Resection of malignant tumors involving the jugular foramen

RESECTION OF MALIGNANT TUMORS INVOLVING THE JUGULAR FORAMEN VICTOR L. SCHRAMM, JR, MD

Surgical resection of the jugular foramen may be used as part of temporal bone resection, extended radical parotidectomy or middle fossa-infratemporal fossa resection. Detailed tumor imaging and carotid test occlusion, team planning, and extensive patient counseling are required. Exposure from retromastoid to the lateral orbit allows facial nerve and mandibular transposition, temporary removal of the zygomatic arch, and exposure of the petrous carotid. When tumor location and the height of the jugular bulb are favorable, hearing may be preserved. The final dissection in a 3-×-4 cm area of skull base requires expert anesthesia and surgery to limit bleeding and maintain hemodynamic stability during the petrous carotid and jugular bulb dissection. Rehabilitation most often requires free flap reconstruction of bone and soft tissue defects and facial reanimation. Aspiration pneumonia is the most common postoperative sequela. Functional long-term survival has been possible for a majority of patients undergoing jugular foramen resection.

Surgical management of malignancy involving the area of the jugular foramen and the adjacent carotid artery, lower cranial nerves, and dura is the most difficult of all skull base surgery procedures. Not even cavernous sinus dissection rivals the potential for blood loss and patient morbidity. Management in these challenging situations begins with a great deal of patient consultation, detailed planning, and extensive discussion of sequelae and potential complications. Expert anesthesia, advanced surgical technique, and intensive postoperative care are required. Despite the potential availability of surgical resection, a dilemma remains for physicians and patients, related in great part to advanced disease and delayed diagnosis, failed prior therapy, and the ethical considerations related to the short- and long-term functional and tumor outcome. This article relates the present approach to patients and surgical technique that has evolved from many years of skull base experience. It is hoped that with the following discussion, the reader will be able to integrate and improve on these techniques.

SYMPTOMS OF JUGULAR FORAMEN INVOLVEMENT Severe and unrelenting pain deep in the ear and suboccipi-

tal region is the usual motivating factor for patients to be evaluated and to consider therapy. Often there is hoarseness that may be followed b y progressive difficulty swallowing related to vagus nerve and pharyngeal plexus involvement. As symptoms advance, ipsilateral tongue paralysis and subtle sternocleidomastoid paresis occurs. Conductive hearing loss occurs as a result of eustachian tube compression from skull base tumor extension. Uncommonly, sudden or progressive sensory neural hearing loss is noted if tumor erosion of the otic capsule occurs. Trismus may be associated with parapharyngeal or pterygomaxillary tumor involvement. From the Department of Otolaryngology--Head and Neck Surgery, Universityof Colorado Health Sciences Center, Denver,CO. Address reprint requeststo Victor L. Schramm, Jr, MD, 1601 E 19thAve, #3100, Denver,CO 80218. Copyright © 1996 by W.B. Saunders Company 1043-1810/96/0702-0017505.00/0 1 68

INDICATIONS AND CONTRAINDICATIONS The approach to the jugular foramen may be used with appropriate surgical extensions to address tumors arising in the oral cavity and extending to the jugular foramen via the parapharyngeal space, as well as parotid tumors following the facial nerve to the temporal bone. Ear canal malignancy and parapharyngeal sarcomas, particularly of neural origin, are also candidates for this type of surgical resection. Certainly, benign tumors arising in the jugular bulb expand the potential usefulness of this surgical approach. Low grade or slowly progressive tumors, such as adenoidcystic or mucoepidermoid carcinoma, have relatively more favorable outcomes, although squamous carcinoma arising in the ear canal or oral cavity may also be successfully resected. The patient's inability to withstand the difficult surgery from a medical perspective, and the presence of distant metastatic disease remain the only absolute contraindications to surgery. Individual patient considerations such as the tumor biological behavior, inability of the patient to tolerate temporary carotid artery occlusion, and dural or superficial brain invasion may be considered as relative contraindications. The final decision regarding surgery is based on a precise definition of the tumor location with magnetic resonance imaging complemented by fine cut CT scanning. High-resolution thin cut magnified MR images with fat suppression techniques are most useful. Most patients should undergo angiography with carotid test occlusion, supplemented by the techniques of hypotension and some form of quantifiable evaluation of cerebral circulation during and after occlusion. Special attention must be given to the venous phase of the arteriogram to document collateral venous flow and the anatomical relationship of major venous outflow tracks to the margins of tumor resection.

