Eight patients representing the entire spectrum of Cantrell's syndrome are presented. While a combined diaphragmatic and pericardial defect is an essential feature of the syndrome, other manifestations form a continuum ranging from innocent upper abdominal midline defects to the full-blown pentalogy sometimes incompatible with life. In only two cases was the diagnosis established preoperatively. One patient presented uniquely with bilateral chylothoraces. Clinical suspicion, plain chest roentgenograms, and ultrasonography proved the most definitive diagnostic aids. Two patients died early due to their severe cardiac malformations. Six children are alive 16 months to 23 years postoperatively. Seven patients underwent operative correction of their defects. A primary repair of the abdominal and diaphragmatic lesions was performed trans-abdominally or via a sternotomy in all but one case. Two patients had late repairs of their intracardiac defects. Although rare, Cantrell's syndrome must be actively sought in every patient with an omphalocele or an atypical diaphragmatic hernia. These patients need a full cardiac evaluation before operation. The Diaphragmatic defect must be recognized and repaired before closure of the omphalocele. The prognosis depends mainly on the cardiac lesion.
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Cantrell JR, Haller JA, Ravitch MM (1958) A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, Pericardium and heart. Surg Gynecol Obstet 107: 602–614
Eraklis AJ, Trump DS, Longino LA (1967) Omphalocele with diaphragmatic and pericardial defects: diagnosis and repair. J Ped Surg 2: 354–358
Farriaux J-P, Ridet M, Lefebvre P, Ryckewaert Ph, Fontaine G (1966) Une curieuse forme de hernie diaphragmatique: la hernie péricardio-diaphragmatique. Ann Pédiatrie 50: 778–787
Haller JA, Cantrell JR (1966) Diagnosis and surgical correction of combined congenital defects of supra-umbilical abdominal wall, lower strrnum and diaphragm. J Thorac Cardiovasc Surg 51: 286–291
Janneck C, v Ekesparre W, Wesener G (1983) Kongenitale intraperikardiale Zwerchfellhernie. Ein kasuistischer Beitrag. Z Kinderchir 38: 31–33
Larrieu AJ, Wiener I, Alexander R, Wolma FJ (1980) Pericardiodiaphragmatic Hernia. Am J Surg 139: 436–440
Milne LW, Morosin A-M, Campbell JR, Harrison MW (1990) Pars sternalis diaphragmatic hernia with omphalocele: a report of two cases. J Pediatr Surg 25: 726–730
Pérez-Alvarez JJ, Pérez-Trevino C, Domene-Flor R (1966) Successful total correction of venticular septal defect, diverticulum of the left ventricle, and anomaly of the thoracoabdominal wall in an 11-year-old boy with cardiac dextroversion. J Thorac Cardiovasc Surg 52: 386–394
Ravitch MM (1977) Congenital deformities of the chest wall and their operative correction. Saunders, Philadelphia, London, Toronto
Ravitch MM (1986) The chest wall. In: Welch KJ, Randolph JG, Ravitch MM, O'Neill Jr JA, Rowe MI (eds) Pediatric surgery, 4th edn. Year Book Medical Publishers, Chicago, London, pp 566–567
Reese HE, Stracener CE (1966) Congenital defects involving the abdominal wall, sternum, diaphragm and pericardium: case report and review of embryologic factors. Ann Surg 163: 391–393
Symbas PN, Ware RE (1973) A syndrome of defects of the thoracoabdominal wall. Diaphragm, pericardium and heart. One-stage surgical repair and analysis of the syndrome. J Thorac Cardiovasc Surg 65: 914–919
Wesselhoeft CW, DeLuca FG (1984) Neonatal septum transversum diaphragmatic defects. Am J Surg 147: 481–485
Zachariou Z, Daum R, Roth H, Benz G (1987) Das Cantrellsche Syndrom. Z Kinderchir 42: 255–259
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Vanamo, K., Sairanen, H. & Louhimo, I. The spectrum of Cantrell's syndrome. Pediatr Surg Int 6, 429–433 (1991). https://doi.org/10.1007/BF00185335
- Diaphragmatic hernia
- Pentalogy of Cantrell