Diaphragmatic Fenestrations in Catamenial Pneumothorax: a Management Strategy
Mohammad Rafay, FRCS Ed1,
Hatem El-Bawab, MD1,
Wesam Kurdi, MRCOG2,
Khaled Al Kattan, FRCS Ed1
1 Thoracic Surgery Unit. Department of Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
2 Department of Obstetric and Gynecology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
Hatem El Bawab, PhD, Tel: +966 1 442 7776, Fax: +966 1 442 7772, Email: hysahmed{at}gmail.com, Thoracic Surgery Unit, Department of Surgery (MBC 40), King Faisal Hospital and Research Center, PO Box 3354, Riyadh 11211, Saudi Arabia.
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ABSTRACT
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Video-assisted thoracoscopic exploration of the right hemithorax in a 37-year-old woman with recurrent catamenial pneumothorax revealed multiple diaphragmatic fenestrations. She underwent successful plication of the diaphragm, with no recurrence of pneumothorax after 4 years of follow-up.
Key Words: Diaphragm • Endometriosis • Pneumothorax
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INTRODUCTION
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Diaphragmatic fenestrations play a fundamental role in the pathogenesis of catamenial pneumothorax.1–5 There is no consensus in the literature on a closure procedure for diaphragmatic fenestrations.
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CASE REPORT
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A 37-year-old woman was referred for assessment of recurrent right-sided chest pain and dyspnea coinciding with her menstrual period since she was 19-years old. She attained menarche at the age of 13 years, and chest symptoms commenced at 16 years of age. She was diagnosed to have pelvic and umbilical endometriosis at 26-years old. She had hormonal therapy with danazol for 8 months following umbilectomy, with inadequate relief of her chest symptoms. The first documented pneumothorax was at the age of 35 years, which was drained by thoracostomy. Pneumothorax recurred during the next menstrual period and she underwent video-assisted thoracoscopy and pleural abrasion. Pneumothorax recurred again at the next menstrual period and was drained by thoracostomy, with connection to a Heimlich valve. The patient was evaluated by radiography and computed tomography of the chest, which showed right pneumothorax and normal lung parenchyma. Video-assisted thoracoscopic exploration of the right hemithorax revealed multiple diaphragmatic fenestrations and no other abnormality. The fenestrations were localized to the tendinous portion of the right hemidiaphragm and circular in shape, measuring 2–5 mm in diameter (Figure 1
). The margins were well defined with sloping edges, covered with thin pale-colored transparent epithelium. The superior surface of the liver could be seen as a brown color through the fenestrations. Some of the fenestrations were completely perforated, while others showed incomplete perforation with a thin layer of epithelium in the floor of shallow defects (Figure 1, see arrows 1 and 3). There were multiple 1–2-mm dark-red shallow depressions (Figure 1, see arrow 2). The procedure was converted to a right posterolateral thoracotomy, and a biopsy was taken from the center of the fenestrated portion of the diaphragm. The diaphragm was plicated with nonabsorbable Prolene suture and covered with a Gore-Tex graft that was fixed at the periphery with continuous running 2/0 Prolene sutures. The parietal pleura was abraded, and the thoracotomy was closed in layers. Histopathology of the specimen indicated endometriosis. No hormonal therapy was given postoperatively. There was no recurrence of pneumothorax after 4 years of follow-up.
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Figure 1. Types of diaphragmatic fenestration. Arrows 1 and 3 indicate incompletely perforated fenestrations. Arrow 2 indicates a dark-red shallow depression.
