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OESO©2011
 
Volume: Primary Motility Disorders of the Esophagus
Chapter: Diffuse esophageal spasms (Corkscrew esophagus)
 

Is thickening of the esophageal wall a significant finding ?

N. Ectors, K. Geboes, V. Desmet (Leuven)

Introduction

Independently of the final application a criterion should always be scrutinized upon its specificity and sensitivity.

Quoting Ferguson et al. [1] diffuse esophageal spasm (DES) is referred to by gastroenterologists as diffuse spasm, by radiologists as segmental spasm or corkscrew esophagus and by surgeons as giant muscular hypertrophy. Since the

description of a « peculiar form of esophagismus » in 1889 by Osgood [2], diffuse esophageal spasm has been recognized as a primary motility disorder. As such the diagnosis is based upon a combination of clinical symptoms, radiographic and manometric analyses.

The increased wall thickness of the esophagus, as considered in this clinical context of primary motility disorder, corresponds to an increase in smooth muscular mass whereby as well the etiopathogenesis as the mechanism remain unclear.

This morphological finding has been reported in a descriptive and temptatively pathogenetic way as giant muscular hypertrophy, idiopathic muscular hypertrophy and diffuse and/or nodular leiomyomatosis. Muscular hypertrophy is mainly situated along the distal 1/2-2/3 of the esophagus, reaching frequently from just above or below the aortic arch to the proximal end of the gastroesophageal junction.

The junction being only occasionally involved. The appearance of the esophagus is fusiform.

Esophageal wall thickening in diffuse esophageal spasm. Review of the literature

Only occasionally all diagnostic features and criteria of diffuse esophageal spasm are documented in one and the same article, hampering review of the literature. However discrepancies in the «synonyms» used are detectable. A number of articles, with a more or less well-documented diagnosis of diffuse esophageal spasm, show a variable association of diffuse esophageal spasm and esophageal wall thickening ranging from 0 up to 100 p. cent [3] (table ]).

The mean percentage of association is 40 p. cent, while the median percentage is 36 p. cent. This large range is indicative of a lack of well structured, systematic studies. The studied entities, diffuse esophageal spasm and (idiopathic) muscular hypertrophy of the esophagus (IMHE), should be defined more clearly and all criteria should be fulfilled.

The rarity of the disease remains however a drawback. The studies of Chen el al. and Loebenberg [11, 12] testify of a well structured set-up. In 17 cases of diffuse esophageal spasm no significant esophageal wall thickening could however be demonstrated. These recent studies therefore clearly question the value of increased esophageal wall thickness as a « criterion » for diffuse esophageal spasm.

Esophageal wall thickening in other conditions. Review of the literature

Esophageal wall thickening has been described as well in achalasia [13-15] as in non-specific dysphagias [16-20]. These observations raise questions concerning diffuse esophageal spasm as part of the primary motility disorders of the esophagus and may be as part of a spectrum of diseases. To pathologists, this morphological change is mainly known as an « incidental autopsy finding » in patients having had

Table 1. Diffuse esophageal spasm (DES) and esophageal wall thickening.

Authors

Diagnosis

DES with esophageal wall thickening

Diagnostic method

Johnstone, 1960

« diffuse spasm »

26%(n= 11/43)

RX and/or OP

(all grades)

Craddock, 1966

DES

60%(n = 3/5)

OP

Gillies, 1967

DES

19%(n = 4/21)

RX

Westgaard, 1968

DES

100%(n = 2/2)

RX

Gonzalez, 1973

DES

45%(n = 5/ll)

RX and /or OP

Henderson, 1974

PDMA

72% (11 = 8/11)

RX

Flye, 1975

DES

18%(n = 4/22) 71 %(n = 10/14)

RX OP

Loebenberg, 1988

DES

0%

EUS

Chen, 1989

DES

0%(n = 0/l7)

RX

RX: radiographical measurement

OP: intra-operative measurement

EUS : endoscopic ultrasound

DES : diffuse esophageal spasm

PDMA : primary disordered motor activity

no related symptoms and an unrelated cause of death [21 ]. When associated with diffuse esophageal spasm and other motility disorders, an esophageal wall thickening caused by an increased muscular mass raises questions concerning its primary or secondary nature. The finding of asymptomatic idiopathic, muscular hypertrophy of the esophagus suggests it might be a secondary, adaptive phenomenon.

It would however be of interest to know the results of esophageal manometry in these cases of idiopathic muscular hypertrophy. «Idiopathic » muscular hypertrophy of the esophagus can also be an « incidental » finding when associated with malignant processes nearby or at some distance of the esophagus [22-25]. As far as etiopathogenesis is concerned, this association is intriguing.

