Concurrent Meckel’s Diverticulum and Sjögren Syndrome: A Case of Intestinal Obstruction

Introduction

Meckel’s diverticulum (MD) is a congenital diverticulum that contains all normal layers of the gastrointestinal wall [1,2]. It is proximal to the ileocecal valve by 7 to 200 cm and is about 0.4 to 11 cm long. The incomplete eradication of the omphalomesenteric duct is the main cause of MD. The omphalomesenteric duct’s major role is to provide nutrition to the fetus, as it forms an attachment between the yolk sac and the gut. The duct dissociates from the intestine at week 7 of gestation. Failed dissociation results in omphalomesenteric cysts, omphalomesenteric fistulas that drain to the umbilicus, leading to obstruction. MD refers to the absence of any more attachments [3]. Most MD cases are asymptomatic. Yet, in the adult population, it can present with intestinal obstruction in 35.6%, hemorrhage in 27.3%, and inflammation in 29.4% of cases. MD is more common in men than in women, with ratio of 4: 1 [2].

A study has shown that complicated MD presents with small bowel obstruction in 35.1% and gastrointestinal bleeding in 29.8% of patients [4]. Moreover, in a study done in Taiwan on the characteristics of MD, the presentations of MD were intestinal obstruction in 48%, acute appendicitis in 38%, and gastrointestinal bleeding in 23% of patients [5]. Sjögren syndrome is a rare systemic autoimmune disorder. The pathological theory is that lymphocyte-rich mononuclear cell foci infiltrate exocrine glandular tissue, and humoral factors, such as antibodies and cytokines, cause dysfunction to the exocrine glands, leading to diminished saliva and tear production and skin, tracheobronchial, and vaginal dryness. Sjögren syndrome can be associated with other autoimmune disorders, such as rheumatoid arthritis and systemic lupus erythematosus. Patients with Sjögren syndrome present with eye and mouth dryness from inflammation and pathology of the lacrimal and salivary glands. Extra-glandular involvement presents in 50% of patients in the joints, skin, lungs, gastrointestinal tract, nervous system, and kidneys [6]. Additionally, the chronic inflammation in Sjögren syndrome results in gastrointestinal tract dysmotility, thus mimicking mechanical obstruction, which is a rare complication [6]. This case study features a 25-year-old woman with a known case of Sjögren syndrome who presented with symptoms of small bowel obstruction that were later shown in laparoscopic exploration to be caused by MD.

Case Report

A 25-year-old woman with a known case of Sjögren Syndrome, and who was not on any medication, presented to the Emergency Department with abdominal pain and bilious vomiting on September 5, 2023.

The pain was generalized, mainly in the suprapubic region, accompanied by nausea with repeated episodes of bilious vomiting. The patient denied a history of fever, urinary symptoms, or alterations in bowel habits.

Her vital signs were stable, with a temperature of 36.6°C, blood pressure of 128/78 mmHg, heart rate of 80 beats per min, respiratory rate of 18 breaths per min, and SpO2 of 99.0%. On examination, she looked uncomfortable and pale and was dehydrated. She had concerns of intense abdominal pain. Her abdomen was soft and lax with generalized abdominal tenderness, but mainly in the right iliac fossa region.

After the completion of the medical history and physical examination, the laboratory investigations were sent, including a full blood count, liver function tests, and renal function tests, which were all within reference values. Abdominal radiography showed multiple air fluid levels and distended small bowel loops.

Subsequently, computed tomography (CT) with contrast was completed, initially showing right ovarian cyst/follicle of 3 cm, minimal adjacent free fluid, mild fluid in a polycystic ovary, ileal loops appearing slightly prominent in caliber, dilated small bowel loops, and ileus reactionary to the free fluid in the pelvis, which was most probably due to the ruptured ovarian cyst (Figure 1A, 1B).

The patient’s symptoms were primarily assumed to be linked to the ovarian cyst that was revealed on the CT scan. Subsequently, the team of obstetricians and gynecologists dismissed this possibility, following an ultrasound examination. The ultrasound revealed the presence of numerous dilated non-peristaltic small bowel loops, accompanied by hyperechoic fat planes and a slight accumulation of free fluid in the right iliac fossa and pouch of Douglas. These findings indicated the presence of ileus, rather than bowel, obstruction. Consequently, she was admitted to the General Surgery Department for comprehensive assessment and treatment.

