Intestinal obstruction by eosinophilic jejunitis

J. M. Álamo Martínez, F. Ibáñez Delgado, A. Galindo Galindo, C. Bernal Bellido, I. Durán Ferreras,
G. Suárez Artacho and A. Martínez Vieira

Department of Surgery. Hospital Universitario Virgen del Rocío. Sevilla, Spain

 

ABSTRACT

Eosinophilic enteritis is an uncommon disease that rarely devel-ops as a surgical emergency. Although it may be associated with infestation by Ancylostoma caninum, its etiology is unknown and often related to a personal or family history of atopy. A transmural involvement may cause intestinal obstruction -more frequently in the jejunum- or even acute abdomen, which may or may not be accompanied by intestinal perforation. The latter two conditions tend to be more commonly associated with ileum disease, causing pain in the lower right quadrant of the abdomen. Patient history, eosinophil count -which may be paradoxically reduced when the disease appears in this way-, ultrasonography, and/or CT lead to the suspicion of this condition before a surgical procedure is considered. A definitive diagnosis, however, must be reached by means of an anatomopathological study. Macroscopically, intestinal loops exhibit a thickened appearance with an elastic consistency. Laparoscopic intestinal biopsy may play a major role in the diagnosis of disease.

Key words: Intestinal obstruction. Eosinophilic enteritis. Urgent surgery.

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Álamo Martínez JM, Ibáñez Delgado F, Galindo Galindo A, Bernal Bellido C, Durán Ferreras I, Suárez Artacho G, Martínez Vieira A. Intestinal obstruction by eosinophilic jejunitis. Rev Esp Enferm Dig 2004; 96: 279-283.

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Recibido: 15-04-03.
Aceptado: 09-07-03.

Correspondencia: José María Álamo Martínez. C/ José Laguillo, 27-3, 7º C. 41003 Sevilla. Telf.: 954418636

 

INTRODUCTION

Eosinophilic enteritis is an uncommon condition that rarely presents as a surgical emergency. Infiltration of the intestinal wall by eosinophils causes a variety of clinical features depending on their intensity and location. Thus, the disease may present itself as a digestive malabsorption syndrome, intestinal stenosis, or ascites, which will depend on whether eosinophils predominate in the mucosa, muscularis or serosa layer (1). However, cellular infiltration of the intestinal loop may cause an abdominal syndrome requiring urgent care and a prompt surgical procedure.

There have been described few cases of urgent surgical intervention for eosinophilic enteritis, but data collected point to the suspicion that location of infiltrates will condition the clinical presentation. It appears that jejunal involvement is more frequently associated with intestinal occlusion while ileitis complicates more commonly with acute abdomen in the lower right quadrant of the abdomen or loop perforation.

Peripheral blood eosinophil count and a personal and family history of atopy may help in diagnosing the disease. Imaging studies using ultrasounds or computerized tomography, or even an exploratory laparoscopy can complete the diagnosis in cases of suspected eosinophilic enteritis.

CASE REPORT

A 50-year old woman presented to the Emergency Room complaining of epigastric pain, biliary vomiting, absence of passage of flatus and stool for 48 hours. A physical examination revealed a distended abdomen with no signs of peritonitis. Abdominal auscultation revealed no peristalsis. A rectal palpation revealed an empty ampulla. A small, uncomplicated umbilical hernia was also present. The patient had a history of anxiety-depression syndrome, arterial hypertension, umbilical hernia, and frequent upper abdominal disconfort, all diagnosed by her family practitioner, and latter considered to be secondary to an esophageal hiatal hernia. Previous surgery included appendectomy and tubal ligation.

A hemogram and blood chemistry revealed leukocytosis of 17,000/cc with neutrophilia of 85%, as well as monocytosis and eosinopenia. Plain abdominal radiograph in upright position demonstrated air-fluid levels and an empty rectal ampulla.

As conservative measures yielded unsatisfactory results, an emergency laparotomy was performed with the diagnosis of intestinal obstruction caused by surgical adhesions.

During the procedure a segment of thickened and rigid jejunum was found, which caused stenosis of the lumen and pre-stenotic intestinal dilatation. An enterotomy with extraction of non-digested food was carried out, along with a subsequent resection of 20 cm of jejunum and an end-to-end anastomosis with manual suture. No mesenteric adenopathies or other macroscopic alterations were observed prior to closure. There were no postoperative complications except for an easily resolved ileus.

The histopathological study of the resection piece gave the diagnosis of “eosinophilic enteritis”.

DISCUSSION

Eosinophilic enteritis is a rare pathological condition. It is pathologically characterized by an infiltration of the intestinal wall by mature polymorphonuclear eosinophil cells. The presence of eosinophils in the intestinal wall may be diffuse (gastroenteritis) or, more commonly, segmentary (even in the form of an eosinophilic granuloma, common mainly in the gastric antrum). Cases of esophagitis unresponsive to medical and surgical treatment, which were later diagnosed of eosinophilic enteritis, have been described; these patients usually improved with corticosteroid treatment (2). Some cases have also been reported with eosinophilic infiltration in a number of organs such as the gallbladder, liver, and urinary tract.

