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OESO©2015
 
Volume: Primary Motility Disorders of the Esophagus
Chapter: Idiopathic motor disorders of the esophagus in the elderly
 

Are the causes of this achalasia known ?

M. Gosselin, A. Gosselin, A. Ropert, J.F. Bretagne (Rennes)

Difficulty in swallowing can develop at any age owing to a variety of disorders such as cerebral disease, peripheral neuropathies, muscular diseases, local factors and idiopathic upper esophageal sphincter dysfunction. The latter is an extremely rare condition but it is becoming increasingly recognized particularly in the elderly in whom this dysfunction can result in severe defects in eating and nutrition. Upper esophageal sphincter (UES) dysfunction may be a part of a more generalized disorder and the mechanisms responsible for abnormalities of Cricopharyngeal function resulting in oropharyngeal dysphagia (OD) remain controversial.

The various diseases listed in the table must be excluded by history, careful inspection of pharynx, hypopharynx and larynx, radiology and endoscopy, before the disorder is diagnosed as being a primary UES dysfunction. The specific defects more likely to cause OD in the aging population are strokes, Parkinson's disease, primary myositis, hyperthyroidism, cervical tumors, surgical resection of the

hypopharynx and cervical spine osteophytes. Neurogenic and myogenic causes account for 80 per cent of instances, whereas local lesions and dysfunction are responsible for the rest.

The neurologic system may be involved at any level of the afferent or efferent pathway or in the brain stem and the cortex, where integration of the information of swallowing takes place. Cerebrovascular accidents frequently bring on swallowing problems in the elderly people, particularly when the brain stem is involved as in pseudobulbar palsy. In other neurologic diseases UES dysfunction is also largely due to destruction of the motor nuclei in the brain stem. No single and specific disorder of swallowing is observed in multiple sclerosis and Parkinsonism. In the latter, lingual hesitancy and piece-meal deglutition may induce difficulties in bolus formation but the incomplete UES relaxation observed in several studies is not found by Logemann in a series of more than 100 patients [8]. In chronic schizophrenia, there is a striking incidence of cinefluorographic abnormalities of swallowing [2] regardless of the neuroleptic agents that occasionally have been discussed as a possible cause of laryngopharyngeal dystonia [9].

Peripheral nerve involvement usually due to a lesion in the upper portion of the vagus nerve trunk may result from neck surgery, neoplasm, injury by lead or bacterial toxins (botulism, rabies, tetanus) or mononeuritis multiplex. Some of the esophageal muscular dysfunction observed in diabetics is independent of neuropathy yet is strongly associated with psychiatric disorder.

Diseases that directly affect muscular activity also may result in swallowing difficulties in the elderly. Most common among these diseases are myotonic and oculopharyngeal dystrophies, myasthenia gravis, myositis and myopathy of the thyrotoxicosis.

When neurologic or muscular disease and also iatrogenic (neck operations, radiation therapy) or mechanical causes have been excluded OD can be attributed to an idiopathic dysfunction limited to the UES. Ever since its introduction in 1950 by Asherson [ 1 ], the term cricopharyngeal achalasia (CPA) has been widely accepted and indiscriminately applied to patients with a wide variety of abnormalities of UES function such as altered resting tone (hypertensive UES), abnormal relaxation (absent, incomplete or delayed relaxation) or premature closure of UES.

Strictly speaking, the term CPA denotes absence of relaxation or incomplete relaxation in response to swallowing; it must be reserved for this type of UES motor disorder and the diagnosis confirmed with a high fidelity manometric device such as a low compliance system and spatially oriented manometry.

Radiologists introduced the term CPA to describe a horizontal indentation that persists on the posterior wall of the pharyngoesophageal function during swallowing. This phenomenon, observed in about 5 per cent of symptomatic subjects over the age of 40 years [14] and 22 per cent of subjects aged 65 and over [12], has been described in a wide variety of diseases causing OD : cerebrovascular diseases, bulbar poliomyelitis, oculopharyngeal muscular dystrophy, thyroid myopathy, dermatomyositis, high unilateral vagotomy, pharyngeal diverticula and after laryngectomy [10].

