Sincipital encephalocele: A case report

Abstract

Encephalocele is a congenital anomaly characterized by herniation of brain and meninges through a defect along the mid line of the cranial vault or at the base of skull. It occurs in 14 cases per 10,000 live births.Commonest site is occipital (75%) whereas frontonasal is rare site. We report a case of four months old male baby who presented to us with gradually progressive swelling at the root of nose since birth. Initially it used to increase in size on crying but since 1 month there was no change in size noticed. On examination a midline solitary swelling of 80cms in the frontonasal region was noted. It was non tender, cystic, non-pulsatile, non-transilluminant, without cough impulse, non- reducible with streched hyperpigmented overlying skin and normal surrounding skin. Underlying bone defect could not be made out. Intraoperatively, there was 5 cms bony defect (in anterior half of cribriform plate up to frontonasal junction) through which herniation of the frontal lobe covered by thinned out duramater was noted along with CSF. Gliotic changes were seen at the tip of herniated brain matter. Bifrontal craniotomy followed by circumferential dissection and repositioning of herniated frontal lobe was done. Primary dural closure with excision of redundant skin sac was done. Postoperative period was uneventfull.