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Colonic Perforation in Crohn's Disease
Vol. 71. No.5 Print,d," U.S.A.
GASrROENTElIOLOGY 71:835-838. 1976 Copyright ~ 1976 by The Williams & Wilkins Co.
COLONIC PERFORATION IN CROHN'S DISEASE JOHN FISHER, M.D., FRANK MANTZ, M.D., AND W. GRAHAM CALKINS, M.D. Department8 of Medicine and Pathology University of Kansas Medical Center, Kansas City. Kansas, and Veterans Administration Hospital. Kansas City. Missouri
Three cases of acute, free perforation of the colon in Crohn's disease are described and their clinical and pathological features are detailed. Colonic perforation in Crohn's disease bears no definite relationship to obstruction or adrenocorticosteroid therapy. It may occur with or without toxic megacolon, and pathological study has not thus far suggested a common pathogenetic mechanism . Although free perforation has been considered to be extremely rare in Crohn's colitis, it is likely that this complication is more common than previously believed. Free intestinal perforation during the course of Crohn's disease is an infrequent complication. In 1957 Crohn had not yet encountered this complication in his own experience. 1 However, a case of free perforation of the small intestine in Crohn's disease had been reported as early as 1935 by Arnheim t and was followed in 1937 by a second report. a Since that time, DeCourcy' has collected 14 cases from the medical literature, and Menguy· states that at least 100 instances of this complication have been reported. Thus, it appears that free perforation of the small intestine, although unusual, is a well documented complication of Crohn's disease. On the other hand, free perforation of the colon in the course of Crohn's disease has been considered exceedingly rare. Farmer et al. have recently reported a study of 615 patients with Crohn's disease of whom 166 had only colon involvement.' Although 18 of these patients had megacolon, none had free perforation of the colon. Greenstein et al. found only 1 patient who had a free perforation of the transverse colon in a review of 160 patients with Crohn's colitis. 7 Since the description of this complication by Javett and Brooke in 1970,' we have been able to find only 4 additional reported cases. 7011 Nevertheless, 3 cases of free colonic perforation reported here were encountered at the University of Kansas Medical Center and its affiliated Veterans Administration hospital in the relatively short period of 8 months, suggesting that this complication ofCrohn's disease may not be as rare as previously believed. Case 1 W. D., a 17-year-old boy, was admitted to the University of Kansas Medical Center with persistent abdominal pain, diarrhea, and fever. Eleven months previOllsly an abdominal laparotomy was performed with resection of the terminal ileum and ascending colon and the construction of an end-to-end Received July 24. 1975. Accepted May 3. 1976. Address requests for reprints to: W. Graham Calkins, M.D., Vet· erans-Administration Hospital. Kansas City. Mi6SOUri 61428. Dr. Fisher's present address is: 1800 Sullivan Avenue. Suite 304. Daly City. California 94015.
