A rare variant of neuroenteric cyst: split notochord syndrome

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[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...

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A 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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A 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.

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Neuroenteric Cyst: a Rare Cause of Childhood Paraplegia

Neuroenteric cysts are rare congenital anomalies derived from the displaced endodermal tissue around the third week of the embryonic stage. We report a case of neuroenteric cyst of the spinal canal presenting with paraplegia in a 2.5-year-old boy. Despite the 12-day delay in surgical decompression, he made a complete neurological recovery. This is a unique case in regard to the clinical present...

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Holt-Oram Syndrome: A Rare Variant

Holt-Oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial and ventricular septal defects. Skeletal defects exclusively affect the upper limbs in the preaxial radial ray distribution and are bilateral and asymmetrical. They range from clinodactyly, absent or digitalised thumb, hypoplastic...

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ژورنال

عنوان ژورنال: Jornal de Pediatria

سال: 2004

ISSN: 0021-7557

DOI: 10.2223/1138