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Wydanie 4/2011
str. 156

Objawy okulistyczne w zespole Parry’ego–Romberga

Ocular Symptoms in Parry–Romberg Syndrome

Ewa Langwińska-Wośko¹, Agnieszka Radomska², Agnieszka Kucewicz-Szulborska², Piotr Skopiński^1,3

¹ Katedra i Klinika Okulistyki II Wydziału Lekarskiego Warszawskiego Uniwersytetu Medycznego
  Kierownik: prof. dr hab. n. med. Jerzy Szaflik
² Zakład Ortodoncji Instytutu Stomatologii Warszawskiego Uniwersytetu Medycznego
  Kierownik: dr n. med. Barbara Siemińska-Piekarczyk
³ Katedra i Zakład Histologii i Embriologii Instytutu Biostruktury Warszawskiego Uniwersytetu Medycznego
  Kierownik: prof. dr hab. n. med. Jacek Malejczyk

Summary: Background: The progressive hemifacial atrophy, known also as the Parry-Romberg syndrome, is characterised by unilateral, slowly progressive atrophy of the skin, underlying subcutaneous tissue, muscles and bones. The craniofacial growth results in facial asymmetry. Ophthalmic involvement is common, with progressive enophthalmos as a frequent finding. This study presents a case report of 15-year-old patient with Parry-Romberg syndrome. Based on the literature, authors also present ocular findings in progressive hemifacial atrophy.
Material and Methods: A 15-year-old female patient with Parry-Romberg syndrome underwent full clinical examination. X-ray of the skull, visual field, ultrasound biometry and echography, OCT, fluorescein angiography and visual evoked potentials were also performed.
Results: Clinical observation revealed classic hemifacial atrophy with enophthalmos. In the presented case of Parry-Romberg syndrome non significant association to ophthalmological involvement was found. The ultrasound biometry and echography, OCT, fluorescein angiography and visual evoked potentials revealed no abnormalities.
Conclusions: The patients with Parry-Romberg syndrome need interdisciplinary investigation and complex treatment: orthodontic treatment, maxillofacial and plastic surgery. The therapeutic goal is to improve aesthetics and function. The important role for ophthalmologist is to exclude severe complications such as papilloedema, neuroretinitis or retinal vasculitis, and to treat keratopathy or strabismus.

Słowa kluczowe: zespół Parry’ego–Romberga, połowiczy zanik twarzy, wzrost twarzoczaszki, analiza cefalometryczna, diagnostyka okulistyczna, wzrokowe potencjały wywołane.

Keywords: Parry–Romberg syndrome, progressive hemifacial atrophy, craniofacial growth, cephalometric analysis, ophthalmic examination, visual evoked potentials.

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