ANESTHESIA TECHNIQUES Because of the magnitude of the surgical procedure, an anesthesia team with neurological, cardiac, and trauma surgery experience is mandatory. The key to interoperative and postoperative survival is the management of fluid and

OPERATIVE TECHNIQUES IN OTOLARYNGOLOGY--HEADAND NECK SURGERY, VOL 7, NO 2 (JUN), 1996: PP 168-174

blood replacement. Placement of a subclavian oximetric Swanz-Ganz catheter (Abbott Critical Care Systems, North Chicago, IL) to monitor mixed venous oxygen saturation has proven to be the most reliable method of determining blood replacement needs. Experience has also shown that if more than 4 L of crystalloid are administered even for a 20-hour surgical procedure, postoperative central nervous system, wound, and pulmonary complications markedly increase. The patients are routinely monitored with continuous arterial blood pressure, end tidal CO2, pulse-oximetry, central temperature, and urinary output. A lumbar subarachnoid drain is essential, and patient temperature control is adjusted with fluid warming and warming blankets. Pulsatile compression leg stockings are used. Antibiotic prophylaxis is routinely administered. Because blood replacement requirement is generally great, routine use of two cell-saver suctions, along with interoperative hemodilution and plasma phoresis, saves many unit equivalents of homolagous transfusion. Controlled hypotension, with a combination of inhalant and intravenous narcotic technique avoiding fluctuations in blood pressure, greatly facilitates the surgical dissection.

SURGICAL TECHNIQUE The tumor removal in the area of the jugular foramen and jugular bulb is the pinnacle of a surgical technique that can be done safely only after an encompassing surgical approach. Although the need for exposure will depend on the location of the primary tumor and surrounding extensions, a lateral skull base approach that gives exposure to the neck, suboccipital area, temporal fossa, face and oral cavity is necessary. A preliminary tracheostomy is done, after which a separate surgical preparation includes the scalp, face, circumferential neck, shoulders, and anterior chest. Further preparation for a free flap reconstruction is done simultaneously or with a secondary prep after resection, depending on the requirements for reconstruction and free flap donor site.

CRANIAL-CERVICOFAClAL APPROACH Depending on the presence of prior incisions, and the potential for preserving the ear canal and tympanic membrane, a preauricular or postauricular incision may be chosen. If a prior parotidectomy incision is present, this may be used along with a bicoronial and cervical extension, complemented by a horizontal suboccipital incision, maintaining an adequate blood supply to the external ear. If the middle ear is to be sacrificed, a postauricular incision beginning 3 cm behind the ear is preferable (Fig 1). The safest and most convenient cranial exposure involves a bicoronial extension of the upper cervicofacial portion of the incision with reflection of the scalp flap across the entire frontal bone to the orbital rim. The facial portion of the craniofacial skin flap is elevated superficial to the parotid fascia, mobilizing the skin flap to the malar eminence and around the lateral orbit superiorly and anterior to the masseter muscle inferiorly.

PAROTID AND PERIPHERAL FACIAL NERVE MANAGEMENT In situations in which the parotid and facial nerve are to be spared, the parotid and contained facial nerve are mobilized completely away from the mandible from the styloVICTOR L. SCHRAMM, JR

FIGURE 1. Incisions for cervical and craniofacial approach. Preauricular incision with suboccipital extension (dashed line) or postauricular incision behind level of sigmoid sinus connected to bicoronal and upper cervical incisions. McFee incision is preferable for neck dissection. mastoid foramen to the peripheral facial nerve branches. The mobilization begins during the scalp flap transposition by developing an envelope of soft tissue around the upper branches of the facial nerve. This is accomplished by dissecting at the level of the temporalis fascia, developing a plane beneath the facial nerve to the zygomatic arch. Secondarily during the facial skin transposition, the soft tissue is separated from the facial skin flap to within I or 2 cm of the lateral orbital rim. The soft tissue around the facial nerve is then mobilized between the stylomastoid foramen and the mandible, without skeletonizing the trunk of the facial nerve. The entire parotid is then mobilized away from the masseter muscle fascia, continuing the plane beneath the parotid, over the zygomatic arch and mandible from a superior dissection, and connecting this with mobilization of the parotid done from below over the masseter fascia. With complete mobilization of the parotid, the mandible may be subsequently mobilized without producing facial nerve traction (Fig 2).

