Diagnostic Radiology
Retractile Mesenteritis: Initial Presentation as Colonic Obstruction 1 R. Gayle Williams, M.D.,2 and James A. Nelson, M.D.3 Retroperitoneal fibrosis has been associated with a variety of sclerosing diseases. Among these is the variant known as retractile mesenteritis, which involves predominantly the mesentery of the small intestine and associated vessels; involvement of the mesocolon and the colon is less frequent. Two patients with a dominating clinical picture of colonic obstruction are described. The radiographic presentations were similar and should serve to remind radiologists and clinicians of this entity. INDEX TERMS: (Colon, obstruction as unusual manifestation, 7[5].726) • (Gastrointestinal system, other vasculitis, 7[8].629). Intestines, diseases • Intestines, obstruction. Retroperitoneal space, fibrosis. Mesentery, inflammation Radiology 126:35-37, January 1978
Ogden's description of the variety of symptoms that have been popularized as retractile mesenteritis. The predominant symptoms consisted of alternating episodes of obstipation and diarrhea. Age ranged from the second to the eighth decade, with a slight predominance in men. Baum's particular case evolved primarily as a small bowel and ureteral obstruction following extensive mesenteric involvement by fibrosis. Very few cases of this entity have shown primary involvement of the colon, mesocolon, or colonic vasculature with subsequentobstructive signs and symptoms (13). This paper presents two cases of apparent idiopathic mesenteric fibrosis in which the most significant problem was directly referable to the colon.
FIBROSIS, with its multiple variants, was initially described by Albarran in 1909 (1). The best-known variant was popularized by Ormond, who described approximately 100 cases in 1948 (14). His typical patient was a middle-aged man who presented with a variety of symptoms, including anorexia, weight loss, nausea and vomiting, and a backache that was most often lumbar in location. Laboratory findings were usually nonspecific, including an elevated white cell count (usually with a left shift) and an increase in acute phase reactants, indicating the presence of inflammation. The etiology remains a mystery despite numerous attempts to categorize it. It seems to manifest itself as a period of active inflammation with lymphocyte and polymorphonuclear leukocyte infiltration, with subsequent development of a fibrotic process that constitutes the healing phase. The contractures resulting from the fibroblastic proliferation of fibrocollagen cause the chronic symptoms. Exploration reveals a fibrous mass in the region of Gerota's fascia. The mesentery is frequently thickened, with loss of the normal fat lobulatlons. The latter areas appear as fat necrosis, suggesting Weber-Christian disease (3). A gamut of other disease states have been associated with this process, including inflammatory conditions of the bowel (diverticulitis, Crohn's disease, chronic ulcerative colitis, and appendicitis), and various neoplastic processes including Hodgkin's disease (12), prostatic carcinoma (14), and other undifferentiated sarcomas (15). It is often either multifocal or related to a similar fibrotic process elsewhere in the body, e. g., the peri cardiac area (9), bladder neck (10), thyroid (5), bile ducts (5), and lung or mediastinum (4). Autoimmune and collagen vascular diseases (7, 11) have been implicated as well. Dr. Stanley Baum (6) discussed this topic and noted
R
ETROPERITONEAL
CASE REPORTS CASE I: This 54-year-old white woman was initially admitted in 9/75 with symptoms and excretory urographic findings compatible with left ureteral obstruction. Subsequent surgery revealed retroperitoneal fibrosis, and ureterolysis was performed. There was no evidence of any underlying tumor or inflammatory process. Despite a history of vascular headaches she denied the use of methysergide. She was subsequently readmitted in 12/75 following several bouts of severe colicky abdominal pain and nonbloody vomiting. Physical examination revealed a firm mass measuring 30 cm X 20 cm. There was no evidence of hepatosplenomegaly or adenopathy. Laboratory data were unremarkable. A preoperative plain radiograph of the abdomen revealed a pattern consistent with mechanical obstruction of the small bowel. A barium enema revealed obstruction of the descending colon (splenic flexure). There was apparent mucosal thumbprinting associated with a mass lesion (Fig. 1). Operatively, the patient had multiple small and large bowel obstructions with progressive mesenteric and colonic fibrosis and extensive fibrotic implants over the small bowel. A subtotal colectomy with an ileostomy and mucus fistula formation was performed. Pathologically, the specimen revealed fibrovascular thickening and infiltration of scattered polymorphonuclear neutrophilic leukocytes. There was no evidence of necrosis, chronic infection, or tumor.
