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Table 3 Recommendations for radiological examination

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

  Grade Consensus agreement
R3.10 Evaluation by ultrasound is recommended to assess pectoralis major, subcutaneous tissue and breast characteristics of the two halves of the thorax. US examination, because of its more availability, not radiation exposure for the patients and cost-effectiveness, should be the first line (and often the only one) imaging tool in order to confirm the clinical suspect of PS (agenesia or hypoplasia of the pectoralis major muscle) and to assess the severity of anomalies. Definitely useful/strong literature 92,3%
R3.11 Evaluation by CT (without contrast medium) is recommended only for abnormalities of the rib cage that require surgery in non-adult patients Possibly useful/modest literature 100%
R3.12 Pre-natal suspect of PS is sometimes possible as a collateral finding in routine US morphological examination if there are severe bone manifestations involving hands or rib cage, but it has to be confirmed with clinical examination after birth. On the basis of the current state of knowledge no further radiological analysis are indicated in uterus if parents are affected by the syndrome. GCP (no literature available) 100%
R3.13 Radiological workup includes: ultrasound of the pectoralis region, chest X-ray, cardiac evaluation with echocardiography, abdominal US, other examinations on the basis of the specific phenotype Definitely useful/strong literature 100%
R3.14 Ultrasound is able to categorize pectoralis major abnormalities in three classes (i.e. i, total agenesis of the muscle; ii, agenesis of the sternocostal part with normal costoclavicular component; iii, partial agenesis of the sternocostal part with normal costoclavicular component) as well as to recognize regional anomalies affecting the pectoralis minor and regional vessels Definitely useful/strong literature 90,9%
R3.15 MR imaging has limited indications, particularly when ultrasound is non-conclusive Possibly useful/modest literature 77,8%
R3.16 Chest radiography should be obtained routinely for gross evaluation of the rib cage and the heart Definitely useful/strong literature 88,9%
R3.17 Imaging is not always necessary for the diagnosis of PS, however it may be helpful for the surgical planning Definitely useful/strong literature 77,8%
R3.18 CT or MRI often more clearly depict the absence of the pectoralis major muscle and allows better appreciations of other nearby associated musculoskeletal anomalies but should not be indicated in a routine radiologic evaluation in a primary diagnostic phase Definitely useful/strong literature 88,9%
R3.19 US examination should be performed with an high frequency probe, with a musculoskeletal preset, and should be bilateral and comparative, detecting all the 3 heads of the pectoralis major muscle (clavicular, sternocostal and abdominal) with sagittal and trasverse scans, the pectoralis minor muscle and the mammary gland Definitely useful/strong literature 100%
R3.20 X-rays of the thorax or of the ribs are not specific for PS and not often necessary in diagnostic phase but can help showing associated malformation of the rib cage Possibly useful/modest literature 90,0%
R3.21 Only in few selected patients should be considered a complete radiologic study of the skeletal and muscles of hands, forearms, upper arms and/or scapulas with X-rays or CT (other than MRI) in order to better detect complex anomalies clinically evident and define a pre-surgical assessment. GCP (no literature available) 62,5%
R3.22 Chest X-Ray if there is clinical suspicion of rib agenesis. CT scan if severe deformity of the rib cage is observed; cardiac and renal US evaluations could be performed to exclude cardiac or renal anomalies. Possibly useful/modest literature 100%
R3.23 In complex deformities of the chest wall, CT scan may provide a more detailed depiction of bone anomalies and vascular relationships. Multi-imaging evaluation may be needed in case of hand deformities to support clinical assessment. Definitely useful/strong literature 81,8%