Abstract
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.
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00383-005-1604-5/MediaObjects/383_2005_1604_Fig1_HTML.gif)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00383-005-1604-5/MediaObjects/383_2005_1604_Fig2_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00383-005-1604-5/MediaObjects/383_2005_1604_Fig3_HTML.jpg)
Similar content being viewed by others
References
Dejardin A, Goffette P, Moulin P, Verhelst R, Cornu G, De Plaen JF, Persu A (2004) Severe hypoplasia of the abdominal aorta and its branches in a patient and his daughter. J Intern Med 255:130–136
Terramani TT, Salim A, Hood DB, Rowe VL, Weaver FA (2002) Hypoplasia of the descending thoracic and abdominal aorta: a report of two cases and review of the literature. J Vasc Surg 36:844–848
Asabe K, Nagasaki A (2004) Double atresia of hindgut with ileal stenosis: a case report. Asian J Surg 27:49–51
Ein SH (1997) Imperforate anus (anal agenesis) with rectal and sigmoid atresias in a newborn. Pediatr Surg Int 12:449–451
Nitta K, Iwafuchi M, Ohsawa Y, Uchiyama M, Yamagiwa I, Hirota M, Naito M, Hirokawa K (1987) A case of congenital colonic atresia associated with atresia ani. J Pediatr Surg 22:1025–1026
Heinen FL, Prieto F (1987) Rectovestibular fistula associated with colonic atresia. J Pediatr Surg 22:1021–1022
Al-Wafi A, Morris-Stiff G, Lari A (1998) Colonic atresia secondary to a choledochal cyst. Pediatr Surg Int 13:422–423
McHugh K, Daneman A (1991) Multiple gastrointestinal atresias: sonography of associated biliary abnormalities. Pediatr Radiol 21:355–357
Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K (1977) Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263–269
Komuro H, Makino S, Tahara K (2000) Choledochal cyst associated with duodenal obstruction. J Pediatr Surg 35:1259–1262
da Gama AD (2004) Hypoplasias of the thoracic and abdominal aorta: presentation of two cases, with evaluation 11 and 20 years after surgical management. Rev Port Cir Cardiotorac Vasc 11:205–211
Kishi M, Sakaguchi A, Tanaka S, Nisiwaki K, Horimatsu T, Takeda A, Oonishi Y, Koike T, Hujisawa T, Maeda M, Ueda T, Kawabata Y, Miyamoto K, Kusumoto N (2004) Small aorta syndrome in Japan. Nippon Naika Gakkai Zasshi 93:1186–1188
Connolly JE, Wilson SE, Lawrence PL, Fujitani RM (2002) Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg 194:774–781
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Komuro, H., Takahashi, M.I., Matoba, K. et al. Rare association of severe hypoplasia of the abdominal aorta with imperforate anus, colonic atresia, and choledochal cyst. Ped Surgery Int 22, 289–292 (2006). https://doi.org/10.1007/s00383-005-1604-5
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-005-1604-5