ileocolostomy. At that time the diagnosis of Crohn's disease was established. After surgery he continued to have frequent diarrhea, abdominal cramps, and a low grade fever. Corticosteroids and salicylazosulfapyradine were begun, but the patient's symptoms continued. The patient was subsequently referred to this hospital for further management. On admission, the physical examination revealed a well nourished young man in no distress. The only abnormal physical finding was mild, diffuse abdominal tenderness. No masses were felt. Bowel sounds were normal. Rectal and proctosigmoidoscopic examinations were normal. The initial laboratory examinations were unremarkable except Cor barium X-rays of the upper and lower gastrointestinal tract, which showed changes in the jejunum and ileum consistent with Crohn's disease. Neither obstruction nor fistulae were identified . In spite of medical management with corticosteroids, adrenocorticotrophic hormone, and salicylazosulfapyradine, the patient continued to have abdominal pain and occult blood in his frequent diarrheal stools. Twenty·two days later the patient was allowed home on a weekend pass. Within a few hours after leaving the hospital, the patient returned complaining of severe abdominal pain. At that time the abdomen was tense and tympanic and had markedly diminished bowel sounds. Abdominal X·rays showed a pneumoperitoneum, and an emergency exploratory abdominal laparotomy was immediately performed. The previous anastamotic site between ileum and colon was found to have marked inflammatory reaction with omental adhesions. There was a free colonic perforation just distal to the anastamosis. The perforation was closed and the involved bowel was resected. An end-to-end ileocolonic anastamosis was reconstructed. The postoperative recovery was uneventful. Eleven months after surgery, the patient continues to have active inflammatory bowel disease in spite of intensive chemotherapy. Case 2 J. H., a 28-year-old male, was admitted to the Kansas City Veterans Administration Hospital 24 hr after the sudden onset of severe, diffuse abdominal pain. The patient's illness began approximately 4 years before the current admission. At that time. the patient presented with frequent bloody stools and the diagnosis of Crohn's disease of the small and large bowel was established. Since that time, the patient had been treated with corticosteroids and salicylazo-
8.15
8:36
Vol. 71, No ..5
CASE REPORTS
sulfapyradine. During the 2 years before the present admission he was maintained in clinical remission on these drugs. On admission the patient appeared critically ill and was in shock. His abdomen was distended and had board like rigidity. Bowel sounds were ahsent. The WAC count was 22,800. An emergency exploratory laparotomy was performed . On entering the peritoneal cavity, purulent fluid was encountered. Limited exploration revealed inflammatory changes of the ileum and colon. A 5-mm free perforation in the sigmoid colon was found. Because of the patient's poor condition. a diverting colostomy was performed and peritoneal irrigation with kanamycin was carried out. Postoperatively the patient did poorly, developing multiple intraahdominal abscesses. Surgery was again performed 22 days later. At that time multiple abscesses were drained. Two ileal perforations were also noted and were treated by simple closure. A partial left colectomy was performed with the formation of a proximal transverse loop colostomy. The patient did well for 4 weeks, at which time signs of an acute ahdomen developed. A third abdominal exploration was performed. Purulent fluid was found, hut no frank perforation was grossly demonstrated. The remaining colon distal to the loop colostomy was markedly dilated and hyperemic . This remaining segment of colon was removed and a right transverse colostomy was refashioned. After this procedure the patient's course was uneventful and he was discharged on continued medical management. Case 3 R. Z.. a 64-year-old male, first developed symptoms of "colitis" at age 1:1. He had no gastrointestinal complaints until 19:>8, when an 8·month history of diarrhea prompted admis· sion to the Kansas City Veterans Administration Hospital. A right abdominal mass was felt and a harium enema X-ray suggested a malignancy of the ascending colon. An exploratory laparotomy was performed. Inflammatory