Myelocystocele

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Status

Done

Parent item

Spinal anomalies

Definition

• Marked dilatation of the central canal of the spinal cord, herniating posteriorly through a dorsal spinal defect.

Numbers

• More common in females

• Rare
• 1/400000 births

• No genetic component

• Risk increased with siblings having it
• No nutritional risk factors

Pathology

• Skin-covered mass in the lower lumbar region

• Sac lined by ependyma

 

Pathophysiology

• A combination of 2 theories
• Cloaca exstrophy → Herniation of the lower part of the abdomen and pelvis → disruption in the secretion by the notochord of inductive factors (normally function to regulate development of the neural tube and regression) → Without the secretion of these factors, the process of secondary neurulation fails to occur.
• Myelocystocele is associated with cloaca exstrophy
• CSF is unable to exit from the early neural tube as it should → mechanical distention of the distal terminal ventricle after canalization → The terminal ventricle then distends the surrounding dorsal membrane and arachnoid, creating the surrounding meningocele.

Clinical presentation

• Skin covered lower lumbar mass

• with or without neurological deficits

Associated abnormalities

• OEIS complex (omphalocele, bladder exstrophy, imperforate anus, and sacral agenesis)

Treatment

• Surgery
• Goal:
• To prevent tethering
• Timing
• Cyst is intact so no rush for surgery
• But should be done within 6 months of age
• Prevent neurological loss from spinal tethering
• Images