Indian J Thorac Cardiovasc Surg (July–September 2014) 30(3):226–228 DOI 10.1007/s12055-014-0299-4
CASE REPORT
Catamenial pneumothorax with diaphragmatic defects associated with Morgagni hernia Yasser Aljehani & Yasser El-Ghoneimy
Received: 27 August 2012 / Accepted: 21 September 2013 / Published online: 28 August 2014 # Indian Association of Cardiovascular-Thoracic Surgeons 2014
Introduction The association between catamenial pneumothorax (CP) and Morgagnian hernia (MH) has never been reported to our knowledge. The presence of anterior diaphragmatic defect could be part of a delayed presentation of MH or part of diaphragmatic involvement in diffuse endometriosis. Such a case highlights the wide variation inthe presentations of thoracic endometriosis. Morgagni described a substernal herniation of abdominal contents in 1769. Larrey described a surgical approach addressing the anterior diaphragmatic defect in 1828 [1]. Synonyms for MH include retrosternal hernia, subcostosternal hernia, and Larrey’s hernia [1]. CP is considered to be a sequel to thoracic endometriosis. Schwarz described lung parenchymal endometriosis in 1938 [2]. In 1958, Maurer described CP and later in 1972, and Lillington coined the name [3]. The ongoing flow of literature over the years has contributed to better understanding of this entity evidenced by the many theories of etiology and various treatment approaches. Theories presented over the years include retrograde menstruation through lymphatics or hematogenous spread as described by Schron and Ruysh [2]. Maurer described the intraperitoneal air theory in 1958 followed in 1972 by Lillington who described the subpleural implants theory [4]. Rossi and Goplerud in 1974 described the Prostaglandin F2α and its bronchvascular spasm effect in the pathogenesis of CP [4]. Sloughing and desquamation of these endometrial tissues cause the rupture of bullae and blebs or creation of diaphragmatic defects. Here, we raise the speculation of acquired MH
Y. Aljehani (*) : Y. El-Ghoneimy Department of Surgery, Cardiothoracic Units, and King Fahd Hospital of the University, University of Dammam, Dammam, Saudi Arabia e-mail:
[email protected]
due to the sloughing effect or being a genuine case of associated congenital diaphragmatic hernia.
Case report A 40-year-old married lady presented with progressive shortness of breath for the preceding 5 days. There was no history of cough, hemoptysis, or chest pain. There was no fever, night sweats, or other constitutional symptoms. She gave a history of spontaneous pneumothorax 2 years earlier, which had been treated conservatively with chest tube drainage. She underwent right oopherosalpingectomy for pelvic endometriosis 11 months prior to her current presentation. Her gynecological history revealed menarche at 12 years, and her menstruation was irregular. The review of systems was unremarkable. On examination, her vital signs were as follows: pulse 90/min; blood pressure 126/68 mmHg; respiratory rate 20/ min; temperature 37.1 °C; and room air saturation 96 %. General examination was unremarkable. Chest examination revealed diminished air entry to the right lung with hyper resonant percussion note over the right hemithorax. The rest of the examination was unremarkable. Her routine laboratory test results were within normal ranges. A plain chest x-ray (CXR) was done and showed moderate to large right-sided pneumothorax and near complete collapse of the right lung. It showed a nodular lesion over the right copula of the diaphragm (Fig. 1). Computed tomography (CT) revealed a large pneumothorax and a collapsed lung with multiple septations in the pleural cavity. A soft tissue component was seen in the retrosternal area with fat density (Fig. 2). Since this was a recurrent pneumothorax, we elected to proceed with right video-assisted thoracoscopic surgery (VATS). Upon exploration, diaphragmatic defects were seen through which liver tissue was herniating. The decision was taken to convert to open thoracotomy for better visualization and better repair of
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Fig. 3 Intraoperative picture demonstrating the right diaphragmatic holes with liver tissue herniating through Fig. 1 Plain chest film showing right tension pneumothorax. The tracing of the visceral pleura is irregular. The surface of the right diaphragm shows a nodule like structure. The right heart border is obscured
the Morgagni hernia that was found during the exploration. The multiple purplish nodular lesions over the liver (Fig. 3) were reduced, and the defect was repaired. The anterior defect was repaired as well. The patient had an uneventful postoperative course and was discharged within a week. Due to patient’s desire for continued fertility, it was decided to continue on follow-up without prescribing oral contraceptives. Followup after a year did not show any recurrence.
