neurenteric cyst

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.

Localization

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

Localization

 cervical neurenteric cyst
 abdominal neurenteric cyst

Associations

 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)

References

 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