Spinal Dysraphism (Occult Spinal Closure Defects)

A general term for congenital spinal anomalies that result from incomplete fusion of the spinal cord and surrounding structures in utero and mostly remain hidden beneath the skin.

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Congenital Anomalies

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1Overview

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What is this Condition?

Spinal dysraphism is a general term for congenital anomalies that occur when the spinal cord and surrounding structures fail to completely fuse during fetal development. While some appear as an open sac on the outside, the skin usually remains intact and the problem remains hidden beneath; therefore, it is also known as occult spinal closure defects. The most common forms include situations where the spinal cord is anchored and tense below (tethered spinal cord), forms where fatty tissue adheres to the spinal cord, a thin channel extending from the skin to the spinal canal, and situations where the spinal cord is divided in two. Symptoms may appear or worsen as the child grows. Diagnosis is made by MRI, and in suitable cases, surgery relieves tension on the nerves.

Symptoms

Skin marks on the lower-mid back such as hairy patch, birthmark (hemangioma), small dimple, or soft mass under the skin

Weakness in the legs, usually one-sided, gait disturbance, foot dragging, or inability to lift the foot

Decreased sensation or numbness in the leg or hip-buttock area

Urinary and fecal control problems: bedwetting (over age 5), urinary incontinence, constipation, recurrent urinary tract infections

Foot deformities (high-arched or inward-turning feet), length difference between legs, spinal curvature (scoliosis)

Back pain or pain radiating to the leg that increases with movement

Diagnosis Methods

The first step in diagnosis is careful physical examination evaluating skin marks on the back and neurological symptoms. Spinal MRI is used for definitive diagnosis; this imaging reveals whether the spinal cord is tense and anchored below, and identifies findings such as thick ligament, fatty tissue, skin canal, or spinal cord splitting. Bladder function and kidneys can also be evaluated with urodynamic testing and ultrasound.

Treatment Methods

The primary goal of treatment is to prevent or stop progressive nerve damage. In symptomatic cases or those at risk of progression, surgery to relieve tension on the spinal cord (untethering) is recommended; during this procedure, fatty tissue, thick ligament, or bone septum causing adhesion can be corrected as needed. Mild asymptomatic cases can be monitored with close follow-up. When properly timed, surgery can help preserve existing functions; treatment is accompanied by urological and orthopedic follow-up and physical therapy.

Spinal cord untethering surgery: The thick ligament, fatty tissue, or adhesions anchoring the spinal cord below are released to reduce tension.

Correction of associated structures: Skin-to-spinal canal tract (dermal sinus), fatty mass (lipoma), or bone septum can be appropriately removed during the same procedure.

Monitoring and follow-up: In mild asymptomatic cases, the child's neurological status is closely monitored with regular physical examination and MRI.

Supportive treatments: The child's development is supported through bladder-bowel management, orthopedic treatment for foot and spinal problems, and physical therapy.

Treatment Path

Related Treatments

The procedures and interventions most commonly explored with this condition.

4 links

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

Open treatment detail

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. Tethered Cord ve Filum/Sekonder Nörülasyon Defektleri. 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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Spinal Dysraphism (Occult Spinal Closure Defects)

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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