View metadata, citation and similar papers at core.ac.uk brought to you by CORE

provided by Elsevier - Publisher Connector J Ped Surg Case Reports 1 (2013) 129e131

Contents lists available at SciVerse ScienceDirect

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Wandering and splenic torsion associated with upper respiratory tract infection

Rashmi N. Samarasinghe a, Bogdan Protyniak b,*, Colin A.I. Bethel b

a School of Medicine, St. George’s University, St. George’s, Grenada b Department of Surgery, Newark Beth Israel Medical Center, 201 Lyons Avenue, Newark, NJ 07112, USA

article info abstract

Article history: Torsion of a wandering spleen is a rare cause of acute abdomen in pediatric patients. Congenital absence Received 10 March 2013 of the splenic ligaments predisposes the spleen to axial rotation around its vascular pedicle and may lead Received in revised form to infarction. Computed tomography and/or ultrasound are valuable in making a timely diagnosis. 8 May 2013 Detorsion and splenopexy permit splenic salvage, potentially reducing late post- associated Accepted 8 May 2013 complications. We report the case of a 9-year-old female with an upper respiratory tract infection and infarction of a wandering spleen. We review the literature on the management of this condition and hypothesize that vigorous coughing associated with upper respiratory infections may have caused the Key words: Wandering spleen wandering spleen to undergo axial rotation around its pedicle. Ó Spleen infarction 2013 Elsevier Inc. Open access under CC BY-NC-ND license. Spleen torsion Acute abdomen Splenectomy Splenopexy

Wandering spleen was first described in children by Jozef Dietl The diagnosis of splenic torsion with infarction was made and in 1854 [1]. Little has been published on the subject in the current exploratory laparotomy was performed through a left subcostal literature. Wandering spleen is a rare condition characterized by incision. The spleen measured 15 cm in length. The normal splenic splenic hypermobility due to laxity or absence of its suspensory suspensory ligaments (gastrosplenic, colicosplenic, phrenocolic, ligaments [2e4]. The spleen can migrate to the pelvis, attached only and splenorenal) were absent (Fig. 3). by its lengthened vascular pedicle [3,5]. This single point of Doppler probe revealed pulsatile flow proximal to the clock- attachment predisposes the spleen to torsion, parenchymal wise 360 splenic pedicle torsion but there was no flow at the congestion, and ensuing infarction [5]. distal hilum. The spleen was detorsed by rotating it counter- clockwise. Hilar doppler signals remained absent and the spleen 1. Case report remained cyanotic. Splenectomy was performed. Histopathologic examination demonstrated diffuse hemorrhagic necrosis of the The patient is a 9-year-old girl who presented to the emergency spleen. department with a 4-day history of fever and spasmodic cough. On The patient was discharged 2 days later after receiving vacci- fl the day prior to admission she complained of left upper quadrant nations against in uenza, meningococcus, and pneumococcus. An fi abdominal pain. Physical examination was remarkable for a fever inde nite period of prophylactic antibiotics was planned. of 100.6 F and a tender left upper quadrant mass. Complete blood count, urinalysis, and chest X-ray were normal. A computed tomog- 2. Discussion raphy (CT) scan of the abdomen and pelvis showed a large, non- fl enhancing spleen extending into the pelvis with trace free uid Splenic hypermobility (wandering spleen) occurs from the fail- (Fig. 1). The splenic artery ended abruptly proximal to the splenic ure of fusion between the dorsal mesogastrium and the posterior hilum (Fig. 2). abdominal wall during embryogenesis [6]. The wandering spleen has occasionally been seen in association with other conditions in fi * Corresponding author. Tel.: þ972 973 704 1023; fax: þ972 973 923 8757. which normal intraabdominal xation has not occurred (prune- E-mail address: [email protected] (B. Protyniak). belly syndrome, renal agenesis, gastric volvulus, diaphragmatic

2213-5766 Ó 2013 Elsevier Inc. Open access under CC BY-NC-ND license. http://dx.doi.org/10.1016/j.epsc.2013.05.006 130 R.N. Samarasinghe et al. / J Ped Surg Case Reports 1 (2013) 129e131

