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Closing gastroschisis: The good, the bad, and the not-so ugly
Erin E Perrone1, Jacob Olson2, Jamie M Golden3
1University of Michigan, Michigan Medicine, Department of Surgery, Section of Pediatric Surgery, C.S. Mott Children's Hospital, Ann Arbor, MI; University of Michigan, Fetal Diagnosis and Treatment Center.
Insights
A new classification system for closing gastroschisis improves disease understanding and patient counseling. This study found survival rates for closing gastroschisis are better than previously reported.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Abnormalities
Background:
- Closing gastroschisis involves bowel incarceration within a fascial ring, often with atresia.
- This condition has historically been associated with high morbidity and mortality rates.
Purpose of the Study:
- To develop and validate a new classification system for closing gastroschisis.
- To better characterize the spectrum of disease and associated outcomes.
- To improve long-term outcome prediction and patient counseling.
Main Methods:
- Retrospective case series of 53 infants with closing gastroschisis (2000-2016).
- Development of a novel classification system (Types A-D) based on bowel viability and atresia.
- IRB approval obtained prior to data collection.
Main Results:
- A new classification system (Types A-D) was developed, categorizing cases based on bowel status.
- Type A: Ischemic but viable bowel (15%). Type B: Intestinal atresia with viable external bowel (51%). Type C: Nonviable external bowel +/- atresia (26%). Type D: Completely closed defect (8%).
- Overall infant survival rate was 87%, exceeding previous reports. Long-term data provided for each type.
Conclusions:
- The new classification system accurately reflects the closing gastroschisis spectrum and aids in predicting long-term results.
- Early reduction and evaluation of external bowel are recommended unless clearly necrotic.
- Survival rates for closing gastroschisis are significantly better than previously understood.
Purpose:
The diagnosis of "closing" or "closed gastroschisis" is made when bowel is incarcerated within a closed or nearly closed ring of fascia, usually with associated bowel atresia. It has been described as having a high morbidity and mortality.
Methods:
A retrospective review of closing gastroschisis cases (n = 53) at six children's hospitals between 2000 and 2016 was completed after IRB approval.
Results:
A new classification system for this disease was developed to represent the spectrum of the disease: Type A (15%): ischemic bowel that is constricted at the ring but without atresia; Type B (51%): intestinal atresia with a mass of ischemic, but viable, external bowel (owing to constriction at the ring); Type C (26%): closing ring with nonviable external bowel +/- atresia; and Type D (8%): completely closed defect with either a nubbin of exposed tissue or no external bowel. Overall, 87% of infants survived, and long-term data are provided for each type.
Conclusions:
This new classification system better captures the spectrum of disease and describes the expected long-term results for counseling. Unless the external bowel in a closing gastroschisis is clearly necrotic, it should be reduced and evaluated later. Survival was found to be much better than previously reported.
Type Of Study:
Retrospective case series with no comparison group.
Level Of Evidence:
Level IV.
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