Respir Case Rep. 2014; 3(1): 66-70 | DOI: 10.5505/respircase.2014.68442

Upper extremity muscle weakness in a case with Poland syndrome

Ebru Çalýk Kütükcü¹, Naciye Vardar Yaðlý¹, Deniz Ýnal Ýnce¹, Melda Saðlam¹, Hülya Arýkan¹, Ebru Güneþ Yalçýn^2
1Hacettepe University, Faculty Of Health Sciences, Department Of Physiotherapy And Rehabilitation
²Hacettepe University, Faculty Of Medicine, Department Of Pediatry, Unit Of Pediatric Chest Diseases

Poland syndrome is a rare genetic disorder characterized by a partial or total absence of the pectoralis major muscle. In this study, a case of a 13-year-old diagnosed with Poland syndrome is presented with clinical, laboratory, and functional findings. The case presented with brachydactyly and right side pectoral muscle hypoplasia. The respiratory functions and respiratory muscle strength were within normal limits. Chest X-ray was normal. The loss of strength in the upper extremity muscles (shoulder abductors, horizontal adductors, horizontal abductors, elbow flexors, and hand grip) was determined with a hand-held dynamometer. The posture analysis revealed that the patient had moderate to severe postural disorders. In addition to the appearance defect, Poland syndrome can also lead to functional effects such as muscle weakness, postural deformities, and decrease in the exercise capacity at the same time.

Keywords: Poland anomaly, pectoralis muscle, muscle strength dynamometers, muscle strength, respiratory muscles

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Poland sendromlu bir olguda üst ekstremite kas zayýflýðý

Ebru Çalýk Kütükcü¹, Naciye Vardar Yaðlý¹, Deniz Ýnal Ýnce¹, Melda Saðlam¹, Hülya Arýkan¹, Ebru Güneþ Yalçýn^2
1Hacettepe Üniversitesi, Saðlýk Bilimleri Fakültesi, Fizyoterapi Ve Rehabilitasyon Bölümü
²Hacettepe Üniversitesi, Týp Fakültesi, Çocuk Saðlýðý Ve Hastalýklarý Anabilim Dalý, Göðüs Hastalýklarý Ünitesi

Poland sendromu, pektoralis major kasýnýn kýsmen veya tamamen yokluðu ile karakterize nadir görülen bir sendromdur. Bu çalýþmada; 13 yaþýnda Poland sendromu tanýlý olgu klinik, laboratuar ve fonksiyonel özellikleri ile sunulmaktadýr. Olgunun sað taraf pektoral kas hipoplazisi yanýnda brakidaktilisi vardý. Solunum fonksiyonlarý ve solunum kas kuvveti normal sýnýrlardaydý. Akciðer grafisi normal olarak izlendi. El dinamometresi ile üst ekstremite kaslarýnda (omuz abduktör, horizontal adduktör, horizontal abduktör, dirsek fleksör kaslarý ve el kavrama) kuvvet kaybý saptandý. Postür analizinde, hastanýn orta þiddetli postür bozukluklarý olduðu bulundu. Poland sendromu, görünüm defektine ek olarak ayný zamanda kas zayýflýðý, postural deformiteler ve egzersiz kapasitesinde azalma gibi fonksiyonel etkilere yol açabilmektedir.

Anahtar Kelimeler: Poland anomalisi, pektoral kas, kas kuvveti dinamometreleri, kas kuvveti, solunum kaslarý

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