Rhabdomyomatous mesenchymal hamartoma of the eyelid - Report of a case andliterature review

Purpose: To report a rare case of rhabdomyomatous mesenchymal hamartoma and to compare its features with those cases previously reported. Design: interventional case report and literature review. Intervention: Complete ophthalmologic and systemic examinations followed by excisional biopsy and histopathologic examination, Main Outcome Measures: Clinical examination features and histopathologic fi ndings. Results: A 6-month-old Latino male presented with a congenital, elevated, s mooth, flesh-colored right lower eyelid lesion. An ipsilateral right limbal dermoid and an upper eyelid coloboma were also present. Excisional biopsy of the eyelid lesion revealed randomly oriented mature striated muscle tiss ue with associated adipose tissue, blood vessels, pilosebaceous units, and peripheral nerves, findings consistent with rhabdomyomatous mesenchymal ham artoma, Of the 24 reported cases (including the current case), eight had as sociated congenital anomalies. Conclusions: Although rhabdomyomatous mesenchymal hamartomas are rare and b enign, they may be associated with other congenital anomalies and anomaly s yndromes. As a result, we recommend systemic evaluation of patients diagnos ed with this entity. (C) 2001 by the American Academy of Ophthalmology.