Proximal jejunal obstruction due to eosinophilic gastroenteritis.TT Karande, SN Oak, AA Trivedi, SS Karmarkar, BB Kulkarni, AA Kalgutkar
Department of Paediatric Surgery, LTMG Hospital, LTM Medical College, Sion, Mumbai, Maharashtra.
Eosinophilic enteritis or gastroenteritis is a rare disease characterised by tissue eosinophilia which can affect different layers of bowel wall. It can affect any area of gastrointestinal tract from the esophagus to the rectum, although stomach and small intestine are sites most frequently reported. It is important to recognize this disease early and institute the necessary treatment. An eight year old girl presented with acute intestinal obstruction. Exploration revealed a structure of proximal jejunum. Histopathology demonstrated eosinophilic jejunitis. The case is reported owing to this rarity and relevant literature is reviewed.
Keywords: Acute Disease, Case Report, Child, Eosinophilia, complications,pathology,Female, Gastroenteritis, complications,pathology,Human, Intestinal Obstruction, etiology,surgery,Jejunal Diseases, etiology,surgery,
Eosinophilic gastroenteritis was initially characterised by the triad of peripheral eosinophilic, eosinophilic infiltration of segments of gastrointestinal tract and abnormalities of gastrointestinal function. Kaijser in 1937 is credited with the first description of eosinophilic gastroenteritis. Exact pathogenesis is poorly understood but allergy seems to play a role. The clinical picture is dominated by abdominal symptoms. Eosinophilic involvement of one or more areas of the gastrointestinal tract is conspicuous at biopsy. In 20% patients peripheral blood eosinophilic is absent and hence this criteria cannot be included in diagnostic requirements-.
We report a case of an eight-year-old girl who presented with symptoms suggestive of proximal small bowel obstruction and wherein exploration revealed jejunal obstruction. Histo-pathology confirmed the diagnosis of eosinophilic gastroenteritis.
An eight-year-old girl was brought to us with history of intermittent colicky abdominal pain with distension of abdomen of one month duration. Patient was empirically given antituberculous therapy prior to hospitalisation for one month. There was no history of any food or drug allergy. On examination the patient was mildly dehydrated, vital parameters were normal. There was fullness and generalised distention of abdomen. Bowel sounds were present. Per rectal examination was normal.
Blood investigations did not reveal anaemia, eosinophilia or hypoproteinaemia. X-ray abdomen showed air fluid levels. A barium meal follow through examination revealed narrowing and suggested partial obstruction at proximal jejunum hence surgery was contemplated. Exploration revealed narrowing of jejunum 10 cms. from duodenojejunal flexure. The proximal bowel was hypertrophied and distal bowel was collapsed. [Figure - 1]
The lumen was opened to find thickening of the bowel wall at that site. [Figure - 2]. A Ryle's tube No. 14 was passed to rule out any other additional obstruction proximally and distally. There was mesenteric lymphadenopathy. The narrowed jejunal segment was resected and end-to-end anastomosis performed Mesenteric lymph node was taken for histo-pathological examination.
Histopathological examination of the stenotic lesion showed marked hypertrophy of muscularis mucosa with changes of obstruction in the mucosa and submucosa oedema. There was dense lymphoblastic infiltrate with prominent admixture of eosinophils infiltrating the lamina propria and submucosa and sparse inflammation of muscularis propria and serosa.
There was no evidence of granuloma or caseous necrosis or malignancy. Accompanying mesenteric lymph node showed reactive hyperplasia with infiltration of eosinophils in the sinusoids. Histological features were in favour of eosinophilic gastroenteritis.
The first description of eosinophilic gastroenteritis dates back to 1937 and is credited to Kaijser. The disease is uncommon, less than 300 cases have been reported till 1993 in medical literature. This usually presents in third to fifth decade and is almost unheard of in children.
A definite case needs to fulfil the following criteria:
1. Presence of gastrointestinal symptoms.
2. Demonstration of eosinophilic infiltration in one or more areas of gastrointestinal tract in biopsy.
3. Absence of eosinophilic involvement of multiple organs outside the gastrointestinal tract.
4. Absence of parasitic infestation,.
Moreover, food intolerance or history of food allergy is not mandatory for the diagnosis. To establish food allergy as a definite precipitating factor, some criteria need to be fulfilled. According to Ingelfinger and associates, symptoms must invariably follow contact with a specific food substance that is harmless to most people; immune mechanisms should be evident; other possible pathogenic mechanisms should be absent: and lesions or functional abnormalities should be demonstrable after contact. Leinbach and Rubin have suggested that eosinophilic gastroenteritis is not a single reversible allergic reaction to specific foods, but rather a chronic process that may be symptomatically aggravated by different foods. In many patients, however, objective allergic responses are absent or are inadequate to account for clinical findings and elimination of diet fails to relieve symptoms.
Recent data suggest that eosinophils may directly damage the gastrointestinal tract wall in eosinophilic gastroenteritis. Tissue eosinophilia is the hallmark of the disease process. Normally after eosinophils leave the circulation, they preferentially get distributed in the stomach and small bowel. It is likely that eosinophils play a role in the gut in immediate hypersensitivity reactions involving gut.
The histological characteristic is oedema and an inflammatory cell infiltrate, composed of eosinophils, which may appear in clumps. Maximum infiltration of eosinophils is seen in submucosa. Stomach and small intestine are most commonly affected though it can affect any area of gastrointestinal tract.
Based on the predominant layer of gut wall involvement, it can be classified as mucosal, muscle layer and serosal-layer disease. Mucosal layer disease typically presents as colicky abdominal pain, nausea, vomiting, diarrhoea and weight loss. Fifty percent of patients have past of family history of allergy. Predominant muscle layer disease presents as pyloric or intestinal obstruction, usually localised. Food intolerance or allergy is uncommon in this type of variant. Our patient presented with this type of picture. The rarest form is serosal layer disease, where the whole bowel is involved and in this pattern eosinophilic ascites is noted. Steroids have a role in management of disease in patients with non-allergic origin. Serosal disease responds dramatically to steroids ,. Low dose maintenance therapy to maintain remission is advocated. Immunosuppressive therapy with azathioprine can be used as an adjunct to steroids. If in acute exacerbations steroids are unable to control the morbidity, then sodium cromoglycate is employed. It prevents the release of mast cell toxic mediators and can also reduce absorption of antigens by the small bowel. Chromoglycate has also been used to treat milk allergy and other gastrointestinal allergic reactions in children. Surgery should be considered in patients with localised disease who present with obstruction and if treatment with steroids is unsuccessful, In case of the present report as the entity was not considered prior to exploratory laparotomy and as the patient had presented with acute obstruction, surgical resection was offered.
The long-term outlook for patient with eosinophilic gastroenteritis is generally favourable i.e. mortality related to the disease itself is rare, however progressive weight loss leading to profound cachexia unresponsive to treatment has been reported. Eosinophilic gastroenteritis does not predispose a patient for a gastrointestinal malignancy.
[Figure - 1], [Figure - 2]