A unique case of gastrointestinal bleeding with splenomegaly

Abstract

We present the case of a 61-year-old man with a 30-year history of iron-deficiency anemia and recurrent gastrointestinal bleeding, accompanied by splenomegaly and hypersplenism. Despite multiple endoscopy and imaging examinations, the source of bleeding remained elusive. The patient ultimately underwent exploratory laparotomy with splenectomy, which revealed a Meckel’s diverticulum with stale hemorrhage, along with congestive changes in the spleen. Following surgery, the patient experienced a complete resolution of symptoms with normalization of hematological parameters. This case highlights Meckel’s diverticulum as a rare cause of obscure gastrointestinal bleeding, with the unique presentation of splenomegaly due to extramedullary hematopoiesis.

Introduction

Obscure gastrointestinal bleeding (OGIB) is defined as persistent or recurrent bleeding originating from the gastrointestinal (GI) tract that remains unexplained despite comprehensive initial evaluation. ¹ Most cases of OGIB are attributed to lesions in the small bowel, which are challenging to detect using conventional endoscopic and radiological methods.

In cases of OGIB, Meckel’s diverticulum (MD) should be considered as an important differential diagnosis. Bleeding from MD is typically due to ectopic gastric or pancreatic mucosa within the diverticulum, which can cause ulceration of the adjacent intestinal mucosa. ² MD is the most common congenital anomaly of the GI tract, with an estimated prevalence ranging from 0.3% to 4% in the general population. ³ Although it is typically asymptomatic and often discovered incidentally, MD can present with a range of clinical manifestations in adults, including GI bleeding (GIB), anemia, intestinal obstruction, inflammation, and abdominal pain. ⁴ Only about 16% of cases may present clinical manifestations, with GIB being the most frequent one. The bleeding can range from occult blood loss to life-threatening hemorrhage. However, nonspecific symptoms and negative findings of routine endoscopic and radiological evaluations frequently lead to delayed or missed diagnosis. ⁵

Splenomegaly, although well-recognized in conditions such as portal hypertension, hematologic malignancies, autoimmune diseases, and infectious diseases, is extremely rare in patients with MD. Here, we report a unique case: a 61-year-old man with recurrent OGIB, severe iron-deficiency anemia, and splenomegaly, who was ultimately diagnosed with MD complicated by hypersplenism.

Case report

A 61-year-old man presented with iron-deficiency anemia and recurrent GIB spanning over 30 years. As the anemia progressively worsened and repeated episodes of significant hematochezia began to severely affect his quality of life, the patient finally sought medical attention at our hospital. During his adolescence, he suffered from intermittent abdominal pain and fatigue. Subsequently, iron-deficiency anemia, thrombopenia, and splenomegaly were incidentally discovered during a routine medical examination. Throughout follow-up, he exhibited recurrent positive fecal occult blood (FOB) tests and experienced two episodes of hematochezia, with hemoglobin levels dropping as low as 3 g/dL (normal range: 13–17.5 g/dL). Although his hemoglobin levels temporarily increased after blood transfusions and iron supplementation, they consistently fell when treatment was discontinued. Repeated gastroduodenoscopy, colonoscopy, and double contrast radiography failed to identify any source of GI hemorrhage. The patient denied any history of alcohol consumption or drug use. On admission, a physical examination revealed pale skin and an enlarged spleen. Laboratory tests showed significantly decreased hemoglobin at 5.8 g/dL, reduced platelet count at 71 × 10⁹/L (normal range: 100–300 × 10⁹/L), and leukocyte count at 1.68 × 10⁹/L (normal range: 3.5–9.5 × 10⁹/L) with normal liver and renal function. Bone marrow examination revealed iron-deficiency and enhanced myeloproliferative activity. Computed tomography enterography showed thickened and enhanced small intestinal walls in the left middle abdomen (Figure 1(a), white arrow), along with splenomegaly and dilated portal and splenic veins (Figure 1(b), white arrow and red arrow). No GI hemorrhage lesions were found by capsule endoscopy. To address hypersplenism and explore the source of bleeding, the patient finally agreed to surgical diagnosis and treatment. During the operation, we observed congestive splenomegaly with tortuous, clustered vessels at the splenic hilum. The vessels surrounding the gastric cardia and fundus were dilated and tortuous. Dense adhesions were noted between the pancreatic tail and the diaphragm at the splenic hilum. The gallbladder was enlarged with edematous walls, containing multiple palpable stones, the largest measuring ~1.0 cm in diameter. About 30 cm proximal to the ileocecal valve, a 3 × 4 cm ileal diverticulum was identified, with the adjacent intestinal lumen containing dark fluid. The intestine proximal to the diverticulum was dilated and edematous, while the distal segment collapsed. Accordingly, we performed a splenectomy, devascularization around the cardia and gastric fundus, cholecystectomy, bowel resection with anastomosis, diaphragm repair, and repair of the pancreatic body and tail. The procedure was successful, with an estimated blood loss of ~200 mL. Histopathological examination of the resected spleen showed evidence of congestion, while the resected bowel revealed MD (Figure 1(c) and (d)). The patient fully recovered and was discharged without complications 5 days after surgery. During follow-up, he experienced no recurrence of hematochezia, and FOB tests returned negative. His hemoglobin, platelet, and leukocyte counts gradually normalized.

