Turgut IŞITMANGİL, Nurettin YİYİT, Serkan ARIBAL

Is isolated dextrocardia a feature of left-sided Poland's syndrome?

Amaç: Bu çalışmada, Poland sendromu ve dekstrokardiarasındaki ilişki araştırıldı. Çalışmaplanı:Ocak 2005 - Aralık 2013 tarihleri arasındasekiz erkek hastaya (ort. yaş 21 yıl; dağılım 19-25 yıl)dekstrokardi ile birlikte Poland sendromu tanısı konuldu.Tüm hastalarda sol taraflı sendrom vardı. Hastalar ekanormallikler açısından değerlendirildi. Bul gu lar: İzole dekstrokardi, sol pektoral kas yokluğuve meme anomalileri tüm hastalarda tespit edildi. Solserratus anterior kasının yokluğu veya hipoplazisiüç hastada, kısmi kaburga agenezisi beş hastada,kontralateral pektus karinatum deformitesi üç hastada,skolyoz bir hastada, brakisindaktili bir hastada ve palmarhiperhidroz bir hastada tespit edildi. Üç hastada kaburgaagenezisi olmaksızın dekstrokardi ve Poland sendromubirlikteliği vardı. Sol taraflı Sprengel deformitesi üçhastada gözlendi. So nuç: Olgu serimiz dekstrokardinin sol taraflı Polandsendromunun bir özelliği olabileceği ve Poland sendromuile izole dekstrokardi kombinasyonunun kaburga agenezisiolmaksızın da seyredebileceği görüşünü desteklemektedir.

İzole dekstrokardi sol taraflı Poland sendromunun bir özelliği midir?

Background: In this study, we investigate the relationshipbetween Poland s syndrome and dextrocardia. Methods: The diagnosis of Poland s syndrome withdextrocardia was made in eight male patients (mean age21 years; range 19 to 25 years) between January 2005 andDecember 2013. All patients had left-sided syndrome. Thepatients were evaluated in terms of additional anomalies. Results: Isolated dextrocardia, the absence of the leftpectoral muscle, and breast anomalies were detected inall patients. The absence or hypoplasia of the left serratusanterior muscle in three patients, partial rib agenesis infive patients, contralateral pectus carinatum deformity inthree patients, scoliosis in one patient, brachysyndactyly inone patient, and palmar hyperhidrosis in one patient wereidentified. Three patients had Poland s syndrome withdextrocardia without rib agenesis. Left-sided Sprengel sdeformity was observed in three patients. Conclusion: Our case series may support the view thatdextrocardia may be a feature of left-sided Poland ssyndrome and the combination of Poland s syndrome andisolated dextrocardia may be present without rib agenesis.

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