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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 3  |  Page : 206-209

A rare ca(u)se of dysphagia


1 Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
2 Health Centre, Central University of Tamil Nadu, Thiruvarur, Tamil Nadu, India
3 Department of Paediatrics, Government Villupuram Medical College, Villupuram, Tamil Nadu, India

Date of Submission21-Jan-2019
Date of Acceptance10-Jun-2019
Date of Web Publication05-Aug-2019

Correspondence Address:
Dr. Prem Davis
Health Centre, Central University of Tamil Nadu, Thiruvarur - 610 005, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijhas.IJHAS_4_19

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  Abstract 


Achalasia is a rare lower esophageal motility disorder, in which there is destruction of myenteric plexi leading to the loss of esophageal peristalsis and inadequate relaxation of the lower esophageal sphincter in response to swallowing. Although the etiology of achalasia cardia is unclear, it is likely to be caused by autoimmune etiology. It is characterized by dysphagia of solids and liquids, regurgitation, weight loss, chest pain, heartburn, and nocturnal cough. Achalasia can be diagnosed on the clinical history and confirmed by barium swallow, and esophageal manometry which is the gold standard technique for diagnosis. Even though achalasia cannot be cured permanently, pneumatic balloon dilatation and myotomy remain the definitive treatment of choice in spite of availabilities of pharmacological therapies. We report a case of 25-year-old male admitted with chronic dysphagia and weight loss, diagnosed to be having achalasia cardia and treated accordingly.

Keywords: Achalasia cardia, barium swallows, motility disorder, pneumatic dilation


How to cite this article:
Balamurugesan K, Davis P, Ponprabha R. A rare ca(u)se of dysphagia. Int J Health Allied Sci 2019;8:206-9

How to cite this URL:
Balamurugesan K, Davis P, Ponprabha R. A rare ca(u)se of dysphagia. Int J Health Allied Sci [serial online] 2019 [cited 2019 Aug 20];8:206-9. Available from: http://www.ijhas.in/text.asp?2019/8/3/206/263947




  Introduction Top


Achalasia cardia is a rare primary neuromuscular motility disorder of the esophagus due to a reduction in ganglion cells of the myenteric plexus of the lower esophagus.[1] Achalasia cardia was first described by Sir Thomas Williams in 1672. In 1929, Hurt and Rake described the disease which is due to failure of relaxation of the lower esophageal sphincter (LES), and they coined the term achalasia, means failure to relax.[2] It has an incidence of 1.6 cases/lakh population and the prevalence of 10 cases/lakh individuals. However, the incidence of achalasia cardia in India is not known due to underreporting of the cases. It affects both genders equally and can affect any age group, more commonly seen between 25 and 60 years of age group individuals.[3] The autoimmune etiology is being proposed in majority of the cases that usually follows the viral infection.[4] Since achalasia cardia is a rare disease, therefore, we report a case of primary achalasia cardia in an adult.


  Case Report Top


A male in his 27-years of age, presented with the complaints of difficulty in swallowing for both solids and liquids for the duration of 2 years. Initially, he had dysphagia for liquids only and he consulted many physicians. He was treated with H2 blockers and proton-pump inhibitors, for which there was no improvement in the symptoms. Over a period of time, he developed dysphagia for both solid and liquids and approached our hospital. He also had the complaint of recurrent episodes of vomiting and cough which is aggravated with the food intake. The dysphagia was associated with the loss of weight. There is no other history suggestive of chest pain, heartburn, hematemesis, and recurrent respiratory tract infections. His bowel and bladder habits were normal.

In general physical examination, the patient had mild pallor, and his vitals were stable; and in the systemic examination, the patient was found to be having scattered basal crepts with the bilateral normal vesicular breath sounds. His other systemic examinations were found to be normal.

His investigations revealed anemia with the mild microcytic hypochromic picture, other parameters of complete blood count were normal. Moreover, the details of the other investigations are as shown in [Table 1].
Table 1: Blood investigations

