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脐膨出和腹裂的临床特征和结局:20 年多中心区域性经验。

Clinical characteristics and outcome of omphalocele and gastroschisis: a 20-year multicenter regional experience.

机构信息

Department of Surgery, Hong Kong Children's Hospital, Hong Kong, China.

Division of Paediatric Surgery, Department of Surgery, The University of Hong Kong, Hong Kong, China.

出版信息

Pediatr Surg Int. 2024 Jul 25;40(1):210. doi: 10.1007/s00383-024-05783-0.

DOI:10.1007/s00383-024-05783-0
PMID:39052072
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.

摘要

脐膨出和腹裂是最常见的腹壁缺陷类型。全面的本地经验有助于父母做出关于妊娠的决策,并预见疾病的进程。对 2003 年 1 月至 2023 年 2 月期间香港所有 3 家儿科外科中心的腹壁缺陷患者进行了回顾性研究。所有连续诊断为脐膨出和腹裂的患者均被纳入研究,排除其他形式的腹壁缺陷。收集了人口统计学和短期及长期结果参数的数据。共回顾了 99 例病例,其中 85 例符合纳入标准。诊断包括巨大脐膨出(n=49,57.6%)、小脐膨出(n=22,25.9%)和腹裂(n=14,16.5%),平均胎龄为 37 周(标准差 2.2),出生体重为 2.7 公斤(标准差 0.6)。脐膨出最常与心血管疾病(n=28,39.4%)和染色体缺陷(n=11,15.5%)相关。手术方式包括一期修复(n=38,53.5%)、分期关闭(n=30,42.3%),平均 8.6 天(标准差 4.7)进行减仓,以及保守治疗(n=3,4.2%)。死亡率为 14.1%(n=10),并发症发生率为 36.6%(n=26)。大多数患者在最近的随访中具有正常的智力发育(92.5%)和生长(79.2%)。对于腹裂,有 1 例(7.1%)患儿存在肠闭锁。手术方式包括一期修复(n=9,64.3%)和分期关闭(n=5,35.7%),平均 8 天(标准差 3.5)进行减仓。并发症发生率为 21.4%(n=3),死亡率为 7.1%。所有患者均具有正常的智力发育和生长。本研究的平均随访时间为 76.9 个月(标准差 62.9)。本研究中大多数腹壁缺陷患者均接受了手术治疗,总体生存率和长期预后良好。这些信息对于父母的产前和产后咨询至关重要。

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Abdominal Wall Defects-Current Treatments.腹壁缺损——当前的治疗方法
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