Los Angeles, Calif. From the Division of Plastic and Reconstructive Surgery, David Geffen School of Medicine, University of California, Los Angeles School of Medicine.
Plast Reconstr Surg. 2010 Jul;126(1):197-204. doi: 10.1097/PRS.0b013e3181dbbf6c.
Transsphenoidal encephaloceles are rare cystic herniations of meninges, cerebrospinal fluid, and/or brain matter resulting from incomplete closure of the cranial base and may be associated with midfacial, central nervous system, and endocrine anomalies. Although some centers choose not to operate because of risks, the authors document their staged operative approach to avoid recurrent meningitis, progressive neurologic decline, and other symptoms.
Patients with symptomatic transsphenoidal encephaloceles who underwent staged treatment with intracranial and transpalatal cyst correction, facial bipartition, and cleft palate repair were studied (n = 4). Outcome measures included perioperative complications, recurrence, interdacyron distance comparison, and speech and developmental assessments.
The authors' staged correction of transsphenoidal encephaloceles as detailed in this article proved successful in all four patients, with no recurrence of meningitis, no cerebrospinal fluid leakage, alleviation of headaches, aesthetic improvement, and no encephalocele relapse. Skeletal correction by computed tomographic scan showed correction of interdacyron distance with a mean 22-mm reduction (56 percent). After the initial procedure of encephalocele correction, speech scores fell from 2.2 (borderline incompetent) to 7.9 (incompetent) but improved after the cleft palate repair and speech therapy to 1.4 (borderline competent). Follow-up developmental tests showed normal global evaluations in memory and attention skills in all but one patient (who had persistent deficiencies consistent with preoperative evaluations).
A staged operative treatment for symptomatic transsphenoidal encephaloceles offers functional and morphologic correction.
经蝶窦脑膨出是由于颅底不完全闭合导致的脑膜、脑脊液和/或脑组织的罕见囊性疝出，可能与面中部、中枢神经系统和内分泌异常有关。尽管一些中心因风险而选择不手术，但作者记录了他们的分期手术方法，以避免复发性脑膜炎、进行性神经功能下降和其他症状。
对接受颅内和经腭裂囊肿矫正、面部二分法和腭裂修复的有症状经蝶窦脑膨出患者进行了分期治疗（n = 4）。结果测量包括围手术期并发症、复发、眶距差比较以及言语和发育评估。
作者在本文中详细介绍的经蝶窦脑膨出分期矫正术在所有 4 例患者中均取得成功，无脑膜炎复发、无脑脊液漏、头痛缓解、美容改善、无脑膨出复发。CT 扫描显示的骨骼矫正结果显示眶距差平均减少 22mm（56%）。在初次脑膨出矫正手术后，言语评分从 2.2 分（边缘性不称职）降至 7.9 分（不称职），但在腭裂修复和言语治疗后提高至 1.4 分（边缘性称职）。随访发育测试显示，除 1 例患者（存在与术前评估一致的持续性缺陷）外，所有患者的记忆和注意力技能均有正常的总体评估。
对有症状的经蝶窦脑膨出进行分期手术治疗可提供功能和形态矫正。