INFRATEMPORAL EXPOSURE The key to obtaining exposure in the infratemporal fossa for pterygomaxillary and distal carotid dissection is the removal of the zygomatic arch and lateral orbital bone complex. This is accomplished by incising the periosteum from the frontozygomatic suture along the lateral orbital rim and superior zygomatic arch to the posterior root of the zygoma. The periosteum is then elevated circumferen169

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FIGURE 3. Infratempofal exposure for removal of zygomatic-orbital bone complex. Bone cuts made with fine oscillating saw blade through frontozygomatic suture to temporal squama and with V configuration across lateral malar eminence. Posterior zygomatic cut tangential to glenoid fossa. Lateral orbital cuts connect to inferior orbital fissure. 170

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JUGULAR FORAMEN RESECTION

tially around the arch, and the orbital contents are mobilized around the lateral half of the orbit. The temporalis muscle is dissected away from the lateral orbital wall with cutting cautery, and bone cuts are made at the frontozygomatic suture line, posterior zygomatic root and across the junction of the inferior and lateral orbital rim, extending this bone cut through the lateral malar eminence. The final bone cut across the lateral orbit from the frontozygomatic suture line to the inferior orbital fissure allows mobilization and removal of the zygomatic-orbital bone complex after transecting the masseter muscle attachment (Fig 3). Management of the temporalis muscle depends on the extent of infratemporal resection that is required. The muscle may be mobilized away from the temporal squama and transposed, allowing pterygomaxillary access and low temporal craniotomy. If the third division of the trigeminal nerve is to be sacrificed, along with the internal maxillary artery, the muscle is best transsected across the infratemporal fossa, leaving it attached to the temporal squama.

MANDIBULAR MOBILIZATION To obtain adequate exposure of the distal internal carotid and jugular foramen, the mandible is translocated anteriorly and inferiorly. This is accomplished by dissecting the joint capsule from the glenoid fossa with a periosteal elevator and cutting cautery and transecting the attach-

ment at the glenoid tubercle. Once the medial aspect of the joint capsule has been mobilized, it is possible to transect the sphenomandibular ligament. The parapharyngeal space is then entered by dividing the stylomandibular ligament, thus completing a medial tunnel, allowing the mandible to be retracted out of the glenoid fossa (Fig 4).

SUBOCCIPITAL AND SIGMOID SINUS APPROACH The carotid artery and internal jugular vein are mobilized during the cervical approach, along with a neck dissection appropriate to the disease. The sternocleidomastoid and anterior portion of the trapezius muscles are released from the skull base by cutting cautery from the mastoid tip and suboccipital area to the level of the mastoid emissary vein. A complete mastoidectomy is then accomplished and the sigmoid sinus exposed at the sinodural angle. The sigmoid is then completely decompressed and posterior fossa dura exposed anterior and posterior to the venous sinus to the level of the jugular bulb (Fig 4). The bone of the mastoid tip is removed with rongeur to the level of the ear canal. If hearing is to be preserved, the dissection continues as an infralabrynthine exposure. If the parotid and facial nerve are to be sacrificed, they are resected at this time (Fig 5).

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CAROTID TRANSPOSITION With the mandible displaced, the internal carotid artery is followed and decompressed to the skull base (Figs 5, 6). Cranial nerve IX is usually transsected, but cranial nerves X, XI, and XII are isolated and preserved. A drill is used to remove the bone of the medial glenoid fossa to follow the vertical segment of the petrous carotid artery to its turn at the horizontal segment. The fibrous ring at the carotid canal is divided and mobilized away from the carotid, following which circumferential bone removal in the vertical petrous segment may be accomplished. If total carotid transposition is required for petroclival resection, a temporal craniotomy, including the superior aspect of the glenoid fossa, is required. Complete transposition of the petrous carotid artery requires division of the third segment of the trigeminal nerve and removal of the bony eustachian tube.

