The Multidisciplinary Management of Paragangliomas of the Head and Neck, Part 1
By Kenneth Hu, MD1, Mark S. Persky, MD2 |
July 1, 2003
1Assistant Professor of Radiation Oncology, Albert Einstein College of Medicine, Beth Israel Medical Center
2Vice Chairman, Department of Otolaryngology-Head and Neck Surgery; Associate Director, Continuum Cancer Center, Codirector, Institute for Head and Neck Cancer, Beth Israel Medical Center, New York, New York; Professor of Clinical Otolaryngology–Head and Neck Surgery, Albert Einstein College of Medicine, Bronx, New York
Traditionally, surgical resection has been the mainstay of treatment for these tumors, but the outcome is dependent on many factors that may influence the ideal result of total tumor removal and minimal postoperative complications. Relative contraindications to surgery include extensive skull-base or intracranial involvement, advanced age of the patient, medical comorbidities, and bilateral or multiple paragangliomas, which may result in unacceptable postoperative morbidity and bilateral lower cranial nerve palsies.
If surgery is the chosen course of treatment, preoperative embolization is performed. Embolization of paragangliomas has been an extremely useful adjunct to our treatment protocol. Although not uniformly accepted,[ 52-56] there are major advantages to performing endovascular embolization prior to subsequent surgery, assuming that certain criteria have been met before the embolization is performed.[41,57-61] An experienced vascular radiology team must be thoroughly familiar with the complexities and possible variations in the vascular anatomy of the head and neck. Many anastomoses exist between the external and internal carotid system, and without this knowledge, disastrous neurologic consequences may result. The safe performance of this procedure by interventional vascular radiology must be established and documented with an acceptable rate of morbidity and mortality.[60,63]
Surgery is performed within 2 days of angiography and embolization to avoid recruitment of collateral tumor blood supply and prior to the onset of a significant postinflammatory effect.[ 28] If there is concern about tumor edema, which may compromise the dissection, short-term steroids are administered. The anesthesiologist must be prepared to counteract the alpha- and beta-adrenergic catecholamine- induced cardiovascular effect when dealing with "secreting" tumors.
Adequate removal of the tumor requires a subadventitial dissection of the carotid artery. Nerves adherent to but not infiltrated by these tumors can usually be mobilized; these include the vagus, hypoglossal, and, occasionally, glossopharyngeal nerves. The sympathetic chain and the superior laryngeal nerve are often adherent to tumor, especially in cases with medial extension into the parapharyngeal space.
Tympanic paragangliomas confined to the middle ear (Glasscock- Jackson type I, Fisch class A) can be accessed via a transcanal approach.[ 64] These small tumors do not require preoperative embolization.
Involvement of the jugular bulb requires a combined transmastoid and transcervical approach. Tumor dissection is meticulously performed off the internal carotid artery. The exiting cranial nerves are preserved if they can be adequately separated from the tumor. Additional exposure into the infratemporal fossa can be accomplished with resection of the mandibular condyle and zygomatic arch. Such an approach allows access to the posterior and middle cranial fossa, and, if necessary, further tumor resection can be accomplished through a neurosurgical, intracranial approach. A pedicled temporalis, temporal- parietal, or sternomastoid flap is used to reconstruct and obliterate the defect. The external auditory canal is closed at the meatus. Spinal drainage catheters are used postoperatively for significant cerebrospinal fluid leaks.
Vagal paragangliomas may vary in the extent of skull-base or intracranial involvement. Most vagal paragangliomas originate in the nodose (inferior) ganglion, approximately 2 cm below the jugular foramen. When these tumors grow, they will extend to the skull base and involve the post styloid parapharyngeal space as well. Vagal paragangliomas arising in the middle or superior vagal ganglia are associated with early skull-base involvement with intracranial extension.
As with all paragangliomas, a complete radiographic evaluation with contrast-enhanced MRI and CT stud- ies will define the extent of involvement. A combined cervical-mastoid approach to the skull base is best for achieving safe and wide exposure. The tumor almost always involves sacrifice of the vagus nerve and additional cranial nerves according to its size and local involvement.
