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Primary cricopharyngeal dysfunction: Treatment with balloon catheter dilatation
CASE STUDIES Primary cricopharyngeal dysfunction: treatment with balloon catheter dilatation Jeno˝ Solt, MD, PhD, Judit Bajor, MD, Mariann Moizs, MD, Erzsébet Grexa, MD, PhD, Péter Ö. Horváth, MD, PhD, ScD Background: Primary cricopharyngeal dysfunction (PCD) is a rare, idiopathic, functional disorder of the upper esophageal sphincter, characterized by dysphagia, frequent aspiration, and functional narrowing at the level of the upper esophageal sphincter. Methods: Five of 29 patients with oropharyngeal dysphagia were found to have PCD. Patients presented with severe dysphagia and predisposition to aspiration. Radiography demonstrated narrowing at the level of the upper esophageal sphincter and aspiration. An endoscope could be introduced into the esophagus in only 2 patients before dilation. Observations: In contrast to organic stenoses, these functional upper esophageal sphincter stenoses were dilated without difficulty with a balloon catheter. After low-pressure (1.5-2 atm) progressive balloon dilation (to 20 mm), superficial mucosal injury was observed only in one patient. After dilatation, symptoms resolved and barium swallow demonstrated normal passage through the upper esophageal sphincter. During a mean followup of 21 months (7-33), redilation was necessary in only 1 case. Conclusions: Balloon catheter dilatation of PCD is minimally invasive and provides both important diagnostic information and effective therapy. It should be the first choice of therapy for PCD.
It is often difficult to determine the etiology of oropharyngeal dysphagia (OPD). In particular, the endoscopic and radiographic evaluation of esophageal inlet stenosis can be extremely challenging. Primary cricopharyngeal dysfunction (PCD) is characterized by dysphagia, frequent aspiration, and idiopathic functional narrowing at the level of the upper esophageal sphincter (UES).1 The characteristic feature is a reduction in maximal opening of Received October 14, 2000. For revision November 15, 2000. Accepted June 29, 2001. From the I. Departments of Medicine and Radiology, Baranya County Hospital, and the Department of Surgery, Faculty of Medicine, University of Pécs, Pécs, Hungary. Presented at the Groupe Europien d’Etude des Maladies de l’Oesophage meeting, March 2-4, 2000, Würzburg, Germany. Reprint requests: Jenö Solt, MD, I. Department of Medicine, Baranya County Hospital, Raksczi u. 2., 7623, Pécs, Hungary. Copyright © 2001 by the American Society for Gastrointestinal Endoscopy 0016-5107/2001/$35.00 + 0 37/1/118442 doi:10.1067/mge.2001.118442 VOLUME 54, NO. 6, 2001
Table 1. Differential diagnosis of oropharyngeal dysphagia Secondary cricopharyngeal dysfunction Neurogenic Cerebral vascular accident Poliomyelitis Amyotrophic lateral sclerosis Parkinson’s disease Multiple sclerosis Peripheral neuropathies (diphtheria, botulism, rabies, diabetes mellitus) Diseases of striated muscles Inflammatory myopathies Polymyositis Dermatomyositis Muscular dystrophies Myotonic dystrophy Oculopharyngeal dystrophy Metabolic myopathy (thyrotoxicosis, myxedema, corticosteroid myopathy) Local structural lesions Inflammatory Neoplastic Extrinsic compression Cervical webs Zenker’s diverticulum Bilateral recurrent laryngeal nerve paralysis Surgical resection of oropharynx Other postsurgical changes Primary cricopharyngeal dysfunction Hypertensive UES Abnormal UES relaxation and/or pharyngo-esophageal incoordination Incomplete relaxation (cricopharyngeal achalasia) Premature closure Delayed relaxation (familial dysautonomia) Impaired UES compliance (cricopharyngeal bar, restricted sphincter opening)
the UES during transphincteric flow. This is caused by failed or partial sphincter relaxation, a lack of pharyngoesophageal coordination, or a reduction in the muscular compliance of the UES. PCD must be differentiated from numerous neuromuscular diseases and local structural lesions that cause secondary cricopharyngeal dysfunction (Table 1).2 PCD is rare, occurring mainly in elderly individuals. The most frequent symptom is dysphagia. Swallowing is often followed by regurgitation, coughing, choking, and aspiration, which may lead to pneumonia. Manometric characteristics include a normal or increased resting UES pressure, with incomplete relaxation, loss of pharyngoesophageal coordination, or both. A prominent “cricopharyngeal bar” can often be demonstrated by contrast radiography.2,3 Endoscopy and contrast radiography are performed to exclude mechanical obstruction. After exclusion of secondary neurologic and myogenic causes as well as diseases of the pharynx and larGASTROINTESTINAL ENDOSCOPY
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Table 2. Demographic data for patients with primary cricopharyngeal dysfunction Patient No. 1 2 3 4 5
Patient’s age
End
Diameter of narrowing (mm)
Type of dysphagia
Aspiration clinical/radiologic
Dilatation (mm)
Reflux
Follow-up (mo)
79 74 71 62 70
No No Push Push No
2-3 4 6 8 4-5
