Rare case of a Congenital Arteriovenous malformation (Park Weber angiodysplasia) around the elbow with median nerve compression

摘要

A 28 yr old male presented to us with pain and sudden increase in preexisting swelling around the left elbow of one year duration. Examination revealed an arteriovenous malformation with signs of median nerve compression and a fixed flexion deformity of the elbow. Xray revealed erosion of the bone in the upper and the lower end humerus . CT/MR Angiography proved invaluable in delineating the extent of the bony and soft tissue changes. Trauma is one of the factors implicated in the sudden increase of a quiescent AV malformation as in this case.This form of presentation as a high flow congenital arteriovenous malformation around the elbow with osseous changes and nerve compression, we believe is a rare presentation. Introduction Arteriovenous Malformations (AVMs) belong to a group of disorders known as vascular malformations. AVMs are defects of the circulatory system that generally arise during embryonic or fetal development or soon after birth. They consist of masses of abnormal blood vessels. Peripheral arteriovenous (AV) malformations may present with a plethora of clinical symptoms such as paradoxical emboli, severe hypertension, nerve palsies or pain syndromes. Hemodynamically compromising lesions of the limbs are rare and involve high-flow AV fistulae with marked arteriovenous shunting1.Lesions may be located superficially with only minimal arterio-venous shunting or more deeply with significant, high flow, AV shunting.AVMs consist of a blood vessel “nidus” (nest) through which arteries connect directly to veins, instead of through the elaborate collection of very small vessels called capillaries.Complete excision of the malformation is required because subtotal resections result in recurrence. Preoperative embolization may be used, followed shortly with operative resection. Simple ligation or embolization is contraindicated for arteriovascular anomalies because rapid collateral arterial flow develops. Case Report Presenting complaints -A 28 yr old male c/o pain and increase of swelling around the left elbow. Swelling noticed by the parents since the age of 1 year, slowly progressed up to the age of 15 yrs,remained static until a trivial trauma 6 mths back following which a rapid increase in the size of the swelling was noted. The patient was operated for the same complaints at the age of 15 yrs, operative details not known.On examination- Diffuse circumferential swelling from the insertion of deltoid to upper third forearm, firm to cystic in consistencySkin – shiny, multiple engorged veins with thickened walls. Temp. raised ,erythema of the distal limb present. Operated scar over the medial armLimb lengthening of 5 cms noted with significant increase in girthPalpable and an audible continous machinery murmur. Nicoladinus and Branham sign positive.Gross restriction of shoulder abduction(60 deg),flexion(40 deg) and external rotation(20 deg).Fixed flexion deformity of the elbow of 30 deg, gross restriction of forearm pronation(30 deg).Paraesthesia in the area of distribution of the median nerve noted, motor system normal.. Schobinger classification- Features of both stage 2 and stage 3(persistent pain).