bowel disease involving the right colon . cecum. appendix . and terminal ileum was found . A resection of the right colon and 22 em of ileum was performed. with the formation of ileotransverse colostomy . The patient remained asymptomatic until 197:l when he was readmitted to the hospital complaining of bloody diarrhea and abdominal cramping. He appeared acutely ill. Vital signs were normal. The abdomen was soft and slightly tender, without guarding or rebound. Howel sounds were normal. Several hours after hospitalization the patient 's condition deteriorated. with the dev(>lopment of abdominal distention. rigidity, and dimin· ishing bowel sounds. Abdominal X·rays revealed massive pneumoperitoneum and the pat ient was taken to surgery. On opening of the peritoneal ('avity, a large amount of air was pxpelled . There was one colonic perfroation proximal to the spleniI' flexure and seven perforations in the mid , descending "olon. Thl' ('olon and 10 e lll of the distal ileum were reseded. An ilpostomy was formed and tbe rectal slllmp was closed, creat ing a Hart mann's pouch. The day after surgery, massive bleeding from the remaining rectum occurred. An emergency procl<'('/om ,v was thell ('arried Ollt. The patient followed a progn',si\'elv septic and deteriorating course and died I" da~" s latN, Path{)l{)~i('al
phangitis, and interstitial fibrosis. In every instance, there were multiple scattered narrow ulcerations with slightly overhanging borders. Multiple scattered noncaseating granulomas were found in all colons. No acid-fast or fungal organisms were demonstrated in any . There was nothing in the history or surgical findings of any of the 3 patients to suggest that perforation was the result of a foreign body, manipulation, or trauma. In case I, a 4-mm free perforation was identified in the sigmoid colon just distal to a previous ileocolic anastamosis (fig. 1). In case 2, the perforation was 5 mm in diameter and located at the splenic flexure. A second sinus tract extending to the serosa was well sealed off by adhesions to adjacent structures. In both instances, perforations had occurred on the free or anti mesenteric surfaces and were surrounded by fibrinopurulent inflammation on their peritoneal aspects. In both instances, the perforations were located within the base of preexisting active ulcers and appeared to represent somewhat chronic penetrating sinuses which had blown out as the serosa was approached (fig. 2). Their chronicity is attested to by their granulation tissue linings, in which a somewhat granulomatous inflammatory reaction was apparent (fig. 3). The entire picture in these 2 cases indicates a long standing indolent disorder in acute exacerbation. Case :3 likewise showed evidence of chronic active Crohn 's disea~e in acute exacerbation, with changes similar to those noted above. There were at least seven perforations, however, in the transverse and descending colon, also occurring through the base of otherwise typical ulcerations of Crohn 's disease. They were exceedingly wide, measuring up to 3 em in diameter, and appeared infinitely more acute, with little granulation tissue in their lining and much acute inflammatory infiltration (fig. 4). This seems to fit the clinical history of an acute fulminating exacerbation of a process which had long been clinically quiescent. From the pathologist's point of view, it appears that the perforations in case 1 and case 2 occurred from
Features
All :\ cases exhibitecl the typical features of chronic active (:rohn's disease. with transmural int1ammation most marked in the mucosa and submucosa. These feature s were associated with lymphangiectasia . telangit·(·tilsia . interstitial edema, nodular periphlebitis. ly'm-
Fu;, I
(;ro~s
s pf'('irnl'n
a :-;. m .. 11 uh'{>r jus t dis tal
l ' (b('
tlllhll
appt·ar . . n -Ial i\' ~ I .\ · Ilormal.
I . Tht.· ornnl' illdi n lll':o. a pt·rtoratinl\ in
ile(l("olic
anastaJllo~i :-. .
Tllf' ih'uIH Iri)!htl
November 1976
CASE REPORTS
....
" ..... ~.:.. ......... . .~
,
FI(:. 2. Case I-perforated sinus penetrating wall of eolon from base of ulcer. A similar perforation was noted in ease 2.
FIG. :\. Cas~ 2. Chronically inflamed granulation tissue lining perforated sinus suggesting COIl1"iderahle duration of lesion. ~ott~ LanKhans Iype Kianl cell. This degree of
rt'ad ion
was noled likewise in
the perforated sinus in case 1.
