An Unusual Case of Pediatric Painless Ophthalmoplegia

Abstract

Acquired unilateral ophthalmoplegia in childhood has many potential causes. Tolosa-Hunt Syndrome is characterized by painful ophthalmoplegia caused by nonspecific inflammation of cavernous sinus or superior orbital fissure. It is rarely present in children. Corticosteroid treatment is the current mainstay of treatment but cases of THS that failed to respond to steroids have been reported. We report a case of cavernous sinus pseudotumour presenting as a painless ophthalmoplegia in a child, in which complete resolution was spontaneous. To our knowledge, such a case has never been reported in the literature.

Bilateral Tolosa-Hunt syndrome mimicking pituitary adenoma

“…The course of the disease in our patient supports current evidence of a possible spontaneous remission in certain cases [ 19 ]. Up to now, i.e.…”

Tolosa-Hunt Syndrome in Childhood and Adolescence: A Literature Review in the Last 10 Years

“…In one patient, pain was absent. 9 The diagnosis of THS is based on clinical, radiological (magnetic resonance) and histologic (biopsy) criteria proposed by ICHD-3, 17 which have a sensitivity of 95.0 to 100.0%. 10 The biopsy of the granulomatous lesion is rarely performed and this fact was observed in all of our patients.…”

“…Since the initial description of THS by Tolosa and Hunt the effectiveness of his treatment with steroids is known. 19,20 However, there is no consensus on the route of administration or dose of this medication, 9 but the suggested dose is equivalent to 1 mg/kg/d of prednisone, and should be gradually reduced within 12 to 16 weeks. 13 THS occurs in episodes with recurrent character, both during and after weaning from corticosteroid therapy.…”

“…However, there are several reports of cases of childhood involvement, showing a similar clinical presentation to adult patients. [5][6][7][8][9][10][11][12][13][14][15][16] The etiology of THS has not yet been clarified and its diagnosis is one of exclusion. During the investigation of THS it is necessary to exclude other causes of recurrent multiple cranial neuropathies, such as infectious (bacterial, viral, and fungal), neoplastic, vascular diseases (aneurysms, carotidcavernous fistula, or thrombosis) and other etiologies (sarcoidosis, Wegener's granulomatosis, and eosinophilic granuloma) that may present the same signs and symptoms.…”

“…During the investigation of THS it is necessary to exclude other causes of recurrent multiple cranial neuropathies, such as infectious (bacterial, viral, and fungal), neoplastic, vascular diseases (aneurysms, carotidcavernous fistula, or thrombosis) and other etiologies (sarcoidosis, Wegener's granulomatosis, and eosinophilic granuloma) that may present the same signs and symptoms. 9 According to ICHD-3 diagnostic criteria for THS, 17 the patient must have orbital or periorbital pain ipsilateral to granulomatous inflammation preceded by paresis of the IIIrd, IVth, or VIth cranial nerves, for 2 weeks or developed at the same time. In addition, brain MRI or biopsy should show granulomatous inflammation in the cavernous sinus, in the superior orbital fissure or in the orbit (►Table 1).…”