Henry Ford Hospital Medical Journal

Volume 17 | Number 4 Article 8

12-1969 Cystoides Intestinalis: The Report of a Case Associated with Severe Pulmonary Disease and Chronic Lymphocytic Leukemia Russell J. Crider

Philip Bentlif

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Recommended Citation Crider, Russell J. and Bentlif, Philip (1969) "Pneumatosis Cystoides Intestinalis: The Report of a Case Associated with Severe Pulmonary Disease and Chronic Lymphocytic Leukemia," Henry Ford Hospital Medical Journal : Vol. 17 : No. 4 , 285-294. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol17/iss4/8

This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp. Med. Journal Vol. 17, No. 4, 1969

Pneumatosis Cystoides Intestinalis: The Report of a Case Associated with Severe Pulmonary Disease and Chronic Lymphocytic Leukemia

Russell J. Crider, Jr., M.D.* and Philip Bentlif, M.D.**

Pneumatosis cystoides intestinalis is discussed with emphasis on its clinical, radiologic, sigmoidoscopic, surgical and pathological characteristics. The four theories of the etiology of P.Cl. are neoplastic, infectious, nutritional, and mechanical. These theories are discussed, and a case of P.C.I, associated with severe pulmonary disease and chronic lymphocytic leukemia is presented to lend support to the mechanical theory.

First noted by Duvernoy in 1730, submucosal are characterized pneumatosis cystoides intestinalis chiefly by the firm, spongy sensation (P.C.I.) is a rare disorder character­ they present on palpation. Although ized by submucosal or subserosal gas size varies, the majority are 0.5 to 1.0 cysts in the intestinal wall. Other names cm in diameter with thin, smooth, given to the disorder include diffuse bluish walls, which are usually tense emphysema of the intestinal wall, mul­ but break readily with the release of tiple gas cysts of the intestine, emphy­ enclosed gas. sema of the cecum and gas cysts of the Often there are associated lesions intestine, t such as , , or Gross Anatomy . Although the distribution 1. Appearance at laparotomy and of the lesions is variable, the small autopsy. bowel is most commonly affected and The gross appearance of P.C.I, has is often associated with other pyloric been described as resembling soap lesions (eg, stenosis). Involvement of bubbles, hydatid cysts, hydatidiform the iliocecal region is also common and moles, clusters of grapes, etc. Not is usually associated with no other readily apparent on laparotomy, the demonstrable gastrointestinal lesion. Only 13 of the 213 cases investigated

* Kaiser Foundation Hospital, San Fran­ by Koss had lesions distal to the hepatic cisco, California flexure, indicating the rarity of P.C.I, * * Baylor University College of Medicine, in the distal colon, sigmoid and rec­ Houston, Texas tum.^ Presented at the Resident Education Seminar of the Henry Ford Hospital 2. Appearance at sigmoidoscopy. House Staff Association, May 17, 1969. In 1944 K. Rojel made the first t There have been several excellent articles written on P.C.I, including sources 1-7. diagnosis of P.C.I, by proctoscopy. He

