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Prenatal Diagnosis of a Case of Pentalogy of Cantrell with Spina Bifida

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Prenatal Diagnosis of a Case of Pentalogy of Cantrell with Spina Bifida � Letters administered. Thus, the PPE was attributed to temozolomide. Successful treatment of palmar- He was treated with intravenous dexamethasone at a dose of 32 plantar erythrodysesthesia mg/d for seven days. As the skin lesions resolved with brownish discoloration, this therapy was tapered over the following one possibly due to temozolomide with week. The chemotherapy regimen of the patient had to be ceased because of the rapid progression in the clinical course. dexamethasone In this patient, temozolomide-induced PPE might be caused by a cumulative toxic effect on the skin, since PPE occurred after fifth cycle of therapy. The patient received a combination Sir, of temozolomide and phenytoin, thus it is difficult to decide Palmar-plantar erythrodysesthesia (PPE) is a cutaneous toxicity which drug or their combination is responsible for the skin of some chemotherapeutic agents such as 5-FU, doxorubicine lesions. However, as suggested by the patient’s history and and capecitabine.[1,2] Clinicians could also encounter this available data in literature, it is most likely that the skin reaction reaction in response to treatment with novel agents. was induced by temozolomide. Temozolomide is a new alkylating agent that has demonstrated promising activity in the treatment of primary or metastatic We treated our patient with intravenous dexamethasone and brain tumors.[3] Herein, we present a case of PPE possibly due his symptoms and signs disappeared within one week. The to temozolomide therapy. prompt improvement of skin lesions as compared with the normal course of the disease (five to six weeks) confirmed the A 67-year-old man presented with headache caused by a large efficiency of dexamethasone therapy previously reported.[1] intracerebral frontal tumor. The tumor was totally resected and a diagnosis of glioblastoma multiforme was established. In conclusion, chemotherapy-induced PPE is a fascinating Following the surgery, he received radiation therapy. Three clinical problem that can occur when using new and months later, locally tumor recurrence was detected and investigational agents as in this patient. We conclude that the temozolomide was started at a dose of 200 mg/m2 for five days patient is possibly a case of PPE to temozolomide. Therefore, every four weeks. A stable disease was achieved. Following the we suggest that clinicians should be aware of the potential for completion of the fifth cycle, the patient complained of a burning PPE in patients receiving temozolomide. sensation at the lateral edges of hands and feet. This dysthesia .com). was followed by the development of symmetric erythema on Kanat O, Baskan BE*, Kurt E, Evrensel T the palms and soles [Figure 1]. The hands were more severely Uludag University Faculty of Medicine, Departments of affected than feet. The painful and well-demarcated Medical Oncology and *Dermatology, Gorukle, Bursa, Turkey erythematous lesions on the palms and soles and periungual erythema and swelling were consistent with Grade 3 PPE Correspondence: according to World Health Organization criteria.[1] Infectious Dr. Ozkan Kanat, E-mail: [email protected] causes were ruled out. The skin biopsy demonstrated nonspecific.medknow histopathological findings including papillary dermal edema, References dilated blood vessels and perivascular lymphocytic infiltration. The patient’s only concurrent medications were multivitamins(www 1. Nagore E, Insa A, Sanmartin O. Antineoplastic therapy-induced palmar and phenytoin, which were taken for more than five months. plantar erythrodysesthesia (‘hand-foot’) syndrome. Incidence, recognition and management. Am J Clin Dermatol 2000;1:225-34. His metabolic status was normal and blood transfusion was not 2. Bhasin S, Sunita, Gupta DK, Kataria SP, Saluja S, Sharma M. This PDF is available for free Chemotherapy-induceddownload from palmer planter erythrodysesthesia. J Assoc a site hosted by MedknowPhysicians Publications India 2005;53:155-6. 3. O’Reilly SM, Newlands ES, Glaser MG, Brampton M, Rice-Edwards JM, Illingworth RD, et al. Temozolomide: A new oral cytotoxic chemotherapeutic agent with promising activity against primary brain tumours. Eur J Cancer 1993;29A:940-2. Prenatal diagnosis of a case of pentalogy of Cantrell with spina bifida Sir, In 1958, Cantrell described a syndrome in which a ventral Figure 1: Symmetric erythema and edema on both palms diaphragmatic hernia occurred in association with � 146 J Postgrad Med April 2007 Vol 53 Issue 2 Letters � omphalocele.[1] This rare syndrome is now called the pentalogy of defects present (but always including a sternal of Cantrell and it consists of the following five anomalies: a abnormality).