Lessons Learned in Jugular Paraganglioma Surgery

Objective: The anatomy of the jugular foramen (JF) is complex. It contains the lower cranial nerves and major vascular structures. Jugular paragangliomas (JPs) are the most common primary neoplasms of the JF, arising from the paraganglion cells within the adventitia of the jugular bulb. They are slow-growing, highly vascularized tumors. Although considered histologically benign, the management of jugular paragangliomas is challenging because of their infiltrative nature and proximity to the facial and lower cranial nerves (CN), carotid canal, posterior fossa meninges, and otic capsule. Although radiosurgery has shown an important role in the treatment of JPs, surgery is the main treatment strategy in a selected group of young and symptomatic patients. In this lecture, we present the experiences learned in a single surgeon series, which have led to the formation of an institutional protocol.

Methods: The authors retrospectively reviewed the operative notes of a consecutive database of procedures in which a surgical treatment of JPs was performed. The preoperative MRI, angio CT, angiogram as well as the surgical notes and videos were reviewed and analyzed. Special consideration was done in the postoperative complications to establish an institutional protocol for the management of JPs.

Results: Between January 2014 and August 2018, twelve patients (3 males, 9 females) aged 19 to 62 years underwent a total of 12 surgeries for JPs by the primary author (DMR). All cases were staged, where the first surgery included a cervical vascular control and a posterior petrosal approach tailored for each case. All the tumors were preoperatively embolized prior to surgery (24–96 hours). CSF leak presented in 25% of the cases requiring a lumbar drainage. Multiple full HD videos are collected to analyze the surgical technique.

Conclusion: Surgical management of JPs is challenging and requires a thorough knowledge of the surgical anatomy of the JF and craniocervical areas. A selected group of patients require surgery due to progressive neurological deficit. Preoperative embolization and retrofacial infralabyrinthine approach provide a wide exposure for the removal of JPs. An adequate selection of the patients is crucial to avoid complications.