285 Crider and Bentlif recorded the following description of level is 80% nitrogen, 20% oxygen the circumferential lesion: "From the and .04% carbon dioxide. appearance of the nodules, they could There are, however, several difficul­ have acted like mucous cysts, but when ties in determining accurate average the top of two were pierced, they de­ values for the gas contents of the cysts. flated without evidence of mucus. They The major problem is that the methods seemed to be filled with air."^ The of obtaining the gas samples are in­ diagnostic feature seen at sigmoido­ accurate, especially since the cystic scopy is the rapid deflation of the cysts gas contents change drastically in 20 upon biopsy. In a case report by Doub minutes (eg, oxygen concentration and Shea, "Each bleb when biopsied changes from 1.4% to 13.1% in 20 yielded a report as of a balloon pop­ minutes). 13 Thus all postmortem anal­ ping, clearly audible throughout the yses are unacceptable. room."» Radiologic Appearance Microscopic Appearance In 1924 Reverdin made the first The microscopic features of P.C.I, roentgenographic description of P.C.I. vary with the stage of development of However, it was diagnosed by laparot­ the lesion. Koss postulates the follow­ omy. Lemer and Gazin were the first ing microscopic stages in the develop­ to make the diagnosis by x-ray before ment of P.C.I.: by nodng in 1946 that, "The 1. Formation of cysts with simple presence of inconsistent filling defects endothelial lining; of increased translucency is patho- 2. Formation of giant cells; gomonic." The radiologic finding of 3. Inflammatory reaction in the sur­ interposition of bowel between the rounding ; and the diaphragm (the Moreau- 4. Sloughing off of giant cells with Chilaiditi sign) is common in P.C.I, concomitant of surround­ and should immediately arouse suspi­ ing stroma; cion of its presence. 5. Diminution of the size of the cysts to the point of complete Theories of Etiology fibrosis with ultimate disappear­ Although the etiology of P.C.I, is ance as scarring progresses.^ not known, four major theories have The lesions are usually submucosal been put forth: in infants, but in adults (except those 1. The neoplastic theory, with so-called primary P.C.I.), they 2. The infectious theory,^' are generally subserosal. 3. The nutritional theory, and Analyses of the gaseous content of 4. The mechanical theory. the cysts is estimated at 70%-90% ni­ The neoplastic and infectious trogen, 3%-20% oxygen and 0%- theories have few supporters and little 15% carbon dioxide with traces of supporting evidence. The nutritional methane.i'' " !^ Intraluminar bowel gas theory is based on the premise that contains about 0.5% oxygen, 45% ni­ the imbalance of certain nutritional trogen, 40% carbon dioxide and 14% factors changes the permeability of the hydrogen.-- By comparison, air at sea bowel wall allowing the collection of

286 Pneumatosis Cystoides Intestinalis

gas in the intestinal lymphatics. P.C.I, lesion from a disease process. has been produced in 100% of a test Koss found that 75% of 213 group of swine fed 78% polished rice cases of P.C.I, had other gastroin­ and 20% skimmed milk. If 5% baker's testinal lesions. Of these, 76% had yeast or 50% whole grain were added pyloric or gastric disease, the most to this diet, very few of the swine common being pyloric stenosis with developed P.C.I. However, similar die­ peptic ulcer. He postulates that, un­ tary deficiencies in humans do not der increased pressure at the site of appear to produce P.C.I. the stenosis, the gas enters the lym­ Some investigators believe the dis­ phatic channels via the ulcer and is order is due to malnutrition, eg, as forced along the gut lymphatics by caused by pyloric stenosis.However, peristalsis. On the other hand, Key- there is no increased incidence of tingi-' suggests that the ulcer often P.C.I, in people who are malnourished allows communication with the because of lack of food. blood vessels at the roots of the Some authors have proposed that mesentery and the gas follows these an increase of lacdc acid in the intestine vessels to the subserosal of the gut (possibly secondary to the high milk wall. It was found that, in dogs, air intake on an ulcer diet) causes the entering through experimentally pro­ lactic acid to be absorbed into the duced ulcers passed along the bowel lacteals where it combines with alka­ via lymphatics with vigorous peris­ line chyme producing carbon dioxide talsis.A great deal of direct sup­ and nitrogen (from the blood).i^'i'^ port was given to the mechanical Added support for this theory comes theory when a patient vomited dur­ from Domini and Matolise who sup­ ing laparotomy under spinal anesthe­ port an eclectic theory of "biochemical sia. The cysts were seen to increase dystrophic and nutritional deficiency," in size during the act of after reviewing 711 cases of entero- with no increase of the bowel size. peritoneal pneumatosis. 2. Secondary to a gastrointestinal Many authors subscribe to some lesion resulting from a surgical pro­ form of the nutritional theory, even cedure. though lesions often occur in the bowel It was first noticed in 1956 that in the area of least absorbency. there had been several instances The mechanical theory states that when P.C.I, of the descending colon the air arrives in the cysts by dissecting closely followed proctoscopy, with along fascial and vascular planes. Sub­ and without biopsy.^o -s It also fol­ groups of the mechanical theory are lowed rectal surgery.2-* that the cysts are: 1. secondary to gas­ 3. Secondary to severe pulmonary trointestinal lesions resulting from a disease. disease process, 2. secondary to gas­ The most recent addition to the trointestinal lesions from operative mechanical theory was introduced trauma, and 3. secondary to severe by Keydngi'* in 1961. He notes that pulmonary disease. several of his cases of P.CL, as well 1. Secondary to a gastrointestinal as others from the hteraturc-'^^'-"

