Idiopathic sclerosing mesenteritis in paediatrics: Report of a successfully treated case and a review of literature
© Viswanathan and Murray; licensee BioMed Central Ltd. 2010
Received: 25 June 2009
Accepted: 21 January 2010
Published: 21 January 2010
A 6 year old female with symptoms of small bowel obstruction underwent an exploratory laparotomy which revealed widespread evidence of inflammatory fibrotic adhesions involving the jejunal mesentery. In view of persistent growth failure, chronic anaemia, elevated acute phase reactants and imaging evidence of a diffuse progressive inflammatory process, the child was treated with corticosteroids and methotrexate with complete response. The literature on juvenile idiopathic sclerosing mesenteritis has been reviewed.
Idiopathic sclerosing mesenteritis (ISM) is an uncommon disease involving the small bowel mesentery and characterised by chronic inflammatory changes ultimately progressing to fibrosis. The first reported series featured 34 cases of "retractile mesenteritis and mesenteric sclerosis"; published in 1924 [1–3]. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, liposclerotic mesenteritis, xanthogranulomatous mesenteritis, mesenteric Weber Christian disease and systemic nodular panniculitis . These names possibly reflect the underlying pathology involved with mesenteric lipodystrophy (predominant fatty degeneration), mesenteric panniculitis (chronic inflammation) and retractile mesenteritis (predominant fibrosis) being the common histologic variants. It has been postulated that these variants represent the varied spectrum of a single disease process which could be termed idiopathic sclerosing mesenteritis .
Though the exact aetiology remains obscure, proposed mechanisms include prior abdominal trauma/surgery, autoimmunity, infection and ischemia . A wide range of therapeutic initiatives have been reported including corticosteroids, colchicine, immunosuppressives (cyclophosphamide and azathioprine), tamoxifen, thalidomide and hormonal therapies with varying success [6, 7]. The disease is considered rare in the pediatric age group with only 16 cases reported to date. We describe a case of ISM in a young girl who was treated successfully with corticosteroids and methotrexate, and briefly review the pediatric literature on this subject.
A 6 year old girl presented at a local hospital with an acute onset of abdominal pain, nausea and bilious vomiting. A history of constipation, recurrent vomiting associated with high fever in the preceding 12 months was elicited. Clinical examination was reported to be normal at the time. Complete blood counts revealed haemoglobin (Hb) 94 g/l, WBC 18.4 × 09/l (Absolute PMN 15.6 × 109/l, Lymph 2.0 × 109 /l) and platelets 663 × 109 /l. Her ESR was elevated at 54 mm/hr [1-15 mm/hr) and CRP 84 mg/l (<10 mg/l). Ultrasonography of the abdomen revealed diffuse, mesenteric lymphadenopathy. A barium meal with follow through suggested a small bowel obstruction. A laparotomy showed extensive intraperitoneal adhesions, mesenteric lymphadenopathy and a tight jejunal band with obstruction. The adhesions were divided, the obstruction was relieved and the patient's bowel function recovered steadily. Tissue cultures were negative. Histopathological analysis of the peritoneal tissue revealed fibro-fatty tissue with extensive fibrosis throughout, infiltrated by many scattered lymphocytes, plasma cells and occasional neutrophils but no malignant cells. The single lymph node examined revealed dilated sub-capsular sinuses with an expanded para-cortex with mixed histiocytes and occasional immunoblast with no granulomas, Reed Sternberg cells or any evidence of lymphoma. Ziehl-Neelsen stains were negative for tuberculosis organisms as was subsequent culture. The clinical condition improved slowly over the subsequent 2 weeks and she was discharged on oral iron supplements. No evidence of mono-clonality was seen on flow cytometry for immuno-phenotyping of lymph node tissue.
A rheumatology consultation was made. Review of the case revealed multiple episodes of subacute small bowel obstruction with chronic anaemia, weight loss, and a chronic inflammatory process with fibrosis on mesenteric biopsy and elevated acute phase reactants. Further investigations revealed a borderline positive ANA of 1:40 (speckled pattern), a negative dSDNA and rheumatoid factor (RF). Total serum proteins were 82 g/l, albumin 40 g/l (32-48 g/l), globulins 42 g/l (23-35 g/l), and serum IgG 15.7 g/l (6-12.3 g/L) indicating mild hypergammaglobulinemia. IgG subclasses IgG1 11.1(4.0-10.8 g/L), IgG2 4.3 (0.8-4.1 g/L), IgG 30.4 (0.1-1.4 g/L), IgG4 1.47 (<1.9 g/L) were unremarkable. An abdominal angiogram was done to screen for major vessel vasculitis such as polyarteritis nodosa, and was negative.
