Case Report: Massive epistaxis from juvenile angiofibroma in an adolescent with severe haemophilia A.

Jose Florencio F Lapeña,Olivia Agnes D Mejia

doi:10.12688/f1000research.20147.2

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.

Highlights

Epistaxis may be profuse in individuals with normal bleeding parameters, but in an individual with haemophilia, it may be lifethreatening
Any further responses from the reviewers can be found at the end of the article Introduction Juvenile angiofibroma (JA) is a benign vascular tumour accounting for 0.5% of all head and neck neoplasms[1]
The preliminary diagnosis of JA was confirmed by biopsy at a different medical centre, where massive haemorrhage jeopardized the patient’s life

Summary

Discussion

There is only one previous case of JA and concomitant haemophilia in the English literature, twice reported by Ozturk et al in 19993 and by Celiker et al in 20044. Confirmed by biopsy at a different medical centre, where massive haemorrhage jeopardized the patient’s life On referral to their institution, preoperative embolization, surgical excision, and adequate Factor VIII replacement saved the patient[4]. While recent-onset of bleeding from “mucous membranes in the mouth, gums, nose, and genitourinary tract” was serious, massive bleeding with “neck/throat” involvement was “life-threatening.” This degree of epistaxis should not have been expected in patients with haemophilia A alone, where major bleeding from these areas only occurs 5–10% of the time[5]. Current haemophilia guidelines[5] advise otolaryngologist referral only for “persistent or recurrent” epistaxis, but the emphasis in this recommendation is for control of bleeding only and not to investigate a different underlying cause such as JA. Consent Written informed consent for publication of his clinical details and clinical images was obtained from the patient

National ENT Trainee Research Network

Findings