Guillen Arguello Roboan, Sader Nicholas, McComb J Gordon
Department of Neurosurgery, Louisiana State University Health Sciences Center, New Orleans, Louisiana.
Department of Neurosurgery, Oklahoma Children's Hospital, The University of Oklahoma College of Medicine, Oklahoma City, Oklahoma.
J Neurosurg Case Lessons. 2024 Dec 16;8(25). doi: 10.3171/CASE24590.
Camurati-Engelmann disease (CED) is an extremely rare autosomal dominant genetic disorder that can cause increased intracranial pressure (ICP) secondary to cranial hyperostosis, which decreases intracranial volume. Surgical procedures to reduce ICP in medically refractory cases include intracranial volume expansion and ventriculoperitoneal shunting.
The authors present the case of a pediatric patient with CED and medically refractory increased ICP who underwent unilateral hemicraniectomy with titanium cranioplasty, resulting in a complete long-term resolution of symptoms.
Unilateral hemicraniectomy with titanium cranioplasty is a feasible surgical treatment for CED in pediatric patients with medically refractory increased ICP and papilledema. https://thejns.org/doi/10.3171/CASE24590.
卡穆拉蒂-恩格尔曼病（CED）是一种极其罕见的常染色体显性遗传病，可因颅骨增生导致颅内压（ICP）升高，进而使颅内容积减小。在药物治疗无效的情况下，降低颅内压的外科手术包括扩大颅内容积和脑室腹腔分流术。
作者报告了1例患有CED且药物治疗无效的颅内压升高的儿科患者，该患者接受了单侧颅骨切除术及钛网颅骨成形术，症状得到了长期完全缓解。
对于药物治疗无效且伴有视乳头水肿的CED儿科患者，单侧颅骨切除术及钛网颅骨成形术是一种可行的外科治疗方法。https://thejns.org/doi/10.3171/CASE24590 。
