Split Cord Malformations: Report of Three Unusual Cases

Associated cranial abnormalities with spinal dysraphism are not uncommon. We came across an unusual case of a 1-year-old male child with spinal dysraphism having lumbar meningomyelocele, who also had split cord malformation (hemicord with intervening bony spur) with lipoma of one of the hemicord and filum terminale. The patient also had communicating hydrocephalus without Chiari malformation and also near-total frontal bone agenesis. Single photon emission computed tomography scanning of brain revealed normal perfusion. In the first stage of repair, the patient had postoperative CSF leak for which ventriculo-peritoneal shunt was performed. This constitutes a rare anomaly associated with spinal dysraphism.

Section: Discussionsupporting

confidence: 74%

Frontal Bone Agenesis in a Patient of Spinal Dysraphism

Nayak

Mahapatra²

2006

“…Erşahin et al [7]supported this theory with their study and also emphasized the presence of unusual forms of this malformation [8]. …”

Section: Discussionmentioning

confidence: 71%

Dorsal Bony Septum: A Split Cord Malformation Variant

Akay

İzci

Baysefer³

2002

Split cord malformations (SCMs) are rare spinal anomalies and their classification is still a matter of debate. There is no widespread consensus on the embryological basis of this entity. The unified theory, proposed by Pang et al. [Neurosurgery 1992;31:451–480], was an attempt to explain the embryogenetic mechanism as a basic error occurring around the time when the primitive neuroenteric canal closes. We report two unusual cases of SCMs with a dorsally situated bony spur. We analyzed the radiological, clinical and surgical features of the lesions and were not able to classify these cases according to the unified theory. Further embryological studies should be conducted to elucidate the mechanisms of occurrence of these lesions, and the dorsal bony septum variant should be considered in SCM surgery.

“…Among these, the present case is the only one with a dorsally attached bony spur. Extensive search in the English literature has not revealed a similar case [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19]. Even the recently published series on SCM does not mention such an entity [14].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Dorsally Situated Bony Spur in a Lumbar Split Cord Malformation

Chandra

Kamal

Mahapatra³

1999

A case of lumbar split cord malformation (SCM) with a bony spur situated dorsally is presented. This was associated with a hypertrophied posterior arch. The ventral dura was totally intact, and there was no fibrous septum connecting the bony arch to the dura. To our knowledge, such a case has not been reported earlier. In view of this unique finding, we propose a slight modification in Pang's unified theory of embryogenesis in the development of SCM.