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Published on: May 23, 2025
Diagnosis and management of cephaloceles
George A Alexiou1, George Sfakianos, Neofytos Prodromou
1Department of Neurosurgery, Agia Sofia Children's Hospital, Athens, Greece. alexiougrg@yahoo.gr
Insights
Cephaloceles, rare neural tube defects, are often occipital and associated with hydrocephalus. Early surgical intervention and careful planning improve outcomes for affected children.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neurology
- Congenital Malformations
Background:
- Cephaloceles are uncommon neural tube defects.
- Surgical management is crucial for improving patient outcomes.
Purpose of the Study:
- To review surgical outcomes for patients with cephaloceles.
- To identify common types and associated conditions.
Main Methods:
- Retrospective chart review of 27 pediatric patients.
- Analysis of surgical procedures and follow-up data over 14 years.
Main Results:
- Occipital encephaloceles were most common (17/27).
- Hydrocephalus was present in 16 patients.
- No postoperative neurologic deficits or deaths occurred.
Conclusions:
- Occipital cephaloceles are frequent and often linked to hydrocephalus.
- Early surgical intervention is recommended, particularly for anterior cephaloceles requiring meticulous preoperative planning.
Abstract:
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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