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Eight Patients With Multiple Bilateral Thoracic Anomalies: A New Syndrome or Bilateral Poland's Syndrome?
MedLine Citation:
PMID:  24630765     Owner:  NLM     Status:  Publisher    
BACKGROUND: Poland's syndrome is a rare congenital anomaly characterized by an absence or hypoplasia of the greater and smaller pectoral muscles, breast or nipple anomalies, hypoplasia of subcutaneous tissue, chest wall deformities, pectoral and axillary alopecia, and hand anomalies. Poland's syndrome is usually unilateral. We present 8 patients with multiple bilateral thoracic anomalies.
METHODS: Eight patients were admitted to our clinic with an abnormal thoracic appearance and restriction of shoulder mobilization. Bilateral multiple muscles, including the greater pectoral muscle and some other thoracic muscles, could not be palpated during physical examination. All patients were evaluated, with a preliminary diagnosis of bilateral Poland's syndrome.
RESULTS: All patients exhibited partial or complete absence of bilateral greater pectoral muscles, absence or hypoplasia of bilateral smaller pectoral muscles, bilateral shoulder protrusion to the front, limited abduction of both shoulders, absence or hypoplasia of other bilateral thoracic muscles (serratus anterior, latissimus dorsi, and trapezius muscles), and scapula alata.
CONCLUSIONS: All patients with Poland's syndrome have unilateral hypoplasia or absence of the greater pectoral muscle as the main feature. Poland's syndrome is routinely described as a unilateral syndrome. Cases of Poland's syndrome are typically sporadic. Our patients had different additional bilateral anomalies. In particular, the main problems our patients had were the position of the shoulders and limited abduction of both upper extremities. In contrast to patients with Poland's syndrome, half of our cases were familial. We consider our patients important examples in that they either present a new syndrome or show that Poland's syndrome can be bilateral.
Nurettin Yiyit; Turgut Işıtmangil; Hasan Saygın
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-3-12
Journal Detail:
Title:  The Annals of thoracic surgery     Volume:  -     ISSN:  1552-6259     ISO Abbreviation:  Ann. Thorac. Surg.     Publication Date:  2014 Mar 
Date Detail:
Created Date:  2014-3-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  15030100R     Medline TA:  Ann Thorac Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
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