Spontaneous multiple small bowel perforations due to ischemic enteritis: a case report

Abstract

Introduction

Spontaneous multiple small bowel perforation refers to the complete transmural disruption of the intestinal wall and is often associated with conditions such as inflammatory bowel disease, infectious enteritis, drugs or autoimmune diseases, and it affects all age groups. Spontaneous multiple small bowel perforations due to ischemic enteritis is a rare clinical entity.

Case presentation

A 75-year-old man was admitted to our hospital with acute abdominal pain, vomiting, low-grade fever, bloody diarrhea, and abdominal distension. He had no history of trauma, comorbidities, or medications. Erect chest X-ray showed air under the diaphragm on the right side. An emergency laparotomy was performed. Intraoperatively, ischemic lesions and multiple perforations were localized to the jejunum and ileum and sent for histopathology which showed features of ischemic enteritis. Finally, the patient’s postoperative course was uneventful.

Conclusion

While ischemic enteritis is uncommon, multiple small bowel ruptures are a rare but real complication.

Introduction

Ischemic enteritis (IE) occurs when the blood supply to the small intestine is either blocked or severely reduced. Ischemic enteritis is significantly less common than ischemic colitis (IC) due to the ample blood supply to the small intestine. Although current knowledge on the epidemiology of ischemic enteritis is limited, studies have shown that the incidence is rare, yet increases in octogenarians from 2/100,000 person-years before age 80 to 40/100,000 person-years after age 80 [1]. However, younger individuals, particularly those with diabetes, lupus, or sickle cell disease, can also be affected. Histopathological examination is necessary to confirm a diagnosis. This disease progresses rapidly and has a high mortality rate of approximately 95% [2]. This report describes a rare case of acute abdomen resulting from multiple small bowel ruptures due to Ischemic enteritis.

Case presentation

A 75-year-old Ethiopian farmer male presented with seven days history of abdominal pain, which was initially on periumbilical, crampy type a day prior to his admission the pain became continuous, severe and generalized. Associated symptoms included low-grade fever, abdominal distension, vomiting of ingested food two to three times daily, and bloody diarrhea occurring up to three times per day. The patient had no prior history of trauma or recent endoscopic examinations. He reported no evidence of blood in the stool, nor was it oily, unusually large or abnormal stool consistency. There was no record of diabetes, high blood pressure, heart disease, allergies, or tobacco use. He was screened for retroviral infection a month ago and found to be seronegative and was not taking any medications for long time. On examination, he was acutely sick looking, vital signs were, pulse rate 104 beats per minute regular on radial artery, the other vital signs blood pressure, respiratory rate, oxygen saturation and temperature were in the normal range. Abdominal examination revealed a full abdomen moving minimally with respiration. Diffuse tenderness was present across the abdomen. Digital rectal examination showed the presence of stool. Otherwise, the rest of the examinations were unremarkable. On investigation, his complete blood count (CBC) was normal white blood cell count with left shift 89% of neutrophilia, anemia-HGB 10 g/dl but the rest CBC components were in normal range. Except hypoalbuminemia 2.48 mg/dl, otherwise liver, renal function test and coagulation profile were normal. Echocardiography showed diastolic dysfunction with ejection fraction 41% but electrocardiogram was normal. Upright chest X-ray showed free air under the diaphragm (Fig. 1).

Fig. 1

Upright chest X-ray showed free air under the diaphragm; labeled with the arrow

The patient underwent emergency laparotomy with diagnosis of generalized peritonitis, under general anesthesia and supine position by a general surgeon and general surgery residents. Intraoperatively, around 800 ml pus mixed with small bowel content all over the peritoneum with edematous bowel, more than nine small perforations were found on both mesenteric and anti- mesenteric sides of the small bowel, extending from 80 centimeters distal the ligament of Treitz to 40 centimeters proximal the ileocecal valve. Major arterial pulsations were palpable, and thrombosed veins were not seen. Intestinal resection (about 70 cm in length) followed by double barrel stoma and peritoneal lavage was performed. Macroscopically, the intestinal lesions were characterized by multiple small perforations in a paper-thin bowel variable length along the resected segments of small bowel, progressing to full thickness necrosis of the intestinal wall (Figs. 2 and 3). Then the sample was sent for histopathology; histopathologic examination of the sample showed, marked inflammatory infiltrate with diffuse crypt loss, submucosal venules thrombus and transmural inflammation necrosis (Figs. 4 and 5).

Fig. 2

Multiple small perforations showed by the blue arrows

Fig. 3

Some of the perforations in a paper-thin bowel are indicated by the hemostat

Fig. 4

Intense mixed inflammatory infiltrate with crypt loss and a submucosal venules luminal fibrin thrombus (arrow)

Fig. 5

Flattened mucosal surface with ulceration, marked crypt destruction and transmural inflammation and necrosis (arrow)

Postoperatively, the patient was started on Proton pump inhibitor (PPI), loperamide, and opioids to manage the high-output stoma. Ceftriaxone, metronidazole and unfractionated heparin were added. Subsequently, vital signs stabilized, urine output normalized, and fluid balance was restored. On his eighth postoperative day, the abdomen was explored aiming stoma closure meanwhile there was no additional perforations or ischemic change on the small bowel or colon. Subsequently an end-to-end ileo-jejunal anastomosis was performed. The patient had unremarkable recovery and was discharged improved after seventh day of hospital stay after stoma reversal (total of two weeks stay) with no perioperative complications. On subsequent follow-up at the general surgery referral clinic, the wound healed well and the patient did not have any other complaints.

