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Successful treatment of a patient with retractile mesenteritis with prednisone and azathioprine
GASTROENTEROLOGY
79:352-356.1966
Successful Treatment of a Patient with Retractile Mesenteritis with Prednisone and Azathioprine G. N. TYTGAT, K. ROOZENDAAL, and M. R. ESSEVELD Department Amsterdam, Amsterdam,
W. WINTER,
of Internal Medicine and Radiology, Onze Lieve Vrouwe Gasthuis, and Division of Gastroenterology, University Hospital, Wilhelmina The Netherlands
A patient
with retractile mesenteritis is discussed, who presented with a huge 15 x 25-cm inflammatory mass involving the root of the mesentery. Gradually the patient developed duodenal obstruction for which it was necessary to perform a gastroileostomy. Because of a relentless downhill course with fever, weight loss, general malaise, and fatigue, it was finally decided to treat the patient with prednisone and azathioprine. Upon institution of this treatment there was a steady, progressive clinical amelioration with disappearance of fever, improvement in laboratory findings, and gradual regression of the lesion until it became a well-delineated hard mass that was thought to be the end stage of fibrosis. Early trial with such a treatment, once the diagnosis is firmly established, should be considered. Retractile mesenteritis is a rather rare disease of unknown cause, characterized by nonspecific inflammation of the adipose tissue of the mesentery, leading to fibrosis and retraction. The latter was first described by Jura in 1924.’ Aach et al.’ suggested that the term “mesenteric panniculitis” should be used for cases with acute or subacute inflammation of the mesentery and “retractile mesenteritis” for those in whom the chronic protracted inflammatory process was complicated by fibrosis and retraction; the latter condition was also called sclerosing lipogranulomatosis. A more comprehensive review of this puzzling disease entity has been published by Kipfer et al.” and by Durst et al.’ The purpose of this case report is to stress the complexity of the clinical presentation, the problems Received November 26, 1979. Accepted March 14, 1960. Address requests for reprints to: Professor Dr. G.N. Tytgat, Wilhelmina Gasthuis, le Helmersstraat 104, 1054 EG Amsterdam, The Netherlands. 0 1980 by the American Gastroenterological Association 0016-5085/80/080352-05$0X5
Gasthuis,
related to the well-known complication of intestinal obstruction, and especially, to stress the quite striking regression of the mesenteric tumorlike mass together with unequivocal amelioration of the clinical condition of the patient upon treatment with prednisone and azathioprine.
Case Report C.B., a Caucasian female born in 1928, had always enjoyed good health until she first presented in February, 1974,with 3 mo of vague chronic pain and discomfort in the upper abdomen. There was no anorexia, and her body weight had been stable. She denied episodes of fever. She had never been operated upon in the past. Physical examination was unremarkable except for a large, deeply seated, rather hard, and poorly delineated mass in the left upper abdomen of an otherwise healthy looking woman. Extensive laboratory examination was normal except for an ESR of 53 mm/hr. Upper gastrointestinal series, barium enema, intravenous pyelography, and angiography of the celiac trunk and superior mesenteric artery did not show any abnormality. In particular there was no evidence of a pancreatic malignancy nor of retroperitoneal displacement and narrowing of the ureters. During subsequent exploratory laparotomy, an indurated 15 x Is-cm mass was found behind the stomach and the transverse colon, involving the root of the mesentery. The overall macroscopic aspect of the lesion was highly suspicious for malignancy. Resection of the mass was considered impossible because of the danger of compromising the blood supply to the small bowel. A large surgical biopsy was obtained that revealed dense collagenous tissue with patchy foci of chronic inflammation consisting of lymphocytes (Figure 1). Postoperatively the clinical situation of the patient gradually deteriorated. She started complaining of anorexia, general malaise, progressive weight loss up to 10 kg, intermittent bouts of nausea and vomiting, and episodes of fever. At regular follow-up, the mass was felt to become larger.
