Journal List > Korean J Obstet Gynecol > v.55(9) > 1088512

Korean J Obstet Gynecol. 2012 Sep;55(9):655-658. Korean.
Published online September 17, 2012.  https://doi.org/10.5468/KJOG.2012.55.9.655
Copyright © 2012. Korean Society of Obstetrics and Gynecology
Colon atresia, a rare comorbid condition in VACTERL association
Ji Hye Hwangbo, MD, Eun Young Heo, MD, Youn Sil Choo, MD, Jin Young Bae, MD, Mi Ju Kim, MD and Won Joon Seong, MD, PhD
Department of Obstetrics and Gynecology, Kyungbook National University School of Medicine, Deagu, Korea.

Corresponding author: Won Joon Seong, MD, PhD. Department of Obstetrics and Gynecology, Kyungbook National University School of Medicine, 130 Dongdeok-ro, Jung-gu,Deagu 700-721, Korea. Tel: +82-53-420-5724, Fax: +82-53-423-7905, Email: wjseong@knu.ac.kr
Received May 03, 2012; Revised June 29, 2012; Accepted July 24, 2012.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


Abstract

Vertebral, anal, cardiac, tracheoesophageal, renal and limb (VACTERL) association is defined by the presence of at least three of the following six congenital malformations: vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies and limb abnormalities. The abdominal cystic mass, suspicious of colon atresia was identified on prenatal ultrasonography. The new born infant exhibited single umbilical artery, hemivertebra, hypoplastic nasal bone, ventricular septal defect, patent ductus arteriosus, colon atresia. For treatment of colon atresia, two staged surgery was provided by a pediatric surgeon. We experienced colon atresia, a very rare comorbid condition in VACTERL association. Prompt diagnosis and surgical correction was possible with the cooperation of pediatric surgeons, obstetricians and pediatricians.

Keywords: VACTERL association; Colon atresia; Congenital malformations

Figures


Fig. 1
At 28/6 weeks of gestation, the hemivertebra was identified between thoracic vertebra No.10 and No.11 on prenatal ultrosonogram (arrow).
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Fig. 2
At 38/1 weeks of gestation, the abdominal cystic mass 3×7 cm, suspicous of colon atresia was identified on ultrasonogram.
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Fig. 3
At 38/1 weeks of gestation, a left ectopic kidney was identified.
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Fig. 4
On the date of birth the baby showed a hypoplastic nasal bone (arrow).
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Fig. 5
On the first day of birth, the lopogram showed narrowing colon near the hepatic flexure, representing colon atresia (arrow).
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References
1. Botto LD, Khoury MJ, Mastroiacovo P, Castilla EE, Moore CA, Skjaerven R, et al. The spectrum of congenital anomalies of the VATER association: an international study. Am J Med Genet 1997;71:8–15.
2. Källén K, Mastroiacovo P, Castilla EE, Robert E, Källén B. VATER non-random association of congenital malformations: study based on data from four malformation registers. Am J Med Genet 2001;101:26–32.
3. Evans JA, Vitez M, Czeizel A. Patterns of acrorenal malformation associations. Am J Med Genet 1992;44:413–419.
4. Khoury MJ, Cordero JF, Greenberg F, James LM, Erickson JD. A population study of the VACTERL association: evidence for its etiologic heterogeneity. Pediatrics 1983;71:815–820.
5. Czeizel A, Ludányi I. An aetiological study of the VACTERL-association. Eur J Pediatr 1985;144:331–337.
6. Damian MS, Seibel P, Schachenmayr W, Reichmann H, Dorndorf W. VACTERL with the mitochondrial np 3243 point mutation. Am J Med Genet 1996;62:398–403.
7. de Jong EM, Douben H, Eussen BH, Felix JF, Wessels MW, Poddighe PJ, et al. 5q11.2 deletion in a patient with tracheal agenesis. Eur J Hum Genet 2010;18:1265–1268.
8. Solomon BD. VACTERL/VATER Association. Orphanet J Rare Dis 2011;6:56.
9. Oral A, Caner I, Yigiter M, Kantarci M, Olgun H, Ceviz N, et al. Clinical characteristics of neonates with VACTERL association. Pediatr Int 2012;54:361–364.
10. Mittal A, Airon RK, Magu S, Rattan KN, Ratan SK. Associated anomalies with anorectal malformation (ARM). Indian J Pediatr 2004;71:509–514.
11. Evans CH. Atresias of the gastrointestinal tract. Int Abstr Surg 1951;92:1–8.
12. Franken EA, Smith WL. In: Gastrointestinal imaging in pediatrics. 2nd ed. New York: Harper & Row; 1982.
13. Boles ET Jr, Vassy LE, Ralston M. Atresia of the colon. J Pediatr Surg 1976;11:69–75.