Clinical manifestations and course of illness in 17 children with sclerosing mesenteritis
Special features (if any)
McGee et.al (1965)
A, Asc/unknown duration
Died ,3 weeks post surgery (Bacterial endocarditis )
No 'persistent fibrosis on autopsy.'
A, WL, F/Recurrent symptoms over 12 yrs
Died ,12 yrs after initial symptom
Steatorrhea, secondary amyloidosis
Black et.al (1968)
A, N, V, D, F, M/6 weeks
Sx, Partial resection and ileocolostomy
UR/NF ,5 mths
Blunt trauma one week prior.
A/M Two weeks
Sx/Hemi colectomy with ileo colic anastomosis
UR/NF ,9 mths
Misaka et.al (1977)
Sx/enterostomy with resection
UR/NF ,12 mths
Melo et.al (1980)
A, N, V, M
Dor et.al (1982)
Sx/enterectomy, partial splenectomy and partial pancreatectomy
Cakmak 22et. al (1986)
A/C/F/V, acute onset
UR/NF, 20 mths.
Recurrence after 2 weeks.
Jona et.al (1987)
A, F/7 days
UR/NF ,51/2 yrs
Davis et.al 24(1992)
A, An, V/one day
UR/NF 2 mths.
Ueda et.al (1997)
V, M/2 days
UR/NF ,6 months
Fulminant hep A with recurrent ascites
Ito et.al (1998)
UR/NF, 9 years
Hakguder et.al 11(2000)
A, N, An/Not specified
UR/NF, 6 months.
Kawano et.al (2003)
Kawano et.al (2003)
F, M/Not specified,
Kawano et.al (2003)
F, M/Not specified
Our case (2008)
A, V, WL, C/Recurrent episodes 6 months
Sx, Steroids, Methotrexate
UR/NF, 6 months.
Methotrexate first reported pediatric case.
Various patho-physiological mechanisms suggested include previous surgery, trauma, hypoxia, allergy, infection and autoimmunity [6, 9, 10]. Concurrent intra abdominal pathologies have been reported in adults including lymphoma and ovarian tumours. One child had a coincidental presence of a Meckels diverticulum  while another had a blunt trauma to the abdomen a week before developing clinical manifestations. (See table 1). Other associated immuno-hematological anomalies such as common variable immunodeficiency, polyclonal gammopathies, acute myeloid leukaemia and myelodysplasias have been reported in adults. Understandably monoclonal/oligoclonal B cell expansion has been suggested as a possible mechanism, as it has in a possibly related disorder, retroperitoneal fibrosis. Inflammation in ISM has been attributed to adipocytokines, including adiponectin, resistin, leptin, IL-6, and TNF-α. Macrophages found in adipose tissue may trans-differentiate from local preadipocytes, supporting the hypothesis that adipocytes and macrophages may be interconvertible, implicating mesenteric adipose tissue in disorders such as ISM . Autoimmunity has been associated with ISM, including the co-occurrence of Sjogren syndrome, sarcoidosis, rheumatoid arthritis and ankylosing spondylitis. Similarly an association of ISM and other sclero-fibrosing disorders in other organs has been reported in adults [13, 14]. (More recently, ISM has been regarded as a possible subset of IgG4 related sclerosing disorders with increased expression in the lesional plasma cells. No evidence of elevated IgG4 was found in this case but the biopsy was not specifically stained for subclasses . Apart from one child with SLE none of these has been reported in pediatric literature to date . Our case did exhibit some features of autoimmunity with a positive ANA and hypergammaglobulinemia.
The onset of the disease may be acute, insidious or have recurring episodes before the actual diagnosis is made . Varied clinical manifestations have been reported, primarily due to mechanical effects of the encasing mass/inflammation involving bowel loops, mesenteric vessels and lymphatics. These include abdominal pain with a clinically palpable mass, bilious vomiting, diarrhoea, constipation and abdominal distension. Associated features of chronic inflammation including prolonged pyrexia, weight loss, and progressive anaemia can be seen but may be more prominent in the early phase.
In the pediatric cases, of the 17 children, 4 were described as mesenteric panniculitis while the remaining 13 termed sclerosing mesenteritis. Eleven (64.7%) children presented with abdominal pain, 7 (41%) had vomiting, 4 (23.5%) with bowel symptoms in the form of constipation or diarrhoea, 8 (47%) with associated fever and 2 (11.7%) with weight loss. Only 5 (29%) children had a clinically palpable mass corroborated on exploration. Laboratory investigations were usually non specific ranging from normal to neutrophilic leucocytosis and variable elevation of acute phase reactants. Five of the 13 (38%) children tested demonstrated anemia and 9 (62%) leucocytosis. 4 (30%) children had normal white cell counts. Elevated acute phase reactants ESR/CRP were only reported in 5 children [18–26].
