Surgical treatment of thoracic deformity in Poland's syndrome. 1989

R C Shamberger, and K J Welch, and J Upton
Department of Surgery, Children's Hospital, Harvard Medical School, Boston, MA.

In 1841, Poland described congenital deficiency of the pectoralis major and minor muscles associated with syndactyly. This syndrome is a spectrum, often involving chest wall and breast deformity as well. Identification of the various musculoskeletal components involved permits optimal thoracic reconstruction in the small proportion of patients who will require it. From 1955 to 1988, 75 patients (40 males and 35 females) with Poland's syndrome were treated or evaluated. Patients with isolated deficiencies of the pectoral muscles, breast, or hand deformity were excluded. The complex was right-sided in 44 patients, left-sided in 30, and bilateral in one. The pectoralis minor and the costal portion of the pectoralis major muscle were absent in all patients. Hand anomalies were present in 50 patients. Athelia and/or amastia were noted in 37 patients. In ten patients, the rib cage deformity required reconstruction, and in three cases, rib or cartilage grafts were needed for complete repair. Often unappreciated in these cases is the significant rotation of the sternum toward the involved side and contralateral carinate deformity. Correction is achieved by bilateral subperichondrial costal cartilage resection and sternal osteotomy (seven of ten patients), thus allowing anterior displacement and orthorotation of the sternum. Chest wall reconstruction must be tailored to the requirements of each patient. No intraoperative or postoperative complications occurred in these ten patients. In males without rib cage deformity, generally no treatment is required to replace the absent pectoral muscles, although in two cases rotation of the latissimus dorsi muscle was performed. In all females, reconstruction of the ipsilateral breast is required at full development.(ABSTRACT TRUNCATED AT 250 WORDS)

UI MeSH Term Description Entries
D008297 Male Males
D008488 Medical Illustration The field which deals with illustrative clarification of biomedical concepts, as in the use of diagrams and drawings. The illustration may be produced by hand, photography, computer, or other electronic or mechanical methods. Illustration, Medical,Illustrations, Medical,Medical Illustrations
D008722 Methods A series of steps taken in order to conduct research. Techniques,Methodological Studies,Methodological Study,Procedures,Studies, Methodological,Study, Methodological,Method,Procedure,Technique
D011045 Poland Syndrome A syndrome which is characterized by symbrachydactyly and aplasia of the sternal head of pectoralis major. Poland Anomaly,Poland Sequence,Poland Syndactyly,Anomaly, Poland,Syndactyly, Poland,Syndrome, Poland
D001940 Breast In humans, one of the paired regions in the anterior portion of the THORAX. The breasts consist of the MAMMARY GLANDS, the SKIN, the MUSCLES, the ADIPOSE TISSUE, and the CONNECTIVE TISSUES. Breasts
D005260 Female Females
D006228 Hand Deformities, Congenital Alterations or deviations from normal shape or size which result in a disfigurement of the hand occurring at or before birth. Congenital Hand Deformities,Congenital Hand Deformity,Deformities, Congenital Hand,Deformity, Congenital Hand,Hand Deformity, Congenital
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths

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