Dorsal enteric sinus with spina bifida: a rare form of split notochord syndrome.
Split notochord syndrome is a rare congenital malformation that results in spinal anomalies associated with anomalies of the gastrointestinal tract and central nervous system. This report presents an infrequently described variant of this syndrome. A 9-month-old female presented with a dorsal midline mass since birth which was partially covered by skin, and part of the lesion gave a gross appearance of intestinal mucosa. MRI was suggestive of partial sacral agenesis with spinal dysraphism with tethered cord. Excision of the mass with repair of the spinal defect was done. Histopathology confirmed the presence of gastrointestinal mucosa.
Nanda
Monika
M
Department of Pediatric Surgery, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Bawa
Monika
M
Kanojia
Ravi Prakash
RP
Rao
K L N
KL
D005260:Female; D006801:Humans; D007223:Infant; D007413:Intestinal Mucosa / Q000002:abnormalities / Q000601:surgery; D007422:Intestines / Q000002:abnormalities* / Q000601:surgery*; D009436:Neural Tube Defects / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D009672:Notochord / Q000002:abnormalities* / Q000601:surgery; D016135:Spinal Dysraphism / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D013577:Syndrome