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Central Hypoventilation Syndrome (CHS) or Ondine’s curse is a rare disorder that results from the impairment of autonomic control of breathing during sleep with preserved voluntary control. 

Its occurrence post-excision of hemangioblastoma has been well documented. However, its association with Von Hipppel Lindau syndrome (VHL), a well known cause of hemangioblastoma, has never been described.

The clinical details, laboratory reports and imaging findings were reviewed.

A 19-year-old male patient with VHL, underwent removal of a medullary hemangioblastoma. Post-operatively he experienced difficulty in weaning off from the ventilator and required tracheostomy. After weaning he was found to have recurrent episodes of desaturation, predominantly during sleep. On evaluation, the polysomnography(PSG), showed evidence of central sleep apnoea during sleep and MRI brain showed post-operative changes with a well-defined cystic lesion at cervicomedullary junction compressing the medulla. Therapeutic CT-guided aspiration of the cyst was performed resulting in significant clinical improvement. The PSG showed a betterment of the respiratory disturbance index and desaturation index.

This case is a unique scenario of a patient with VHL presenting with symptoms of CHS, due to brainstem disruption caused by post-operative CSF cyst formation following surgical removal of a cervico-medullary hemangioblastoma. Direct pressure effects from this cyst likely caused blunted response to chemosensitivity, resulting in loss of involuntary respiratory drive during sleep.

Cases of CHS following posterior cranial fossa hemangioblastoma resection are known, however, they were noticed immediately after the surgery.

Our case highlights the importance of long term follow up of patients undergoing posterior cranial fossa surgery, for surveillance of potential late post-operative complications.