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Neurofibromatosis type 1 (NF 1) or von Recklinghausen's disease mainly involves the peripheral nervous system. Vascular involvement of this disease is a recognised feature involving primarily the arterial tree. Venous invasion is rare, but may have serious consequences. A case of an internal jugular vein dissection secondary to neurofibroma invasion is presented. A medline search did not reveal any previous reported cases of this type.
Key Words: Neurofibromatosis Type 1 ; Jugular Veins; Neck Dissection
Introduction
Neurofibromatosis type 1 (NFl) or von Recklinghausen's disease is an autosomal dominant disorder that mainly involves the peripheral nervous system. The most frequent clinical manifestations include café au lait spots, freckling, peripheral neurofibromas and Lisch nodules. Less frequent disease features include short stature, macrocephaly, central nervous system tumours, plexiform neurofibromas and intellectual handicap. '
Vascular neurofibromatosis is a recognised form of the disease, the pathology of which was first described by Reubi in 1945.2 However, the majority of the reported lesions have been in the arterial tree involving the abdominal aorta and its branches, especially the renal and coeliac arteries, but also the mesenteric, iliac, subclavian, pulmonary and cervicocerebral arteries.3-6
This case study describes a patient with NFl who presented with an acute neck mass secondary to a dissection of his left internal jugular vein (IJV). Parts of the IJV wall were completely replaced by neurofibroma. In contrast to arterial involvement with neurofibromatosis, the involvement of large veins is extremely rare. In the literature, there has only been one case of neurofibroma involving the IJV causing an aneurysm. However, there have been no previous reports of UV dissection secondary to neurofibromatosis.
Case Report
A 43 year old male farmer with NFl presented with an acute onset of a left sided neck mass. The mass appeared over 24 hours and was painful. There was no history of trauma and there were no vocal, swallowing or breathing problems. There were no neurological symptoms and no localising neurological features. He was a non smoker. His past medical history was associated with his NFl. Over...