353
RETRACTILE MESENTERITIS
August 1980
The prednisone medication was gradually tapered over a period of some 8 mo. At present, the patient is feeling well and performing all her normal duties. Her body weight has increased to 65 kg. There is no evidence of diarrhea. The urinary d-xylose excretion is 5.1 g/5 hr after a 25-g load. Vitamin B,, is normal whereas carotene and folic acid levels fluctuate around the lower limit of normalcy. Extensive malabsorption work-up was turned down by the patient. Upon physical examination, a shrunken, hard, well-delineated mass, measuring approximately 7 x 8 cm, is still palpable to the left of the umbilicus. Now, well over 1 yr after stopping the prednisone medication, and more than one-half year after stopping the azathioprine treatment, there is no evidence of reactivation of the process, the patient remaining symptom-free in a clinically stable and satisfactory condition.
Discussion Figure 1. Superficial part of a large surgical biopsy showing dense collagenous tissue (C) and a focal mainly lymphocytic inflammatory infiltrate around adipocytes (arrow).
About 1 yr after her first laparotomy, incessant vomiting because of duodenal obstruction (Figure 2) occurred, leading to dehydration, metabolic alkalosis, and renal insufficiency. For relief of her progressive obstructive symptoms, a second laparotomy was performed, demonstrating that the mass had obviously progressed, involving and fixing all of the jejunum to the dense fibrotic process and causing total obstruction at the duodenojejunal angle. Only the distal 1.4 m of ileum could be mobilized and anastomosed side-to-side to the greater curvature of the stomach followed by a Braun-type anastomosis between the distal jejunum and ileum. Biopsies of the mass showed abnormalities similar to those previously seen, again without evidence of malignancy. Postoperatively the patient continued to go downhill. Stomach emptying via the gastroileostomy was rather slow. In addition the patient complained of chronic abdominal pain, episodes of vomiting, general malaise, and fatigue, and her temperature ranged between 38 and 39.5’C. She kept losing weight and reached a body wt of 52 kg. Because of this deterioration of her condition, she was hospitalized again in April, 1976. Upon admission, a chronically ill, feverish, slightly emaciated woman was found. The abdominal mass had further progressed (Figure 3, Table 1). The ESR had risen to 105 mm/hr, and her hemoglobin level dropped to 9.7 g/dl. In view of the dismal outlook and the relentlessly downhill course, it was finally decided to treat the patient with prednisone and azathioprine in a desperate attempt to slow down further progression of the lesion (see Table 1 for dosages). Upon institution of this treatment, the fever disappeared within a few days. Together with gradual improvement of her general condition and well being, there was progressive fall of her ESR to normal. Hemoglobinlevel and body-weight responses are summarized in Table 1. As judged by palpation, the mass gradually decreased in volume. Shrinkage of the mass was confirmed by ultrasonographic measurement (Figure 4).
Retractile mesenteritis (or mesenteric panniculitis) is an intriguing disease of unknown cause despite extensive speculation. In his literature survey of 71 patients, Jura’ considered, as a cause, preceding trauma of pathogenetic importance because the majority of his patients had experienced abdominal surgery, abdominal tuberculosis, or mesenteric thrombosis, conditions all considered to be potentially traumatic to the mesentery with ultimate evolution toward fibrosis and retraction. In support of this hypothesis, Tedeschi and Botta” injected bac-
Figure
2. Barium meal showing nearly third duodenum (arrow) with proximal duodenum.
total obstruction marked dilatation
of the of the
354
TYTGAT ET AL.
Figure 3. Transverse echogram at the cus (LJ) in August, 1977. The in the left abdomen between measures 8 x 9 cm. To the bowel gas (B) are seen.
GASTROENTEROLOGY
Vol. 79, No. 2
level of the umbilitumor is visualized the two arrows and right, reflections of
teria or bacterial extracts in the mesentery of animals and produced degeneration of mesenteric fat cells, inflammation, and subsequent fibrosis. In many patients, however, as in this case report, preceding mesenteric trauma was absent. Thus surgery is not a prerequisite for mesenteric fibrosis, and other hypotheses were proposed. Steinberg” presented 2 patients with systemic nodular panniculitis with widespread involvement of the adipose tissue, and he considered this illness to be a systemic form of Weber-Christian’s subcutaneous nodular panniculitis. Crane et al.’ considered the mesenteric lesion to be a localized form of lipodystrophy. Similarly Soergel and Hensley” described a patient who initially had mesenteric lipogranulomata and lipodystrophy, which, upon further evolution, led to complete mesenteric fibrosis and retraction. Finally, other authors’,” relate retractile mesenteritis to retroperitoneal fibrosis, stressing the similarites be-
Figure 4. Transverse echogram at the level of the umbilicus (U) in January 1978. The tumor between arrows has regressed to 5 x 6 cm. Some internal reflections within the tumor are now visible.
