Lipomyelocele

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Lipomyelocele, also known as lipomyeloschisis, is one of the most common closed spinal dysraphism. It is seen in the thoracolumbar region and usually presents as a fatty subcutaneous mass.

It is twice as common as lipomyelomeningocele.

Clinical presentation

Affected individuals are usually asymptomatic at birth, but many (~ 50%) show the presence of high-risk cutaneous markers as listed below:

  • hypertrichosis
  • atypical dimples
  • acrochordons (pseudo-tails)
  • lipomas
  • hemangiomas
  • aplasia cutis
  • dermoid cyst or sinus

As the spinal canal grows, there is a distortion of nerve roots with growth thereby leading to neurological deficits, highlighting the importance of early diagnosis.

Pathology

There is premature separation of surface ectoderm before the formation of proper neural tube with the ingress of mesoderm (which forms fatty elements). The mesoderm prevents proper neurulation.

Radiographic features

MRI

Posterior spinal defect noted which is covered with skin and shows interspersed lipomatous tissue. The neural placode-lipoma interface lies within the spinal canal or at its edge with normal anterior subarachnoid space.

There is usually associated tethered low-lying cord or syrinx of the terminal spinal cord.

Differential diagnosis

Clinical differential diagnosis

Closed spinal dysraphism of thoracolumbar region presenting as subcutaneous mass may be due to:

Imaging differential diagnosis