International Journal of Phonosurgery & Laryngology

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VOLUME 12 , ISSUE 1 ( January-June, 2022 ) > List of Articles


Congenital Cricopharyngeal Achalasia in an Infant Treated by External Cricopharyngeal Myotomy: A Case Report

S Vivek, Jayakumar R Menon, Manju E Issac, Jayanthy Pavithran

Keywords : Achalasia, Aspiration, Cricopharyngeus, Dysphagia, Myotomy

Citation Information : Vivek S, Menon JR, Issac ME, Pavithran J. Congenital Cricopharyngeal Achalasia in an Infant Treated by External Cricopharyngeal Myotomy: A Case Report. Int J Phonosurg Laryngol 2022; 12 (1):16-18.

DOI: 10.5005/jp-journals-10023-1230

License: CC BY-NC 4.0

Published Online: 30-07-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Congenital cricopharyngeal atresia is a rare but serious cause of dysphagia in infants. The failure of the cricopharyngeus muscle (CPM) to adequately relax on food intake is considered as the cause. This condition usually presents early in life with symptoms that include regurgitation, nasopharyngeal reflux, aspiration, choking episodes, and failure to thrive. Videofluoroscopy helps to clinch the diagnosis, demonstrating a prominent cricopharyngeal bar in most cases. This describes the case report of a male infant who presented with cricopharyngeal achalasia (CPA) and underwent open cricopharyngeal myotomy at 6 months of age, leading to the resolution of symptoms.

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  1. Jackson C. Diseases of the esophagus. In: Jackson C, (ed.) Peroral endoscopy and laryngeal surgery. St Louis (Mo): Laryngoscope Co 1915;507–508.
  2. Utian HL, Thomas RG. Cricopharyngeal incoordination in infancy. Pediatrics 1969;43:399–406. DOI: 10.1542/peds.43.3.402
  3. Bedwell JR. Cricopharyngeal dysfunction in children. Pediatric Dysphagia 2018;207–213. Springer, Cham.
  4. Sewell RK, Bauman NM. Congenital cricopharyngeal achalasia: management with botulinum toxin before myotomy. Arch Otolaryngol Head Neck Surg 2005;131(5):451–453. DOI: 10.1001/archotol.131.5.451
  5. Salib SA, Aubert D, Valioulis I, et al. Cricopharyngeal achalasia–a cause of major dysphagia in a newborn. A case report. Eur J Pediatr Surg 1999;9(6):406–408. DOI: 10.1055/s-2008-1072293
  6. Korakaki E, Hatzidaki E, Manoura A, et al. Feeding difficulties in a neonate with primary cricopharyngeal achalasia treated by cricopharyngeal myotomy. Int J Pediatr Otorhinolaryngol 2004;68(2):249–253. DOI: 10.1016/j.ijporl.2003.10.008
  7. Kuhn MA, Belafsky PC. Management of cricopharyngeus muscle dysfunction. Otolaryngol Clin N Am 2013;46(6):1087–1099. DOI: 10.1016/j.otc.2013.08.006
  8. Goldani HA, Staiano A, Borrelli O, et al. Pediatric esophageal high-resolution manometry: utility of a standardized protocol and size-adjusted pressure topography parameters. Am J Gastroenterol 2010;105(2):460–467. DOI: 10.1038/ajg.2009.656
  9. Nikaki K, Ooi JL, Sifrim D. Chicago classification of esophageal motility disorders: applications and limits in adults and pediatric patients with esophageal symptoms. Curr Gastroenterol Rep 2016;18(11):59. DOI: 10.1007/s11894-016-0532-y
  10. Sondheimer JM. Upper esophageal sphincter and pharyngoesophageal motor function in infants with and without gastroesophageal reflux. Gastroenterology 1983;85(2):301–305. DOI: 10.1016/0016-5085(83)90314-1
  11. Scholes MA, McEvoy T, Mousa H, et al. Cricopharyngeal achalasia in children: botulinum toxin injection as a tool for diagnosis and treatment. Laryngoscope 2014;124(6):1475–1480. DOI: 10.1002/lary.24464
  12. Jain V, Bhatnagar V. Cricopharyngeal myotomy for the treatment of cricopharyngeal achalasia. J Pediatr Surg 2009;44(8):1656–1658. DOI: 10.1016/j.jpedsurg.2009.04.031
  13. Blank RH, Silbiger M. Cricopharyngeal achalasia as a cause of respiratory distress in infancy. J Pediatr 1972;81(1):95–98. DOI: 10.1016/S0022-3476(72)80383-4
  14. De Caluwe D, Nassogne MC, Reding R, et al. Cricopharyngeal achalasia: case reports and review of the literature. Eur J Pediatr Surg 1999;9(2):109–112. DOI: 10.1055/s-2008-1072223
  15. Erdeve O, Kologlu M, Saygili B, et al. Primary cricopharyngeal achalasia in a newborn treated by balloon dilatation: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 2007;71(1):165–168. DOI: 10.1016/j.ijporl.2006.08.012
  16. Skinner MA, Shorter NA. Primary neonatal cricopharyngeal achalasia: a case report and review of the literature. J Pediatr Surg 1992;27(12):1509–1511. DOI: 10.1016/0022-3468(92)90487-r
  17. Muraji T, Takamizawa S, Satoh S, et al. Congenital cricopharyngeal achalasia: diagnosis and surgical management. J Pediatr Surg 2002;37(5):E12. DOI: 10.1053/jpsu.2002.32298
  18. Brooks A, Millar AJ, Rode H. The surgical management of cricopharyngeal achalasia in children. Int J Pediatr Otorhinolaryngol 2000;56(1):1–7. DOI: 10.1016/s0165-5876(00)00365-7
  19. Martin N, Prince JM, Kane TD, et al. Congenital cricopharyngeal achalasia in a 4.5-year-old managed by cervical myotomy: a case report. Int J Pediatr Otorhinolaryngol 2011;75(2):289–292. DOI: 10.1016/j.ijporl.2010.11.013
  20. Chun R, Sitton M, Tipnis NA, et al. Endoscopic cricopharyngeal myotomy for management of cricopharyngeal achalasia (CA) in an 18-month-old child. Laryngoscope 2013;123(3):797–800. DOI: 10.1002/lary.23545
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