular cannabis abuse who required excessive doses of 0.5 g, Inresa Arzneimittel, Freiburg, Germany), that different anesthetic drugs for induction of anesthesia. We finally induced anesthesia. However, the patient was still postulate that this might be related to a pharmacological breathing spontaneously with a tidal volume of 100–150 interaction of cannabis and anesthetic drugs resulting in ml and a respiratory rate of 20/min requiring assisted tolerance of higher anesthetic dosages. ventilation using a face mask that was not tolerated by In literature, the effect of cannabis on anesthetic drugs the patient. 4 vol% of isoflurane (Forane®, Abbott, are discussed controversially. Ludwigshafen, Germany) was added to achieve tolerance Flisberg et al. [2] could show, that patients with a of the assisted ventilation. history of cannabis consumption needed significant After three minutes of isoflurane-supplemented more propofol to successfully achieve the insertion of a assisted ventilation a size 5 laryngeal mask (LMA/ laryngeal mask, than the control group. They concluded Unique™ Single Use, Teleflex Medical, Kernen, that regular cannabis users show a more variable response Germany) was inserted to further improve ventilation. to induction of anesthesia with higher doses of propofol The tidal volume was increased to 200 ml requiring a needed [2]. These human data are supported by Brand peak inspiratory pressure of 30 cm H2O. A further dose et al. [3] who reported a dose-dependent antagonistic of 200 mg of propofol was administered to deepen the interaction between tetrahydrocannabinol (THC) and anesthesia so that the patient was finally sufficiently propofol in mice. ventilated. Due to the need to position the patient on In contrast, several experimental studies have the left side, neuromuscular blockade was achieved with also shown an enhancement of the sedative effect of 100 mg succinylcholine and endotracheal intubation intravenous [4] and inhalational [5] anesthetic drugs by was performed without any complications (Cormack II, THC, so that an individual dose titration and continuous tube size 8 mm, Covidien Mallinckrodt™ Hi−Contour, monitoring of the anesthetic depth is recommended. Covidien Germany, Neustadt/Donau, Germany). In our case, the use of thiopental and propofol did After intubation sufficient ventilation was achieved not seem to have the expected effect on the patient’s by a tidal volume of 650 ml, a respiratory rate of 16 and consciousness. Furthermore, the induction and an etCO2 of 40 to 45 mmHg. In the operating room maintenance of anesthesia with high concentrations anesthesia was maintained with an end-tidal volume of isoflurane and sevoflurane did not achieve adequate concentration (etVol%) of 2.4% sevoflurane (Sevorane®, anesthetic depth. Only when nitrous oxide had been Abbott, Ludwigshafen, Germany). Nitrous oxide was added to sevoflurane, sufficient tolerance of the surgical

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):436–439. Richtig et al. 438 www.ijcasereportsandimages.com procedure could be provided. In connection with CONCLUSION pharmacokinetics, similar findings have been reported by Symons [6] describing a patient with a history of cannabis Patients with regular cannabis consumption are smoking, who required repetitive doses of propofol (3x50 recommended to closely monitored for adequate mg), midazolam (2x1 mg) and ketolorac (10 mg), besides anesthetic depth and analgesia, treated by experienced high concentrations of isoflurane and nitrous oxide to staff only and regional anesthetic techniques should maintain anesthesia for a tooth extraction. be favored. Patients are recommended not to consume A possible pharmacodynamic explanation could cannabis as long as possible before the surgical procedure. be that propofol and thiopental mediate their effects over the gamma-aminobutyric acid type a (GABA (A)- ********* receptor), like the volatile anesthetics, sevoflurane and isoflurane, which may have been influenced by THC and Author Contributions other cannabinoid compounds. Georg Richtig – Substantial contributions to conception Liu et al. [7] observed that GABA (A)-receptors could and design, Acquisition of data, Analysis and form a complex with G-Protein-Coupled-Receptors interpretation of data, Drafting the article, Revising (Dopamine-5-Receptor) resulting in a reduced efficacy of it critically for important intellectual content, Final the GABA transmission. approval of the version to be published So a possible explanation for the pharmacodynamic Götz Bosse – Substantial contributions to conception and interactions in our case could be the ability of the design, Acquisition of data, Analysis and interpretation CB1-Receptor and the GABA (A)-Receptor to form of data, Drafting the article, Revising it critically for such a receptor complex resulting in a lower GABA important intellectual content, Final approval of the neurotransmission. A hint for these theory, could be the version to be published fact that in our case no anesthetic - which mediated its Friederike Arlt – Substantial contributions to effect through the GABA (A)-receptor - could provide conception and design, Acquisition of data, Analysis and optimal conditions for intubation. Only when nitrous interpretation of data, Drafting the article, Final approval oxide, which blocks the NMDA-receptor, was provided to of the version to be published our patient, sufficient anesthesia could be achieved and Christian von Heymann – Substantial contributions maintained. to conception and design, Analysis and interpretation It also needs to be mentioned, that plasma and blood of data, Drafting the article, Revising it critically for concentrations of THC in frequent cannabis users (four important intellectual content, Final approval of the times per week a cannabis cigarette with 54 mg THC version to be published content) are detectable up to more than 30 hours after consumption [8]. Moreover it has to be considered, that Guarantor the amount of THC depends on the type of plant species, The corresponding author is the guarantor of submission. cultivation, climate and soil and ranges from 1–20% in herbal cannabis [9, 10]. Therefore, we would recommend, Conflict of Interest that surgical patients refrain from consuming cannabis Authors declare no conflict of interest. as long as possible prior to surgery, since the way of interaction with anesthetic drugs is not safely predictable. Copyright The interaction of THC with anesthetic drugs is complex, © 2015 Georg Richtig et al. This article is distributed possibly resulting in increased sensitivity [4] or greater under the terms of Creative Commons Attribution tolerance to anesthetic drugs, the latter with a higher need License which permits unrestricted use, distribution of anesthetic agents to induce anesthesia. In patients with and reproduction in any medium provided the original greater tolerance of anesthetic drugs nitrous oxide, could author(s) and original publisher are properly credited. be one possible solution to avoid possible interactions Please see the copyright policy on the journal website for between GABA (A)-receptor signaling and THC. Since more information. the mode and the effect of interaction is not predictable history of cannabis intake should be taken into routine preoperative assessment. REFERENCES Limitations of our reported case are, that we did not have any monitoring of depth of anesthesia applied, a 1. Murray RM, Morrison PD, Henquet C, Di Forti M. relatively low fentanyl induction dose (200 µg, 2 µg/kg), Cannabis, the mind and society: the hash realities. no THC blood level measurements and no knowledge of Nat Rev Neurosci. 2007 Nov;8(11):885–95. any genetic mutation of the patients GABAA-receptor. 2. Flisberg P, Paech MJ, Shah T, Ledowski T, Kurowski I, Parsons R. Induction dose of propofol in patients using cannabis. Eur J Anaesthesiol 2009 Mar;26(3):192–5. 3. Brand PA, Paris A, Bein B, et al. Propofol sedation is reduced by delta9-tetrahydrocannabinol in mice. Anesth Analg 2008 Jul;107(1):102–6.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):436–439. Richtig et al. 439 www.ijcasereportsandimages.com

4. Frizza J, Chesher GB, Jackson DM, Malor R, Starmer between dopamine D5 and gamma-aminobutyric acid GA. The effect of delta 9-tetrahydrocannabinol, A receptors. Nature 2000 Jan 20;403(6767):274–80. cannabidiol, and cannabinol on the anaesthesia 8. Desrosiers NA, Himes SK, Scheidweiler KB, induced by various anaesthetic agents in mice. Concheiro-Guisan M, Gorelick DA, Huestis MA. Psychopharmacology (Berl) 1977 Nov 24;55(1):103–7. Phase I and II cannabinoid disposition in blood and 5. Stoelting RK, Martz RC, Gartner J, Creasser plasma of occasional and frequent smokers following C, Brown DJ, Forney RB. Effects of delta-9- controlled smoked cannabis. Clin Chem 2014 tetrahydrocannabinol on halothane MAC in dogs. Apr;60(4):631–43. Anesthesiology. 1973 Jun;38(6):521–4. 9. Adams IB, Martin BR. Cannabis: pharmacology and 6. Symons IE. Cannabis smoking and anaesthesia. toxicology in animals and humans. Addiction 1996 Anaesthesia. 2002 Nov;57(11):1142–3. Nov;91(11):1585–614. 7. Liu F, Wan Q, Pristupa ZB, Yu XM, Wang YT, Niznik 10. Ashton CH. Adverse effects of cannabis and HB. Direct protein-protein coupling enables cross-talk cannabinoids. Br J Anaesth 1999 Oct;83(4):637–49.

