www.elsevier.com/locate/jpedsurg

Journal of Pediatric Surgery (2009) 44, 1656–1658

Cricopharyngeal myotomy for the treatment ofcricopharyngeal achalasiaVishesh Jain, Veereshwar Bhatnagar⁎

The Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029, India

Received 12 February 2009; revised 24 April 2009; accepted 26 April 2009

0d

Key words:Cricopharyngealachalasia;

Dysmotility;Myotomy

Abstract Three cases of cricopharyngeal achalasia are being presented. The preoperative diagnosis wasestablished by a contrast swallow. Cricopharyngeal myotomy was performed in all patients. One childdied in the postoperative period because of respiratory failure secondary to aspiration pneumonitis,whereas in the other two, symptoms were relieved, and they remain asymptomatic on follow-up.© 2009 Elsevier Inc. All rights reserved.

Cricopharyngeal achalasia is a condition characterized byfailure of the cricopharyngeus muscle to relax duringswallowing and thereby preventing the entry of a food bolusfrom the pharynx to the esophagus [1]. Dysphagia because ofspasm of upper esophageal sphincter was first described in1915 by Jackson [2]; this condition was seen in adults inwhom it was usually secondary to a neurologic disease. Thefirst report of cricopharyngeal achalasia in infancy was byUtian and Thomas [3] in 1967. It is a rare cause of dysphagiain children and is therefore often diagnosed late. Opencricopharyngeal myotomy, performed through a cervicalapproach, has been the standard treatment of this condition.We report 3 cases along with a review of the literature.

1. Case reports

1.1. Case 1

A 3.5-month-old male child, previously reported [4],presented with complaints of choking and regurgitation

⁎ Correponding author. Tel.: +91 11 26593309; fax: +91 11 26588663.E-mail address: veereshwarb@hotmail.com (V. Bhatnagar).

022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved.oi:10.1016/j.jpedsurg.2009.04.031

during feeds. He also had cleft lip and palate, bilaterallobed ears, and a left preauricular sinus. Antenatalultrasonography was suggestive of polyhydramnios. Hewas born by a normal abdominal delivery after a full-termgestation. The child had 4 episodes of aspiration pneumo-nia before admission. On feeding through a nasogastrictube, the patient became asymptomatic. A contrast studyrevealed a shelflike projection posteriorly in the pharynx atthe C6 level. Upper gastrointestinal endoscopy revealedspasm of the cricopharyngeus muscle, but it allowed thepassage of the endoscope. Cine contrast esophagogramunder fluoroscopy showed partial obstruction to the flow ofbarium at the C6 level with a persistent smooth narrowingat the same level. Twenty-four–hour pH monitoringrevealed no gastroesophageal reflux (GER). The childhad undergone cricopharyngeal dilatation thrice withoutmuch relief. Cricopharyngeal myotomy was performed bya cervical approach through a right supraclaviculartransverse incision, and the myotomy was extended for1.5 cm on to the esophagus. All the muscle fibers weredivided until the mucosa was seen bulging out. In thepostoperative period, the child developed respiratoryfailure because of aspiration pneumonitis and did notsurvive despite appropriate ventilatory therapy and othersupportive treatment.

1657Cricopharyngeal achalasia

1.2. Case 2

An 8-month-old male child presented with fever for thelast 2 months along with recurrent episodes of chest infectionsince birth. His gestation period was normal, and the birthwas uneventful. The child had sialorrhea since birth and hadbeen on nasogastric tube feeds since day 10 of life. In the last8 months, the child had been hospitalized for more than 5months for recurrent pneumonia. After the pneumonia wastreated, a cine barium study revealed a narrowing andregurgitation of contrast at the cricopharyngeal level of theupper esophagus and absence of GER. Bronchoscopy wasalso performed to rule out a H-type tracheoesophagealfistula, and subsequently, cricopharyngeal myotomy wasperformed as described before. The child required ventilatorysupport for a short period in the postoperative period. Oralfeeds were initiated and were well tolerated from the secondpostoperative day onward. The child remained asymptomatictill the last follow-up at the age of 5 years.

Fig. 1 Contrast swallow study reveals persistent narrowing at thelevel of cricopharynx (arrow) with partial obstruction to the distalflow of the barium. The contrast is also seen refluxing into thenasopharynx.

1.3. Case 3

A 2-month-old male child presented with excessivesalivation since birth along with recurrent chest infections.His gestation period was normal, and the birth wasuneventful. Bronchoscopy and laryngoscopy revealed diffuseinflammation of the respiratory tract. Cine barium studyshowed dysmotility in the area of the cricopharynx along witha narrowing suggestive of cricopharyngeal achalasia (Fig. 1).Cricopharyngeal myotomy was performed as describedbefore. The postoperative period was uneventful. The childwas fed on the second day after surgery and was dischargedon the seventh day on full oral feeds. The child remainedasymptomatic till the last follow-up at 3 years of age.

2. Discussion

Cricopharyngeal achalasia is a rare cause of dysphagia inchildren and is therefore often diagnosed late. The exactetiology of this condition remains unknown although insome patients it can be attributed to central nervous system orneuromuscular disorders [5]. The effects of cricopharyngealachalasia can be better understood if functions of this muscleare taken into consideration. This muscle remains in a state oftonus thereby preventing passage of air into esophagusduring inspiration and passage of regurgitated gastriccontents in the pharynx. It relaxes during the second phaseof swallowing thereby allowing the food bolus to pass intothe esophagus. Therefore, failure of this muscle to relaxcompletely results in a resistance or in some cases inability ofthe food bolus to pass into the esophagus. This results inpersistence of food bolus in the pharynx that may then refluxthrough the nasopharynx or is aspirated into the airwaycausing coughing, choking, and chest infections.

