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[波兰综合征——临床研究]

[Poland syndrome--clinical study].

作者信息

Vasileva P, Radev R

出版信息

Akush Ginekol (Sofiia). 2007;46 Suppl 2:16-9.

PMID:18173004
Abstract

UNLABELLED

Poland's Syndrome is a rare congenital abnormality involving functional and aesthetic impairments. Constitutive mark of the condition: partial or total absence of the pectoralis major and minor muscles, symmetric chest wall deformity, brachydactyly/syndactyly. This syndrome is occasionally associated with either unilateral or bilateral hypoplasia of breast.

AIM

We present a case of a patient with Poland's Syndrome. This association has not been previously reported in the medical literature. Difficulties by preoperative diagnosis. The purpose of this article--to propose a classification system for the treatment of this anomaly requiring defining the age of the patients for plastic surgery on the chest.

METHODS AND MATERIALS

A 16-year-old female with Poland's Syndrome complained of drastic mood changes consisting of depression and anxiety, regular menses, dysmenorrhea and hypomastia. Two reconstructive surgeries at the age of 12 years--surgical corrections of the sternal malformation.

RESULTS AND CONCLUSION

This work shall describe Poland's Syndrome from a clinical, diagnostic, genetic and therapeutic point of view. The Italian Association for Poland Syndrome founded in October 2003 and two nationwide scientific conferences dealt with the position of scientific research and future prospects with regards to Poland Syndrome. Patients are subject to multi-specialist (surgical, gynecological, genetic, psychological, orthopaedic and plastic surgical) checkups, aiming at an accurate genetic and clinical organisation of a therapeutic programme.

摘要

未标注

波兰综合征是一种罕见的先天性异常,涉及功能和美学障碍。该病症的主要特征:胸大肌和胸小肌部分或完全缺失、胸壁对称性畸形、短指/并指。此综合征偶尔与单侧或双侧乳房发育不全相关。

目的

我们报告一例波兰综合征患者。这种关联此前在医学文献中未见报道。术前诊断存在困难。本文的目的是——提出一种针对该异常情况的治疗分类系统,这需要确定进行胸部整形手术的患者年龄。

方法与材料

一名16岁患有波兰综合征的女性,主诉有由抑郁和焦虑组成的剧烈情绪变化、月经规律、痛经和乳房发育不良。12岁时进行了两次重建手术——胸骨畸形的手术矫正。

结果与结论

本文将从临床、诊断、遗传和治疗的角度描述波兰综合征。2003年10月成立了意大利波兰综合征协会,两次全国性科学会议探讨了关于波兰综合征的科研状况和未来前景。患者需接受多专科(外科、妇科、遗传学、心理学、骨科和整形外科)检查,旨在对治疗方案进行准确的遗传和临床规划。

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