As a matter of fact, the indentation does not closely correlate with UES failure to relax during manometry. Incomplete manometric relaxations have been found

in amyotrophic lateral sclerosis, neuritis, polymyositis, oculopharyngeal dystrophy, cervical osteoarthritis [5] and in some patients with neck surgery [4], but no recent systematic study using a high fidelity manometric device has been reported.

Much remains to be studied about the functional disturbances in deglutition complicating varied diseases.

When cricopharyngeal dysfunction occurs as an isolated condition, the adjectives « idiopathic » or « primary » are used to cover an ignorance of the syndrome. This condition, if it exists, represents only 3 per cent of causes of benign dysphagia requiring dilatations [17] and occurs particularly in the elderly ; the average age of patients quoted in 4 series is over 65 years [13].

Some believe that this condition may be the first stage of the pulsion pharyngeal diverticulum which occurs predominantly in the elderly but, to date, studies using a specifically designed low compliance manometric recording system provide no firm evidence to support this concept [6].

It is not clear to which extent aging per se contributes to some UES dysfunction, particularly CPA. The changes in resting pressure in the UES measured by a low compliance infusion system as a function of age have been reported by Pelemans and Vantrappen [11]. It appears that, with increasing age, the UES gets especially hypotonic[l1]. In the other hand, in 14 patients with a permanent indentation at the cricopharyngeal level, none had complete absence of relaxation and three patients had incomplete relaxation in 20 to 50 per cent of the swallows [16].

Incomplete opening of the UES after swallowing might be due 1/to a continuing contraction of the cricopharyngeal muscle, 2/to insufficiency of the pulling forces of other muscles that must overcome the elasticity that keeps the sphincter closed, or 3/to a stenosing process.

The first mechanism which is suggested by the term achalasia cannot be explained like achalasia of the lower esophageal sphincter by defective inhibitory innervation since, for the UES, no such VIP-ergic innervation has been demonstrated. In that case, the defective inhibitory mechanism must be in the brain or at the level of the afferent impulses [16].

Idiopathic UES dysfunction could be related to the degeneration of the nervous system and the weakness or atrophy of oropharyngeal muscles that might become insufficient to pull the sphincter open that frequently occurs in old age. This dysfunction could also be due to unrecognized or subclinical transient ischemic episodes.

Lastly, the elasticity of the cricopharyngeus muscle (CPM) may be diminished in some cases: reports of histologic examination of biopsy specimens of the CPM in the literature are scanty but varying degrees of muscle damage with principally interstitial fibrosis[3] or rare granulomatous reaction [7, 15] were observed. In assessing the significance of these changes, it must be born in mind that fibrosis was not observed in controls or in pouch cases, although regeneration or slight atrophy were seen. It would therefore appear that minor degrees of muscle damage and regeneration can occur in the CPM from the act of swallowing over a lifetime but that the degree of fibrosis in the symptomatic group is such that some other explanation must be sought [3]. It is probable that fibrosis will follow when the

degeneration process results in desintegration of the myotube basement membranes but, there was nothing to indicate the cause of myopathy, in particular there was no interstitial inflammatory or vascular change.

In conclusion, even though the respective roles of incomplete sphincter opening, failure of UES elasticity or weak propulsive forces are to be established, it appears that in the elderly, idiopathic CPA, if it exists, must be rare and that in this population disordered swallowing motility is most likely caused by disease, rather than by aging. Recognition of the etiology of the UES dysfunction is crucial because patients with primary UES dysfunction respond more favorably to the cricopharyngeal myotomy than patients whose neurological disease is also proximal to UES.

Table 1. Likely causes of oropharyngeal dysphagia

I.

Neurogenic causes

Central nervous system disease

Cerebrovascular accidents * (R) (bulbar or pseudobulbar palsy)

Amyotrophic lateral sclerosis *

Disseminated sclerosis

Parkinson's disease* ?

