Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
Department of Plastic and Reconstructive Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
Oper Neurosurg (Hagerstown). 2021 Jul 15;21(2):73-80. doi: 10.1093/ons/opab088.
Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis.
To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue.
A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac.
We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula.
The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.
脑膨出是指颅内神经组织和脑膜通过颅骨缺陷疝出。颅底脑膨出是一种罕见的前颅底缺陷，发生率为每 35000 例活产中 1 例。蝶筛型脑膨出更为罕见，发生率为每 70 万例活产中 1 例。前颅底脑膨出在婴儿中可能危及生命，表现为气道阻塞和呼吸功能受损。它们也可能表现为脑脊液（CSF）鼻漏、脓性鼻分泌物或脑膜炎。
报告一种修复含有重要神经组织的巨大蝶筛型脑膨出的新方法。
一名 16 个月大的女孩因巨大的蝶筛型脑膨出导致进行性气道阻塞而就诊，该脑膨出充满了她的口腔。她存在多种先天性异常，包括胼胝体发育不全、唇腭裂。计算机断层扫描显示前颅底完全缺失，磁共振成像显示疝囊内存在垂体腺和下丘脑。
我们采用联合显微镜和内镜的多学科方法，通过颅内外、经鼻和经腭通道进行修复。手术在一个阶段内完成，同时还修复了中线唇裂。患儿恢复良好，神经和美容效果均佳，保留了垂体和下丘脑功能，无脑脊液漏的证据。
作者描述了一种治疗含有重要脑区的巨大蝶筛型脑膨出的新的多学科方法。同时还修复了唇裂。通过多个通道进行操作的能力可以提高这种通常具有挑战性的手术的安全性和疗效。