Figure 1.

(a) Computed tomography enterography showed thickened and strengthened small intestine in the left middle abdomen (white arrow). (b) Computed tomography enterography showed splenomegaly with expanded portal and splenic vein (white arrow and red arrow). (c) Hematoxylin and Eosin staining of the resected spleen demonstrated congestive changes. (d) Postoperative pathology confirmed the presence of MD.

Discussion

MD, resulting from incomplete closure of the omphalomesenteric duct, is the most common congenital malformation of the small intestine, affecting about 2% general population. ⁶ Although generally asymptomatic and discovered fortuitously, MD can present with complex clinical manifestations and individual variations in adults, leading to frequent misdiagnosis or delayed diagnosis. Approximately 10%–60% of symptomatic patients present with GIB, which may vary from a positive FOB test to life-threatening hematochezia. ⁷ Despite its rarity, MD should not be overlooked as a potential cause of obscure GIB, especially in patients with unexplained iron-deficiency anemia or positive FOB patients when routine endoscopic evaluations yield negative findings. Various diagnostic modalities are available for MD diagnosis, but they each come with inherent limitations. In our case, the patient had a 30-year history of recurrent GIB with no identifiable source despite multiple gastroduodenoscopy, colonoscopy, capsule endoscopy, and imaging studies. The noninvasive methods failed to locate the bleeding. When noninvasive methods fail, a laparoscopic approach can be considered as a final diagnostic step. According to the literature, laparoscopy offers a safe, direct, and efficient way to examine the entire intestinal serosa, mesentery, and small intestine in detail. Its dual role in both diagnosis and therapy is widely acknowledged.^8,9 Moreover, this patient presented with splenomegaly and hypersplenism. The patient’s pancytopenia requires long-term medication and repeated blood transfusions to improve the anemic condition, which has seriously affected the patient’s quality of life. Therefore, there is a clear indication for splenectomy. Based on the above considerations, we ultimately opted for laparotomy rather than laparoscopic surgery.

Actually, there are very few discussing the association between splenomegaly and MD. One such instance, reported by Mirpour et al., described a 16-year-old boy with intermittent lower GIB who was referred for a radionuclide Meckelogram. Interestingly, they incidentally detected focal tracer accumulation in the left side of the abdomen, which was subsequently confirmed as splenomegaly secondary to non-cirrhotic portal fibrosis. Their study demonstrated that Meckel scans can provide valuable diagnostic information beyond their primary purpose of detecting ectopic gastric tissue. ¹⁰ Notably, even current guidelines for diverticular disease have not mentioned splenomegaly as an associated sign.^11,12

In this study, we identified that an adult MD patient may manifest iron-deficiency anemia, splenomegaly, and hypersplenism, caused by extramedullary hematopoiesis resulting from long-term recurrent hemorrhagic anemia. To the best of our knowledge, no similar cases have been reported previously.

Conclusion

This case highlights the potential association of splenomegaly and hypersplenism with MD, offering a novel perspective for clinical differential diagnosis. Early recognition of this uncommon presentation and timely surgical intervention can significantly improve patient outcomes and long-term prognosis.