{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T09:47:15Z","timestamp":1767174435297,"version":"build-2238731810"},"update-to":[{"DOI":"10.12688\/f1000research.20147.1","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2019,10,4]],"date-time":"2019-10-04T00:00:00Z","timestamp":1570147200000}}],"reference-count":6,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2019,10,4]],"date-time":"2019-10-04T00:00:00Z","timestamp":1570147200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"Epistaxis may be profuse in individuals with normal bleeding parameters, but in an individual with haemophilia, it may be life-threatening. It is even more dangerous when epistaxis is caused by an undetected concomitant juvenile angiofibroma, and only one such case has been reported in the English literature. We report another case, of an 18-year-old Filipino adolescent with severe haemophilia A who was referred for repeated massive epistaxis. The epistaxis had been attributed to his haemophilia and managed with nasal packing, multiple blood transfusions and Factor VIII administration. After two years of unsuccessful management, nasal endoscopy was performed for the first time, revealing an intranasal mass. Imaging showed a right intranasal vascular tumour supplied mainly by the right sphenopalatine artery. He subsequently underwent preoperative embolization and endoscopic excision of the tumour with Factor VIII transfused pre-, intra-, and post-operatively, and recombinant Factor VII added post-operatively. Final histopathology was consistent with juvenile angiofibroma. There has been no nasal obstruction or recurrence of epistaxis seven years since the surgery. Clinicians should be more meticulous in assessing epistaxis in any patient with a bleeding disorder and investigate more subtle symptoms such as nasal obstruction. Verification of the source by direct visualization and ancillary diagnostic techniques (such as imaging) when indicated should be the standard of care for all patients presenting with epistaxis, whether or not a concomitant bleeding disorder exists. A high index of suspicion for juvenile angiofibroma should be maintained in adolescent males with epistaxis and nasal obstruction.<\/ns4:p>","DOI":"10.12688\/f1000research.20147.2","type":"journal-article","created":{"date-parts":[[2019,10,4]],"date-time":"2019-10-04T07:10:05Z","timestamp":1570173005000},"page":"1593","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Massive epistaxis from juvenile angiofibroma in an adolescent with severe haemophilia A"],"prefix":"10.12688","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5794-1878","authenticated-orcid":false,"given":"Jose Florencio F.","family":"Lape\u00f1a","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6180-2950","authenticated-orcid":false,"given":"Olivia Agnes D.","family":"Mejia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2019,10,4]]},"reference":[{"key":"ref-1","first-page":"1-143","article-title":"European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base.","volume":"22","author":"V Lund","year":"2010","journal-title":"Rhinol Suppl."},{"key":"ref-2","doi-asserted-by":"publisher","first-page":"412545","DOI":"10.1155\/2012\/412545","article-title":"Juvenile angiofibroma: evolution of management.","volume":"2012","author":"P Nicolai","year":"2012","journal-title":"Int J Pediatr."},{"key":"ref-3","doi-asserted-by":"publisher","first-page":"207-8","DOI":"10.1046\/j.1365-2516.1999.00296.x","article-title":"Nasopharyngeal angiofibroma in a patient with haemophilia A: a bleeding tumour in a bleeding-prone patient.","volume":"5","author":"M Ozt\u00fcrk","year":"1999","journal-title":"Haemophilia."},{"key":"ref-4","doi-asserted-by":"publisher","first-page":"819","DOI":"10.1053\/j.jvca.2004.08.030","article-title":"Anesthesia in a patient with nasopharyngeal angiofibroma and hemophilia A.","volume":"18","author":"V Celiker","year":"2004","journal-title":"J Cardiothorac Vasc Anesth."},{"key":"ref-5","doi-asserted-by":"publisher","first-page":"e1-47","DOI":"10.1111\/j.1365-2516.2012.02909.x","article-title":"Guidelines for the management of hemophilia.","volume":"19","author":"A Srivastava","year":"2013","journal-title":"Haemophilia."},{"key":"ref-6","doi-asserted-by":"publisher","first-page":"1142-1156","DOI":"10.1017\/S0022215117002018","article-title":"The British Rhinological Society multidisciplinary consensus recommendations on the hospital management of epistaxis.","volume":"131","year":"2017","journal-title":"J Laryngol Otol."}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,14]],"date-time":"2021-10-14T08:28:48Z","timestamp":1634200128000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,4]]},"references-count":6,"URL":"https:\/\/doi.org\/10.12688\/f1000research.20147.2","relation":{},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,4]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.22132.r53496, Alberto Maria Saibene, Department of Otolaryngology, San Paolo Hospital, University of Milan, Milan, Italy, 09 Sep 2019, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v1#referee-response-53496","order":0,"name":"referee-response-53496","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"Jose Florencio Lapeña<\/b>; \nPosted: 02 Oct 2019<\/i>; We thank the reviewer for his excellent review and valuable comments and recommendations: 1. "\nFirst of all, it might be worth mentioning that in good rhinologic practice, performing sinonasal tumors biopsies without adequate imaging can result to harmful or fatal incidents. Had the clinicians decided to perform a biopsy in an outpatient setting, a massive epistaxis could have led to serious complications for this patient, without helping further clinical decisions. This is the case of the - correctly cited - case report already published by Ozturk.<\/i>"Yes, indeed. A biopsy in the case of our patient would have been catastrophic (and we have witnessed this happen to patients of other unsuspecting physicians). Any nasal mass suspicious for angiofibroma should not be manipulated (unless in the operating theatre under double set-up). However, we felt that mentioning this important management caveat would detract from the main message of initial diagnosis necessitating visualising the bleeding source (directed toward general practitioners, primary care providers, family physicians, and paediatricians), and opted to reiterate the point in our response to the review instead.2.  "\nSecondly, while it is true that haemophilia guidelines do not advise routine evaluation for epistaxis, on the other hand epistaxis guidelines (1) do recommend for a thorough evaluation of the patient in order to identify the bleeding source in all case, starting with anterior rhinoscopy and escalating to nasal endoscopy whenever the source of bleeding cannot be easily identified. Therefore, it is worth mentioning that correct management of all epistaxis cases required identification of the bleeding source<\/i>."We have added the statement "\nOn the other hand, epistaxis guidelines(6) do recommend 'anterior rhinoscopy with headlight following nasal decongestion' escalating to 'rigid endoscopy or microscopy \u2026 where anterior rhinoscopy fails to identify a bleeding point.' <\/i>" to the Discussion, for which the additional reference (6) provided by the reviewer was cited:National ENT Trainee Research Network. The British Rhinological Society multidisciplinary consensus recommendations on the hospital management of epistaxis. J Laryngol Otol. 2017;131(12):1142-1156. 10.1017\/S0022215117002018.3.  "\nLast, while the CT scan the authors provided allows a good depiction of the clinical picture, it would be interesting to see whether the CT scans showed enlargement of the sphenopalatine foramen. While such enlargement is not constant in all JA patients, an enlargement >3mm in presence of a unilateral sinonasal mass can point the diagnosis towards JA."<\/i>We totally agree that the educational value of the article can be enhanced by mentioning that "\nin the presence of a unilateral sinonasal mass<\/i>," such an enlargement of the sphenopalatine foramen "\ncan point to the diagnosis of JA<\/i>." However (and in relation to the first point above), we opted to maintain the primary focus of our discussion and perhaps encourage our non-otolaryngologist colleagues to consider urgent referrals to ENT surgeons following initial diagnosis in such cases.","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2#referee-comment-4944","order":1,"name":"referee-comment-4944","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.22132.r53498, J. Paul Moxham, Division\u00a0of\u00a0Otolaryngology-Head and Neck Surgery, University of British Columbia, Vancouver, BC, Canada, 23 Sep 2019, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v1#referee-response-53498","order":2,"name":"referee-response-53498","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"Jose Florencio Lapeña<\/b>; \nPosted: 02 Oct 2019<\/i>; We thank the reviewer for his clear and concise review, recapitulating the main theme and "take-home" message of our case report "\nthat just because someone has a bleeding disorder does not mean they cannot also have a rare vascular tumour<\/i>."","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2#referee-comment-4945","order":3,"name":"referee-comment-4945","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.22132.r53497, Robert G. Berkowitz, Department of Otolaryngology, Royal Children's Hospital,\u00a0Melbourne, Parkville, Vic, Australia, 24 Sep 2019, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v1#referee-response-53497","order":4,"name":"referee-response-53497","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"Jose Florencio Lapeña<\/b>; \nPosted: 02 Oct 2019<\/i>; We thank the reviewer for his kind review and for raising minor (but important) points for consideration:1. \n"It is implied, but not actually stated, that during the two year period where the JA was overlooked, the patient's haemophilia was poorly controlled (which presumably contributed to overlooking the diagnosis of JA). Is this the case?"<\/i>Yes, "\nduring the two year period where the JA was overlooked, the patient's haemophilia was poorly controlled (which presumably contributed to  overlooking the diagnosis of JA)<\/i>." We were hoping the first paragraph conveyed a sense of this poor control without being overly dramatic. As mentioned in the Case presentation, this can be attributed to inadequate factor VIII replacement therapy ("\nseldom available due to shortage of supply and cost<\/i>").  Indeed, "\nthis makes the case even more interesting, and highlights that not only when there is a known coagulopathy present, and even when it is not being adequately treated, a possible intra-nasal cause should still be considered.<\/i>" 2. \n"If treatment proceeded uneventfully, why did the patient require hospitalisation for a period of 2 months?"<\/i>I hope we did not give the impression that "\ntreatment proceeded uneventfully<\/i>." Our "\npatient require(d) hospitalisation for a period of 2 months<\/i>" because of a very stormy preoperative course wherein "\nhe suffered from hypovolemic shock several times due to difficulty in acquiring blood<\/i>" and blood products, and "\nhis condition was compounded by development of Factor VIII antibodies.<\/i>" He also "\nreceived 39,500 units of commercially available pFVIII, 24 mg of rFVIIa, 22 units of packed red blood cells (PRBC), 301 units of cryoprecipitate, 1 unit of whole blood and 3 units of fresh frozen plasma (FFP)<\/i>."  We have revised the Case presentation by adding the statement "\nPost-operative recovery was uneventful and the patient was discharged within a week of surgery (after two months in hospital)<\/i>." to clarify that the prolonged hospitalisation was due to the stormy pre-operative course.","URL":"https:\/\/f1000research.com\/articles\/8-1593\/v2#referee-comment-4943","order":5,"name":"referee-comment-4943","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The authors applied for reimbursement of Article Processing Charges by the University of the Philippines Manila. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.","order":6,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.","order":0,"name":"copyright-info","label":"Copyright"}]}}