8:3/
Although free perforation of the colon in Crohn's disease had not been generally appreciated until Javett and Brooke's report in 1970,' a careful review of the medical literature has revealed 2 possible additional cases of free cecal perforation in Crohn's ileocolitis preceding their report. Fischman and Gruzowoy'3 may have been the first to document colonic perforation in 1965, and in 1968 Kyle, et al." included 1 case of cecal perforation in their 8-year experience. More recent cases of free colonic perforation in Crohn's colitis have been reported by others·' '6-12 It is likely that additional cases have been mistakenly attributed to patients with idiopathic ulcerative colitis. The :J patients reported here represent unequivocal cases of free colonic perforation in Crohn's disease. Free perforation of the colon in Crohn's disease differs from the similar complication in the small intestine. It is less frequent and tends to occur apart from the site of mesenteric attachment. There is no association with obstruction, a feature commonly described in free perforation of the small intestine.· The complication may occur with or without toxic megacolon and may arise as an extension from a preexistent penetrating sinus as well as by penetration of an acutely necrotic ulcer. Age, duration of clinical illness, and the use of corticosteroids do not appear to be of significance. However, the role of corticosteroids in the pathogenesis remains uncertain. Of our:3 patients, 2 were on corticosteroids at the lime of perforation. It appears that free colonic perforation can occur in Crohn's colitis both in patients who are taking corticosteroids and those who are not. 1'\0 specific pathogenetic mechanisms are apparent in our study. Indeed, the variability of the clinical pictures in these :3 patients is noteworthy. It has previously been emphasized by .Javett and Brooke" that Crohn's disease of the colon mav not be distinguished from other inflammatory bowel' diseases by the presence or absence of acute toxic megacolon. The increasing recognition of free perforation also makes this
indolent penetrating sinuses. which had not previously approached the serosal surface closely enough to incite the usual adhesive peritoneal inflammatory reaction. In case :3, on the other hand, the entire picture was one of exceedingly acute fulminating disease with dissolution of the bowel wall at the base of relatively broad ulcers. In no instance was there evidence of an obstructive component and only in case :3 were there features suggesting toxic megacolon.
Discussion l;ntil recently it was axiomatic that free intestinal perforat ion did not occur in ('rohn's disease. It is now recognized that free perforation as well as the more commonl~' occurring closed perforation does occur. Williams 12 has reported a 1'; incidence of small intestinal perforations in collected reports of chronic ('mhn's disease and a ~'; incidence of free perforation of the small bowel in his own experience of over :lOO pat ienls.
FI(;, -1. ('asp :~-hordpr of :~'('Ill pt'rtorated ulrPL 'ntp mft~si\'t>
npcrosis of howel wall wit hOllt ~lgJlifil'nnt (lTgalli/at jlln or gral111lat ion tissue.
838
CASE REPORTS
complication a poor differential feature . It can no loger be assumed that persons with established Crohn's disease involving the colon will not develop this catastrophic complication. REFERENCES 1. Crohn BB: Indications for surgical intervention in regional ileitis. Arch Surg 74:305-311, 1957
2. Arnheim EE: Regional i1eitia with perforation, abscess and peritonitis. J Mt Sinai HCllp 2:61-63, 1935 3. Hallegan EJ, Halligan HJ: Acute free perforation as a fint sign of regional enteritis. Am J Surg 37:493-495. 1937 4. DeCourcy CB: Regional enteritis with free perforation of the jejunum and generalized peritonitis. South Med J 66:1145-1146, 1973
5. Menguy R: Surical management of free perforation of the small intestine complicating regional enteritis. Ann Surg 175:178-189, 1972
Vol . 71, No . 5
6. Farmer RG, Hawk WA, Turnbull RB Jr: Clinical patterns in Crohn'. disease: a statistical study of 615 cases. Gastroenterology 68:627-635, 1975
7. Greenstein AJ, Kark AE, Dreiling DA: Crohn's disease of the colon. TIl. Toxic dilatation of the colon in Crohn's colitis. Am J Gastroenterol 63(2):117-128, 1975 8. Javett SL, Brooke BN: Acute dilatation of colon in Crohn's disease. Lancet 2:126-128, 1970 9. Shamanesh J, Wilken BJ: Perforated Crahn'•. Lancet 2:363-364, 1970 10. Tugwell P, Southcott C, Walmesley PL: Free perforation of colon in Crohn's disease. Br J Clin Pract 26:44-45, 1972
11. Steinberg DM, Cooke WT, Williams JA: Free perforation in Crahn's disease. Gut 14:187-190, 1973 12. Williams JA: The place of surgery in Crohn's disease. Gut
12:739-749, 1971 13. Fischman DM, Gruzowoy J: Ileocolitis de Crohn can perforation libre. Prensa Med Argent 52:2116-2119, 1965 14. Kyle J , Caridis T: Duncan T, et al: Free perforation in regional enteritis. Am J Dig Dis 13:275-283, 1968
GASrROENTElIOLOGY 71:835-838. 1976 Copyright ~ 1976 by The Williams & Wilkins Co.