Solid and liquid Solid and liquid Solid Solid and liquid Solid
+/+ +/+ –/– +/+ +/+
20 20 20 20 20
+ + – – +
33 23 25 17 7
ynx, it is necessary to distinguish among the primary causes of cricopharyngeal dysfunction. The cricopharyngeal musculature was first described by Valsalva in 1717.4 In 1946, Lahey5 proposed cricopharyngeal dilation in addition to surgical procedures for the treatment of patients with pharyngoesophageal diverticulum. In 1950, Asherson1 described “cricopharyngeal achalasia” as defective relaxation of the UES. The first cricopharyngeal myotomy was performed by Kaplan6 in 1951 for secondary cricopharyngeal dysfunction caused by poliomyelitis. This operation was performed for PCD by Sutherland7 in 1962. Subsequently Blitzer and Brin8 reported the use of botulinum toxin for the management of PCD. These are the results of balloon catheter dilation of the UES in 5 patients with PCD. PATIENTS AND METHODS Twenty-nine patients with OPD were evaluated between 1995 and 1999. To exclude secondary causes of OPD, a medical history, physical examination, and radiographic swallowing study (fluoroscopic or video) were performed, followed by upper endoscopy (with biopsies if necessary). Esophageal manometry was performed before and after UES dilation. Synchronous fluoromanometry was not performed. To exclude extraluminal organic processes, radiologic studies and CT were obtained. Neurologic and otolaryngologic consultations were part of the routine evaluation. Six patients were found to have malignancy, 6 corrosive strictures, 5 postoperative stenoses, 1 amyotrophic lateral sclerosis, 4 Zenker’s diverticula, 1 cervical osteophytes, and 1 had bilateral recurrent nerve paresis. A diagnosis of PCD was made in 5 patients. The average age of patients (3 men, 2 women) with PCD was 71 years (62-79 years) (Table 2). All presented with dysphagia. Three had difficulty swallowing liquids. On average, symptoms had been present for 25 months (2 weeks to 5 years). Four patients had clinical symptoms of aspiration, which was confirmed by radiographic contrast studies. In 2, the radiographic study had to be terminated because of aspiration. Before dilation, the maximal UES diameter ranged from 2 to 8 mm as determined by contrast radiography. An endoscope could be inserted distal to the UES in 3 patients only after dilation and in the other 2 patients only with pushing. Three patients had hiatal hernias and gastroesophageal reflux. Two patients had 768
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mild scarring just proximal to the cardia and were treated with a proton pump inhibitor initially, and H2 receptor blockers, subsequently. Dilation of UES Pharyngeal anesthesia was obtained by spraying the pharynx with a topical anesthetic (lignocaine). In 3 patients dilation with a balloon catheter was performed after it was determined that the endoscope could not be introduced into the esophagus. In these patients, a 0.035inch guidewire (Zebra, Microvasive Endoscopy, Boston Scientific Corp., Natick, Mass.) was passed through the endoscope and through the UES under fluoroscopic guidance. Then a 12-mm or 15-mm diameter balloon dilation catheter (Microvasive Endoscopy) was passed over the wire and positioned across the UES under fluoroscopy. If the UES expanded easily and no mucosal damage was evident, dilation was performed to 18- or 20-mm diameter, either immediately or at a subsequent procedure. The balloon was inflated with a water-soluble contrast medium to 1.5 to 2 atm under fluoroscopic guidance to eliminate the waist caused by the UES. The pain noted by the patients in response to dilation was minimal. After dilation, the UES and adjacent structures were examined endoscopically for evidence of mucosal damage. Subsequently, patients were observed closely for 3 hours for complications. Barium contrast radiography and manometry were performed 2 days later.
OBSERVATIONS Balloon dilation of the UES was initiated at 15 mm in 2 cases, 18 mm in 1, and at 20 mm in 2 patients. The maximum diameter achieved in all cases was 20 mm (Table 2). With a distention pressure of 2 atm, the waist on the balloon disappeared in all cases. Superficial mucosal splitting was observed in 1 patient in whom a 20-mm diameter balloon catheter was used at the first dilation. After balloon dilation, all patients were free of dysphagia, coughing, and aspiration, and radiographically the passage of barium was normal. Before treatment, manometry demonstrated a hypertensive UES with normal relaxation in patient number 1; incomplete and failed relaxations in patients 2, 3, and 4, and lack of pharyngoesophageal coordination and incomplete relaxation in patient number 5. After dilation the UES resting pressure VOLUME 54, NO. 6, 2001
Primary cricopharyngeal dysfunction and balloon catheter dilatation
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A A
B
Figure 1. A, Barium contrast radiograph showing short 3mm diameter narrowing at level of upper esophageal sphincter with prenarrowing dilation and residual contrast. B, Barium contrast radiograph obtained after dilatation showing open esophageal inlet.
