A rare case of split pons with double encephalocoele, dermal sinus tract, and lipomeningomyelocele: a case report and review of literature

Abstract

Cases of split pons and double encephalocoeles in combination with other spinal anomalies are very rare, and to the best of author's knowledge, this is the first case report of these multiple anomalies in world literature.

Supra- and infra-torcular double occipital encephalocele

“…Classical theory explains that the neural tube closure starts from the mid-cervical region, and continuously reaches to the rostral and caudal ends in a bidirectional, zipper-like fashion. 5,6,8,10,11 However, this theory is unable to explain the presence of double neural tube defects or defects in the cervical region. 8 The classification of encephaloceles is based on the cause and anatomical location of the skull defect.…”

“…To the best of our knowledge, this is only the fourth case of double occipital encephalocele published in the English literature. 5,7,8 Case report…”

“…The presence of multiple (two or more) neural tube defects along the neural axis is extremely rare and mostly encountered in separate locations. [4][5][6] The current study presents a neonate, who had two encephaloceles that arose from two different bone defects. The superior encephalocele was supra-torcular and the other was infratorcular.…”

Double encephalocele: An unusual presentation

“…However, this theory is unable to explain the presence of double NTDs or defects in the cervical region. [ 3 ]…”

Prenatal Imaging of Occipital Encephaloceles

“…Although postnatal MRI features of meningoencephaloceles have been systematically analyzed [12], prenatal MRI data of these defects are limited to rare case reports [13,14,15,16,17,18,19,20,21,22]. Due to the absence of systematically assessed imaging data, our confidence in predicting the neurological outcome in these cases is generally low.…”