Intermittent claudication due to right common femoral artery compression by a solitary neurofibroma

Abstract

Common femoral artery narrowing secondary to compression by a solitary neurofibroma arising from the adjacent femoral nerve is an interesting problem that does provide a previously unreported differential diagnosis for an IC in children. Intermittent claudication (IC), a characteristic pain in the calf muscles that appears on exercise and is relieved by rest, is a frequent symptom in the adult age group; however, it is unusual in children [3,6]. Although commonly arising from diseases which have arterial involvement during the course, claudication-like symptoms can also be produced by non-arterial conditions. These are rare but can present a diagnostic dilemma [6]. Neurofibromas (NFs) may occur as part of neurofibromatosis or as a solitary tumour. They usually arise from small nerves, grow slowly as painless nodules and produce few signs and symptoms. Such tumours are well circumscribed and not encapsulated. The clinical features of these tumours are non-specific and are generally due to displacement of the adjacent structures by a tumour mass as well as increasing functional disorders or paresthesias as a result of nerve atrophy as the tumour displaces nerve tissue. As a result, clinical presentation of NFs depends mainly upon the site of their origin [1]. A 10-year-old girl with slowly progressive IC in her right calf was referred to our vascular surgical service in 1996. The first episode of IC had occurred about 1 year previously. At admission, her pain-free walking distance was 20 m. On physical examination, she had a full set of peripheral pulses at rest. The temperature on the right forefoot was a little lower than the left one, but they showed no difference in hair or skin structure or atrophy of muscles or nails. Examination of the other systems was normal. There were no signs of NF1 such as cafe au lait spots, Lisch nodules or another NF. Family history was also negative for NF1. Ankle branchial index at rest was 0.6 and after exercise was 0.4 on the right. At rest, it was 1.07 on the left. A distal aortogram with lower extremity runoff views was performed via a right femoral artery percutaneous access. This revealed a 2.5 cm stenotic segment narrowing about 70% of the lumen of the right common femoral artery (CFA) resulting in decreased superficial femoral arterial flow (Fig. 1). With these findings, adventitial cystic disease was suspected, and a right femoral exploration was performed. After dissection, the CFA was found to be enveloped anteriorly and on either side by an encapsulated solid mass behind the inguinal ligament. The CFA appeared markedly narrowed due to compression of this lesion. Lateral to the CFA, the main trunk of the femoral nerve was adherent to the dorso-lateral side of the mass. The encapsulated mass was separated cleanly from both the femoral nerve and the CFA releasing the arterial compression. Most significantly, the CFA itself showed no evidence of vascular occlusion or focal stenotic areas. It immediately re-expanded and normal arterial flow was confirmed by intraoperative angiography. Histopathology of the specimen revealed a benign solitary NF. The postoperative period was uneventful, and the ankle branchial index at rest was over 1 on the right. We monitored the patient for 6 years, and neither recurrence nor any evidence of NF1 was found. IC is well defined symptom in adults and the most common cause is peripheral arterial occlusive disease secondary to arteriosclerosis. Other arterial causes are very rare, including inflammatory disorders (Takayasu disease), adventitial cystic disease, popliteal entrapment syndrome, thromboemboli, irradiation injury, remote Z. Pulathan AE Y. K. Guven Department of Cardiovascular Surgery, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey