Surgical considerations in Labrune syndrome.

Area of Science:

• Neurology
• Neuroscience
• Medical Genetics

Background:

• Labrune syndrome (LS) is a rare leukoencephalopathy with intracranial calcification and cysts (LCC).
• Intracranial cysts in LS are typically stable but can necessitate intervention due to hemorrhage or mass effect.
• Surgical outcomes for cyst decompression in LS have not been previously reviewed.

Observation:

• A 16-year-old girl with LS presented with worsening right hemiparesis due to a hemorrhagic thalamic cyst.
• The patient underwent successful frameless stereotactic cyst aspiration and Ommaya reservoir placement.
• Post-operative monitoring confirmed stable cyst decompression and symptom improvement.

Findings:

• The pathophysiology of LS involves diffuse cerebral microangiopathy, potentially leading to cyst formation and calcifications.
• Indications for surgical intervention in LS are not well-defined, requiring individualized management.
• Literature review suggests stereotactic approaches with potential reservoir placement are favorable for progressive LS lesions.

Implications:

• This case highlights the efficacy of stereotactic cyst aspiration in managing symptomatic LS intracranial cysts.
• Less-invasive surgical techniques may be the preferred treatment for progressive lesions in Labrune syndrome.
• Further research is needed to establish clear surgical guidelines for LS management.