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Laparoscopic Heller’s cardiomyotomy for paediatric achalasia cardia

Ramnik Patel, Joseph Curry, Edward Kiely, Keith Lindley
Department of Paediatric Surgery, Great Ormond Street Children’s Hospital, London, United Kingdom

 

Abstract

We wish to report a case of achalasia cardia in a young girl who presented with dysphagia and on investigation was found to have achalasia cardia. She underwent laparoscopic Heller’s cardiomyotomy uneventfully. She presented at 5 years with dyspeptic symptoms and repeat investigations did not show any evidence of recurrence or gastro-esophageal reflux disease but esophageal dysmotility was evident.

Key words: Achalasia cardia, Heller’s cardiomyotomy, gastro-esophageal reflux, laparoscopy, laparotomy

 

Correspondence

Ramnik Patel
Department of Paediatric Urology
University College London Hospitals and
Great Ormond Street Children’s Hospital NHS Foundation Trust
London, United Kingdom
Phone: 00447956896641, Fax: 00441162586089
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it. , This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Introduction

Achalasia cardia is an oesophageal motility disorder associated with raised lower oesophageal sphincter (LES) pressure, abnormal peristalsis in the oesophagus, and absence of co-ordination in LES relaxation during swallowing. We wish to report a case which had laparoscopic Heller’s cardiomyotomy uneventfully with a long term follow up.

Case report

A 10-year-old girl presented with few months history of anorexia, progressively worsening vomiting and weight loss.

Initial antireflux treatment did not improve symptoms. Barium swallow showed a dilated fluid and food filled oesophagus with abnormal contractions in the dilated part and a smooth narrowing at the oesophago-gastric junction in keeping with achalasia (Figure 1a). Oesophageal manometry confirmed the diagnosis.

Oesophago-gastro-duodenoscopy showed dilatation of oesophagus with narrowing at lower oesophagus and biopsies showed no evidence of inflammation or infection.

Dietician commenced her on high energy soft feeds. Management options with nifedipine, interventional radiologic or endoscopic dilatation and laparoscopic Heller’s cardiomyotomy were discussed with the parents who selected the latter option.

She underwent laparoscopic Heller’s oesophagocardiomyotomy without any antireflux fundoplication. She recovered well and remained asymptomatic for 5 years when she presented with mild dysphagia and occasional vomiting. Repeat upper gastrointestinal contrast study showed normal calibre oesophagus without any evidence of achalasia or reflux but had significant abnormal oesophageal motility (Figure 1b).

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Figure 1: (A) Barium swallow showing dilated oesophagus with slow contractions and smooth narrowing at lower end with absent gastric air bubble. (B) Post-operative changes at 6 year follow up.

Discussion

Presenting symptoms of vomiting, dysphagia and weight loss mimics gastro-oesophageal reflux and achalasia may be evident on investigation [1-3]. Oesophageal manometry is confirmatory.

Therapeutic valid options include pharmacotherapy with using smooth muscle relaxants such as calcium channel blockers and long acting nitrites,botulinum toxin injections, interventional radiologic or endoscopic balloon dilatation, combined Botox and pneumatic dilatation, peroral endoscopic myotomy and robotic /laparoscopic /thoracoscopic or open oesophagocardiomyotomy with or without antireflux procedure.

The laparoscopic Heller’s oesophagocardiomyotomy is safe, feasible, cosmetic, associated with less post-operative pain and leads to quick recovery. The medical and balloon dilatation options are unpredictable, have short lived effects and may need repeated sessions.

The role of a synchronous fundoplication after oesophagocardiomyotomy has been a controversial subject and some of the studies has shown incidence of gastro-esophageal reflux post cardiomyotomy up to 40 %. This controversy is partly due to the fact that several studies have shown that a fundoplication is not routinely needed after cardiomyotomy as it does not confer a significant benefit to the patients and the total (Nissen) fundoplication may actually increase the risk of post-operative dysphagia due to inherent oesophageal motility disorder in these patients.

However, in selected group of high risk patients, synchronous fundoplication may be considered during cardiomyotomy. The optimal type of partial fundoplication is again debated (posterior 270 degree-Toupet vs. anterior 180 degree-Dor), but partial fundoplication should be favoured over total fundoplication, because it is associated with decreased dysphagia rates and similar reflux control. Additional evidence is required to demonstrate which partial fundoplication provides the best reflux control after myotomy.

Conclusion

Achalasia cardia is rare in children and high index may lead to early diagnosis. Medical and interventional or endoscopic management does not have consistent long term results. Laparoscopic Heller’s cardiomyotomy is safe, effective and cosmetic with rapid recovery.

Conflict of Interest: None

 

 

References

1. Arun S, Senthil R, Bhattacharya A, et al. Incidental detection of pediatric achalasia cardia during gastroesophageal scintigraphy. Clin Nucl Med 2013; 38:228-9.

2. Kale A, Walker J, Natarajan A. Achalasia cardia masquerading as gastroesophageal reflux disease: case report and review of literature. Welsh Paed J 2010; 34:15-17.

3. Zhang Y, Xu CD, Zaouche A, et al. Diagnosis and management of esophageal achalasia in children: analysis of 13 cases. World J Pediatr 2009; 5:56-9.