Esophageal achalasia of unknown etiology in infants

  • Vijay D. UpadhyayaEmail author
  • A. N. Gangopadhyaya
  • D. K. Gupta
  • S. P. Sharma
  • Vijayendra Kumar
  • S. C. Gopal
Clinical Summary



Achalasia cardia is an uncommon disease in children particularly in infants. We present 8 cases of achalasia who were encountered over a 12-year period. In infantile achalasia, respiratory symptoms predominate and vomiting may easily be mistaken for gastroesophageal reflux (GER). Vomiting of uncurdled milk is characteristic of achalasia.


In this retrospective study, the data were obtained from records of the Department of Pediatric Surgery IMS, BHU Varanasi, India. The patients were diagnosed by clinical examination and barium study. Any other associated anomalies were noted in these patients. The patients underwent esophagocardiomyotomy with antireflux procedure via the abdominal route.


In the present series, 7 patients survived. The follow-up study after operation showed remarkable relief of symptoms with satisfactory weight gain. No post-operative death occurred in the patients. Achalasia associated with alacrimia was noted in one infant who was lost to follow up. One infant was initially diagnosed as having gastric volvulus, but exploration revealed achalasia cardia.


Esophageal achalasia is a rare disease in children and its origin is generally indeterminable. Achalasia cardia should be emphasized in the differential diagnosis of an infant presenting with signs and symptoms of esophageal obstruction.

Key words

esophageal achalasia esophagomyotomy etiology gastroesophageal reflux infants 


  1. 1.
    Hamza AF, Awad HA, Hussein O. Cardiac achalasia in children. Dilatation or surgery? Eur J Pediatr Surg 1999;9:299–302.PubMedCrossRefGoogle Scholar
  2. 2.
    Jolley SG, Baron HI. Disorders of esophageal function. In: O’Neill JA, eds. Pediatric Surgery, 5th ed. Boston: Mosby, 2000: 997–1007.Google Scholar
  3. 3.
    Ashcraft KW. The esophagus. In: O’Neill JA, eds. Pediatric Surgery, 5th ed. Boston: Mosby, 2000: 325–347.Google Scholar
  4. 4.
    Patti MG, Albanese CT, Holcomb GW 3rd, Molena D, Fisichella PM, Perretta S, et al. Laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia in children. J Pediatr Surg 2001;36:1248–1251.PubMedCrossRefGoogle Scholar
  5. 5.
    Porras G, Hernández MH, Izundegui G, Porras JD. Selecting the surgical procedure for simple and complicated esophageal achalasia in children. J Pediatr Surg 1997;32:1595–1597.PubMedCrossRefGoogle Scholar
  6. 6.
    Nakajima K, Wasa M, Kawahara H, Soh H, Taniguchi E, Ohashi S, et al. Laparoscopic esophagomiotomy with Dor anterior fundoplicacion in a child with achalasia. Surg Laparosc Endosc Percutan Tech 1999;10:236–239.CrossRefGoogle Scholar
  7. 7.
    Chelimsky G, Hupertz V, Blanchard T. Manometric progression of achalasia. J Pediatr Gastroenterol Nutr 2000;31:303–306.PubMedCrossRefGoogle Scholar
  8. 8.
    Tovar JA, Prieto G, Molina M, Arana J. Esophageal function in achalasia: preoperative and postoperative manometric studies. J Pediatr Surg 1998;33:834–838.PubMedCrossRefGoogle Scholar
  9. 9.
    Lan LC, Wong KK, Lin SC, Sprigg A, Clarke S, Johnson PR, et al. Endoscopic balloon dilatation of eshophageal strictures in infants and children: 17 years’ experience and a literature review. J Pediatr Surg 2003;38:1712–1715.PubMedCrossRefGoogle Scholar
  10. 10.
    Rothenberg SS, Patrick DA, Bealer JF, Chang JH. Evaluation of minimally invasive approaches to achalasia in children. J Pediatr Surg 2001;36:808–810.PubMedCrossRefGoogle Scholar
  11. 11.
    Morris-Stiff G, Khan R, Foster ME, Lari J. Long-term results of surgery for childhood achalasia. Ann R Coll Surg Engl 1997;79:432–434.PubMedGoogle Scholar
  12. 12.
    Liu JF, Zhang J, Tian ZQ, Wang QZ, Li BQ, Wang FS, et al. Long-term outcome of esophageal myotomy for achalasia. World J Gastroenterol 2004;10:287–291.PubMedGoogle Scholar
  13. 13.
    Mattioli G, Esposito C, Pini Prato A, Doldo P, Castagnetti M, Barabino A, et al. Results of the laparoscopy Heller-Dor procedure for pediatric esophageal achalasia. Surg Endosc 2003;17:1650–1652.PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2008

Authors and Affiliations

  • Vijay D. Upadhyaya
    • 1
    Email author
  • A. N. Gangopadhyaya
    • 1
  • D. K. Gupta
    • 1
  • S. P. Sharma
    • 1
  • Vijayendra Kumar
    • 1
  • S. C. Gopal
    • 1
  1. 1.Department of Pediatric SurgeryIMS, BHUVaranasiU. P. India

Personalised recommendations