Discussion

Disrupted intestinal blood flow can lead to bowel infarction, necrosis, and perforation. Ischemic colitis occurs when the mesenteric vessels are under perfused without a major vessel blockage [3]. Typically, it resolves after an acute colonic event. In contrast, ischemic enteritis, the equivalent condition in the small intestine, often requires surgical intervention. Due to its irreversible pathophysiology, ischemic enteritis is a rare disease with a poor prognosis [4]. There are two primary causes of IE described in the literature: occlusive and non-occlusive. The most common occlusive cause is acute arterial embolism of cardiac origin which is less likely in the absence of cardiac thrombose or atrial fibrillation, although the embolus in superior mesenteric artery embolism is usually installed in the middle colic artery, small intestinal branches are also sometimes occluded. Acute arterial thrombosis and mesenteric venous thrombosis account for 25% and 15% of occlusive cases, respectively [5, 6].

Non-occlusive IE affects approximately 25% of patients [6]. This condition is caused by inadequate blood flow due to various factors, including heart failure, acute myocardial infarction, arrhythmias, aortic dissection, sepsis, and diabetes mellitus. Prolonged vasoconstriction of the mesenteric vessels can reduce blood flow by up to 80%, exacerbating IE [2]. In another report several uncommon mesenteric vascular conditions have been linked to small intestinal perforation [7]. These include giant cell arteritis (even in its isolated form), Churg-Strauss syndrome (or allergic granulomatous angiitis) and other eosinophilic enteritis variants, Wegener’s granulomatosis (characterized by necrotizing vasculitis, granulomatous respiratory tract involvement, and glomerulonephritis), and Buerger’s disease with multiple small intestinal sites affected [7]. The jejunum is affected in 20% of patients, while the ileum is affected in 45–55% of patients. Thrombosis of the superior mesenteric artery or vein is often diagnosed without an identifiable cause. However, it can also be linked to conditions such as portal hypertension, sepsis, deficiencies in antithrombin III, protein C, or protein S, and long-term use of birth control pills [8, 9]. A case report presented on a 65-year-old male patient with multiple small bowel perforations caused by intestinal ischemia, similar to the presentation of our patient [2]. Another study revealed that acute ischemic small bowel enteritis can occur similarly to ischemic colitis [10]. This suggests that mesenteric ischemia can manifest as a clinical syndrome resembling ‘regional enteritis,’ as observed in our patient [11].

Initial symptoms in our patient, including acute abdominal pain and bloody diarrhea, aligned with those described in the literature [12]. Subsequently, the patient developed complications consistent with known outcomes of this condition: intestinal necrosis, peritonitis, sepsis, and ileus. Angiography is the gold standard for visualizing and diagnosing abnormalities in the mesenteric vessels. A further benefit is the ability to leave the catheter in place for direct administration of vasodilating agents. Recent studies have shown that CT scans can accurately diagnose approximately 85% of cases of bowel infarction, regardless of whether the cause is occlusive or non-occlusive [13]. Neither imaging study was performed in our case due to symptoms of peritonitis and accessibility issues. In our case, arrhythmia was considered unlikely due to a normal electrocardiogram and the absence of cardiac chamber thrombosis. In the absence of liver failure, disseminated intravascular coagulation (DIC), a history of proteinuria, anticoagulant use, or hemodialysis, acquired antithrombin deficiency was also considered less likely. However, regarding hereditary antithrombin deficiency, which occurs in 0.02-0.17% of the general population, antithrombin assays (activity or protein quantity) were not performed due to availability issues [14]. Even though there are no established diagnostic criteria for IE, the presence of palpable major arterial pulsations, the absence of grossly visible thrombosed veins, non-anatomic multiple focal ischemic perforations, and low ejection fraction with left ventricular diastolic dysfunction strongly suggest nonocclusive causes [15, 16]. Studies have shown that the histologic depth of IE varies from submucosal to transmural, depending on the severity of ischemia. The thrombosed venules observed in our case can be explained by the extent of inflammation [4, 17].

IE due to a superior mesenteric artery embolism requires immediate intervention with arteriotomy and embolectomy. If the superior mesenteric artery is thrombosed, an aorto-mesenteric bypass is indicated. Initial management of superior mesenteric vein thrombosis involves conservative treatment with high-dose heparin. While treatment guidelines for non-occlusive IE are limited, angiography with papaverine infusion has shown some efficacy. Unfortunately, in most cases, the diagnosis is made during laparotomy when patients present with advanced symptoms such as gangrene, perforation, or peritonitis. Our patient was no exception [2, 18].

Conclusion

Ischemic enteritis is a rare but life-threatening condition. This can lead to severe complications including hemorrhage, intestinal infraction, bowel perforation, peritonitis, septic shock, and ultimately, death. Early diagnosis and prompt medical or surgical intervention are essential for survival. Unfortunately, definitive diagnosis typically requires histopathologic examination. Given its serious nature and potential for rapid progression, ischemic enteritis should be considered in the differential diagnosis of acute abdominal pain, especially when accompanied by bloody or bloody tingled diarrhea.