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Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature

Hemophilic pseudotumor is a rare, yet dangerous complication of hemophilia. It has been reported previously at sites prone to recurrent trauma like long bones and pelvis. However, in the field of otorhinolaryngology, few cases are reported and therefore there is no established protocol for managemen...

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Detalles Bibliográficos
Autores principales: Farag, Nouran, Alwasiyah, Bashair, Marglani, Osama
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10630648/
https://www.ncbi.nlm.nih.gov/pubmed/37954105
http://dx.doi.org/10.1093/jscr/rjad586
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author Farag, Nouran
Alwasiyah, Bashair
Marglani, Osama
author_facet Farag, Nouran
Alwasiyah, Bashair
Marglani, Osama
author_sort Farag, Nouran
collection PubMed
description Hemophilic pseudotumor is a rare, yet dangerous complication of hemophilia. It has been reported previously at sites prone to recurrent trauma like long bones and pelvis. However, in the field of otorhinolaryngology, few cases are reported and therefore there is no established protocol for management. We hereby report a case of a 2-year-old boy, a known case of hemophilia A (factor VIII deficiency), who presented with recurrent epistaxis not responding to medical management. Imaging was done and revealed a heterogenous nasal mass compressing the left orbital wall, extending to the sphenoid sinus, and causing skull base erosion. The patient was successfully managed by evacuation and drainage of the pseudotumor via endoscopic endonasal approach and replacement of factor VII pre-and post-operatively. To our knowledge, this is the first case of nasal hemophilic pseudotumor managed by evacuation and drainage through an endoscopic endonasal approach, which was deemed successful.
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spelling pubmed-106306482023-11-11 Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature Farag, Nouran Alwasiyah, Bashair Marglani, Osama J Surg Case Rep Case Report Hemophilic pseudotumor is a rare, yet dangerous complication of hemophilia. It has been reported previously at sites prone to recurrent trauma like long bones and pelvis. However, in the field of otorhinolaryngology, few cases are reported and therefore there is no established protocol for management. We hereby report a case of a 2-year-old boy, a known case of hemophilia A (factor VIII deficiency), who presented with recurrent epistaxis not responding to medical management. Imaging was done and revealed a heterogenous nasal mass compressing the left orbital wall, extending to the sphenoid sinus, and causing skull base erosion. The patient was successfully managed by evacuation and drainage of the pseudotumor via endoscopic endonasal approach and replacement of factor VII pre-and post-operatively. To our knowledge, this is the first case of nasal hemophilic pseudotumor managed by evacuation and drainage through an endoscopic endonasal approach, which was deemed successful. Oxford University Press 2023-11-03 /pmc/articles/PMC10630648/ /pubmed/37954105 http://dx.doi.org/10.1093/jscr/rjad586 Text en Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2023. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Farag, Nouran
Alwasiyah, Bashair
Marglani, Osama
Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
title Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
title_full Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
title_fullStr Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
title_full_unstemmed Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
title_short Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
title_sort nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10630648/
https://www.ncbi.nlm.nih.gov/pubmed/37954105
http://dx.doi.org/10.1093/jscr/rjad586
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