287 Crider and Bentlif

had severe pulmonary disease us­ cussion note over the distended ab­ ually of the obstructive type.* The domen and a palpable . proposed mechanism of the pulmon­ Other symptoms may arise from ary cause of P.C.I, is that coughing complications of P.C.I, including spon­ or straining to exhale leads to dis­ taneous ,*" volvu­ section of air from ruptured alveoli lus," syndrome,*! ir­ to the mediastinum, then by peri­ ritable bowel syndrome,!! and a sprue­ vascular tissue to the mesentery and like syndrome.3- the intestinal wall. There is a great The symptoms are so nonspecific deal of experimental evidence to that the diagnosis is usually made by support this theory on animals-* and radiological examinations, sigmoido­ on cadavers.13 It should be noted scopy, or laparotomy. that most of the so-called primary lesions and the lesions associated Treatment and Prognosis with pulmonary disease occur distal Most authors prefer conservative to the splenic flexure. treatment with the use of stool soften­ ers, because lesions will often regress Clinical Picture o£ P.C.I. spontaneously. Resection is reserved The incidence of all forms of adult for localized disease that is intractable P.C.I, is highest in the 30- to 50-year- or life threatening.* " ''It should be old group with a 3.5 to 1 ratio of males noted that surgical correction of a to females. Before 1951, only 44 cases lesion causing P.C.I, (eg, pyloric sten­ of pediatric P.C.I., a much more ser­ osis and peptic ulcer) cures the P.C.I. ious disorder, had been reported. The adult disease seems to be world-wide Comparative studies of the treatment with no apparent racial predisposition. of P.C.I, were done by Pemberton'^ and The presenting symptoms of P.C.I, Smith." Because of the generally poor are usually those of an associated dis­ conditions of the patients, conservative order (eg, pyloric stenosis). When the measures should be attempted with P.C.I, is symptomatic, the patient us- surgery as a final measure or for cor­ uaUy complains of vague abdominal rection of other lesions or complica­ cramping , intermittent constipa­ tions. The prognosis for adult P.C.I, tion and mucoid (sometimes blood- in any location is good, so the patient's tinged) , and inter­ ultimate course usually depends upon mittent abdominal distention. The dis­ underlying disease. order is very often asymptomatic and Case Report may appear and disappear rapidly.' Mrs. A. O., a 62-year-old white house­ Often the disease may become asymp­ wife, was diagnosed as having chronic tomatic with no appreciable change in lymphocytic leukemia in August 1962. She had chronic with a severe chronic the radiologic and sigmoidoscopic find­ cough, occasional shortness of breath on ings." is usually exertion and occasional wheezing. Leukeran therapy was begun in 1963 with good results negative except for a tympanitic per- until a sudden drop in her white cell count and red blood cell count necessitated mul­ * Since this article there have been many tiple transfusions with the discontinuance of reports of the association of respiratory Leukeran. Prednisone therapy then proved disease and P.CL: 2, 9, 15, 18, 25, 27, 28, effective with no serious side effects. 29. On March 13, 1967, she was admitted to

288 Pneumatosis Cystoides Intestinalis

Figure 1 Photomicrograph of sigmoid biopsy specimen showing the typical pathologic changes of pneumatosis cystoides intestinals (see text).