Wall thickening due to an increase in fibrous tissue, as seen in reflux esophagitis or some secondary motility disorders; or due to the presence of « foreign material », as seen in an infiltrating carcinoma or amyloidosis should be excluded. The diagnosis of wall thickening can be based upon radiographical, surgical and morphological examination and more recently upon echo-endoscopy. The combination of the finding of an increased esophageal wall thickness with other findings, radiographical and others, should allow an accurate diagnosis of diffuse esophageal spasm or other conditions.

Measurements of the esophageal wall

The esophageal wall thickness corresponds almost to the thickness of both internal and external muscle layers. The «normal» thickness ranges from 2 to 4 mm depending on the author and most likely the method used. The measurements found in literature mainly concern adult patients. They are obtained via radiographical examination, surgical examination and autopsy, from diffuse esophageal spasm and idiopathic muscular hypertrophy cases.

The results obtained by these different techniques are perhaps not merely comparable, but this problem has never been studied for the esophagus. Yet, the data show a clear distinction between the normal esophagus and the pathological esophagi. The different measurements found in the literature are shown in figure I. The range varies from almost normal to a five-fold increase, as could be expected and no significant difference is noted between both groups. From our present

0215F1.JPG

Figure 1. Measurements of esophageal wall thickness in normal[33, 7, 26, 27, 12, 20, 11], diffuse esophageal spasm (DES)[6, 4, 22, 7, 5, 28, 29, 30, 9, 8] and idiopathic muscular hypertrophy of the esophagus (IMHE) [34, 31, 32, 33, 26, 35, 22, 17, 36, 20, 37], (Predominantly male, adult population).

knowledge of the primary motility disorders of the esophagus, we can conclude that the increased mass in the esophageal wall is an acquired, probably secondary, phenomenon [22, 38, 13]. Therefore, this feature will develop from nihil and non-existing to a prominent, occasionally extreme change. Since the «wall sign» described by Johnstone in 1960 on barium radiographical studies more sophisticated techniques have become available. Endoscopic ultrasound, CT scan and NMR open new perspectives. Accurate investigation should be possible even in minor, initial changes in wall thickness.

Conclusion

In conclusion, seemingly not all cases of diffuse esophageal spasm are associated with a thickening of the esophageal wall neither are all thickenings of the esophageal wall associated with diffuse esophageal spasm. Specificity and sensitivity of esophageal wall thickness as a diagnostic criterion for diffuse esophageal spasm are not absolute. Exact figures are not yet available. Further studies of diffuse esophageal spasm, together with other (primary) motility disorders and (idiopathic) muscular hypertrophy of the esophagus are mandatory, especially since now both diagnostic criteria and technical methods are available (figure 2).

0215F2.JPG

Figure 2. Transmural esophageal biopsy from a child operated for idiopathic muscular hypertrophy (a) showing the epithelium (E), the submucosa (S) and the prominent thickening of the muscularis. (Haematoxylin-eosin X6); For comparison a transmural esophageal biopsy from a normal adult is shown in b. The lumen is located centrally (L) and the two layers of the muscularis can easily be distinguished (M). (Haematoxylin-eosin X6).

References

1. Ferguson TB, Woodbury JD, Roper CL, et al. (1969) Giant muscular hypertrophy of the esophagus. Ann Thorac Surg 8 : 209-218.

2. Osgood H (1989) A peculiar form of esophagismus. Boston Medical Surg J 120: 401-405.

3. Bennett JR, Hendrix TR (1970) Diffuse esophageal spasm : a disorder with more than one cause. Gastroenterology 59 (2): 273-279.

4. Johnstone AS (1960) Diffuse spasm and diffuse muscular hypertrophy of lower esophagus. Br J Radiol XXXIII (396): 723-735.

5. Craddock DR, Logan A, Walbaum PR (1966) Diffuse esophageal spasm. Thorax 21:511-517.

6. Gillies M, Nicks R, Skyring A (1967) Clinical, manometric and pathological studies in diffuse esophageal spasm. Br Med J 2 : 527-530.

7. Westgaard T, Keats TE (1968) Diffuse spasm and muscular hypertrophy. Radiology 90 : 1001-1005.

8. Gonzalez G (1973) Diffuse esophageal spasm Am J Roentgenol Radium Ther Nucl Med 117 (2): 251-258.

9. Henderson RD, Ho CS, Davidson JW (1974) Primary disordered motor activity of the esophagus (diffuse spasm). Ann Thorac Surg 18 (4): 327-336.

