Pentology of Cantrell: a rare case report with review of literature
Keywords:Ectopia cordis, Omphalocoele, Pentology of Cantrell
Pentology of Cantrell a rare congenital disorder of unknown etiology consists of pentad of: defect in the diaphragm, abdominal wall defect, deficiency of the pericardium and intracardiac defects. The hallmark diagnostic findings being ectopia cordis and omphalocoele. The exact pathogenesis is not clear, and the prognosis of this lethal condition depends on the severity of anterior abdominal wall defect and associated cardiac anamoly. Antenatal ultrasonography is a reliable tool of diagnosis and allow for termination of pregnancy if done before 20weeks of pregnancy. First described by Cantrell in 1958, very few cases have been reported till now in the literature. Authors describe here a case of a neonate delivered at 32weeks of gestation weighing 2.3kg at their hospital with typical features of Cantrells’ Pentology associated with a rare interesting finding of absent external genitalia and presence of lumbar meningocoele.
Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 1958;107(5):602-14.
Suresh Chandran DA. Pentalogy of Cantrell: an extremely rare congenital anomaly. Journal of clinical neonatology. 2013 Apr;2(2):95.
Önderoðlu L, Baykal C, Tulunay G, Talim B, Kale G. Prenatal diagnosis of Cantrell’s pentalogy: a case report. The Turkish journal of pediatrics. 2003 Oct;45:357-8.
Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome. Pediatrics. 1972;50:778-92.
Abdelmoneim EM Kheir, Elghazali A Bakhiet, Salma MM Elhag, Mohamed Z Karrar. Pentalogy of Cantrell: A case report and review of the literature. Sudan J Pediatr. 2014;14(1):85-8
Jafarian AH, Omidi AA, Fazel A, Sadeghian H, Joushan B. Pentology of Cantrell: A rare case report. J Res Med Sci. 2011 Jan;16(1):105-9
McMahon CJ, Taylor MD, Cassady CI, Olutoye OO, Bezold LI, Diagnosis of pentology of Cantrell in the fetus using magnetic resonance imaging and ultrasound. Pediatr Cardiol 2007;28:172-5.
Ghidini A, Sirtori M, Romero R, Hobbins JC. Prenatal diagnosis of pentology of Cantrell. J Ultrasound Med. 1998;7(10):567-62
Norma BS, Alfredo VA, Erika SS, Begona SS, Luis AA, Lorenzo RC, et al. pentology of Cantrell. Fourty two years of experience in the hospital infantile de Mexico ferico Gomez. World J Pediatr Congenital Heart Surg. 2011;2:211-8.
Hornberger LK, Colan SD, Lock JE, Wessel DL, Mayer JE. Outcome of patients with ectopia cordis and significant intracardiac defectsw. Circulation. 1996;94:32-7.