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Fibrous tumor of the superior oblique tendon in Proteus syndrome

Dipti Trivedi1, Samuel Y S Lee, Marie-Anne Brundler

  • 1Birmingham Children's Hospital, Steelhouse Lane, United Kingdom.

Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
|July 31, 2013
PubMed

Insights

Proteus syndrome, a mosaic overgrowth disorder, can affect eye muscles. A case study details successful surgical removal of a fibrous tumor in the superior oblique tendon, resolving vertical strabismus.

Area of Science:

  • Genetics
  • Ophthalmology
  • Pathology

Background:

  • Proteus syndrome is a rare mosaic disorder characterized by postnatal overgrowth, often linked to somatic AKT1 gene mutations.
  • Ocular manifestations like epibulbar tumors and strabismus are documented, but involvement of extraocular tendons remains unreported.

Observation:

  • A patient with Proteus syndrome presented with vertical strabismus.
  • Diagnostic imaging and subsequent examination revealed a fibrous tumor within the superior oblique tendon as the cause.

Findings:

  • This is the first reported case of a lesion affecting the extraocular tendons in Proteus syndrome.
  • Surgical excision of the fibrous tumor in the superior oblique tendon led to complete resolution of the patient's vertical strabismus.

Implications:

  • This case expands the known spectrum of ocular and tendinous involvement in Proteus syndrome.
  • Highlights the importance of considering tendinous structures in the evaluation of strabismus in patients with Proteus syndrome.
  • Successful surgical intervention demonstrates a viable treatment option for this specific complication.

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