Scleroderma of the Colon with Obstruction: Report
ROBERT P. DAVIS, M.D.,
,]AIVIES R.
of a Case*
HINES, M.D., WILLIAM R. FI,INN, M.D.
From the Department o] Surgery, Northwestern University Medical School, Chicago, Illinois
foul" years earlier. Past history revealed t h a t t h e patient h a d tlad a Billroth II gastrectomy for peptic ulcer disease in 1965, a n d h a d been treated for R a y n a u d ' s p h e n o m e n o n for seven years. She h a d heen treated for myocardial c o n d u c t i o n defects for two years. D u r i n g the past year she had h a d two hospital admissions for large-bowel o b s t r u c t i o n secondary to colonic impaction a n d h a d been treated with nasogastric suction, cathartics, Anvac, m i n e r a l oil, Potoba, p o t a s s i u m chloride, a n d daily enemas. At this admission the p a t i e n t was thin, dehydrated, a n d h a d thickened, s m o o t h skin with a tightly d r a w n facies. T h e a b d o m e n was grossly distended and h y p e r r e s o n a n t , a n d bowel s o u n d s were absent. Diffuse tenderness was present in a n otherwise soft, " d o u g h y " a b d o m e n . T h e r e was n o stool in the a m p u l l a of the r e c t u m . T h e leukocyte c o u n t was 5,900, with a m a r k e d shift to the left. H e m a t o c r i t was 46 per cent. Laboratory studies revealed s e r u m s o d i u m 133 m g / 1 0 0 ml, p o t a s s i u m 4.7 rag/100 ml, chloride 93 m g / 1 0 0 m l a n d a blood urea n i t r o g e n of 27 rag/100 ml. R o e n t g e n o g r a m s of the a b d o m e n revealed a colon full of s e m i o p a q u e material a n d m u l t i p l e air fluid levels in the small bowel. T h e p a t i e n t was treated with nasogastric suction, i n t r a v e n o u s fluids, a n d a r e g i m e n of m u l t i p l e daily enemas. T e n days later she was operated u p o n . T h e colon was f o u n d to be filled with thick stool. A r i g h t transverse colostomy was performed, as colonic resection was d e e m e d inadvisable. D u r i n g the n e x t two m o n t h s the p a t i e n t was treated with h y p e r a l i m e n t a t i o n , nasogastric feedings of Vivonex@ a n d daily colostomy a n d rectal irrigations. In D e c e m b e r 1973, a second l a p a r o t o m y was carried o u t a n d subtotal colectomy with end-to-end ileoproctostomy was performed. T h e resected s e g m e n t of colon was 130 cm long a n d 8.5 to 10.5 cm in circumference. T h e wall of the colon was markedly t h i n n e d , a n d was filled w i t h a yellowish-white dense, putty-like material. Microscopic sections showed focal areas of fibrosis w i t h i n the s u b m u c o s a a n d a t t e n u a t i o n of the m u s c u l a r i s propria. T h e intermyenteric plexus a p p e a r e d normal. T h e small a n d medium-sized vessels were n o r m a l in a p p e a r a n c e (Figs. 1 a n d 2).
RAKE x~ is credited with the first description of a patient with scleroderma of the colon. His description of the dilated colon filled with dense, putty-like masses is classic. However, his interpretation of the pathophysiology was incorrect, as he believed that the underlying defect was in the sympathetic nervous system rather than in the collagen deposition in the bowel wall. Reports of surgical intervention for intestinal scleroderma are few, and the results of operation often have been poor. Prior to 1962 there were only ten such instances reported, and five patients had died in the postoperative period, s Lately the outlook for patients receiving surgical treatment has improved; one such patient who had coIonic perforation and three who had largebowel obstrnction have been successfully treated by colonic resection. 2, 5. s T h e current case would appear to be the fifth instance of a patient with colonic sclerod e r m a having survived resection of the colon, and the first case in which the patient was treated by subtotal colectomy. R e p o r t of a Case A 35-year-old white w o m a n k n o w n to have scleroderma was a d m i t t e d to hospital in October 1973, with a four-day history of obstipation, n a u s e a a n d vomiting. T h e diagnosis of scleroderma h a d been established by skin, muscle a n d rectal biopsies * Received for publication J u n e 11, 1975. Address r e p r i n t requests to Dr. Hines: N o r t h western M e m o r i a l Hospital, D e p a r t m e n t of Surgery --Suite 728, 250 East Superior Street, Chicago, Illinois 60611.
