Published: 2018-07-25

Poland syndrome with associated synbrachydactyly and dextrocardia: a rare case

Govind Singh Rajawat, Narendra Khippal, Rajendra Prasad Takhar


Poland’s Syndrome is a rare congenital condition. It is classically characterized by absence of unilateral chest wall muscles and sometimes ipsilateral symbrachydactyly (abnormally short and webbed fingers). The condition typically presents with unilateral absence of the sternal or a breast portion of the pectoralis major muscle which may or may not be associated with the absence of nearby musculoskeletal structures. Authors report a 13-year-old male patient with typical features of Poland syndrome associated with synbrachydactyly and dextrocardia. This association is very rare, till 2010, only 19 cases have been described in scientific literature (Poland syndrome with dextrocardia), in this case synbrachydactyly also associated on ipsilateral side. To the best of our knowledge, this is the first documented case of the left sided Poland syndrome with dextrocardia and symbrachydactyly reported from the South East Asia Region (SEAR).


Dextrocardia, Poland syndrome, Synbrachydactyly

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Fokin AA, Robicsek F. Poland’s syndrome revisited. Ann Thorac Surg. 2002;74:2218-25.

Ferraro GA, Perrotta A, Rossano F, D’Andrea F. Poland syndrome: description of atypical variant. Aesth Plast Surg. 2005;29:32-3.

Fokin AA, Robicsek F: Poland syndrome revisited. Ann Thorac Surg. 2002;74:903-18.

Lagbo JN. Poland syndrome: report of a variant. J Natl Med Assoc 2006; 98: 97-99.

Moir CR, Johnson CH. Poland’s syndrome. Semin Pediatr Surg. 2008;17:161-6.

Urschell HC. Poland syndrome. Chest Surg Clin North Am. 2000;10:393-403.

Bavinch JNB, Weaver DD. Subclavian artery disruption sequence: hypothesis of vascular etiology for Poland, Klippel-Feil and Mobius anomalies. Am J Med Genet. 1986;23:903-18.

Fraser FC, Ronen GM, O’Leary E. Pectoralis major defect and Poland sequence in second cousins: extension of the Poland sequence spectrum. Am J Med Genet. 1989;33:468-70.

Baban A, Torre M, Bianca S, Buluggiu A, Rossello MI, Calevo MG, et al. Poland Syndrome with bilateral features: case description with review of the literature. Am J Med Genet. 2009;149A(7):1597-602.

Cobben JM, Robinson PH, van Essen AJ, van der Wiel HL, ten Kate LP. Poland anomaly in mother and daughter. Am J Med Genet. 1989;33:519-21.

Czeizel A, Vitéz M, Lenz W. Birth prevalence of Poland sequence and proportion of its familial cases. Am J Med Genet. 1990;36:524.

Hoyme HE, Der Kaloustian VM, Hogg H, Entin MA, Guttmacher AE. Possible common pathogenetic mechanisms for Poland sequence and Adams-Oliver syndrome: an additional clinical observation. Am J Med Genet. 1992;42:398-9.

David TJ. Familial Poland anomaly. J Med Genet. 1982;19:293-6.

Lowry RB, Bouvet JP. Familial Poland anomaly. J Med Genet. 1983;20:152-4.

Garcìa CC, Castilla AN, Jiménez EL, Garcìa IA. Dextrocardia Associated with Left-Sided Poland Syndrome. Am J Phys Med Rehabil. 2009;88(2):168.

Mutlu H, Sildiroglu O, Basekim CC, Kizilkaya E. A variant of Poland Syndrome associated with dextroposition. J Thorac Imaging. 2007;22:341-2.

Eroglu A, Yildiz D, Tunc H. Dextrocardia is a component of left-sided Poland syndrome. The J Thorac Cardiovasc Surg. 2005;130(5):1471-2.

da Silva Freitas R, Tolazzi AR, Martins VD, Knop BA, Graf RM, e Cruz GA. Poland’s syndrome: different clinical presentations and surgical reconstructions in 18 cases. Aesthetic plastic surgery. 2007 Apr 1;31(2):140-6.