The complications associated with head and paraganglioma surgery can be grouped into three main categories: cranial nerve injury, vascular injury, and injury to the carotid body/ carotid sinus complex.
Lower cranial nerve dysfunction is a common complication of surgical resection of head and neck paragangliomas and often requires postoperative rehabilitation. Indeed, larger tumors that involve the temporal bone can infiltrate between the fascicles of the cranial nerves, even with normal nerve function. These involved nerves should be sacrificed to effect a total tumor resection. Our experience with postoperative cranial nerve dysfunction reflects a combination of total paralysis, due to nerve sacrifice and paresis, with ultimate recovery of function due to nerve traction.
Postoperative Cranial Nerve Deficits in Paraganglioma Patients
The frequency of affected nerves is related to tumor type and size and the surgical approach used (Table 5). Although isolated injury to one of the lower cranial nerves (IX-XII) sometimes causes only temporary minor difficulty in swallowing, aspiration, phonation, shoulder mobility, or tongue motion, vagus nerve and multiple cranial nerve injury may result in significant morbidity.[ 67] Multiple cranial nerve deficits are particularly poorly tolerated by elderly patients. Bilateral lower cranial nerve palsies most often represent a severe, potentially life-threatening situation.
Paraganglioma surgery generally produces postoperative nerve dysfunction, and familiarity with rehabilita tion techniques is necessary for proper patient care. If tumor resection results in nerve transection, then primary anastomosis, if possible, results in acceptable function. This is especially true of the facial, accessory, and hypoglossal nerves. Cable grafts using the greater auricular or sural nerve are also an option, especially in the case of facial and accessory nerve dysfunction.
Rehabilitation for patients with postoperative nerve dysfunction depends on the functional deficit and may require additional surgical intervention, including upper-lid gold-weight implants for corneal protection, transoral hemipalatal adhesion for palatal insufficiency, temporalis and masseter muscle/ fascial sling or adynamic fascial or allograft slings for facial muscle weakness, and gelatin (Gelfoam) injection/ silastic implant of a paralyzed vocal cord for medialization to potentiate swallowing and improve voice quality.
The incidence of intraoperative or postoperative stroke has decreased dramatically as surgical and anesthetic techniques have improved. This improvement has been attributed to many factors, including detailed preoperative imaging and angiographic evaluation to determine vessel involvement by tumor, carotid occlusion testing, correlation of bilateral cerebral angiography findings with postocclusion cerebral function, and advances in surgical arterial revascularization techniques. Recent series report a 0% to 2% rate of major strokes as a complication of carotid body tumor resection[ 1,50,55,69-72]; earlier series reported a rate of 10% to 20%.[72,73]
Compared to carotid body paragangliomas, vagal paragangliomas are usually not as intimately associated with the great vessels, making vascular injury less likely. Netterville et al reported carotid artery encasement in 5 of 46 patients with vagal paragangliomas. Other paragangliomas associated with vascular injury include large jugular paragangliomas, which commonly involve the internal carotid artery, especially if there is erosion of the bony partition between the internal jugular vein and the internal carotid artery. Internal carotid artery damage occurred in only 1% of patients with large paragangliomas of the skull base.
Resection of bilateral carotid body tumors can result in baroreceptive dysfunction due to bilateral denervation of the carotid sinus. This dysfunction manifests as sustained hypertension and tachycardia. Netterville et al reported that 10 of 11 patients who underwent bilateral carotid sinus denervation demonstrated severe labile hypertension/hypotension, headache, diaphoresis, and emotional instability.
As the parasympathetic response is lost, unopposed sympathetic stimuli result in cardiovascular morbidity, which is usually successfully managed postoperatively with alphaadrenergic antagonists. Sodium nitroprusside is administered in the early postoperative period to prevent hypertension. The long-term cardiovascular effects are controlled with clonidine or phenoxybenzamine (Dibenzyline).
Financial Disclosure: The authors have no significant financial interest or other relationship with the manufacturers of any products or providers of any service mentioned in this article.
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