Methylmalonic aciduria combined with hydrocephalus treated with ventriculoperitoneal shunt
10.3760/cma.j.issn.2095-428X.2017.10.018
- VernacularTitle:脑室-腹腔分流术治疗甲基丙二酸尿症并脑积水
- Author:
Hui LI
;
Yangxu GAO
;
Hongwu ZHANG
;
Baofu LIU
;
Lixue SHEN
;
Hongxin YAO
- Keywords:
Methylmalonic aciduria;
Hydrocephalus;
Ventriculoperitoneal shunt
- From:
Chinese Journal of Applied Clinical Pediatrics
2017;32(10):788-790
- CountryChina
- Language:Chinese
-
Abstract:
Objective To summarize the experience of the ventriculoperitoneal shunt in treating children with methylmalonic aciduria combined with hydrocephalus,and to assess the clinical value.Methods From September 2012 to May 2016,a total of 12 patients with methylmalonic aciduria combined with hydrocephalus in Peking University First Hospital were enrolled,including 7 boys and 5 girls.All the 12 patients underwent ventriculoperitoneal shunt.Drug therapy was performed after surgery.The clinical manifestations and imaging findings were used as the basis for adjusting the pressure of the diverter valve appropriately.The clinical condition of patients were evaluated retrospectively.The patients' clinical symptoms,signs,imaging materials,surgical complications and postoperative prognosis were analyzed.Results All the cases were followed up for 3 to 36 months,no death case and no serious postoperative complications of hydrocephalus occurred.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement stopped.The anterior fontanelle tension decreased significantly.Setting-sun sign of eyes disappeared.One out of 4 cases with convulsion and epilepsy was relieved after the operation.Seven cases of poor vision or vision loss,postoperative visual acuity were improved though not recover to normal eyesight.One case of the children with hearing loss,postoperative hearing recovered.During the follow-up period,the head CT showed that the ventricle was narrowed significantly,interstitial brain edema improved obviously.Conclusion Ventriculoperitoneal shunt is a effective method for treating children with methylmalonic aciduria combined with hydrocephalus,which is beneficial for patients with these diseases.
