Split notochord syndrome

Case contributed by Dr Sebsbie Admas

Presentation

Lower back swelling since birth

Patient Data

Age: 10 days
Gender: Female
MRI

There is posterior midline lumbosacral vertebral diastasis through which intestinal loops are exteriorized, and myelomeningocele is present. There is also splitting of the spinal cord in this region.

Case Discussion

Split notochord syndrome is an extremely rare complex form of spinal dysraphism characterized by a persistent communication between the endoderm and the ectoderm. And usually, it is associated with other congenital anomalies like meningomyelocele or myelocele and genitourinary anomalies.

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