decreased from a median pretreatment value of 88.8 mm Hg to 43.9 mm Hg (p < 0.05), the median UES residual pressure decreased from 9.7 mm Hg to 1.4 mm Hg (p < 0.05), and relaxation improved from 89% to 96.8%. Clinical symptoms and barium contrast radiography were more sensitive indicators of the efficacy of treatment than manometry. The average length of follow-up was 21 (7-33) months. Dysphagia returned after 18 months in 1 patient (patient No. 2) (Table 2), but was treated successfully by a further balloon dilation. This patient died 23 months later of heart failure. An autopsy including histopathologic examination did not reveal any pathologic alteration in the UES region. Representative case histories follow. Case 1 A 79-year-old man (patient 1, Table 2) presented with a 10-year history of OPD. Initially, solid food would lodge and then be regurgitated, accompanied VOLUME 54, NO. 6, 2001
B
Figure 2. A, Barium contrast radiograph showing arched narrowing at level of seventh cervical vertebra 15 mm long and 6 mm wide. B, Barium contrast radiograph obtained after dilation showing disappearance of narrowing at inlet to esophagus.
by coughing. In the 2.5 months before evaluation, he experienced difficulty with swallowing even soft food, which also provoked coughing. A contrast radiographic study was performed with a small amount of water-soluble contrast medium initially, followed by barium. In the proximal esophagus, there was a conical narrowing 20 mm in length with a moderately irregular contour (Fig. 1A). With swallowing, the lumen above the narrowing was briefly distended and then emptied. However, the patient aspirated barium and the examination was aborted. Perfusion manometry demonstrated a hypertensive UES, with normal relaxation. It was possible to pass an endoscope into the esophagus only after dilation of the UES with a 15-mm diameter balloon catheter. Endoscopically, the UES was located 18 cm from the teeth. The mucosa of the UES and esophagus was intact. There was a thin fibrotic ring at the level of the cardia without significant narrowing of the lumen; a 4-cm hiatal hernia was evident. Subsequently, the UES was dilated with a balloon catheter to 18 mm and then 20 mm. The dysphagia resolved after dilaGASTROINTESTINAL ENDOSCOPY
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tion. A follow-up radiographic swallowing study demonstrated normal passage of contrast through the UES (Fig. 1B). Case 2 A 71-year-old woman (patient 3, Table 2) presented with dysphagia for solid food of 2 weeks’ duration. A radiographic swallowing study demonstrated a narrowing 6 mm wide and 15 mm long at the level of the seventh vertebra (Fig. 2A). Manometry demonstrated poor pharyngeal contractions and failure of UES relaxation. At endoscopy, the UES was at 17 cm; it opened only when pressure was exerted with the endoscope. The esophageal mucosa was intact. After insertion of an atraumatic guidewire, the narrowed UES was dilated with an 18-mm and then a 20-mm balloon catheter inflated to 2 atm. After the dilation there was no mucosal damage evident endoscopically. On subsequent barium swallow the sharply contoured, arched narrowing noted on the initial study was no longer evident (Fig. 2B). DISCUSSION PCD is a rarely diagnosed disorder that usually goes untreated. The leading symptoms are dysphagia, weight loss, regurgitation, aspiration, and coughing. The symptoms are evoked by functional obstruction of the UES, which can be detected by endoscopy and radiographic swallowing studies. In most cases there is either incomplete relaxation of the UES or a functional lack of coordination between the pharynx and UES. Premature closure is often demonstrated in patients with Zenker’s diverticulum,9 whereas delayed relaxation is found in patients with familial dysautonomia.10 The diagnosis of PCD requires exclusion of secondary causes of OPD (Table 1). A thorough history, physical examination, radiographic swallowing study, and endoscopy with biopsy are necessary. Balloon catheter dilation also provides information that is useful for diagnosis: the extent of narrowing and the compliance of the UES. Furthermore, dilation may be necessary before endoscopic examination can be performed. Pharyngoesophageal manometry provides additional information, including the peak pharyngeal pressure, hypopharyngeal intrabolus pressure, UES resting pressure, UES relaxation, and the degree of coordination between pharyngeal contraction and UES relaxation.11-13 However, perfusion manometry of the UES is technically difficult.1 The measured manometric pressure values are highly influenced by the orientation of the perfusion ports on the catheter, and the results are often discordant with the clinical and 770
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Primary cricopharyngeal dysfunction and balloon catheter dilatation
