Jogani Abhinav D, George Prakash K, Marathe Nandan A, Shah Swapneel S, Desai Jigar R
Department of Orthopaedics, Seth G.S. Medical College and KEM Hospital, Parel, Mumbai, India.
J Orthop Case Rep. 2019;9(4):3-5. doi: 10.13107/jocr.2019.v09i04.1452.
Poland Syndrome(PS) is a rare congenital condition associated with the 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 portion of the pectoralis major muscle which may or may not be associated with a hypoplasia of the breast, an ipsilateral webbing of the fingers (cutaneous syndactyly) and agenesis of 2, 3, 4, and 5 ipsilateral costal cartilages, and athelia.
We report a 13-year-old patient with an atypical variant of PS without any limb anomalies. In view of the good function of the upper limb, no surgical treatment was offered, and the patient and his family were counseled regarding the condition. A follow-up of the patient at 2 years revealed that the patient is still asymptomatic with good functional status of the upper limb.
It is hoped that this paper will further improve our understanding of this rare syndrome and its atypical presentations.
波兰综合征（PS）是一种罕见的先天性疾病，与单侧胸壁肌肉缺失有关，有时还伴有同侧并指畸形（手指异常短且有蹼）。该病症通常表现为单侧胸大肌胸骨部缺失，这可能与乳房发育不全有关，也可能无关，同时伴有同侧手指蹼状（皮肤并指）以及同侧第2、3、4和5肋软骨发育不全和乳头缺失。
我们报告了一名13岁患有波兰综合征非典型变体的患者，其无任何肢体异常。鉴于上肢功能良好，未提供手术治疗，并就该病症向患者及其家属提供了咨询。对该患者进行2年随访发现，患者仍无症状，上肢功能状态良好。
希望本文能进一步增进我们对这种罕见综合征及其非典型表现的理解。