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Primary hydatid cyst of gallbladder: A case report

Pronoy Kabiraj, Shib Shankar Kuiri, Utpal De

Abstract INTRODUCTION

Introduction: Hydatid cyst is a common clinical Hydatid cyst in humans is an accidental dead end entity in India. Liver and lungs are the common infestation caused by Echinococcus granulosus. It is organs involved. No organ is immune to infection. endemic in sheep rearing countries. In India, Jammu Case Report: We report a case of primary hydatid and Kashmir is an endemic region though reports of cyst of gallbladder presenting as a gallbladder hydatid disease has been reported from almost whole of lump. Conclusion: In endemic regions hydatid the Indian subcontinent [1]. Liver (75%) and lungs (15%) cyst should be considered in patients presenting are common sites of infection in humans [1, 2]. Primary with gallbladder lump. extrahepatic intra-abdominal hydatid cyst is a rare clinical entity. This article reports and reviews a case of Keywords: Echinococcus granulosus, Echino- primary gallbladder hydatid (Table 1). coccus multilocularis, Gallbladder, Hydatid cyst, Liver, Lungs CASE REPORT How to cite this article A 16-year-old female patient admitted suffering from Kabiraj P, Kuiri SS, De U. Primary hydatid cyst of intermittent mild pain in the right hypochondrium and gallbladder: A case report. Int J Case Rep Images epigastrium associated with nausea since six months. 2015;6(7):440–443. There was no history of fever or jaundice. Physical examination revealed mild tenderness in the right upper quadrant of the abdomen. On deep palpation a globular doi:10.5348/ijcri-201574-CR-10535 lump (4 cmx3 cm), moving with respiration was noted in the right hypochondrium. The upper margin of the lump was impalpable while the other margins were well defined. Murphy’s sign was positive. Base line hematological examination was Pronoy Kabiraj1, Shib Shankar Kuiri2, Utpal De3 unremarkable. Plain abdominal X-ray was normal. The Affiliations: 1MS, Postgraduate Trainee, Department of ultrasound examination showed that her gallbladder was Surgery, Bankura Sammilani Medical Colege, Kenduadih, distended with a localised thickening of its wall. A single Bankura, West Bengal, India; 2MS, Clinical Tutor, Department cystic lesion was noted inside the gallbladder lumen of Surgery, Bankura Sammilani Medical Colege, Kenduadih, 3 giving it an impression of double gallbladder. There was Bankura, West Bengal, India; MS, Professor, Department no image of gallstone. of Surgery, Bankura Sammilani Medical Colege, Kenduadih, Bankura, West Bengal, India. An abdominal CT scan was performed showing inflammatory gallbladder wall with a cyst within cyst Corresponding Author: Dr. Utpal DE, L-4/9, Phase – 3, Dankuni Housing Complex, Dankuni, Hooghly 712311; Ph: appearance (Figure 1). Hepatic parenchyma and other 091-9434156664; Email: [email protected] abdominal organs proved absolutely normal. Anti-Echinococcus antibodies were not found in serum. The diagnosis of primary hydatid cyst of Received: 04 April 2015 gallbladder was made and surgery was decided. The patient Accepted: 28 April 2015 underwent right subcostal laparotomy. Intraoperatively, Published: 01 July 2015 the gallbladder was found to be tense and hugely

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Table 1: Published reports of primary gallbladder hydatid. Serial No Author Title Journal Year Pubmed PMID 1 Pautov FN. A case of primary alveolar Med Parazitol 1964 14250141 echinococcosis of the gallbladder (Mosk). 2 Rigas et al. Primary hydatid cyst of the Br J Surg. 1979 466022 gallbladder.

3 Kapoor et al. Sonographic diagnosis of a J Clin Ultrasound. 2000 10602107 ruptured primary hydatid cyst of the gallbladder.

4 Wani et al. Primary extrahepatic Int J Surg 2005 17462273 abdominal hydatidosis. 5 Pitiakoudis et al. Primary hydatid disease in a J Gastrointestin 2006 17205152 retroplaced gallbladder. Liver Dis. 6 Krasniqi et al. Primary hydatid J Med Case Rep. 2010 20205877 cyst of the gallbladder: a case . report. 7 Mushtaque et al. Atypical Niger J Surg 2012 24027383 localizations of hydatid disease: experience from a single institute.

8 Noomene et al. Primary hydatid Pan Afr Med J. 2013 23504393 cyst of the gallbladder: an unusual localization diagnosed by magnetic resonance imaging (MRI).

distended (Figure 2). The wall appeared oedematous and or meat [1–9]. Cystic transformation of Echinococcus inflamed. After proper precaution the gallbladder wall granulosus in human organs results in varied symptoms. was opened (Figure 3). A single cyst with germinal layer No organ is immune to echinococcal infection [5]. Liver was delivered intact. No other cysts were found in the liver (first filter) and Lungs (second filter) are the commonest and peritoneal cavity. Cholecystectomy was performed. organs involved worldwide. In children, due to unknown It permitted a total removal of the cyst without rupture. cause lung infection (41–70%) is more common than liver A peroperative cholangiography searching daughter (42–48%) [5–7]. Other organs are involved secondary to cysts in the common bile duct proved unremarkable. pulmonary or hepatic involvement. Among the unusual The patient’s postoperative course was uneventful and primary sites, in order of frequency, central nervous she was discharged on tenth postoperative day after system (4–5%), musculoskeletal system (0.5–4%) and stitch removal. The patient was put on albendazole (10 cardiovascular system (0.5-2%) are involved [2, 5, 6, 9]. mg/kg) in divided doses. The histopathology confirmed The incidence of primary extrahepatic intra the presence of hydatid cyst (Figure 4). At sixth month abdominal hydatid cyst is 6.5% [1–9]. Spleen (2–3%) is follow-up, the patient was well and had no recurrence of the predominant organ involved [1, 3–5]. Gallbladder hydatid disease. is primarily involved in 0.6%. GB cysts may be located intraluminally or on the surface [1–9]. As a rule luminal cysts are single and parietal cysts are multiple containing DISCUSSION daughter cyst [2, 5]. Luminal cyst may attain enormous size before they are symptomatic due to distensile Human hydatosis is a zoonosis caused by Echinococcus capacity of the gallbladder. On the contrary parietal cysts granulosus and Echinococcus multilocularis (malignant produce early symptoms. The pathogenesis of gallbladder hydatid). Echinococcus granulosus predominates in infection is not well documented. The hypothetical routes humans. Cattles (sheep, goat, dogs) are primary (definitive, of spread include portal circulation, biliary, lymphatic sexual cycle) host. Human beings are secondary dead end and peritoneum [1–9]. (asexual cycle) host [1–5]. Human infection is caused by Univariate presentation of gallbladder hydatid fecal-oral route or ingestion of contaminated vegetables include acute cholecystitis with cholangitis, gallbladder

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):440–443. Kabiraj et al. 442 www.ijcasereportsandimages.com

Figure 3: Computed tomography scan showing inflammatory gallbladder wall with a cyst within cyst appearance.

Figure 1: Tense and hugely distended gallbladder at laparotomy.

Figure 4: Histopathology of the cyst wall.

lump, obstructive jaundice or peritonitis with shock due to rupture. A combination of the above symptoms is not infrequent. Gallbladder hydatid with daughter cysts in the common duct producing obstructive symptoms may give rise to a false positive Courvoisier’s law [2, 5–9]. So in endemic areas this should be included in the differential diagnosis of patients with obstructive jaundice. Abdominal sonography is highly sensitive and specific for diagnosing hydatid cyst but accurate extrahepatic organ localization is at times doubtful [5, 8, 9]. Computed tomography, and magnetic resonance imaging, are adjuncts to sonography for definitive diagnosis. Serological (ELISA) and hematological (eosinophilia) tests may clinch the diagnosis along with radiology [4, 6–9]. Cystoprostatectomy (cholecystectomy) is the treatment of choice [2–5, 8, 9]. Apart from the usual per- operative precautions gentle handling of the gallbladder Figure 2: View of the hydatid cyst after opening the gallbladder. is of utmost importance. An intraluminal rupture may

