Colonic Atresia, Malrotation, and Hirschsprung’s Disease in a Newborn: Report of 2 Cases

The concurrent occurrence of colonic atresia, malrotation, and Hirschsprung's disease in neonates is extremely rare.These anomalies often share embryologic origins and present overlapping clinical symptoms that complicate diagnosis and management.We report two neonatal cases with this rare triad.Case 1 involved a term neonate initially diagnosed with esophageal atresia and later found to have colonic atresia, malrotation, and Hirschsprung's disease.Case 2 was a preterm neonate presenting with abdominal distension and perforation, ultimately diagnosed with the same triad.Both underwent staged surgical management, including Duhamel's procedures after confirming aganglionosis.Awareness of the possible coexistence of these anomalies is essential in neonates with colonic atresia and non-fixed colon.Surgical planning should anticipate aganglionosis and include rectal biopsy.This report emphasizes the importance of early suspicion and multidisciplinary approach for optimal outcomes.

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Publication Details

Journal
Advances in Pediatric Surgery
Published
2025-01-01
DOI
https://doi.org/10.13029/aps.2025.31.1.41
Primary Topic
Intestinal Malrotation and Obstruction Disorders
Type
article
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article

Colonic Atresia, Malrotation, and Hirschsprung’s Disease in a Newborn: Report of 2 Cases

Sungjoo Park, Jeong-Meen Seo, Sanghoon Lee, Won‐Tae Kim
Advances in Pediatric Surgery
Intestinal Malrotation and Obstruction Disorders
article

Colonic Atresia, Malrotation, and Hirschsprung’s Disease in a Newborn: Report of 2 Cases

Sungjoo Park, Jeong-Meen Seo, Sanghoon Lee, Won‐Tae Kim
article en

Abstract

The concurrent occurrence of colonic atresia, malrotation, and Hirschsprung's disease in neonates is extremely rare.These anomalies often share embryologic origins and present overlapping clinical symptoms that complicate diagnosis and management.We report two neonatal cases with this rare triad.Case 1 involved a term neonate initially diagnosed with esophageal atresia and later found to have colonic atresia, malrotation, and Hirschsprung's disease.Case 2 was a preterm neonate presenting with abdominal distension and perforation, ultimately diagnosed with the same triad.Both underwent staged surgical management, including Duhamel's procedures after confirming aganglionosis.Awareness of the possible coexistence of these anomalies is essential in neonates with colonic atresia and non-fixed colon.Surgical planning should anticipate aganglionosis and include rectal biopsy.This report emphasizes the importance of early suspicion and multidisciplinary approach for optimal outcomes.

Advances in Pediatric SurgeryVol. 31(1)
Samsung Medical Center (KR), Sungkyunkwan University (KR)
Zero hunger
Openalex Percentile: Top 74%
Intestinal Malrotation and Obstruction Disorders
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