A Review on Cutaneous and Musculoskeletal Manifestations of CLOVES Syndrome

Durmaz, Emel Öztürk; Demircioğlu, Deniz; Dikmen, Pınar Yalınay; Alanay, Yasemin; Alanay, Ahmet; Demirkesen, Cüyan; Tokat, Fatma; Karaarslan, Ercan

doi:10.2147/ccid.s351637

Cited by 5 publications

(2 citation statements)

References 60 publications

(243 reference statements)

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“…Clinical differentiation between CLOVES and KTS consists of the absence of truncal involvement and the preferential location of the anomalies in limbs in KTS patients [ 54 ]. Moreover, high-flow atrio-ventricular malformations (AVM) and spinal/paraspinal AVM are features of CLOVES and PWS but have not been reported in patients with KTS [ 58 ].…”

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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Section: Discussionmentioning

confidence: 99%

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

Pavone,

Marino,

Cacciaguerra

et al. 2023

Children

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“…CLOVES syndrome, which stands for Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi and Spinal (scoliosis) and/or Skeletal anomalies, is a rare sporadic segmental overgrowth syndrome currently classified under PROS (PIK3CA-related overgrowth spectrum) disorders. 1 , 2 The estimated incidence of CLOVES syndrome is less than 1:1,000,000. No gender predilection is reported and the syndrome can be observed in all races and ethnic groups equally.…”

Section: Dear Editormentioning

confidence: 99%