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):440–443. Kabiraj et al. 443 www.ijcasereportsandimages.com result in spillage into common bile duct. Manual handling Copyright instead of instrumental (application of Moynihan’s © 2015 Pronoy Kabiraj et al. This article is distributed clamps) holding of the gallbladder during dissection is under the terms of Creative Commons Attribution warranted [1, 7]. Cystic duct should be identified early License which permits unrestricted use, distribution and stay suture passed in order to prevent accidental and reproduction in any medium provided the original spillage of contents into CBD in the event of rupture of author(s) and original publisher are properly credited. cyst. Careful ligation of cystic duct and artery together Please see the copyright policy on the journal website for with removal of gallbladder from liver bed along with a more information. portion of the liver tissue prevents rupture and spillage. Postoperative treatment with albendazole (10–15 mg/ kg) in divided doses should be continued for a period of REFERENCES four months to prevent recurrence [1–9]. 1. Mushtaque M, Mir MF, Malik AA, Arif SH, Khanday SA, Dar RA. Atypical localizations of hydatid disease: CONCLUSION experience from a single institute. Niger J Surg 2012 Jan;18(1):2–7. 2. Noomene R, Ben Maamer A, Bouhafa A, Haoues In endemic countries, hydatid cyst should be N, Oueslati A, Cherif A. Primary hydatid cyst of the considered in the differential diagnosis in patients gallbladder: an unusual localization diagnosed by presenting with gallbladder lump. magnetic resonance imaging (MRI). Pan Afr Med J 2013;14:15. ********* 3. Krasniqi A, Limani D, Gashi-Luci L, Spahija G, Dreshaj IA. Primary hydatid cyst of the gallbladder: a Author Contributions case report. J Med Case Rep 2010 Jan 29;4:29. Pronoy Kabiraj – Substantial contributions to 4. Wani RA, Malik AA, Chowdri NA, Wani KA, Naqash conception and design, Acquisition of data, Analysis and SH. Primary extrahepatic abdominal hydatidosis. Int J Surg 2005;3(2):125–7. interpretation of data, Revising it critically for important 5. Pitiakoudis MS, Tsaroucha AK, Deftereos S, Laftsidis intellectual content, Final approval of the version to be P, Prassopoulos P, Simopoulos CE. Primary hydatid published disease in a retroplaced gallbladder. J Gastrointestin Shib Shankar Kuiri – Substantial contributions to Liver Dis 2006 Dec;15(4):383–5. conception and design, Drafting the article, Critical 6. Safioleas M, Stamoulis I, Theocharis S, Moulakakis revision of the article, Final approval of the version to be K, Makris S, Kostakis A. Primary hydatid disease of published the gallbladder: a rare clinical entity. J Hepatobiliary Utpal De – Substantial contributions to conception and Pancreat Surg 2004;11(5):352–6. design, Revising it critically for important intellectual 7. Kapoor A, Sarma D, Gandhi D. Sonographic diagnosis of a ruptured primary hydatid cyst of the gallbladder. content, Final approval of the version to be published J Clin Ultrasound 2000 Jan;28(1):51–2. 8. Cangiotti L, Muiesan P, Begni A, et al. Unusual Guarantor localizations of hydatid disease: a 18 year experience. The corresponding author is the guarantor of submission. G Chir 1994 Mar;15(3):83–6. 9. Rigas AM, Karatzas GM, Markidis NC, Bonikos DS, Conflict of Interest Sotiropoulou GG, Skalkeas G. Primary hydatid cyst of Authors declare no conflict of interest. the gallbladder. Br J Surg 1979 Jun;66(6):406.

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Occult hip fracture diagnosed by MRI scan after inconclusive X-ray and CT scan

Bogdan Deleanu, Radu Prejbeanu, Florin Birsasteanu, Dinu Vermesan, Liviu-Ionut Micle, Eleftherios Tsiridis, Vlad Predescu

Abstract prosthesis on the affected hip considering the age and the condition of the patient. Conclusion: Introduction: Hip fractures are common, most Occult hip fractures should be suspicioned in all of them being easily diagnosed with the clinical elderly patients who have a history of hip trauma examination and X-ray. Few cases need a even if X-ray and CT scan do not reveal a fracture. computed tomography (CT) scan and even fewer If it’s available, the use of MRI scan is the best an magnetic resonance imaging (MRI) scan. Here option for diagnose of occult hip fractures. we report a case that was diagnosed with occult femoral neck fracture only after undergoing a Keywords: CT-scan, MRI-scan, Occult hip frac- pelvic MRI scan. Case Report: The patient has tures presented a normal X-ray at the first exam with minimal clinical signs. The emergency CT scan How to cite this article shown no injury and the patient was left home. Three days after, the patient returned to hospital Deleanu B, Prejbeanu R, Birsasteanu F, Vermesan D, with increased pain and limited mobility to the left Micle CI, Tsiridis E, Predescu V. Occult hip fracture hip. Following the emergency MRI scan a femoral diagnosed by MRI scan after inconclusive X-ray and neck fracture has been revealed. The patient was CT scan. Int J Case Rep Images 2015;6(7):444–447. soon operated and we used a total hip cemented doi:10.5348/ijcri-201575-CR-10536 Bogdan Deleanu1, Radu Prejbeanu2, Florin Birsasteanu3, Dinu Vermesan1, Liviu-Ionut Micle4, Eleftherios Tsiridis5, Vlad Predescu6 Affiliations: 1Assistant Professor, I-st Clinic of Orthopedics and Trauma, University of Medicine and Pharmacy ‘Victor INTRODUCTION Babes’, Timisoara, Romania; 2Professor, I-st Clinic of Orthopedics and Trauma, University of Medicine and Hip fractures are one of the most frequent types of Pharmacy ‘Victor Babes’, Timisoara, Romania; 3Associate fractures seen in orthopedic practice [1, 2]. Considering Professor, Department of Radiology, University of Medicine that life expectancy increased more and more in the and Pharmacy ‘Victor Babes’, Timisoara, Romania; 4Resident past few decades the elderly population increased too Doctor, I-st Clinic of Orthopedics and Trauma, University of [3], so much more attention is needed to properly Medicine and Pharmacy ‘Victor Babes’, Timisoara, Romania; diagnose hip fractures which occur more often in this 5 Associate Professor, Imperial College London Medical segment of population [4]. Standard anterior-posterior School; Aristotle University Medical School, Thessaloniki, X-ray may help us to diagnose displaced fractures but Greece; 6Associate Professor, St. Pantelimon’ Emergency Hospital, ‘Carol Davila’ University of Medicine and Pharmacy, in non-displaced fractures a CT scan or an MRI scan Bucharest, Romania. is recommended [5, 6]. Studies show that occult hip Corresponding Author: Micle Liviu-Ionut, Str Corneliu Coposu, fractures represent 2–10% of total hip fractures [5, 7], Nr 20, sc A, Ap 29, Timisoara, Romania. Ph: +40769870430 and the MRI scan has the best accuracy in detecting Email: [email protected] these fractures [5–9]. It is considered that late diagnose of occult hip fractures occur in 2–9% of the cases [10]. Holder et al. showed that CT scan performed in less Received: 29 January 2015 than 3 days after trauma has 93% sensitivity and 95% Accepted: 11 March 2015 specificity, so there is a significant percent of occult hip Published: 01 July 2015 fractures missed by CT scan [11, 12].

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Here we report a case of occult femoral fracture that was diagnosed using a pelvic MRI scan 3 days after an X-ray and a CT scan shown no injury. The purpose is to show the importance of MRI scan in diagnosing occult hip fractures.

CASE REPORT

A 72-year-old female had a history of left hip trauma after tripping and falling at home. She was brought immediately to the emergency department and an anterior-posterior hip X-ray was done (Figure 1). As seen in the X-ray there was no sign of fracture but the clinical examination revealed pain at the left hip and limited mobility. Thus, considering the age of the patient, a pelvic CT scan was requested which also revealed no injury to the left hip (Figure 2). The patient was discharged and left home with anti-inflammatory and pain medication, being programmed to return after 7 days for a follow-up. After three days the patient returned to emergency department with increased pain to the left hip and almost no active mobility possible to the affected hip. We decided to do a pelvic MRI scan which revealed an occult left femoral neck fracture (Figure 3). The second day the patient was operated. We have chosen a hip direct lateral approach (Hardinge) with the patient in supine position. Considering the age of the patient and her associated conditions we opted for a cemented total hip arthroplasty in order to have the Figure 1: X-ray at the first presentation showing no fracture. best results (Figure 4). The second day postoperative the patient started rehabilitation with bed side exercises and at third day postoperatively weight bearing walk was possible. Follow-up at 45th day, the patient had an overall good recovery with a hip Harris score of 78.