256 Dis. Col. & Rect. April 1976
Volume 19 Number 3
Volume 19 Number 3
SCLERODERMA OF THE COLON
257
FIG. 1. Photomicrograph of the colon, demonstrating submucosal fibrosis and marked atrophy of the muscularis (• The patient had an uneventful immediate postoperative course and did well for 18 months. She returned to work and gained 20 pounds of weight. Lomotil| therapy was necessary to control diarrhea, but later she had two stools a day without medication. At that time the diarrhea gradually began to return and she began to lose weight. X-rays revealed scleroderma of the distal small intestine. She is currently being treated for malabsorption syndrome.
most c o m m o n i n i t i a l s y m p t o m is R a y n a u d ' s p h e n o m e n o n . S y m p t o m s r e f e r a b l e to the g a s t r o i n t e s t i n a l tract a p p e a r in only 10 p e r cent of p a t i e n t s p r i o r to c u t a n e o u s m a n i festations. 1 I n t e s t i n a l s c l e r o d e r m a may be m a n i f e s t e d by b l e e d i n g , o b s t r u c t i o n , perf o r a t i o n or even m a l a b s o r p t i o n . Most investigators state that systemic sclerosis
Comment
affects the g a s t r o i n t e s t i n a l tract in decreas-
Systemic sclerosis (scleroderma) affects w o m e n three times as o f t e n as men. I t occurs a l m o s t solely in Caucasians, and in 75 per cent of the p a t i e n t s it occurs between the ages of 30 and 60 years. 9 T i l e
ing i n c i d e n c e f r o m the e s o p h a g u s caudad. In P o i r i e r and R a n k i n ' s 9 r e t r o s p e c t i v e analysis of 364 cases, the i n c i d e n c e of smallbowel i n v o l v e m e n t was 47.7 per cent, a n d the colon was f o u n d to be i n v o l v e d in 8.9
958
DAVIS, E T AL.
Dis. Col. &Rect. April 1976
Fig. 2. Photomicrograph showing submucosal fibrosis, a normal myenteric plexus, and normal vasculature (• 125). per cent of the cases. Patients with intestinal scleroderma are usually asymptomatic, but may have malabsorption or gastrointestinal atony.
Pathologic changes in scleroderma of tile colon are frequently reflected by the roentgenographic findings. Replacement of the smooth muscle of the muscularis mucosae
Volume 19 Number 3
SCLERODERMA
by collagen tissue in a patchy d i s t r i b u t i o n produces d i v e r t i c u l a or "sacculations." T h e s e saccnlations are caused by i n a b i l i t y of the collagenous, fibrotic areas to withs t a n d the i n t r a l u m i n a l pressure p r o d u c e d by the c o n t r a c t i n g colonic m u s c u l a t u r e . ~ T h e sacculations are true d i v e r t i c u l a , in that each c o n t a i n s all layers of the wall of the colon. T h e s e sacculations are best demo n s t r a t e d on the p o s t e v a c u a t i o n r o e n t g e n ograms, as they c o n t a i n inspissated fecal material.4.7 T h e s e areas of sacculation often a l t e r n a t e with areas of rigidity, as is seen in ulcerative colitis? As the disease 1)rogresses to involve larger p o r t i o n s of the colon, tim sacculations tlisappear a n d b a r i u m - e n e m a studies d e m o n s t r a t e a r162 colon, full of fecal tnaterial. 8 Medial hypertrol)hy of the sntall vessels of the colon ntay give rise to ulceration, l e a d i n g to either b l e e d i n g or p e r f o r a t i o n : s " L u s h b a u g h et el. 6 d e m o n s t r a t e d a n ulceration in the d e s c e n d i n g colon in a p a t i e n t who died of this c o m p l i c a t i o n . 6 Jayson et el. s reported survival of a p a t i e n t whose p e r f o r a t i o n was i n the sigmoid colon. Both p a t i e n t s had p e r f o r a t i o n s w i t h i n areas of c h r o n i c u l c e r a t i o n , the former w i t h i n a d i v e r t i c t d n m a n d the latter b e t w e e n two sacculations. T h e descriptions of the p a t h o p h y s i o l o g y in the three r e p o r t e d instances of colonic atony treated by resection were essentially the same as i n the c u r r e n t case. 2, s All the p a t i e n t s had several years of c o n s t i p a t i o n , at times a l t e r n a t i n g with diarrhea. H o s p i t a l admissions were necessary in each i n s t a n c e because of o b s t r u c t i o n of the large bowel secondary to fecal i m p a c t i o n . I n a case rep o r t e d by C o m p t o n , 2 a loop colostomy was necessary to p r e p a r e the bowel adequately. O u r case r e p o r t is obviously one of technical f r u s t r a t i o n i n cleansing the bowel, as the stool in this p a t i e n t was thick a n d very
O1" T H E
259
COLON
