Mechanism and surgical management of transsellar transsphenoidal encephalocele

We performed a retrospective study to assess the mechanisms and surgical strategies for transsellar transsphenoid encephalocele, a rare type of basal encephalocele. Its clinical presentations include multiple endocrine disturbances, visual deficits, cerebrospinal fluid rhinorrhea and dyspnea. However, little is known about the occurrence and optimal treatment of this disease. We retrospectively reviewed six patients who were treated in our hospital from October 2003 to September 2013; five male patients and one female, with an average age of 10 years (range: 2 - 28). We collected data on their general condition, medical history, clinical features, and outcomes. An endoscopic transsphenoidal approach was used for five patients, and one refused surgery. All patients had similar imaging findings, and their pituitary gland could not be seen on MRI. Five of the patients showed gradual disease progression. The clinical symptoms were endocrine disturbance (n=6), decreased visual acuity (n=5), dyspnea (n=3) and cerebrospinal fluid rhinorrhea (n=2). Three of the patients also had a cleft palate. Two patients suffered serious symptoms of fever, tachyarrhythmia, and electrolyte disturbance postoperatively. After a long follow-up period, the symptoms remained stable or improved in all patients postoperatively, but worsened in the patient who did not have a surgical intervention. No mortalities were recorded. This disease may result from pituitary dysplasia, and the symptoms develop as the patients grow. Surgical interventions can be helpful for symptom management, the optimal treatment being a transsphenoidal approach. Those patients with milder symptoms preoperatively have a better prognosis.