Severity grading of 56 patients with Poland’s syndrome
MA Wei
TIAN Wen
Abstract：Objective Poland’s syndrome is a rare congenital malformation characterized by the combination of unilateral aplasia of the pectoralis major muscle and an ipsilateral hypoplastic hand with syndactyly and short fingers. Besides, patients with Poland’s syndrome have various anomalies of limb and body with various extent. However, previous clinical studies only focused on the anomalies of the hand and chest. In this paper, the authors review 56 cases of Poland’s syndrome and offer a comprehensive scoring method to grade the severity of the anomalies. Methods Based on the physical and imaging examinations, the hand ( H ), arm ( A ) and chest ( C ) were scored from 0 to 10 points for severity. The maximum severity index was 30 points. Fifty-six patients were divided into 3 severity grades:mild ( 4-8 points ), moderate ( 9-16 points ), severe ( 17-30 points ). Results In our series, all patients were thoroughly evaluated:mild in 8 patients ( 12.3%), moderate in 22 patients ( 39.3%) and severe in 26 patients ( 46.4%). Conclusions The severity grading system is an objective, effective and overall method to grade the severity of Poland’s syndrome, and is helpful to direct clinical treatment and evaluate outcomes.
Keywords：Poland syndromeCongenital abnormalitiesUpper extremity deformitiescongenitalHand deformitiesSeverity of illness index
Publication Date：2016-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：8( 661-668 )