Dahalani S, Demonceau N, Mulder A, Maton P, Henroteaux A, Thimmesch M
Service de Pédiatrie, CHC Clinique du MontLégia, Liège, Belgique.
Service de Neurochirurgie, CHC Clinique du MontLégia, Liège, Belgique.
Rev Med Liege. 2020 Dec;75(12):761-762.
Severe hydrocephalus in a child with congenital myotonic dystrophy X A young patient with congenital myotonic dystrophy, or Steinert's disease, presented at the age of 4.5 months with an increase of his head circumference and signs of intracranial hypertension. The results of the radiological exams reveal a major hydrocephalus. The patient condition evolved favourably after ventriculoperitoneal bypass. While ventriculomegaly is common in congenital myotonic dystrophy, hydrocephalus with signs of intracranial hypertension is rare, hence the need of regular monitoring of head circumference.
先天性肌强直性营养不良患儿的重度脑积水 X 一名患有先天性肌强直性营养不良（即斯坦纳特病）的幼儿在4.5个月大时出现头围增大和颅内高压症状。影像学检查结果显示存在重度脑积水。该患者在接受脑室腹腔分流术后病情向好发展。虽然脑室扩大在先天性肌强直性营养不良中很常见，但伴有颅内高压症状的脑积水却很罕见，因此需要定期监测头围。
