Management Strategies of Cranial Encephaloceles: A Neurosurgical Challenge.

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.

Tectal Tuberculoma: An Unusual Cause of Parinaud's Syndrome.

INTRODUCTION: Parinaud's Syndrome is an inability to move the eyes upwards which is caused by damage to the tectal plate of midbrain. Commonest causes implicated are the Pineal tumors in children, Multiple Sclerosis in adults and stroke in elderly patients. We present a rare case of Tectal plate tuberculoma leading to Parinaud's Syndrome. DISCUSSION: Parinaud's syndrome is caused by damage to the tectal plate or posterior commissure of midbrain. Our patient presented with upward gaze paresis and was diagnosed to have tuberculoma involving the midbrain region. He was managed conservatively and responded to the treatment. CONCLUSION: Tectal plate tuberculoma though a rare possibility, warrants proper diagnostic workup in order to prevent unnecessary brainstem surgery.

Silent Migration of Ventriculoperitoneal Shunt per Anus in a Child: Management and Review of Literature.

The aim of this paper is to report a case of ventriculoperitoneal (VP) shunt tube coming out through the anus in a 6-year-old boy, who had undergone shunt revision surgery for the malfunctioning of the peritoneal end 6 months back. Among the complications of VP shunt surgery, such unusual migration of peritoneal end of the VP shunt is very rare. The possible factors responsible for this complication, in our case, were abdominal adhesions and thin bowel wall in the children. Although this complication has been previously reported, it remains an exceedingly rare case. Risk factors and possible mechanisms of migration are discussed.

Reduction techniques in the management of atlantoaxial subluxation.

BACKGROUND: The traditional approach to atlantoaxial subluxation which is irreducible after traction is transoral decompression and reduction or odontoid excision and posterior fixation. Transoral approach is associated with comorbidities. However using a posterior approach a combination of atlantoaxial joint space release and a variety of manipulation procedures, optimal or near optimal reduction can be achieved. We analysed our results in this study based on above procedure. MATERIALS AND METHODS: 66 cases treated over a 5 year period were evaluated retrospectively. Three cases treated by occipito cervical fusion were not included in the study. The remaining 63 cases were classified into three types. All except two cases were subjected to primary posterior C1-C2 joint space dissection and release followed by on table manipulation which was tailored to treat the type of atlantoaxial subluxation. Optimal or near optimal reduction was possible in all cases. An anterior transoral decompression was needed only in two cases where a bony growth (callus) between the C1 anterior arch and the odontoid precluded reduction by posterior manipulation. All cases then underwent posterior fusion and fixation procedures. Patients were neurologically and radiologically evaluated at regular followups to assess fusion and stability for a minimum period of 6 months. RESULTS: Of the 63 cases who underwent posterior manipulation, 49 cases achieved optimum reduction and the remaining 14 cases showed near optimal reduction. Two cases expired in the postoperative period. None of the remaining cases showed neurological worsening after the procedure. Evaluation at 6 months after surgery revealed good stability and fusion in all except three cases. CONCLUSION: Atlantoaxial joint release and manipulation can be used to achieve reduction in most cases of atlantoaxial subluxation, obivating the need of transoral odontoid excision.