Treatment of Congenital Microgastria

被引:3
作者
Ruczynski, Larissa I. A. [1 ]
Botden, Sanne M. B. I. [2 ]
Daniels-Scharbatke, Horst E. [2 ]
Schurink, Maarten [2 ]
de Blaauw, Ivo [2 ]
机构
[1] Radboudumc Hlth Acad Radboudumc, Dept Res Learning & Educ, Nijmegen, Netherlands
[2] Radboudumc Amalia Childrens Hosp, Dept Pediat Surg, Nijmegen, Netherlands
关键词
microgastria; congenital microgastria; Hunt– Lawrence pouch; total esophageal gastric dissociation; intestinal malrotation; FOLLOW-UP; GASTRIC DISSOCIATION; ROBIN-SEQUENCE; MANAGEMENT; DEFICIENCY; ASSOCIATION; NEWBORN;
D O I
10.1055/s-0040-1710027
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Introduction Congenital microgastria is an extremely rare birth defect. The aim of this study was to present an overview of existing literature on the treatment of microgastria. Materials and Methods The term "microgastria" was used in a PubMed and Medline search. Since merely case reports were found, only a narrative synthesis with limited statistical analysis can be given. Data of different treatment modalities were collected and divided into two groups: conservative or less invasive treatment (C/LT, i.e., modified diet or a gastrostomy/jejunostomy) and extensive gastric surgery (EGS, i.e., Hunt-Lawrence pouch or total esophageal gastric dissociation). Clinical outcome parameters (nutrition, growth pattern, and mortality) were compared. Results Out of 73 articles published from 1973 to 2019, 38 articles describing 51 cases were included. In four patients, microgastria was an isolated anomaly (8%). Type of treatment was described in only 46 patients, 19 were treated by C/LT. Mortality was 9/19 (47%) in the C/LT group versus 4/27 (15%) in the EGS group (chi-square=5.829, p =0.016, Fisher=0.022). There was a negative correlation between the invasiveness of the treatment and both mortality ( r =-0.356, p =0.015) and comorbidity ( r =-0.506, p <0.001). Patients in the C/LT group had significantly more comorbidity than in the EGS group (mean=4.32 vs. 2.26, p =0.001). There was a positive correlation between comorbidity and mortality ( r =0.400, p =0.006). Median follow-up was 42 months (range: 1-240). Type and way of nutrition were poorly described. In at least 9 of the 33 surviving patients, oral feeding was reported as normal, of whom 8 belonged to the EGS group. In all patients, growth could be acknowledged, but in comparison to peers, final body length was less. There was no difference in final body length between the two treatment groups. Conclusion In patients with congenital microgastria, only minimal differences in clinical outcome in terms of type of nutrition and body growth were found when C/LT was compared with treatment by EGS. Mortality was significantly higher in the first group as well as the amount of comorbidities.
引用
收藏
页码:129 / 134
页数:6
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