1 From the Department of Radiology and Division of Gastroenteroloqy, Department of Medicine, College of Medicine, University of Utah, Salt Lake City, Utah. Accepted for publication in August 1977. 2 Resident in Diagnostic Radiology, University of Utah Medical Center. 3 James Picker Foundation Scholar in Radiological Research. wjw Supported in part by NIH Research Career Development Award (1 K04 GM00164 01) from NIGMS.
35
36
R. GAYLE WILLIAMS AND JAMES A. NELSON
January 1978
The patient suffered postoperative complications including pulmonary hemorrhage and subsequently died of respiratory insufficiency.
~ ~i'/?
•• Fig. 1.
Barium enema study demonstrating diffuse "thumbprinting" of the transverse colon to the splenic flexure with almost complete luminal obliteration.
Fig. 2. Fig. 3.
CASE II: This 47-year-old white man presented with hypogastric colicky pain of unknown duration. He also complained of some nonspecific nausea and vomiting and a 22-33 kg weight loss over the previous 4 to 6 weeks. Otherwise he was devoid of any system-specific symptoms. Physical examination revealed a moderately obese white man in no apparent distress. He had a distinctly nontender abdomen despite the absence of bowel sounds. Palpation of the abdomen revealed a firm, iII-defined left-upper-quadrant mass. Laboratory data included a hematocrit of 37.4, white cell count of 14,000 with a left shift, an erythrocyte sedimentation rate of 46, and a normal urinalysis and SMA 12. An excretory urogram revealed questionable deviation of the distal left ureter with minimal proximal hydronephrosis and cortical thinning. The barium enema demonstrated partial obstruction at the splenic flexure with evidence of luminal narrowing and mucosal thumbprintng (Fig. 2). Although retroperitoneal fibrosis was considered in the preoperative diagnosis, the primary consideration was ischemic colitis. The patient subsequently underwent an exploratory laparotomy and a side-to-side bypass transverse-to-distal sigmoid colostomy because of a near complete obstruction of the distal colon by a huge mesenteric fibrotic mass. Biopsy of the large mass was interpreted as idiopathic retroperitoneal fibrosis. The mass extended from the midline to the left paracolic gutter, and from the posterior portion of the pancreas inferiorly
Similar findings are seen in the rnid-descending colon. Follow-up barium enema study showing no evidence of the previous process.
37
RETRACTILE MESENTERITIS
Vol. 126
to the lower sigmoid. Encasement of the colon and many mesenteric vessels was noted. The patient improved considerably postoperatively while on steroid therapy. He gained weight and had an increased sense of well being. A follow-up barium enema showed no evidence of recurrence or residua (Fig. 3).