Fig. 1. Computed tomography scan showing a large, non-enhancing spleen extending into the pelvis with trace free fluid. eventration, and congenital diaphragmatic hernia) [7]. Generalized connective tissue diseases such as marfanoid hypermobility can lead to increased laxity of existing splenic ligaments and wandering Fig. 3. The spleen has no ligamentous attachments and is twisted 360 degrees on its spleen [8]. , resulting from lipid accumulation due to vascular pedicle. type C NiemannePick disease has also been reported to be a cause of a wandering spleen [9]. Children with wandering spleen most commonly present with an acute abdomen after torsion of the splenic vascular pedicle [7]. Subsequent infarction, necrosis, and gangrene may cause perito- nitis, intestinal obstruction, variceal hemorrhage, and necrosis of the pancreatic tail [6,8,10,11]. Patients may also present with chronic or intermittent pain when partial torsion followed by spontaneous detorsion of the splenic pedicle occurs [3,4,10]. These patients may present with nausea, emesis, fever, , and peritoneal irritation [6,10]. CT and ultrasonography may demonstrate a distinctive comma- shaped spleen in an abnormal location [10]. Doppler flow studies may reveal absent flow within the splenic artery and vein, and CT may show the classical “whorl” sign indicative of a twisted pedicle [5,8,10]. The disadvantages of CT include the need for general anesthesia in younger children and radiation exposure [6]. Ultra- sonography is useful and avoids radiation, but in our experience, it may be impossible to clearly evaluate the splenic pedicle in the common situation of massive splenic enlargement [12]. Historically, the treatment for a wandering spleen, whether torsion was present or not, was splenectomy [4,10,11]. However, preservation of the spleen is desirable to avoid overwhelming post- splenectomy sepsis [3,4,11]. Splenopexy is performed if there is no evidence of infarction, thrombosis, or hypersplenism [2e4]. The procedure may involve creating an in-situ tissue pouch using omentum or colon, or by using an absorbable or synthetic mesh to fix the spleen [3,7]. Splenectomy is the treatment of choice if the spleen is infarcted, in danger of rupture, or if there is splenic vein thrombosis [2,4].

3. Conclusion

Most cases of wandering spleen complicated by splenic torsion occur in the setting of nonexistent/abnormal splenic attachment in the absence of other predisposing factors. However this patient presented after a period of vigorous coughing raising the possibility that abdominal wall spasm/contraction may have predisposed to Fig. 2. Computed tomography scan showing the splenic artery ending abruptly prox- torsion. Centonze et al. report a patient who presented with a imal to the splenic hilum. wandering spleen in the setting of chronic cough [9]. R.N. Samarasinghe et al. / J Ped Surg Case Reports 1 (2013) 129e131 131

Torsion of a wandering spleen is a rare cause of an acute [4] Kapan M, Gumus M, Onder A, Gumus H, Aldemir M. A wandering spleen e abdomen in pediatric patients. Optimal treatment requires a presenting as an acute abdomen: case report. J Emerg Med 2012;43:e303 5. [5] Liu HTM, Lau KK. Wandering spleen: an unusual association with gastric heightened awareness, early diagnosis, and prompt surgical inter- volvulus. Am J Roentgenol 2007;188:W328e30. vention. If diagnosed promptly, detorsion and splenopexy permit [6] Di Crosta I, Inserra A, Gil CP, Pisani M, Ponticelli A. Abdominal pain and splenic salvage, avoiding post-splenectomy associated complica- wandering spleen in young children: the importance of an early diagnosis. J Pediatr Surg 2009;44:1446e9. tions. CT scan with intravenous contrast and/or color doppler [7] Brown CVR, Virgilio GR, Vazquez WD. Wandering spleen and its complications ultrasonography are useful adjuncts in making the diagnosis. in children: a case series and review of the literature. J Pediatr Surg 2003;38: 1676e9. fl [8] Sodhi KS, Gupta P, Rao KLN, Marwaha RK, Khandelwal N. Marfanoid hyper- Con ict of interest mobility syndrome and skeletal abnormalities in a rare case of torsion of The authors have no conflicts of interest. wandering spleen. Br J Radiol 2008;81:e145e8. [9] Centonze A, Mazzei A, Castaldo P, Raiola G, Salerno D, Porta IP, et al. Atypical presentation of a wandering spleen in NiemannePick disease. J Emerg Med References 2013;44:851e2. [10] Sodhi KS, Saggar K, Sood BP, Sandhu P. Torsion of a wandering spleen: acute [1] Magowska A. Wandering spleen: a medical enigma, its natural history and abdominal presentation. J Emerg Med 2003;25:133e7. rationalization. World J Surg 2013;37:545e50. [11] Lopez-Tomassetti FEM, Arteaga GI, Martin MA, Carrillo PA. An unusual case of [2] Kim SC, Kim DY, Kim IK. Avulsion of wandering spleen after traumatic torsion. hemoperitoneum owing to acute splenic torsion in a child with immuno- J Pediatr Surg 2003;38:622e3. globulin deficiency. J Postgrad Med 2006;52:41e2. [3] Bouassida M, Sassi S, Chtourou MF, Bennani N, Baccari S, Chebbi F, et al. [12] Ayaz UY, Dilli A, Ayaz S, Api A. Wandering spleen in a child with symptoms of A wandering spleen presenting as a hypogastric mass: case report. Pan Afr acute abdomen: ultrasonographic diagnosis. Case report. Med Ultrason 2012; Med J 2012;11:31. 14:64e6.