Chiari III Malformation with a Giant Encephalocele Sac: Case Report and a Review of the Literature

A Brief Review of Giant Occipital Encephalocele

“…[ 2 3 ] Giant occipital encephalocele is a rare entity, and mostly reported as case reports. [ 4 5 ] The internet search revealed two series published on giant occipital encephaloceles. [ 3 6 ] Mahapatra published a series of 14 cases of giant encephaloceles, which included thirteen cases of giant occipital encephaloceles, and one case of anterior encephalocele.…”

Giant Occipital Encephalocele with Chiari Malformation Type 3

“…Nearly 15%–20% of children have additional congenital anomalies, including neural tube defects, microcephaly, CM Type 2 or 3, craniosynostosis, and syringomyelia. [ 2 3 5 6 ] For this reason, MRI coupled with MRA and MRV is the optimal investigation to visualize the contents of the sac and its relationship to venous sinuses during the surgical planning. [ 7 ]…”

“…Even today, it is a formidable challenge to neurosurgeons. [ 1 2 ] This is due to the amount of brain tissue, which forms the content of the encephalocele, intraoperative blood loss, and intra- and postoperative hypothermia. CM Type 3 with a giant occipital encephalocele (GOE) is rare, and to the author's knowledge, only a handful of published reports are available till date, mostly reporting a poor outcome which was contradictory in our case.…”

“…CM Type 3 with a giant occipital encephalocele (GOE) is rare, and to the author's knowledge, only a handful of published reports are available till date, mostly reporting a poor outcome which was contradictory in our case. [ 2 3 4 5 ]…”

Chiari Malformations and Syringohydromyelia in Children

“…76 The severity of symptoms is correlated with the amount of brain structures within the encephalocele. 77 In an increasing number of patients, the diagnosis of C3M is made prenatally by fetal MRI.…”