Disclosures: Sean Lam, DO: No financial relationships or conflicts of interest

Case Diagnosis: A 12 year old female with medullary Ganglioglioma [BRAF v600e mutation].

Case Description: A 12 year old patient presented with insidious onset of intermittent clumsiness, falling, blurry vision, headaches, nausea, and lower limb paresthesias. MRI brain detected a large intraaxial enhancing and infiltrative brainstem mass with involvement of the medulla. The patient underwent craniotomy and partial tumor resection with biopsy revealing ganglioglioma with BRAF v600e mutation. After a course of inpatient rehab post-resection, she made significant functional gains and was discharged home, ambulatory with a walker and able to complete most ADLs (activities of daily living) with none to minimal assistance. However, 1 month following discharge she developed acute onset altered mental status and seizure. Imaging revealed intratumoral hemorrhage, which resulted in new symptoms including: significant postural hypotension, respiratory failure and inability to clear secretions. Her profound orthostatic hypotension limited her ability to participate in therapy sessions. Her orthostasis was remarkable for a 30 mmHg drop in systolic blood pressure from supine to sit and pressures as low as 60/25. She was given an abdominal binder, compression stockings, and salt tablet supplementation with minimal results. Given the efficacy of Midodrine in symptomatic orthostasis in other populations, a trial was initiated.

Setting: Pediatric Inpatient Rehabilitation FacilityAssessment/

Results: The patient was started on Midodrine 5mg twice daily preceding therapy sessions and resulted in resolution of symptoms. She was able to tolerate sitting and standing exercises with minimal symptoms eventually leading to tolerance of twice daily therapies and readmission onto the rehab unit.

Discussion: There is a paucity of literature to direct treatment of orthostatic hypotension in pediatric patients with intramedullary lesions. When first line approaches (such as use of external compression devices) fail, midodrine may help to address functionally inhibiting postural hypotension.

Conclusion: Midodrine may be effective in treating patients with postural hypotension in the setting of intramedullary lesion.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/postural-hypotension-treatment-in-an-adolescent-patient-with-ganglioglioma-braf-v600e-mutation-complicated-by-intratumoral-hemorrhage-a-case-report/