DISCUSSION

The best imaging option for diagnosing hip fractures is still a subject of discussion. Obviously this discussion applies to the undisplaced and occult fractures. X-ray exam is a primary investigation used in pre- operative planning but by definition an occult hip fracture is not visible on X-ray exam so using only this exam is not helpful in diagnosing such cases. Due to increased cost and limited availability in some hospitals the MRI-scan is not a primary imaging option, as seen in this case. Studies show that MRI scan has the best sensitivity Figure 2: Computed tomography scan at the first presentation showing no fracture and it should be used as a primary choice for diagnosing this type of fractures [6–9, 13]. Both orthopedic surgeons and radiologists agree that MRI scan is the gold standard inconclusive or can give false-negative results. The CT in diagnosing occult hip fractures [5–9, 13]. It is scan has the advantages to be widely available with a recommended that MRI scan to be done in the first 24 lower cost comparative to MRI scan [5, 14], but even so hours after the hip trauma. If not available, a 72-hour CT the MRI is preferable. The advantages of MRI scan over scan or MRI scan is recommended. CT scan are the better sensitivity (100%) and no exposure CT scan is another imaging option which can be to harmful radiation [15]. The literature also mentions the helpful but as seen in this case the results may be bone scintigraphy using technetium polyphosphate with

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available remains the MRI scan and it should be the first to be used in cases with hip trauma and no X-ray signs, especially in the elderly population. Preferably the MRI should be performed in the first 24 hours after trauma to decrease the complications and obtain the best curative results.

*********

Author Contributions Bogdan Deleanu – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Radu Prejbeanu – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be Figure 3: Magnetic resonance imaging scan at 3rd day after published trauma showing femoral neck fracture. Florin Birsasteanu – Analysis and interpretation of data, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published Dinu Vermesan – Analysis and interpretation of data, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published Liviu-Ionut Micle – Analysis and interpretation of data, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published Eleftherios Tsiridis – Analysis and interpretation of data, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published Vlad Predescu – Analysis and interpretation of data, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published

Guarantor The corresponding author is the guarantor of submission.

Conflict of Interest Figure 4: The postoperative recovery was good and at sixth day the patient was discharged. Authors declare no conflict of interest. a high sensitivity in detecting occult fractures (98%) but Copyright this technique is less available and with limited reliability © 2015 Bogdan Deleanu et al. This article is distributed [16]. Another useful option is the sonography which is under the terms of Creative Commons Attribution mentioned to have 100% sensibility and 60% specificity License which permits unrestricted use, distribution compared to MRI [17]. and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for CONCLUSION more information.

Occult hip fractures can easily be under-diagnosed or missed using X-ray and even a CT scan. The best option

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REFERENCES 11. Levitsky KA, Hozack WJ, Balderston RA, et al. Evaluation of the painful prosthetic joint. Relative 1. Ballane G, Cauley JA, Luckey MM, Fuleihan Gel-H. value of bone scan, sedimentation rate and joint Secular trends in hip fractures worldwide: opposing aspiration. J Arthroplasty 1991 Sep;6(3):237–44. trends East versus West. J Bone Miner Res 2014 12. Guyton JL. Intertrochanteric femoral fractures. Aug;29(8):1745–55. Fractures of hip, acetabulum and pelvis. In: 2. Azagra R, López-Expósito F, Martin-Sánchez JC, et al. Campbell’s Operative Orthopaedics. Ninth edition. Changing trends in the epidemiology of hip fracture Mosby 1998;3:2181–208. in Spain. Osteoporos Int. 2014 Apr;25(4):1267–74. 13. Hakkarinen DK, Banh KV, Hendey GW. Magnetic 3. Liu P, Li C, Wang Y, et al. The impact of the major resonance imaging identifies occult hip fractures causes of death on life expectancy in China: a 60-year missed by 64-slice computed tomography. J Emerg longitudinal study. BMC Public Health 2014 Nov Med 2012 Aug;43(2):303–7. 20;14:1193. 14. Jordan RW, Dickenson E, Baraza N, Srinivasan K. 4. Cooper C, Cole ZA, Holroyd CR, et al. Secular trends in Who is more accurate in the diagnosis of neck of the incidence of hip and other osteoporotic fractures. femur fractures, radiologists or orthopaedic trainees? Osteoporos Int 2011 May;22(5):1277–88. Skeletal Radiol 2013 Feb;42(2):173–6. 5. Gill SK, Smith J, Fox R, Chesser TJ. Investigation 15. Israel GM, Cicchiello L, Brink J, Huda W. Patient of occult hip fractures: The use of CT and MRI. Size and Radiation Exposure in Thoracic, Pelvic, ScientificWorldJournal 2013;2013:830319. and Abdominal CT Examinations Performed With 6. Nachtrab O, Cassar-Pullicino VN, Lalam R, Tins B, Automatic Exposure Control. AJR Am J Roentgenol Tyrrell PN, Singh J. Role of MRI in hip fractures, 2010 Dec;195(6):1342–6. including stress fractures, occult fractures, avulsion 16. Brunner LC, Eshilian-Oates L, Kuo TY. Hip fractures fractures. Eur J Radiol 2012 Dec;81(12):3813–23. in adults. Am Fam Physician 2003 Feb 1;67(3):537– 7. Lim AK. Occult Hip Fractures in Elderly Patients: Is 42. CT Enough? 2013:1–2. 17. Safran O, Goldman V, Applbaum Y, et al. 8. Cannon J, Silvestri S, Munro M. Imaging choices in Posttraumatic painful hip: sonography as a screening occult hip fracture. J Emerg Med 2009 Aug;37(2):144– test for occult hip fractures. J Ultrasound Med 2009 52. Nov;28(11):1447–52. 9. Pandey R, McNally E, Ali A, Bulstrode C. The role of MRI in the diagnosis of occult hip fractures. Injury 1998 Jan;29(1):61–3. 10. Gangopadhyay S, Akra GA, Nanu AM. Occult hip fractures in the elderly: A protocol for management. Eur J OrthopSurgTraumatol 2007;17:153–6.

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CASECase REPORTreport Peer Reviewed OPEN| OPEN ACCESS ACCESS Intramedullary spinal cord abscess by Nocardia: A case report

Khaled Alshabani, Joseph Adrian L. Buensalido, Milagros P. Reyes, Ayman O. Soubani

Abstract months. Discussion: Nocardia species are able to cause different kinds of disease in man. Spinal Introduction: Nocardia is a Gram-positive cord abscess are extremely rare and high index of bacteria that usually cause opportunistic clinic suspicion is required for diagnosis. Initial infections but can affect the immunocompetent combination intravenous antibiotics treatment host. The central nervous system can be the is essential and should be continued for at least primary site of infection in up to 40% of the three to six weeks. Treatment can be switched to cases but the involvement of the spinal cord is oral and continued for a minimum of six months. extremely rare. Case Report: We present a case Surgical intervention might be indicated in of a middle age man with history of alcoholic liver some patients. 16s rRNA gene sequencing allows cirrhosis and diabetes mellitus who presented earlier identification and thus adjustment of with acute lower back pain that is associated antibiotics. Conclusion: This report illustrates with right leg weakness and numbness. Magnetic that a diagnosis can be made by neuroimaging resonance imaging (MRI) scan showed a spinal and cerebrospinal fluid (CSF) 16s ribosomal cord intramedullary abscess. The cerebrospinal RNA sequencing, allowing early and effective fluid analysis was consistent with meningitis and antibiotic therapy and obviating the need for a the organism was eventually identified with CSF high-risk neurosurgical intervention. culture as Nocardia Farcinica. Patient was treated with prolonged intravenous trimethoprim/ Keywords: Gram-positive bacteria, Intramedul- sulfamethoxazole and meropenem. No lary abscess, Nocardia, Spinal cord neurosurgical intervention was done and the patient had full neurological recovery in few How to cite this article

Khaled Alshabani1, Joseph Adrian L. Buensalido2, Milagros P. Alshabani K, Buensalido JAL, Reyes MP, Soubani AO. Reyes3, Ayman O. Soubani4 Intramedullary spinal cord abscess by Nocardia: A case Affiliations: 1MD, Wayne State University, Department of report. Int J Case Rep Images 2015;6(7):448–453. Internal Medicine, Detroit, MI, USA; 2MD, Wayne State University, Department of Internal Medicine, Division doi:10.5348/ijcri-201576-CR-10537 of Infectious Diseases, Current affiliation: University of the Philippines - Philippine General Hospital, Section of Infectious Diseases, Manila, Philippines; 3MD, FACP, FIDSA, Professor of Medicine, Wayne State University, Department of Internal Medicine, Division of Infectious Diseases, Detroit, MI, USA; 4MD, Professor of Medicine, Department of Internal INTRODUCTION Medicine, Division of Pulmonary and Critical Care Medicine, Detroit, MI, USA. Nocardia is known to be ubiquitous in the Corresponding Author: Khaled Alshabani, 4201 St. Antoine, environment and is able to cause different kinds of Suite 2E, Detroit, MI 48201, USA; Tel: 1-313-725-0204; diseases in man, including skin and soft tissue infections, Email: [email protected] pneumonia, central nervous system (CNS) infection and bacteremia [1]. Nocardia infections commonly affect Received: 17 April 2015 immunocompromised patients and those who are on Accepted: 23 May 2015 corticosteroid therapy. The lungs are usually the first Published: 01 July 2015 organs that are infected, but the CNS eventually becomes

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):448–453. Alshabani et al. 449 www.ijcasereportsandimages.com infected in around 40% of cases if the primary infection is left untreated [2]. Brain abscess formation is the most common presentation of Nocardia CNS infection. Spinal cord involvement with intramedullary abscess formation is exceedingly rare. We present a rare case of such a neurological involvement as the initial presentation of Nocardia infection. We also review literature (Table 1) for this rare presentation [3–12].

