Ramesh Hebbale, Kaushik Ramachandran, Varadaraju R, Amulya Cherukumudi*

Department of Cardiothoracic and Vascular surgery 
Sri Jayadeva Institute of Cardiovascular Sciences and Research
Bangalore, Karnataka, India

Abstract

 Objective: The aim of this article was to present the results of thoracotomy performed in three symptomatic adult patients with congenital diaphragm eventration in the light of current literature data.

Methods: Along with representation of the surgical management of each case, a brief review of literature was performed.

Results and Conclusion:  Diaphragmatic eventration is a rare clinical entity that may be congenital or acquired. Surgery, which may be open or endoscopic, is the primary treatment modality for symptomatic patients.

Key words: Diaphragmatic eventration, congenital, surgery

Introduction

Eventration of the diaphragm is an abnormally high or elevated position of one leaf of the diaphragm as a result of paralysis, or atrophy of varying degree of the muscle fibers (1). Diaphragmatic eventration affects less than 0.05 % of the population including children and adults with male preponderance (2). It commonly affects the left hemidiaphragm and is usually unilateral (3) ; bilateral affliction is rare. It can be congenital or acquired; in the congenital variant, the phrenic nerve is normal. Eventrations are typically asymptomatic and are incidentally diagnosed (4). Rarely, it can cause progressive dyspnea and frequent respiratory infections and surgery is indicated in such patients (5, 6).

There is a bulging of the entire diaphragmatic leaflet in contrast to a hernia, which has a localized defect. The diaphragm is replaced with fibro-elastic tissue which causes displacement of the hemidiaphragm on the affected side(1). This leads to a compliant thin membrane formation which causes reduced movements leading to poor lung expansion and reduced blood flow with protrusion of abdominal contents to the thoracic cavity (Fig.1) (2, 5).

Figure 1. Schematic view of diaphragmatic eventration with compression of thoracic organs

Here, we discuss three cases of congenital diaphragmatic eventration and the management strategies.

Case 1

A 55-year-old male with no significant co-morbidities and no history of trauma reported with chief complaints of worsening dyspnea since a month. Physical examination showed reduced breath sounds in the right infra-mammary, infra-axillary and infra-scapular areas with bowel sounds in the right infra-mammary region.

Case 2

A 28-year-old male, a known case of biopsy proven nephrotic syndrome with no history of trauma, presented with chief complaints of swelling of face with worsening dyspnea for 3 months. Physical examination revealed reduced breath sounds in the left infra-mammary, infra-axillary, and infra-scapular areas with bowel sounds in the left infra-mammary region.

Case 3

A 20-year-old male with no comorbidities and no history of trauma, that presented with cough and dyspnea, was incidentally diagnosed with a right diaphragmatic hernia after undergoing a chest radiograph.  No abnormalities were detected on physical examination other than reduced breath sound in the right infra-mammary, infra-axillary, and infra-scapular areas with bowel sounds in the right infra-mammary region.

All the patients were evaluated with a pre-operative chest radiograph which revealed substantially elevated hemidiaphragm on the affected side (Fig.2). A contrast enhanced computerized tomography of the thorax and abdomen revealed a displacement of the respective organs into the thoracic cavity with collapse of the lower lobe of the ipsilateral lung on the affected side (Fig. 3). Sniff test (fluoroscopic assessment of diaphragmatic function) showed paradoxical movement of elevated hemidiaphragm. In view of worsening dyspea, it was decided that all 3 patients require surgical management.

Figure 2.  Preoperative chest radiograph of case 2 – see text for explanation

Figure3. Preoperative computed tomography scan of case 3 – see text for explanation

After adequate preoperative bowel preparation in all 3 patients, they underwent a muscle sparing anterolateral thoracotomy that was performed in the 5th intercostal space on the affected side. With this approach, the free edge of m. lattismus dorsi was mobilized posteriorly and the m. serratus anterior is freed at the level of its anterior insertion on the chest wall allowing earlier access to the anterior portion of the diaphragmatic leaflet. The diaphragm appeared morphologically normal but thinned out. The affected dome was opened anteriorly and towards the periphery following the line of insertion into the chest wall in order to avoid injury to the major branches of the phrenic nerve. The  contents were displaced inferiorly through the opened diaphragm into the abdominal cavity. The diaphragm was reconstructed  overlapping the two edges of the opened muscle with interrupted size 0 non absorbable U shaped sutures in a double breasted reconstruction technique, reinforced by a second running layer. Following which, the diaphragm was reinforced with a prolene mesh of 15x 15 cm in size with a non-absorbable interrupted suture.

We had to perform a midline laparotomy in case 2 as the right kidney was adherent and required mobilization of the Gerota’s fascia to reduce the contents and the mesh was placed on the undersurface of the diaphragm through the laparotomy incision using trochars.

In case 3, the mesh was anchored to the subcutaneous tissue with 1 -0 prolene as an innovative method. This was done to strengthen the diaphragm additionally from the intraabdominal forces.

All the patients underwent thoracotomy with diaphragmatic plication and mesh reinforcement. The patient’s postoperative course was uncomplicated and they were discharged on the 4th postoperative day and were seen in the outpatient clinic a week later and a chest X ray demonstrated a normal diaphragm contour of the previously affected side. There was a marked reduction of dyspnea which was appreciated among all the patients. After an initial follow-up of one month they are asymptomatic and are on further follow-up regime. These patients all underwent pulmonary function tests, which were found to be normal for their age.                                                                  

Figure 4. Intraoperative excision of diaphragm with defect closure of case 1

Figure 5. Intraoperative view – after mesh placement in case 1

Discussion