DISCUSSION
Retroperitoneal fibrosis has been implicated as part of a spectrum of sclerosing diseases occurring in a diffuse distribution. The variant involving mesenteric fibrosis has been related to systemic involvement by Weber-Christian disease, to autoimmune processes stimulating fibrosis, and occasionally to occult underlying lymphomas and sarcomas. Despite the frequency of associated pathology, the majority of patients are at least tentatively diagnosed as having idiopathic retroperitoneal or mesenteric fibrosis. Involvement of the mesentery itself usually leads to small bowel involvement with infarction secondary to encasement and obstruction of primary vessels. A distinct mass may be present within the mesentery in addition to sharply-angulated narrowed segments of bowel, which otherwise may appear intrinsically normal (8). Occasionally the primary process itself will cause luminal obliteration and subsequent obstruction. To our knowledge there are few reports of patients presenting with predominant colonic involvement. Both of our patients presented with colonic symptoms and a history compatible with idiopathic retroperitoneal fibrosis. Presenting histories included signs and symptoms of bowel obstruction and a firm abdominal mass. Both patients had been relatively healthy prior to the onset of illness. Neither had a known family history of this or other fibrosing processes, or of exposure to allergens. There was no evidence of any coexistent neoplastic entity. Neoplastic involvement was not found in the autopsied patient and seems unlikely in the patient who has survived and shown clinical improvement for five years. These cases further illustrate the protean manifestations of idiopathic fibrosis. Colonic obstruction in these patients, whether by luminal obliteration or vascular compromise, illustrates another presentation of the spectrum of idiopathic fibrosis. The exact nature of the fibroblastic stimulus remains unknown. Empirical steroid therapy is the only successful treatment (2). The radiographic manifestations vary. Angiography may show vascular obstruction; barium studies demonstrate luminal narrowing or obstruction, often in association with ipsilateral hydronephrosis. Early changes include mild
Diagnostic Radiology
mucosal irregularity with apparent mucosal edema ("thumbprinting"). The thumbprinting in both cases was pathologically proved to be secondary to extensive fibrotic implants on the serosal portion of the bowel wall and adjoining mesentery. Associated venous congestion can, of course, contribute to the radiographic appearance. The clinical history and findings of an obstructive lesion in the colon (usually mucosal thumbprinting associated with an ill-defined mass), where cancer is not necessarily suspected, should lead to a consideration of this entity for the radiographic diagnosis. Department of Radiology University of Utah Medical Center 50 N. Medical Drive Salt Lake City, Utah 84132 ACKNOWLEDGMENTS: The authors wish to thank Dr. Ralph J. Meyer for the radiographs on CASE I and Ms. Joan Moody for her secretarial assistance.
REFERENCES 1. Albarran J: Medicine operatoire des voies urinaires. Paris, Masson et Cie, 1909, p 991 2. Apalakis A, McCollum JK: Corticosteroids in retroperitoneal fibrosis. Br Med J 4:791,30 Dec 1972 3. Arnold HA, Bainborough AR: Weber-Christian disease with visceral involvement. Can Med Assoc J 89: 1138-1142, 30 Nov 1963 4. Barrett NR: Idiopathic mediastinal fibrosis. Br J Surg 46: 207-218, Nov 1958 5. Bartholomew LG, Cain JC, Woolner LB, et al: Sclerosing cholangitis: its possible association with Riedel's struma and fibrous retroperitonitis. N Engl J Med 269:8-12,4 Jul 1963 6. Baum S: Case records of the MassachusettsGeneral Hospital. Case 72-1972. N Engl J Med 287:34-40,6 Jul 1972 7. Blau EB: Relapsing polychondritis and retroperitoneal fibrosis in an 8-year-old boy. Am J Dis Child 130: 1149-1152, Oct 1976 8. Clemett R, Tracht DG: The roentgen diagnosis of retractile mesenteritis. Am J Roentgenol 107:787-790, Dec. 1969 9. Dineen J, Asch T, Pearce JM: Retroperitoneal fibrosis. Radiology 75:380-390, Sep 1960 10. Hewett AL, Headstream JW: Pericystitis plastlca. J Urol 83: 103-1 07, Feb 1960 11. Hoffman WW, Trippel OH: Retroperitoneal fibrosis: etiologic considerations. J Urol 86:222-231, Aug 1961 12. Kendall AR, Lakey WH: Sclerosing Hodgkin's disease vs. idiopathic retroperitoneal fibrosis. J Urol 86:217-221, Aug 1961 13. McCarthy JG, Porter MR, Veenema R: Retroperitoneal fibrosis and large bowel obstruction: case report and review of the literature. Ann Surg 176:199-204, Aug 1972 14. Ormond JK: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory process. J UroI59:1072-1079, Jun 1948 15. Rutter AG: Idiopathic retroperitoneal fibrosis simulating advanced pelvic carcinoma. Br J Urol 37:302-306, Jun 1965