CASE REPORT

A 53-year-old Caucasian male with past medical history of alcoholic liver cirrhosis, diabetes mellitus, and hypertension presented to the emergency department with acute onset lower back pain and inability to walk for two days. He also had numbness and weakness in the right leg, urinary retention and constipation. He had not had preceding respiratory or gastrointestinal illnesses or any sick contact. Physical examination was remarkable for weakness in the lower extremities that was worse on the right side, diminished pinprick and vibration sensation Figure 1: Magnetic resonance imaging scan of lumbar spine below the mid-thighs bilaterally, and hyporeflexia in both showing progression of signal abnormality and enhancement legs. He had mild tenderness over the lower lumbar spine. consistent with intramedullary abscess (arrow). Initial blood tests included complete blood count, basic metabolic profile, Erythrocyte sedimentation rate, and C-reactive protein were all within normal limits discharged to a subacute rehabilitation center on IV except for thrombocytopenia. antibiotics. A six-month follow-up MRI scan showed The patient was admitted to the general medical resolution of the intramedullary abscess. floor and started on intravenous (IV) dexamethasone. Computed tomography (CT) scan of the spine showed no cord compression. However, given the high clinical DISCUSSION suspicion, a magnetic resonance imaging (MRI) showed a signal abnormality and enhancement of the spinal Nocardia is a Gram-positive bacteria of the cord, including the conus medullaris, consistent with actinomycetes group. It is responsible for nocardiosis, an intramedullary abscess (Figure 1). uncommon infection. Nocardia species can cause localized A lumbar puncture was performed and cerebrospinal or systemic disease usually in immunocompromised fluid analysis (CSF) showed a red blood cell (RBC) count patients as an opportunistic infection, but one third of the of 1621 RBCs/µL, nucleated cells of 4822 WBCs/µL with cases occur in healthy individuals [13]. neutrophilic predominance (84% neutrophils), protein of Nocardia is defined as a complex that includes N. 306 mg/dL, and glucose < 1 mg/dL. The Gram stain was asteroides sensu stricto, N. farcinica, N. nova, and N. negative. The patient was then started on broad spectrum transvalensis complex. Nocardia species are not part of IV antibiotics. the human flora. They are known to be ubiquitous in the The initial CSF culture showed Gram-positive bacilli environment [1]. Inhalation is the most common mode of partially acid fast with some branching. Mycobacterium entry and that explains the lung involvement in a majority tuberculosis PCR was negative and patient was initiated of the infections. It can also result from trauma-related on intravenous trimethoprim/sulfamethoxazole (TMP/ introduction of the organism [12]. SMX) and meropenem for Nocardia double coverage. Immunosuppression is a major risk factor for attracting Patient was evaluated by neurosurgery and it was the disease. HIV, malignancies, corticosteroid therapy, decided that it was in his best interest not to intervene organ and hematopoietic stem cell transplantation are on the abscess as the risks outweighed the benefits. On the most common causes of immunosuppression leading day 10 of admission, and using 16s ribosomal RNA gene to Nocardia infections. Diabetes mellitus is a prominent sequencing, the organism was identified as N. farcinica. risk factor. Alcoholism and COPD are important risk The CSF culture was finalized on day 20 of his factors for pulmonary nocardiosis [13]. admission and showed Nocardia farcinica, sensitive to Nocardia species are able to cause different kinds of TMP/SMX, amikacin, and imipenem, but resistant to disease in man, including skin and soft tissue infections, ceftriaxone. Patient weakness started to improve slowly pneumonia, CNS infection and bacteremia. The most towards the end of his admission. He was eventually common site of primary infection is the lung (up to

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70%) [5]. CNS involvement is common in disseminated with Nocardia treatment. Table 1 summarizes the main nocardiosis, but can also be the primary site [9]. CNS features of these cases. involvement can also occur in up to 40% of untreated Antimicrobial susceptibility patterns are variable primary lung lesions [2]. Brain abscess is the most between different studies, countries and species. common presentation of CNS nocardiosis [10]. Nocardia Therefore, formal antimicrobial susceptibility testing epidural abscesses have also been described. is always necessary to ensure optimal therapy. There are Spinal cord involvement with intramedullary abscess rapid tests that can identify the organism much faster formation is exceedingly rare and only ten cases have than culture and knowing the organism itself with these been reported in literature [3–12]. Our case is number 11. tests, can allow us to pattern our two empiric antibiotics Yenrudi et al. reported a case of disseminated Nocardia, by relying on studies that show the susceptibility profiles but we excluded it because the spinal cord involvement of specific species [1]. 16s ribosomal RNA gene sequencing was secondary to thromboses of several blood vessels is also a quicker method for coming up with the specific supplying the spinal cord resulting in widespread species of Nocardia, and thus allowing an earlier and myelomalacia and not by direct infection by the organism improved empiric therapy while cultures are pending [14]. Of these cases, six patients were males. The mean age [15], as we did in our case. was 58.27 years (SD +/- 16.11). Comorbid conditions were Treatments strategies are based on cumulative reported in seven cases besides our patient, and included retrospective experience, as well as in vitro studies, as no hypertension, diabetes mellitus (in four patients), end- prospective randomized trials have been performed to stage renal disease, renal transplant, liver cirrhosis, determine the most effective treatment. Given the variable chronic obstructive pulmonary disease, and ulcerative antibiotics resistance of Nocardia, empiric coverage with colitis. One patient was mistakenly diagnosed with two or three agents in a patient with severe disease is allergic alveolitis, but her symptoms were secondary to warranted. TMP/SMX is a first line and standard therapy the pulmonary nocardiosis as he improved dramatically and should be included in these regimens. Combination

Table 1: Characteristics and summary of Nocardia intramedullary spinal cord abscess reported in literature Author and Patient’s age Extension of Type of Diagnosis Treatment Outcome year reported and gender nocardiosis Nocardia Welsh [3] 38 y/o female Disseminated: N. asteroides Sputum and PO Sulfadiazine+ IM Died on 1957 lungs, kidneys, cutaneous streptomycin hospital pancreas, abscess culture day 20 abdominal + autopsy wall, brain and spinal cord Kampfl [4] 63 y/o male Primary CNS: N. asteroides CSF consistent TMP/SMX + amikacin Partial 1992 multiple with bacterial recovery cerebral and meningitis + 3 months cervical spinal high titers of after cord abscesses serum Nocardia discharge IgG + complete resolution of radiological findings and partial clinical recovery with treatment Hiller [5] 56 y/o female Disseminated: N. asteroides Spinal cord IV TMP/SMX Partial 1999 Lung + brain biopsy recovery + single spinal (consistent with in two cord lesion abscess) + months BAL Culture positive for Nocardia Mehta [6] 30 y/o female Disseminated: Not speciated CSF suggestive TMP/SMX + Partial 1999 Lung + Skin + of aseptic amoxicillin/clavulanic recovery in single spinal meningitis acid + amikacin + 20 days cord lesion + cutaneous clarithromycin abscess culture