radiologic findings.10 Solid-state catheter manometry may be more useful. In recent years the importance of simultaneous videoradiography and manometry with specific catheters (Dent sleeve, Castell-type solid state) has been emphasized.13-16 These types of studies may provide information on the mechanism of oropharyngeal swallowing and differentiate among the types of cricopharyngeal dysfunction. Other studies, such as 24-hour pH measurement, endosonography with a narrow-diameter probe17 CT, and electromyography may also play important roles in the evaluation. The average age of our patients with PCD was 71 years. Three of the 5 patients had a history of gastroesophageal reflux, and 2 had a moderate cicatrization proximal to the cardia. Patients with gastroesophageal reflux may have a significantly increased resting UES pressure, which normalizes with effective treatment.18 This may elicit a globus sensation,19 and our hypothesis is that in a small number of cases it may lead to cricopharyngeal dysfunction. A possible pathogenetic role for gastroesophageal reflux is supported by the fact that 2 of our patients had both Zenker’s diverticulum and severe reflux esophagitis. Another elderly patient, who refused surgery for Zenker’s diverticulum, was treated by UES dilation to 20 mm, resulting in a significant improvement in dysphagia. Many investigators have used cricopharyngeal myotomy with success in the treatment of primary and secondary cricopharyngeal dysfunction.6,17,20,21 The best outcome is achieved in patients with PCD.22,23 According to Bonavina et al.,24 cricopharyngeal myotomy should be considered in cases of cricopharyngeal dysfunction when the pharyngeal contraction pressure is inadequate, when the movement of the pharynx and UES is uncoordinated, or when the relaxation of the UES is incomplete. McKenna and Dedo25 considered myotomy to be both a diagnostic and therapeutic intervention. The transmucosal myotomy was introduced by Halvarson26 in 1994. Blitzer and Brin8 described the treatment of “cricopharyngeal achalasia” with botulinum toxin in 1997; they also considered it to be useful as a diagnostic test. PCD and the dysfunction of the lower esophageal sphincter in achalasia have similarities, but the simultaneous occurrence of these 2 disorders has been described in only 1 case.27 In our experience, achalasia is 40 times more common than PCD. There are few reports of cricopharyngeal dilation,13,28,29 but results support its use in patients with PCD. Myotomy, dilation, and botulinum toxin injection all decrease UES resting tone and result in the recovery of normal pharyngoesophageal funcVOLUME 54, NO. 6, 2001
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tion. The effect of treatment with botulinum toxin is temporary. In our opinion, dilation causes functional damage to muscle, but the procedure is less invasive than surgical myotomy. Furthermore, it is simple and less troublesome for the patient. REFERENCES 1. Asherson N. Achalasia of cricopharyngeus sphincter: record of cases with profile pharyngograms. J Laryngol Otol 1950;64: 747-58. 2. Castell DO. Approach to the patent with dysphagia. In: Yamada T. editor. Textbook of gastroenterology. Philadelphia: JB Lippincott; 1991. p. 562-72. 3. Dantos RO, Cook IJ, Dodds WJ, Kern MK, Lang IM, Brasseur JG. Biomechanics of cricopharyngeal bars. Gastroenterology 1990;99:1269-74. 4. Ross ER, Green R, Auslander MO, Biler HF. Cricopharyngeal myotomy: management of cervical dysphagia. Otolaryngol Head Neck Surg 1982;90:434-41. 5. Lahey FH. Pharyngoesophageal diverticulum: its management and complications. Ann Surg 1946;124:617-36. 6. Kaplan S. Paralysis of deglution, a post-poliomyelitis complication treated by section of the cricopharyngeus muscle. Ann Surg 1951;133:572-3. 7. Sutherland HD. Cricopharyngeal achalasia. J Thorac Cardiovasc Surg 1962;43:114-26. 8. Blitzer A, Brin MF. Use of botulinum toxin for diagnosis and management of cricopharyngeal achalasia. Otolaryngol Head Neck Surg 1997;116:328-30. 9. Ellis FH, Schlegel JF, Lynch VP, Payne WP. Cricopharyngeal myotomy for pharyngeal diverticulum. Ann Surg 1969;170: 340-9. 10. Marguiles SI, Brunt PW, Donner MW, Silbiger ML. Familial dysautonomia: a cinematographic study of the swallowing mechanism. Radiology 1968;90:107-12. 11. Hurwitz AL, Nelson JA, Hadded JK. Oropharyngeal dysphagia. Manometric and cine esophagraphic findings. Digest Dis 1975;20:313-24. 12. Schneider I, Thumfait WF, Pototsching C, Eckel H. Treatment of dysfunction of the cricopharyngeal muscle with botulinum toxin: introduction of a new, noninvasive method. Ann Otol Laryngol 1994;103:31-5. 13. Ali GN, Wallace KL, Laundl TM, Hunt DR, deCarle DJ, Cook
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14.
15. 16.
17.
18. 19.
20. 21.
22. 23.
24. 25. 26.
27. 28.
29.