10. Flye MW, Sealy WC (1975) Diffuse spasm of the esophagus. Ann Thorac Surg 19(6): 677-687.

11. Loebenberg MJ, Lewis JH, Fleischer DE, Jaffe MH, Bertagnolli ME, Cattau EL, Collen MJ, Benjamin SB (1988) Endoscopic ultrasound (EUS) for evaluating esophageal wall thickness (EWT) in esophageal motility disorders (EMD). Gastroenterology 94: 267.

12. Chen YM, Ott DJ, Hewson EG, Richter JE, Wu WC, Gelfand DW, Castell DO (1989) Diffuse esophageal spasm : radiographic and manometric correlation. Radiology 170: 807-810.

13. Cassella RR, Ellis FH, Brown AL (1965) Fine-structure changes in achalasia of the esophagus. II. Smooth muscle Am J Pathol 46 (3): 467-475.

14. MacCready PB (1935) Cardiospasm: report of two cases with postmortem observations. Arch Otolaryngol 21 : 633-647.

15. Moore I (1927) The pathology of esophagectasia (dilatation of the esophagus without anatomic stenosis at the cardiac orifice); demonstration of five further specimens. Proc Roy Soc Med 20 (Laryng Sec): 31-41.

16. Fernandes JP, Mascarenhas MJ, DaCosta JC, Correia JP (1975) Diffuse leiomyomatosis of the esophagus. A case report and review of the literature. Dig Dis 20(7): 684-690.

17. Demian SDE, Vargas-Cortes F (1978) Idiopathic muscular hypertrophy of the esophagus. Chest 73 : 28-32.

18. Wood DA (1932) Primary idiopathic muscular hypertrophy of the esophagus with narrowing of the lumen. Arch Pathol 14: 766-773.

19. Hall AJ (1916) A case of diffuse fibromyoma of the esophagus causing dysphagia and death. Q J Med 9 : 409-427.

20. Zeller R, McLelland R, Myers B, Thompson WM (1979) Idiopathic muscular hypertrophy of the esophagus: a case report Gastrointest Radiol 4 : 121-125.

21. Postlethwait RH, Sealy WE (1961) In : Surgery of the esophagus. Charles C. Thomas, Springfield, III.

22. Sloper JC (1954) Idiopathic diffuse muscular hypertrophy of the lower esophagus. Thorax 9 : 136-146.

23. Reher H (1885) Beitrage zur Kasuistiek der Esophaguserkrankungen. Dtsch Arch Klin Med 36: 455.

24. Rössle R (1935) Die Pylorushypertrophie des Erwachsenen. Schweiz Med Wschr 174.

25. Bühler J (1943) Schweiz Z Path Bakt 6, 193 (referred to by Abbo I and Giampalmo A, 1952).

26. Katz SJ, Lieberman A, Hechtman HB (1974) Spontaneous perforation of the esophagus associated with smooth muscle hypertrophy Am J Surg 127 : 338-341.

27. Henderson RD (1987) Esophageal motor disorders. Surg Clin North Am 67 (3): 455-474.

28. Ramsay BH (1967) Esophageal hypertrophy with esophagospasm (curling). Ann Thor Surg 4(1), 66-70.

29. Lummert H (1960) Eine besondere Form der idiopathischen Hypertrophie des Osophagus. Thoraxchirurgie 8 : 436-450.

30. Enterline H, Thompson J (1984) In : Pathology of the esophagus. Springer-Verlag, New York Ch. 4.

31. Giampalmo A (1946) Informatore Medico I (2), (referred to by Abbo I and Giampalmo A, 1952).

32. Buccellato G (1948) Sul'ipertrofia dell'esofago (contributo casistico). Pathologia 40,24.

33. Abbo I, Giampalmo A (1952) Contributo alia conoscenza della — ipertrofia idiopatica -dell'esofago. Rivista di anatomia patologica e di oncologia 6: 1-14.

34. Jung J (1945) Zbl Path 83,3 (referred to by Abbo I and Giampalmo A, 1952).

35. Peison B (1971) Idiopathic muscular hypertrophy of the lower esophagus and pylorus in an adult. Chest 59 (6): 682-687.

36. Agostini S, Grimaud JC, Salducci J, Clement JP (1988) Idiopathic muscular hypertrophy of the esophagus : CT features. J Comp Ass Tomogr 12 (6): 1041-1043.

37. Legius E, Proesmans W, Van Damme B, Geboes K, Lerut T, Eggermont E (1989) Idiopathic muscular hypertrophy of the esophagus and Alport-like syndrome in a boy. Submitted.

38. Rolleston HD (1899) Trans Path Soc London 50, 69 (referred to by Sloper JC, 1954).

39. Cassella RR, Ellis FH, Brown AL (1965) Diffuse spasm of the lower part of the esophagus. JAMA 191 (5): 107-110.


Publication date: May 1991 OESO©2011