Discussion The purpose of reporting this case is to explore the association between catamenial pneumothorax and Morgagnian hernia. With reporting of such cases, the pathogenesis is better understood and proper effective management is initiated. Diaphragmatic hernia is categorized into congenital and acquired types. Among the congenital types is the Morgagnian
Fig. 2 Axial CT chest demonstrating the collapsed lung and an anterior mediastinal soft tissue component in the retrosternal area. This is the omental content of the hernia as confirmed intraoperatively
hernia. Larrey and Morgagni described a triangular space between muscle fibers originating from costal margin and xiphisternum inserting into the central tendon. This space is called Larrey’s triangle. The internal mammary artery passes through this triangle [1]. MH is considered rare and is about 20 times less frequent than Bochdalek hernia. It accounts for 3 % of all surgically treated diaphragmatic hernias [1]. Among delayed presentation of diaphragmatic hernias (more than 2 months of age), MH reaches up to 30 % in some series (5). It is more common in females—70 % of cases [1]. It is more common on the right side (90 %) but left-sided and bilateral cases have been reported [5]. The reason for the right-side predominance is explained by partial obstruction of the defect by the liver and reinforcement of the left-sided diaphragm by the heart and pericardium [6]. With chronicity, 40 % develop a sac (5). The omentum is the most common content followed by the colon, stomach, and small bowel [1]. The presentation varies from an incidental finding to a surgical emergency resulting from complications of hernia—mainly volvulus, strangulation, hemorrhage, or perforation. Other presentations include abdominal symptoms, nonspecific symptoms, or chest symptoms including recurrent chest infections. Risk factors for MH include pregnancy, trauma, or obesity [1, 6]. CT has a sensitivity reaching 100 % and demonstrates a fatty mass radiating from parasternal aspect [1]. The differential diagnosis includes pneumothorax, pleural effusion, empyema, necrotizing pneumonia, or anterior mediastinal mass [5]. The management is surgical— transabdominal or transthoracic, open, or minimally invasive. Thoracic endometriosis is the presence of functional endometrial tissue in the pleural cavity, airways, or lung parenchyma [7]. It presents as CP (73 %), catamenial hemothorax (14 %), catamenial hemoptysis (7 %), pulmonary nodule (6 %), isolated catamenial chest pain, or catamenial pneumomediastinum [7]. It is considered rare with an incidence of around 2.8–5.6 %, but increased awareness and reporting, it is expected to rise. The temporal relation defines CP which takes place within 24–72 h from the onset of
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menstruation [8]. Ninety percent have their symptoms within 2 years of menarche with a mean age of onset of 34 years (15– 47) [9]. It is mainly on the right side (91 %), left-sided in 5 %, and bilateral in 4 % (9). The reason for the right side predominance is the clockwise movement of intraperitoneal fluid reaching the right subphrenic area where the liver acts as a piston to push the fluid to the right thoracic cavity [9]. CP should be suspected in every ovulating woman with emphasis on gynecological history. Diagnosis is difficult and there is no clear established relation to pelvic endometriosis especially since CP has been reported with pelvic endometriosis in less than 30 % of the cases [8]. Our interest in this case is the type of diaphragmatic involvement which includes multiple defects, multiple nodular brown lesions, or associated defects and nodules [10]. The case demonstrated defects on the diaphragmatic surface and liver tissue was herniating through in addition to an anterior retrosternal hernia defect. Reviewing operative findings in reported series showed basically diaphragmatic lesions, endometrial lesions, or no lesions. Diaphragmatic lesions were seen in almost 40 % in form of endometriosis and defects (12 %), endometriosis without defects (10 %) or defects without endometriosis—as our case (16 %) [9]. Endometriosis on the visceral pleura was found in 29 % of cases. Bullae or blebs were found in 23 % of cases. Interestingly, 8.5 % did not show any significant intraoperative findings [9]. The management of CP is controversial and is tailored according to the patients’ age, desire for pregnancy, severity, and frequency of attacks. Options include observation, medical management including oral contraceptives or gonadotropin-releasing hormone analog, chest tube insertion, surgical management, or a combination of the above. Surgical options include pleurodesis for recurrent pneumothorax, diaphragmatic intervention including excision of defects with plication with or without mesh and pleurodesis, wedge resection, or anatomical resection of the diseased lung and visceral pleura [9]. Surgical treatment shows superiority to the less invasive medical management alone.
Conclusion Thoracic endometriosis is becoming a more established entity with better understanding of its pathogenesis and treatment options. Controversy still exists due to its rarity. We have described a new associationbetween CP and MH that could be a purely incidental association or an extensive diaphragmatic involvement that reach caused omental herniation through a retrosternal diaphragmatic defect resembling MH.
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