COLONIC PERFORATION IN CROHN'S DISEASE JOHN FISHER, M.D., FRANK MANTZ, M.D., AND W. GRAHAM CALKINS, M.D. Department8 of Medicine and Pathology University of Kansas Medical Center, Kansas City. Kansas, and Veterans Administration Hospital. Kansas City. Missouri
Three cases of acute, free perforation of the colon in Crohn's disease are described and their clinical and pathological features are detailed. Colonic perforation in Crohn's disease bears no definite relationship to obstruction or adrenocorticosteroid therapy. It may occur with or without toxic megacolon, and pathological study has not thus far suggested a common pathogenetic mechanism . Although free perforation has been considered to be extremely rare in Crohn's colitis, it is likely that this complication is more common than previously believed. Free intestinal perforation during the course of Crohn's disease is an infrequent complication. In 1957 Crohn had not yet encountered this complication in his own experience. 1 However, a case of free perforation of the small intestine in Crohn's disease had been reported as early as 1935 by Arnheim t and was followed in 1937 by a second report. a Since that time, DeCourcy' has collected 14 cases from the medical literature, and Menguy· states that at least 100 instances of this complication have been reported. Thus, it appears that free perforation of the small intestine, although unusual, is a well documented complication of Crohn's disease. On the other hand, free perforation of the colon in the course of Crohn's disease has been considered exceedingly rare. Farmer et al. have recently reported a study of 615 patients with Crohn's disease of whom 166 had only colon involvement.' Although 18 of these patients had megacolon, none had free perforation of the colon. Greenstein et al. found only 1 patient who had a free perforation of the transverse colon in a review of 160 patients with Crohn's colitis. 7 Since the description of this complication by Javett and Brooke in 1970,' we have been able to find only 4 additional reported cases. 7011 Nevertheless, 3 cases of free colonic perforation reported here were encountered at the University of Kansas Medical Center and its affiliated Veterans Administration hospital in the relatively short period of 8 months, suggesting that this complication ofCrohn's disease may not be as rare as previously believed. Case 1 W. D., a 17-year-old boy, was admitted to the University of Kansas Medical Center with persistent abdominal pain, diarrhea, and fever. Eleven months previOllsly an abdominal laparotomy was performed with resection of the terminal ileum and ascending colon and the construction of an end-to-end Received July 24. 1975. Accepted May 3. 1976. Address requests for reprints to: W. Graham Calkins, M.D., Vet· erans-Administration Hospital. Kansas City. Mi6SOUri 61428. Dr. Fisher's present address is: 1800 Sullivan Avenue. Suite 304. Daly City. California 94015.
