Published online Dec 16, 2017. doi: 10.12998/wjcc.v5.i12.419
Peer-review started: March 2, 2017
First decision: May 23, 2017
Revised: September 10, 2017
Accepted: November 22, 2017
Article in press: November 22, 2017
Published online: December 16, 2017
Processing time: 284 Days and 3.9 Hours
Neurofibromatosis type 1 is a congenital condition affecting neurons and connective tissue integrity including vasculature. On extremely rare occasions these patients present with venous aneurysms affecting the internal jugular vein. If they become large enough there presents a risk of rupture, thrombosis, embolization or compression of adjacent structures. In these circumstances, or when the patient becomes symptomatic, surgical exploration is warranted. We present a case of one of the largest aneurysms in the literature and one of only five associated with Neurofibromatosis type 1. A 63-year-old female who initially presented for a Hinchey III diverticulitis requiring laparotomy developed an incidentally discovered left neck swelling prior to discharge. After nonspecific clinical exam findings, imaging identified a thrombosed internal jugular vein aneurysm. Due to the risks associated with the particularly large size of our patient’s aneurysm, our patient underwent surgical exploration with ligation and excision. Although several techniques have been reported, for similar presentations, we recommend this technique.
Core tip: Neurofibromatosis type 1 is a congenital condition occasionally affecting vascular connective tissue integrity. On extremely rare occasions these patients present with internal jugular venous aneurysms. We present a case of the successful ligation and excision of one of the largest internal jugular vein aneurysms in the literature and one of only five associated with Neurofibromatosis type 1.