idiopathic muscular strictures of the sigmoid colon · 330 c. cassanoanda. torsoli 4', ir...

Gut, 1968, 9, 325-331

Idiopathic muscular strictures of the sigmoid colonC. CASSANO AND A. TORSOLI1

From the Istituto di Clinica Medica Generale (II)dell' Universita?, Rome (Italy)

In a paper dealing with diverticular disease, Morson(1963) stated that four out of 155 specimens ofsigmoid colon removed at St. Mark's Hospital,London, for 'diverticulitis' showed an increase ofthe muscular thickness of the wall without anydetectable diverticula. There were no signs ofinflammation in the wall, and the muscular ab-normality could be compared to that seen in patientswith diverticulosis. From this observation, hesuggested that the muscular thickening could precedethe appearance of diverticula or predispose to theoccurrence of diverticulosis. This hypothesis seemsto have been confirmed by Arfwidsson (1964) whofound initial intramural diverticula in cases ofspastic colon with remarkable thickening of themusculature. The muscular involvement of thecolon in diverticulitis has also been pointed out byCelio (1952) who compared the myopathic com-ponent of the disease to the hypertrophic stenosisof the pylorus.

Idiopathic muscular strictures of the large bowelin human beings have not been described until now.In the horse, segmental muscular stenosis has beenmentioned in the oesophagus and small intestine(Helmke, 1939). We have observed two cases ofmuscular stricture of the sigmoid not related todiverticular or other diseases. It seems helpful togive a brief description of our experience.

CASE REPORTS

CASE 1 This is a necropsy study of a 21-year-old womanwho died of renal failure. She had never complained ofdigestive trouble; her bowels had always been regular.

After the necropsy the large bowel was fixed in dis-tension with 10% saline formaldehyde at a pressure of70 cm of water. After fixation, radiographs were takenwith air contrast, and transverse sections of the largebowel, embedded in paraffin and sectioned at 5 microns,were stained with haematoxilyn and eosin, Mallory,and Pas.

Mforbid anatomy All the large intestine from theileo-caecal valve to the anal canal was studied. Theoutside of the fixed specimen showed a stricture in the

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sigmoid which was harder and more fibrous than the restof the large bowel. This narrowing was also evident inthe air-contrast film (Fig. la).On opening the specimen, the caecum, right transverse,

left colon, and the rectum were normal. The sigmoidshowed reduction of the lumen in its entire length exceptat one point where a circumscribed dilatation of the wallwas seen. The mucosa had redundant folds with alongitudinal pattem (Fig. lb); the walls showed a con-siderable increase in the thickness of the muscular layerexcept at the dilatation where it was thinner than thesurrounding parts (Figs. la, lc).

Microscopically (Figs. lc, 2a, 3a) the mucosa and thesubmucosa were normal and showed some signs of

FIG. la. Case 1. X-ray air-contrast picture of theisolated colon, showing the normal aspect of the ascendingtract and the stricture of the sigmoid with remarkablethickening of the wall: there is a break between thestenotic sigmoid and the normal rectum and at about themiddle of the sigmoid a circumscribed dilatation of thewall.FIG. lb. Macroscopic appearance ofthe specimen betweenthe normal descending colon and the narrowed sigmoid;in the narrowed sigmoid, thickening of the wall andmany mucosal folds with a longitudinal pattern.FIG. Ic. Transverse sections of the sigmoid colon (4 x)at the level of the dilatation of the wall. The thinning of thelongitudinal musculature and the almost complete dis-appearance of the circular musculature are noted. Alsothe mucosal folds are missing at this level.

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C. Cassano and A. Torsoli

FIG. 2a FIG. 2bFIG. 2a. Case 1. Transverse section of the sigmoid colon (x 24), showing the increase in thickness of both muscularlayers.FIG. 2b. Normal aspect ( x 80) of the ganglion cells of Auerbach's plexus.

vascular congestion and a few focal collections ofneutrophilic granulocytes. Thickening of both circularand longitudinal musculature could be easily recognizedand was more evident in the inner layer. The musculatureappeared to be normal in structure, but in some sectionsoedema was evident. The ganglions of Auerbach'splexus seemed to be cytologically normal and morenumerous in the narrowed portion of the bowel than inthose adjacent. The serous membrane showed a normalaspect.

At the level of the dilatation of the wall (Fig. Ic),the mucosa was flattened, the mucosal folds had dis-appeared and the muscular layer was thin. The dilatationinvolved about one third of the entire circumference andits radius was wider than that of the common diverticula.The picture resembled in some way the sacculations thatat times can be seen in the sclerodermic colon.