At the outset, the patient received conservative management consisting of intravenous fluids, analgesic medication, and bowel rest. Nevertheless, her condition progressively deteriorated, with an increased frequency of vomiting. In response, a nasogastric tube was inserted. However, no marked improvement was achieved, and the intensity of her discomfort continued to escalate. On further review of the CT scan by a senior radiologist, there appeared a possible existence of a transition zone at the level of the ileum (Figure 1A, 1B). Subsequently, the patient was sent to the operating room for laparoscopic exploration, which showed an obstruction located approximately 1 m from the ileocecal valve in the ileum. The condition appeared to be due to MD (Figure 2). The MD was excised, and the sample was sent for pathological analysis. The following was found on macroscopic examination: small fragment of intestinal wall measured 3×1.2×1.2 cm (RPT ×2), sectioning revealed inked black margin, and sectioning was unremarkable. The following was found on microscopic examination: small bowel resection showed invagination of mucosa and submucosa within the wall, consistent with MD.

The patient recovered well from her surgery, was able to tolerate oral intake, and the pain and nausea improved dramatically. The patient was discharged home in a stable state. Two weeks later, a colonoscopy was done for the patient, which showed a normal study.

Discussion

Identifying MD is difficult because traditional imaging methods, including abdominal X-rays, ultrasounds, and CT scans, are often unsuccessful at precisely diagnosing the ailment [1]. Technetium-99m pertechnetate scanning, also known as the Meckel’s scan, is the primary diagnostic method for MD, although there is little study in this field. Although the Meckel’s scan is commonly used, research has indicated that it has low specificity in adult patients, thus low overall reliability in diagnosing MD [8]. A literature analysis conducted in 2018 focused on MD and included detailed information about its clinical characteristics, diagnostic techniques, and treatment options. The review found that technetium-99m pertechnetate scanning has a sensitivity of 62.5% and a specificity of 9% in adults [8].

Although laparoscopic exploration or laparotomy is commonly used as the main diagnostic method in many cases [1], published examples highlight the heterogeneity and complexity of detecting MD with imaging tests. For example, the cases of a 25-year-old man, 18-year-old patient, and 44-year-old woman demonstrate the difficulties in diagnosing MD. These cases show discrepancies between the results of imaging tests and what is found during surgery. This emphasizes the need for surgical exploration to accurately diagnose and treat MD [9–11].

MD can be identified by imaging techniques in some rare circumstances, such as when a 17-year-old boy experienced sudden pain in the lower abdomen that lasted for 1 day. A CT scan revealed the existence of air pockets in the abdominal cavity, specifically in the front and middle areas, as well as aberrant thickening of the ileal loops, indicating a possibility of MD [12]. Similarly, in another instance, a grown patient who had pain in the navel area underwent a Meckel’s scan, which showed increased activity in the jejunum beyond that location. This finding led to the diagnosis of MD [13]. Although these diagnostic approaches may have lower specificity for diagnosing MD, they can nevertheless be beneficial in some situations for facilitating prompt diagnosis and treatment.

A case report and literature review in 2023 of a 36-year-old pregnant woman with a known case of Sjögren syndrome, gravida 3 para 0 at 29 weeks’ gestation, describes that the patient presented with a 1-day history of acute abdominal pain, nausea, and vomiting. Appropriate investigations were done, including CT of the abdomen, which showed a dilated small bowel, with the widest diameter of 45 mm, suggestive of small bowel obstruction; however, no anatomical lesions leading to obstruction were identified on CT imaging. Therefore, they suspected acute intestinal pseudo-obstruction secondary to underlying Sjögren syndrome. The patient was treated with immunosuppressive therapy and supportive treatment [7]. Similarly, our patient presented with the same symptoms of obstruction; however, in the CT, mechanical obstruction was identified, making it an intestinal obstruction not pseudo-obstruction. Nonetheless, studies have shown that gastric and liver biopsies taken from patients with Sjögren syndrome have revealed chronic inflammation, aggregates of lymphocytes and plasma cells [14]. In our case, the pathology report showed invagination of mucosa and submucosa within the wall, consistent with MD. Therefore, findings were not consistent with Sjögren syndrome, which emphasizes the fact that MD and Sjögren syndrome are distinct entities.

To summarize, MD must be included in the list of possible causes of sudden abdominal pain in adults, as it can commonly present with intestinal obstruction. The literature we found emphasized the significance of maintaining a vigilant attitude toward MD, particularly when traditional diagnostic techniques do not provide conclusive outcomes. Prompt identification and diagnosis of MD is essential for guaranteeing timely intervention and proper treatment, highlighting the importance of thorough evaluation in instances of sudden stomach symptoms in adult individuals.

Conclusions

It is important to consider MD as a possible cause for sudden abdominal pain in adults, as it can have catastrophic consequences. Furthermore, upon searching for hypotheses to support the relationship between MD and Sjögren syndrome, no clear link between the two was found. MD and Sjögren syndrome are distinct entities. However, both conditions can present with symptoms of intestinal obstruction.