The two jugular foramina are openings in the skull base located on either side, anterolateral to the foramen magnum. The main structures which pass through this foramen are the glossopharyngeal (IX), vagus (X), and spinal accessory (XI) nerves and the internal jugular vein (IJV). The foramen is divided into two parts by a fibro-osseous bridge connecting the jugular spine of the temporal bone and jugular process of the occipital bone. The anteromedial compartment (pars nervosa) contains the cranial nerve IX, the tympanic branch of IX (Jacobson's nerve), and the inferior petrosal sinus. The posterolateral component (pars venosa or vascularis) contains the IJV, jugular bulb, cranial nerves X and XI, the auricular branch of vagus (Arnold's nerve), and the posterior meningeal branch of the ascending pharyngeal artery.
The jugular foramen syndrome (JFS or Vernet syndrome) refers to paralysis of the IX, X, and XI cranial nerves traversing the jugular foramen. Vernet first described it in the Paris Medical Journal (1917).
The following etiologies of jugular foramen syndrome have been described in the literature:
The exact incidence of jugular foramen syndrome is challenging to estimate due to its rarity. Metastatic disease is the most common cause of JFS but is often underdiagnosed. Skull base metastasis is usually considered a late event in the course of systemic malignancy. The most common primary cancers which are likely to metastasize to the jugular foramen are those of breast, lung, kidney, and prostate.
Paragangliomas are the most frequent primary lesions, followed by schwannomas and meningiomas.
Recent literature suggests that primary lesions are more common than metastases.
Cranial nerve IX is primarily sensory, receiving general visceral and special sensory input from tonsils, larynx, middle ear, posterior one-third of the tongue, and carotid bodies. It has a small general motor efferent to the stylopharyngeus muscle, which aids in the elevation of the larynx and pharynx. Cranial nerve X has a wide range of functions and controls heart rate, peristalsis, sweating, speech phonation, and elevation of the palate. Cranial nerve XI is a pure motor efferent supplying the ipsilateral sternocleidomastoid and trapezius muscles. Derangement in the functions of these nerves occurs when they become compressed in jugular foramen syndrome.
Even though most cases of jugular foramen syndrome occur due to compression of structures within the jugular foramen, some other mechanisms act in viral infections. These factors can lead to the spread of the virus into the lower cranial nerves, which are as follows:
The most common presenting symptom of jugular foramen syndrome is hoarseness of voice or dysphagia. Pain is also a common associated symptom (persistent unilateral periauricular pain and headache).
Clinical features depend on the structures affected by JFS:
The clinician should specifically note the following findings in the case of JFS:
Investigations aimed at diagnosing the etiology of jugular foramen syndrome:
The finding characteristic of glomus jugulare tumors is ‘‘moth-eaten’’ pattern of destruction of the temporal bone (in CT), ‘‘salt-and-pepper’’ pattern on long TR–long TE images (in MRI), and ‘‘dropout’’ phenomenon in time-intensity curves on contrast MRA.
Treatment of JFS depends on the etiology:
Clinical variants of jugular foramen syndrome include:
The prognosis depends on the type of compression at jugular foramen. If it is due to a benign condition, curative resection may be feasible. The determining factor in survival for patients with metastatic compression is the status of the primary malignancy and the extent of other systemic metastases.
Infectious and vascular causes may respond adequately to appropriate therapy. Post-traumatic lower cranial nerve palsy is associated with incomplete recovery in the majority of cases.
The main complications which are likely to occur in patients with jugular foramen syndrome, especially in the post-operative period are:
It is needed to make the patient with JFS understand their condition and the cause. If he/she needs surgery, counseling needs to be done based on the approach. The need for tracheostomy and feeding gastrostomy should be stressed pre-operatively if necessary.
The effective management of jugular foramen syndrome requires an interprofessional approach.
The clinical skills of an astute physician or neurologist are needed to diagnose this condition. Confirmation of the diagnosis requires the presence of a neuroradiologist, specialized in interventional neuroradiology, who is skilled in interpreting the MRI and DSA images. If the patient needs surgery, the active involvement of a neurosurgeon, otorhinolaryngologist, plastic surgeon, and neuro anesthetists is necessary for a good outcome.
In the postoperative period, the timely decisions to do tracheostomy and feeding gastrostomy, with appropriate care by neuroscience specialty nurses is mandatory. A dietitian should be involved in the care to determine the type of nutrition. Also, since most patients need a tracheostomy, respiratory therapists need to educate the caregivers on its management. Nurses should provide and administer deep venous thrombosis and pressure ulcer prophylaxis, and alert the clinicians of any concerns they encounter. The social worker should be involved to ensure that the patient has adequate home facilities and support services. Since many patients do become depressed, enlisting a mental health nurse consult is a recommended step.
Both the physiotherapist and voice therapist have their unique roles in the long run. Open communication among members of the healthcare team is vital if one wants to improve patient outcomes. [Level 5]
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