RESUMEN
OBJECTIVE: To describe presenting ophthalmic signs and symptoms in children with parameningeal rhabdomyosarcoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Twenty-three children with parameningeal rhabdomyosarcoma treated from 1978 through 1998. MAIN OUTCOME MEASURES: Each patient's presenting symptoms and history, the ophthalmic signs at presentation, the location of the tumor on computed tomographic scanning, the pathologic diagnosis after tumor biopsy, and the child's outcome after chemotherapy and radiotherapy. RESULTS: Eight of 23 patients with parameningeal rhabdomyosarcoma had ophthalmologic signs at presentation. All patients had sixth nerve palsies, and four had additional third nerve palsies. Fifth and seventh nerve involvement was seen in three children each, and two manifested additional fourth nerve palsies. In two patients, invasion of the optic canals and posterior orbit resulted in unilateral blindness in one and marked unilateral visual loss in the other. In no patient was the symptomatic history longer than 12 weeks. Seven of eight patients were treated with a combination of surgery, chemotherapy, and radiotherapy. The mean survival time for five patients who died from parameningeal rhabdomyosarcoma associated with ophthalmic signs was 27.2 months. Three patients are currently alive, with a mean survival time of 33.7 months after diagnosis. The survival time of 12 patients with parameningeal rhabdomyosarcoma and without ophthalmologic signs at presentation was better, in that nine are alive, with a mean survival time of 54 months. CONCLUSIONS: Advanced parameningeal rhabdomyosarcoma with skull base invasion may present with ophthalmic signs. These patients sought treatment late and had advanced tumors because their initial signs and symptoms were often attributed to more common ailments. Usually, the onset of the cranial nerve palsy initiated referral to the tertiary care center. The sixth nerve was the most vulnerable. Further invasion of the skull base or cavernous sinus may cause additional third, fourth, and fifth nerve palsies. The optic nerve was involved when the optic canals and orbit were encroached on by the tumor. The prognosis for survival was poor when these tumors were accompanied by ophthalmic signs.
Asunto(s)
Enfermedades de los Nervios Craneales/diagnóstico , Oftalmopatías/diagnóstico , Neoplasias Meníngeas/diagnóstico , Rabdomiosarcoma Embrionario/diagnóstico , Niño , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Masculino , Neoplasias Meníngeas/terapia , Estudios Retrospectivos , Rabdomiosarcoma Embrionario/mortalidad , Rabdomiosarcoma Embrionario/terapia , Tasa de Supervivencia , Tomografía Computarizada por Rayos XRESUMEN
PURPOSE: We describe 6 cases of a previously unreported variation of bilateral Brown's syndrome that presented in congenital form in one eye and developed later in the fellow eye with no underlying cause. METHODS: We reviewed the clinical records of 6 patients from 6 separate practices to determine whether there were any common clinical features on presentation or in their clinical courses. RESULTS: All 6 patients were diagnosed with unilateral congenital Brown's syndrome at the first ophthalmologic assessment but showed no evidence of the syndrome in the fellow eye. In 5 cases the contralateral syndrome developed in the second eye after surgery was performed on the first eye, and in 1 case it developed before any surgery was done. The ages at onset of the syndrome in the second eye ranged from 2 to 8 years. None of the children had any evidence of systemic illness or local orbital disease to explain an acquired Brown's syndrome. CONCLUSION: To our knowledge, this is the first reported series of cases of bilateral Brown's syndrome that manifested sequentially in the eyes with no known causes for an acquired syndrome in the second eye. This finding supports the premise that congenital and acquired Brown's syndrome are on a continuum with a common pathophysiology of restriction of free movement of the superior oblique tendon in the trochlea.