Endoscopic Third Ventriculostomy in Children with Myelomeningocele: A Case Series

Abstract

Hydrocephalus develops in up to 80-90% of children with myelomeningocele (MM) after closure of the defect. Traditionally, ventriculoperitoneal shunts have been used to manage hydrocephalus in these patients. A role for endoscopic third ventriculostomy (ETV) in MM has provoked much debate, principally due to anatomical variants described, which may complicate the procedure. We present 7 cases of children with MM and hydrocephalus undergoing a total of 10 ETV procedures. All patients demonstrated clinical improvement (in acute/subacute cases) or stabilization (in chronic cases). Three patients requiring a second ETV have shown clinical stability and renewed radiological evidence of functioning ventriculostomies in follow-up since reintervention. ETV can be used, albeit cautiously, in selected cases of hydrocephalus associated with MM. However, the frequency with which anatomical variation is encountered and the difficulty of the assessment of success make the procedure more challenging than usual.

Third ventricle floor variations and abnormalities in myelomeningocele associated hydrocephalus: an experience in 455 endoscopic third ventriculostomy procedures

“…Although the incidence of MAH at birth is 15 to 20%, it increases up to 80 to 90% after the closure of the defect (10). However, the exact etiology of MAH still remains unclear.…”

“…Kulkarni et al (6) reported that the incidence of complications such as new neurological deficit or meningitis was less than 2%. Although the recent development of ETV has provided a challenging way of treatment, it still has a very limited role in the first-line treatment of MAH patients due to the immature CSF reabsorption system and anatomical difficulties in these patients; therefore, this method should be suggested only in selected cases (3,10).…”

“…choice for obstructive hydrocephalus in recent years (10). Although, myelomeningocele-associated hydrocephalus (MAH), which can be congenital or acquired after the myelomeningocele closure, has an obstructive basis, the use of ETV for the management of hydrocephalus in these patients is still controversial (9,10,13).…”

“…choice for obstructive hydrocephalus in recent years (10). Although, myelomeningocele-associated hydrocephalus (MAH), which can be congenital or acquired after the myelomeningocele closure, has an obstructive basis, the use of ETV for the management of hydrocephalus in these patients is still controversial (9,10,13). Anatomical variations and abnormalities of the ventricular system and the confusing topography of the floor of the third ventricle are of utmost importance in the ETV procedure, since all these factors play an important role in the limitations and current risks of surgery.…”

Myelomeningocele-associated hydrocephalus: nationwide analysis and systematic review

“…From papers we identified, the reported success rates for ETV as a primary treatment ranged from 29% to 37.5%, whereas success rates for ETV after shunt failure ranged from 50% to 92%. 22,46,50,54 Long-term ETV outcomes after 5 years follow-up demonstrated ETV success rates of 53.3% and 64.3% when used as a first or second treatment modality, respectively. 53 Long-term neurocognitive outcomes between ETV and VPS groups are similar.…”