|Ahead of print publication
An overview of achalasia cardia in children
Rajendra K Ghritlaharey
Department of Pediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals, Bhopal, Madhya Pradesh, India
|Date of Submission||20-Jul-2020|
|Date of Decision||28-Jul-2020|
|Date of Acceptance||07-Aug-2020|
Rajendra K Ghritlaharey,
Department of Pediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals, Bhopal - 462 001, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
Achalasia cardia is an idiopathic, motility disorder of the esophagus, characterized by failure of the lower esophageal sphincter to relax during the deglutition., Achalasia is rare in children with an estimated annual incidence of only 0.02–0.11 cases per 100,000 children, and below 5-year of age, it is more infrequent.,, Most common symptoms relating to the achalasia are regurgitation, vomiting, dysphagia, nocturnal cough, recurrent pneumonia, and poor weight gain/weight loss.,, The “bird's beak” appearance of the lower esophagus on the esophagogram (barium swallow) is a classical radiological finding of the cases of esophageal achalasia.,, Although esophagogram is diagnostic of achalasia, esophagoscopy and manometry are also helpful not only in making a diagnosis, but also for the peroperative and postoperative assessment of the therapy offered for the treatment.,, During the esophageal manometry evaluation, an elevated and nonrelaxing lower esophageal pressure with poor or low peristalsis are characteristic findings with achalasia, but absent of above findings does not exclude the achalasia in children.,,
The objectives of therapy for achalasia are to reduce the lower esophageal sphincter pressure and improve the esophageal emptying. The various modalities used for the treatment of achalasia are; oral nitrates and calcium channel antagonists (blockers), botulinum toxin, pneumatic dilatation, and Heller's cardiomyotomy with or without an anti-reflux procedure.,,,,, The efficacy of the use of endoscopic injection of botulinum toxin at the lower esophageal sphincter as therapeutic option for achalasia in children is not well proven, it require multiple injections and the failure rate is also high. Pneumatic balloon dilatation of the lower esophageal sphincter is also one of the therapies for the achalasia in children, but multiple dilatations are frequently required, and failure rate is also high., Medical therapy offered for the management of achalasia in children often fails, resulting in recurrence of symptoms and ultimately surgical therapy is required.,,
Surgical cardiomyotomy is definitive, most effective, and successful treatment for achalasia, not only in adults, but also in children.,,, Ernst Heller (1877–1964), Director of the surgical clinic of the University of Leipzig in Germany, while operating a patient with idiopathic achalasia of the esophagus, he performed extramucosal cardiomyotomy for the first time on April 14, 1913. Heller cardiomyotomy remains the treatment of choice for achalasia cardia, it can be executed through open or laparoscopic technique and can also be accomplished by thoracic or abdominal approach. Heller myotomy done for the surgical correction of achalasia is done either alone or added with fundoplication as an anti-reflux procedure.,,, With the advancement in the field of minimal invasive surgical techniques, laparoscopic Heller myotomy with an anti-reflux procedure/partial fundoplication is a gold standard procedure for the correction of achalasia cardia, not only in adults, but also in children. The added advantages of minimal invasive surgery are minimal postoperative pain, better cosmetic result, shorter hospital stay, and faster recovery as well.,, Per-oral endoscopic myotomy is a new endoscopic technique for the treatment of esophageal achalasia with excellent results in adults, has also been added as a newer technique for children. More clinical studies/trials are required to establish not only the safety and efficacy, but also the feasibility of this technique to treat esophageal achalasia in children. Robotic-assisted Heller cardiomyotomy has also been added recently as a newer modality for the treatment of esophageal achalasia in children. Dor fundoplication is most frequently done along with Heller myotomy in children to prevent gastroesophageal reflux, although the role of fundoplication is debatable, and it is not clear whether all children should undergo a concomitant anti-reflux procedure or not.,,,
Complications may occur during or following the surgical procedures done for the treatment of achalasia in children as well, and are esophageal perforation, recurrent dysphagia, and gastroesophageal reflux.,,, Perforation of the esophagus during Heller myotomy is reported in 0%–20% of the cases.,,, Esophageal perforation is mostly detected during the surgical procedure, and very rarely during the postoperative period. If the perforation is detected during operative procedure, it is manageable and required repair of the perforation. Esophageal perforations detected during postoperative period are managed either by aggressive conservative therapy, re-operation and repair of the perforation, or by placement of self-expanding plastic stent. The decision regarding which therapy to be instituted depends on the duration of perforation, amount of leakage, and the clinical condition of the patient.,,, [6,]
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