Congenital orbital fibrosis: report of two cases and review of literature.

Orbit

Larson and Toubro Department of Ocular Pathology, Vision Research Foundation, Sankara Nethralaya, Chennai, India.

Published: October 2024

AI Article Synopsis

  • A 7-year-old boy had eyelid issues since birth, with MRI revealing thickening of certain eye muscles and an irregular lesion by the lacrimal gland, confirmed to be orbital fibrosis through a biopsy.
  • A 3-year-old girl also showed symptoms of a smaller right eye and limited movement, with MRI indicating thickened eye muscles and fibrotic strands, again suggesting orbital fibrosis.
  • Congenital orbital fibrosis is rare, presenting with symptoms like restricted eye movement and lid retraction, diagnosed through imaging but needing a biopsy for confirmation, with treatment focused on conservative methods like therapy for vision issues.

Article Abstract

A 7-year-old male child presented with complaints of lagophthalmos and lid retraction of the right eye since birth. Magnetic resonance imaging (MRI) showed diffuse thickening of right superior rectus and levator-palpebrae complex along with a hypointense, irregular, and ill-defined lesion in the adjoining fat abutting the lacrimal gland. Biopsy from the lesion showed diffuse orbital fibrosis. Another 3-year-old female child presented with complaints of her right eye appearing smaller and inability to move the right eye freely since birth. MRI showed thickening of right superior and medial recti with diffuse retrobulbar hypointense fibrotic strands. The findings were suggestive of orbital fibrosis. Congenital orbital fibrosis is an extremely rare orbital pathology with very few cases described in the literature. The most common clinical features are motility restriction, restrictive strabismus, upper lid retraction, enophthalmos, and proptosis. The diagnosis can be made on imaging but requires biopsy for confirmation. Management is mostly conservative in the form of refractive and amblyopia therapy.

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Source
http://dx.doi.org/10.1080/01676830.2023.2223686DOI Listing

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