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Published on: November 11, 2021
Diffuse midline glioma presenting with central sleep apnea and pulmonary hypertension in a 4-year-old patient: a case
Szu-Wei Huang1,2, Pei-Lin Lee3, Pi-Chuan Fan2
1Department of Emergency Medicine, Taipei Municipal Wan Fang Hospital, Taipei, Taiwan.
Insights
Central sleep apnea, a rare pediatric disorder, was diagnosed in a 4-year-old boy presenting with syncope and pulmonary hypertension. This case links these symptoms to a brainstem glioma, highlighting a novel diagnostic pathway.
Area of Science:
- Pediatric Neurology
- Sleep Medicine
- Neuro-oncology
Background:
- Central sleep apnea (CSA) is uncommon in children and often secondary to other conditions.
- Pediatric CSA can present with diverse symptoms, complicating diagnosis.
- Underlying etiologies for pediatric CSA require thorough investigation.
Observation:
- A 4-year-old boy presented with recurrent syncope, pulmonary hypertension, and cardiomegaly.
- Polysomnography revealed severe central sleep apnea with a high apnea-hypopnea index.
- Brain imaging identified an ill-defined lesion in the medulla oblongata and lower pons.
Findings:
- Brainstem biopsy confirmed a low-grade glioma as the cause of the neurological findings.
- The central sleep apnea, syncope, and pulmonary hypertension were attributed to the brainstem glioma.
- This case represents the first report linking syncope and pulmonary hypertension workup to CSA and a brain neoplasm.
Implications:
- This case expands the known clinical spectrum of pediatric low-grade gliomas.
- It underscores the importance of considering central nervous system pathology in pediatric syncope and pulmonary hypertension.
- Highlights a novel diagnostic pathway for central sleep apnea in children, originating from investigations into syncope and pulmonary hypertension.
Abstract:
Central sleep apnea is a rare disorder in the pediatric population with various initial presentations and is secondary to many underlying diseases. We report on a 4-year-old boy with episodes of syncope. He also had pulmonary hypertension and cardiomegaly. Polysomnography showed the finding for central sleep apnea with a high apnea-hypopnea index (up to 138.1 events/h). Brain magnetic resonance imaging showed an ill-defined area near the medulla oblongata and lower pons. The lesion from a brainstem biopsy confirmed the diagnosis of low-grade glioma. Conservative medical follow-up was suggested, and brain magnetic resonance imaging 6 months later showed no obvious tumor progression. To our best knowledge, this is the first case report that workup on the cause of syncope and pulmonary hypertension led to the final diagnosis of central sleep apnea and a brain neoplasm.
