History: A35-year-old woman with recurrent chest pain.(反復(fù)胸痛)
Which of the following is the mostlikely diagnosis?
A. Granulomatous polyangiitis（GPA）
B. Goodpasture syndrome
C. Catamenial pneumothorax（月經(jīng)性氣胸）
D. Birt-Hogg-Dubé syndrome（BHD）
E. Malignant mesothelioma（惡性間皮瘤）
Findings
Chest radiograph: Thereis a moderate-sized right hydropneumothorax(液氣胸) with apical and basal pneumothoraxcomponents. There is no mediastinal shift to suggest tension physiology and,therefore, no need for emergent needle decompression(穿刺減壓).The basal air-fluid level corresponds to layering hemorrhagic effusion in thebasal component of the pneumothorax.
Chest CT: ChestCT demonstrates multiple septations(分隔) in the pleural space, secondary toadhesions from recurrent hydropneumothoraces.There are small nodules along the right hemidiaphragm and right lateralparietal pleura corresponding to endometrial deposits of thoracicendometriosis, which was confirmed intraoperatively.（胸部CT顯示胸膜腔多個(gè)由于反復(fù)液氣胸所致胸膜粘連形成的分隔。右側(cè)膈面和壁層胸膜見多個(gè)小結(jié)節(jié)，手術(shù)證實(shí)系胸部子宮內(nèi)膜異位癥所致子宮內(nèi)膜在胸膜的種植）
Diagnosis:
Catamenialpneumothorax from thoracic endometriosis
胸部子宮內(nèi)膜異位癥所致月經(jīng)性氣胸
Differential diagnosis
secondaryspontaneous pneumothorax（繼發(fā)性自發(fā)性氣胸）
Chronic obstructive pulmonary disease(COPD)
Cystic lung diseases（囊性肺?。?/p>
Malignancy（惡性腫瘤）
Pneumonia（肺炎）
Granulomatous polyangiitis（GPA）
Collagen vascular disease（膠原血管病）
Catamenial pneumothorax（月經(jīng)性氣胸）
Spontaneous, recurrent pneumothorax inwomen of reproductive age, occurring in association with menstruation.生育年齡的女性，與月經(jīng)周期有關(guān)的自發(fā)性、反復(fù)發(fā)生的氣胸。
Associated with thoracic endometriosis inapproximately two-thirds of cases, with idiopathic etiology in the remainder ofcases. Notably, thoracic endometriosis can also cause noncatamenialpneumothorax. 約2/3病例與胸部子宮內(nèi)膜異位癥有關(guān)，其余為特發(fā)性。值得注意的是，胸部子宮內(nèi)膜異位癥也能引起非月經(jīng)性氣胸。
Diagnostic criteria:診斷標(biāo)準(zhǔn)
Recurrent pattern of pneumothorax,typically occurring within 24 hours prior to and 72 hours following onset ofmenses. 反復(fù)發(fā)生的氣胸，典型地發(fā)生在月經(jīng)來(lái)潮前24小時(shí)內(nèi)和來(lái)潮后72小時(shí)內(nèi)。
Right sided in approximately 92% ofcases, although left-sided or bilateral pneumothoraces canoccur. 約92%的病例發(fā)生在右側(cè)，盡管左側(cè)或雙側(cè)氣胸均可發(fā)生。
Pathophysiology remains to be fullyelucidated. Proposed mechanisms include the following.其病理生理學(xué)尚未完全闡明，可能的機(jī)制如下：
Ectopic endometrial implants in thediaphragm undergo cyclical necrosis, forming diaphragmatic defects, which thenpredispose to intrathoracic endometriosis and transdiaphragmatic passage ofperitoneal air.種植于膈肌的異位子宮內(nèi)膜周期性壞死，形成膈肌缺損，易導(dǎo)致胸內(nèi)子宮內(nèi)膜異位癥，且腹部空氣易經(jīng)膈肌缺損進(jìn)入胸腔。
Endometrial implants in the visceralpleura, deposited through lymphovascularspread, slough with the menstrual cycle and create small air leaks.子宮內(nèi)膜組織通過(guò)淋巴血管播散并種植入到臟層胸膜，隨月經(jīng)周期脫落，引起少量空氣滲漏。
Endometrial implants in the bronchiolescontribute to prostaglandin-mediated bronchiolar constriction/obstruction andconsequent alveolar rupture.子宮內(nèi)膜種植于細(xì)支氣管，引起前列腺素介導(dǎo)的細(xì)支氣管收縮/閉塞，隨之肺泡破裂
Clinical presentation
Synchronyand recurrence of symptoms with menstrual cycle should raise clinical concern.The most common symptoms include the following:與月經(jīng)周期同步和反復(fù)的癥狀應(yīng)引起臨床注意，常見癥狀如下：
Pain、Dyspnea、Cough、Hemoptysis（咯血）
Imaging findings
Pneumothorax(73%)氣胸
Hemothorax(12%)血胸
Lungnodules (2%)肺結(jié)節(jié)
Radiographic
Pneumothorax, most often right-sided,often in association with hemothorax
Occasional concurrent pneumoperitoneum偶爾發(fā)生氣腹
Gas bubbles along the diaphragmcorresponding to diaphragmatic defects (rare finding)罕見膈肌缺損區(qū)
CT
Findings corresponding to aboveradiographic findings
Fibrosis due to recurrent pneumothoraces isfairly common.由于反復(fù)的氣胸，纖維化很常見
Diaphragmatic or pleural nodules,corresponding to endometrial implants膈肌或胸膜結(jié)節(jié)，與子宮內(nèi)膜種植有關(guān)
Posterosuperior diaphragm is mostcommonly involved.
Diaphragmatic defects or thinning膈肌缺損或變薄
Lung nodules corresponding to parenchymalendometrial implants or hematomas, most often peripherally located肺結(jié)節(jié)，與子宮內(nèi)膜種植于肺實(shí)質(zhì)、出血有關(guān)，外周分布多見
Fluctuate in size with menstrual cycle.大小歲隨月經(jīng)周期變化
Intraoperative findings手術(shù)中所見
Definitive diagnosis remains by surgicalexploration with video-assisted thoracoscopy (VATS). Findings include thefollowing:確診需要電視輔助的胸腔鏡手術(shù)探查，術(shù)中所見：
Diaphragmatic defects, single or multiple膈肌缺損，單或多個(gè)
Endometriosis implants, manifesting aspigmented nodules or spots, most commonly along the diaphragm, but alsoinvolving the parietal and visceral pleura子宮內(nèi)膜種植，表現(xiàn)為著色的結(jié)節(jié)或斑點(diǎn)，最多見于膈肌，但也可見于壁層與臟層胸膜
Treatment
Combined surgical and medical approach.
VATS with pleurodesis, possible repair ofdiaphragmatic defects if present, and lung wedge resection for involved lesions電視胸腔鏡胸膜固定術(shù)，盡可能的膈肌缺損修補(bǔ)，肺楔形切除術(shù)
Postoperative hormonal treatment withgonadotropin-releasing hormone (GnRH) analogues to induce amenorrhea手術(shù)后使用荷爾蒙激素治療，誘導(dǎo)閉經(jīng)
Recurrence rates remain high, at morethan 30% in some series.復(fù)發(fā)率較高，超過(guò)30%
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