Introduction Colonic atresia (CA) is a very rare disease. Two entities of CA can be differentiated: primary CA and CA with abdominal wall defects (AWD). This study aimed to investigate the differences between these two entities, especially for long-term outcomes.

Materials and Methods Data from the major health insurance company (AOK) were analyzed. Sixty-two patients with ICD-10 codes Q42.1–2 and Q42.8–9 (atresia of the colon and rectum) who underwent surgery within 10 days after their first admission between 2007 and 2016 were obtained.

Results Twenty-nine patients had an AWD, and 33 patients had primary colonic atresia (PCA). Significant differences between patients with PCA and AWD were found regarding prematurity but not for other concomitant malformations. Ostomy was the initial therapy of choice for 87.9% (29/33) of patients with PCA and 65.5% (19/29) with AWD. Central venous access was significantly more often in patients with AWD. Overall, patients with CA have an excellent short-term outcome. The 1-year mortality was 3% (1/33) in PCA and 6.9% (2/29) in AWD. Delayed management did not lead to higher mortality. Higher number of admission days and higher hospital costs were related to gastrostomy and short bowel in the long term.

Conclusion The long-term outcome of CA is related to short bowel and need for initial gastrostomy but not related to AWD, prematurity, or associated anomalies.