Kennedy E M, Gruber D P, Billmire D A, Crone K R
Department of Plastic Surgery, University of Cincinnati, Children's Hospital Medical Center, and the Mayfield Clinic, Ohio, USA.
J Neurosurg. 1997 Nov;87(5):677-81. doi: 10.3171/jns.1997.87.5.0677.
The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.
儿童经蝶骨脑膨出的外科治疗存在争议。经颅入路进行复位和修复与术后高发病率、死亡率及下丘脑功能障碍相关。在本研究中，4例手术时年龄为3至35个月的患儿成功接受了经腭部修复2例脑膨出和2例脑膜膨出的手术。2例患儿在婴儿期出现鼻塞，1例出现不明原因的脑膜炎，1例患儿在癫痫评估时偶然发现病变。2例患儿患有正中面裂综合征，另1例伴有I型阿诺德 - 奇亚里畸形，第4例无其他颅骨异常。所有患者均接受了包括磁共振（MR）成像在内的术前评估。根据需要进行听觉、眼科、遗传学、内分泌学或其他评估。通过硬腭和软腭的正中缝进入病变。在切开鼻腭黏膜后，所有脑膨出均易于显露和分离。在切开并分离覆盖的黏膜后，通过表面凝固使硬脑膜囊及其内容物复位。复位后，4例患者中有3例骨缺损被闭塞。未打开硬脑膜，未切除异常神经组织。在随访评估中,所有患者术前症状均消失，无感染或持续发病的迹象。随访MR成像显示所有病例均有缩小。作者得出结论，这种经腭部入路治疗幼儿经蝶骨脑膨出是安全可靠的。