Imaging modalities in idiopathic sclerosing mesenteritis in children
Displaced DC, Small bowel obstruction, splenomegaly
Initial normal, then thickened folds, segmentation of contrast material.
Mass displacing cecum &AC.
Mass & narrowing AC.
Heterogeneous, solid TM between spleen, left kidney and pancreas
Mass on medial AC
Narrowing of SC
Soft Tm in mid abdomen around AC to DC. Retraction of TC, Dilated AC
Solid soft TM over the lower TC
E/o TM over the hepatic flexure
Tumor mass displacing the Splenic flexure
Anatomic Involvement and pathological findings on biopsy.
Anatomic area of involvement
Mesentery (AC and TC)
FB with LI
Mesentery (SB, largely at root)
Fat necrosis(early phase )→Extensive FB (later )
Mesentery (TI), plaques on UT, OV, PP.
FB with absent residual fat
T M in mesentery (TI, IC and Ap )
FB with LI and PI.
Mesentery (I, AC, TC, UT)
FB with LI and PI.
FB with EI.
TM (MG adherent to the Splenic artery)
Panniculitis and severe fibrinoid necrosis.
Mesentery (GC and TC )
Severe inflammatory changes minimal FB.
TM ( right colon and H (No obstruction ))
Hemoperitoneum, TM (SI and TC )
FB with LI and foamy macrophages.
TM (Whole colon and MC (except RS) with perforation, normal SI.
FB with necrosis and LI.
Increased adipose tissue with FB with necrosis and LI.
FB with inflammatory infiltration.
Mesentery (TC )
FB with inflammatory infiltration
Greater omentum (TC)
FB with LI and PI.
FB with LI and F.
Extensive intraperitoneal adhesions, jejunal band with evidence of proximal obstruction
Fibro fatty changes with extensive LI and PL
No specific guidelines regarding the management of ISM exist and various medical and surgical modalities that have been tried. Use of anti-inflammatory, anti fibrotic and immunomodulatory agents such as cortico-steroids, methotrexate, azathioprine, colchicine, thalidomide and Tamoxifen have all been reported either alone or in combination with varied degrees of success [6, 7]. Thalidomide might act by modulating the production of tumor necrosis factor-alpha (TNF-α) and other cytokines along with its inhibitory effects on angiogenesis . Though reported in adults , our case is to our knowledge the first child to be treated with methotrexate. We used methotrexate because of the evidence of ongoing chronic inflammation and in part the reported role of methotrexate in ameliorating adiponectin which may thus inhibit the migration of macrophages to the mesenteric adipose tissue and suppress local TNF-α driven proinflammatory pathways .
Surgical exploration for diagnostic and therapeutic (adhesiolysis, debulking with or without resection anastomosis) purposes has been reported with all the children having some form of surgical intervention with only two cases not having actual tissue resection. Resection or debulking probably has a role in alleviating symptoms but may not help in prevention of disease progression.
Widely believed to have a good outcome in adults, the paucity of reported pediatric cases and long term follow up makes it difficult to plan management and prognosticate in children.
Decisions regarding the most appropriate form and duration of therapy in children should probably be guided by the severity and chronicity of symptomatology, growth of the child, and inflammatory markers. It seems rational that unless there is severe obstruction, medical/non-surgical management might be considered. Serial imaging is probably not indicated unless warranted by a clinical deterioration. Studies in adults have reported median duration of medical treatment between 2-30 months with recurrences up to 2 years after discontinuation though long term outcomes are not well reported ). Acute management of the condition with corticosteroids and introduction of a steroid sparing agent such as methotrexate if treatment seems likely be required for 3 months or more could be considered a reasonable. We suggest that treatment might be continued from at least 6 months from the time clinical and laboratory remission, as for other similar chronic inflammatory conditions of childhood.
Mortality secondary to ISM has been reported in 2 children and 4 adults [16, 18, 33]). Chronic inflammatory diseases have a much higher morbidity in growing children and a close follow up and individualisation of care is warranted with such a potentially variable course and outcome.
Written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
The authors would like to acknowledge the efforts of Ms Ayako Nakayama, pediatric nurse for helping with linguistic interpretation of some of the literature.
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