289 Crider and Bentlif

The Methodist Hospital, Houston, Texas, gave the initial impression of rectal polyps. with the complaint of diarrhea which had The pathologic specimen showed the typical begun one month earlier and had progressed changes of P.CL (Fig 1 and 2) with mul­ to a watery, mucoid diarrhea without blood tiple small submucosal lesions partly lined the week before. Although her appetite dur­ by multinucleated giant cells. Later, barium ing this period remained normal, she lost examination of the bowel showed typical ten pounds. She also reported intermittent radiologic features of P.CL Multiple radio- abdominal , increased flatulence and lucent filling defects extended from the belching and an occasional mild intermittent lower sigmoid to the upper portion of the cramping gastric pain. She had no previous descending colon (Fig 3). history of changing bowel habits, tarry A liver biopsy showed hemachromatosis, stools or abdominal discomfort. thought to be secondary to multiple earlier She had smoked heavily until 1957, when blood transfusions. she stopped because of her chronic bron­ Normal bowel movements began 10 days chitis. Surgical operations included removal after the patient was treated conservatively of a portion of grangrenous bowel in 1948. with the stool softeners, magnesium sulfate This came as a result of an acute intestinal and sulfonamides, which were continued obstruction due to volvulus following a after discharge. On March 28, 1967, another unilateral oophorectomy and appendectomy barium study showed no significant change in 1947. No note was made of P.CL at this in her P.C.I. Although she has remained time. asymptomatic, except for occasional abdom­ Physical examination showed an obviously inal cramping, repeat proctoscopy and bar­ Cushingoid woman, not chronically or ium enema studies six months later showed acutely distressed, with a blood pressure of no significant changes from the examinations 160/70, temperature 98.4°F, pulse 96/min on the day after her admission to The and regular, and respiratory rate 22/min. Methodist Hospital. Pertinent physical findings included dry, thin skin with multiple ecchymotic lesions; a Discussion slightly distended, tympanitic with normal bowel sounds; a firm splenic edge This is the second reported case of felt 3-4 cm below the left costal margin at the mid-clavicular line and a large, fixed, P.C.I, associated with chronic lympho­ non-tender mass palpable in the left lower cytic leukemia,^'' although there is one quadrant. Her chest was clear to auscultation and percussion, and there were no palpable previously reported case of P.C.I, with lymph nodes. lymphosarcoma.-'! Our case differs Admitting laboratory results were: hemo­ from these other two in that our pa­ globin, 14.9 gms; hematocrit, 46%; white blood count, 12,600/cu mm, with 36% tient's leukemia is well controlled and segmented cells, 62% mature lymphocytes, there is no lymphocytic infiltrate sur­ 1% eosinophils, and 1% basophils. Reticu­ locytes, 3.4%; platelets, 176,000; urinalysis, rounding the cysts. Since the lesions normal; serology, nonreactive; BUN, 43 were of the descending and sigmoid mg %; creatinine, 2.0 mg %; electrolytes, normal; prothrombin time, 100%; total colon and the patient had severe serum protein, 6 mg %; serum electro­ disease with vigorous coughing epi­ phoresis showed normal protein fractions; ova and parasite study of stools was nega­ sodes, the pulmonary etiology seems tive; BSP, 25% (on repeat, 21% retention to be the most likely basis for her in 30 minutes); alkaline phosphatase, 1.7 Bessey-Lowry units; total bilirubin was 0.4 P.C.I. mg %; thymol turbidity, 1 unit; SGOT, 126 units; and SGPT was 250 units. The correct diagnosis was made Proctoscopy performed the day after ad­ both by proctoscopic biopsy and by mission revealed large, boggy, polypoid x-ray. It saved unnecessary surgery on lesions scalloped into the lumen from 18 to 23 cm. Biopsies of the pale friable mucosa a poor-risk patient.

290 Pneumatosis Cystoides Intestinalis

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* *

Figure 2 Higher power of Figure 1 showing giant cells and gas cysts (top) immediately under the colonic mucosa (bottom).

