Split notochord syndrome variant: prenatal findings and neonatal management.
Spilt notochord syndrome is an extremely rare form of spinal dysraphism characterized by a complete cleft of the spine and a persistent communication between endoderm and ectoderm. A variant of split notochord syndrome was diagnosed in a 25-week-old fetus showing a prolapsed congenital colostomy and a spinal cystic lesion. The final diagnosis included protruding colon segment, imperforate anus with a rectourethral fistula and lipomyelomeningocele. The ultrasound features of the condition and the post-natal management are discussed. The neonate was successfully treated with a posterior sagittal anorectoplasty, while the lipomyelomeningocele was resected at a later stage.
Agangi
A
A
Prenatal Diagnosis Unit, Department of Gynecology and Obstetrics, University Federico II of Naples, Naples, Italy.
Paladini
D
D
Bagolan
P
P
Maruotti
G M
GM
Martinelli
P
P
D000015:Abnormalities, Multiple / Q000601:surgery*; D000328:Adult; D003106:Colon / Q000002:abnormalities; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007412:Intestinal Fistula / Q000151:congenital / Q000601:surgery; D008297:Male; D008591:Meningomyelocele / Q000473:pathology / Q000601:surgery; D009672:Notochord / Q000002:abnormalities*; D011247:Pregnancy; D016135:Spinal Dysraphism / Q000150:complications / Q000473:pathology / Q000601:surgery; D013577:Syndrome; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal