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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 96-98

Achalasia in a child confirmed by high-resolution manometry


1 Department of Pediatric Medicine, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
2 Department of Pediatric Surgery, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Date of Web Publication18-Nov-2019

Correspondence Address:
Dr. Anirban Chatterjee
Flat-BB29/6, Salt Lake, Sector-1, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ami.ami_14_19

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  Abstract 

Achalasia is a primary motor disease of the esophagus. It is rare in the pediatric population; fewer than 5% of patients are below 15 years of age. Progressive dysphagia is the most common presentation in adults. The clinical presentation in children does differ from adults. The various clinical features are nocturnal cough, recurrent pneumonia, vomiting, and feeding difficulties. The symptoms of achalasia may mimic gastroesophageal reflux disease in children. We report a 4-year-old boy presented with increasing difficulty in swallowing. Esophagram showed a smooth narrowing of the distal end and dilated esophagus. High-resolution manometry (HRM) had confirmed the diagnosis by fulfilling the criteria for achalasia Type 2. HRM has high sensitivity than conventional and may predict prognosis. He underwent Heller's myotomy with an anti-reflux procedure. The patient was asymptomatic after curative surgery. We report a rare case of achalasia in a 4-year-old boy confirmed by novel diagnostic technique HRM; hence, pediatric data of HRM are limited.

Keywords: Achalasia, high-resolution manometry, pediatric


How to cite this article:
Chatterjee A, Saha S. Achalasia in a child confirmed by high-resolution manometry. Acta Med Int 2019;6:96-8

How to cite this URL:
Chatterjee A, Saha S. Achalasia in a child confirmed by high-resolution manometry. Acta Med Int [serial online] 2019 [cited 2019 Dec 14];6:96-8. Available from: http://www.actamedicainternational.com/text.asp?2019/6/2/96/271105


  Introduction Top


Achalasia is characterized manometrically[1] by impaired synchronized relaxation of the lower esophageal sphincter (LES) and aperistalsis in the body of the esophagus. It creates functional obstruction of the esophagus.

The etiology is unknown.[2] Viral infection, autoimmune, or neurodegenerative process can destroy ganglion cells in the myenteric plexus. Consequently, lack of the release of neuroinhibitory substances, nitric oxide, and vasoactive intestinal polypeptide from inhibitory neurons has been diminished. Eventually, excitatory cholinergic becomes dominant and its uninhibited activity is the cause of manometric presentation of achalasia.[1],[2]

The diagnosis is made by clinical history and radiography. Achalasia is confirmed by manometry.

Esophageal manometry is a procedure to determine how various factors come to play in the motility of the esophagus and function of the sphincter by measuring pressures developed by the esophageal muscles and the sphincter. A catheter is introduced from the nostril and is placed beyond the esophagogastric junction. The catheter carries a sensor that captures intraesophageal pressure signals and transmits those signals to receiving gadget. The reordered signals in receiving device represent the data.

New diagnostic manometry – high-resolution manometry (HRM) – is replacing conventional manometry. The distinguishing points between HRM and conventional presented in [Table 1]. Recent data coming out from HRM are showing greater sensitivity than the conventional one.[1],[3] Furthermore, HRM also predicts treatment outcome in adults. There is the dearth of HRM experience in children.[4]
Table 1: The difference between high resolution manometry and conventional manometry

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The treatment is aimed to the reduction of LES pressure and smooth esophageal emptying. Myotomy with partial fundoplication is most commonly recommended. The outcome is excellent with normal life expectancy.

We report the rare of achalasia and describe HRM in a child.


  Case Report Top


A 4-year-old boy presented with increasing difficulty of swallowing for the past 3 months. At the time of admission, he had persisting vomiting, some dehydration, and pallor, with weight being 12 kg and height 96.5 cm (weight-for-height Z score <2 standard deviation). No abnormality was found on physical examination. Before admission, he has been receiving a proton-pump inhibitor trial in suspicion of gastroesophageal reflux disease (GERD), but that did not work. A routine blood test showed anemia. Serum creatinine, sugar, and electrolytes were normal. Upper gastrointestinal endoscopy showed large dilated esophagus and retained food material. The esophagram revealed smooth narrowing of the esophagus near to the cardia with a hugely dilated esophagus [Figure 1] and slow passage of contrast and no GERD demonstrated. HRM tracing [Figure 2] revealed aperistalsis and absent LES relaxation that is definitive diagnostic criteria for achalasia.
Figure 1: Esophagogram shows classical “bird beak” appearance tapering of massively dilated distal esophagus

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Figure 2: High-resolution manometry tracing [Figure 2] revealed aperistalsis and absent lower esophageal sphincter relaxation that is definitive diagnostic criteria for achalasia

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After initial conservative management nutrition by nasogastric tube, he was transferred to the pediatric surgery department. He was managed by Heller's cardiomyotomy and anterior fundoplication that was uncomplicated. The boy started to gain weight and nutritional recovery.


  Discussion Top


Achalasia is a primary motility disease of the esophagus, presents with a slowly progressive manner.

The incidence of this rare disease is 2 per 105 per year[5] and peak incidence age group is 30–60 years.[6] It is rarely symptomatic in the pediatric population with an incidence of 0.11 per 105 children per year and <5% of patients are below 15 years of age.[4] A high prevalence in male (male:female = 6:1)[7] is reported, and the mean age of presentation in children approximately is 5 years.[8] Our patient is male and admitted in the institute at the age of 4 years.

