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neurenteric cyst

Friday 10 February 2006

Definition: A neurenteric cyst is a rare congenital disorder secondary to alimentary duplication and vertebral malformation.

Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and

Neurenteric cysts are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis.

Neurenteric cyst are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults.

Although usually located in the thorax, neurenteric cysts may be found along the entire spine.


- Spinal neurenteric cysts should be included in the differential diagnosis of an intradural, extramedullary spinal lesion.


- cervical neurenteric cyst
- abdominal neurenteric cyst


- Klippel-Feil syndrome (1764851)
- hydrocephalus

  • aqueductal stenosis

- vertebral segmentation anomalies
- fused costae
- anal atresia
- renal dysplasia
- bicornuate uterus
- double blind vagina
- transdiaphragmatic duodenal duplication (15108090)


- Abdominal neurenteric cyst. Colović R, Micev M, Jovanović M, Matić S, Grubor N, Atkinson HD. World J Gastroenterol. 2008 Jun 21;14(23):3759-62. PMID: 18595146 [Free]

- Giant transdiaphragmatic duodenal duplication with an intraspinal neurenteric cyst as part of the split notochord syndrome: report of a case. Wakisaka M, Nakada K, Kitagawa H, Shimada H, Nosaka S. Surg Today. 2004;34(5):459-62. PMID: 15108090

- VACTERL or MURCS association in a girl with neurenteric cyst and identical thoracic malformations in the father: a case of gonosomal mosaicism? Orstavik KH, Steen-Johnsen J, Foerster A, Fjeld T, Skullerud K, Lie SO. Am J Med Genet. 1992 Aug 1;43(6):1035-8. PMID: 1415331

- Neurenteric cysts—a spectrum. Alrabeeah A, Gillis DA, Giacomantonio M, Lau H. J Pediatr Surg. 1988 Aug;23(8):752-4. PMID: 3171846