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DISCUSSION
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Recurrent pneumothorax associated with menstruation was first described by Maurer and colleagues1 in 1958. Catamenial pneumothorax was until recently considered a rare entity, occurring in less than 5.6% of women presenting with spontaneous pneumothorax. However, in a recent prospective study, 8 of 32 women hospitalized in an 18-month period for surgical treatment of spontaneous pneumothorax had a catamenial pneumothorax.2
Numerous hypotheses regarding the pathogenesis of catamenial pneumothorax have been advanced: spontaneous rupture of blebs; alveolar rupture caused by prostaglandin-induced bronchiolar constriction; sloughing of endometrial implants of visceral pleura, resulting in air leaks; and passage of air from the genital tract through diaphragmatic defects, in the absence of the cervical mucous plug during menses.3 Medical treatment of thoracic endometriosis in general has been shown to prevent recurrence in 50% of cases at most. A sequential medical-surgical or surgical-medical approach may become necessary for patients who do not achieve a satisfactory response to the first treatment option employed. Gonadotropin-releasing hormone agonist appears to effectively suppress catamenial pneumothorax, but its long-term use is not recommend as it is poorly tolerated and the outcome of chronic hormonal ablation in premenopausal women is unknown.4
Diaphragmatic fenestrations may be congenital or acquired due to sloughing of diaphragmatic endometrial foci. How the endometrial tissue reaches the thoracic cavity remains unknown. Currently, the most favored hypothesis suggests endometrium autotransplantation to ectopic sites through lymphatic or vascular embolization, or more probably, after retrograde menstruation with subsequent transabdominal and transdiaphragmatic passage of endometrial tissue.5 The frequency and severity of symptoms suggest progressive pathogenesis of pleurodiaphragmatic endometriosis. Scrutiny of the size, shape, and appearance of the fenestrations suggests that they are of different ages; some appear to be close to inception and others are fully developed (Figure 1
). Understanding the spectrum of fenestrations has implications for the choice of procedure for closure of the diaphragm. There is no standard method; simple closure, partial resection and closure, plication closure, staple resection and simultaneous closure of the defects, and covering of the diaphragm with mesh with or without resection or closure of fenestrations have been described with variable rates of success.2,6,7 We want to emphasize the fact that simple closure, partial resection, and plication may close fenestrations that are fully developed at the time of surgery, but will this strategy deal with the ongoing pathogenesis of new fenestrations? This concern was appreciated by Bagan and colleagues7 who proposed closure of the diaphragm with mesh. They reported recurrence of pneumothorax following simple closure of diaphragmatic fenestrations in 3 patients, and justified prophylactic use of mesh to cover fenestrations undetected during surgery. Recurrence of catamenial pneumothorax after surgery is common and frustrating, so the ideal procedure would be closure of the existing diaphragmatic defects as well as any fenestrations that might develop in the future due to ongoing pleurodiaphragmatic endometriosis.
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REFERENCES
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- Maurer ER, Schaal JA, Mendez Jr FL. Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm. JAMA 1958;168:2013–4.[Abstract/Free Full Text]
- Alifano M, Roth T, Broët SC, Schussler O, Magdeleinat P, Regnard JF. Catamenial pneumothorax: a prospective study. Chest 2003;124:1004–8.[Abstract/Free Full Text]
- Alifano M, Trisolini R, Cancellieri A, Regnard JF. Thoracic endometriosis: current knowledge. Ann Thorac Surg 2006; 81:761–9.[Abstract/Free Full Text]
- Marshall MB, Ahmed Z, Kucharczuk JC, Kaiser LR, Shrager JB. Catamenial pneumothorax: optimal hormonal and surgical management. Eur J Cardiothorac Surg 2005;27:662–6.[Abstract/Free Full Text]
- Alifano M, Jablonski C, Kadiri H, Falcoz P, Gompel A, Camilleri-Broet S, et al. Catamenial and noncatamenial, endometriosis-related or nonendometriosis-related pneumothorax referred for surgery. Am J Respir Crit Care Med 2007;176:1048–53.[Abstract/Free Full Text]
- Cowl CT, Dunn WF, Deschamps C. Visualization of diaphragmatic fenestration associated with catamenial pneumothorax. Ann Thorac Surg 1999;68:1413–4.[Abstract/Free Full Text]
- Bagan P, Le Pimpec Barthes F, Assouad J, Souilamas R, Riquet M. Catamenial pneumothorax: retrospective study of surgical treatment. Ann Thorac Surg 2003;75:378–81.[Abstract/Free Full Text]
Asian Cardiovasc Thorac Ann 2009;
17:70-72
© 2009 by SAGE Publications
DOI: 10.1177/0218492309102507
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ABSTRACT
INTRODUCTION
