Abstract
Split notochord syndrome (SNS) is an extremely rare anomaly. This report presents the case of a male infant with SNS associated with congenital ileal atresia and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula. Dorsal fistulography and vesicography were useful and essential for the detailed study of the topology in this patient. The embryological mechanism and etiologic theories are discussed with a review of 19 cases reported in the literature.
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Keen WW, Coplin WML (1906) Sacrococcygeal tumor (teratoma). Surg Gynecol Obstet 3:661–671
Saunders RL (1943) Combined anterior and posterior spina bifida in a living neonatal female. Anat Rec 87:255–278
Rosselet PJ (1995) A rare case of rachischisis with multiple malformations. Am J Roentgenol Radium Ther Nucl Med 73:235–240
Bentley JFR, Smith JR (1960) Developmental posterior enteric remnants and spinal malformations. Arch Dis Child 35:76–86
Faris JC, Crowe JE (1975) The split notochord syndrome. J Pediatr Surg 4:467–472
Singh A, Singh R (1982) Split notochord syndrome with dorsal enteric fistula. J Pediatr Surg 17:412–413
Gupta DK, Deodhar MC (1987) Split notochord syndrome presenting with meningocele and dorsal enteric fistula. J Pediatr Surg 22:382–383
Burrows FGO, Sutcliffe J (1968) The split notochord syndrome. Br J Radiol 41:844–847
Kheradpir MH, Ameri MR (1983) Dorsal herniation of the gut with posterior opening of the terminal colon: a rare manifestation of the split notochord syndrome. Z Kinderchir 38:186–187
Kramer EL, Giacoia GP, Say B, Jarolim KL, Miller-Hardy D (1988) Split notochord syndrome with dorsal enteric fistula and sacral agenesis. Teratology 38:1–5
Meller JL, Loeff DS, Reyes HM (1989) A variant of the split notochord syndrome. J Pediatr Surg 24:733–735
Hoffman CH, Dietrich RB, Pais MJ, Demos DS, Pribram HF (1993) The split notochord syndrome with dorsal enteric fistula. J Neuroradiol 14:622–627
Razack N, Page LK (1995) Split notochord syndrome: case report. Neurosurgery 37:1006–1008
Akgur FM, Ozdemir T, Olguner M, Erbayraktar S, Ozer E, Aktug T (1998) A case of split notochord syndrome: presence of dorsal enteric diverticulum adjacent to the dorsal enteric fistula. J Pediatr Surg 8:1317–1319
Kiristioglu I, Teitelbaum DH, Dogruyol H (1998) Split notochord syndrome with prolapsed congenital colostomy. J Pediatr Surg 33:525–528
Pathak VB, Singh S, Wakhlu AK (1988) Double split of notochord with massive prolapsed of the gut. J Pediatr 23:1039–1040
Kanmaz T, Demirbilek RB, Ozturk A, Baykara S, Yucesan S (2002) The split notochord syndrome with dorsal enteric fistula. Indian J Pediatr 69:729–730
Jesus LE, Franca CG (2004) A rare variant of neuroenteric cyst: split notochord syndrome. J Pediatr 80:77–80
Agangi A, Paladini D, Bagolan P, Maruotti GM, Martinelli P (2005) Split notochord syndrome variant: prenatal findings and neonatal management. Prenat Diagn 25:23–27
Bell HH (1923) Anterior spina bifida and its relation to a persistence of the neuroenteric canal. J Nerv Ment Dis 57:445–462
Bremer JL (1952) Dorsal intestinal fistula; accessory neuroenteric canal; diastematomyelia. Arch Pathol 54:132–138
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Asagiri, K., Yagi, M., Tanaka, Y. et al. A case of split notochord syndrome with congenital ileal atresia, the total absence of a colon, and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula. Pediatr Surg Int 24, 1073–1077 (2008). https://doi.org/10.1007/s00383-008-2206-9
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DOI: https://doi.org/10.1007/s00383-008-2206-9