ileocolostomy. At that time the diagnosis of Crohn's disease was established. After surgery he continued to have frequent diarrhea, abdominal cramps, and a low grade fever. Corticosteroids and salicylazosulfapyradine were begun, but the patient's symptoms continued. The patient was subsequently referred to this hospital for further management. On admission, the physical examination revealed a well nourished young man in no distress. The only abnormal physical finding was mild, diffuse abdominal tenderness. No masses were felt. Bowel sounds were normal. Rectal and proctosigmoidoscopic examinations were normal. The initial laboratory examinations were unremarkable except Cor barium X-rays of the upper and lower gastrointestinal tract, which showed changes in the jejunum and ileum consistent with Crohn's disease. Neither obstruction nor fistulae were identified . In spite of medical management with corticosteroids, adrenocorticotrophic hormone, and salicylazosulfapyradine, the patient continued to have abdominal pain and occult blood in his frequent diarrheal stools. Twenty·two days later the patient was allowed home on a weekend pass. Within a few hours after leaving the hospital, the patient returned complaining of severe abdominal pain. At that time the abdomen was tense and tympanic and had markedly diminished bowel sounds. Abdominal X·rays showed a pneumoperitoneum, and an emergency exploratory abdominal laparotomy was immediately performed. The previous anastamotic site between ileum and colon was found to have marked inflammatory reaction with omental adhesions. There was a free colonic perforation just distal to the anastamosis. The perforation was closed and the involved bowel was resected. An end-to-end ileocolonic anastamosis was reconstructed. The postoperative recovery was uneventful. Eleven months after surgery, the patient continues to have active inflammatory bowel disease in spite of intensive chemotherapy. Case 2 J. H., a 28-year-old male, was admitted to the Kansas City Veterans Administration Hospital 24 hr after the sudden onset of severe, diffuse abdominal pain. The patient's illness began approximately 4 years before the current admission. At that time. the patient presented with frequent bloody stools and the diagnosis of Crohn's disease of the small and large bowel was established. Since that time, the patient had been treated with corticosteroids and salicylazo-
8.15
8:36
Vol. 71, No ..5
CASE REPORTS
sulfapyradine. During the 2 years before the present admission he was maintained in clinical remission on these drugs. On admission the patient appeared critically ill and was in shock. His abdomen was distended and had board like rigidity. Bowel sounds were ahsent. The WAC count was 22,800. An emergency exploratory laparotomy was performed . On entering the peritoneal cavity, purulent fluid was encountered. Limited exploration revealed inflammatory changes of the ileum and colon. A 5-mm free perforation in the sigmoid colon was found. Because of the patient's poor condition. a diverting colostomy was performed and peritoneal irrigation with kanamycin was carried out. Postoperatively the patient did poorly, developing multiple intraahdominal abscesses. Surgery was again performed 22 days later. At that time multiple abscesses were drained. Two ileal perforations were also noted and were treated by simple closure. A partial left colectomy was performed with the formation of a proximal transverse loop colostomy. The patient did well for 4 weeks, at which time signs of an acute ahdomen developed. A third abdominal exploration was performed. Purulent fluid was found, hut no frank perforation was grossly demonstrated. The remaining colon distal to the loop colostomy was markedly dilated and hyperemic . This remaining segment of colon was removed and a right transverse colostomy was refashioned. After this procedure the patient's course was uneventful and he was discharged on continued medical management. Case 3 R. Z.. a 64-year-old male, first developed symptoms of "colitis" at age 1:1. He had no gastrointestinal complaints until 19:>8, when an 8·month history of diarrhea prompted admis· sion to the Kansas City Veterans Administration Hospital. A right abdominal mass was felt and a harium enema X-ray suggested a malignancy of the ascending colon. An exploratory laparotomy was performed. Inflammatory