difficult to remove. Even w i t h daily irrigations t h r o u g h b o t h ends of the colostomy a n d the rectum, the colon still c o n t a i n e d a large q u a n t i t y of stool at the time of subtotal colectomy. Summary A case of a p a t i e n t who h a d colonic obstruction caused by scleroderma is described. T i l e o b s t r u c t i o n i n i t i a l l y was treated by means of a colostomy, a n d later s u b t o t a l colectomy was carried out. T h i s is the fifth report of a p a t i e n t with s y m p t o m a t i c coIonic scleroderma successfully treated 1)y c:olonic resection. A review of colonic sclerotlerma is inclutled. References I. Cassada WA, Armstrong RH Jr, Neal MP Jr: Involvement of the gastrointestinal tract by progressive systemic sclerosis. South Med J 6t: 475, 1968 2. Compton R: Scteroderma with diverticuIosis and colonic obstructinn. Am J Surg 118:602. 1969 3. Hale CH, Schatzki R: The roentgenolo~cal a.ppearance of the gastrointestinal tract m scleroderma. Am J Roentgenol Radium Ther Nucl Med 51: 407, 1944 4. Heinz ER, Steinberg AJ, Sackner MA: Roentgenographic and pathologic aspects of intestinal scleroderma. Ann Intern Med 59: 822, 1963 5. Jayson MI, Salmon PR, Gough J, et al: Spontaneous bowel perforation in intestinal scleroderma: First report of a non4atal case. Postgrad Med J 48: 56, 1972 6. Lushbaugh CC; Rubin L, Rothman S: Scleroderma of the intestinal tract: First report of a fatal case. Gastroenterology 11: 382, 1948 7. Meszaros WT: The colon in systemic sclerosis (scleroderma). Am J Roentgenol Radium Ther Nucl Med 82: 100, 1959 8. Norton RA, Monroe LS: The surgical approach to gastrointestinal scleroderma: Report of a case with partial colonic resection. Am J Dig Dis 7: 766, 1962 9. Poirier TJ, Rankin GB: Gastrointestinal manifestations of progressive systemic scleroderma based on a review of 364 cases. Am J. Gastroenterol 58: 30, 1972. 10. Rake G: On the pathology and pathogenesis of scleroderma. Bull Johns Hopkins Hosp 48: 212, 1931
ROBERT P. DAVIS, M.D.,
,]AIVIES R.
of a Case*
HINES, M.D., WILLIAM R. FI,INN, M.D.
From the Department o] Surgery, Northwestern University Medical School, Chicago, Illinois
foul" years earlier. Past history revealed t h a t t h e patient h a d tlad a Billroth II gastrectomy for peptic ulcer disease in 1965, a n d h a d been treated for R a y n a u d ' s p h e n o m e n o n for seven years. She h a d heen treated for myocardial c o n d u c t i o n defects for two years. D u r i n g the past year she had h a d two hospital admissions for large-bowel o b s t r u c t i o n secondary to colonic impaction a n d h a d been treated with nasogastric suction, cathartics, Anvac, m i n e r a l oil, Potoba, p o t a s s i u m chloride, a n d daily enemas. At this admission the p a t i e n t was thin, dehydrated, a n d h a d thickened, s m o o t h skin with a tightly d r a w n facies. T h e a b d o m e n was grossly distended and h y p e r r e s o n a n t , a n d bowel s o u n d s were absent. Diffuse tenderness was present in a n otherwise soft, " d o u g h y " a b d o m e n . T h e r e was n o stool in the a m p u l l a of the r e c t u m . T h e leukocyte c o u n t was 5,900, with a m a r k e d shift to the left. H e m a t o c r i t was 46 per cent. Laboratory studies revealed s e r u m s o d i u m 133 m g / 1 0 0 ml, p o t a s s i u m 4.7 rag/100 ml, chloride 93 m g / 1 0 0 m l a n d a blood urea n i t r o g e n of 27 rag/100 ml. R o e n t g e n o g r a m s of the a b d o m e n revealed a colon full of s e m i o p a q u e material a n d m u l t i p l e air fluid levels in the small bowel. T h e p a t i e n t was treated with nasogastric suction, i n t r a v e n o u s fluids, a n d a r e g i m e n of m u l t i p l e daily enemas. T e n days later she was operated u p o n . T h e colon was f o u n d to be filled with thick stool. A r i g h t transverse colostomy was performed, as colonic resection was d e e m e d inadvisable. D u r i n g the n e x t two m o n t h s the p a t i e n t was treated with h y p e r a l i m e n t a t i o n , nasogastric feedings of Vivonex@ a n d daily colostomy a n d rectal irrigations. In D e c e m b e r 1973, a second l a p a r o t o m y was carried o u t a n d subtotal colectomy with end-to-end ileoproctostomy was performed. T h e resected s e g m e n t of colon was 130 cm long a n d 8.5 to 10.5 cm in circumference. T h e wall of the colon was markedly t h i n n e d , a n d was filled w i t h a yellowish-white dense, putty-like material. Microscopic sections showed focal areas of fibrosis w i t h i n the s u b m u c o s a a n d a t t e n u a t i o n of the m u s c u l a r i s propria. T h e intermyenteric plexus a p p e a r e d normal. T h e small a n d medium-sized vessels were n o r m a l in a p p e a r a n c e (Figs. 1 a n d 2).
