Peripheral Arteriovenous Malformations-A Case Series

Abstract

Context: Arteriovenous malformations (AVMs) are aggressive vascular malformations that often result in significant morbidity. Patients may present to a dermatologist due to associated skin changes. Early diagnosis is important as treatment is available to halt their progression toward irreversible destruction of adjacent tissues.

Aims: To study the clinical profile of peripheral AVMs presenting to the dermatologist and to provide a diagnostic algorithm.

Settings and design: A retrospective study of patients of all age groups with peripheral AVMs who presented to the Department of Dermatology at a tertiary care hospital in India was performed. Syndromic forms were also included.

Subjects and methods: We conducted a search of patients with peripheral AVMs, which were seen over a period of 51 months, i.e., from July 2014 to September 2018, from electronic medical records and reviewed their clinical details.

Statistical analysis used: Descriptive statistics such as frequency, mean, and median were computed.

Results: We report a series of 13 patients with peripheral AVMs, which constituted 6.7% (13/193) of all vascular malformations during this period. Of these, 8.3% (1/12) belonged to Schobinger's stage 1, 41.7% (5/12) to stage 2, 50% (6/12) to stage 3, and one with subcutaneous involvement devoid of cutaneous changes. The most common location was the extremities, which was seen in 53.8% (7/13). Syndromic association was present in 46.2% (6/13). Management included embolization, surgery, and medical treatment.

Conclusions: The proportion of peripheral AVMs out of all vascular malformations was similar to reported studies. The extremities were more frequently involved as compared to the head and neck. The diagnostic algorithm provided will help us to optimize investigations and direct early management.

Keywords: Angiogram; embolization; peripheral arteriovenous malformations; syndromic.

Figure 1

AVM of the right ring finger showing macrodactyly, purplish hue with angiokeratoma like papules and crusted ulcers at the distal phalange

Figure 2

WB syndrome showing pulsatile, bluish tinged tortuous vessels on the left side of the forehead and frontal scalp, and ill-defined swelling of the left cheek

Figure 3

CM-AVM syndrome showing (a) macrodactyly of the right middle and ring fingers with overlying capillary malformation extending onto the palm; (b) capillary malformations on the lumbar areas

Figure 4

Selective angiogram showing hypertrophy of digital arteries to the right middle finger with a nidus at the distal phalange in the CM-AVM syndrome

Figure 5

Axial pulse Doppler showing arterialization of the right middle finger digital vein due to shunting in the CM-AVM syndrome

Figure 6

T2 STIR images of the right hand showing flow voids in AVM of the right ring finger

Figure 7

Diagnostic algorithm for peripheral AVMs