Radiological aspects of the Poland syndrome and implications for treatment: a case study and review.
Poland syndrome (PS) (OMIM 173800) is a rare congenital anomaly classically consisting of the combination of unilateral aplasia of the sternocostal head of the major pectoral muscle and an ipsilateral hypoplastic hand with simple syndactyly and short fingers. The aetiology is most probably a vascular disruption sequence of the subclavian arteries. In most cases, PS is sporadic. Familial occurrence suggests that genes exist which are involved in the pathogenesis as paradominant traits. The syndrome may include mammary hypoplasia and further muscle abnormalities which can be accurately defined by sonography and MRI. The evaluation of the vascular status can be performed using colour coded duplex sonography for peripheral arteries and contrast-enhanced MR-angiography for supra-aortic arteries.


CONCLUSIONS
We report a 7-year-old girl with unilateral right sided Poland syndrome with particular emphasis on the radiological investigations of vascular abnormalities. A review of the literature concerning the origin, outcome, and implications for treatment is given.
Mentzel
Hans-Joachim
HJ
Institute of Diagnostic and Interventional Radiology, Friedrich-Schiller-University, Bachstrasse 18, 07740 Jena, Germany. hans-joachim.mentzel@med.uni-jena.de
Seidel
Jörg
J
Sauner
Dieter
D
Vogt
Susanna
S
Fitzek
Clemens
C
Zintl
Felix
F
Kaiser
Werner A
WA
D002648:Child; D005260:Female; D005542:Forearm / Q000254:growth & development; D006801:Humans; D007621:Karyotyping; D018810:Magnetic Resonance Angiography; D011045:Poland Syndrome / Q000150:complications / Q000175:diagnosis* / Q000196:embryology / Q000235:genetics / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D018615:Ultrasonography, Doppler, Color; D018616:Ultrasonography, Doppler, Duplex