Diastematomyelia in congenital scoliosis: a report of two cases

Gavriliu, Ştefan; Vlad, C; Georgescu, I; Burnei, Gheorghe

doi:10.1007/s00586-014-3218-x

Cited by 6 publications

(4 citation statements)

References 10 publications

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“…Children presenting with vertebral and rib anamolies-syndromic association like JLS should be kept in mind and also should evaluate for diplomyelia, fibrous band, bony septum, syrinx and lesions like dermoid, epidermoid etc [15].…”

Section: Discussionmentioning

confidence: 99%

Epidermoid Causing Split Cord Malformation in a Case of Jarcho Levin Syndrome (JLS)

Sura

2023

J Anaesth Surg Res.

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Jarcho-Levin syndrome (JLS) (costovertebral syndrome) is a rare congenital anomaly with multilevel vertebrocostal deformities. Intraspinal epidermoid cyst is a rare tumor. The incidence in adults is lesser than 1% and in children lesser than 3%. Epidermoid is predominantly seen at the dorsal spinal level. These may be congenital or acquired with known association with spinal dysraphism. We hereby report a rare case of split cord malformation with epidermoid in a 7-year female with JLS. The patient presented with complaints of progressive scoliosis, since3 years of age. On Neurological examination revealed no motor deficit. Patient had a dorsolumbar scoliosis with a hairy patch. X-ray revealed irregular fusion of left sided ribs and scoliosis of dorsolumbar spine with convexity towards left. Magnetic resonance imaging (MRI) and computerized tomography (CT) of the spine were done which revealed bifid spine from D11-L1, diplomyelia with two hemicords in a single dural sac with no membranous or bony septum. Intra operatively incompletely fused spinous process were seen from D11-L1. The patient underwent D10 to L2 laminectomy; there was a fibrous band which was leading into the bifid spine which was followed till the dura after removal of the laminae. After opening the dura this fibrous band was seen extending into the split cord. To our surprise split cord was continuing above the fibrous band following it revealed an epidermoid at the junction of the split cord, which was excised completely and fibrous band also removed. Histopathology of lesion confirmed as epidermoid.

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Section: Discussionmentioning

confidence: 99%

Epidermoid Causing Split Cord Malformation in a Case of Jarcho Levin Syndrome (JLS)

Sura

2023

J Anaesth Surg Res.

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“…In retrospect, the presence of multiple anomalies commonly associated with split‐cord malformations including multilevel lumbar spina bifida occulta, lumbar hypertrichosis, and scoliosis may have warranted additional diagnostic workup for concomitant dysraphism [7]. Indications for surgical treatment with laminectomy and septal resection are defined inconsistently but generally include progressive neurologic decline regardless of malformation type and lower back and/or radicular pain [1,2,8]. Some recommend prophylactic surgery for type I malformations given the high rate of cord tethering and resultant low‐lying conus with cord traction, ischemia, and progressive symptoms including pain, weakness, ataxia, sensory changes and incontinence [1,9].…”

Section: Discussionmentioning

confidence: 99%

Diastematomyelia as an Incidental Finding Lumbar on Magnetic Resonance Imaging

Lersten

Duhon

Laker

2016

PM&R

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“…The literature on concurrent neural axis intervention with definitive fusion is growing and the practice appears to be is safe [26][27][28]. The rates of neurologic injury with posterior spinal instrumented fusion and simultaneous spur resection in type 1 split cord malformation are low [29][30][31][32]. In a mixed group of diastematomyelia patients (20) and those with syrinx with tethered cord (2) there was neurologic injury in 2 patients, both with type 1 split cord malformation, 1 from an intracanal screw in nonsimultaneous surgery and 1 from simultaneous spur excision in a patient with preexisting weakness [33].…”

Section: Discussionmentioning

confidence: 99%