tween the two disease entities. In some patients, both diseases even appeared to coexist.‘.” Presumably retractile mesenteritis has to be considered as the nonspecific end stage of a variety of injuries, particularly of the mesenteric adipose tissue, leading to fibrosis and retraction. This mesenteric scarring can lead to secondary occlusion of lymph vessels causing lymph stagnation and occasionally to obstruction of mesenteric veins with fatal ischemic bowel damage.’ The diagnosis of retractile mesenteritis in our patient was based upon the macroscopic and histologic findings and upon the clinical course of the disease. The common gross pathologic appearance of retractile mesenteritis consists of a markedly thickened mesentery of rubbery consistency, or a large solitary mass in the root of the mesentery or of several masses stuck together, showing puckering of the mesenteric surface.3.4 Usually the peritoneum firmly
August
1980
RETRACTILE
MESENTERITIS
355
adheres to the inflammatory mass. Occasionally there is conspicuous mesenteric shortening and fibrosis. In some patients, a collection of chylous fluid, apparently subsequent to liquefaction of adipocytes, is present within the mesenteric mass.8.“,‘2 The corresponding histologic features include: foci of degeneration and necrosis of adipocytes, accumulation of lipophages, chronic predominantly lymphocytic inflammation mixed with histiocytes, and occasionally foreign body giant cells, and, in addition, various degrees of fibrosis with massive accumulation of dense collagenous tissue. In the majority of the patients the clinical course is characterized by vague abdominal fullness and heaviness, pain and discomfort, bouts of vomiting, fever, development of a deep-seated and usually poorly defined abdominal mass, and occasionally signs and symptoms of partial intestinal obstruction.2-4 The prognosis of retractile mesenteritis is variable. In some patients, the inflammatory process resolves, and there is more or less complete recovery. In others, the disease takes a more protracted course with fibrosis and retraction of the mesentery leading to partial intestinal obstruction. The course in our patient was dominated by the progressive enlargement of the tumorlike mass, which caused intestinal obstruction. Because of the unrelenting, gradual deterioration of her general clinical condition, a fatal outcome was thought to be imminent and unavoidable, as has previously been noted by other authors.R,“’ In view of this dismal outlook, treatment with prednisone and azathioprine was initiated in a desperate attempt to arrest the basic chronic inflammatory process, presumably related to degeneration of the mesenteric adipose tissue, in analogy with therapeutic attempts in Weber-Christian disease, although admittedly the cause of this illness remains obscure. Azathioprine was added to the prednisone to potentiate its antiinflammatory activity and because of its well-known steriod-sparing effect, in analogy with the treatment of many other chronic inflammatory conditions. Upon institution of this treatment, there was a steady progressive improvement of general well being, reversion of the biochemical abnormalities towards normal values, disappearance of fever, and regression of the mass to a smaller well-delineated, hard lesion, thought to be the end-stage of scarring. Other authors also noted a beneficial effect of corticosteroid therapy.“.“~‘” Other treatments (antibiotics, irradiation, emetine, and azathioprine) have been tried without obvious success4 Surgical treatment should be limited to exploration and biopsy with intestinal bypass procedures whenever indicated. Resection of the tumor mass is often impossible, or does not seem to be justified,
356
TYTGAT ET AL.
when considered feasible, especially if surgery involves resection of large segments of small bowel or possibly compromises the mesenteric arterial circulation.‘,” In view of the unequivocal improvement in our patient upon treatment with prednisone and azathioprine, we would like to recommend an early trial of such treatment once the diagnosis is established in an attempt to prevent progression of the lesion and perhaps to avoid surgical bypass procedures. Why the disease apparently remains in remission in our pateint after tapering and stopping the corticosteroid and azathioprine therapy is difficult to understand and will presumably remain unknown until more information on the cause becomes available.
