College of Medical and Dental Sciences, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom.
Department of Paediatric Surgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, United Kingdom.
J Pediatr Surg. 2022 Sep;57(9):39-44. doi: 10.1016/j.jpedsurg.2021.10.057. Epub 2021 Nov 7.
Nutritional management of children with epidermolysis bullosa (EB) presents multiple challenges including reduced oral intake compounded by mucosal fragility. Gastrostomy tube feeding is effective in improving nutritional status however there is limited data on the safety and tolerance of this technique in EB children. We aim to review the effectiveness and morbidity of our minimally invasive two-port laparoscopic-assisted gastrostomy (LAG) approach using Seldinger techniques with serial dilatations in children with EB.
A retrospective, observational cohort study was conducted on all consecutive EB patients who underwent LAG tube insertion between 2009 and 2019. Patient demographics, admission details and 12-month clinical outcomes were reported.
32 EB patients underwent LAG placement. Median age at insertion was 7.3 (IQR ± 6.3) years, with 8 (25.0%) and 3 (9.4%) of patients also undergoing oesophageal dilatation and fundoplication, respectively. Minor complications arose in 58.1% of patients including: peri-stomal overgranulation (25.8%), gastrostomy infection (22.6%), pain (22.6%), mild gastrostomy leakage (16.1%), blockage (9.7%) and device failure (3.2%). 2 patients (6.5%) developed major complications with extensive gastrostomy site leakage. Improvements in growth were reflected in mean height Z-scores (-1.99 to -1.71). Mean weight Z-scores improved in patients aged 0-10 years (-2.30 to -1.61) and mean BMI Z-scores increased in patients more than 10 years (-2.71 to -1.46). No cases of gastrostomy-related mortality were reported.
LAG is well-tolerated in EB patients with improvements in growth and minimal morbidity 12-months post-gastrostomy insertion. An extended follow-up period is required to ascertain the long-term implications of gastrostomy feeding.
大疱性表皮松解症（EB）患儿的营养管理存在多种挑战，包括因口腔黏膜脆弱而导致的摄入减少。胃造口管喂养可有效改善营养状况，但关于该技术在 EB 患儿中的安全性和耐受性的数据有限。我们旨在回顾使用 Seldinger 技术进行的微创两孔腹腔镜辅助胃造瘘术（LAG）的效果和发病率，该技术通过连续扩张在 EB 患儿中进行。
对 2009 年至 2019 年间接受 LAG 管插入的所有连续 EB 患者进行回顾性、观察性队列研究。报告患者人口统计学、入院详细信息和 12 个月的临床结果。
32 名 EB 患者接受了 LAG 放置。插入时的中位年龄为 7.3（IQR ± 6.3）岁，8 名（25.0%）和 3 名（9.4%）患者还分别进行了食管扩张和胃底折叠术。58.1%的患者出现轻微并发症，包括：吻合口周围过度肉芽组织形成（25.8%）、胃造口感染（22.6%）、疼痛（22.6%）、轻度胃造口漏（16.1%）、堵塞（9.7%）和设备故障（3.2%）。2 名患者（6.5%）出现广泛的胃造口部位漏并发症。生长的改善反映在平均身高 Z 评分（-1.99 至-1.71）上。0-10 岁患者的平均体重 Z 评分改善（-2.30 至-1.61），10 岁以上患者的平均 BMI Z 评分增加（-2.71 至-1.46）。没有与胃造口相关的死亡病例报告。
LAG 在 EB 患者中耐受性良好，在胃造口插入后 12 个月内生长改善，发病率低。需要延长随访期以确定胃造口喂养的长期影响。