bowel disease involving the right colon . cecum. appendix . and terminal ileum was found . A resection of the right colon and 22 em of ileum was performed. with the formation of ileotransverse colostomy . The patient remained asymptomatic until 197:l when he was readmitted to the hospital complaining of bloody diarrhea and abdominal cramping. He appeared acutely ill. Vital signs were normal. The abdomen was soft and slightly tender, without guarding or rebound. Howel sounds were normal. Several hours after hospitalization the patient 's condition deteriorated. with the dev(>lopment of abdominal distention. rigidity, and dimin· ishing bowel sounds. Abdominal X·rays revealed massive pneumoperitoneum and the pat ient was taken to surgery. On opening of the peritoneal ('avity, a large amount of air was pxpelled . There was one colonic perfroation proximal to the spleniI' flexure and seven perforations in the mid , descending "olon. Thl' ('olon and 10 e lll of the distal ileum were reseded. An ilpostomy was formed and tbe rectal slllmp was closed, creat ing a Hart mann's pouch. The day after surgery, massive bleeding from the remaining rectum occurred. An emergency procl<'('/om ,v was thell ('arried Ollt. The patient followed a progn',si\'elv septic and deteriorating course and died I" da~" s latN, Path{)l{)~i('al
phangitis, and interstitial fibrosis. In every instance, there were multiple scattered narrow ulcerations with slightly overhanging borders. Multiple scattered noncaseating granulomas were found in all colons. No acid-fast or fungal organisms were demonstrated in any . There was nothing in the history or surgical findings of any of the 3 patients to suggest that perforation was the result of a foreign body, manipulation, or trauma. In case I, a 4-mm free perforation was identified in the sigmoid colon just distal to a previous ileocolic anastamosis (fig. 1). In case 2, the perforation was 5 mm in diameter and located at the splenic flexure. A second sinus tract extending to the serosa was well sealed off by adhesions to adjacent structures. In both instances, perforations had occurred on the free or anti mesenteric surfaces and were surrounded by fibrinopurulent inflammation on their peritoneal aspects. In both instances, the perforations were located within the base of preexisting active ulcers and appeared to represent somewhat chronic penetrating sinuses which had blown out as the serosa was approached (fig. 2). Their chronicity is attested to by their granulation tissue linings, in which a somewhat granulomatous inflammatory reaction was apparent (fig. 3). The entire picture in these 2 cases indicates a long standing indolent disorder in acute exacerbation. Case :3 likewise showed evidence of chronic active Crohn 's disea~e in acute exacerbation, with changes similar to those noted above. There were at least seven perforations, however, in the transverse and descending colon, also occurring through the base of otherwise typical ulcerations of Crohn 's disease. They were exceedingly wide, measuring up to 3 em in diameter, and appeared infinitely more acute, with little granulation tissue in their lining and much acute inflammatory infiltration (fig. 4). This seems to fit the clinical history of an acute fulminating exacerbation of a process which had long been clinically quiescent. From the pathologist's point of view, it appears that the perforations in case 1 and case 2 occurred from
Features
All :\ cases exhibitecl the typical features of chronic active (:rohn's disease. with transmural int1ammation most marked in the mucosa and submucosa. These feature s were associated with lymphangiectasia . telangit·(·tilsia . interstitial edema, nodular periphlebitis. ly'm-
Fu;, I
(;ro~s
s pf'('irnl'n
a :-;. m .. 11 uh'{>r jus t dis tal
l ' (b('
tlllhll
appt·ar . . n -Ial i\' ~ I .\ · Ilormal.
I . Tht.· ornnl' illdi n lll':o. a pt·rtoratinl\ in
ile(l("olic
anastaJllo~i :-. .
Tllf' ih'uIH Iri)!htl
November 1976
CASE REPORTS
....
" ..... ~.:.. ......... . .~
,
FI(:. 2. Case I-perforated sinus penetrating wall of eolon from base of ulcer. A similar perforation was noted in ease 2.
FIG. :\. Cas~ 2. Chronically inflamed granulation tissue lining perforated sinus suggesting COIl1"iderahle duration of lesion. ~ott~ LanKhans Iype Kianl cell. This degree of
rt'ad ion
was noled likewise in
the perforated sinus in case 1.