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Table 1: (Continued) Author and Patient’s age Extension of Type of Diagnosis Treatment Outcome year reported and gender nocardiosis Nocardia Mukunda [7] 52 y/o male Primary CNS: N. asteroides Laminectomy IV TMP/SMX + Full 1999 single spinal for abscess imipenem recovery in cord lesion aspiration and 16 months culture Durmaz [8] 59 y/o male Primary CNS: N. asteroides Craniotomy / TMP/SMX + amikacin + Died on 2000 Brain and laminectomy ceftriaxone hospital spinal cord for abscess day-12 lesions aspiration and culture Hussain [9] 75 y/o female Primary CNS: N. asteroides Laminectomy IV TMP/SMX + Partial 2004 multi-loculated for abscess cefotaxime recovery in lesions aspiration and 10 days culture Samkoff [10] 79 y/o male Disseminated: N. farcinica Laminectomy IV TMP/SMX Partial 2008 lung + cervical for abscess recovery spinal cord aspiration and in 10 days single lesion culture but died at 8 weeks from cardiac arrest Lee [11] 82 y/o male Primary CNS; N. Laminectomy IV meropenem followed Partial 2010 single spinal cyriaciagerogica for abscess by IV TMP/SMX recovery in cord lesion aspiration and 7 weeks culture Hong [12] 54 y/o female Primary CNS; N. nova Laminectomy amikacin + cefotaxime + Full 2012 multiple spinal for abscess vancomycin followed by recovery in cord abscess aspiration amikacin+ cefotaxime one year and culture + 16s rRNA sequencing Current report 53 y/o male Primary CNS: N. farcinica CSF culture IV TMP/SMX + Partial meningitis + meropenem recovery at single spinal 3rd weeks lesion † y/o years old therapy is warranted in severe infections, defined as High index of suspicion of Nocardia infection is CNS involvement, disseminated disease, and infections necessary in patients with suggestive neuroimaging. involving more than one site in immunocompromised Spinal tap and stains and cultures for Nocardia are patients. Agents available for combination therapy include important part of the work up, but 16s rRNA allows earlier amikacin, imipenem, meropenem, third generation identification of the specific species, allowing adjustment cephalosporins, minocycline, extended spectrum of antibiotics, given the distinctly variable antibiotic fluoroquinolones (e.g., moxifloxacin), tigecycline, and susceptibilities of Nocardia species. A multidisciplinary dapsone [1, 16]. approach that includes infectious disease, neurosurgical Initial treatment should be administered intravenously and neurological specialists is essential for early diagnosis for at least three to six weeks and/or until clinical and appropriate management of these patients. improvement is documented. Treatment can be switched to oral and continued for a minimum of six months and up to 12 months in immunocompromised patients and CONCLUSION those with CNS involvement. Surgical intervention might be indicated in some This report describes a rare case of Nocardia situations, such as abscesses that do not respond to intramedullary spinal abscess. It illustrates that a antibiotic therapy [12]. Prognosis is good in general, even diagnosis can be made by neuroimaging and CSF in disseminated cases, if therapy is initiated early and 16s ribosomal RNA, with subsequent CSF culture continued for appropriate duration. confirmation. Appropriate antibiotic therapy was

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):448–453. Alshabani et al. 452 www.ijcasereportsandimages.com effective and resulted in significant recovery and obviated 6. Mehta RS, Jain D, Chitnis DS. Nocardial abscess of the need for a high-risk neurosurgical intervention. spinal cord. Neurol India 1999 Sep;47(3):243–4. 7. Mukunda BN, Shekar R, Bass S. Solitary spinal ********* intramedullary abscess caused by Nocardia asteroides. South Med J 1999 Dec;92(12):1223–4. 8. Durmaz R, Atasoy MA, Durmaz G, et al. Multiple Author Contributions nocardial abscesses of cerebrum, cerebellum and Khaled Alshabani – Substantial contributions to spinal cord, causing quadriplegia. Clin Neurol conception and design, Acquisition of date, Analysis Neurosurg 2001 Apr;103(1):59–62. and interpretation of date, Drafting the article, Revising 9. Hussain N, Srinivasan A, Paquette S, Agbi C, Lum it critically for important intellectual content, Final C. Neuroimaging highlight. MRI of spinal nocardial approval of the version to be published abscess. Can J Neurol Sci 2004 Aug;31(3):404–5. Joseph Adrian L. Buensalido – Substantial contributions 10. Samkoff LM, Monajati A, Shapiro JL. Teaching to conception and design, Acquisition of date, analysis NeuroImage: nocardial intramedullary spinal cord abscess. Neurology 2008 Jul 15;71(3):e5. and interpretation of date, Drafting the article, Revising 11. Lee J, Whitby M, Hall BI. Nocardia cyriacigeorgica it critically for important intellectual content, Final abscess of the conus medullaris in an approval of the version to be published immunocompetent host. J Clin Neurosci 2010 Milagros P. Reyes – Substantial contributions to Sep;17(9):1194–5. conception and design, Analysis and interpretation 12. Hong SB, Han K, Son BR, Shin KS, Rim BC. of date, Revising the article critically for important First case of Nocardia nova spinal abscess in an intellectual content, Final approval of the version to be immunocompetent patient. Braz J Infect Dis 2012 published Mar-Apr;16(2):196–9. Ayman O. Soubani – Substantial contributions to 13. Sorrell TC, Mitchell DH, Iredell JR, Chen SC-A. Nocardia Species. In: Principles and Practice of conception and design, Analysis and interpretation Infectious Diseases, 7, Mandell GL, Bennett JE, Dolin of date, Revising the article critically for important R (Eds), Churchill Livingstone Elsevier, Philadelphia; intellectual content, Final approval of the version to be 2010. p. 3199. published 14. Yenrudi S, Shuangshoti S, Pupaibul K. Nocardiosis: report of 2 cases with review of literature in Thailand. Guarantor J Med Assoc Thai 1991 Jan;74(1):47–54. The corresponding author is the guarantor of submission. 15. Angeles RM, Lasala RP, Fanning CV. Disseminated subcutaneous nocardiosis caused by Nocardia Conflict of Interest farcinica diagnosed by FNA biopsy and 16S ribosomal gene sequencing. Diagn Cytopathol 2008 Authors declare no conflict of interest. Apr;36(4):266–9. 16. Vera-Cabrera L, Gonzalez E, Choi SH, Welsh O. In Copyright vitro activities of new antimicrobials against Nocardia © 2015 Khaled Alshabani et al. This article is distributed brasiliensis. Antimicrob Agents Chemother 2004 under the terms of Creative Commons Attribution Feb;48(2):602–4. License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.

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1. Schlaberg R, Fisher MA, Hanson KE. Susceptibility profiles of Nocardia isolates based on current taxonomy. Antimicrob Agents Chemother 2014;58(2):795–800. 2. Radhakrishnan K, Heaton D. Disseminated nocardiosis. Intern Med J 2008 Sep;38(9):740. 3. Welsh JD, Rhoades ER, Jaques W. Disseminated nocardiosis involving spinal cord. Case report. Arch Intern Med 1961 Jul;108:73–9. 4. Kampfl A, Auckenthaler A, Berek K, et al. [Cerebral and spinal nocardia infection. A case report]. Wien Klin Wochenschr 1992;104(6):174–7. 5. Hiller R, Singh H, Crone M. Left leg paralysis in a renal transplant. Am J Kidney Dis 1999 Jan;33(1):E4.

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CASEClinica REPORTl Imag es Peer Reviewed OPEN| OPEN ACCESS ACCESS Perianal aggressive angiomyxoma in a male patient

Zainab Taha ALHumoud, Najla Aldaoud, Hussain Abrar, Amro Salem

Case Report cut surface (Figure 3). Microscopically, it showed an ill- defined tumor composed of small spindle cells embedded A 45-year-old male presented with a swelling in in an abundant myxoid stroma interspersed with a thick the perianal area that had begun to grow four months wall and hyalinized blood vessels. There was no evidence previously. He complained of pain, and problems of mitotic activity or nuclear atypia (Figure 4A–C). Based defecating. Physical examination located a soft lump in the on these histological features, a diagnosis of aggressive perianal area, with ill-define edges near to the posterior angiomyxoma (AAM) was confirmed. midline (Figure 1). Although it is a rare condition, one of our preliminary differential diagnoses was lipomas. Other differential diagnosis was possible Desmoids Type DISCUSSION Tumor. Magnetic resonance imaging (MRI) scan of the pelvis Aggressive angiomyxoma (AAM) is a rare benign soft showed a large flask shaped mass sagging from the tissue tumor of mesenchymal origin, characterized by its perineum, with fat lobules apparently originating from perineum, extending into the intergluteal cleft. There was concern about the swirled appearance noted post contrast study, which suggested possible diagnosis of a myxoid type tumor and less likely a lipoma (Figure 2A–B). Surgical excision of the mass was performed. The gross appearance of the specimen was a disoriented mushroom shaped mass, about 380 grams in weight, with a fatty

Zainab Taha ALHumoud1, Najla Aldaoud2, Hussain Abrar3, Amro Salem4 Affiliations: 1MD, Postgraduate Physician, Department of General Surgery, King Fahad Specialist Hospital in Dammam, Saudi Arabia; 2MD, JBP, EBP, Assistant Professor, Medical School, Jordan University of Science and Technology, Irbid, Jordan, Consultant Pathology, King Abdullah University Hospital, Irbid, Jordan; 3FRCS, Consultant Plastic Surgeon, Department of Plastic Surgery, King Fahad Specialist Hospital in Dammam, Saudi Arabia; 4MSc, FRCS, Consultant Colorectal Surgeon, Department of Colorectal Surgery, King Fahad Specialist Hospital in Dammam, Saudi Arabia. Corresponding Author: Zainab Taha ALHumoud, Saihat, B.O.BOX, 1977, Eastern Province Postal Code: 31972, Saudi Arabia; Mob: +966551104300, Fax: 0138372323; Email: [email protected]

Received: 20 February 2015 Figure 1: Gross appearance of soft lump in the perianal area, Accepted: 21 March 2015 adjacent to posterior midline with ill-defined edges and about Published: 01 July 2015 28 x 22 cm.