[3] A ventricular septal defect was not observed deficiency of the anterior diaphragm, a midline in our case, although Cantrell noted that congenital supraumbilical abdominal wall defect, a defect in the intracardiac anomalies are constant elements of the diaphragmatic pericardium, various congenital intracardiac pentalogy, with ventricular septal defects seen in every case abnormalities and a defect of the lower sternum. We report a (100%), atrial septal defects in 53%, pulmonary stenosis in case of pentalogy of Cantrell associated with thoraco-lumbar 33%, tetralogy of Fallot in 20% and left ventricular spina bifida, hydrocephalus and lemon sign diagnosed diverticulum in 20%. Only seven of the 60 reported cases of prenatal in the second trimester, a finding not previously Cantrell’s pentalogy involved the central nervous system and recorded in the literature. craniofacial malformations, which included anencephaly, meningocele, cephalocele, hydrocephaly and exencephaly.[4,5] An 18-year-old woman (gravida 1, para 0), presented for To our knowledge, Cantrell’s pentalogy with thoraco-lumbar routine ultrasound examination at 20 weeks gestation. spina bifida, hydrocephaly and lemon sign was not previously Detailed ultrasonography demonstrated omphalocele with reported in this association. Our case is a variant form of abdominal visceral eventration (liver, stomach and intestines), the classic pentalogy of Cantrell and extends the spectrum ectopia cordis and hydrocephalus (lemon sign), thoraco­ of Toyama’s classification, particularly Class 3. Termination lumbar spina bifida with a femur length corresponding to of pregnancy can be offered before viability. After viability, the menstrual age. An anterior thoraco-abdominal wall defect a periodic ultrasonographic evaluation of the lesions, fetal was seen with the contents herniating into the amniotic cavity growth and delivery in a tertiary center is recommended. covered by a thin sac. The fetal heart was seen over the omphalocele and clearly out of the thorax [Figures 1, 2]. On the basis of the sonographic findings, we diagnosed the pentalogy of Cantrell with thoraco-lumbar-rachischisis. After we discussed the diagnosis with the parents, the pregnancy was terminated. There was an upper abdominal omphalocele defect with covering membranes getting ruptured during extraction at labor, due to which the liver and intestines were seen protruding out [Figure 3]. .com). The incidence of this syndrome is less than 1 in 100 000, with a 2:1 male predominance.[2] Based on a review of 61 cases of Cantrell’s pentalogy, Toyama suggested the following classification for the syndrome: Class 1, certain diagnosis, with all five defects present; Class.medknow 2, probable diagnosis, with four defects (including intracardiac and ventral abdominal wall abnormalities) Figure 2: Transverse section of the fetal head showing hydrocephalus, lemon present; and Class 3, incomplete, with various combinations sign and another section posterior view of the fetus shows the spinal spinal (www bifida This PDFa site is availablehosted by for Medknow free download Publications from Figure 1: Sagittal image of the fetus showing large omphalocele and heart Figure 3: Postmortem photograph depicts a large thoraco-abdominal wall seen out of the thorax over omphalocele defect with eviscerated abdominal organs and another photograph of the thoraco-lumbar spina bifida J Postgrad Med April 2007 Vol 53 Issue 2 147 � � Letters Dane C, Dane B, Yayla M, Çetin A Department of Perinatology, Clinics of Gynecology and Obstetrics, Haseki Education and Research Hospital, Istanbul, Turkey Correspondence: Cem Dane, E-mail: [email protected] References 1. Cantrell JR, Haller JA, Ravith MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602-14. 2. Thamboo TP, Chan NH. Pentalogy of Cantrell with a double-outlet left ventricle. Pathology 2005;37:87-9. 3. Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium and heart: A case Figure 1: Top strip: ventricular bigeminy with prolonged PR interval of the report and review of the syndrome. Pediatrics 1972;50:778-92. successive sinus beats. Middle strip: progressive prolongation of the PR 4. Polat I, Gul A, Aslan H, Cebeci A, Ozseker B, Caglar B, et al. Prenatal interval of the successive sinus beats followed by AV block in every third diagnosis of pentalogy of Cantrell in three cases, two with sinus beat. Bottom strip: AV block of alternate sinus beats following the craniorachischisis. J Clin Ultrasound 2005;33:308-11. ventricular ectopics 5. Denath FM, Romano W, Solcz M, Donnelly D. Ultrasonographic findings of exencephaly in pentalogy of Cantrell: Case report and review of the literature. J Clin Ultrasound 1994;22:351-4. alternatively conducted
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