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[Proteus syndrome in the practice of an otorhinolaryngologist: a clinical case]

I V Stozhkova1, E V Pchelenok1, S Ya Kosyakov1

  • 1Russian Medical Academy for Continuing Professional Education of the Ministry of Health of Russia, Department of Otorhinolaryngology, Moscow, Russia.

Vestnik Otorinolaringologii
|June 2, 2020
PubMed

Insights

This case study details a unique patient with chronic otitis media and Proteus syndrome, highlighting extensive surgical history for vascular malformations and ear infections. The observation is considered rare in medical literature.

Area of Science:

  • Medicine
  • Genetics
  • Otolaryngology

Background:

  • Proteus syndrome is a rare congenital disorder characterized by asymmetric, segmental overgrowth of tissues.
  • Chronic otitis media is a persistent inflammation of the middle ear, often requiring surgical intervention.

Observation:

  • An 18-year-old patient with Proteus syndrome presented with chronic left-sided otitis media.
  • The patient had a history of multiple surgeries for lymphangiomas, hemolymphangiomas, and angiokeratoma.
  • Multiple surgical procedures were also performed for the left ear's chronic infection.

Findings:

  • The co-occurrence of Proteus syndrome and chronic otitis media in this patient is exceptionally rare.
  • The extensive surgical history underscores the complexity of managing associated conditions in Proteus syndrome.

Implications:

  • This case highlights the importance of comprehensive management for patients with Proteus syndrome.
  • Further research may elucidate potential links between Proteus syndrome and chronic ear infections.
  • Such rare observations contribute valuable data to understanding complex genetic disorders and their clinical manifestations.
Keywords:
Proteus syndromecholesteatomaovergrowth

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