RAKE x~ is credited with the first description of a patient with scleroderma of the colon. His description of the dilated colon filled with dense, putty-like masses is classic. However, his interpretation of the pathophysiology was incorrect, as he believed that the underlying defect was in the sympathetic nervous system rather than in the collagen deposition in the bowel wall. Reports of surgical intervention for intestinal scleroderma are few, and the results of operation often have been poor. Prior to 1962 there were only ten such instances reported, and five patients had died in the postoperative period, s Lately the outlook for patients receiving surgical treatment has improved; one such patient who had coIonic perforation and three who had largebowel obstrnction have been successfully treated by colonic resection. 2, 5. s T h e current case would appear to be the fifth instance of a patient with colonic sclerod e r m a having survived resection of the colon, and the first case in which the patient was treated by subtotal colectomy. R e p o r t of a Case A 35-year-old white w o m a n k n o w n to have scleroderma was a d m i t t e d to hospital in October 1973, with a four-day history of obstipation, n a u s e a a n d vomiting. T h e diagnosis of scleroderma h a d been established by skin, muscle a n d rectal biopsies * Received for publication J u n e 11, 1975. Address r e p r i n t requests to Dr. Hines: N o r t h western M e m o r i a l Hospital, D e p a r t m e n t of Surgery --Suite 728, 250 East Superior Street, Chicago, Illinois 60611.
256 Dis. Col. & Rect. April 1976
Volume 19 Number 3
Volume 19 Number 3
SCLERODERMA OF THE COLON
257
FIG. 1. Photomicrograph of the colon, demonstrating submucosal fibrosis and marked atrophy of the muscularis (• The patient had an uneventful immediate postoperative course and did well for 18 months. She returned to work and gained 20 pounds of weight. Lomotil| therapy was necessary to control diarrhea, but later she had two stools a day without medication. At that time the diarrhea gradually began to return and she began to lose weight. X-rays revealed scleroderma of the distal small intestine. She is currently being treated for malabsorption syndrome.
most c o m m o n i n i t i a l s y m p t o m is R a y n a u d ' s p h e n o m e n o n . S y m p t o m s r e f e r a b l e to the g a s t r o i n t e s t i n a l tract a p p e a r in only 10 p e r cent of p a t i e n t s p r i o r to c u t a n e o u s m a n i festations. 1 I n t e s t i n a l s c l e r o d e r m a may be m a n i f e s t e d by b l e e d i n g , o b s t r u c t i o n , perf o r a t i o n or even m a l a b s o r p t i o n . Most investigators state that systemic sclerosis
Comment
affects the g a s t r o i n t e s t i n a l tract in decreas-
Systemic sclerosis (scleroderma) affects w o m e n three times as o f t e n as men. I t occurs a l m o s t solely in Caucasians, and in 75 per cent of the p a t i e n t s it occurs between the ages of 30 and 60 years. 9 T i l e
ing i n c i d e n c e f r o m the e s o p h a g u s caudad. In P o i r i e r and R a n k i n ' s 9 r e t r o s p e c t i v e analysis of 364 cases, the i n c i d e n c e of smallbowel i n v o l v e m e n t was 47.7 per cent, a n d the colon was f o u n d to be i n v o l v e d in 8.9
958
DAVIS, E T AL.
Dis. Col. &Rect. April 1976
Fig. 2. Photomicrograph showing submucosal fibrosis, a normal myenteric plexus, and normal vasculature (• 125). per cent of the cases. Patients with intestinal scleroderma are usually asymptomatic, but may have malabsorption or gastrointestinal atony.
