Catamenial pneumothorax: a rare entity? Report of 5 cases and review of the literature

Abstract

Objective: Spontaneous recurrent pneumothorax during menstruation is reported as catamenial pneumothorax. It is encountered in 3-6% of spontaneous pneumothorax cases among menstruating women. The percentage among women referred for surgery is significantly higher (25-30%). Although it usually involves the right-side (85-95%) it can be left-sided or bilateral. It is associated with diaphragmatic perforations and/or thoracic endometriosis. There is pelvic endometriosis in up to 30-51% of cases. The lesions that are not always found may present as small or larger holes at the central tendon of the diaphragm, as red, blueberry, brown spots or larger nodules at the diaphragm, the visceral or parietal pleura. Lesion histology may reveal endometriosis. We present 5 cases of catamenial pneumothorax treated surgically during the last 6 years.

Patients and methods: Five women, with a mean age of 34+/-9.9 years (median 38, range, 19-45 years) presented with right-sided recurrent catamenial pneumothorax. In 3 patients diaphragmatic perforation(s) were found; perforation suturing (n=1), and diaphragmatic plication reinforced with bovine pericardial patch (n=1) were performed. All patients underwent atypical resection of upper and/or middle lobe segments of lung parenchyma that appeared abnormal (haemorrhagic/emphysematous or blebs). Four patients underwent pleurodesis and 1 patient underwent pleurectomy. Four interventions were performed through video assisted thoracoscopic surgery, while diaphragmatic plication was performed through a video assisted mini-thoracotomy. Histology did not reveal endometriosis tissue.

Results: The postoperative course was uneventful. The patients were extubated in theatre and were discharged home at a mean of 7+/-4 days (median 6 days, range, 4-14 days). Two of them received hormonal therapy [Gonadotropin Releasing Hormone (GnRH) analogue] postoperatively. At a follow-up of 14.16 patient-years (mean 2.83+/-1.08 years, range, 1.33-3.83 years) there was recurrence, 6.5 months postoperatively, in one patient that had not undergone closure of a tiny diaphragmatic hole and had not received hormonal treatment postoperatively. She was treated medically (amenorrhea for 6 months with GnRH analogue) and had no further recurrences (in 3.3 years).

Conclusions: Surgery is the treatment of choice of catamenial pneumothorax. It should aim to complete management of all lesions. The most common complication is recurrence. Early diagnosis and multidisciplinary treatment including hormonal therapy may be beneficial in high risk patients.

Keywords: Catamenial pneumothorax; thoracic endometriosis; video-assisted thoracoscopic surgery.

Figure 1

Images of recurrent spontaneous catamenial pneumothoraces with progressively increasing severity in the 1^st patient. A. Thoracic CT during the 6th episode (9 months preoperatively): small, apical, right-sided pneumothorax; B. Thoracic CT (supine position) during the 7th episode, 2 months preoperatively: medium-sized, right-sided pneumothorax; C. Posteroanterior chest radiogram (erect position) during the 8th episode, 2 days preoperatively: large, right-sided pneumothorax without mediastinal shift. Basal air-fluid level. (Visceral pleural line - red arrows, air-fluid level - blue arrow).

Figure 2

Preoperative posteroanterior chest radiogram (erect position) of the 2nd patient: Medium-sized, apical and subpulmonary, right-sided pneumothorax without mediastinal shift. Basal air-fluid level. (Visceral pleural line - red arrows, air-fluid level - blue arrow).

Figure 3

Preoperative imaging of the 4^th patient. A. Small apical pneumothorax, basal air-fluid level on erect, posteroanterior, chest radiogram; B. Small apical pneumothorax on transverse plane of (supine) thoracic CT. (Visceral pleural line - red arrows, air-fluid level - blue arrow).

Figure 4

Very rare findings on thoracic imaging of the 5^th patient. A. Posteroanterior (erect) chest radiogram: Large, right-sided pneumothorax (red arrows), without mediastinal shift. Basal air-fluid level (blue arrow). “Multinodular appearance” of the contour of the right hemidiaphragm (within oval shaped white line); B. Enlarged image of the right hemidiaphragm: multiple “nodules” extending from the diaphragmatic dome to the costophrenic recess. C. Transverse plane of thoracic contrast CT: large, right-sided pneumothorax; D. Transverse plane of thoracic contrast (supine) CT: large right-sided pneumothorax (posterior air-fluid level, small quantity of pleural fluid), five “nodules” of various sizes and circular contour at the central tendon of the right hemidiaphragm.

Figure 5

Coronal (frontal) planes of thoracic, contrast CT: large, right-sided pneumothorax, multiple “nodules” on the dome and the lateral surface of the right hemidiaphragm.

Figure 6

Characteristic diaphragmatic lesions on intraoperative photographs of the 2^nd patient. A and B. Multiple diaphragmatic red spots (red arrows) and holes (black arrows) at the periphery of the right leaflet of the central tendon, most of them less than 1cm in their maximal dimesion; B. the liver can be seen underneath the perforations (black arrows). The black, dashed arrow shows a spot of thin transparent diaphragmatic central tendon (as if a partial-thickness implant has undergone apoptosis, without resultant perforation).

Figure 7

Very rare intraoperative findings on intraoperative photographs of the 5th patient. A. Liver protrusion through a large, central, multipartitioned defect of the tendinous part of the left hemidiaphragm, consisted of multiple confluent diaphragmatic fenestrations of various sizes; B. Thin, elongated, connective tissue bridges between the confluent diaphragmatic defects; C. Smaller, oval shaped perforation at the periphery of the large multipartitioned central defect. Although very rare, these intraoperative findings share common characteristics with at least 2 individually reported cases of catamenial pneumothorax, and furthermore they bare characteristic similarity with a third individually reported case of catamenial pneumothorax. Thus, in our opinion, despite the rareness, these findings can be considered characteristic of catamenial and/or endometriosis related pneumothorax.