A case of split notochord syndrome with congenital ileal atresia, the total absence of a colon, and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula.
Split notochord syndrome (SNS) is an extremely rare anomaly. This report presents the case of a male infant with SNS associated with congenital ileal atresia and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula. Dorsal fistulography and vesicography were useful and essential for the detailed study of the topology in this patient. The embryological mechanism and etiologic theories are discussed with a review of 19 cases reported in the literature.
Asagiri
Kimio
K
Department of Pediatric Surgery, School of Medicine, Kurume University, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan. asagiri@med.kurume-u.ac.jp
Yagi
Minoru
M
Tanaka
Yoshiaki
Y
Akaiwa
Masao
M
Asakawa
Takahiro
T
Kaida
Akiko
A
Kobayashi
Hidefumi
H
Tanaka
Hiroaki
H
D000015:Abnormalities, Multiple / Q000601:surgery; D002432:Cecum / Q000002:abnormalities* / Q000601:surgery; D003106:Colon / Q000002:abnormalities* / Q000601:surgery; D017809:Fatal Outcome; D006801:Humans; D007082:Ileum / Q000002:abnormalities* / Q000601:surgery; D007231:Infant, Newborn; D008297:Male; D009436:Neural Tube Defects / Q000150:complications* / Q000601:surgery; D009672:Notochord / Q000002:abnormalities* / Q000601:surgery; D012187:Retroperitoneal Space; D013577:Syndrome; D001747:Urinary Bladder Fistula / Q000150:complications* / Q000601:surgery