Pathologic changes in scleroderma of tile colon are frequently reflected by the roentgenographic findings. Replacement of the smooth muscle of the muscularis mucosae
Volume 19 Number 3
SCLERODERMA
by collagen tissue in a patchy d i s t r i b u t i o n produces d i v e r t i c u l a or "sacculations." T h e s e saccnlations are caused by i n a b i l i t y of the collagenous, fibrotic areas to withs t a n d the i n t r a l u m i n a l pressure p r o d u c e d by the c o n t r a c t i n g colonic m u s c u l a t u r e . ~ T h e sacculations are true d i v e r t i c u l a , in that each c o n t a i n s all layers of the wall of the colon. T h e s e sacculations are best demo n s t r a t e d on the p o s t e v a c u a t i o n r o e n t g e n ograms, as they c o n t a i n inspissated fecal material.4.7 T h e s e areas of sacculation often a l t e r n a t e with areas of rigidity, as is seen in ulcerative colitis? As the disease 1)rogresses to involve larger p o r t i o n s of the colon, tim sacculations tlisappear a n d b a r i u m - e n e m a studies d e m o n s t r a t e a r162 colon, full of fecal tnaterial. 8 Medial hypertrol)hy of the sntall vessels of the colon ntay give rise to ulceration, l e a d i n g to either b l e e d i n g or p e r f o r a t i o n : s " L u s h b a u g h et el. 6 d e m o n s t r a t e d a n ulceration in the d e s c e n d i n g colon in a p a t i e n t who died of this c o m p l i c a t i o n . 6 Jayson et el. s reported survival of a p a t i e n t whose p e r f o r a t i o n was i n the sigmoid colon. Both p a t i e n t s had p e r f o r a t i o n s w i t h i n areas of c h r o n i c u l c e r a t i o n , the former w i t h i n a d i v e r t i c t d n m a n d the latter b e t w e e n two sacculations. T h e descriptions of the p a t h o p h y s i o l o g y in the three r e p o r t e d instances of colonic atony treated by resection were essentially the same as i n the c u r r e n t case. 2, s All the p a t i e n t s had several years of c o n s t i p a t i o n , at times a l t e r n a t i n g with diarrhea. H o s p i t a l admissions were necessary in each i n s t a n c e because of o b s t r u c t i o n of the large bowel secondary to fecal i m p a c t i o n . I n a case rep o r t e d by C o m p t o n , 2 a loop colostomy was necessary to p r e p a r e the bowel adequately. O u r case r e p o r t is obviously one of technical f r u s t r a t i o n i n cleansing the bowel, as the stool in this p a t i e n t was thick a n d very
O1" T H E
259
COLON
difficult to remove. Even w i t h daily irrigations t h r o u g h b o t h ends of the colostomy a n d the rectum, the colon still c o n t a i n e d a large q u a n t i t y of stool at the time of subtotal colectomy. Summary A case of a p a t i e n t who h a d colonic obstruction caused by scleroderma is described. T i l e o b s t r u c t i o n i n i t i a l l y was treated by means of a colostomy, a n d later s u b t o t a l colectomy was carried out. T h i s is the fifth report of a p a t i e n t with s y m p t o m a t i c coIonic scleroderma successfully treated 1)y c:olonic resection. A review of colonic sclerotlerma is inclutled. References I. Cassada WA, Armstrong RH Jr, Neal MP Jr: Involvement of the gastrointestinal tract by progressive systemic sclerosis. South Med J 6t: 475, 1968 2. Compton R: Scteroderma with diverticuIosis and colonic obstructinn. Am J Surg 118:602. 1969 3. Hale CH, Schatzki R: The roentgenolo~cal a.ppearance of the gastrointestinal tract m scleroderma. Am J Roentgenol Radium Ther Nucl Med 51: 407, 1944 4. Heinz ER, Steinberg AJ, Sackner MA: Roentgenographic and pathologic aspects of intestinal scleroderma. Ann Intern Med 59: 822, 1963 5. Jayson MI, Salmon PR, Gough J, et al: Spontaneous bowel perforation in intestinal scleroderma: First report of a non4atal case. Postgrad Med J 48: 56, 1972 6. Lushbaugh CC; Rubin L, Rothman S: Scleroderma of the intestinal tract: First report of a fatal case. Gastroenterology 11: 382, 1948 7. Meszaros WT: The colon in systemic sclerosis (scleroderma). Am J Roentgenol Radium Ther Nucl Med 82: 100, 1959 8. Norton RA, Monroe LS: The surgical approach to gastrointestinal scleroderma: Report of a case with partial colonic resection. Am J Dig Dis 7: 766, 1962 9. Poirier TJ, Rankin GB: Gastrointestinal manifestations of progressive systemic scleroderma based on a review of 364 cases. Am J. Gastroenterol 58: 30, 1972. 10. Rake G: On the pathology and pathogenesis of scleroderma. Bull Johns Hopkins Hosp 48: 212, 1931
