(Spasm of the crico-pharyngeal muscle)

Rare functional disorder, sometimes referred to as cricopharyngeal achalasia, due to lack of or incomplete relaxation of the cricopharyngeal muscle as the food bolus passes from the hypopharynx into the esophagus. The cricopharyngeal muscle is the main component of the upper esophageal sphincter, along with the lower pharyngeal constrictor muscle. This pathology is often associated with a neurological problem: spastic tetraparesis, myelomeningocele, Arnold-Chiari malformation, trisomy 21, myotonia, Kearns-Sayre syndrome (see these terms).

Clinical signs:

-        clinical onset between birth and 6 months of age; in older children, this problem is sometimes associated with the presence of a cricopharyngeal diverticulum, which contains all 3 tissue layers of the esophagus, as opposed to a pulsion-type diverticulum, which contains only the mucosa covered by a thin muscular layer

-        feeding difficulties: immediate regurgitation, choking episodes, coughing, dysphagia, nasal food reflux,

-        recurrent respiratory infections.

Diagnosis is difficult (dynamic opacification, esophageal manometry). Palliative treatment: tube feeding or gastrostomy, sometimes administration of nifedipine before meals.

Curative treatment: either endoscopic balloon dilatation, extramucosal myotomy of the cricopharyngeal muscle by cervical approach (anterior or posterior), endoscopic laser myotomy or botulinum toxin injection.

This disease should not be confused with the transient crico-pharyngeal incoordination of the newborn: the signs are identical to those of esophageal fistula or laryngeal diastema, but there is no anatomical abnormality. This incoordination disappears at around 6 months of age, and until then the infant is fed by feeding tube.

Differential diagnosis: H-type esophageal fistula, vocal cord paralysis, laryngeal cleft.

Anesthetic implications:

according to the associated neurological pathology; check chest XRay (atelectasis, bronchiectasis); risk of regurgitation.

References : 

-        De Caluwe D, Nassogne MC, Reding R, de Ville de Goyet J, Clapuyt P, Otte JB.
Cricopharyngeal achalasia: case reports and review of the literature.
Eur J Pediatr Surg 1999; 9:109-12. DOI: 10.1055/s-2008-1072223

-        Drendel M, Carmel E, Kerimis P, Wolf M, Finkelstein Y.
Cricopharyngeal achalasia in children: surgical and medical treatment.
IMAJ 2013 ; 15: 498-501

-        Soman V, Menon JR, Issac ME, et al.
Congenital cricopharyngeal achalasia in an infant treated by external cricopharyngeal myotomy: A Case Report.
Int J Phonosurg Laryngol 2022;12:16-8.

-        Mohajeri S, Ansari G, Isaac DM, Adsett AR, Zhang H, Isaac A.
Pediatric cricopharyngeal achalasia: a systematic review.
Int J Pediatr Otorhinolaryngol 2024;186:112112. doi: 10.1016/j.ijporl.2024.112112.

Updated: December 2024