Erin E Perrone1, Jacob Olson2, Jamie M Golden3, Gail E Besner4, Christopher P Gayer5, Saleem Islam6, Gerald Gollin7. 1. University 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. Electronic address: eperrone@med.umich.edu. 2. Loma Linda University Children's Hospital, Loma Linda, CA; Nationwide Children's Hospital, Columbus, OH 43205. Electronic address: JKOlson@llu.edu. 3. Children's Hospital of Los Angeles, Los Angeles, CA. Electronic address: Jamie.m.golden@gmail.com. 4. Nationwide Children's Hospital, Columbus, OH 43205. Electronic address: gail.besner@nationwidechildrens.org. 5. Children's Hospital of Los Angeles, Los Angeles, CA. Electronic address: cgayer@chla.usc.edu. 6. University of Florida, Gainesville, FL. Electronic address: saleem.islam@surgery.ufl.edu. 7. Rady Children's Hospital, San Diego, CA. Electronic address: ggollin@rchsd.org.
Abstract
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. Published by Elsevier Inc.
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. Published by Elsevier Inc.
Entities:
Keywords:
Atresia; Classification; Closed/closing; Gastroschisis; Short gut