IJ. Predictors of outcome following cricopharyngeal disruption for pharyngeal dysphagia. Dysphagia 1997;12:133-9. American Gastroenterological Association medical position statement on management of oropharyngeal dysphagia. Gastroenterology 1999;116:452-4. Cook IJ, Kahrilas PJ. AGA technical review on management of oropharyngeal dysphagia. Gastroenterology 1999;116:455-78. Stendal S. Practical guide to gastrointestinal function testing (Medtronic Synectics). Oxford: Blackwell Science; 1997. p. 156-73, 188-93. Sobin J, Nathanson A, Engstrom CF. Endoluminal ultrasonography: a new method to evaluate dysphagia. J Otorhinolaryngol Relat Spec 1996;58:105-9. Hunt PS, Connell AM, Smiley TB. The cricopharyngeal sphincter in gastric reflux. Gut 1970;11:303-6. Watson WC, Sukkivan, SN. Hypertonicity of the cricopharyngeal sphincter: a cause of globus sensation. Lancet 1974;14: 1417-9. Lebo CP, Sang K, Norris F. Cricopharyngeal myotomy in amyotrophic lateral sclerosis. Laryngoscope 1976;86:862-8. Berg HM, Persley MS, Jakobs JB, Cohen NL. Cricopharyngeal myotomy: a review of surgical results in patients with cricopharyngeal achalasia of neurogenic origin. Laryngoscope 1985;85:1337-40. Belsey R. Functional disease of the esophagus. J Thorac Cardiovasc Surg 1966;52:164-88. Orringer MB. Extended cervical esophagomyotomy for cricopharyngeal dysfunction. J Thorac Cardiovasc Surg 1980; 80:669-78. Bonavina L, Khan NA, De Meester TR. Pharyngoesophageal dysfunction. Arch Surg 1985;120:541-9. McKenna JA, Dedo HH. Cricopharyngeal myotomy: indication and technique. Ann Otol Rhinol Laryngol 1992;101:216-21. Halvarson DJ. The treatment of cricopharyngeal dysmotility with transmucosal cricopharyngea myotomy using the potassium-titanyl-phosphate (KTP) laser. Endoscopy 1998;30:46-50. Nobre-e-Sauza MA, Dantas RO. Cricopharyngeal dysfunction in patient with achalasia. J Clin Gastroenterol 1998;26:216-8. Lernau OZ, Sherzer E, Mogle P, Nissan S. Congenital cricopharyngeal achalasia treatment by dilatations. J Pediatric Surg 1984;19:202-3. Mathieu J, Lapointe G, Brassard A, Tremblay C, Brais B, Rouleau GA, et al. A pilot study on upper esophageal sphincter dilatation for the treatment of dysphagia in patients with oculopharyngeal muscular dystrophy. Neuromusc Disorders 1977;1(suppl):S100-4.
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It is often difficult to determine the etiology of oropharyngeal dysphagia (OPD). In particular, the endoscopic and radiographic evaluation of esophageal inlet stenosis can be extremely challenging. Primary cricopharyngeal dysfunction (PCD) is characterized by dysphagia, frequent aspiration, and idiopathic functional narrowing at the level of the upper esophageal sphincter (UES).1 The characteristic feature is a reduction in maximal opening of Received October 14, 2000. For revision November 15, 2000. Accepted June 29, 2001. From the I. Departments of Medicine and Radiology, Baranya County Hospital, and the Department of Surgery, Faculty of Medicine, University of Pécs, Pécs, Hungary. Presented at the Groupe Europien d’Etude des Maladies de l’Oesophage meeting, March 2-4, 2000, Würzburg, Germany. Reprint requests: Jenö Solt, MD, I. Department of Medicine, Baranya County Hospital, Raksczi u. 2., 7623, Pécs, Hungary. Copyright © 2001 by the American Society for Gastrointestinal Endoscopy 0016-5107/2001/$35.00 + 0 37/1/118442 doi:10.1067/mge.2001.118442 VOLUME 54, NO. 6, 2001
Table 1. Differential diagnosis of oropharyngeal dysphagia Secondary cricopharyngeal dysfunction Neurogenic Cerebral vascular accident Poliomyelitis Amyotrophic lateral sclerosis Parkinson’s disease Multiple sclerosis Peripheral neuropathies (diphtheria, botulism, rabies, diabetes mellitus) Diseases of striated muscles Inflammatory myopathies Polymyositis Dermatomyositis Muscular dystrophies Myotonic dystrophy Oculopharyngeal dystrophy Metabolic myopathy (thyrotoxicosis, myxedema, corticosteroid myopathy) Local structural lesions Inflammatory Neoplastic Extrinsic compression Cervical webs Zenker’s diverticulum Bilateral recurrent laryngeal nerve paralysis Surgical resection of oropharynx Other postsurgical changes Primary cricopharyngeal dysfunction Hypertensive UES Abnormal UES relaxation and/or pharyngo-esophageal incoordination Incomplete relaxation (cricopharyngeal achalasia) Premature closure Delayed relaxation (familial dysautonomia) Impaired UES compliance (cricopharyngeal bar, restricted sphincter opening)