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In the X-ray of the chest, there is a mediastinal widening with retrocardiac mass and absent gastric air bubble as in [Figure 1]. Based on the history with long duration of symptoms and X-ray, he was advised to undergo barium swallow. The barium swallow revealed dilated esophagus with tapering at the lower esophageal junctio, as shown in [Figure 2] and [Figure 3]. And then, the patient was proceeded with computed tomography (CT) chest to rule out any secondary causes such as carcinoma of the gastric cardia, esophageal carcinoma, and metastatic disease. CT chest revealed dilated esophagus with air-fluid level as in [Figure 4]. Biopsy and manometry were not done in this case. Based on the clinical and radiological features, the diagnosis of primary achalasia cardia was made. He was treated with the pneumatic dilation (PD). The patient was advised to come with liquid diet 2 days before the procedure and fasting for 12 h. Under sedation, the balloon dilator is positioned across the LES with the fluoroscopic guidance. About 50-cm long gastric over tube is passed into the proximal stomach. The correct positioning of the overtube is confirmed, and a guide wire is passed into the stomach, and the location of the gastroesophageal junction is measured and marked. After overtube placement, a 3-cm balloon dilator is passed into the gastroesophageal junction, and the gastric overtube with the balloon dilator is retracted back proximally to the marked level for proper positioning at the LES. After correct positioning of the balloon waist at the gastroesophageal junction, and after no esophageal leakage confirmed radiologically, the balloon is slowly inflated to the pressure of 15 psi for 15–20 s, and the patient was discharged after 12 h of observation. The patient advised to review immediately if any severe chest pain, shortness of breath or fever, and hematemesis due to the risk of delayed perforation because of vomiting after the procedure. The patient was advised for follow-up after 4 weeks for assessment, and the patient improved symptomatically and he is on regular follow-up on yearly basis clinically and radiographically with a barium swallow.
Figure 1: Plain X-ray showing mediastinal widening with retrocardiac mass and absent gastric air bubble

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Figure 2: Barium swallow X-ray showing dilated esophagus with tapering at the lower esophageal junction

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Figure 3: Barium swallow X-ray showing classical bird-beak appearance

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Figure 4: Computed tomography chest showing dilated esophagus with air-fluid level without any secondary pathology

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  Discussion Top


Achalasia cardia is the primary neurodegenerative disorder of the esophagus and characterized by abnormal motility and the failure of the distal esophageal relaxation.[5] Achalasia is due to the loss of the ganglion cells in the myenteric plexus of the esophagus. The myenteric plexus consists of excitatory neurons which have cholinergic action and inhibitory neurons which produce nitric oxide. The inhibitory neurons of the myenteric plexus are only responsible for the relaxation of the distal esophageal sphincter and the sequential forward propagation of the peristalsis.[6] However, the achalasia cardia involves both the excitatory and inhibitory neurons which lead to loss of the lower esophageal relaxation and the absence of the peristalsis.[6] It is proposed that the decrease or the absence of the myenteric plexus is due to autoimmune-mediated ganglionitis.[4] The latent infection of the patient with the viruses such as herpes simplex virus 1, measles, and human papillomavirus acts as a potential antigen for the development of the autoimmune response in the myenteric plexus of the esophagus.[7] The achalasia usually presents with dysphagia for both solid and liquid foods, as the most common symptoms, followed by regurgitation. Most of the times, it is misdiagnosed as gastrointestinal reflux disease which delays the treatment. The differentiating feature of the regurgitation in achalasia from gastrointestinal reflux disease is that, it occurs during and immediately taking meals, and the patient's complaints of the regurgitation of the undigested foods.[8] Other clinical features include weight loss, chest pain, heartburn, nocturnal cough, and aspiration.[9] The achalasia is diagnosed with the help of X-ray and esophageal manometry. In plain X-ray of chest, the radiological features include widening of the mediastinum, absence of gastric air bubble, and presence of air-fluid level in the mediastinum. In barium swallow, the achalasia is characterized by dilated esophagus with tapering at the lower esophageal junction giving a bird beak-like appearance. Although high-resolution manometry is not done in this case, it is a gold standard technique for the diagnosis of achalasia. CT scan has no diagnostic value in achalasia cardia. However, it helps in differentiating primary and secondary achalasia.[10] The management of achalasia cardia includes pharmacological, nonsurgical, and surgical management. This case has been treated by pneumatic balloon dilation which is the most effective nonsurgical treatment for achalasia. PD uses air pressure of around 8–15 psi across the level of LES for 15–60 s to dilate intraluminally. This radial force disrupts the circular muscle fibers of the LES, which decreases the hypertonicity. All patients undergoing PD are also prepared for surgical interventions due to the risk of esophageal perforation which is the most serious complication on doing the procedure. The rigiflex dilator is the commonly used balloon dilator currently which is nonradiopaque graded size polyethylene balloon. The correct position of Rigiflex balloon dilator across the LES has to be confirmed before undergoing the procedure for its effectiveness. After dilation, all patients must undergo radiographic testing by Gastrografin study followed by barium esophagram to exclude esophageal perforation.[11] The aim of PD is to neglect the functional obstruction at the level of the gastroesophageal junction. Postdilation LES pressure has been considered the single most important factor for the long-term prediction of clinical response.[12] Patients benefit from a graded approach with increased diameter of 3–4 cm which may provide 90% response rate and 44% response rate at 6 months and 6 years, respectively.[9] The other treatment modalities are also explained which includes pharmacological management of calcium channel blocker (Nifedipine) and long-acting nitrates (sublingual isosorbide dinitrate). Other drugs include sildenafil, atropine, dicyclomine, cimetropium bromide, β-adrenergic agonists (terbutaline), theophylline, and botulinum toxin injection. Overall, pharmacological treatment is the least effective in the management of achalasia cardia.[13] Surgical myotomy, which has the equal effectiveness with the PD can be performed as open or laparoscopic procedure. An endoscopic myotomy approach has been introduced since 2009. It involves creating a tunnel in the esophageal wall, through which the circular muscles of the LES and distal esophagus are resected.[14]


