Fraser syndrome: About A Case and Review of the Literature

Author(s): Laouali Laminou, Oumarou Habou, Magagi Amadou, Abba Kaka Yakoura Hadjia, Amza Abdou.

Purpose: The aim of this study was to describe the management of a case of Fraser syndrome at the National Hospital of Zinder in Niger and review of the literature.

Observation: This was a newborn at day 1 of life, female, from a first degree consanguineous marriage referred from the mother and child health center (CSME) to the ophthalmology department of the national hospital of Zinder for polymalformative syndrome. On admission, he had complete bilateral cryptophthalmia, nasal depression and polydactyly. Bilateral ocular examination noted on bidigital palpation the presence of a renal globular mass behind the skin tissue. The general physical examination noted an umbilical hernia and simple polydactyly and the otorhinolaryngological examination did not reveal any other abnormalities apart from nasal depression.

Bilateral aphakia was found on ocular ultrasound and the abdomino-pelvic ultrasound showed a uterine inversion with no abnormalities in the urinary tree. Surgical exploration revealed an opaque cornea in the right eye that prevented further examination of the anterior segment.

Conclusion: Frazer's syndrome is a malformative syndrome that poses serious management difficulties, particularly in facilities with limited resources such as ours. An antenatal diagnosis and a multidisciplinary management are a guarantee of a functional and cosmetic prognosis.

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