Case Presentations

In Depth Case Review-Weiss

HISTORY AND PHYSICAL EXAM:

CC: Right-sided facial pain for six years.

HPI: JG is a 54-year-old Hispanic male with a six-year history of progressive right-sided facial pain. Patient states that the pain is always present, and relieved only briefly with Vicodin or Neurontin.

The pain is a 10/10 ‘burning’ pain localizing to his right mid-face, and extends back to the scalp above his ear.
PMH: none
PSH: none
NKDA
Medications: Neurontin 1200 mg q6 hr, Vicodin prn
SH: no tobacco or alcohol use, denies other drugs.
ROS: negative

PE: T 98.8 F BP 130/80 P 86 RR 18 Rt facial pain 10/10

General: WD, WN, HM in mild distress
HEENT: NCAT, no lymphadenopathy
CVS: regular rate and rhythm, no murmurs
Resp: clear to auscultation bilaterally
Abd: soft, nontender, nondistended, normal bowel sounds
Extremities: no edema, cyanosis or clubbing

MS: AAO x 4, speech fluent, follows commands well
CN: PERRL 3 to 2, EOM intact, face symmetrical, sensation intact over V1-3 except hyperesthesia over the right V2 distribution, tongue midline,
Motor: MAE with 5/5 strength, reflexes 2+ and symmetric bilaterally, toes down-going, no clonus
Sensory: sensation intact to light touch and pinprick in all extremities
Cerebellar: gait normal, finger to nose normal

Labs: Na 138 K 4.3 Cl 105 HCO3 24 BUN 10 Cr. 0.7 Glucose 170
WBC 9.6 Hgb 13.1 HCT 38.7 Plt 121
UA negative

DISCUSSION AND QUESTIONS PT. 1:

Consultant: What are the triggers to pain?
Presenter: There are no triggers to pain. The symptoms are constant.

Dr. Weiss: How effective is using Neurontin for pain management?
Dr. Gruen: Neurontin is not effective in patients with failed back syndrome.
Dr. Samudrala: There is some subset of patients who do benefit from Neurontin. This subset of patients usually includes patients that can tolerate high enough effective doses of neurontin without limiting side effects (e.g. drowsiness, cognitive problems). We don’t know the exact mechanism of neurontin’s effects however there has been some benefit of giving neurontin particularly early in the course of patient’s with chronic pain for example patients with crush injuries to peripheral nerves.
Dr. J. Chen: Neurontin is effective for pain management however does not appear to be any more effective than Elavil or Nortriptyline. Patients who benefit from neurontin are probably the same patients who benefit from Elavil or Nortriptyline

Dr. Weiss: What is the most sensitive manner in which to test for CN V dysfunction?
Consultant: Check for corneal reflex with a wisp of cotton. One does not need to have an intact CN VII to test for sensation from the cornea.

Dr. Weiss: How does one test for CN V motor function?
Consultant: Open, close jaw. Also, check for symmetric elevation of the soft palate (tensor veli palatini function). The soft palate will pull to the side of normal CN V function (i.e. opposite from the affected side).

Dr. Weiss: Why is this history not consistent with typical trigeminal neuralgia?
Consultant: Trigeminal neuralgia is usually intermittent pain with pain-free periods. Trigeminal neuralgia also typically has triggers such as chewing, yawning, talking, brushing teeth. The description of the pain is usually like an electric shock.

Dr. Weiss: Can you have persistent pain with trigeminal neuralgia?
Answer: Patients with severe trigeminal neuralgia will have baseline chronic pain with episodes of worsening exacerbations.

Dr. Weiss: Can you have sensory loss with trigeminal neuralgia?
Answer: 10% of patients with trigeminal neuralgia will have hypesthesia in the distribution of the pain

Dr. Weiss: Where is the lesion localized to?
Consultant: 2nd division of the R trigeminal nerve.

Dr. Weiss: Where do the three divisions of the trigeminal nerve exit the skull from?
Consultant: V1 – superior orbital fissure (with CN III, IV, VI)
V2 – Foramen rotundum
V3 – Foramen ovale

Dr. Weiss: With what division does the motor branch of V run along with?
Consultant: V3

Dr. Weiss: What is your differential diagnosis?
Consultant: meningioma, schwannoma, epidermoid, arachnoid cyst, inflammatory disorder, tuberculosis, lymphoma, basilar ectasia, clival chordoma

Dr. Weiss: Is the history of pain without numbness consistent with an intrinsic lesion of CN V (i.e. CN V schwannoma) or an extrinsic lesion compressing CN V?
Answer: Intrinsic lesions generally cause numbness via disruption of sensory fibers. Extrinsic lesions primarily cause pain secondary to irritation of the nerve.

Dr. Weiss: What is the most likely diagnosis if the patient has bilateral trigeminal neuralgia?
Answer: Multiple sclerosis. The plaque is usually in root at the root entry zone. Microvascular decompression has a higher failure rate for trigeminal neuralgia secondary to multiple sclerosis.

Dr. Weiss: What imaging studies do you want?
Consultant: Head CT, Brain MRI +/- contrast.

PREOPERATIVE IMAGING:

Figure 1

Head CT: Calcified structure adjacent to right carotid canal causing remodeling of petrous apex with mild sclerotic changes, meningioma vs. osteoma.

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Figure 6

Brain MRI +/- contrast: 1.5 x 1.2 x 1.3 cm calcified lesion in right Meckel’s cave with compression of trigeminal nerve, most likely calcified meningioma.

DISCUSSION AND QUESTIONS PT. 2:

Dr. Weiss: What nerves pass through the cavernous sinus?
Consultant: CN III, IV, V1, V2, VI, sympathetics along carotid artery

Dr. Weiss: What syndrome involves CN V distribution pain and CN VI palsy?
Answer: Gradenigo’s syndrome, apical petrositis

Dr. Weiss: What is Tolosa Hunt syndrome?
Consultant: Inflammatory disorder of the superior orbital fissure resulting in pain with ophthalmoplegia, however, vision is not affected. Usually occurs in elderly women.

Dr. Weiss: How do you distinguish giant cell arteritis vs. Tolosa Hunt syndrome?
Consultant: Giant cell arteritis results in visual loss secondary to ophthalmic artery distribution ischemia

Dr. Weiss: How would you approach this lesion surgically?
Consultant: Infratemporal, extradural, middle fossa approach

Dr. Weiss: What structure sits 1 mm from the foramen ovale that you need to be aware of?
Consultant: Foramen spinosum and middle meningeal artery

Dr. Weiss: What has occurred if the patient develops a CN VII palsy on POD #2 from the above procedure
Answer: Patient has avulsed greater superficial petrosal nerve which causes swelling and inflammation to track back to the geniculate ganglion resulting in a facial nerve palsy (may be permanent).

PROCEDURE:

Figure 7


Figure 8

Supine position, Right temporal craniotomy, extended middle fossa approach for tumor resection

PATHOLOGY:

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Report: Meningioma WHO grade I with extensive calcifications.
Immunohistochemistry positive for EMA and negative for S-100, chromogranin, and synaptophysin.

POSTOPERATIVE IMAGING

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Figure 16

Brain MRI +/- contrast: interval removal of calcified mass from lateral dura of right cavernous sinus