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家族性月经性气胸:两姐妹的病例报告及文献简要综述

Catamenial pneumothorax in a family: Case reports of two sisters and a brief literature review.

作者信息

Dezfouli Mojtaba Mokhber, Hasanzade Arman, Sheikhy Kambiz, Ghorbani Fariba, Dizaji Mohsen Herik

机构信息

Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Tracheal Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

Int J Surg Case Rep. 2024 Dec;125:110529. doi: 10.1016/j.ijscr.2024.110529. Epub 2024 Oct 28.

Abstract

INTRODUCTION AND IMPORTANCE

Catamenial pneumothorax (CP) is a condition characterized by recurrent spontaneous pneumothoraxes in women, in temporal pattern with menstrual bleeding. This report presents two cases of CP from a family of three sisters affected by this condition.

CASE PRESENTATION

The first case involved a 38-year-old woman with a history of recurrent pneumothoraxes. Following the diagnosis of CP, she underwent wedge resection with video-assisted thoracoscopic surgery (VATS). However, due to recurrence and the discovery of a diaphragmatic endometrial nodule, she required nodule excision and diaphragm reconstruction through open thoracotomy. The second case concerned a 47-year-old woman who had a single episode of pneumothorax. After being diagnosed with CP, she also underwent VATS. During the procedure, multiple endometrial diaphragmatic lesions were found, necessitating excision via open thoracotomy. Despite the surgery, the pneumothorax reoccurred, which was managed with a hysterectomy and bilateral salpingo-oophorectomy. At the one-year follow-up, both patients were well.

CLINICAL DISCUSSION

CP is a rare disorder with unclear pathophysiology. Additionally, the impact of genetic factors has not been fully evaluated, and familial cases are rarely reported.

CONCLUSION

We believe that this report of CP in three sisters suggests a potential genetic role in its occurrence, necessitating further investigation. A better understanding of genetic factors may improve our knowledge of the pathophysiology of CP, ultimately aiding in the management of the condition. We also want to emphasize the importance of collaboration between thoracic surgeons and gynecologists in managing CP, as well as improving prevention of its recurrence.

摘要

引言与重要性

月经性气胸(CP)是一种女性反复出现自发性气胸的病症,发作时间与月经出血有时间规律。本报告介绍了一个家庭中三名姐妹患此病的两例CP病例。

病例介绍

首例病例为一名38岁有反复气胸病史的女性。诊断为CP后,她接受了电视辅助胸腔镜手术(VATS)下的楔形切除术。然而,由于复发以及发现膈肌子宫内膜结节,她需要通过开胸手术切除结节并重建膈肌。第二例病例是一名47岁女性,她有过一次气胸发作。诊断为CP后,她也接受了VATS。手术过程中发现多个子宫内膜膈肌病变,需要通过开胸手术切除。尽管进行了手术,气胸仍复发,通过子宫切除术和双侧输卵管卵巢切除术进行了处理。在一年的随访中,两名患者情况良好。

临床讨论

CP是一种罕见疾病,病理生理机制尚不清楚。此外,遗传因素的影响尚未得到充分评估,家族性病例报道很少。

结论

我们认为,这例三姐妹患CP的报告表明遗传因素在其发病中可能起作用,需要进一步研究。更好地了解遗传因素可能会增进我们对CP病理生理学的认识,最终有助于该病的治疗。我们还想强调胸外科医生和妇科医生在CP治疗中合作以及改善预防复发方面的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd64/11638649/036afc228cad/gr1.jpg

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