Management strategies of cranial encephaloceles: A neurosurgical challenge

Abstract

Background: Encephalocele is defined as herniation of cranial contents beyond the normal confines of the skull through a defect in the calvarium either along the midline or at the base of skull. These anomalies should be repaired in the first few months of life to prevent neurological deficits and facial disfigurement. The aim of the surgery is water tight dural closure at the level of internal defect, closure of skull defect, and reconstruction of external bony deformity. Materials and Methods: Fifty-four cases of encephalocoeles were studied in our hospital over a 6-year period from 2010 to 2016. Computed tomography (CT) and magnetic resonance imaging (MRI) brain were performed to delineate the bony defect and associated anomalies. Reconstruction of the bony defect was done using autologous calvarial bone graft, Osteopore polycaprolactone (PCL) bone scaffold filler and titanium mesh. Results: In our study, 54 patients (34 boys and 20 girls) whose age varied between 2 months and 14 years were evaluated. Frontoethmoidal (44.5%) and occipital encephaloceles (25.9%) were the most frequently seen varieties. Repair of the dural defect either primarily or using pericranium was done in all cases. Closure of the bony defect was done using autologous calvarial bone graft in 12 (22.22%) patients. Titanium mesh was used in eight and Osteopore PCL bone scaffold filler in four children. Cranioplasty was not done in remaining thirty children because of the small bone defect. Overall, 80% had no postoperative problem and were discharged between 7 and 10 days of surgery. Cerebrospinal fluid leak was the most frequent postoperative complication, noted in five patients. Re-exploration with repair was done in one and remaining four were managed conservatively. Overall, cosmetic results were acceptably good, with parents judging the cosmetic outcome as good to excellent in 70%, satisfactory in 18%, and poor in 3% at the last follow-up. Conclusion: Our study demonstrated that encephaloceles are associated with complex deformities and pose a technical challenge to the neurosurgeon. A multidisciplinary approach is necessary to manage these cases. MRI brain and three dimensional CT aids in evaluating the deformity better and surgical correction should be performed as soon as possible to prevent a further neurological deficit. Repair of dural defect and reconstruction of the skull defect results in a good long-term outcome. We present our experience on 54 cases of cranial encephaloceles managed surgically over a period of only 6 years which is one of the largest series reported from Asia.