8:3/
Although free perforation of the colon in Crohn's disease had not been generally appreciated until Javett and Brooke's report in 1970,' a careful review of the medical literature has revealed 2 possible additional cases of free cecal perforation in Crohn's ileocolitis preceding their report. Fischman and Gruzowoy'3 may have been the first to document colonic perforation in 1965, and in 1968 Kyle, et al." included 1 case of cecal perforation in their 8-year experience. More recent cases of free colonic perforation in Crohn's colitis have been reported by others·' '6-12 It is likely that additional cases have been mistakenly attributed to patients with idiopathic ulcerative colitis. The :J patients reported here represent unequivocal cases of free colonic perforation in Crohn's disease. Free perforation of the colon in Crohn's disease differs from the similar complication in the small intestine. It is less frequent and tends to occur apart from the site of mesenteric attachment. There is no association with obstruction, a feature commonly described in free perforation of the small intestine.· The complication may occur with or without toxic megacolon and may arise as an extension from a preexistent penetrating sinus as well as by penetration of an acutely necrotic ulcer. Age, duration of clinical illness, and the use of corticosteroids do not appear to be of significance. However, the role of corticosteroids in the pathogenesis remains uncertain. Of our:3 patients, 2 were on corticosteroids at the lime of perforation. It appears that free colonic perforation can occur in Crohn's colitis both in patients who are taking corticosteroids and those who are not. 1'\0 specific pathogenetic mechanisms are apparent in our study. Indeed, the variability of the clinical pictures in these :3 patients is noteworthy. It has previously been emphasized by .Javett and Brooke" that Crohn's disease of the colon mav not be distinguished from other inflammatory bowel' diseases by the presence or absence of acute toxic megacolon. The increasing recognition of free perforation also makes this
indolent penetrating sinuses. which had not previously approached the serosal surface closely enough to incite the usual adhesive peritoneal inflammatory reaction. In case :3, on the other hand, the entire picture was one of exceedingly acute fulminating disease with dissolution of the bowel wall at the base of relatively broad ulcers. In no instance was there evidence of an obstructive component and only in case :3 were there features suggesting toxic megacolon.
Discussion l;ntil recently it was axiomatic that free intestinal perforat ion did not occur in ('rohn's disease. It is now recognized that free perforation as well as the more commonl~' occurring closed perforation does occur. Williams 12 has reported a 1'; incidence of small intestinal perforations in collected reports of chronic ('mhn's disease and a ~'; incidence of free perforation of the small bowel in his own experience of over :lOO pat ienls.
FI(;, -1. ('asp :~-hordpr of :~'('Ill pt'rtorated ulrPL 'ntp mft~si\'t>
npcrosis of howel wall wit hOllt ~lgJlifil'nnt (lTgalli/at jlln or gral111lat ion tissue.
838
CASE REPORTS
complication a poor differential feature . It can no loger be assumed that persons with established Crohn's disease involving the colon will not develop this catastrophic complication. REFERENCES 1. Crohn BB: Indications for surgical intervention in regional ileitis. Arch Surg 74:305-311, 1957
2. Arnheim EE: Regional i1eitia with perforation, abscess and peritonitis. J Mt Sinai HCllp 2:61-63, 1935 3. Hallegan EJ, Halligan HJ: Acute free perforation as a fint sign of regional enteritis. Am J Surg 37:493-495. 1937 4. DeCourcy CB: Regional enteritis with free perforation of the jejunum and generalized peritonitis. South Med J 66:1145-1146, 1973
5. Menguy R: Surical management of free perforation of the small intestine complicating regional enteritis. Ann Surg 175:178-189, 1972
Vol . 71, No . 5
6. Farmer RG, Hawk WA, Turnbull RB Jr: Clinical patterns in Crohn'. disease: a statistical study of 615 cases. Gastroenterology 68:627-635, 1975
7. Greenstein AJ, Kark AE, Dreiling DA: Crohn's disease of the colon. TIl. Toxic dilatation of the colon in Crohn's colitis. Am J Gastroenterol 63(2):117-128, 1975 8. Javett SL, Brooke BN: Acute dilatation of colon in Crohn's disease. Lancet 2:126-128, 1970 9. Shamanesh J, Wilken BJ: Perforated Crahn'•. Lancet 2:363-364, 1970 10. Tugwell P, Southcott C, Walmesley PL: Free perforation of colon in Crohn's disease. Br J Clin Pract 26:44-45, 1972
11. Steinberg DM, Cooke WT, Williams JA: Free perforation in Crahn's disease. Gut 14:187-190, 1973 12. Williams JA: The place of surgery in Crohn's disease. Gut
12:739-749, 1971 13. Fischman DM, Gruzowoy J: Ileocolitis de Crohn can perforation libre. Prensa Med Argent 52:2116-2119, 1965 14. Kyle J , Caridis T: Duncan T, et al: Free perforation in regional enteritis. Am J Dig Dis 13:275-283, 1968