Eosinophilic enteritis appears mainly in the third decade of life, but its diagnosis during childhood is not uncommon. Occasionally, a family history of atopy (asthma, rhinitis, conjunctivitis,...) (1) may be found. Although its etiology is not clear, it has been unsuccessfully related to food allergy. Its association with an infestation by Ancylostoma caninum is more evident. This disease has been widely studied, particularly by Croese and Prociv (3-6) in Australia where this disease is specially frequent. This entity has also been described in patients treated with carbamazepine (7).

The pathophysiology of this disease depends upon the cellular infiltration of the intestinal wall. Increased eosinophilic cellularity is generalized throughout the intestinal wall, but is always more severe in some of its layers. Although the most frequent form of presentation is a failure of the intestinal absorptive function resulting in a malabsorption syndrome, the condition may occasionally present itself with intestinal obstruction, intestinal perforation, or pain in the lower right quadrant of the abdomen, which may resemble appendicitis. Thus, infiltration of the mucosa would lead to a bowel malabsorption syndrome whose severity would depend on the affected area. Serosal infiltration results in ascites. Most important for surgeons is the transmural infiltration of the muscular layer, which may result in one of the following syndromes:

1. Intestinal occlusion, potentially requiring an emergency laparotomy. Few cases of this complication have been reported, but they result from jejunal involvement (8-10) and cause small intestinal type of obstruction.

2. Acute abdomen, the most frequent surgical complication. It usually resembles and is managed as an acute appendicitis. The diagnosis is established during surgery (11-15).

3. Intestinal perforation syndrome. This has been described mainly in the distal ileum (16,17).

The rare incidence of this disease makes it difficult to differentiate it from patients with intestinal obstruction, acute abdomen, or hollow visceral perforation. Although peripheral blood eosinophilia can seemingly be important in the diagnosis of this condition, it frequently does not develop, or may develop only after surgery. This circumstance has been described by Croese and Prociv in a review of 6 surgically operated patients (12) and in 9 patients who were studied for abdominal pain, where 33.3% did not present eosinophilia (13). Buchman also reported a patient who underwent surgery for intestinal perforation (17). Although peripheral blood eosinophilia is a very important fact when this pathology is suspected, a normal count of eosinophils or even, as in our case, eosinopenia does not rule out the disease. In some occasions, eosino-philia develops after surgery.

Pozniak suggests using ultrasounds as a useful method to differentiate eosinophilic gastroenteritis from Crohn’s enteritis, lymphoma, intestinal ischemia, and appendicitis (18). In cases of eosinophilic enteritis, a thickening of the intestinal wall is usually found (11). In the presence of intestinal obstruction and in patients with acute abdomen who undergo a diagnostic procedure, ultrasounds can offer interesting data. The finding of a thickened intestinal segment will support the clinical and analytical suspicion of eosinophilic enteritis; more easily, ultrasounds will rule out this diagnosis by finding signs of appendicitis. A radiological study with CT imaging may also be very useful (19), but the administration of an oral contrast is not appropriate in cases of intestinal occlusion. Diagnostic laparoscopy may show a thickened intestine with or without mesenteric adenopathies (20), and eventually intestinal biopsy can be taken as described by Edelman (21,22). Diagnostic laparoscopy is indicated in patients with intestinal obstruction or abdominal pain in whom exist the clinical suspicion of this disease based on a history of atopy, increased eosinophil count in peripheral blood, and suggestive ultrasound findings.

The diagnosis, which may only be reached by histopathological analysis, reveals an infiltration of the gut with polymorphonuclear eosinophils, which should be differentiated from that appearing in Crohn’s disease, intestinal lymphomas, or hypereosinophilic syndromes.

Mesalamina, azathioprine and cromoglycate have been successfully used in the treatment of intestinal obstruction caused by eosinophilic enteritis. It would be interesting to investigate whether immunosuppressive treatment would avoid surgery in patients with this disease, particularly in those with intestinal obstruction (23).

The following conclusions can be drawn:

-Eosinophilic enteritis is an uncommon condition that usually is associated with an intestinal malabsorption syndrome. However, it may acutely present as an intestinal perforation, intestinal occlusive syndrome or, more frequently, as abdominal pain or acute abdomen.

-Intestinal perforation tends to occur in the terminal ileum (eosinophilic ileitis), and a surgical procedure without further delay is mandatory. Ileitis may cause an acute abdomen resembling acute appendicitis.

-Intestinal obstruction is more frequently due to jejunitis. Thus, it should be suspected in occlusive syndromes at upper intestinal levels.

-Eosinophilia is suggestive of the disease, but a normal eosinophil count does not exclude this disease. A personal or family history of allergic manifestations can assist in the diagnosis.

-Ultrasounds or emergency CT may either reveal a thickened intestinal loop suggestive of eosinophilic enteritis or rule out other causes of acute abdomen or intestinal obstruction. Exploratory laparoscopy with biopsy is an option for cases with a high suspicion of this disease.

It would be interesting to see whether these diagnostic efforts could be directed toward finding a medical treatment with corticosteroids that could preclude surgery in cases of subocclusive syndrome or acute abdomen. However, the low frequency of this disease as a condition requiring emergency surgery will render it difficult to make a reliable statistical study.

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