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locally infiltrative nature and high recurrence rate. It was given this name due to the pathological findings of the stellate and spindled cells, along with variable-sized blood vessels, which were intricately entwined within a myxoid matrix [1]. This presentation occurs predominantly in woman of reproductive age (female to male ratio of approximately 6:1), and exclusively in the pelvi-perianal region. To our knowledge, only 43 cases occurring in men have been reported in literature [1]. In men, AAM usually involves the scrotum (38%), spermatic cord (33%), and Figure 2: Magnetic resonance imaging (MRI) scan of pelvis (A) perineal region (13%) [2]. Coronal section T1 W image showing Large Flask shaped mass Although clinical diagnosis of AAM may be difficult, the sagging from Perineum and measures 29 x 22 x 17 cm. MRI of typical MRI features of AAM are swirled strands, aligned pelvis, (B) Sagittal Section image T2 W image : Swirls of T2 with the craniocaudal axis. This particular imaging feature hypointensity is noted within the Mass which is characteristic is caused by a stretching of the fibrovascular stroma [3]. of AAM (arrow) . Confirmation of diagnosis is based on a histopathology study. AAM should be distinguished from other benign, potential lesions with low local recurrence, and malignant tumors with widespread metastatic potential. Surgery is the mainstay of treatment for AAM, although achieving negative resection margins is complicated, because of the infiltrative nature of the tumor. Local recurrence rate falls between 36% and 72% and is usually seen in the first three years. Recurrences of the disease are usually controlled with surgery [1]. Several reported attempts at using chemotherapy and radiotherapy as part of the treatment protocol for AAM have proved disappointing; probably due to the low mitotic activity/ growth fraction of cells. Primary treatment with GnRH agonists has proved successful, as many of angiomyxoma are positive for estrogen and progesterone receptors [4]. Figure 3: Gross appearance of the specimen showing Disoriented However, the duration of the responses and an optimal mushroom shaped mass, and about 380 grams in weight, with treatment schedule are still unknown [1]. a fatty cut surface.

CONCLUSION

In conclusion, whether the treatment of aggressive angiomyxoma (AAM) is surgery, hormonal therapy or a combination of the two, it is apparent that adequate management of AAM necessitates close, and long- term follow-up at sixth month intervals, to monitor for recurrence. Multiple relapses can occur but metastases are unusual.

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ALHumoud ZT, Aldaoud N, Abrar H, Salem A. Perianal aggressive angiomyxoma in a male patient. Int J Case Rep Images 2015;6(7):454–456.

Figure 4: (A) An ill-defined tumor composed of oval to spindle doi:10.5348/ijcri-201522-CL-10077 shaped cells lacking nuclear atypia with no mitotic activity, (B) A characteristic features of aggressive angiomyxoma is the appearance of lesional cells spinning off a vessel that they ********* encircle (arrow), (C) Note the thick-walled larger vessels (H&E stain, x100).

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):454–456. ALHumoud et al. 456 www.ijcasereportsandimages.com

Acknowledgements REFERENCES We would like to thank Dr. Thabet Ghazal who was involved in the Management of the case. 1. Rocco F, Cozzi G, Spinelli MG, Rocco BM, Albo G, Finkelberg E, Oliva I, et.al. Massive recurring Author Contributions angiomyxoma of the scrotum in an obese man. Rare Tumors 2011;volume 3:e31. Zainab Taha ALHumoud – Substantial contributions to 2. Morag R, Fridman E, Mor Y. Aggressive angiomyxoma conception and design, Acquisition of data, Drafting the of the scrotum mimicking huge hydrocele: case report article, Revising it critically for important intellectual and literature review. Case Rep Med. 2009;volume content, Final approval of the version to be published 2009: 157624. Najla Aldaoud – Substantial contributions to conception 3. Karwacki GM, Stöckli M, Kettelhack C, Mengiardi B, and designm, Drafting the article, Final approval of the Studler U. Radiographic diagnosis and differentiation version to be published of an aggressive angiomyxoma in a male patient. J Hussain Abrar – Substantial contributions to conception Radiol Case Rep. 2013 Jul;7(7): 1–6. and design, Drafting the article, Final approval of the 4. Seema Narang, Supreethi Kohli, Vinod Kumar, Raj Chandoke. Aggressive Angiomyxoma with Perineal version to be published Herniation. J Clin Imaging Sci. 2014;4:23. Amro Salem – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the SUGGESTED READING version to be published 1. Sivasubramanian Srinivasan, Vijay Krishnan, Syed Guarantor Zama Ali, Natesan Chidambaranathan. “Swirl sign” of The corresponding author is the guarantor of submission. aggressive angiomyxoma—a lesser known diagnostic sign. Clinical Imaging 38 (2014);751–754. Conflict of Interest 2. Bhavna Nayal, Lakshmi Rao, Anuradha C.K Rao, Swati Authors declare no conflict of interest. Sharma, Rajgopal Shenoy. Extragenital aggressive angiomyxoma of the axilla and the chest wall. J Clin Copyright Diagn Res.2013 Apr;7(4):718-20. © 2015 Zainab Taha ALHumoud et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.

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CASEClinica REPORTl Imag es Peer Reviewed OPEN| OPEN ACCESS ACCESS Bowen’s disease involving the dorsal and volar aspects of left thumb: An unusual site diagnostic entity

Shagufta Rather, Peerzada Sajad, Iffat Hassan

Case Report

Herein, we report a case of a 40-year-old normotensive, non-diabetic, euthyroid male patient who presented with a non-healing erythematous slightly scaly plaque involving the volar and dorsal aspects of left thumb from last eight months (Figures 1 and 2). There was history of pruritus and slight burning sensation. There was a history of application of potent topical steroids for few months with no response. A punch biopsy was done which confirmed the diagnosis of eczema psoriasis and Bowen’s disease. On histopathology, the patient showed thickened epidermis with full thickness dysplasia of squamous epithelium and atypical keratinocytes with numerous mitotic figures. The basement membrane was intact with no focus of invasion or solar elastosis (Figures Figure 1: Bowen’s disease involving the volar aspect of left 3 and 4). Thus a diagnosis of Bowen’s disease was made. thumb. The patient was put on topical 5-fluorouracil and is doing well.

Shagufta Rather1, Peerzada Sajad2, Iffat Hassan3 Affiliations: 1MBBS, MD, Consultant, Postgraduate, Department of Dermatology, STD and Leprosy,Government Medical College Srinagar, J&K, India; 2MBBS,MD, Senior Resident, Postgraduate, Department of Dermatology, STD and Leprosy, Government Medical College, Srinagar, J&K, India; 3MBBS, MD, Professor and Head, Postgraduate, Department of Dermatology, STD and Leprosy, Government Medical College, Srinagar, J&K, India. Corresponding Author: Dr. Peerzada Sajad, Room No 405, Doctors Hostel Opposite Casuality Block, SMHS Hospital, Karan Nagar, Srinagar,J&K 190010, India; Ph: 0990570569; Email: sajads112@gmail. com

Received: 25 February 2015 Accepted: 24 March 2015 Figure 2: Bowen’s disease involving the dorsal aspect of left Published: 01 July 2015 thumb.

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):457–459. Rather et al. 458 www.ijcasereportsandimages.com

Histopathology is characterized by full-thickness dysplasia of the epidermis, with loss of the normal maturation of its components. Keratinocytes are atypical and disorderly, often described as having a windblown appearance. Basement membrane is intact. Topical 5-fluorouracil, imiquimod, photodynamic therapy, cryotherapy and excision are the various treatment modalities [4].

CONCLUSION

Figure 3: Histopathological picture (x400) of Bowen’s disease . The head, neck, and extremities are the most commonly affected anatomic locations in men, while the lower limbs and cheeks are most commonly affected in women. Involvement of palms and soles is an unusual site of occurrence, but it should be considered in the differential diagnosis of non-healing erythematous scaly plaques.

