Pediatr Gastroenterol Hepatol Nutr.  2013 Mar;16(1):28-33. 10.5223/pghn.2013.16.1.28.

Anorectal Malformations Associated with Esophageal Atresia in Neonates

• Byun SY
• Lim RK
• Park KH
• Cho YH
• Kim HY

• KMID: 1428804
• DOI: http://doi.org/10.5223/pghn.2013.16.1.28

Abstract

PURPOSE
Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia.
METHODS
Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted.
RESULTS
The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems.
CONCLUSION
This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

Keyword

Anorectal malformation; Esophageal atresia; Neonates

MeSH Terms

Accounting
Anus, Imperforate
Colostomy
Early Diagnosis
Esophageal Atresia
Extremities
Female
Fistula
Heart
Humans
Incidence
Infant, Newborn
Male
Retrospective Studies
Tracheoesophageal Fistula
Anus, Imperforate

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