291 Crider and Bentlif

REFERENCES L Finney, J.M.T.: Gas cysts of the intestine. JAMA 51:1291-8, 1908. 2. Jackson, J.A.: Gas cysts of the intestine. Surg Gynec Obstet 71:675-8, Nov 1940. 3. Koss, L.G.: Abdominal gas cysts (pneumatosis cystoides intestinorum hominis). Arch Path 53:523-49, Jun 1952. 4. MacKenzie, E.P.: . Pediatrics 7:537-49, Apr 1951. 5. Moore, R.A.: Intestinal pneumatosis. Amer J Dis Child 38:818-23, Oct 1929. 6. Smith, W.G.; Anderson, M.L; and Pemberton, H.W.: Pneumatosis cystoides intestinalis involving left portion of colon: Report of four cases diagnosed at sigmoidoscopy. Gastroenterology 35:528-33, Nov 1958. 7. Pemberton, H.W.; Smith, W.G.; and Holman, C.B.: Pneumatosis cystoides intestinalis diagnosed sigmoidoscopically. Amer J Surg 94:472-7, Sept 1957. 8. Rojel, K.: Un cas de pneumatose kystique intestinale diagnostique par la rectoscopie. Acta Chir Scand 97:276-82, 1949. 9. Doub, HP., and Shea, LL: Pneumatosis cystoides intestinalis. JAMA 172:1238-42, 19 Mar 1960. 10. McGregor, J.K., and McKinnon, D.A., Jr.: Intestinal interstitial emphysema. Gastroen­ terology 35:206-11, Aug 1958. 11. Kushlan, S.D.: Pneumatosis cystoides intestinalis. JAMA 179:699-702, 3 Mar 1962. 12. Druckmann, A., et al: Pneumatosis of the intestines. Amer J Roentgen 86:911-9, Nov 1961. 13. Keyting, W.S., et al: Pneumatosis intestinalis: A new concept. Radiology 76:733-41, May 1961. 14. Bang, B.L.F.: Luftholdige Kyster i Vaggen af og i nydannet bindevav pa sammes serosa (Gaseous cysts in wall of ileum and in serous part of newly formed connective tissue). Nord med Ark 8:1-16, 1876. 15. Alva, L, and Schuster, M.M.: Pneumatosis cystoides coli. Gastroint Endosc 12:19-24, May 1966. 16. Thorpe, G.L: Pneumatosis cystoides intestinalis associated with abdominal carcinomatosis. Postgrad Med J 41:41-2, Jan 1965. 17. Villaume, C: Pneumatosis cystoides intestinalis. Nord Med 49:912-4, 26 Jun 1953. 18. Culver, G.L: Pneumatosis intestinalis with associated retroperitoneal air: Report of a complication of severe . JAMA 186:160-2, 12 Oct 1963. 19. Tung, P.C, and Ngai, S.K.: Gas cysts of the intestines. Chinese Med J 47:1-14, Jan 1933. 20. Kreeger, N., and Littman, L.: Pneumatosis cystoides intestinorum. Report of a case confirmed by sigmoidoscopic biopsy. New Eng J Med 251:779-81, 4 Nov 1954. 21. Matthews, F.J.C: Enteric pneumatosis. Brit Med J 1:851-2, 10 Apr 1954. 22. Marshak, R.H.; Bluhm, S.D.; and Eliasoph, L: Pneumatosis involving the left side of the colon. JAMA 161:1626-8, 25 Aug 1956. 23. Sedgwick, C.E., and Ruddell, K.S.: Pneumatosis cystoides intestinalis. Lahey Clin Found Bull 8:238-43, Apr 1954. 24. Burt, C.A.V.: Emphysematous and from intra­ rectal operation. Ann Surg 130:277-80, Aug 1949.

292 Pneumatosis Cystoides Intestinalis

Figure 3 Roentgenogram of barium enema of patient on March 14, 1967. Note the radiolucent filling defects in the descending and sigmoid colon of air density characteristic of P.CL

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25. Mujahed, Z., and Evans, J: Gas cysts of the intestine. Surg Gynec Obstet 107:151-60, Aug 1958. 26. Macklin, CC: Transport of air along sheaths of pulmonic blood vessels from alveoli to mediastinum. Arch Intern Med 64:913-26, Nov 1939. 27. Plachta, A., and Speer, F.D.: Mechanism of nonbacterial gastric emphysema. Gastroen­ terology 40:248-52, Feb 1961. 28. Creevey, K.; Cohen, LL., and Riley, G.L: Pneumatosis of the descending colon. New Eng J Med 259:912-5, 6 Nov 1958. 29. Montani, S., and Landry, M.: La pneumatose kystique intestinale est-elle une complica­ tion de I'emphyseme pulmonaire? Gastroenterologia (Basel) 103:234-44, 1965. 30. Hahn, E.: Uber Pneumatosis Cystoides Intestorum und einen durch Laparatomie behandebten Fall. Comentarii Acad Suintarum Imp Petrop 5:213. (20) 31. Williams, LL; Sutherland, D.H., and Clark, C.G.: Lymphosarcoma of the with a malabsorption syndrome and pneumatosis intestinalis. Gastroenterology 45:550-7, Oct 1963. 32. Yunich, A.M., and Fradkin, N.F.: Fatal sprue (malabsorption) syndrome secondary to extensive pneumatosis cystoides intestinalis. Gastroenterology 35:212-7, Aug 1958.

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