CASE 2 A 53-year-old woman had suffered fromconstipation from the age of 13. At first the bowels wereopen at intervals of a few days, then at longer periods,till she had to use laxatives and enemas. Neverthelessshe had stopped using laxatives during the last few yearsbecause they were no longer effective. Lately the con-stipation had become worse and she began to suffer froma tight, painful sensation in the left hypochondrium.This seemed apparently related to the intestinal stasis.She complained also of headache, abdominal distension,and listlessness; all this had worsened in the last twoyears.An x-ray examination of the large intestine taken

about 10 years ago revealed that the calibre of the sigmoidwas slightly reduced. However, there were no detectablechanges in the wall.

Physical examination showed a fit woman with anormal abdomen. The colon, increased in consistencyand slightly painful, was felt in the left iliac fossa. Thelaboratory tests did not show any abnormal pattern.Proctosigmoidoscopy was also normal.The gastrointestinal transit time, studied by means of

radiopaque markers (Halls, 1965; Hinton, 1967), revealedthat after 10 days all the markers were still retained inthe large intestine; the greater number seemed to besituated in the left colon above the sigmoid (Fig. 4a).X-ray examination of the large intestine by double-contrast enema (Figs. 4b, 5a, b) showed a segmentalstricture in the descending and sigmoid colon and therectosigmoid junction. The change of luminal calibrewas gradual, cranially, but clear cut at the distal end.The outline of the wall appeared to be normal everywhereand also in the stenotic tract.A provisional diagnosis of muscular stricture of the

sigmoid colon was made. It was believed that thisstricture could be the cause of the constipation andsurgical treatment was suggested.At laparotomy the sigmoid appeared normal, with a

slightly reduced calibre. A left hemicolectomy was per-formed, and the surgical specimens were fixed accordingto the procedure described in the previous case. Afterfixing the sigmoid was found to be stenotic; it felt harderthan the descending colon.Upon opening the specimen a stricture of the sigmoid

was evident and the radiological changes of intestinalcalibre, gradual in the upper end and clear cut in thelower one, were confirmed. A noticeable corrugation ofthe mucosa was seen, the folds having sometimes nodefinite orientation, sometimes a transverse direction(Fig. 6). Thickening of the muscular layer was seen

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Idiopathic muscular strictures of the sigmoid colon

FIG. 3c

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FIGS. 3a - 3b. Transversesections of the sigmoid colon( x 4) in the first, and secondcase of muscular stricture.In case 1, both layers of themusculature have increased inthickness. In case 2, thethickening involves only thecircular layer. The mucosaand the submucosa have anormal aspect; severalmucosal folds are evident.

FIG. 3b

FIG. 3c. Transverse sectionsof a normal sigmoid colon( x 4) of a 45-year-old man.The thickness of the muscularwall is shown for comparisonwith the above findings.

(Fig. 3b). Microscopically (Figs. 7 and 8) mild congestionof the mucosa and submucosa was seen. The muscularismucosae seemed slightly thicker, with an irregularpattern of its muscular cells. The circular muscle wasremarkably thickened, while the taeniae appeared to bequite normal. No diverticula were found. The ganglia ofthe myenteric plexus appeared to be numerous and morevoluminous in the stenotic portion, but were structurallynormal. No changes in the serosa were noted. No partof the wall components showed signs of inflammation ordegeneration.

After the operation the patient's symptoms disappearedand her bowel movements returned to normal.

DISCUSSION

Our cases show a hitherto unknown muscularinvolvement of the sigmoid colon. The most import-ant finding is the considerable increase in thicknessof the musculature with a consequent remarkablereduction in calibre of the damaged segment. In


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C. Cassano and A. Torsoli

FIG. 4a FIG. 4b

FIG. 4a. Case 2. X-ray film 10 days after the administration ofradiopaque markers which are still retained in the largeintestine; most of them are situated above the sigmoid.FIG. 4b. X-ray film during the first phase ofa double-contrast enema; the stricture of the sigmoid is clearly visible.

ti1(. 5a ic:iC. 5b

FIGS. Sa - 5b. Case 2. X-ray films after gas insufflation (double-contrast enema). The stricture beginsfrom the descendingcolon to the sigmoid and ends abruptly at the wall of the rectum. The outlines and the inner surface of the sigmoid areregular.

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FIG. 6a. Case 2. Macroscopicappearance of the isolated distalcolon. The corrugation of themucosa and the development of themucosalfolds are noted. Theincrease in thickness of the wall ismore evident at the highermagnification.