Retractile Mesenteritis: Initial Presentation as Colonic Obstruction 1 R. Gayle Williams, M.D.,2 and James A. Nelson, M.D.3 Retroperitoneal fibrosis has been associated with a variety of sclerosing diseases. Among these is the variant known as retractile mesenteritis, which involves predominantly the mesentery of the small intestine and associated vessels; involvement of the mesocolon and the colon is less frequent. Two patients with a dominating clinical picture of colonic obstruction are described. The radiographic presentations were similar and should serve to remind radiologists and clinicians of this entity. INDEX TERMS: (Colon, obstruction as unusual manifestation, 7[5].726) • (Gastrointestinal system, other vasculitis, 7[8].629). Intestines, diseases • Intestines, obstruction. Retroperitoneal space, fibrosis. Mesentery, inflammation Radiology 126:35-37, January 1978
Ogden's description of the variety of symptoms that have been popularized as retractile mesenteritis. The predominant symptoms consisted of alternating episodes of obstipation and diarrhea. Age ranged from the second to the eighth decade, with a slight predominance in men. Baum's particular case evolved primarily as a small bowel and ureteral obstruction following extensive mesenteric involvement by fibrosis. Very few cases of this entity have shown primary involvement of the colon, mesocolon, or colonic vasculature with subsequentobstructive signs and symptoms (13). This paper presents two cases of apparent idiopathic mesenteric fibrosis in which the most significant problem was directly referable to the colon.
FIBROSIS, with its multiple variants, was initially described by Albarran in 1909 (1). The best-known variant was popularized by Ormond, who described approximately 100 cases in 1948 (14). His typical patient was a middle-aged man who presented with a variety of symptoms, including anorexia, weight loss, nausea and vomiting, and a backache that was most often lumbar in location. Laboratory findings were usually nonspecific, including an elevated white cell count (usually with a left shift) and an increase in acute phase reactants, indicating the presence of inflammation. The etiology remains a mystery despite numerous attempts to categorize it. It seems to manifest itself as a period of active inflammation with lymphocyte and polymorphonuclear leukocyte infiltration, with subsequent development of a fibrotic process that constitutes the healing phase. The contractures resulting from the fibroblastic proliferation of fibrocollagen cause the chronic symptoms. Exploration reveals a fibrous mass in the region of Gerota's fascia. The mesentery is frequently thickened, with loss of the normal fat lobulatlons. The latter areas appear as fat necrosis, suggesting Weber-Christian disease (3). A gamut of other disease states have been associated with this process, including inflammatory conditions of the bowel (diverticulitis, Crohn's disease, chronic ulcerative colitis, and appendicitis), and various neoplastic processes including Hodgkin's disease (12), prostatic carcinoma (14), and other undifferentiated sarcomas (15). It is often either multifocal or related to a similar fibrotic process elsewhere in the body, e. g., the peri cardiac area (9), bladder neck (10), thyroid (5), bile ducts (5), and lung or mediastinum (4). Autoimmune and collagen vascular diseases (7, 11) have been implicated as well. Dr. Stanley Baum (6) discussed this topic and noted
R
ETROPERITONEAL
CASE REPORTS CASE I: This 54-year-old white woman was initially admitted in 9/75 with symptoms and excretory urographic findings compatible with left ureteral obstruction. Subsequent surgery revealed retroperitoneal fibrosis, and ureterolysis was performed. There was no evidence of any underlying tumor or inflammatory process. Despite a history of vascular headaches she denied the use of methysergide. She was subsequently readmitted in 12/75 following several bouts of severe colicky abdominal pain and nonbloody vomiting. Physical examination revealed a firm mass measuring 30 cm X 20 cm. There was no evidence of hepatosplenomegaly or adenopathy. Laboratory data were unremarkable. A preoperative plain radiograph of the abdomen revealed a pattern consistent with mechanical obstruction of the small bowel. A barium enema revealed obstruction of the descending colon (splenic flexure). There was apparent mucosal thumbprinting associated with a mass lesion (Fig. 1). Operatively, the patient had multiple small and large bowel obstructions with progressive mesenteric and colonic fibrosis and extensive fibrotic implants over the small bowel. A subtotal colectomy with an ileostomy and mucus fistula formation was performed. Pathologically, the specimen revealed fibrovascular thickening and infiltration of scattered polymorphonuclear neutrophilic leukocytes. There was no evidence of necrosis, chronic infection, or tumor.
