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Dextrocardia Associated With Left-Sided Poland Syndrome

Cordero García, Carlos MD; Nieto Castilla, Andrés MD; López Jiménez, Emilio MD; Amores García, Isabel MD, PhD

American Journal of Physical Medicine & Rehabilitation: February 2009 - Volume 88 - Issue 2 - p 168
doi: 10.1097/PHM.0b013e318197e819
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From the Departments of Physical Medicine and Rehabilitation (CCG, ANC, IAG) and Radiology (ELJ), Juan Ramón Jiménez Hospital, Huelva, Spain.

All correspondence and requests for reprints should be addressed to Andrés Nieto Castilla, MD, Department of Physical Medicine and Rehabilitation, Juan Ramón Jiménez Hospital, Ronda Norte s/n, 21005 Huelva, Spain.

A 21 yr-old man was referred to our hospital because of occasional discomfort in the left costal region. The clinical examination revealed agenesis of the pectoralis major, without evidence of associated syndactylism or brachysyndactyly. The left areola was raised and of smaller size in comparison with the right. There was no family history of interest. The radiologic examination revealed associated dextrocardia. Echocardiography showed dextrocardia with the apex pointing to the right, without other disorders. Multislice computed tomography with 64 detector rings revealed agenesis of the left pectoralis major muscle associated with a deformity of the anterior wall of the same hemithorax characterized by concavity in the area corresponding to the cartilage on the left side with lack of costochondral fusion of the fourth left rib. An alteration of normal sternal anatomy was detected with hypoplasia of the xiphoid apophysis and alteration of the body with multiple enthesophytes in chondral insertions (Figs. 1 and 2).





Poland syndrome is a rare congenital anomaly characterized by the absence of the costosternal portion of the pectoralis major muscle and ipsilateral brachysyndactyly. The estimated incidence of Poland syndrome is between 1/20,000 and 1/30,000 live births. There are many clinical variations of the syndrome, as agenesis of the pectoralis major may seem as an isolated or associated phenomenon, in addition to brachysyndactyly, rib defects, or other abnormalities such as absence of shoulder girdle muscles, hypoplasia, or agenesis of the breast and of the subcutaneous cell tissue. It may also be associated with disorders such as dextrocardia, vertebral defects, Moebius syndrome, and renal aplasia or hypoplasia.1

The association of dextrocardia and Poland syndrome is very rare, however, as only 18 cases have been described in the scientific literature to date. In all the described cases of association of dextrocardia with Poland syndrome, as in the case presented here, the manifestations of Poland syndrome were on the left side, although they usually occur on the right.2

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1. Fokin AA, Robicsek F: Poland's syndrome revisited. Ann Thorac Surg 2002;74:2218–25
2. Fraser FC, Teebi AS, Walsh S, et al: Poland sequence with dextrocardia: Which comes first? Am J Med Genet 1997;73:194–6
© 2009 Lippincott Williams & Wilkins, Inc.