Case of the Week: November 11-November 18, 2009

Teenager with asymmetric chest

Diagnosis Please

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Case Details

Study shows hypoplasia of the pectoralis muscles on the right. The major is absent and the minor is hypoplastic. The breast and subcutaneous tissue is normal.

Answer

Diagnosis: Poland's Syndrome

    Poland’s syndrome consists of absence of the sternal head of the pectoralis major muscle as well as variable other deformities of the upper chest and extremity including ipsilateral breast and nipple hypoplasia, deficiency of subcutaneous fat, absence of ipsilateral adjacent musles, ribs or upper extremity.

 Patients may also have complete or partial syndactyly of the ipsilateral hand(10%), dextrocardia.     

It is estimated to occur in about 1/30,000 individuals and occurs equally in boys and girls, more commonly on the right than the left.

Patients present usually with aesthetic concerns. Prostheses can be placed when patients are fully grown. 

Reported associations with increase incidence of lung hernia, leukemia,possibly NHL, neuroblastoma, leiomyosarcoma, and Wilm’s.   

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