Diastematomyelia (Spinal Cord Splitting)

A rare congenital spinal cord anomaly in which a portion of the spinal cord is longitudinally divided into two halves.

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Congenital Anomalies

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1Overview

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What is this Condition?

Diastematomyelia is a rare congenital spinal cord anomaly in which a portion of the spinal cord is longitudinally divided into two halves. A bone, cartilage, or fibrous tissue septum may exist between the two halves of the spinal cord. It occurs due to an abnormality during early fetal development and is frequently accompanied by vertebral deformities (curvature, fused or hemivertebrae). In many children, the spinal cord is tense and anchored below; therefore, symptoms such as leg weakness, leg length discrepancy, foot deformity, and spinal curvature may develop over time. Skin marks such as hairy patch or birthmark may be seen on the back. Diagnosis is made by MRI; surgery is recommended in symptomatic cases or when a prominent bone septum is present.

Symptoms

Skin marks on the midline of the back such as hairy patch, birthmark, small dimple

Weakness and gait disturbance, usually more pronounced in one leg

Leg length discrepancy or foot deformity (high-arched, inward-turning feet)

Spinal curvature (scoliosis); particularly curvature that begins early and may progress

Urinary and fecal control problems (bedwetting, constipation)

Back or leg pain, decreased sensation in the leg

Diagnosis Methods

In diagnosis, skin marks on the back and neurological symptoms are carefully evaluated. Plain spinal X-rays may show clues such as widened spacing between vertebrae. Definitive diagnosis is made by spinal MRI; this imaging clearly shows the region where the spinal cord is divided, the septum between them, and whether the spinal cord is tense. Computed tomography (CT) can also be used to determine the structure of the bone septum.

Treatment Methods

The goal of treatment is to prevent progressive nerve damage over time. Surgery is recommended in symptomatic children or when there is a hard bone septum between the two halves of the spinal cord. During surgery, this septum is appropriately removed and tension on the spinal cord is relieved (untethering). Associated spinal curvature is separately followed up orthopedically and treated if necessary. Mild asymptomatic cases can be managed with close monitoring; treatment is accompanied by physical therapy and urological follow-up.

Removal of bone/cartilage septum: The hard septum between the two halves of the spinal cord is surgically cleared.

Spinal cord untethering surgery: Structures anchoring the spinal cord below are released to reduce tension.

Monitoring and treatment of spinal curvature (scoliosis): Curvature is managed by the orthopedic team with bracing or, if necessary, surgery.

Monitoring and supportive care: In asymptomatic cases, regular MRI and physical examination; also bladder-bowel monitoring with physical therapy.

Treatment Path

Related Treatments

The procedures and interventions most commonly explored with this condition.

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Craniosynostosis Surgeries

Craniosynostosis Surgeries procedure

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Fronto-Orbital Advancement

Fronto-Orbital Advancement procedure

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Calvarial Reshaping

Calvarial Reshaping procedure

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Oksipitoservikal posterior dekompresyon + duraplasti

Oksipitoservikal posterior dekompresyon + duraplasti procedure

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References

Greenberg MS. Handbook of Neurosurgery. 10th ed. Thieme; 2023.
Cochrane DD. Occult Spinal Dysraphism. In: Principles and Practice of Pediatric Neurosurgery. 3rd ed. New York: Thieme; 2014.
Pang D, et al. Split Cord Malformation. In: Di Rocco C, Pang D, Rutka JT, eds. Textbook of Pediatric Neurosurgery. Springer; 2020.

This content is for informational purposes and based on academic sources; consult your physician for diagnosis and treatment.

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Diastematomyelia (Spinal Cord Splitting)

Congenital Anomalies

1Overview

What is this Condition?

Symptoms

Diagnosis Methods

Treatment Methods

Related Treatments

Craniosynostosis Surgeries

Fronto-Orbital Advancement

Calvarial Reshaping

Oksipitoservikal posterior dekompresyon + duraplasti

References

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