Department of Thoracic Surgery, GMMA Haydarpasa Training Hospital, Istanbul, Turkey.
Department of Thoracic Surgery, GMMA Haydarpasa Training Hospital, Istanbul, Turkey.
Ann Thorac Surg. 2014 May;97(5):1758-63. doi: 10.1016/j.athoracsur.2014.01.027. Epub 2014 Mar 12.
Poland's syndrome is a rare congenital anomaly characterized by an absence or hypoplasia of the greater and smaller pectoral muscles, breast or nipple anomalies, hypoplasia of subcutaneous tissue, chest wall deformities, pectoral and axillary alopecia, and hand anomalies. Poland's syndrome is usually unilateral. We present 8 patients with multiple bilateral thoracic anomalies.
Eight patients were admitted to our clinic with an abnormal thoracic appearance and restriction of shoulder mobilization. Bilateral multiple muscles, including the greater pectoral muscle and some other thoracic muscles, could not be palpated during physical examination. All patients were evaluated, with a preliminary diagnosis of bilateral Poland's syndrome.
All patients exhibited partial or complete absence of bilateral greater pectoral muscles, absence or hypoplasia of bilateral smaller pectoral muscles, bilateral shoulder protrusion to the front, limited abduction of both shoulders, absence or hypoplasia of other bilateral thoracic muscles (serratus anterior, latissimus dorsi, and trapezius muscles), and scapula alata.
All patients with Poland's syndrome have unilateral hypoplasia or absence of the greater pectoral muscle as the main feature. Poland's syndrome is routinely described as a unilateral syndrome. Cases of Poland's syndrome are typically sporadic. Our patients had different additional bilateral anomalies. In particular, the main problems our patients had were the position of the shoulders and limited abduction of both upper extremities. In contrast to patients with Poland's syndrome, half of our cases were familial. We consider our patients important examples in that they either present a new syndrome or show that Poland's syndrome can be bilateral.
波兰氏综合征是一种罕见的先天性异常，其特征为胸大肌和胸小肌缺失或发育不全、乳房或乳头畸形、皮下组织发育不良、胸廓畸形、胸肌和腋窝部秃发以及手部畸形。波兰氏综合征通常为单侧。我们报告 8 例双侧多发性胸壁畸形。
8 例患者因胸廓异常和肩关节活动受限而就诊。体格检查时双侧多块肌肉，包括胸大肌和其他一些胸肌，均无法触及。所有患者均经评估，初步诊断为双侧波兰氏综合征。
所有患者均表现为双侧胸大肌部分或完全缺失、双侧胸小肌缺失或发育不全、双侧肩部前突、双肩外展受限、其他双侧胸肌（前锯肌、背阔肌和斜方肌）和肩胛骨翼状肩缺失。
所有波兰氏综合征患者均以单侧胸大肌发育不全或缺失为主要特征。波兰氏综合征通常被描述为单侧综合征。波兰氏综合征的病例通常为散发性。我们的患者除了具有不同的双侧额外畸形外。与波兰氏综合征患者不同，我们的一半患者具有家族性。我们认为我们的患者是重要的例子，因为他们要么表现出一种新的综合征，要么表明波兰氏综合征可以是双侧的。
