Background: Enlargement of extraocular muscles is an uncommon finding
in children. Little has been written in the literature about possible
causes. Methods: The authors present the clinical findings of a young
girl who had unilateral congenitally enlarged extraocular muscles at 8
months of age. She underwent computed tomography (CT) and magnetic re
sonance imaging (MRI) scans, systemic assessment, and extraocular musc
le biopsy to determine a cause for the enlarged muscles. Results: The
patient had a unilateral left esotropia and hypotropia at birth that w
as nonprogressive. High-resolution CT and MRI scans showed enlargement
of the left inferior rectus, lateral rectus, and medial rectus muscle
s. Strabismus surgery undertaken at 2 years of age showed that the aff
ected muscles were restricted on forced duction testing but were macro
scopically normal in appearance. A biopsy specimen of the left lateral
rectus muscle was processed for histologic, histochemical, and electr
on microscopic studies, but no abnormal pathologic findings were found
. Conclusion: This patient's constellation of findings appears to be u
nique: it does not follow any previously reported pattern of disorders
of extraocular muscle enlargement.