Rare association of severe hypoplasia of the abdominal aorta with imperforate anus, colonic atresia, and choledochal cyst.
Hypoplasia of the abdominal aorta (HAA) is a rare condition that causes marked hypertension. Although multiple etiologies have been postulated for HAA, congenital structural anomalies are rarely observed except in cases associated with some hereditary syndromes. The authors describe a neonatal case with HAA complicated by multiple anomalies including colonic atresia (CA), imperforate anus, choledochal cyst, facial cleft, and brain defects. This patient showed CA in the descending colon and caliber change in the transverse colon mimicking Hirschsprung disease, both of which were thought to be caused by vascular insult to the mesentery due to HAA. Although multiple surgical corrections were successfully performed, the hypertension was uncontrollable.
Komuro
Hiroaki
H
Department of Pediatric Surgery, Graduate School of Comprehensive Human Sciences, University of Tsukuba, 1-1-1 Tennodai, 305-8575 Tsukuba, Ibaraki, Japan. hiro-kom@md.tsukuba.ac.jp
Takahashi
Miho I
MI
Matoba
Kimio
K
Hori
Tetsuo
T
Hirai
Misako
M
Gotoh
Chikashi
C
Kaneko
Michio
M
D000015:Abnormalities, Multiple; D001006:Anus, Imperforate / Q000175:diagnosis* / Q000601:surgery; D001012:Aorta, Abdominal / Q000002:abnormalities* / Q000601:surgery; D001018:Aortic Diseases / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D015529:Choledochal Cyst / Q000175:diagnosis* / Q000601:surgery; D044683:Colon, Descending / Q000002:abnormalities* / Q000601:surgery; D003108:Colonic Diseases / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D003937:Diagnosis, Differential; D013505:Digestive System Surgical Procedures / Q000379:methods; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D014656:Vascular Surgical Procedures / Q000379:methods