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Table 2 Recommendations for diagnosis of PS (principal diagnostic criteria)

From: Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence)

  Grade Consensus agreement
R2.4 The mandatory feature of PS is the agenesis or hypoplasia of the pectoralis major muscle (the sterno-costal head is always affected). In most cases, PS is unilateral. Presumed bilateral PS needs a more extensive differential diagnosis. Additional diagnostic criteria are hypo/aplasia of the omolateral mammary gland and nipples, and malformations of the omolateral upper limb (limited to or more severely affecting the central rays). Definitely useful/strong literature 93,8%
R2.5 The diagnosis is made through the physical examination of the patient; an ultrasound of the pectoralis muscles is important but not strictly necessary for the diagnosis Definitely useful/strong literature 92,9%
R2.6 The sterno-costal head of the pectoralis major muscle is involved in most frequently; the other heads of the pectoralis major muscle and the pectoralis minor muscle are involved in different percentages of patients Definitely useful/strong literature 93,3%
R2.7 The latissimus dorsi muscle may be involved too in a minority of cases Definitely useful/strong literature 92,3%
R2.8 Many variable phenotypical characteristics can be associated but we cannot diagnose PS in the absence of the basic diagnostic criterion Definitely useful/strong literature 92,9%
R2.9 The concurrence of rare internal organ malformations, such as kidney agenesis or destrocardia, may ease prenatal detection, but also in these cases, the underlying PS is recognized postnatally Definitely useful/strong literature 71,4%