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CASE REPORT
Year : 2014  |  Volume : 4  |  Issue : 3  |  Page : 58-60  

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


Department of Internal Medicine, Sher-i-Kashmir Institute of Medical Sciences, Soura, Srinagar, Jammu and Kashmir, India

Date of Submission02-Sep-2013
Date of Acceptance14-Mar-2014
Date of Web Publication15-Sep-2014

Correspondence Address:
Faheem Arshad
133 Housing Colony, Sanat Nagar, Rawalpora, Srinagar - 190 005, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-516X.140746

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   Abstract 

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.

Keywords: Dextrocardia, eventration, left hemidiaphragm, respiratory failure


How to cite this article:
Mir MH, Arshad F, Bagdadi FS, Nasir SA, Hajni MR. Eventration of diaphragm with dextrocardia and type 2 respiratory failure: A rare entity. Int J App Basic Med Res 2014;4, Suppl S1:58-60

How to cite this URL:
Mir MH, Arshad F, Bagdadi FS, Nasir SA, Hajni MR. Eventration of diaphragm with dextrocardia and type 2 respiratory failure: A rare entity. Int J App Basic Med Res [serial online] 2014 [cited 2020 Feb 18];4, Suppl S1:58-60. Available from: http://www.ijabmr.org/text.asp?2014/4/3/58/140746


   Introduction Top


Eventration of the diaphragm is a condition where the muscle is permanently elevated, but retains its continuity and attachments to costal margin. [1],[2] 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. [5],[6] Complete eventration almost invariably occurs on the left side is rare on the right side. [7]


   Case Report Top


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 PO 2 4O mmHg, SaO 2 60.3%, PCO 2 87 mmHg, PH 7.161, HCO 3 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].
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

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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 3 rd 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 2: Contrast computed tomography chest showing complete eventration of the left diaphragm with herniation of colon and stomach in the left chest

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   Discussion Top


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 [2],[10] 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.

 
   References Top

1.Donzeau-Gouge GP, Personne C, Lechien J, Colchen A, Leroy M, Seigneur F, et al. Eventration of the diaphragm in the adult-twenty cases (author′s transl). Ann Chir 1982;36:87-90.  Back to cited text no. 1
    
2.Gatzinsky P, Lepore V. Surgical treatment of a large eventration of the left diaphragm. Eur J Cardiothorac Surg 1993;7:271-4.  Back to cited text no. 2
    
3.Prasad R, Nath J, Mukerji PK. Eventration of diaphragm. J Indian Med Assoc 1986;84:187-9.  Back to cited text no. 3
    
4.Thomas TV. Congenital eventration of the diaphragm. Ann Thorac Surg 1970;10:180-92.  Back to cited text no. 4
    
5.Reber PU, Schmied B, Seiler CA, Baer HU, Patel AG, Büchler MW. Missed diaphragmatic injuries and their long-term sequelae. J Trauma 1998;44:183-8.  Back to cited text no. 5
    
6.Shah R, Sabanathan S, Mearns AJ, Choudhury AK. Traumatic rupture of diaphragm. Ann Thorac Surg 1995;60:1444-9.  Back to cited text no. 6
    
7.Seaton A. Abnormalities and diseases of the diaphragm. In: Seaton A, Seaton D, Leitch AG, editors. Crofton and Druglas′s Respiratory Diseases. 5 th ed. Oxford: Blackwell Sciences; 2000. p. 1234-49.  Back to cited text no. 7
    
8.Laxdal OE, McDougall H, Mellin GW. Congenital eventration of the diaphragm. N Engl J Med 1954;250:401-8.  Back to cited text no. 8
    
9.Chin EF, Lynn RB. Surgery of eventration of the diaphragm. J Indian Med 1986;84:187.  Back to cited text no. 9
    
10.Watanabe S, Shimokawa S, Fukueda M, Kinjyo T, Taira A. Large eventration of diaphragm in an elderly patient treated with emergency plication. Ann Thorac Surg 1998;65:1776-7.  Back to cited text no. 10
    
11.Nathani N, Iles PB. Acute respiratory failure with an abnormal chest radiograph. Respiration 2005;72:205-9.  Back to cited text no. 11
    
12.Deslauriers J. Eventration of the diaphragm. Chest Surg Clin N Am 1998;8:315-30.  Back to cited text no. 12
    
13.Shanmuganathan K, Killeen K, Mirvis SE, White CS. Imaging of diaphragmatic injuries. J Thorac Imaging 2000;15:104-11.  Back to cited text no. 13
    
14.Felson B, editor. Chest Roentgenology. I st ed. Philadelphia: WB Saunders; 2004. p. 463.  Back to cited text no. 14
    
15.Weber TR, Tracy TF Jr, Silen ML. The diaphragm: Developmental, traumatic, and neoplastic disorders. Glenn′s Thoracic and Cardiovascular Surgery. 6 th ed. Stamford CT: Appleton and Lange; 1996. p. 618-9.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2]



 

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