Syndrome de Poland
Seance of wednesday 07 june 2023 (Les malformations congénitales thoraco-mammaires)
DOI number : 10.26299/y4f4-0j23/emem.2023.21.03
Abstract
Poland syndrome is a rare congenital malformation associating varying degrees of abnormalities in the thoracic and ipsilateral upper limb (syndactylies).
The characteristic of this syndrome is the agenesis of the sternocostal bundles of the pectoralis major muscle with very often associated breast agenesis.
Its etiology remains unknown and discussed. A vascular anomaly would be the cause, hypo-perfusion of the musculoskeletal elements of the chest wall during fetal life.
The diagnosis is essentially clinical; thwarted adduction maneuver which highlights agenesis of the pectoralis major.
The functional impact of this anomaly is very low but its psychological impact is particularly important.
The purpose of this presentation is to present the thoraco-mammary deformity and the means of correction available and the therapeutic indications in the primary cases and also in the secondary cases.
The characteristic of this syndrome is the agenesis of the sternocostal bundles of the pectoralis major muscle with very often associated breast agenesis.
Its etiology remains unknown and discussed. A vascular anomaly would be the cause, hypo-perfusion of the musculoskeletal elements of the chest wall during fetal life.
The diagnosis is essentially clinical; thwarted adduction maneuver which highlights agenesis of the pectoralis major.
The functional impact of this anomaly is very low but its psychological impact is particularly important.
The purpose of this presentation is to present the thoraco-mammary deformity and the means of correction available and the therapeutic indications in the primary cases and also in the secondary cases.