Pneumatosis cystoides intestinalis diagnosed by colonoscopy

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Page 1: Pneumatosis cystoides intestinalis diagnosed by colonoscopy

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REFERENCESI. Ross ST: Ischemic colitis. POSI Grad Med 51:71, 19722. MARSTON A, PHEILS MT, THOMAS ML, MORSON BC: Ischaemic colitis.

GUI 7: I, 19663. BOLEY SJ, SCHWARTZ S, LASH J, STERNHILL V: Reversible vascular

occlusion of the colon. Surg Gynecol Obslel 116:53, 19634. BOLEY SJ, SCHWARTZ S, KRIEGER H, SCHULTZ L, SlEW F, ALLEN

AC: Further observation in reversible vascular occlusion of the colon.Am J Gaslroenlerol 44:260, 1965

5. BYRD BF, SAWYERS JL, BOMAR RL, KLATTE EC: Reversible vascularocclusion of the colon: recognition and management. Ann Surg 167:901,1968

6. MARSHAK RH, LINDNER AE: Ischemia of the colon. Seminars Raell/­gellol 3:81, 1968

7. THOMAS ML: Further observations on ischaemic colitis. Proc Roy SocMed 61:341, 1968

8. BROWN A: Non-gangrenous ischaemic colitis. Br J Surg 59:463, 19729. SMITH SL, TUTTON RH, OCHSNER SF: Roentgenographic aspects of

intestinal ischemia. Amer J Roen/genol, Rod Ther & Nllc Med 116:249,1972

Pneumatosis cystoides intestinalisdiagnosed by colonoscopy

Laurent G. DesbaiUets, M.D.Jagdish C. Mangla, M.D.*

Gastroenterology DepartmentMonroe Community Hospital

(affiliated with The University of RochesterSchool of Medicine and Dentistry)

435 East Henrietta RoadRochester, New York 14603

Pneumatosis cystoides intestinalis coli is a rare diseasepresenting as intramural collections of gas bubbles withinthe colonic wall, and occasionally in the mesentery .1.2 Twoforms have been described: a primary form in which thecolon involvement is not associated with other diseases,and a secondary form wherein cystic collections of gasin the colon are associated with peptic ulcer, carcinomaof the stomach, or obstructive pulmonary disease. Theprimary disease is very uncommon and the patient's symp­toms, proctosigmoidoscopic, and roentgenologic findingsmay lead to a mistaken diagnosis of colonic cancer andunnecessary surgery.3

The patient described in this report was sent to MonroeCommunity Hospital for custodial care of cancer of therectosigmoid region; at colonoscopy a correct diagnosisof pneumatosis cystoides intestinalis coli was made.

CASE REPORT An 86 year old white man was first seenin another hospital in April 1972 for a routine check-up.On rectal examination, a mass was discovered at the finger­tip, and a subsequent proctosigmoidoscopy led to a diag­nosis of multiple polyps of the rectum. The patient wassigmoidoscoped again in October and December, 1972, anda diagnosis ofcarcinoma of the rectosigmoid area was madedespite a negative biopsy. Due to the extensive bowelinvolvement, the case was considered as terminal, and thepatient was hospitalized at the Monroe Community Hospi­tal. No history of abdominal pain, changes of bowel habits,or rectal bleeding was elicited. Examination of the abdomen

"Reprint requests: J. C. Mangla. M.D., at the address given.

10. WILLlAMSJR LF, BOSNIAK MA, WITTENBURGJ, MANUEL B, GRIMESET, BYRNE JJ: Ischemic colitis. Am J Surg 117:254, 1969

11. CARTER R, VANNI X R, HINSHAW DB, STAFFORD CEo Inferior mesen­teric vascular occlusion: sigmoidoscopic diagnosis. Surgery 46:845,1959

12. LITTMAN L, BOLEY SJ, SCHWARTS S: Sigmoidoscopic diagnosis of rever­sible vascular occlusion of the colon. Dis Colon Rec/um 6: 142, 1963

13. KILPATRIC ZM, FARMAN J, YESNER R, SPIRO HM: Ischemic proctitis.JAMA 205:74, 1968

14. DAWSON MA, SCHAEFERJW: The clinical course of reversible ischemiccolitis. Gas/roen/erology 60:577, 1971

15. DE DOMBAL FT, FLETCHER OM, HARRIS RS: Early diagnosis ofischemic colitis. GUI 10:131,1969

16. PARKS TG, JOHNSTON GW, KENNEDY TL, GOUGH AD: Spontaneousischaemic proctocolitis. Scand J Gas/roen/erol 7:241, 1972

17. BOLEY SJ, AGRAWAL GP, WARREN AR, VEITH VJ, LEVOWITZ BS,TREIBER W, DOUGHERTY J, SCHWARTZ SS, GLiEDMAN ML:Pathophysiologic effects of bowel distention on intestinal blood flow.Amer J Surg 117:228, 1969

was completely negative. Several stool specimens gave noreaction for occult blood. The rest of the physical examina­tion, routine laboratory studies, and x-rays of the chestwere within normal limits.

