A Case of Petrous Apex Cephaloceles with Intracranial Hypertension presenting as Trigeminal Autonomic Cephalalgia-like Attacks responding to Transverse Sinus Stenting
Jane Lee1, Risako Shirane2
1Department of Neurology, Columbia University Irving Medical Center, 2Department of Neurology, Yale School of Medicine
Objective:
To report a case of symptomatic petrous apex cephaloceles successfully treated with transverse sinus venous stenting
Background:
Petrous apex cephalocele (PAC) is a rare structural anomaly characterized by cystic herniation of the arachnoid membrane from the posterolateral aspect of Meckel’s cave into the superomedial portion of the petrous apex. These are typically found incidentally on imaging; however, a link between PAC and idiopathic intracranial hypertension has been suggested. Reports of symptomatic cases describe trigeminal neuropathy, cerebrospinal fluid otorrhea, and meningitis. We present a case of bilateral PACs associated with intracranial hypertension presenting with recurrent trigeminal autonomic cephalalgia (TAC)-like attacks.
Results:
A 55-year-old woman with history of adrenal insufficiency on chronic prednisone, hypertension, Hashimoto’s disease, migraine, obesity status post gastric bypass surgery presented in October 2024 with a year-long history of episodic, severe right-sided retro-orbital throbbing pain accompanied by ipsilateral ptosis, mydriasis, photophobia, facial paresthesia (V2), bilateral pulsatile tinnitus, nausea, and restlessness. Each attack lasted four to twenty-four hours, occurring three times per week.
Both neuro-ophthalmological and neurological examinations were normal. CTA head/neck, and MRI brain/orbit revealed bilateral PACs, more prominent on the right. Lumbar puncture revealed an opening pressure of 38cm H2O, with a normal CSF analysis. MR venogram revealed hypoplastic left transverse and sigmoid sinuses. Acetazolamide was poorly tolerated, and topiramate did not improve her symptoms. The patient was evaluated by neurosurgery and successfully underwent right transverse sinus stenting following venous pressure manometry. Post-procedure, her right-sided facial symptoms resolved.
Conclusions:
This case demonstrates that PACs may present with episodic TAC-like attacks. Additionally, resolution of symptoms following an intracranial pressure-lowering treatment suggests a pathophysiologic link between chronic intracranial hypertension and PACs.
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