Achalasia cardia in an infant - were we too late?
DOI:
https://doi.org/10.18203/2349-3291.ijcp20222776Keywords:
Achalasia cardia, Infant achalasia, MyotomyAbstract
Achalasia cardia is a motility disorder where there is aperistalsis of the body of the esophagus and failure of lower esophageal sphincter relaxation. This manifests as a functional gastroesophageal junction obstruction. This disorder is rare and has a prevalence of 10 in 100,000 population. It has a peak incidence between 30-60 years of age. The incidence in the pediatric population is very low and is even rarer in infants. Here we present a case of achalasia cardia in a 9-month-old male infant weighing 3.1 kilograms. The child had complaints of regurgitation of milk, non-projectile vomiting, fever and cough for the past 3 months along with failure to thrive. He was treated for bronchopneumonia initially and then evaluated with a barium swallow. The barium swallow revealed a hold-up of contrast in the distal esophagus along with a typical bird-beak appearance. Definitive surgical intervention was being planned. However, the child aspirated and expired. Did we delay the procedure? Were we too late? These questions continue to make us self-introspect on the management of infantile achalasia cardia. The paucity of available publications and data is a major roadblock in management.
References
O'Neill OM, Johnston BT, Coleman HG. Achalasia: a review of clinical diagnosis, epidemiology, treatment and outcomes. World J Gastroenterol. 2013;19(35):5806-12.
Hong J. Clinical applications of gastrointestinal manometry in children. Pediatr Gastroenterol Hepatol Nutr. 2014;17(1):23-30.
Franklin AL, Petrosyan M, Kane TD. Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management. World J Gastrointest Endosc. 2014;6(4):105-11.
Ghoshal UC, Daschakraborty SB, Singh R. Pathogenesis of achalasia cardia. World J Gastroenterol. 2012;18(24):3050-7.
Banerjee R, Prasad A, Kumar V, Wadhwa N. Infantile Achalasia Cardia. Indian Pediatr. 2016;53(9):831-2.
Huebner A, Kaindl AM, Knobeloch KP, Petzold H, Mann P, Koehler K. The triple A syndrome is due to mutations in ALADIN, a novel member of the nuclear pore complex. Endocr Res. 2004;30(4):891-9.
Dughera L, Chiaverina M, Cacciotella L, Cisarò F. Management of achalasia. Clin Exp Gastroenterol. 2011;4:33-41.
Patti MG, Fisichella PM. Controversies in management of achalasia. J Gastrointest Surg. 2014;18(9):1705-9.
Chatterjee S, Gajbhiye V, De A, Nath S, Ghosh D, Das SK. Achalasia cardia in infants: report of two cases. JIMA. 2013;44(1).