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1 Case Report

A 59-year-old woman presented with sudden-onset eyelid swelling, ptosis, diplopia, mild pulsation, and mild pain of the right periorbital area for 4 days. She had gone to a local hospital and received antihistamine treatment. She was referred to our hospital for further investigation. The patient had no known history of systemic diseases except for hyperlipidemia. She also denied previous trauma and allergies. Her vision was 6/6 in both eyes. Mild lid ptosis was noted in the right side. A Krimsky test revealed a 35-prism diopter of exotropia in the right eye. In addition, extraocular movement showed moderate to severe limitations in adduction, supraduction, and infraduction of the right eye (Fig. 27.1). Anterior segments and fundoscopic examination were normal. Pupils were symmetric and reactive to light. An MR imaging study showed an enhancing lesion over the right cavernous sinus (Fig. 27.2) and segmental stenosis of the right internal carotid artery (ICA). The patient was admitted for lumbar puncture which showed an opening and closing pressure of 78/55 mmH2O, respectively. CSF analysis showed no white blood cells and a normal protein level and IgG index (0.64). Pulse therapy with methylprednisolone was given. Her orbital pain was relieved after steroid treatment and eye movement improved gradually. Her eye position returned to orthophoric with a full range of motion observed 1 month later (Fig. 27.3).

Fig. 27.1
figure 1

Moderate ptosis and exotropia were noted in the right eye. In addition, extraocular movement showed severe limitations in adduction and infraduction and moderate limitation of supraduction in the right eye

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Fig. 27.2
figure 2

MR imaging study showed a small enhancing lesion (arrow) over the right cavernous sinus on T1-weighted with gadolinium enhancement (T1Gd)

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Fig. 27.3
figure 3

Residual mild ptosis and orthophoric eye position, with a full range of motion 1 month later

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2 Comments

Tolosa-Hunt syndrome (THS) is a recurrent, painful ophthalmoplegia due to an idiopathic granulomatous inflammation of the cavernous sinus or superior orbital fissure [1, 2]. It was named after Dr. Tolosa and Dr. Hunt, who described this syndrome in 1954 and 1961, respectively [3, 4]. THS can occur in patients over a wide range of ages [5]. This syndrome is characterized by an acute or subacute onset of unilateral orbital pain and external ophthalmoplegia, associated with proptosis, ptosis, red eye, chemosis, or even lacrimal gland swelling. The orbital pain is typically severe and located behind the eye. Oculomotor, trochlear, trigeminal, and abducens paresis may occur at the time of onset or a few days later. The pupils are usually normal but may be involved in the cases of associated oculomotor nerve palsy or Horner’s syndrome [5]. Optic nerve involvement with visual losses has been reported, implying that the orbital apex can be involved by an inflammatory process [6]. Histopathologically, THS shows granulomatous inflammation with lymphocytic and plasma cells infiltration in the cavernous sinus. The symptoms of THS may last for days to weeks, or they may resolve spontaneously, even before the use of corticosteroids. However, there is a high recurrence rate with recurrent symptoms commonly manifesting months to years after the initial attack [5, 7].

2.1 Diagnostic Tests

Neuroimaging studies and CSF analyses are the most important tests. MR imaging of the brain or sella may help to detect the focal inflammatory mass lesion in the involved cavernous sinus, which enhances after gadolinium injection. In some patients, the focal mass extends into the orbital apex and intraorbitally [8]. A CSF study is essential, and the result should be unremarkable, with rarely raised protein and/or mild pleocytosis [5]. It is also crucial to obtain CSF cultures to exclude the infectious causes.

2.2 Differential Diagnosis

THS is typically a diagnosis of exclusion, and it is important to exclude other infectious, inflammatory, neoplastic, and/or vascular disorders. Thus, blood tests including complete blood count, ESR, C-reactive protein, thyroid function, liver function, and a chest X-ray should be examined. A wide variety of differential diagnoses for THS includes bacterial sinusitis, mucormycosis, tuberculosis, sarcoidosis, Wegener’s granulomatosis, idiopathic orbital inflammation, giant cell arteritis, pituitary adenoma, meningioma, lymphoma, cancer metastasis, cavernous sinus thrombosis, and carotid-cavernous fistula [1].

2.3 Management

THS may resolve spontaneously without treatment. Corticosteroids have been proven beneficial to decrease orbital pain, though it remains unclear whether steroids improve or hasten recovery. Generally, the corticosteroids are tapered slowly over months since there is a high recurrence rate. Patients with prolonged steroid use especially require careful evaluation and observation during follow-up, because other etiologies may also respond to steroid treatment [5, 7].

2.4 Prognosis

THS is generally a benign process and usually recovers completely. However, in about half of the cases, it may recur several months or years later, either on the ipsilateral or the contralateral side [5, 7]. Since THS is a diagnosis of exclusion, it is essential to follow up using MRI in those cases without a biopsy or CSF study performed. An MRI may help to verify the effects of treatment and detect other etiologies if present.

Keynotes

  • Test for THS: MR imaging of the brain and CSF study.

  • Management: corticosteroid treatment.

  • Protect your patients and others: systemic checkup to exclude other etiologies; CSF study is essential before treatment.

  • DO NOT use corticosteroids if the patient exhibits paranasal sinusitis.

  • Follow-up MRI of the brain to verify if the cavernous lesion resolves after treatment.