Page 74 - World Journal of Laparoscopic Surgery

CASE REPORT
Pediatric Achalasia: A Rare Differential for Failure to Thrive

in a 4-year-old Child

1
2
Dhananjay Pandey , Lokesh Yadav , Lakshmi Kona 3
AbstrAct
Introduction: Achalasia cardia (AC) is a primary motility disorder of esophagus, characterized by aperistalsis and defective relaxation of lower
esophageal sphincter. It is predominantly a disease of adults and the incidence in children is extremely rare, 0.11 in 100,000. The presenting
symptoms among children predominantly are dysphagia, regurgitation, vomiting, and failure to thrive. The diagnosis is made by barium studies
and esophageal manometry. Per oral endoscopic myotomy (POEM) is a novel technique in adult population but its efficacy and safety in pediatric
population is not known. Cardiomyotomy is the treatment of choice for childhood achalasia.
Case description: A 4-year-old boy presented to us with complaints of recurrent vomiting since 6 months of age and failure to thrive. He
used to vomit immediately after ingestion of both solids and liquids. He had history of bronchopneumonia at around 1 year of age. He was
malnourished and less than the third percentile for his age. His barium esophagogram (Fig. 1) showed persistent narrowing at the lower
end of esophagus with proximal dilatation suggestive of achalasia. He was nutritionally rehabilitated and taken up for laparoscopic Heller’s
cardiomyotomy. Post-surgery, he improved well and was able to tolerate both solids and liquids. On follow-up, he had gained weight and
was feeding normally.
This case highlights the importance of recognizing the fact that achalasia though rare can present in pediatric age-group as well. Diagnosis
is usually delayed or misdiagnosed as gastroesophageal reflux disease (GERD), esophageal webs, etc. Patients usually become extremely
malnourished and developmental milestones are delayed. Hence, the early diagnosis and treatment with cardiomyotomy is the key.
Keywords: Cardiomyotomy, Malnourishment, Pediatric achalasia, Per oral endoscopic myotomy.
World Journal of Laparoscopic Surgery (2022): 10.5005/jp-journals-10033-1509

IntroductIon 1 Institute of Minimal Access, Bariatric, and Robotic Surgery, Max Super
Achalasia cardia is a primary motility disorder of esophagus, Specialty Hospital, Ghaziabad, Uttar Pradesh, India
characterized by aperistalsis, raised/normal lower esophageal 2 Department of Surgical Gastroenterology, SMS Medical College and
sphinter (LES) pressure and defective lower esophageal relaxation. Hospital, Jaipur, Rajasthan, India
It is predominantly a disease of adults and extremely rare in children 3 Department of Minimal Access, Bariatric and Surgical Gastroenterology,
1,2
with a reported incidence of 0.11 in 100,000. Per se, there is no Gleneagles Global Hospital, Hyderabad, Telangana, India
population-based epidemiological study in children in India. The Corresponding Author: Dhananjay Pandey. Institute of Minimal
presenting symptoms in children predominantly are vomiting, Access, Bariatric, and Robotic Surgery, Max Super Specialty Hospital,
regurgitation, recurrent chest infections, and failure to thrive. The Ghaziabad, Uttar Pradesh, India, Phone: +91 8588001386, e-mail:
diagnosis is made by barium studies, upper gastrointestinal (GI) pandey.dhananjay83@gmail.com
endoscopy and esophageal manometry. Heller’s myotomy is the most How to cite this article: Pandey D, Yadav L, Kona L. Pediatric Achalasia:
preferred treatment for achalasia in pediatric population as well. Per A Rare Differential for Failure to Thrive in a 4-year-old Child. World J
oral endoscopic myotomy is a novel technique in adult population Lap Surg 2022;15(2):170–173.
but its efficacy and safety in pediatric population is not clearly known. Source of support: Nil
Conflict of interest: None
cAse descrIptIon
A 4-year-old boy presented to us with complaints of recurrent
vomiting since 6 months of age and failure to thrive. History of been 16.5 kg with height of 105.7 cm. He was less than the third
vomiting immediately after ingestion of solids and liquids as well percentile as per centres for disease control and prevention (CDC)
and history of recurrent chest infections was present. His barium growth chart 2004; hence, severely malnourished.
esophagogram (Fig. 1) revealed a persistent narrowing at the lower After the nutritional assessment, a plan was made to
end of esophagus with proximal dilation and minimal passage of improve his nutritional status by giving him parental nutrition
contrast into stomach. Findings were consistent with achalasia (target calories, 1,350 kcal; protein, 24 gm approximately) before
cardia. Esophagogastroduodenoscopy (Fig. 2) showed dilated taking up the patient for any surgical intervention. His blood
esophagus with food residues and narrowing of esophagogastric biochemistry revealed deranged electrolytes which was corrected
junction with non-passage of scope beyond it. High-resolution in the meantime. After a week of nutritional supplementation and
manometry (HRM) could not be done due to technical difficulties. correction of dyselectrolytemia, the patient was reassessed and
At presentation, the boy weighed 8.1 kg, his height was evaluated by our anesthesia and nutritional team and plan was
91 cm, and his body mass index (BMI) was 9.78; hence, severely made to proceed with surgery (laparoscopic Heller’s cardiomyotomy
malnourished (Fig. 3). His ideal bodyweight as per age should have with fundoplication).

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