Page 76 - World Journal of Laparoscopic Surgery

Laparoscopic Management of Pediatric Achalasia
infants. No familial association has been noted and is found to be
more common in male child. 5–8 One of the reasons thought to be
responsible for low incidence reported in pediatric population is
inability to differentiate it from conditions with similar presentation.
5,6
It is often confused with GERD, delaying correct diagnosis.
Typically, symptoms in adults and young children consist of
dysphagia to solids and liquids, regurgitation, weight loss, and chest
pain. It should also be noted that infant as well as preschool children
will not always be able to complain of dysphagia. Therefore, the
presentation in this subgroup of patients will primarily consist of
recurrent vomiting, regurgitation of feeds, failure to thrive/weight
Figs 4A and B: Intraoperative image showing myotomy and Dor loss or recurrent chest infection. Majority of these patient end up in
fundoplication pediatric clinic in place of surgical clinics that could lead to delayed
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presentation in them by 6–10 years. Diagnosis is probably delayed
due to misdiagnosis or presence of associated diseases. Therefore,
it is advised to consider achalasia in differential diagnosis in this
subset of patients.
Achalasia has been found to be associated with Chaga’s disease,
Allgrove syndrome, Congenital hypoventilation syndrome, eating
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disorders, trisomy 21 to name a few. It is also important to confirm
diagnosis prior to instituting treatment and rule out congenital/
acquired causes of OG junction obstruction.
Tools to diagnose this condition are well established. Esoph-
agogram is diagnostic in majority. Esophagogastroduodenoscopy
should be done to rule out other condition that can cause OG
junction obstruction or can be associated with achalasia. High-
resolution manometry is considered investigation of choice for
diagnosing this disorder. However, HRM is not possible in all cases
due to various reasons.
The treatment aims at providing palliation that can be achieved
by lowering the pressure gradient across LES as no treatment
Fig. 5: Follow-up image of child at 9 months reverses underlying neuropathological process. Various treatment
options are available; pharmacological, botulinum injections,
and esophagus to avoid inadvertent injury to esophagus. Anterior endoscopic balloon dilatation (EBD), and open Heller’s myotomy
vagus nerve identified and preserved. As the plan was to do anterior (HM)/laparoscopic Heller’s myotomy (LHM). Calcium channel
fundoplication, esophagus was not dissected posteriorly. Abdominal blockers (CCB) are the most commonly used drugs but are not
part of esophagus was defined and was taken control off by pulling advised in children in view of side effect profile and short-term
cardioesophageal junction by holding pad of fat through left axillary effectiveness. 10–13 Endoscopic injections of botulinum toxin at LES
port. A 12 Fr nasogastric tube was placed orally, over which repair are also reported in children without long-lasting effectiveness.
was done. Myotomy started 2–3 cm above the esophagogastric (OG) Its use described as a bridge to EBD/HM or in cases where later
junction, extended proximally for 5–6 cm and then approximately not possible. Endoscopic balloon dilatation has been described in
2 cm on stomach side (Fig. 4). Longitudinal and circular muscle layer children for long with initial data showing favorable results. Various
were split bluntly providing good exposure to underlying mucosa. studies reveal good short-term results in older children. However, it
Judicious hemostasis achieved using mechanical pressure with is found to be technically difficult in younger (<7 years) children.
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no use of energy sources. Fundus of stomach was mobilized and The risk of recurrence reaches 100% during long-term follow-up,
used for anterior Dor fundoplication using non-absorbable sutures and young age at presentation is an independent predictive
(Fig. 4). Intraoperative period was uneventful. Gastrografin study was factor for the need for repeated treatment. 2,15 The recent and
done on postoperative day (POD) 1 which showed a normal passage majority of data reveal recurrent symptoms in majority requiring
of contrast. The patient tolerated liquid and semisolid diet well and re-interventions.
was discharged on POD3 on soft diet for 1 month, following which Schoenberg et al. in his meta-analysis demonstrated superiority
normal diet was continued. of myotomy over EBD in both short and long-term efficacy.
16
On follow-up at 6 months, weight gain was 5 kg (weight was Heller’s myotomy (lap/open) is an established procedure in
13 kg with a height of 98 cm, and was feeding normally. At 9 months, adults with proven superiority to other means of treatment. With
weight was 15 kg with a height of 102 cm (more than 50th centile) increasing experience, LHM is increasingly being performed in
(Fig. 5).
children as well compared to open HM. The data suggest success
rate of more than 80% for surgical repair in long term studies in
dIscussIon adults. For pediatric achalasia, long term permanent success rate
Achalasia cardia is a primary esophageal motility disorder is highest with myotomy compared to other means of treatment
considered to be neurodegenerative in origin. It is exceedingly rare but these are based on small studies. 2,11 However, the data also
3,4
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in first two decades of life (5%) with only few cases reported in reveal intervention in up to 28% patient in follow-up. The most

172 World Journal of Laparoscopic Surgery, Volume 15 Issue 2 (May–August 2022)