Chorea

Schizophrenia * (R)

Brain stem tumor

Peripheral nerve involvement

Mononeuritis multiplex

Miscellaneous neuropathies (botulism, rabies, tetanus, lead poisoning...)

Injuries (both superior laryngeal* nerves or vagi)

II.

Myogenic causes

Motor end plate disease : myasthenia gravis*

Inflammatory myopathy : polymyositis, dermatomyositis*

Muscular dystrophy : myotonic, oculopharyngeal *

Metabolic myopathy : thyrotoxicosis, hypothyroidism*

III

Iatrogenic causes

Operations

on the neck or on the thorax* Or radiation therapy

IV.

Mechanical causes

Endoluminal : inflammatory disease, foreign body, web, tumor*

Extraluminal : thyromegaly, lymphadenopathy, cervical spur*

V.

UES dysfunction (altered tone, abnormal relaxation, incoordination)

* Likely causes in elderly patients Proved CPA (R = only radiologically)

References

1. Asherson N (1950) Achalasia of the cricopharyngeal sphincter. J Laryng 64 : 747-758.

2. Bragg D, Hussar AE (1971) Cineradiographic evaluation of the swallowing act in schizophrenic patients. Gastroenterology 60 : 299-304.

3. Cruse JP, Edwards DAW, Smith JF, Wyllie JH (1979) The pathology of a cricopharyngeal dysphagia. Histopathology 3 : 223-232.

4. Duranceau A, Jamieson GG, Hurwitz AL, Ones RS, Postlethwait RW (1976) Alteration in esophageal motility after laryngectomy. Am J Surg 131 : 30-35.

5. Hurwitz AL, Nelson JA, Haddad JK (1975) Oropharyngeal dysphagia: manometric and cineesophagogastric findings. Am J Dig Dis 20, 313-324.

6. Knuff TE, Benjamin SB, Castell DO (1982) Pharyngoesophageal (Zenker's) diverticulum: a reappraisal. Gastroenterology 82 : 734-736.

7. Le Clech G, Gosselin M, Hery B, Subileau CI, Bourdiniere J (1977) Une etiologie rare de dysphagie : la pathologic du cricopharyngien. J Franc, d'Oto-Rhinolaryngol 26 : 603-608.

8. Logemann JA, Blonsky ER, Boshes B (1975) Dysphagia in parkinsonism. JAMA 231 : 69-70.

9. Moss HB, Green A (1982) Neuroleptic-associated dysphagia confirmed by esophageal manometry. Am J Psychiatry 139: 515-516.

10. Palmer E (1976) Disorders of the cricopharyngeus muscle : a review. Gastroenterology 71 : 510-519.

11. Pelemans W, Vantrappen G (1985) Esophageal disease in the elderly. Clinics in gastroenterology 14: 635-656.

12. Piaget F, Fouillet J (1959) Le pharynx et 1'oesophage seniles. Etude clinique, radiologique et radiocinematographique. J Med Lyon 40: 951-966.

13. Roed-Petersen K (1979) The pharyngoesophageal sphincter. A review of literature. Dan Med Bull 26:275-281.

14. Seaman WB (1976) Pharyngeal and upper esophageal dysphagia. JAMA 235 : 2643.

15. Siegel CL, Honda M,, Salik J, Mendeloff Al (1961) Dysphagia due to granulomatous myositis of cricopharyngeus muscle. Physiological and Cineradiographic studies prior and following successful surgical therapy. Trans Assoc Am Physicians 74 : 342-352.

16. Vantrappen G (1985) Controversies in esophageal motor disorders. In: Esophageal Disorders: Pathophysiology and Therapy. DeMeester TR, Skinner DB Eds, Raven Press, New York, 413-421.

17. Webb WA, McDaniel L (1984) Endoscopic evaluation of dysphagia in two hundred and ninety three patients with benign disease. Surg Gynecol Obstet 158 : 152-156.


Publication date: May 1991 OESO©2015