the UES during transphincteric flow. This is caused by failed or partial sphincter relaxation, a lack of pharyngoesophageal coordination, or a reduction in the muscular compliance of the UES. PCD must be differentiated from numerous neuromuscular diseases and local structural lesions that cause secondary cricopharyngeal dysfunction (Table 1).2 PCD is rare, occurring mainly in elderly individuals. The most frequent symptom is dysphagia. Swallowing is often followed by regurgitation, coughing, choking, and aspiration, which may lead to pneumonia. Manometric characteristics include a normal or increased resting UES pressure, with incomplete relaxation, loss of pharyngoesophageal coordination, or both. A prominent “cricopharyngeal bar” can often be demonstrated by contrast radiography.2,3 Endoscopy and contrast radiography are performed to exclude mechanical obstruction. After exclusion of secondary neurologic and myogenic causes as well as diseases of the pharynx and larGASTROINTESTINAL ENDOSCOPY
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Table 2. Demographic data for patients with primary cricopharyngeal dysfunction Patient No. 1 2 3 4 5
Patient’s age
End
Diameter of narrowing (mm)
Type of dysphagia
Aspiration clinical/radiologic
Dilatation (mm)
Reflux
Follow-up (mo)
79 74 71 62 70
No No Push Push No
2-3 4 6 8 4-5
Solid and liquid Solid and liquid Solid Solid and liquid Solid
+/+ +/+ –/– +/+ +/+
20 20 20 20 20
+ + – – +
33 23 25 17 7
ynx, it is necessary to distinguish among the primary causes of cricopharyngeal dysfunction. The cricopharyngeal musculature was first described by Valsalva in 1717.4 In 1946, Lahey5 proposed cricopharyngeal dilation in addition to surgical procedures for the treatment of patients with pharyngoesophageal diverticulum. In 1950, Asherson1 described “cricopharyngeal achalasia” as defective relaxation of the UES. The first cricopharyngeal myotomy was performed by Kaplan6 in 1951 for secondary cricopharyngeal dysfunction caused by poliomyelitis. This operation was performed for PCD by Sutherland7 in 1962. Subsequently Blitzer and Brin8 reported the use of botulinum toxin for the management of PCD. These are the results of balloon catheter dilation of the UES in 5 patients with PCD. PATIENTS AND METHODS Twenty-nine patients with OPD were evaluated between 1995 and 1999. To exclude secondary causes of OPD, a medical history, physical examination, and radiographic swallowing study (fluoroscopic or video) were performed, followed by upper endoscopy (with biopsies if necessary). Esophageal manometry was performed before and after UES dilation. Synchronous fluoromanometry was not performed. To exclude extraluminal organic processes, radiologic studies and CT were obtained. Neurologic and otolaryngologic consultations were part of the routine evaluation. Six patients were found to have malignancy, 6 corrosive strictures, 5 postoperative stenoses, 1 amyotrophic lateral sclerosis, 4 Zenker’s diverticula, 1 cervical osteophytes, and 1 had bilateral recurrent nerve paresis. A diagnosis of PCD was made in 5 patients. The average age of patients (3 men, 2 women) with PCD was 71 years (62-79 years) (Table 2). All presented with dysphagia. Three had difficulty swallowing liquids. On average, symptoms had been present for 25 months (2 weeks to 5 years). Four patients had clinical symptoms of aspiration, which was confirmed by radiographic contrast studies. In 2, the radiographic study had to be terminated because of aspiration. Before dilation, the maximal UES diameter ranged from 2 to 8 mm as determined by contrast radiography. An endoscope could be inserted distal to the UES in 3 patients only after dilation and in the other 2 patients only with pushing. Three patients had hiatal hernias and gastroesophageal reflux. Two patients had 768
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mild scarring just proximal to the cardia and were treated with a proton pump inhibitor initially, and H2 receptor blockers, subsequently. Dilation of UES Pharyngeal anesthesia was obtained by spraying the pharynx with a topical anesthetic (lignocaine). In 3 patients dilation with a balloon catheter was performed after it was determined that the endoscope could not be introduced into the esophagus. In these patients, a 0.035inch guidewire (Zebra, Microvasive Endoscopy, Boston Scientific Corp., Natick, Mass.) was passed through the endoscope and through the UES under fluoroscopic guidance. Then a 12-mm or 15-mm diameter balloon dilation catheter (Microvasive Endoscopy) was passed over the wire and positioned across the UES under fluoroscopy. If the UES expanded easily and no mucosal damage was evident, dilation was performed to 18- or 20-mm diameter, either immediately or at a subsequent procedure. The balloon was inflated with a water-soluble contrast medium to 1.5 to 2 atm under fluoroscopic guidance to eliminate the waist caused by the UES. The pain noted by the patients in response to dilation was minimal. After dilation, the UES and adjacent structures were examined endoscopically for evidence of mucosal damage. Subsequently, patients were observed closely for 3 hours for complications. Barium contrast radiography and manometry were performed 2 days later.