  Conclusion Top


Achalasia is a rare case/cause of dysphagia which affects both genders and all age groups. The onset of the disease is insidious in this case which can be misdiagnosed at the early stage of presentation. Hence, the clinician should have knowledge regarding the suspiciousness of achalasia on clinical basis. The etiology is idiopathic in this case. A clinical history and the barium swallow study support the diagnosis of primary achalasia after ruling out the secondary causes by the CT chest. Even though manometry is the gold standard technique, this case has been diagnosed with clinical history and barium study. The patient has been treated with pneumatic balloon dilation after which the patient improved symptomatically. The prognosis is good on regular follow-up yearly with the symptomatic and radiographic studies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Islam MB, Rahman MK, Sarker AK. Achalasia cardia – Case report. J Teach Assoc 2001;14:85-7.  Back to cited text no. 1
    
2.
Sarumpaet F, Dairi L. A case report of achalasia. IOP Conf Ser Earth Environ Sci 2018;125:012209.  Back to cited text no. 2
    
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Spechler SJ. Achalasia: Pathogenesis, clinical manifestations, and diagnosis. UpToDate. Waltham, MA: UpToDate; 2019.  Back to cited text no. 3
    
4.
Furuzawa-Carballeda J, Torres-Landa S, Valdovinos MÁ, Coss-Adame E, Martín Del Campo LA, Torres-Villalobos G, et al. New insights into the pathophysiology of achalasia and implications for future treatment. World J Gastroenterol 2016;22:7892-907.  Back to cited text no. 4
    
5.
Sarangapani A, Vijayaraghavan S, Shanmuganathan S, Rajanikanth S. Achalasia cardia in an infant. Sri Lanka J Child Health 2010;40:74-5.  Back to cited text no. 5
    
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Kahrilas PJ, Hirano I. Disease of the Esophagus. Harrison's Principle of Internal Medicine. 20th ed., Ch. 316. New York: McGraw-Hill Education; 2018. p. 2213.  Back to cited text no. 6
    
7.
Boeckxstaens GE, Zaninotto G, Richter JE. Achalasia. Lancet 2014;383:83-93.  Back to cited text no. 7
    
8.
Pohl D, Tutuian R. Achalasia: An overview of diagnosis and treatment. J Gastrointestin Liver Dis 2007;16:297-303.  Back to cited text no. 8
    
9.
Patel DA, Lappas BM, Vaezi MF. An overview of achalasia and its subtypes. Gastroenterol Hepatol (N Y) 2017;13:411-21.  Back to cited text no. 9
    
10.
Sharma GL, Kumar A, Mukund A, Kedia A. Atypical presentation of achalasia cardia – A case report. Ind J Radiol Imag 2005;15:175-7.  Back to cited text no. 10
    
11.
Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: Diagnosis and management of achalasia. Am J Gastroenterol 2013;108:1238-49.  Back to cited text no. 11
    
12.
Schlottmann F, Patti MG. Esophageal achalasia: Current diagnosis and treatment. Expert Rev Gastroenterol Hepatol 2018;12:711-21.  Back to cited text no. 12
    
13.
Eckardt AJ, Eckardt VF. Current clinical approach to achalasia. World J Gastroenterol 2009;15:3969-75.  Back to cited text no. 13
    
14.
Williams NS, O'Connell PR, McCaskie AW. Motility Disorder and Diverticula. Bailey & Love's Short Practice of Surgery. 27th ed., Ch.11. Boca Raton, Florida: CRC Press, Taylor & Francis Group; 2018. p. 1099.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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