Eventration of diaphragm with dextrocardia and type 2 respiratory failure: A rare entity.

Eventration of the diaphragm is a rare condition where the muscle is permanently elevated, but retains its continuity and attachments to costal margin. In this condition, all or part of the diaphragm is largely composed of fibrous tissue with a few or no interspersed muscle fibers. It can be complete or partial. It is seldom symptomatic and often requires no treatment. We present a 70-year-old male who came with progressive breathlessness and was admitted with type 2 respiratory failure, and on evaluation was found to have complete eventration of the left diaphragm with herniation of colon and stomach in the left chest with dextrocardia. Aim of reporting this rare case is to highlight the importance of history taking, good physical examination, and imaging in the diagnosis of diaphragmatic eventration.

INTRODUCTION

Eventration of the diaphragm is a condition where the muscle is permanently elevated, but retains its continuity and attachments to costal margin.[12] It can be congenital or acquired, consisting of failure of muscular development of part or all of one or both hemidiaphrams.[3] Clinically eventration of the diaphragm refers to an abnormal elevation of one leaf of an intact diaphragm as a result of paresis, paralysis, aplasia, or atrophy of varying degrees of muscle fibers.[4] In some cases, it may be difficult to distinguish it from diaphragmatic paralysis. This condition may be confused with a traumatic rupture of a diaphragm. Early recognition of traumatic rupture of the diaphragm is of utmost importance.[56] Complete eventration almost invariably occurs on the left side is rare on the right side.[7]

CASE REPORT

The case we present here is about a 70-year-man hypertensive presented with a history of progressive exertional breathlessness for last 1 month with worsening from last 5 days. Breathlessness also used to increase during lying down position. There was also history of swelling feet from last 10 days. There was no history of chest pain, paroxysmal nocturnal dyspnea or dry cough. Past history was insignificant. On physical examination, patient was drowsy, tachypnic, and cyanosed with trachea shifted to right. Pedal edema was present and jugular venous pressure was increased. Chest examination revealed decreased movements on the left side in inframamary, infraaxillary and infrascapular areas. Tactile vocal fremitus was decreased and percussion note was decreased on left side. Air entry was decreased in left infra mammary, infra axillary, and infrascapular areas. Apex beat was not felt on left side. Laboratory investigations showed normal hemogram, kidney and liver function tests. Arterial blood gas analysis revealed PO2 4O mmHg, SaO2 60.3%, PCO2 87 mmHg, PH 7.161, HCO3 30.4, Na 136 meq/l, K 4.52 meq/l. X-ray chest showed elevated left dome of diaphragm with bowel loops in left side of chest with mediastinal shift to right side [Figure 1]. Electrocardiogram revealed right axis deviation. Echocardiography showed dextrocardia with concentric left ventricular hypertrophy and severe pulmonary hypertension and tricuspid regurgitation. Contrast computed tomography chest confirmed complete eventration of left diaphragm with hernation of colon and stomach in the left chest [Figure 2]. There was shift of mediastinum to right side. Pulmonary function test revealed restrictive pattern of lung disease with vital capacity 1.31 L, forced vital capacity (FVC) 1.43 L, forced expiratory volume 1 second (FEV1) 1.26 (55%), FEV1/FVC ratio 87.7 (119%). Clinical impression of eventration of diaphragm with restrictive lung disease with congestive cardiac failure and type 2 respiratory failure was made. On the 3rd day of admission, patient developed upper gastrointestinal bleed. Endoscopic findings revealed normal esophagus with stomach anatomically distorted because of which scope falls short of pylorus. No mucosal lesion was seen. Duodenum could not be entered. Endoscopy was tried in right lateral position; even then duodenum could not be entered. Patient was managed with oxygen inhalation, diuretics, and bronchodilators. His oxygenation improved and arterial blood gas at the time of discharge was acceptable.

Figure 1

X-ray chest showing elevated left dome of the diaphragm with bowel loops in the left side of chest with the mediastinal shift to right side

Figure 2

Contrast computed tomography chest showing complete eventration of the left diaphragm with herniation of colon and stomach in the left chest

DISCUSSION

Eventration of the diaphragm is a condition in which all or part of diaphragm is largely composed of fibrous tissue with only a few or no interspersed muscle fibers. It is usually congenital, but may be acquired.[8] It is generally asymptomatic in adults and is discovered incidentally as was seen in this case. Symptoms may be related to gastrointestinal tract, respiratory embarrassment, and rarely cardiac dysfunction.[9] Eventration of the hemidiaphragm, although rare in adults has been reported to be a cause of progressive respiratory failure[210] This patient presented with acute respiratory failure because of a compromised respiratory reserve secondary to long standing eventration, possibly of congenital origin. Eventration of diaphragm causes reduced total lung capacity, vital capacity, expiratory reserve volume and functional residual capacity. Long standing eventration can lead to a state of chronic alveolar hypoventilation and hypercapnea. Any acute illness can easily lead to decompensation and progressive respiratory failure.[11] Although this patient was successfully managed medically, acute presentation have sometimes been successfully managed with surgical plication of the diaphragm. There has a report of successful nonsurgical management of eventration of the hemidiaphragm presenting with acute respiratory distress.[11] In adults the diagnosis of diaphragmatic eventration can usually be made on standard posteroanterior and lateral chest films.[12] A high index of suspicion, past history, previous and present imaging and physical examination of the chest should aid in early and definite diagnosis.

Multiple imaging modalities are available. Chest radiographs are the initial and most commonly performed imaging study. When chest radiographs are intermediate, spiral computed tomography with thin sections are performed and is the imaging study of choice. MRI is used to evaluate the diaphragm for patients with clinical suspicion of but an intermediate diagnosis after chest X-ray and spiral CT.[13] Other imaging modalities are USG chest, fluoroscopy, contrast GI screening. Diagnostic laparoscopy and diagnostic pneumoperitoneum are reliable and simple methods to differentiate eventration from paralysis.[14] Asymptomatic patients are managed conservatively, but symptomatic patients require surgery. Plication of the diaphragm is the procedure of choice.[15] Reinforcement with a synthetic mask may be required. This condition should be recognized as a cause of both progressive and acute respiratory failure. Even in asymptomatic elderly patients, a close follow-up is recommended.[11] Aim of reporting this rare entity is to highlight the importance of history taking, good physical examination and imaging in the diagnosis of diaphragmatic eventration.