Abstract
Two cases of the split notochord syndrome are described. Each patient presented with different clinical features that can be attributed to similar embryological defects. One infant presented with a covered lumbosacral meningocele, caudal to which lay isolated, exposed small-bowel mucosa. This is the second such case described in the literature. The second child presented with respiratory difficulties and was found to have a long thoraco-abdominal bowel duplication. Each child had surgical treatment of the abnormalities and both remain well with no neurological deficits at 6 and 3 years, respectively. The abnormalities are explained as consequences of a division of the notochord resulting in abnormal endo-ectodermal adhesion. Surgery is advocated for excision of an unacceptable skin lesion, spinal dysraphism, and because of the risks of haemorrhage and neoplasm.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Beardmore HE, Wigglesworth FW (1958) Vertebral anomalies in alimentary duplications. Pediatr Clin North Am 5: 457–474
Bentley JRF, Smith JR (1960) Developmental posterior enteric remnants and spinal malformations. Arch Dis Child 35: 76–86
Bower RJ, Sieber WK, Kiesewetter WB (1978) Alimentary tract duplications in children. Am J Surg 188: 669–674
Caruolo JE, Ellis FH Jr, Harris LE (1956) Intrathoracic enterogenous anomaly communicating with the small intestine. Surgery 40: 894–903
D'Almeida AC, Stewart DH Jr (1981) Neurenteric cyst: case report and literature review. Neurosurgery 8: 596–599
Fallon M, Gordon ARG, Lendrum AC (1954) Mediastinal cysts of foregut origin associated with vertebral anomalies. Br J Surg 41: 520–533
Feller A, Sternberg H (1929) Zur Kenntnis der Fehlbildungen der Wirbelsäule. 1. Die Wirbelkörperspalte und ihre formale Genese. Virchows Arch [A] 272: 613
Gdanietz K, Wit J, Heller K, Kriewald M (1983) Complete duplication of the small intestine in childhood. Z Kinderchir 38: 414–416
Goldberg HM, Johnson TP (1963) Posterior abdominothoracic enteric duplications. Br J Surg 50: 445–449
Odake G, Yamaki T, Naruse S (1976) Neurenteric cyst with meningomyelocele. J Neurosurg 45: 352–356
Quayle J (1980) Congenital duplication of the small bowel: severe haemorrhage. J R Soc Med 73: 748–750
Roth M (1881) Ueber Missbildungen in Bereich des Ductus Omphalomesentericus. Arch f Pathol Anat 86: 371–390
Saunders RLdeCH (1943) Combined anterior and posterior spina bifida in a living neonatal human female. Anat Rec 87: 255–278
Superina RA, Ein SH, Humphreys RP (1984) Cystic duplications of the oesophagus and neurenteric cysts. J Pediatr Surg 19: 527–530
Schwartz DL, Becker JM, Schneider KM, So HB (1980) Tubular duplication with autonomous blood supply; resection with preservation of adjacent bowel. J Pediatr Surg 15: 341–342
Veeneklaas GMH (1952) Pathogenesis of intrathoracic gastrogenic cysts. Am J Dis Child 83: 500–507
Author information
Authors and Affiliations
Additional information
Offprint requests to: R. J. Fitzgerald
Rights and permissions
About this article
Cite this article
Byrne, D.J., Grace, P.A. & Fitzgerald, R.J. The split notochord syndrome: alpha and omega. Pediatr Surg Int 4, 52–55 (1988). https://doi.org/10.1007/BF00173085
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00173085