OBSERVATIONS Balloon dilation of the UES was initiated at 15 mm in 2 cases, 18 mm in 1, and at 20 mm in 2 patients. The maximum diameter achieved in all cases was 20 mm (Table 2). With a distention pressure of 2 atm, the waist on the balloon disappeared in all cases. Superficial mucosal splitting was observed in 1 patient in whom a 20-mm diameter balloon catheter was used at the first dilation. After balloon dilation, all patients were free of dysphagia, coughing, and aspiration, and radiographically the passage of barium was normal. Before treatment, manometry demonstrated a hypertensive UES with normal relaxation in patient number 1; incomplete and failed relaxations in patients 2, 3, and 4, and lack of pharyngoesophageal coordination and incomplete relaxation in patient number 5. After dilation the UES resting pressure VOLUME 54, NO. 6, 2001
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A A
B
Figure 1. A, Barium contrast radiograph showing short 3mm diameter narrowing at level of upper esophageal sphincter with prenarrowing dilation and residual contrast. B, Barium contrast radiograph obtained after dilatation showing open esophageal inlet.
decreased from a median pretreatment value of 88.8 mm Hg to 43.9 mm Hg (p < 0.05), the median UES residual pressure decreased from 9.7 mm Hg to 1.4 mm Hg (p < 0.05), and relaxation improved from 89% to 96.8%. Clinical symptoms and barium contrast radiography were more sensitive indicators of the efficacy of treatment than manometry. The average length of follow-up was 21 (7-33) months. Dysphagia returned after 18 months in 1 patient (patient No. 2) (Table 2), but was treated successfully by a further balloon dilation. This patient died 23 months later of heart failure. An autopsy including histopathologic examination did not reveal any pathologic alteration in the UES region. Representative case histories follow. Case 1 A 79-year-old man (patient 1, Table 2) presented with a 10-year history of OPD. Initially, solid food would lodge and then be regurgitated, accompanied VOLUME 54, NO. 6, 2001
B
Figure 2. A, Barium contrast radiograph showing arched narrowing at level of seventh cervical vertebra 15 mm long and 6 mm wide. B, Barium contrast radiograph obtained after dilation showing disappearance of narrowing at inlet to esophagus.
by coughing. In the 2.5 months before evaluation, he experienced difficulty with swallowing even soft food, which also provoked coughing. A contrast radiographic study was performed with a small amount of water-soluble contrast medium initially, followed by barium. In the proximal esophagus, there was a conical narrowing 20 mm in length with a moderately irregular contour (Fig. 1A). With swallowing, the lumen above the narrowing was briefly distended and then emptied. However, the patient aspirated barium and the examination was aborted. Perfusion manometry demonstrated a hypertensive UES, with normal relaxation. It was possible to pass an endoscope into the esophagus only after dilation of the UES with a 15-mm diameter balloon catheter. Endoscopically, the UES was located 18 cm from the teeth. The mucosa of the UES and esophagus was intact. There was a thin fibrotic ring at the level of the cardia without significant narrowing of the lumen; a 4-cm hiatal hernia was evident. Subsequently, the UES was dilated with a balloon catheter to 18 mm and then 20 mm. The dysphagia resolved after dilaGASTROINTESTINAL ENDOSCOPY
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tion. A follow-up radiographic swallowing study demonstrated normal passage of contrast through the UES (Fig. 1B). Case 2 A 71-year-old woman (patient 3, Table 2) presented with dysphagia for solid food of 2 weeks’ duration. A radiographic swallowing study demonstrated a narrowing 6 mm wide and 15 mm long at the level of the seventh vertebra (Fig. 2A). Manometry demonstrated poor pharyngeal contractions and failure of UES relaxation. At endoscopy, the UES was at 17 cm; it opened only when pressure was exerted with the endoscope. The esophageal mucosa was intact. After insertion of an atraumatic guidewire, the narrowed UES was dilated with an 18-mm and then a 20-mm balloon catheter inflated to 2 atm. After the dilation there was no mucosal damage evident endoscopically. On subsequent barium swallow the sharply contoured, arched narrowing noted on the initial study was no longer evident (Fig. 2B). DISCUSSION PCD is a rarely diagnosed disorder that usually goes untreated. The leading symptoms are dysphagia, weight loss, regurgitation, aspiration, and coughing. The symptoms are evoked by functional obstruction of the UES, which can be detected by endoscopy and radiographic swallowing studies. In most cases there is either incomplete relaxation of the UES or a functional lack of coordination between the pharynx and UES. Premature closure is often demonstrated in patients with Zenker’s diverticulum,9 whereas delayed relaxation is found in patients with familial dysautonomia.10 The diagnosis of PCD requires exclusion of secondary causes of OPD (Table 1). A thorough history, physical examination, radiographic swallowing study, and endoscopy with biopsy are necessary. Balloon catheter dilation also provides information that is useful for diagnosis: the extent of narrowing and the compliance of the UES. Furthermore, dilation may be necessary before endoscopic examination can be performed. Pharyngoesophageal manometry provides additional information, including the peak pharyngeal pressure, hypopharyngeal intrabolus pressure, UES resting pressure, UES relaxation, and the degree of coordination between pharyngeal contraction and UES relaxation.11-13 However, perfusion manometry of the UES is technically difficult.1 The measured manometric pressure values are highly influenced by the orientation of the perfusion ports on the catheter, and the results are often discordant with the clinical and 770
