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Case Reports
. 2021 Nov 20:72:103036.
doi: 10.1016/j.amsu.2021.103036. eCollection 2021 Dec.

A case report of a severe form of cogan syndrome

Abdoul Salam Youssoufou Souley  1 , Moctar Issiakou  2 , Oumarou Sambou Khidrou Fadhoullahi  3 , Brah Souleymane  4 , Samir Mainassara Chékaraou  5 , Zeinabou Noura  4
Affiliations
Case Reports

A case report of a severe form of cogan syndrome

Abdoul Salam Youssoufou Souley et al. Ann Med Surg (Lond). .

Abstract

Introduction: the Cogan syndrome is a very rare, systemic disease that affects young adults. Very few cases are described in the literature. We report the case of a patient with a severe form of Cogan syndrome.

Case presentation: This is a young patient who presented with a painful left red eye and bilateral visual impairment evolving for 5 years with ENT signs such as right hypoacusis and vertigo. Clinical examination in this patient found bilateral hypertensive panuveitis, vertigo of peripheral origin and hypoacusis on the right. The patient is currently on corticosteroid therapy with stabilization of the lesions.

Discussion: This pathology is characterized by ocular and audio-vestibular involvement with sometimes other visceral manifestations. The etiopathogeny is not well known, the evolution is marked by the functional prognosis (visual and auditory) and the vital prognosis (aortic insufficiency). The treatment is essentially based on corticotherapy and the treatment of complications.

Conclusion: It is a very rare condition that should be considered when there are suggestive signs, because the evolution is severe without appropriate care. This underlines the importance of early management and the need for optimal follow-up to avoid the occurrence of complications that are disabling or even fatal.

Keywords: Goniosynechia; Optic atrophy; The cogan syndrome.

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Conflict of interest statement

The authors declare that there are no conflicts of interest.

Figures

Fig. 1
Fig. 1
The right eye: papillary pallor evoking an optic atrophy.
Fig. 2
Fig. 2
Disinhabited blood vessels temporally superior to the fundus.
Fig. 3
Fig. 3
The fluorescence angiography shows peripheral retinal ischemia in the left eye with retinal vasculitis.
Fig. 4
Fig. 4
Focal obliteration of the semicircular canals with a spiky appearance of their lumens. Appearance in favor of an ossifying labyrinthitis.
See this image and copyright information in PMC

References

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