Congenital soft tissue dysplasia: a new malformation entity and concept

Progress in Pediatric Surgery
D PellerinF Gasnier

Abstract

We report 185 children with clinical manifestations of various conditions classically described as giant hamartoma, angiodysplasia, congenital hypertrophy, congenital trophoedema, localised gigantism (e.g. macrodactyly), etc. It is proposed to group all these conditions into a single entity: congenital soft-tissue dysplasia (CSTD). According to recent advances in fundamental embryology and cell biology, CSTD appears to be a consequence of embryonal or fetal cell biosynthetic dysregulation. The concept of the CSTD entity leads to a common protocol for clinical investigation and a common therapeutic plan, with special reference to the stability and the benign nature of the condition. Treatment should be confined to improving function rather than attempting to correct cosmetic deficits.

Citations

Dec 1, 1993·Pediatric Dermatology·O Enjolras, J B Mulliken
Sep 16, 2000·Pediatric Dermatology·S KrengelR Ruiz-Maldonado
Sep 1, 1994·Pediatric Pathology·H MartelliM Vekemans
Aug 1, 1991·Foot & Ankle·D P GroganJ A Ogden
Mar 10, 2000·Foot & Ankle International·E SobelT M Chieco
Dec 17, 2010·Orbit·Murtuza NuruddinMalee Fernando

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