References 1. Jura V: Sulfa mesenterite
retrattite e sclerosante. Pohclinico [Prat] 31:575, 1924 2. Aach RD, Kahn LI, French RS: Obstruction of the small intestine due to retractile mesenteritis. Gastroenterology 54:594, 1966
GASTROENTEROLOGY
Vol. 79, No. 2
3. Kipfer RE, Moertel ChG, Dahlin DC: Mesenteric Lipodystrophy. Ann Intern Med 69562, 1974 4. Durst AL, Freund H, Rosenmann E, et al: Mesenteric panniculitis: review of the literature and presentation of cases. Surgery 61:203, 1977 5. Tedeschi GC, Botta GC: Retractile mesenteritis. N Engl J Med 266:1035, 1962 6. Steinberg B: Systemic nodular panniculitis. Am J Path01 29:1059, 1953 7. Crane JT, Aguilar MJ, Grimes OF: Isolated lipodystrophy, a form of mesenteric tumor. Am J Surg 90:169, 1955 6. Soergel KH, Hensley CT: Fatal mesenteric panniculitis. Gastroenterology 51:529, 1966 9. Handelsmann JC, Shelley WM: Mesenteric panniculitis. Arch Surg 91:642, 1965 10. Leger F, Roberti A, Deloche de Noyelle A, et al: Pannicuhte mesenterique, variete de fibrosclerose retro-peritoneale. Presse Med 75:75, 1967 11. Ogden WW, Bradburn DM, Rives GD: Mesenteric pannicuhtis. Ann Surg 161:664, 1965 12. Pallette EM, Pallette EC, Harrington RW: Sclerosing lipogranulomatosis: its several abdominal syndromes. Arch Surg 94:603, 1967 13. Detrie P: Retractile mesenteritis. J Chir (Paris) 88:341. 1964 14. Godquin B, Saout R, Loupias P, et al: Pseudo-tumeur inflammatoire et necrotique du mesentere. J Chir (Paris) 104:167, 1972
79:352-356.1966
Successful Treatment of a Patient with Retractile Mesenteritis with Prednisone and Azathioprine G. N. TYTGAT, K. ROOZENDAAL, and M. R. ESSEVELD Department Amsterdam, Amsterdam,
W. WINTER,
of Internal Medicine and Radiology, Onze Lieve Vrouwe Gasthuis, and Division of Gastroenterology, University Hospital, Wilhelmina The Netherlands
A patient
with retractile mesenteritis is discussed, who presented with a huge 15 x 25-cm inflammatory mass involving the root of the mesentery. Gradually the patient developed duodenal obstruction for which it was necessary to perform a gastroileostomy. Because of a relentless downhill course with fever, weight loss, general malaise, and fatigue, it was finally decided to treat the patient with prednisone and azathioprine. Upon institution of this treatment there was a steady, progressive clinical amelioration with disappearance of fever, improvement in laboratory findings, and gradual regression of the lesion until it became a well-delineated hard mass that was thought to be the end stage of fibrosis. Early trial with such a treatment, once the diagnosis is firmly established, should be considered. Retractile mesenteritis is a rather rare disease of unknown cause, characterized by nonspecific inflammation of the adipose tissue of the mesentery, leading to fibrosis and retraction. The latter was first described by Jura in 1924.’ Aach et al.’ suggested that the term “mesenteric panniculitis” should be used for cases with acute or subacute inflammation of the mesentery and “retractile mesenteritis” for those in whom the chronic protracted inflammatory process was complicated by fibrosis and retraction; the latter condition was also called sclerosing lipogranulomatosis. A more comprehensive review of this puzzling disease entity has been published by Kipfer et al.” and by Durst et al.’ The purpose of this case report is to stress the complexity of the clinical presentation, the problems Received November 26, 1979. Accepted March 14, 1960. Address requests for reprints to: Professor Dr. G.N. Tytgat, Wilhelmina Gasthuis, le Helmersstraat 104, 1054 EG Amsterdam, The Netherlands. 0 1980 by the American Gastroenterological Association 0016-5085/80/080352-05$0X5
Gasthuis,
related to the well-known complication of intestinal obstruction, and especially, to stress the quite striking regression of the mesenteric tumorlike mass together with unequivocal amelioration of the clinical condition of the patient upon treatment with prednisone and azathioprine.
