Cephaloceles are rare neural tube defects. In this study, we retrospectively reviewed the charts of all patients with cephaloceles who underwent surgical treatment in our institute for a 14-year period, between January 1995 and January 2009. There were 27 children (11 boys and 16 girls; mean age, 7.5 mo; range, 1 d to 7 y). Seventeen encephaloceles were occipital; 5, parietal; 2, ethmoidal; 1, frontoethmoidal; 1, nasoethmoidal; and 1, sphenoethmoidal. The mean size of sac was 3 cm, although 2 cases of giant occipital encephaloceles were observed. In 19 cases (70%), the sac contained gliotic brain (encephaloceles) that was excised. All patients were operated on in 1 surgical procedure. Hydrocephalus was found in 16 patients and treated with a ventriculoperitoneal shunt insertion. Postoperatively, there was no neurologic deficit or death. After a mean follow-up period of 7.2 years (range, 6 mo to 11.5 y), all patients were in good condition. In conclusion, occipital cephaloceles are more frequently encountered and are usually associated with hydrocephalus. Surgery should be performed as early as possible and only after careful preoperative planning especially for the anterior cephaloceles.
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