Successful Staged Correction of Transsphenoidal Encephaloceles

Abstract

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.