Case Report C.B., a Caucasian female born in 1928, had always enjoyed good health until she first presented in February, 1974,with 3 mo of vague chronic pain and discomfort in the upper abdomen. There was no anorexia, and her body weight had been stable. She denied episodes of fever. She had never been operated upon in the past. Physical examination was unremarkable except for a large, deeply seated, rather hard, and poorly delineated mass in the left upper abdomen of an otherwise healthy looking woman. Extensive laboratory examination was normal except for an ESR of 53 mm/hr. Upper gastrointestinal series, barium enema, intravenous pyelography, and angiography of the celiac trunk and superior mesenteric artery did not show any abnormality. In particular there was no evidence of a pancreatic malignancy nor of retroperitoneal displacement and narrowing of the ureters. During subsequent exploratory laparotomy, an indurated 15 x Is-cm mass was found behind the stomach and the transverse colon, involving the root of the mesentery. The overall macroscopic aspect of the lesion was highly suspicious for malignancy. Resection of the mass was considered impossible because of the danger of compromising the blood supply to the small bowel. A large surgical biopsy was obtained that revealed dense collagenous tissue with patchy foci of chronic inflammation consisting of lymphocytes (Figure 1). Postoperatively the clinical situation of the patient gradually deteriorated. She started complaining of anorexia, general malaise, progressive weight loss up to 10 kg, intermittent bouts of nausea and vomiting, and episodes of fever. At regular follow-up, the mass was felt to become larger.
353
RETRACTILE MESENTERITIS
August 1980
The prednisone medication was gradually tapered over a period of some 8 mo. At present, the patient is feeling well and performing all her normal duties. Her body weight has increased to 65 kg. There is no evidence of diarrhea. The urinary d-xylose excretion is 5.1 g/5 hr after a 25-g load. Vitamin B,, is normal whereas carotene and folic acid levels fluctuate around the lower limit of normalcy. Extensive malabsorption work-up was turned down by the patient. Upon physical examination, a shrunken, hard, well-delineated mass, measuring approximately 7 x 8 cm, is still palpable to the left of the umbilicus. Now, well over 1 yr after stopping the prednisone medication, and more than one-half year after stopping the azathioprine treatment, there is no evidence of reactivation of the process, the patient remaining symptom-free in a clinically stable and satisfactory condition.
Discussion Figure 1. Superficial part of a large surgical biopsy showing dense collagenous tissue (C) and a focal mainly lymphocytic inflammatory infiltrate around adipocytes (arrow).
About 1 yr after her first laparotomy, incessant vomiting because of duodenal obstruction (Figure 2) occurred, leading to dehydration, metabolic alkalosis, and renal insufficiency. For relief of her progressive obstructive symptoms, a second laparotomy was performed, demonstrating that the mass had obviously progressed, involving and fixing all of the jejunum to the dense fibrotic process and causing total obstruction at the duodenojejunal angle. Only the distal 1.4 m of ileum could be mobilized and anastomosed side-to-side to the greater curvature of the stomach followed by a Braun-type anastomosis between the distal jejunum and ileum. Biopsies of the mass showed abnormalities similar to those previously seen, again without evidence of malignancy. Postoperatively the patient continued to go downhill. Stomach emptying via the gastroileostomy was rather slow. In addition the patient complained of chronic abdominal pain, episodes of vomiting, general malaise, and fatigue, and her temperature ranged between 38 and 39.5’C. She kept losing weight and reached a body wt of 52 kg. Because of this deterioration of her condition, she was hospitalized again in April, 1976. Upon admission, a chronically ill, feverish, slightly emaciated woman was found. The abdominal mass had further progressed (Figure 3, Table 1). The ESR had risen to 105 mm/hr, and her hemoglobin level dropped to 9.7 g/dl. In view of the dismal outlook and the relentlessly downhill course, it was finally decided to treat the patient with prednisone and azathioprine in a desperate attempt to slow down further progression of the lesion (see Table 1 for dosages). Upon institution of this treatment, the fever disappeared within a few days. Together with gradual improvement of her general condition and well being, there was progressive fall of her ESR to normal. Hemoglobinlevel and body-weight responses are summarized in Table 1. As judged by palpation, the mass gradually decreased in volume. Shrinkage of the mass was confirmed by ultrasonographic measurement (Figure 4).