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radiologic findings.10 Solid-state catheter manometry may be more useful. In recent years the importance of simultaneous videoradiography and manometry with specific catheters (Dent sleeve, Castell-type solid state) has been emphasized.13-16 These types of studies may provide information on the mechanism of oropharyngeal swallowing and differentiate among the types of cricopharyngeal dysfunction. Other studies, such as 24-hour pH measurement, endosonography with a narrow-diameter probe17 CT, and electromyography may also play important roles in the evaluation. The average age of our patients with PCD was 71 years. Three of the 5 patients had a history of gastroesophageal reflux, and 2 had a moderate cicatrization proximal to the cardia. Patients with gastroesophageal reflux may have a significantly increased resting UES pressure, which normalizes with effective treatment.18 This may elicit a globus sensation,19 and our hypothesis is that in a small number of cases it may lead to cricopharyngeal dysfunction. A possible pathogenetic role for gastroesophageal reflux is supported by the fact that 2 of our patients had both Zenker’s diverticulum and severe reflux esophagitis. Another elderly patient, who refused surgery for Zenker’s diverticulum, was treated by UES dilation to 20 mm, resulting in a significant improvement in dysphagia. Many investigators have used cricopharyngeal myotomy with success in the treatment of primary and secondary cricopharyngeal dysfunction.6,17,20,21 The best outcome is achieved in patients with PCD.22,23 According to Bonavina et al.,24 cricopharyngeal myotomy should be considered in cases of cricopharyngeal dysfunction when the pharyngeal contraction pressure is inadequate, when the movement of the pharynx and UES is uncoordinated, or when the relaxation of the UES is incomplete. McKenna and Dedo25 considered myotomy to be both a diagnostic and therapeutic intervention. The transmucosal myotomy was introduced by Halvarson26 in 1994. Blitzer and Brin8 described the treatment of “cricopharyngeal achalasia” with botulinum toxin in 1997; they also considered it to be useful as a diagnostic test. PCD and the dysfunction of the lower esophageal sphincter in achalasia have similarities, but the simultaneous occurrence of these 2 disorders has been described in only 1 case.27 In our experience, achalasia is 40 times more common than PCD. There are few reports of cricopharyngeal dilation,13,28,29 but results support its use in patients with PCD. Myotomy, dilation, and botulinum toxin injection all decrease UES resting tone and result in the recovery of normal pharyngoesophageal funcVOLUME 54, NO. 6, 2001
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tion. The effect of treatment with botulinum toxin is temporary. In our opinion, dilation causes functional damage to muscle, but the procedure is less invasive than surgical myotomy. Furthermore, it is simple and less troublesome for the patient. REFERENCES 1. Asherson N. Achalasia of cricopharyngeus sphincter: record of cases with profile pharyngograms. J Laryngol Otol 1950;64: 747-58. 2. Castell DO. Approach to the patent with dysphagia. In: Yamada T. editor. Textbook of gastroenterology. Philadelphia: JB Lippincott; 1991. p. 562-72. 3. Dantos RO, Cook IJ, Dodds WJ, Kern MK, Lang IM, Brasseur JG. Biomechanics of cricopharyngeal bars. Gastroenterology 1990;99:1269-74. 4. Ross ER, Green R, Auslander MO, Biler HF. Cricopharyngeal myotomy: management of cervical dysphagia. Otolaryngol Head Neck Surg 1982;90:434-41. 5. Lahey FH. Pharyngoesophageal diverticulum: its management and complications. Ann Surg 1946;124:617-36. 6. Kaplan S. Paralysis of deglution, a post-poliomyelitis complication treated by section of the cricopharyngeus muscle. Ann Surg 1951;133:572-3. 7. Sutherland HD. Cricopharyngeal achalasia. J Thorac Cardiovasc Surg 1962;43:114-26. 8. Blitzer A, Brin MF. Use of botulinum toxin for diagnosis and management of cricopharyngeal achalasia. Otolaryngol Head Neck Surg 1997;116:328-30. 9. Ellis FH, Schlegel JF, Lynch VP, Payne WP. Cricopharyngeal myotomy for pharyngeal diverticulum. Ann Surg 1969;170: 340-9. 10. Marguiles SI, Brunt PW, Donner MW, Silbiger ML. Familial dysautonomia: a cinematographic study of the swallowing mechanism. Radiology 1968;90:107-12. 11. Hurwitz AL, Nelson JA, Hadded JK. Oropharyngeal dysphagia. Manometric and cine esophagraphic findings. Digest Dis 1975;20:313-24. 12. Schneider I, Thumfait WF, Pototsching C, Eckel H. Treatment of dysfunction of the cricopharyngeal muscle with botulinum toxin: introduction of a new, noninvasive method. Ann Otol Laryngol 1994;103:31-5. 13. Ali GN, Wallace KL, Laundl TM, Hunt DR, deCarle DJ, Cook
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