MANAGEMENT OF THE JUGULAR BULB The exact sequence of dissection around the jugular foramen depends on the disease type and extent. Most often the dissection is not sequential, as will be described, and often debulking of the tumor for exposure is necessary. If the facial nerve is to be preserved, it is decompressed throughout the vertical segment to the stylomastoid foramen and mobilized out of the facial canal. If perineural extension requires facial nerve sacrifice, the nerve is di-

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FIGURE 5. Exposure of jugular foramen. Mandibular condyle mobilized for vertical petrous carotid decompression and cranial nerve isolation to skull base.

vided and frozen section analysis completed for proximal tumor clearance. This may require dissection of the nerve through the otic capsule to the internal auditory canal. The triangular bone segment between the petrous carotid artery and anterior jugular is then removed, following the ear canal and staying below the cochlea superiorly and mobilizing the carotid anteriorly. As the bone is removed around the lateral and anterior aspect of the jugular foramen, the venous wall is decompressed and the vein compressed to allow access. At this juncture it is best to place a vascular tape around the proximal internal jugular vein and to isolate the sigmoid sinus. The sigmoid may be dissected by splitting the dura and passing the vascular tape around the vein, or by penetrating the dura anterior and posterior to the mid sigmoid sinus and passing a silk ligature around the vein intradurally. The very tedious bone removal is then continued, thinning bone with a polishing burr and removing the bone from below and above toward the apex of the jugular bulb. Depending on the height of the jugular bulb in the area of the middle ear and the disease extent, it may be possible to remain below the cochlea and preserve hearing. In favorable situations it is possible at this point to dissect the lower cranial nerves into the anterior neural compartment of the jugular foramen and preserve them. The hypoglossal nerve is next dissected and followed to the hypoglossal canal with the canal being decompressed if necessary (Fig 6). The final tumor removal may require sacrifice of the JUGULAR FORAMEN RESECTION

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jugular bulb or control of bleeding may require occlusion. Because of the bleeding from the inferior petrosal vein, along with multiple secondary venous sites in the anterior wall of the jugular bulb, venous bleeding is often vigorous and two cell-saver suctions are appropriate. A technique for controlling the venous bleeding has been developed that has proven to be most helpful. Ligatures are secured across the sigmoid sinus and internal jugular vein. Following this, Gelfoam-thrombin paste (1 g of Gelfoam powder mixed into 5 mL of thrombin) is injected into the jugular bulb with a 5-mL syringe and 19-gauge needle. As Gelfoam paste fills the jugular bulb, it is compressed within the vein to occlude the inferior petrosal vein. The lateral wall of the jugular bulb may then be removed and the inferior petrosal vein further packed with muscle. Once the jugular bulb is open, and hemostasis obtained, the dissection is completed by further mobilizing the X and XI nerves in the anterior jugular foramen, followed by removal of bone as required (Fig 6). RECONSTRUCTION

REQUIREMENTS

Because of the amount of bone and soft tissue resected, a vascularized flap reconstruction is usually necessary. Dural grafting is completed, using pericranium. When the facial nerve has been sacrificed, nerve grafting or muscle reanimation is completed. Mandibular bone reconstruction, and at times skin resurfacing, is required. A microvascular free flap that has provided virtually all potential VICTOR L. SCHRAMM, JR

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reconstructive possibilities is the scapula serratus muscle myo-oseocutaneous flap. The serratus muscle is reenervated with the proximal facial nerve and oral commissure reanimation accomplished, attaching the split muscle at the lateral corner of the mouth and suspending it from the zygomatic arch. Periscapular soft tissue conveniently supplements the reconstruction and when mandibular bone removal has been required, the spine of the scapular provides a vascularized simultaneous bone reconstruction. When the vagus nerve has been sacrificed, a primary Silastic implant laryngoplasty is completed. Eye reanimation, when required, is done as a secondary procedure with lower lid advancement and upper eye lid gold weight.

EXPECTED OUTCOME AND COMPLICATIONS

The preparation, resection, and free flap reconstruction may be expected to last 16 to 20 hours. Non-colloid fluid overload must be prevented to avoid interoperative and postoperative cerebral, wound, and pulmonary edema. Venous bleeding is relatively easily controlled, although carotid artery injury requires immediate heparin anticoagulation and maintenance of cerebral profusion during repair. There have been no perioperative fatalities. Postoperative pneumonia has occurred in more than one third of patients undergoing prolonged surgery with carotid and lower cranial nerve manipulation. Aspiration is

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expected initially, and oral feeding is most often delayed until the last phase of hospitalization. Permanent gastrostomy feeding has not been required. Stroke has resulted from carotid dissection with or without resection, in 6% of a larger group of 110 patients, but as yet has not occurred in patients undergoing jugular foramen dissection.

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Follow-up is n o w available for I to 7 years. Disease-free survival, with performance status equal to or better than observed preoperatively, has been more than 50%. Although this surgery is a formidable task, the outcome compares favorably to that of surgery for other head and neck malignancy, and therefore seems to be a worthwhile endeavor.

JUGULAR FORAMEN RESECTION