# Pediatric Limb Asymmetry: A Unique Presentation of Angioosteohypertrophic Syndrome

## TL;DR

This paper reports a rare case of angioosteohypertrophic syndrome in a child, marked by limb asymmetry and vascular abnormalities.

## Contribution

The study presents the first documented case of this syndrome at CHU Med VI Oujda, highlighting its unique pediatric presentation.

## Key findings

- The syndrome manifested in a seven-year-old with right upper limb asymmetry, thermal disparity, and a cutaneous nevus.
- Arteriography confirmed an arteriovenous fistula, supporting the diagnosis of angioosteohypertrophic syndrome.
- The case emphasizes the importance of early detection and symptomatic management for this rare condition.

## Abstract

Parks-Weber syndrome (PWS), also known as Klippel-Trenaunay-Weber syndrome, is a rare congenital bone vascular syndrome first described in 1900. It is characterized by arteriovenous malformations in a limb, leading to disproportionate limb growth and potential heart failure. Unlike Klippel-Trenaunay syndrome, PWS manifests arteriovenous malformations with abnormal connections between the arteries and veins of the affected limb. The management of this syndrome, similar to that of Klippel-Trenaunay syndrome, relies mainly on symptomatic treatment.

We report the first case of angioosteohypertrophic syndrome diagnosed at CHU Med VI Oujda, in a patient aged seven years and eight months. This syndrome manifested primarily in the right upper limb, characterized by asymmetry in both upper limbs, thermal disparity, a cutaneous nevus, and venous ectasia in the right arm. The diagnosis was further substantiated through arteriography, confirming the presence of an arteriovenous fistula.