lipomyelomeningocele

Newborn with
a skin covered bump on the back. Sagittal T1 (left), T2 (middle) and T1 with contrast (right) MRI of the lumbar spine show a low-lying conus medullaris at the level of S2, which ends in a lipomatous mass that via a defect in the bone is contiguous with the subcutaneous fat posteriorly.The diagnosis was lipomyelomeningocele.
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Lipomyelomeningocele
with terminal myelocystocele. Lateral view of the chest and abdomen shows a rounded soft tissue mass projecting posterior to the lumbosacral spine.
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Lipomyelomeningocele
with terminal myelocystocele. Notice hydromyelia (yellow arrow), myelocystocele (blue star), lipoma/fat (green star) and thecal sac subarachnoid space (red arrow).
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Lipomyelomeningocele
with terminal myelocystocele. Hydromyelia (yellow arrow) is continuous with the posterior terminal myelocystocele (red arrow).
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Lipomyelomeningocele
with terminal myelocystocele. Notice the dysraphic defect with associated CSF containing lipomatous mass extending dorsally.
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Lipomyelomeningocele
with terminal myelocystocele. Notice the dysraphic defect with associated CSF containing lipomatous mass extending dorsally.
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Lipomyelomeningocele
with terminal myelocystocele. Extension of the neural placode (yellow arrow ) into the CSF-containing mass with broad-based attachment of the neural placode. Nerve roots are visible within the sac.(Red arrows)
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Lipomyelomeningocele
with terminal myelocystocele. The lipomatous component of the mass is predominantly superficial to the thecal sac-distended CSF space, accounting for the dorsal component of the mass.
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Lipomyelomeningoceles are one of the forms of closed spinal dysraphism. They usually present as a subcutaneous fatty mass just above the intergluteal cleft. However, some lipomyelomeningoceles may occur at other locations along the spinal canal.

Clinical presentation

Lipomyelomeningoceles may be clinically detectable as a subcutaneous fatty mass above the intergluteal crease.

Pathology

These lesions are formed mainly due to a defect in primary neurulation in which mesenchymal tissue enters into neural placode and forms lipomatous tissue.

Radiographic features

Plain radiograph

It shows a non-specific splaying / non-fusion of the posterior elements of the spine, which usually cannot be differentiated from the other spinal dysraphisms.

Ultrasound

Ultrasound will show the vertebral arch defector defect associated with an elongated spinal cord with the placode attached to an intrathecal echogenic fatty mass that protrudes in the dorsal subcutaneous tissue (see case 2).

CT
  • spina bifida
  • focal enlargement of the spinal canal at the placode level
  • hypodense fat-attenuated mass in continuity with the subcutaneous fat planes
MRI
  • typically seen as a spinal defect with lipomatous tissue, covered with skin
  • neural placode-lipoma interface lies outside the spinal canal due to enlargement of subarachnoid space
  • a low-lying cord is usually present

Differential diagnosis

  • lipomyelocele: placode-lipoma interface lies within the spinal canal

See also

Siehe auch:
Assoziationen und Differentialdiagnosen zu lipomyelomeningocele:
Myelomeningozele
 
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