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Rather S, Sajad P, Hassan I. Bowen’s disease involving the dorsal and volar aspects of left thumb: An unusual site diagnostic entity. Int J Case Rep Images 2015;6(7):457–459.

doi:10.5348/ijcri-201523-CL-10078

*********

Author Contributions Shagufta Rather – Substantial contributions to Figure 4: Histopathological picture (x100) of Bowen’s disease. conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Peerzada Sajad – Analysis and interpretation of data, Revising it critically for important intellectual content, DISCUSSION Final approval of the version to be published Iffat Hassan – Analysis and interpretation of data, Bowen’s disease (BD) is a form of intra-epidermal or Revising it critically for important intellectual content, in-situ squamous cell carcinoma with a small potential for Final approval of the version to be published invasive malignancy, and commonly involves chronically photo-exposed areas, especially head and neck region. Guarantor Commonly a persistent, non-elevated erythematous The corresponding author is the guarantor of submission. scaly and crusted plaque is seen. Bowen’s disease may occur at any age in adults, but is rare before the age of Conflict of Interest 30 years; most patients are aged over 60. Any site may Authors declare no conflict of interest. be affected, although involvement of palms or soles is uncommon. Bowen’s disease occurs predominantly in Copyright women in whom about 60-85% of patients have lesions © 2015 Shagufta Rather et al. This article is distributed on the lower leg, usually in previously or presently sun- under the terms of Creative Commons Attribution exposed areas of skin. Chronic ultraviolet radiation License which permits unrestricted use, distribution exposure, arsenic exposure, human papillomavirus and and reproduction in any medium provided the original immunosuppression are the various aetiological factors [1–3].

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):457–459. Rather et al. 459 www.ijcasereportsandimages.com author(s) and original publisher are properly credited. 2. Gupta S, Nutan, Dogra S, Kanwar AJ. Bowen Disease Please see the copyright policy on the journal website for over photoprotected site in an Indian male. Dermatol more information. Online J 2009 Oct 15;15(10):16. 3. Kovács A, Yonemoto K, Katsuoka K, Nishiyama S, Harhai I. Bowen’s disease: statistical study of a 10 year period. J Dermatol 1996 Apr;23(4):267–74. REFERENCES 4. Bargman H, Hochman J. Topical treatment of Bowen’s disease with 5-Fluorouracil. J Cutan Med 1. Kossard S, Rosen R. Cutaneous Bowen’s disease. An Surg 2003 Mar-Apr;7(2):101–5. analysis of 1001 cases according to age, sex, and site. J Am Acad Dermatol 1992 Sep;27(3):406–10.

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CASEEditor REPORTial OPEN OPEN ACCESS ACCESS Management of Inflammatory breast cancer: current concepts

Awad Ali M. Alawad

Inflammatory breast cancer (IBC) is considered chemotherapy for IBC [3]. Among patients with as the most aggressive type of locally advanced breast IBC, response to neoadjuvant chemotherapy plays a cancer that carries an appreciably poor prognosis. Sir comparable prognostic role to that observed among Charles Bell described the first case of IBC in literature. It patients with non-IBC. was reported and published in 1814 by Sir Charles Bell Mastectomy is generally considered an important [1]. Clinically, IBC is defined by characteristics features, part of the multimodality treatment of IBC. The only including rapid onset within short time, erythema, edema method of definitive surgery offered to patients with of the breast, and a “peau d’orange” appearance to most IBC following neoadjuvant chemotherapy is modified areas of breast skin. Moreover, patients presented with radical mastectomy. Skin-sparing mastectomy and breast positive metastatic lymph node involvement and up to conserving surgery are contraindicated for patients with one fourth of patients have distant metastases at time IBC [4]. Postmastectomy chest wall radiotherapy (RT) is of diagnosis [2]. Pathologically, the presence of dermal generally indicated for patients with inflammatory breast lymphatic tumor emboli is considered as the hallmark of cancer who are treated with neoadjuvant chemotherapy. IBC. Previously, single modality treatment to manage IBC Understanding the biological characteristics of the had failed. The majority of patients developed recurrence disease has allowed for the development of targeted and/or metastases within short time, and 5-year survival therapies (e.g., trastuzumab and lapatinib) that are rate was less than 5%. improving the outcome of this aggressive disease. Today, the general consensus is that patients Human epidermal growth factor receptor 2 (HER2) with IBC without proof of metastases at the time of positive patients should receive HER2 targeted therapy diagnosis should receive neoadjunant chemotherapy with neoadjuvant chemotherapy. Trastuzumab should be followed by surgery followed by radiotherapy. For continued after surgery to complete one year of treatment patients with human epidermal growth factor receptor [5]. Lapatinibis an orally active small molecule that (HER2) disease, trastuzumab (an antibody targeting reversibly inhibits the tyrosine kinase component of both HER2) is recommended. For patients with hormone HER2 and epidermal growth factor receptor-1 (ErbB-1). receptor–positive disease, hormonal therapy is A recent research showed that lapatinib in conjunction indicated. Regarding chemotherapy, the sequence of with chemotherapy reduces the risk of disease taxane-based chemotherapy followed by anthracycline- progression by 50% in women with inflammatory breast based chemotherapy is the cornerstone of primary cancer whose disease had progressed on trastuzumab- containing chemotherapy regimens [5]. Other agents that are presently being evaluated for the treatment of Awad Ali M. Alawad IBC include antiangiogenic agents and Ras pathway Affiliations: Assistant professor, Department of Surgery, inhibitors. IBC is known to be highly vascular that University of Medical sciences and Technology , Khartoum, express a number of angiogenic factors such as vascular Sudan. endothelial growth factor (VEGF). This encouraged a Corresponding Author: Awad Ali Mohamed Ahmed number of studies looking at the role of anti-VEGF agents Alawad,Department of Surgery, Faculty of Medicine (e.g., bevacizumab) combined with chemotherapy in the University of Medical Sciences and Technology,Postal address: P. O. Box 12810, Khartoum, Sudan; Ph: treatment of IBC, with hopeful results [5, 6]. +966509246018; Email: awadali82@hotmail. com Combinations of neoadjuvant chemotherapy, mastectomy, and radiotherapy have led to an improved prognosis. However, the overall five-year survival rate Received: 04 May 2015 for patients with IBC is still very low, at 20% [7]. Published: 01 July 2015 Proper management of IBC requires close coordination ∼

International Journal of Case Reports and Images, Vol. 6 No. 7, July 2015. ISSN – [0976-3198] Int J Case Rep Images 2015;6(7):460–461. Alawad 461 www.ijcasereportsandimages.com among surgical, radiation oncologists, radiologists, and and reproduction in any medium provided the original pathologists. It is important for health institutions to pool author(s) and original publisher are properly credited. resources by establishing a tumor registry for collecting Please see the copyright policy on the journal website for data from patients with IBC worldwide to deal with this more information. fatal disease because of the infrequency of IBC.

How to cite this article REFERENCES

Alawad AAM. Management of Inflammatory breast 1. Ahmed AA. Clinicopathological profile of female cancer: current concepts. Int J Case Rep Images Sudanese patients with locally advanced breast 2015;6(7):460–461. cancer. Breast Dis 2014;34(3):131–4. 2. Alawad A, Alshiekh A, Alhaj A. Demographic Characteristics and Review of Patients with Locally doi:10.5348/ijcri-201502-ED-10002 Advanced Breast Cancer in Sudan. Afrimedic Journal 2014;4(2):5–8. 3. Alawad AA. Evaluation of clinical and pathological response after two cycles of neoadjuvant chemotherapy ********* on Sudanese patients with locally advanced breast cancer. Ethiop J Health Sci 2014 Jan;24(1):15–20. Author Contributions 4. Abrous-Anane S, Savignoni A, Daveau C, et al. Awad Ali M. Alawad – Substantial contributions to Management of inflammatory breast cancer after conception and design, Acquisition of data, Analysis neoadjuvant chemotherapy. Int J Radiat Oncol Biol and interpretation of data, Drafting the article, Revising Phys 2011 Mar 15;79(4):1055–63. it critically for important intellectual content, Final 5. Dawood S. Biology and management of inflammatory approval of the version to be published breast cancer. Expert Rev Anticancer Ther 2010 Feb;10(2):209–20. 6. Warren LE, Guo H, Regan MM, et al. Inflammatory Guarantor Breast Cancer: Patterns of Failure and the Case for The corresponding author is the guarantor of submission. Aggressive Locoregional Management. Ann Surg Oncol 2015 Mar 20. Conflict of Interest 7. Matro JM, Li T, Cristofanilli M, Inflammatory breast Authors declare no conflict of interest. cancer management in the national comprehensive cancer network: the disease, recurrence pattern, and Copyright outcome. Clin Breast Cancer 2015 Feb;15(1):1–7. © 2015 Awad Ali M. Alawad. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution

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