Clinical and Radiographic Poland Syndrome Classification: A Proposal

Ribeiro, Ricardo Cavalcanti; Saltz, Renato; Mangles, M. Gabriela Moreira; Koch, Hilton Augusto

doi:10.1016/j.asj.2009.09.015

Cited by 24 publications

(9 citation statements)

References 37 publications

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“…An accurate description of soft tissue and chest wall morphological abnormalities, not always easy to identify during clinical assessment, is of outstanding importance since to different types of malformation correspond a different therapeutic approach. Evaluation by US is therefore recommended (Table 3) to assess pectoralis muscle (major and minor), breast characteristics of the two halves of the thorax, and could be used as screening exam for cardiac and renal abnormalities [13]. Associated rib abnormalities are detectable by US but, when suspected, imaging work-up should be completed with other diagnostic modalities described later.…”

Section: Radiological Examinationmentioning

confidence: 99%

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

Baldelli

Baccarani

Barone

et al. 2020

Orphanet J Rare Dis

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Background: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. Main body: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. Conclusion: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.

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Section: Radiological Examinationmentioning

confidence: 99%

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

Baldelli

Baccarani

Barone

et al. 2020

Orphanet J Rare Dis

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“…To our knowledge, less than 460 cases have been published worldwide up to now. In 2009, Ribiero RC et al suggested a clinical and radiographic classification of Poland syndrome as shown in Table II [6]. The three pathogenetic mechanisms that seem to contribute to the development of this syndrome are shown in Table III [7][8][9]].…”

Section: Discussionmentioning

confidence: 99%

A very unusual case of poland syndrome with amastia-athelia and dextrocardia

et al. 2011

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Poland syndrome is a rare congenital anomaly characterized by unilateral aplasia of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, deformity or aplasia of the costal cartilages or ribs II to IV or III to V, hypoplasia or aplasia of the breast and nipple, athelia, scarcity of subcutaneous tissue, alopecia of the axillary and mammary region and unilateral syndactyly or brachysyndactyly. The prevailing theory regarding its cause is that the hypoplasia of the subclavian artery or its branches may lead to a range of developmental changes. The congenital unilateral absence of the breast (amastia) and the nipple (athelia) is a very rare deformity and more often encountered in women. We present one very unusual case of Poland syndrome in a woman referred to the Hospital for the treatment of uterine fibroma with amastia-athelia and dextrocardia without complete mirror-image transposition of the adbominal and thoracic viscera and we also review the literature.

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“…Ipsilateral involvement of the chest muscles is the most striking hallmark, and usually presents as complete or partial agenesis of the pectoralis major, contributing to the abnormal contour of the anterior axillary border and compensatory hypertrophy of the residual synergistic muscles. 1 The insufficient arterial blood supply may also result in mammary hypoplasia; malformation of the shoulder girdle, ribs or digits; axillary alopecia; and malposition of the thoracic organs. 1 Poland syndrome is mostly benign, although there are reports of associated malignancies, including leukemia, non-Hodgkin lymphoma, and breast and lung carcinomas.…”

mentioning

confidence: 99%

“…1 The insufficient arterial blood supply may also result in mammary hypoplasia; malformation of the shoulder girdle, ribs or digits; axillary alopecia; and malposition of the thoracic organs. 1 Poland syndrome is mostly benign, although there are reports of associated malignancies, including leukemia, non-Hodgkin lymphoma, and breast and lung carcinomas. 2,3 Treatment is primarily reconstructive surgery, depending on the severity of the malformation, cosmetic appearance and functional performance.…”

mentioning

confidence: 99%