Table 6.

Treatment of Manifestations in Individuals with NOTCH3-Related Lateral Meningocele Syndrome (LMS)

Manifestation/
Concern		TreatmentConsiderations/Other
Lateral spinal
meningoceles
  • There is no standard operative mgmt of lateral spinal meningoceles [Cuoco et al 2020].
  • Symptomatic treatment of neurologic sequelæ (e.g., neurogenic bladder, paresthesias, back pain, &/or paraparesis) as needed
  • Surgical intervention is generally avoided but may be necessary due to neurologic manifestations secondary to meningocele size & location [Cuoco et al 2020].
  • 1 person benefited from CSF diversion w/a VP shunt for symptomatic relief of thoracolumbar meningocele [Brown et al 2017].
  • When required, surgical approach is individualized & can incl laminectomy for smaller meningoceles, costotransversectomy for larger meningoceles.
Developmental
delay 		Timely supportive interventions as needed to optimize development through OT & education resources
Musculoskeletal
issues
  • PT to ↓ risk for joint subluxation & dislocation
  • Mgmt by specialists in chronic pain mgmt or rehab medicine as needed

OT = occupational therapy; PT = physical therapy

From: NOTCH3-Related Lateral Meningocele Syndrome

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