Fourth Ventricular Outflow Obstruction in an Infant with Ileal Atresia and Laryngomalacia: Endoscopic Management

Abstract

Fourth ventricle outflow obstruction (FVOO) is a rare cause of obstructive hydrocephalus. In this study, we described a case of idiopathic FVOO with ileal atresia and laryngomalacia which was managed with endoscopic third ventriculostomy (ETV) and re-endoscopy. We also described the techniques of fenestration of Liliequist membrane and partial removal of arachnoid membrane over dorsum sella (DS) to prevent closure of fenestration and recurrence of hydrocephalus. The patient was a 4-month-old infant presented with progressively increasing head size, feeding difficulty, respiratory distress, and tense fontanel. The infant had a history of laparotomy for ileal atresia. CT scan showed panventriculomegaly due to FVOO. ETV with fenestration of Liliequist membrane was done on emergency basis. After operation, the patient improved clinically and radiologically. Four weeks later, the patient returned with recurrent hydrocephalus. Endoscopic reoperation showed closure of fenestration in arachnoid membrane (Lilieqiest membrane). Endoscopic refenestration with partial excision of arachnoid on DS was done. The patient again recovered radiologically and clinically till last follow-up. In idiopathic FVOO, ETV with wide fenestration of Liliequist membrane, preferably with partial removal of arachnoid on DS, may be very useful in treating hydrocephalus (HCP) and preventing recurrent HCP even in infants.

Keywords

• hydrocephalus
• fourth ventricle outflow obstruction
• endoscopic third ventriculostomy
• re-endoscopic procedure