Sigmoidoscopic examination revealed at 8 em from theanal verge a cluster of globular masses, projecting in thelumen and obstructing the insertion of the sigmoidoscope.Colonoscopy was performed (Olympus CF), and multiplecystic masses were seen, varying in diameter between 0.5cm and 3.0 em and sometimes giving the appearance ofa grape-like cluster protruding in the lumen (Figure 1). Theoverlying musoca over many of these masses was semi­transparent. The same picture was seen from the upperrectum up to the mid-transverse colon, and the endoscopicfindings were thought to be compatible with penumatosiscystoides intestinalis.

Figure 1: Colonoscopic appearance of pneumatosis cystoidesintestinalis: (A) large cyst, 3 em; (8) gas cysts beyond the spe­nic flexure; (C) cluster of cysts in sigmoid colon; (D) same cystsas seen in C when patient was straining.

GASTROINTESTINAL ENDOSCOPY

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Figure 2: Barium enema show­ing (A) typical scalloped ap­pearance, with barium outliningthe gas cysts; (B) large airspaces outlined by the bariumcolumn seen in the region ofrec­tosigmoid.

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The barium enema (Figure 2) confirmed the findings andrevealed gas bubbles involving only the left side of thecolon. Barium meal examination of the stomach and smallintestine was normal. Biopsies done from several of thesecysts showed normal overlying colonic mucosa.

COMMENT This case impresses the need for thoroughendoscopic examination of the colon in unusual cases andfor an accurate interpretation of the findings. In a reviewof 213 cases of pneumatosis cystoides intestinalis, coloninvolvement was found in only 5% of the cases. 4 Less than100 cases involving only the colon have been reported inthe English literature to date. 5

The etiology of gas cysts in colon is far from clear; thepostulated mechanism in obstructive pulmonary disease isconsidered to be an alveolar rupture in the lungs and thegas tracking down the blood vessels to the colonic wall. 6

Once cysts are formed, they are thought to be replenishedby intraluminal bowel gas. 7•8

Pneumatosis cystoides intestinalis coli may be asymp­tomatic, but diarrhea is a common presenting symptom.Other symptoms may be constipation, flatulence, abdomi­nal discomfort, and occasionally bleeding. The appearanceof these lesions on endoscopic examination is characteris­tic: cysts can be of variable sizes and shapes, projectinginto the lumen in a scalloped manner. The overlying mucosais intact, pale, transparent, and at times hemorrhagic. 3

"Balloon popping" biopsy reveals a normal mucosa withcystic spaces. There may sometimes be an inflammatoryreaction with local collections of histiocytes and giantcells. 4 • 7 On barium enema one can visualize multiplesmooth lucent areas protruding in the lumen, outlined bythe barium column. 1 '

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Nonspecific symptoms and the lack of characteristic find­ings on physical examination make the diagnosis difficult.The appearance of the lesions on colonoscopic examinationis so striking that the diagnosis will usually be apparent.It also allows one to make a full assessment of the colon,since polyps or neoplasm may not be easily differentiatedfrom multiple cysts on x-ray studies. 3

Pneumatosis cystoides intestinalis is a benign disease.Therapy should be expectant and surgery reserved for com-

VOLUME 20, NO.3, 1974

plications such as bleeding, obstruction, or perforation.Recently penumatosis cystoides has been treated with suc­cess by oxygen breathing for several days, with completedisappearance ofthe cystS. 8 •9 It remains to be seen whetheroxygen therapy will be useful on a long-term follow-up.Oxygen therapy was not considered in the patient presentedbecause he was asymptomatic.

REFERENCESI. MARSHAK RH, lIPSAY JJ, FRIEDMAN AI: Penumatosis of the colon.

lAMA 148:1416, 19522. MARSHAK RH, BLUM SO, ELIASOPH J: Pneumatosis involving the left

side of the colon. lAMA 161: 1626, 19563. SMITH WG, ANDERSON MJ, PEMBERTON HW: Pneumatosis cystoides

intestinalis involving left portion of the colon; report of 4 cases diagnosedat sigmoidoscopy. Gastroenterology 35:528. 1958

4. Koss LG: Abdominal gas cysts (Pneumatosis cystoides intestinorumhominis). Arch Pathol 53:523, 1952

5. ECKER JA, WILLIAMS RG, CLAY' KL: Pneumatosis cystoides intes·tina lis-Bullous emphysema of the intestine; a review of the literature.Arnerl Gastroenterol 56: 124, 1971

6. KEYTlNG WS, MCCARVER RR, KOVARIK JL, DAYWITT AL:Pneumatosis intestinalis: a new concept. Radiology 76:733, 1961

7. DOUB HP, SHEA JJ: Pneumatosis cystoides intestinalis. lAMA 172: 1238,1960

8. WYATT AP: Pneumatosis cystoides intestinalis. Proc Roy Soc Med 65:30,1972

9. FORGACS P, WRIGHT PH, WYATT AP: Treatment of intestinal gas cystsby oxygen breathing. Lancet 1:579, 1973

William BeaumontGastrointestinal Symposium

The Third Annual William Beaumont GastrointestinalSymposium for federal service physicians (Army, Navy,Air Force, Veterans Administration, and Public HealthService) interested in the fields of gastroenterology or gas­trointestinal surgery will be held at William Beaumont ArmyMedical Center, EI Paso, Texas, 13-15 March 1974.

Further information may be obtained from J. LorenPitcher, M.D., F.A.C.P., COL MC, Chief, Departmentof Medicine and Chief, Gastroenterology Service, Box70031, William Beaumont Army Medical Center, EI Paso,Texas 79920.