The differential diagnosis of cricopharyngeal achalasiaincludes tracheoesophageal fistula, laryngotracheoesopha-geal cleft, dysphagia lusoria, and congenital esophagealstenosis. Direct examination of the pharynx, esophagus, andupper airway is warranted to rule out a local pathologiccondition.

Cine contrast swallow with thin barium or nonioniccontrast is the most useful study in the diagnosis ofcricopharyngeal achalasia. Findings suggestive of cricophar-yngeal achalasia include dilated pharynx with holdup of thecontrast in the pharynx with little passage into the esophagus[6]. Aspiration or nasal regurgitation of the contrast may benoted. Projection in the pharynx posteriorly at the level of C6level (cricopharyngeal bar) is also seen in most cases. Theendoscopic findings are nonspecific but might show spasm atthe esophageal inlet. Upper esophageal manometry has beenused but is usually difficult to perform in infants and youngchildren, and reference values are lacking.

Although not seen in our series, an interesting relationshiphas been shown in some reports [6-8]. It has been postulated

1658 V. Jain, V. Bhatnagar

that the spasm of the cricopharyngeus is because of itshyperreactivity secondary to GER although the data are con-flicting. However, it is clear that during management in thesepatients, GER needs to be addressed simultaneously or beforetreating cricopharyngeal achalasia as the clinical manifesta-tions of GER may flare up after cricopharyngeal myotomy.

The treatment options for this condition include surgicalmyotomy of the muscle, dilatations, and more recently,injection of botulinum toxin. Of these options, thecricopharyngeal myotomy has been the procedure of choice[5,6,9,10]. Most reports have documented immediate reliefof symptoms after myotomy although the symptoms maypersist for a few months in some patients [11]. An earlysurgical intervention is advisable for the establishment ofswallowing and early recovery [9]. Myotomy is usuallyperformed extramucosally by conventional surgery althoughendoscopic transmucosal myotomy using laser has beendescribed [12]. The endoscopic laser myotomy, whencompared with open myotomy, has the advantages oflower morbidity, absence of an external scar, and comparableearly outcomes [13]. Bouginage and balloon dilatation hasbeen used, and some authors have reported long-term cure[14,15]. Bouginage was tried in one of our patients withoutmuch success. Botulinum toxin has been used for thetreatment of cricopharyngeal achalasia and has been welltolerated with temporary relief for a few months [12,16].

3. Conclusion

Congenital cricopharyngeal achalasia is a rare clinicalentity but needs to be included in the differential diagnosis offeeding difficulty and choking in a neonate. The diagnosis isusually made on contrast swallow, and cricopharyngealmyotomy offers a complete and permanent cure.

References

[1] Eddy D, Palmer MD. Disorders of the cricopharyngeus muscle: areview. Gastroenterology 1976;71:510-9.

[2] Jackson C. Diseases of the esophagus. In: Jackson C, editor. Peroralendoscopy and laryngeal surgery. St Louis (Mo): Laryngoscope Co;1915. p. 507-8.

[3] Utian HL, Thomas RG. Cricopharyngeal incoordination in infancy.Pediatrics 1969;43:399-406.

[4] Mathur NB, Banerjee S, Maria A, et al. Congenital cricopharyngealachalasia. Indian Pediatr 2001;38:783-8.

[5] Caluwe DD, Nassogne MC, de Ville de Goyet A, et al. Cricophar-yngeal achalasia: case reports and review of literature. Eur J PediatrSurg 1999;9:109-12.

[6] Brooks A, Millar AJW, Rode H. The surgical management ofcricopharyngeal achalasia in children. Int J Pediatr Otorhinolaryngol2000;56:1-7.

[7] Ekberg O, Lindgren S. Gastroesophageal reflux and pharyngealfunction. Acta Radiol Diagn 1986;27:421-3.

[8] Hunt PS, Connell AM, Smiley TB. The cricopharyngeal sphincter ingastric reflux. Gut 1970;11:303-6.

[9] Muraji T, Takamizawa S, Satoh S, et al. Congenital cricopharyngealachalasia: diagnosis and surgical management. J Pediatr Surg 2002;37:12.

[10] Mahomed AA. Primary cricopharyngeal achalasia in infancy—myotomy treatment of choice. S Afr J Surg 2000;38:28-30.

[11] Korakaki E, Hatzidaki E, Manoura A. Feeding difficulties in a neonatewith primary cricopharyngeal achalasia treated by cricopharyngealmyotomy. Int J Pediatr Otorhinolaryngol 2004;68:249-53.

[12] Pitman M, Weissbrod P. Endoscopic CO2 laser cricopharyngealmyotomy. Laryngoscope 2009;119:45-53.

[13] Halvorson DJ, Kuhn FA. Transmucosal cricopharyngeal myotomywith the potassium-titanyl-phosphate laser in treatment of cricophar-yngeal dysmotility. Ann Otol Rhinol Laryngiol 1994;103:173-7.

[14] Lernau OZ, Sherzer E, Mogle P, et al. Congenital cricopharyngealachalasia treatment by dilatations. J Pediatr Surg 1984;19:202-3.

[15] Dinari G, Danziger Y, Mimouni M, et al. Cricopharyngeal dysfunctionin childhood: treatment by dilatations. J Padiatr Gastroenterol Nutr1987;6:212-6.

[16] Sewell RK, Bauman NM. Congenital cricopharyngeal achalasia:management with botulinum toxin before myotomy. Arch OtolaryngolHead Neck Surg 2005;131:451-3.