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Excellent long-term outcome for survivors of apple peel atresia
S Festen1, J C Brevoord, G A Goldhoorn
1Amsterdam, Nijmegen, Rotterdam, Maastricht, Groningen, and Utrecht, The Netherlands.
Insights
Apple peel atresia, a rare condition, presents significant early risks but shows low long-term morbidity. Children surviving the critical period often achieve normal growth and development with good bowel function.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatal Care
Background:
- Apple peel atresia is an exceptionally rare form of small bowel atresia.
- Its rarity and high mortality rate limit understanding of long-term outcomes.
Purpose of the Study:
- To investigate the long-term outcomes for children diagnosed with apple peel atresia.
Main Methods:
- Retrospective review of 15 patient charts, operative reports, and office notes from six Dutch pediatric surgical centers.
- Long-term follow-up data obtained through office note review and patient questionnaires.
Main Results:
- The median age at operation was 1.5 days.
- Postoperative complications included cholestasis (53%) and sepsis (40%); 20% of patients died.
- At a median follow-up of 24 months, most children experienced normal growth and development, though some had short bowel syndrome or growth retardation initially.
Conclusions:
- Despite significant short-term morbidity (e.g., short bowel syndrome, sepsis), long-term morbidity in apple peel atresia is low.
- Survival through the operative and immediate postoperative periods, managing malnutrition and total parenteral nutrition, leads to a good prognosis for normal bowel function, growth, and development.
Background:
Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children.
Methods:
The patient charts, operative reports, and office notes of 15 children with apple peel atresia from 6 pediatric surgical centers in the Netherlands were reviewed. Long-term follow-up was assessed through review of office notes and through questionnaires.
Results:
The median age at the time of operation was 1.5 days. Postoperatively, 53% suffered from cholestasis, and 40% were septic. Three patients died (20%). At follow-up at a median age of 24 months, 1 child showed growth retardation and 2 children suffered from short bowel syndrome. At the time of the questionnaire, all children showed normal growth and development.
Conclusions:
Even though children with apple peel atresia often suffer serious morbidity like short bowel syndrome and sepsis during the postoperative course, late morbidity turned out to be low. If the patients survive the operative and direct postoperative period, and survive the morbidity associated with malnutrition and the long-term use of total parenteral nutrition, they have a good chance of having normal bowel function with normal growth and development.
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