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包茎病例中偶然发现波兰综合征:尼泊尔的一例罕见病例报告

Incidental finding of Poland syndrome in a case of phimosis: a rare case report from Nepal.

作者信息

Niraula Zenish, Ghimire Pradip, Pokhrel Sanish, Ale Magar Sugam, Bajracharya Jasmine, Thapa Bineet

机构信息

Department of Pediatric Surgery.

Department of Surgery, Nepal Medical College and Teaching Hospital, Kathmandu, Nepal.

出版信息

Ann Med Surg (Lond). 2023 Feb 17;85(3):490-493. doi: 10.1097/MS9.0000000000000215. eCollection 2023 Mar.

Abstract

UNLABELLED

Poland syndrome is a rare congenital syndrome that mostly includes the absence of unilateral pectoralis major muscle and digit anomalies like symbrachydactyly. It can also present with other varied manifestations like the absence of ribs, bilateral absence of pectoralis major muscle, urogenital anomalies, dextrocardia, etc.

CASE PRESENTATION

We herein present a case of a 6-year-old boy with Poland syndrome as an incidental finding, the second one reported from Nepal, 11 years after the first report. The syndrome was diagnosed after the patient came to the hospital for treatment of phimosis. On examination, the sternocostal head of the right pectoralis major muscle was absent with a palpable clavicular head with symbrachydactyly of the ipsilateral side.

DISCUSSION

Poland syndrome is mostly diagnosed clinically. Its differential diagnosis includes other chest wall anomalies, nipple anomalies, isolated thoracic lipoatrophy, and isolated hand/upper limb anomalies without pectoralis major muscle involvement. Computed tomography and MRI scans can help delineate the exact chest wall abnormality. It also helps to find other manifestations of the syndrome and for the treatment plans.

CONCLUSION

Poland syndrome is a rare syndrome that has the propensity to be missed in clinical settings. Treatment, mainly sought for cosmetic reasons, includes breast augmentation procedures or myocutaneous flap coverage.

摘要

未标注

波兰综合征是一种罕见的先天性综合征,主要表现为单侧胸大肌缺如以及并指畸形等手指异常。它还可能伴有其他多种表现,如肋骨缺如、双侧胸大肌缺如、泌尿生殖系统异常、右位心等。

病例报告

我们在此报告一例6岁男孩,波兰综合征为偶然发现,这是尼泊尔在首例报告11年后报告的第二例。该综合征是在患者因包茎前来医院治疗时被诊断出来的。检查发现,右侧胸大肌的胸肋部缺如,可触及锁骨部,同侧伴有并指畸形。

讨论

波兰综合征大多通过临床诊断。其鉴别诊断包括其他胸壁异常、乳头异常、孤立性胸部脂肪萎缩以及不伴有胸大肌受累的孤立性手部/上肢异常。计算机断层扫描和磁共振成像扫描有助于明确胸壁的确切异常情况。这也有助于发现该综合征的其他表现以及制定治疗方案。

结论

波兰综合征是一种罕见的综合征,在临床环境中容易被漏诊。主要出于美容原因的治疗方法包括隆胸手术或肌皮瓣覆盖。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcd9/10010809/9b44049cfd9b/ms9-85-528-g001.jpg

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