title: Giant encephalocele: a study of 14 patients.
abstract: BACKGROUND
  Giant encephalocele is a rare condition and few published reports are available in the English literature. It is a challenge to neurosurgeons, even today. This series consists of 14 patients with giant encephaloceles treated at our institute.
  Over a period of 8 years, from 2002 to 2009, 110 patients with encephaloceles were managed at our institute. Amongst them, 14 were children with giant encephaloceles. All patients had CT/MRI or both prior to surgery, and all were operated upon. Four patients were neonates, under 1 month of age, and 9/14 patients (64%) were under 3 months. The youngest child was a newborn baby aged 2 days. Except for 1 with an anterior encephalocele, the rest were patients with occipital encephaloceles. A CT scan was performed on 5 and an MRI on 1 patient. Both CT and MRI scans were performed on the other 8 patients. MRI/CT showed hydrocephalus in 10/14 patients. Of these, 7 required ventriculoperitoneal (VP) shunt, and the remaining 3 with mild to moderate hydrocephalus did not. Of the 7 patients who underwent VP shunt, 5 had a shunt during the encephalocele repair and 2 had a postoperative shunt for increasing hydrocephalus.
  
  
  RESULTS
  Other associated anomalies recorded were acquired Chiari malformation in 3 patients, secondary craniostenosis with microcephaly in 5, and syringomyelia in 1 patient. All the patients underwent repair of encephalocele and 4 had suturectomy of coronal suture for the secondary craniostenosis. There were 2 postoperative deaths due to hypothermia. Among the 12 surviving patients, 9 had a good outcome and 3 had poor mental development. The present study shows overall good outcomes in 9/14 (66%) patients.
authors: Department of Neurosurgery, AIIMS, New Delhi, India. akmahapatra_22000@yahoo.com
authors: A K
authors: AK
authors: Mahapatra
mesh_terms: D002675:Child, Preschool; D003398:Craniosynostoses / Q000473:pathology / Q000601:surgery; D004677:Encephalocele / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000473:pathology / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008831:Microcephaly / Q000473:pathology / Q000601:surgery; D019635:Neurosurgical Procedures; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013595:Syringomyelia / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D017287:Ventriculoperitoneal Shunt