Strabismus and diplopia revealing rhabdomyosarcoma in a 7-year-old girl

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Abstract

Purpose

This case report presents the diagnosis and management of seven-years-old girl with persistant diplopia revealing orbital rhabdomyosarcoma,a rare and malign type of tumor among children. Few cases have been reported.The clinical presentation, complementary exams, and treatment for this condition are reviewed.

Methods

A 7-year-old girl was referred to our department for persistant diplopia, and unilateral exophtalmia on her left eye that had been lasting for 5 months.The orthoptic examination showed no visual acuity loss, vicious head tilt bent on the left side, exotropia with quick restitution, limitation of abduction and elevation on left eye. The Lancaster test confirmed the limitation.The eye fundus examination revealed signs of extrinsic compression.

Results

Magnetic resonance imaging, and ocular and orbital echography showed an intra conical tumor of 18x21millimeters, displacing the ocular nerve and invading the superior rectus muscle. Tumor biopsy revealed undifferenciated rhabdomyosarcoma. Metastatic work-up was negative and included thoracic and abdominal CT, bone scintigraphy and bone marrow biopsy. The treatment consisted in chemotherapy and permitted tumor volume regression of 85%. The patient has successfully manage with muscle surgery, not on the cicatricial site but on the other eye.

Conclusion

Ocular rhabdomyosarcoma is a rare tumor among children, but the most frequent malign orbital tumor, with possible secondary localisation. Early diagnosis and early treatment are determinant for the prognosis and the survival rate of these patients. This case report shows all the utility of strabismological examination to detect the first signs of the condition, even without any symptoms relative to visual acuity.

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