Retractile mesenteritis (or mesenteric panniculitis) is an intriguing disease of unknown cause despite extensive speculation. In his literature survey of 71 patients, Jura’ considered, as a cause, preceding trauma of pathogenetic importance because the majority of his patients had experienced abdominal surgery, abdominal tuberculosis, or mesenteric thrombosis, conditions all considered to be potentially traumatic to the mesentery with ultimate evolution toward fibrosis and retraction. In support of this hypothesis, Tedeschi and Botta” injected bac-
Figure
2. Barium meal showing nearly third duodenum (arrow) with proximal duodenum.
total obstruction marked dilatation
of the of the
354
TYTGAT ET AL.
Figure 3. Transverse echogram at the cus (LJ) in August, 1977. The in the left abdomen between measures 8 x 9 cm. To the bowel gas (B) are seen.
GASTROENTEROLOGY
Vol. 79, No. 2
level of the umbilitumor is visualized the two arrows and right, reflections of
teria or bacterial extracts in the mesentery of animals and produced degeneration of mesenteric fat cells, inflammation, and subsequent fibrosis. In many patients, however, as in this case report, preceding mesenteric trauma was absent. Thus surgery is not a prerequisite for mesenteric fibrosis, and other hypotheses were proposed. Steinberg” presented 2 patients with systemic nodular panniculitis with widespread involvement of the adipose tissue, and he considered this illness to be a systemic form of Weber-Christian’s subcutaneous nodular panniculitis. Crane et al.’ considered the mesenteric lesion to be a localized form of lipodystrophy. Similarly Soergel and Hensley” described a patient who initially had mesenteric lipogranulomata and lipodystrophy, which, upon further evolution, led to complete mesenteric fibrosis and retraction. Finally, other authors’,” relate retractile mesenteritis to retroperitoneal fibrosis, stressing the similarites be-
Figure 4. Transverse echogram at the level of the umbilicus (U) in January 1978. The tumor between arrows has regressed to 5 x 6 cm. Some internal reflections within the tumor are now visible.
tween the two disease entities. In some patients, both diseases even appeared to coexist.‘.” Presumably retractile mesenteritis has to be considered as the nonspecific end stage of a variety of injuries, particularly of the mesenteric adipose tissue, leading to fibrosis and retraction. This mesenteric scarring can lead to secondary occlusion of lymph vessels causing lymph stagnation and occasionally to obstruction of mesenteric veins with fatal ischemic bowel damage.’ The diagnosis of retractile mesenteritis in our patient was based upon the macroscopic and histologic findings and upon the clinical course of the disease. The common gross pathologic appearance of retractile mesenteritis consists of a markedly thickened mesentery of rubbery consistency, or a large solitary mass in the root of the mesentery or of several masses stuck together, showing puckering of the mesenteric surface.3.4 Usually the peritoneum firmly
August
1980
RETRACTILE
MESENTERITIS
355
adheres to the inflammatory mass. Occasionally there is conspicuous mesenteric shortening and fibrosis. In some patients, a collection of chylous fluid, apparently subsequent to liquefaction of adipocytes, is present within the mesenteric mass.8.“,‘2 The corresponding histologic features include: foci of degeneration and necrosis of adipocytes, accumulation of lipophages, chronic predominantly lymphocytic inflammation mixed with histiocytes, and occasionally foreign body giant cells, and, in addition, various degrees of fibrosis with massive accumulation of dense collagenous tissue. In the majority of the patients the clinical course is characterized by vague abdominal fullness and heaviness, pain and discomfort, bouts of vomiting, fever, development of a deep-seated and usually poorly defined abdominal mass, and occasionally signs and symptoms of partial intestinal obstruction.2-4 The prognosis of retractile mesenteritis is variable. In