FIG. 7a FIG. 7b

FIG. 7. Case 2. Transverse sections of the sigmoid colon. Fig. 7a shows the normal aspect ( x 50) of the mucosa, theslight increase in thickness of the submucosa and the thickening of the circular musculature.FIG. 7b. An increase in number of Auerbach's ganglions is also evident (x 5O).

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330 C. Cassano and A. Torsoli

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FIG. 8. Case 2. Intramural ganglia in a transversesection of the sigmoid colon (120 x) showing the normalaspect ofthe nervous cell.

the first case, the thickening involved both muscularlayers and it appeared to be asymptomatic. Somefocal inflammatory changes of the mucosa andsubmucosa and furthermore a circumscribed dilata-tion of the wall were noted. In the second case,characterized by severe constipation, the muscularabnormality involved only the circular layer. Thiswas not accompanied by inflammatory or degener-ative change. After surgery (left hemicolectomy) thebowel habit returned to normal.A similarity exists between the muscular changes

in these two cases and the changes of the muscle indiverticular disease. Nevertheless, it is impossibleto say whether or not there is a relationship betweenthe two clinicopathological pictures. In diverticulardisease the increase in thickness of the musclesseems related to the shortening of the intestine(Morson, 1963), while, in at least one of these cases,there was no indication that the sigmoid wasshortened. The almost normal aspect of the sigmoidupon removal and the reproduction of the strictureafter distension and fixing are proof that the mus-cular thickening can have an important functionalcomponent. A pharmacological effect of salineformaldehyde could possibly have contributed to

the recurrence of the stricture (Grupp and Goerttler,1957).

It should be stressed that in both cases thestricture was localized in the sigmoid colon, that is,the colonic segment which normally has the thickestmuscular wall and in which mechanical activity ismost intense (Cassano, Torsoli et al., 1966).

These strictures could be defined as 'idiopathic'in order to point out the unknown aetiology andtheir presumable autonomous meaning; but furtherstudies are necessary to elucidate the backgroundof the pathological picture. It is probable that theyare not often exactly recognized, the diagnosis inmost cases being that of simple chronic constipation,diverticulitis, sigmoiditis, or cancer.

SUMMARY

Two cases of muscular stricture of the sigmoidcolon, apparently idiopathic, have been described.In the first case the increased thickness of the musclewas asymptomatic and involved both the circularand longitudinal layer. It was accompanied by somescattered inflammatory changes. In the second case,characterized by severe constipation, the muscularthickening concerned only the circular layer andthere were no signs of inflammation. After a lefthemicolectomy the constipation disappeared.Some similarities between this pathological

picture and that of diverticular disease are men-tioned. The need for further studies to elucidate themeaning of these muscular thickenings has beenstressed.

REFERENCES

Arfwidsson, S. (1964). Pathogenesis of multiple diverticula of thesigmoid colon in diverticular disease. Acta chir. scand., suppl.342.

Cassano, C., Torsoli, A., et al. (1966). Indirizzi moderni nello studiodelle malattie croniche non neoplastiche del colon. LXVIICongr. Soc. ital. med. int., Rome, Oct. 1966.

Celio, A. (1952). Zur Pathologie der chronischen stenosierendenDiverticulitis coli (sog. Diverticulitistumor). Helv. chir. Acta,2, 93-118.

Grupp, G., and Goerttler, K. (1957). Herbeifuhren und Fixierenverschiedener Kontraktionszustande glatter Muskelfaser.Z.wiss. Mikr., 63, 397-418.

Halls, J. (1965). Bowel content shift during normal defaecation. Proc.roy. Soc. Med., 58, 859-860.

Helmke, K. (1939). Ober Oesophagushypertrophie. Virchows Arch.path. Anat., 304, 79-86. Quoted by Celio, A. (1952).

Hinton, J. M. (1967). Laxatives and anti-diarrhoeal Agents: Transit,studies. Proc. roy. Soc. Med., 60, 215-216.

Morson, B. C. (1963). The muscle abnormality in diverticular diseasesof the sigmoid colon. Brit. J. Radiol., 36, 385-392.

ADDENDUM

Since this article was written, we have seen two newcases of sigmoid muscular stricture. In both the


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pathological findings were strictly comparable withthose described above (severe thickening of bothmuscular layers without evidence of inflammationand the ganglia of Auerbach's plexus appearing tobe normal). The first case concerned a 53-year-oldman with longstanding constipation; in the secondpatient, a 35-year-old man who was operated uponfor a subacute intestinal obstruction, a smalldiverticulum was found in the thickened muscularwall.

FIGS. 1-3. Pathological appearances in a man aged 53with longstanding constipation.

FIc. 3

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idiopathic muscular strictures of the sigmoid colon · 330 c. cassanoanda. torsoli 4', ir...

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