Chiari type III malformation

Abstract

In our case, we introduced a technical variant consisting in primary external drainage from the malformative sac, followed by closure of the encephalocele and, eventually, by CSF shunting.

Chiari III Malformation

“…Chiari type III is the rarest of the Chiari malformations, and its definition has been expanded to include caudal medullary displacement and hindbrain herniation into encephaloceles in lower occipital and high cervical regions [1,2,3,4,5,6,7,8]. This extremely rare congenital anomaly is usually associated with a dismal prognosis in terms of early death or severe disability in long-term survivors [2,8,9].…”

“…This extremely rare congenital anomaly is usually associated with a dismal prognosis in terms of early death or severe disability in long-term survivors [2,8,9]. A 6-month-old female child, born by normal spontaneous vaginal delivery, had presented with a swelling over the nape of the neck since birth.…”

“…The early operative closure of the defect is recommended to remove the encephalocele along with neural tissue and to repair the dura and the skin. It is less important to identify the other contents of the encephalocele as the brain tissue in the herniated sac is believed to be nonviable [2,3,4,5,6]. However, when there is significant hydrocephalus, a CSF diversion including a shunt is necessary [2,4,8].…”

“…It is less important to identify the other contents of the encephalocele as the brain tissue in the herniated sac is believed to be nonviable [2,3,4,5,6]. However, when there is significant hydrocephalus, a CSF diversion including a shunt is necessary [2,4,8]. Chiari type III malformation is very rare and is associated with a high early mortality rate or severe neurological deficits in patients that survive.…”

“…Chiari type III malformation is very rare and is associated with a high early mortality rate or severe neurological deficits in patients that survive. However, if the appropriate surgical procedure is performed at the right time, the outcome for these patients can be satisfactory with a low mortality rate [2,3,4,5]. …”