Clinical characteristics and outcome of omphalocele and gastroschisis: a 20-year multicenter regional experience
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In brief
In brief
This 20-year Hong Kong multicenter study reviews 85 cases of omphalocele and gastroschisis, reporting surgical management approaches, associated anomalies, and long-term outcomes. Most patients achieved normal development with overall survival rates of 85.9% for omphalocele and 92.9% for gastroschisis, providing valuable prognostic data for antenatal counseling.
- Omphalocele has higher mortality (14.1%) than gastroschisis (7.1%) and is frequently associated with cardiac (39.4%) and chromosomal defects (15.5%).
- Primary repair was achieved in 53.5% of omphalocele and 64.3% of gastroschisis cases; staged closure averaged 8-9 days of silo reduction.
- Long-term outcomes are favorable: 92.5% had normal intellectual development and 79.2% normal growth at mean 77-month follow-up.
- Gastroschisis has lower complication rate (21.4%) compared to omphalocele (36.6%), with intestinal atresia occurring in 7.1% of gastroschisis cases.
- Overall survival rate is good for both conditions when managed surgically, providing reassuring data for antenatal and postnatal parental counseling.
Written by the GCMD Library team from the article.
Abstract
Omphalocele and gastroschisis are the most common types of abdominal wall defects. Comprehensive local experience helps parents to make decisions on the pregnancy and foresee the disease journey. A retrospective review of abdominal wall defect patients in all three pediatric surgical centers in Hong Kong between January 2003 and February 2023 was conducted. All patients consecutively diagnosed with omphalocele and gastroschisis were included, excluding other forms. Data of demographics and short- and long-term outcome parameters were collected. A total of 99 cases were reviewed and 85 patients met the inclusion criteria. Diagnoses include omphalocele major (n = 49, 57.6%), omphalocele minor (n = 22, 25.9%) and gastroschisis (n = 14, 16.5%), with mean gestational age 37 weeks (SD 2.2) and birth weight 2.7 kg (SD 0.6). Omphalocele is most commonly associated with cardiovascular (n = 28, 39.4%) and chromosomal defects (n = 11, 15.5%). Surgical procedures including primary repair (n = 38, 53.5%), staged closure (n = 30, 42.3%) with average 8.6 days (SD 4.7) of silo reduction, and conservative management (n = 3, 4.2%) were performed. The mortality rate was 14.1% (n = 10) and the complication rate was 36.6% (n = 26). The majority of patients had normal intellectual development (92.5%) and growth (79.2%) on the latest follow-up. For gastroschisis, one patient (7.1%) had intestinal atresia. Surgical procedures included primary repair (n = 9, 64.3%) and staged closure (n = 5, 35.7%) with average 8 days (SD 3.5) of silo reduction. Complication rate was 21.4% (n = 3), with one mortality (7.1%). All patients had normal intellectual development and growth. The mean follow-up time of this series is 76.9 months (SD 62.9). Most abdominal wall defects in our series were managed surgically with a good overall survival rate and long-term outcome. This information is essential during antenatal and postnatal counseling for parents.