1 From the Department of Radiology and Division of Gastroenteroloqy, Department of Medicine, College of Medicine, University of Utah, Salt Lake City, Utah. Accepted for publication in August 1977. 2 Resident in Diagnostic Radiology, University of Utah Medical Center. 3 James Picker Foundation Scholar in Radiological Research. wjw Supported in part by NIH Research Career Development Award (1 K04 GM00164 01) from NIGMS.
35
36
R. GAYLE WILLIAMS AND JAMES A. NELSON
January 1978
The patient suffered postoperative complications including pulmonary hemorrhage and subsequently died of respiratory insufficiency.
~ ~i'/?
•• Fig. 1.
Barium enema study demonstrating diffuse "thumbprinting" of the transverse colon to the splenic flexure with almost complete luminal obliteration.
Fig. 2. Fig. 3.
CASE II: This 47-year-old white man presented with hypogastric colicky pain of unknown duration. He also complained of some nonspecific nausea and vomiting and a 22-33 kg weight loss over the previous 4 to 6 weeks. Otherwise he was devoid of any system-specific symptoms. Physical examination revealed a moderately obese white man in no apparent distress. He had a distinctly nontender abdomen despite the absence of bowel sounds. Palpation of the abdomen revealed a firm, iII-defined left-upper-quadrant mass. Laboratory data included a hematocrit of 37.4, white cell count of 14,000 with a left shift, an erythrocyte sedimentation rate of 46, and a normal urinalysis and SMA 12. An excretory urogram revealed questionable deviation of the distal left ureter with minimal proximal hydronephrosis and cortical thinning. The barium enema demonstrated partial obstruction at the splenic flexure with evidence of luminal narrowing and mucosal thumbprintng (Fig. 2). Although retroperitoneal fibrosis was considered in the preoperative diagnosis, the primary consideration was ischemic colitis. The patient subsequently underwent an exploratory laparotomy and a side-to-side bypass transverse-to-distal sigmoid colostomy because of a near complete obstruction of the distal colon by a huge mesenteric fibrotic mass. Biopsy of the large mass was interpreted as idiopathic retroperitoneal fibrosis. The mass extended from the midline to the left paracolic gutter, and from the posterior portion of the pancreas inferiorly
Similar findings are seen in the rnid-descending colon. Follow-up barium enema study showing no evidence of the previous process.
37
RETRACTILE MESENTERITIS
Vol. 126
to the lower sigmoid. Encasement of the colon and many mesenteric vessels was noted. The patient improved considerably postoperatively while on steroid therapy. He gained weight and had an increased sense of well being. A follow-up barium enema showed no evidence of recurrence or residua (Fig. 3).