some patients, the inflammatory process resolves, and there is more or less complete recovery. In others, the disease takes a more protracted course with fibrosis and retraction of the mesentery leading to partial intestinal obstruction. The course in our patient was dominated by the progressive enlargement of the tumorlike mass, which caused intestinal obstruction. Because of the unrelenting, gradual deterioration of her general clinical condition, a fatal outcome was thought to be imminent and unavoidable, as has previously been noted by other authors.R,“’ In view of this dismal outlook, treatment with prednisone and azathioprine was initiated in a desperate attempt to arrest the basic chronic inflammatory process, presumably related to degeneration of the mesenteric adipose tissue, in analogy with therapeutic attempts in Weber-Christian disease, although admittedly the cause of this illness remains obscure. Azathioprine was added to the prednisone to potentiate its antiinflammatory activity and because of its well-known steriod-sparing effect, in analogy with the treatment of many other chronic inflammatory conditions. Upon institution of this treatment, there was a steady progressive improvement of general well being, reversion of the biochemical abnormalities towards normal values, disappearance of fever, and regression of the mass to a smaller well-delineated, hard lesion, thought to be the end-stage of scarring. Other authors also noted a beneficial effect of corticosteroid therapy.“.“~‘” Other treatments (antibiotics, irradiation, emetine, and azathioprine) have been tried without obvious success4 Surgical treatment should be limited to exploration and biopsy with intestinal bypass procedures whenever indicated. Resection of the tumor mass is often impossible, or does not seem to be justified,
356
TYTGAT ET AL.
when considered feasible, especially if surgery involves resection of large segments of small bowel or possibly compromises the mesenteric arterial circulation.‘,” In view of the unequivocal improvement in our patient upon treatment with prednisone and azathioprine, we would like to recommend an early trial of such treatment once the diagnosis is established in an attempt to prevent progression of the lesion and perhaps to avoid surgical bypass procedures. Why the disease apparently remains in remission in our pateint after tapering and stopping the corticosteroid and azathioprine therapy is difficult to understand and will presumably remain unknown until more information on the cause becomes available.
References 1. Jura V: Sulfa mesenterite
retrattite e sclerosante. Pohclinico [Prat] 31:575, 1924 2. Aach RD, Kahn LI, French RS: Obstruction of the small intestine due to retractile mesenteritis. Gastroenterology 54:594, 1966
GASTROENTEROLOGY
Vol. 79, No. 2
3. Kipfer RE, Moertel ChG, Dahlin DC: Mesenteric Lipodystrophy. Ann Intern Med 69562, 1974 4. Durst AL, Freund H, Rosenmann E, et al: Mesenteric panniculitis: review of the literature and presentation of cases. Surgery 61:203, 1977 5. Tedeschi GC, Botta GC: Retractile mesenteritis. N Engl J Med 266:1035, 1962 6. Steinberg B: Systemic nodular panniculitis. Am J Path01 29:1059, 1953 7. Crane JT, Aguilar MJ, Grimes OF: Isolated lipodystrophy, a form of mesenteric tumor. Am J Surg 90:169, 1955 6. Soergel KH, Hensley CT: Fatal mesenteric panniculitis. Gastroenterology 51:529, 1966 9. Handelsmann JC, Shelley WM: Mesenteric panniculitis. Arch Surg 91:642, 1965 10. Leger F, Roberti A, Deloche de Noyelle A, et al: Pannicuhte mesenterique, variete de fibrosclerose retro-peritoneale. Presse Med 75:75, 1967 11. Ogden WW, Bradburn DM, Rives GD: Mesenteric pannicuhtis. Ann Surg 161:664, 1965 12. Pallette EM, Pallette EC, Harrington RW: Sclerosing lipogranulomatosis: its several abdominal syndromes. Arch Surg 94:603, 1967 13. Detrie P: Retractile mesenteritis. J Chir (Paris) 88:341. 1964 14. Godquin B, Saout R, Loupias P, et al: Pseudo-tumeur inflammatoire et necrotique du mesentere. J Chir (Paris) 104:167, 1972