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Rhabdomyosarcoma, when confined to the extraocular muscle, is rare, in particular when esotropia with limited abduction is presented as the initial sign. Clinically, this condition is more likely to be misdiagnosed as either concomitant esotropia or paralytic esotropia, especially in an uncooperative child.
A 14-month-old boy was referred with an inward deviation and an outward rotation limitation of the left eye for 6 months. There was no history of trauma or fever prior to the appearance of the deviation, but 2 months prior to referral the boy had been diagnosed with paralytic esotropia, and no specific treatment was administered. The patient had a record of 50 prism diopters of left esotropia and mild thickening of the left medial rectus muscle as revealed by computed x-ray tomography (CT) scan of the orbit and a normal brain CT.
When the boy visited our strabismus clinic, he presented with 80 prism diopters of left esotropia in primary gaze and an inability to abduct to the midline. Slit-lamp and direct ophthalmoscopy examinations were normal and proptosis and retraction were not obvious. Limited movements were present in the doll head test, and the forced abduction test under general anesthesia revealed that the left eye was fixed inward. The confirmation of restrictive esotropia and the mild abnormal changes in previous orbit CT scan suggested an orbit associated disease. Accordingly, consultations with orbital specialists were arranged. A week later, the boy presented with a progressive esotropia of 90 prism diopters, and the orbital specialists advised an orbital CT scan. The CT scan revealed a marked enlargement of the left medial rectus muscle (Fig. 1), and a medial rectus tumor was suspected. An anterior approach to the orbit was chosen to fully expose and remove the medial rectus mass. The histopathology indicated rhabdomyosarcoma, and postsurgical radiotherapy was recommended.
Orbital rhabdomyosarcoma is the most common pediatric primary tumor in the orbit. Rhabdomyosarcoma arises from primitive mesenchymal tissue and can occur in all soft tissues of the orbit. The patient with orbital rhabdomyosarcoma generally presents with proptosis (80% to 100%), globe displacement (80%), blepharoptosis (30% to 50%), conjunctival and eyelid swelling (60%), palpable mass (25%), and pain (10%).
However, rhabdomyosarcoma presented as a deviation with limited ductions as an initial sign and without other orbital signs is rather rare and, accordingly, is more likely to be misdiagnosed. To our knowledge, only 1 case of metastatic orbital rhabdomyosarcoma, which presented as an isolated restrictive strabismus,
have been reported previously. In these reports the patient's history of nonorbital rhabdomyosarcoma alerted the doctor to the correct diagnosis. As for the case reported here, misdiagnosis resulted from a failure to make a prompt diagnosis of restrictive strabismus and attend to the early changes shown in the orbital CT scan, which may be interpreted as an orbital tumor. The doll head test can be useful in uncooperative infants to distinguish between comitant and incomitant strabismus. The patient here showed limited movements in both horizontal and vertical planes with the doll head test, and a forced duction test was then performed to confirm this restriction. In addition, application of various imaging modalities is essential to substantiate the diagnosis of an orbital tumor. For the identification of an early lesion in the extraocular muscle, magnetic resonance imaging or CT scan with contrast may be better for evaluating the lesion and biopsy can then be used to confirm the diagnosis. Finally, an acquired progressive deviation may serve as an important sign suggesting orbital tumors. When a patient presents with an acquired progressive restrictive deviation, an orbital tumor should be considered and orbital imaging should be performed. As for a suspected case in the early stages, follow-up and repeated examinations are warranted.