title: A rare case of a spontaneous neck hematoma in a patient with type 1 neurofibromatosis.
abstract: Neurofibromatosis type 1 (NF-1) is a genetic disorder that affects one in 3000 individuals. Although NF-1 notably involves nerves and connective tissue, vascular involvement in large series is estimated to range from 0.4% to 6.4%. Jugular vein involvement in these patients is rare. Spontaneous neck hematomas and hemorrhages are also unusual. We present a case of a NF-1 patient with a spontaneous neck hematoma with possible leakage from the left internal jugular vein, presenting as a lateral neck mass. The fragility of the vein wall and the surrounding tissue led patient to a severe intraoperative bleeding. Pathological examination revealed degenerated neurofibroma which was in contact with or infiltrated the vein wall. ENT and other clinicians should be aware of this potentially fatal entity considering that it may present as a lateral neck mass.
authors.affiliation: Aristotle University of Thessaloniki, AHEPA University Hospital, A Department of Otorhinolaryngology - Head and Neck Surgery, 1 Stilponos Kyriakidi St, 54636 Thessaloniki, Greece.
authors.forename: Stamatia
authors.initials: S
authors.lastname: Dova
authors.affiliation: Aristotle University of Thessaloniki, AHEPA University Hospital, A Propedeutic Surgery Department, Vascular Surgery Unit, 1 Stilponos Kyriakidi St, 54636 Thessaloniki, Greece.
authors.forename: Kyriakos
authors.initials: K
authors.lastname: Ktenidis
authors.affiliation: Aristotle University of Thessaloniki, AHEPA University Hospital, A Department of Otorhinolaryngology - Head and Neck Surgery, 1 Stilponos Kyriakidi St, 54636 Thessaloniki, Greece.
authors.forename: Petros
authors.initials: P
authors.lastname: Karkos
authors.affiliation: Aristotle University of Thessaloniki, AHEPA University Hospital, A Department of Otorhinolaryngology - Head and Neck Surgery, 1 Stilponos Kyriakidi St, 54636 Thessaloniki, Greece.
authors.forename: Sarantis
authors.initials: S
authors.lastname: Blioskas
authors.affiliation: Aristotle University of Thessaloniki, AHEPA University Hospital, A Department of Otorhinolaryngology - Head and Neck Surgery, 1 Stilponos Kyriakidi St, 54636 Thessaloniki, Greece.
authors.forename: Georgios
authors.initials: G
authors.lastname: Psillas
authors.affiliation: Aristotle University of Thessaloniki, Medical School, Pathology Department, 54124 Thessaloniki, Greece.
authors.forename: Alexandros
authors.initials: A
authors.lastname: Iliadis
authors.affiliation: Aristotle University of Thessaloniki, AHEPA University Hospital, A Department of Otorhinolaryngology - Head and Neck Surgery, 1 Stilponos Kyriakidi St, 54636 Thessaloniki, Greece. Electronic address: kmarkou@med.auth.gr.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Markou
mesh_terms: D015901:Angiography, Digital Subtraction; D000072226:Computed Tomography Angiography; D006258:Head and Neck Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006406:Hematoma / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D007601:Jugular Veins / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D009333:Neck; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed; D020246:Venous Thrombosis / Q000000981:diagnostic imaging / Q000209:etiology*
keywords: Hematoma; Jugular; Neurofibromatosis