Presentation Description
Institution: Children's Hospital Westmead - NSW, Australia
Case Description: A 10-year-old male presented with a one-year history of headaches with acute worsening of pain with vomiting and left middle ear effusion. MRI revealed a 3 cm lesion in the left infratemporal fossa, abutting the middle cranial fossa, petrous internal carotid artery, and displacing pterygoid muscles. Further investigation confirmed elevated serum catecholamines, with uptake on DOTA-TATE PET scan, which indicated no additional lesions. The patient was admitted for blood pressure stabilisation pre-operatively controlled with five antihypertensive agents, coordinated by the nephrology team. The patient underwent interventional radiology guided preoperative embolisation on the day of surgery, followed by an endoscopic trans-nasal resection as well as left-sided grommet placement. Postoperatively, the patient’s blood pressure stabilised and was weaned completely off all blood pressure modifying agents within 24 hours of surgery. He experienced left facial pain, paralysis of muscles of mastication, V2/V3 numbness, and transient allodynia of the limbs, likely due to catecholamine withdrawal. These symptoms resolved within 10 days. Pathology confirmed paraganglioma and genetics consultation was sought.
Discussion: Paragangliomas of the infratemporal fossa are exceptionally rare with only a handful of cases being reported in the literature. They are tumours originating from neural crest cells which may be secretory or non-secretory. This case underscores the complexity of managing catecholamine-secreting skull base tumours in paediatric patients, from preoperative stabilisation and management of hypertension to intraoperative decision-making and postoperative recovery. Successful resection of these tumours requires multidisciplinary coordination, meticulous preoperative planning, and adaptability in surgical approach. This case illustrates potential refinements in endoscopic technique and intraoperative strategy for future cases involving similar lesions in complex anatomical regions.
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Authors
Authors
Dr Aki Flame - , Dr Michael Chin -