Introduction: Encephalocele should be repaired in the first few months of life to prevent neurological deficits and facial disfigurement.Aim of the surgery is water tight dural closure at the level of internal defect,closure of skull defect, reconstruction of external bony deformity. We present our experience on 54 cases of cranial vault encephaloceles managed surgically over a period of only 6 years which is one of the largest series reported from Asia.
Methods: 54 cases of encephalocoeles were studied in our hospital over a 6 year period from 2010 to 2016.CT and MRI brain was performed to delineate the bony defect and associated anomalies. Reconstruction of the bony defect was done using autologous calvarial bone graft,osteopore PCL (polycaprolactone) bone scaffold filler and titanium mesh.
Results: In our study,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 8 and osteopore PCL (polycaprolactone) bone scaffold filler in 4 children. Cranioplasty was not done in remaining 30 children because of the small bone defect.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 last follow-up .
Conclusions: Our study demonstrated that encephaloceles are associated with complex deformities and pose a technical challenge to the neurosurgeon.Repair of dural defect and reconstruction of the skull defect results in good long term outcome.MRI brain and three dimensional CT aids in evaluating the deformity better
Patient Care: Experience demonstrated that a craniofacial centre with interdisciplinary management was necessary to provide proper, early and longitudinal care and to achieve optimum outcomes for the patients with encephaloceles. The collaboration between neurosurgeon and maxillo-facial surgeon and neuro-paediatric anaesthesiologist is fundamental in the management of this anomaly.
Learning Objectives: Multidisciplinary approach is necessary to manage these cases.Surgical correction should be performed as soon as possible to prevent further neurological deficit.