The split notochord syndrome with dorsal enteric fistula.
نویسندگان
چکیده
Split notochord syndrome with dorsal enteric fistula is an extremely rare congenital anomaly that may be associated with meningomyelocele or meningocele, and genitourinary anomalies. This case presented with an additional finding of bladder exstrophy, raising the possibility of a relationship between this syndrome and the OEIS complex.
منابع مشابه
[A rare variant of neuroenteric cyst: split notochord syndrome].
OBJECTIVE We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism. DESCRIPTION We treated a 2 month-old boy presenting with an extensive lumbosacral deformity, hydrocephalus and apparent enteric segments in the dorsal midline, accompanied by an enteric fistula and imperforated anus. The malformation was diagnosed as split notochord syndrome. The baby died as...
متن کاملA Rare Case of Split Notochord Syndrome
We treated a one day old baby presenting with an extensive lumbosacral deformity, hydrocephalus and apparent enteric segments in the dorsal midline with abnormal genitalia, accompanied by an enteric fistula and imperforated anus. The malformation fits into split notochord syndrome. The baby died as a result of sepsis before surgical treatment could be attempted. Patient was full term caesarian ...
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1. Med Imagem – Radiologia, Teresina PI, Brazil. 2. UDI 24 horas, Teresina, PI, Brazil. 3. Hospital de Urgência de Teresina Prof. Zenon Rocha, Teresina, PI, Brazil. Mailing address: Dra. Camila Soares Moreira de Sousa. Med Imagem – Radiologia. Rua Paissandu, 1862, Centro. Teresina, PI, Brazil, 64001-120. E-mail: [email protected]. Although the embryogenesis of the anomaly remains under...
متن کاملA case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis.
The authors describe a case of split notochord syndrome with a neuroenteric fistula in a newborn presenting with meningitis. Associated anomalies included agenesis of the corpus callosum, short colon, malrotation, epispadias, and an abnormally high bifurcation of the abdominal aorta and inferior vena cava. The embryological mechanisms and etiologic theories are discussed in short.
متن کاملMagnetic resonance imaging of spinal dysraphism.
Spinal cord development occurs through three consecutive periods. Gastrulation (weeks 2-3) is characterized by conversion of the embryonic disk from a bilaminar to a trilaminar arrangement and establishment of a notochord. Primary neurulation (weeks 3-4) produces the uppermost nine tenths of the spinal cord. Secondary neurulation and retrogressive differentiation (weeks 5-6) result in formation...
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ورودعنوان ژورنال:
- Indian journal of pediatrics
دوره 69 8 شماره
صفحات -
تاریخ انتشار 1993