Branchial fstula in children with branchio-oto-renal syndrome (Melnick-Fraser syndrome): a clinical case report
https://doi.org/10.33925/1683-3031-2022-22-2-152-157
Abstract
Relevance. Branchio-oto-renal syndrome (also known as Melnick-Fraser syndrome) is a rare syndrome characterized by hearing loss, renal malformations and branchial cyst (fistula) association. The article describes a clinical case of a boy with an atypical clinical picture of branchio-oto-renal syndrome.
Material and methods. The patient was admitted to our clinic with acute inflammation, complaints of hearing loss and a fistulous tract in the right retromandibular region.
Results. After the acute inflammation resolution and necessary investigations, the patient underwent a fistulectomy for diagnosis verification. We analyzed the patient routing from the first presentation to the patient's GP to the final diagnosis in our clinic. Based on this clinical case result analysis, we offered optimal diagnosis and treatment tactics for patients with Melnick-Fraser syndrome.
Conclusion. Correct branchio-oto-renal syndrome diagnosis requires careful history taking, clinical examination and multidisciplinary team involvement. This approach will allow the patient to receive a full range of treatments with quality-of-life preservation.
About the Authors
S. V. YakovlevRussian Federation
Sergey V. Yakovlev, DDS, PhD, Associate Professor, Department of Pediatric Maxillofacial Surgery
Moscow
T. A. Bakshi
Russian Federation
Tatiana A. Bakshi, DDS, resident, Department of Pediatric Maxillofacial Surgery
Moscow
A. A. Beglaryan
Russian Federation
Alina A. Beglaryan, DDS, resident, Department of Pediatric Maxillofacial Surgery
Moscow
O. Z. Topolnitskiy
Russian Federation
Orest Z. Topolnitsky, DDS, PhD, DSc, Professor, Head of the Department of Pediatric Maxillofacial Surgery
Moscow
A. V. Zhuravleva
Russian Federation
Anna V. Zhuravleva, MD, Assistant Professor of the Department of Pathological Anatomy
Moscow
D. A. Lezhnev
Russian Federation
lezhnev Dmitry A, MD, PhD, DSc, Professor, Head of the Department of Radiology
Moscow
References
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2. Mironovich OL, Bliznetz EA, Markova TG, Alekseeva NN, Golybeva TI, Ryzhkova OP, et al. Molecular Genetic Causes and Clinical Description of Branchio-Oto-Renal Syndrome. Russian Journal of Genetics. 2019; 55(5):583-592. doi: 10.1134/S0016675819050114
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Review
For citations:
Yakovlev S.V., Bakshi T.A., Beglaryan A.A., Topolnitskiy O.Z., Zhuravleva A.V., Lezhnev D.A. Branchial fstula in children with branchio-oto-renal syndrome (Melnick-Fraser syndrome): a clinical case report. Pediatric dentistry and dental prophylaxis. 2022;22(2):152-157. (In Russ.) https://doi.org/10.33925/1683-3031-2022-22-2-152-157