Jeon Sungmi, Kim Yumin, Phi Ji Hoon, Chung Jee Hyuk
Division of Pediatric Plastic Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Arch Plast Surg. 2023 Feb 6;50(1):54-58. doi: 10.1055/a-1938-0906. eCollection 2023 Jan.
Patients with Crouzon syndrome have increased risks of cerebrospinal fluid rhinorrhea and meningoencephalocele after LeFort III osteotomy. We report a rare case of meningoencephalocele following LeFort III midface advancement in a patient with Crouzon syndrome. Over 10 years since it was incidentally found during transnasal endoscopic orbital decompression, the untreated meningoencephalocele eventually led to intermittent clear nasal discharge, frontal headache, and seizure. Computed tomography and magnetic resonance imaging demonstrated meningoencephalocele in the left frontal-ethmoid-maxillary sinus through a focal defect of the anterior cranial base. Through bifrontal craniotomy, the meningoencephalocele was removed and the anterior cranial base was reconstructed with a pericranial flap and split calvarial bone graft. Secondary frontal advancement was concurrently performed to relieve suspicious increased intracranial pressure, limit visual deterioration, and improve the forehead shape. Surgeons should be aware that patients with Crouzon syndrome have the potential for an unrecognized dural injury during LeFort III osteotomy due to anatomical differences such as inferior displacement and thinning of the anterior cranial base.
克鲁宗综合征患者在LeFort III截骨术后发生脑脊液鼻漏和脑膜脑膨出的风险增加。我们报告了1例克鲁宗综合征患者在LeFort III面中部前移术后发生脑膜脑膨出的罕见病例。自经鼻内镜眼眶减压术中偶然发现以来的10多年里，未治疗的脑膜脑膨出最终导致间歇性清亮鼻漏、额部头痛和癫痫发作。计算机断层扫描和磁共振成像显示，通过前颅底的一个局灶性缺损，脑膜脑膨出位于左侧额-筛-上颌窦。通过双额开颅手术，切除脑膜脑膨出，并使用帽状腱膜瓣和颅骨劈开骨移植重建前颅底。同时进行二期额部前移，以缓解可疑的颅内压升高、限制视力恶化并改善前额形状。外科医生应意识到，由于前颅底下移和变薄等解剖学差异，克鲁宗综合征患者在LeFort III截骨术中存在未被识别的硬脑膜损伤的可能性。