DISCUSSION
Retroperitoneal fibrosis has been implicated as part of a spectrum of sclerosing diseases occurring in a diffuse distribution. The variant involving mesenteric fibrosis has been related to systemic involvement by Weber-Christian disease, to autoimmune processes stimulating fibrosis, and occasionally to occult underlying lymphomas and sarcomas. Despite the frequency of associated pathology, the majority of patients are at least tentatively diagnosed as having idiopathic retroperitoneal or mesenteric fibrosis. Involvement of the mesentery itself usually leads to small bowel involvement with infarction secondary to encasement and obstruction of primary vessels. A distinct mass may be present within the mesentery in addition to sharply-angulated narrowed segments of bowel, which otherwise may appear intrinsically normal (8). Occasionally the primary process itself will cause luminal obliteration and subsequent obstruction. To our knowledge there are few reports of patients presenting with predominant colonic involvement. Both of our patients presented with colonic symptoms and a history compatible with idiopathic retroperitoneal fibrosis. Presenting histories included signs and symptoms of bowel obstruction and a firm abdominal mass. Both patients had been relatively healthy prior to the onset of illness. Neither had a known family history of this or other fibrosing processes, or of exposure to allergens. There was no evidence of any coexistent neoplastic entity. Neoplastic involvement was not found in the autopsied patient and seems unlikely in the patient who has survived and shown clinical improvement for five years. These cases further illustrate the protean manifestations of idiopathic fibrosis. Colonic obstruction in these patients, whether by luminal obliteration or vascular compromise, illustrates another presentation of the spectrum of idiopathic fibrosis. The exact nature of the fibroblastic stimulus remains unknown. Empirical steroid therapy is the only successful treatment (2). The radiographic manifestations vary. Angiography may show vascular obstruction; barium studies demonstrate luminal narrowing or obstruction, often in association with ipsilateral hydronephrosis. Early changes include mild
Diagnostic Radiology
mucosal irregularity with apparent mucosal edema ("thumbprinting"). The thumbprinting in both cases was pathologically proved to be secondary to extensive fibrotic implants on the serosal portion of the bowel wall and adjoining mesentery. Associated venous congestion can, of course, contribute to the radiographic appearance. The clinical history and findings of an obstructive lesion in the colon (usually mucosal thumbprinting associated with an ill-defined mass), where cancer is not necessarily suspected, should lead to a consideration of this entity for the radiographic diagnosis. Department of Radiology University of Utah Medical Center 50 N. Medical Drive Salt Lake City, Utah 84132 ACKNOWLEDGMENTS: The authors wish to thank Dr. Ralph J. Meyer for the radiographs on CASE I and Ms. Joan Moody for her secretarial assistance.
REFERENCES 1. Albarran J: Medicine operatoire des voies urinaires. Paris, Masson et Cie, 1909, p 991 2. Apalakis A, McCollum JK: Corticosteroids in retroperitoneal fibrosis. Br Med J 4:791,30 Dec 1972 3. Arnold HA, Bainborough AR: Weber-Christian disease with visceral involvement. Can Med Assoc J 89: 1138-1142, 30 Nov 1963 4. Barrett NR: Idiopathic mediastinal fibrosis. Br J Surg 46: 207-218, Nov 1958 5. Bartholomew LG, Cain JC, Woolner LB, et al: Sclerosing cholangitis: its possible association with Riedel's struma and fibrous retroperitonitis. N Engl J Med 269:8-12,4 Jul 1963 6. Baum S: Case records of the MassachusettsGeneral Hospital. Case 72-1972. N Engl J Med 287:34-40,6 Jul 1972 7. Blau EB: Relapsing polychondritis and retroperitoneal fibrosis in an 8-year-old boy. Am J Dis Child 130: 1149-1152, Oct 1976 8. Clemett R, Tracht DG: The roentgen diagnosis of retractile mesenteritis. Am J Roentgenol 107:787-790, Dec. 1969 9. Dineen J, Asch T, Pearce JM: Retroperitoneal fibrosis. Radiology 75:380-390, Sep 1960 10. Hewett AL, Headstream JW: Pericystitis plastlca. J Urol 83: 103-1 07, Feb 1960 11. Hoffman WW, Trippel OH: Retroperitoneal fibrosis: etiologic considerations. J Urol 86:222-231, Aug 1961 12. Kendall AR, Lakey WH: Sclerosing Hodgkin's disease vs. idiopathic retroperitoneal fibrosis. J Urol 86:217-221, Aug 1961 13. McCarthy JG, Porter MR, Veenema R: Retroperitoneal fibrosis and large bowel obstruction: case report and review of the literature. Ann Surg 176:199-204, Aug 1972 14. Ormond JK: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory process. J UroI59:1072-1079, Jun 1948 15. Rutter AG: Idiopathic retroperitoneal fibrosis simulating advanced pelvic carcinoma. Br J Urol 37:302-306, Jun 1965
