Parkes-Weber syndrome in the emergency department

Patel, R; Durant, Edward; Freed, Robert

doi:10.1136/bcr-2021-241649

Cited by 5 publications

(2 citation statements)

References 2 publications

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“…Mild cases commonly exhibit limb length discrepancies, increased skin temperature, dermatitis, skin discoloration, and swelling of the affected limb. Severe forms may involve additional symptoms such as pain, warmth, and pulsations in the affected limb [ 12 ]. Complications of the syndrome can include bleeding, skin infections, thromboses, and heart failure [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Pediatric Limb Asymmetry: A Unique Presentation of Angioosteohypertrophic Syndrome

Salhi,

N'joumi,

Banana

et al. 2024

Cureus

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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.

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Section: Discussionmentioning

confidence: 99%

Pediatric Limb Asymmetry: A Unique Presentation of Angioosteohypertrophic Syndrome

Salhi,

N'joumi,

Banana

et al. 2024

Cureus

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“…PWS has features of KTS, but deep malformations in PWS are arteriovenous, whereas in KTS, they are venous. Genetic analysis provides different results: PIK3CA mutation is indicative for KTS and RASAI for PWS [ 2 , 56 ].…”

Section: Discussionmentioning

confidence: 99%

Klippel–Trenaunay Syndrome, Segmental/Focal Overgrowth Malformations: A Review

Pavone,

Marino,

Cacciaguerra

et al. 2023

Children

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Klippel–Trenaunay syndrome is an uncommon, infrequent, congenital disorder characterized by a triad of capillary malformation, varicosities, and tissue and bone hypertrophy. The presence of two of these three signs is enough to obtain the diagnosis. Capillary malformations are usually present at birth, whereas venous varicosities and limb hypertrophy become more evident later. The syndrome has usually a benign course, but serious complications involving various organs, such as gastrointestinal and genitourinary organs, as well as the central nervous system, may be observed. Recently, Klippel–Trenaunay syndrome has been included in the group of PIK3CA-related overgrowth spectrum (PROS) disorders. In terms of this disorder, new results in etiopathogenesis and in modalities of treatment have been advanced. We report here a review of the recent genetic findings, the main clinical characteristics and related severe complications, differential diagnoses with a similar disorder, and the management of patients with this complex and uncommon syndrome.

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