The clinical presentation in pediatrics is very different than adults and depends on age. The most common symptoms in children are regurgitation, weight loss, and recurrent respiratory tract infections which were found 79.1%, 79.1%, and 73.9%, respectively.[8] Our patient had dysphagia that is also a common symptom[4] and presented in 62.5%[8] of children. The manifestations in infants are atypical: aspiration, difficulty in feeding, nocturnal cough, and pneumonia. Tracheal obstruction by achalasia is a very rare complication.[9] Our patient was initially treated for GERD. Similar wrong diagnosis has been reported and 50% cases are initially misdiagnosed.[4],[7]

Achalasia is suspected by clinical history and is supported by barium swallow studies. The assessment of esophageal motor function by esophageal manometry is strongly recommended[10] to confirm the diagnosis.[4] Although manometry considered as the gold standard for diagnosis, it is not available even in tertiary referral center.[8]

The investigations such as barium esophagram and endoscopy are complementary for management.[10] The esophagram revealed smooth narrowing of the esophagus near to cardia with a hugely dilated esophagus ([Figure 1]) and slow passage of contrast and no GERD demonstrated.

New diagnostic manometry HRM is replacing conventional manometry. Recent data coming out from HRM are showing greater sensitivity than the conventional one.[1],[3]

Aperistalsis is an inability of contractile movement of the esophagus with various pressures in the esophagus. HRM analyzes the dynamic pressure signals through esophagus and depicts continuous topographical esophageal pressure which identifies motor patterns and prognosis after treatment in adults.[1] Three motor patterns of achalasia are recognized in adults according to topographic presentations of body of the esophagus by this novel diagnostic procedure HRM. Those phenotypes are Type 1 – absence of pressurization, Type 2 – isobaric pressurization, and Type 3 – spastic, pressurization with contraction. Type III has worse prognosis than Type I and II.[1]

There is the dearth of HRM experience in children.[4] Our case revealed aperistalsis of the esophagus and high pressure at LES that depiction in our case corresponds to phenotype 2 of the adult. Furthermore, heterogeneous behavior of LES in children needs more experience with HRM for achalasia diagnosis.[11]

HRM has the highest sensitivity to diagnose achalasia comparing others modalities[3],[8] and replacing the conventional manometry.[4] Furthermore, pediatric data of HRM are limited.[4] We have confirmed the achalasia by HRM.

Surgery is the gold standard management of achalasia.[12] Cardiomyotomy is the recommended choice for also in the pediatric age group.[8] Two most common complications of surgery in published series are (i) recurrence of dysphagia (0%–26%)[12] and (ii) perforation of the esophagus (0%–15%).[12]

Overall prognosis is excellent in 90% achalasia.[1] Our case is pursuing a normal life without any complications.

Declaration of patient consent

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

Acknowledgments

We thank the patient and family for giving us informed consent to publish this case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Patel DA, Kim HP, Zifodya JS, Vaezi MF. Idiopathic (primary) achalasia: A review. Orphanet J Rare Dis 2015;10:89.  Back to cited text no. 1
    
2.
Park W, Vaezi MF. Etiology and pathogenesis of achalasia: The current understanding. Am J Gastroenterol 2005;100:1404-14.  Back to cited text no. 2
    
3.
Ghosh SK, Pandolfino JE, Rice J, Clarke JO, Kwiatek M, Kahrilas PJ, et al. Impaired deglutitive EGJ relaxation in clinical esophageal manometry: A quantitative analysis of 400 patients and 75 controls. Am J Physiol Gastrointest Liver Physiol 2007;293:G878-85.  Back to cited text no. 3
    
4.
Rebeca GR, Nayeli XO, Marina GM, Judith FC. Achalasia in pediatric population: Use of high-resolution manometry in children, achalasia in pediatric population. J Gastrointest Dig Syst 2015;5:286.  Back to cited text no. 4
    
5.
Sadowski DC, Ackah F, Jiang B, Svenson LW. Achalasia: Incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil 2010;22:e256-61.  Back to cited text no. 5
    
6.
O'Neill OM, Johnston BT, Coleman HG. Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomes. World J Gastroenterol 2013;19:5806-12.  Back to cited text no. 6
    
7.
Fernandez PM, Lucio LA, Pollachi F. Esophageal achalasia of unknown etiology in children. J Pediatr (Rio J) 2004;80:523-6.  Back to cited text no. 7
    
8.
Singh S, Wakhlu A, Pandey A, Kureel SN, Rawat J. Retrospective analysis of paediatric achalasia in India: Single centre experience. Afr J Paediatr Surg 2012;9:117-21.  Back to cited text no. 8
  [Full text]  
9.
Lee CW, Kays DW, Chen MK, Islam S. Outcomes of treatment of childhood achalasia. J Pediatr Surg 2010;45:1173-7.  Back to cited text no. 9
    
10.
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. 10
    
11.
Morera C, Nurko S. Heterogeneity of lower esophageal sphincter function in children with achalasia. J Pediatr Gastroenterol Nutr 2012;54:34-40.  Back to cited text no. 11
    
12.
Franklin AL, Petrosyan M, Kane TD. Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management. World J Gastrointest Endosc 2014;6:105-11.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

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