Pediatric Crohn Disease Presenting as Isolated Acute Upper-GI Bleed: A Case Report

Introduction

Crohn’s disease (CD) is a chronic inflammatory bowel disease (IBD) characterized by skip lesions of transmural inflammation that can affect any segment of the gastrointestinal (GI) tract, from mouth to anus. The worldwide incidence of IBD is rising in all age groups, but especially among young children and adolescents, with approximately 25% of all new cases diagnosed in children under the age of 18 years, with the highest incidence of new IBD diagnosis occurring in people aged 15–29 years old [1,2]. Common presenting symptoms of CD include abdominal pain, diarrhea, fatigue, weight loss, and rectal bleeding [3,4]. However, other symptoms, both intestinal and extraintestinal, have been reported as the initial presentation of CD, but much more rarely. These include peripheral arthritis, uveitis, erythema nodosum, and ankylosing spondylitis [5]. In this case report, we describe the unusual case of a child who presented with an isolated, life-threatening upper-GI bleed as the initial symptom prior to the diagnosis of CD. He denied any other symptoms prior to or at presentation.

Case Report

A previously healthy 9-year-old boy was transferred to our ED from an outside hospital (OSH) with acute-onset hematemesis described as bright red with large clots. He had 1 episode of hematemesis at home and a second episode at the OSH. Although laboratory workup was done at the OSH, results were not sent with him. A dose of proton pump inhibitor (PPI) and ondansetron were given in the OSH prior to transfer. On arrival to our ED, workup with complete blood count (CBC), complete metabolic profile, and coagulation profile was performed. His hemoglobin was 11.0 g/dL (reference range 10.7–13.4 g/dL). His history was negative for non-steroidal anti-inflammatory drug (NSAID) use. Further, his physical exam and other workup were reassuring with the exception for a leukocytosis; white blood cell count of 18.27×103/uL (reference range 4.31–11×103/uL), slight hypoalbuminemia: 3.5 g/dL (4.1–4.8 g/dL), and mild hypocalcemia: corrected calcium level of 8.8 mg/dL (reference range 9.2–10.5 mg/dL). The gastrointestinal (GI) team was consulted over the phone. Given his stable vital signs, reassuring workup, and tolerance of oral liquids in our ED with no further episodes of hematemesis, a decision was made to discharge him home on a PPI with a close follow-up scheduled in the GI clinic within 2 weeks. He missed his GI clinic follow-up and 3 weeks later presented to the ED again with large-volume, bright red hematemesis. At that presentation, he was hemo-dynamically unstable (tachycardic and hypotensive) and his vital signs remained unstable despite multiple fluid boluses. Labs were notable for leukocytosis: 21.49×103/uL, hypokalemia: potassium: 2.8 mmol/L (reference range: 3.4–4.7 mmol/L), hypocalcemia: corrected calcium 8.1 mg/dL, acidosis: pH 7.23 (reference: 7.31–7.41), and anemia (hemoglobin 9.1 g/dL). He also had 2 large episodes of hematemesis in the ED. He received 2 units of packed red blood cells in the ED and subsequently underwent emergency esophagogastroduodenoscopy (EGD). At endoscopy, gastritis with a single oozing gastric ulcer with a visible vessel was identified in the fundus (Figure 1A). This was injected and clipped, with good visible homeostasis (Figure 1B). A few nonbleeding gastric and duodenal bulb ulcers were also visualized, with no esophageal or gastric varices. Biopsies obtained from the stomach and duodenum demonstrated granulomatous inflammation of the gastric antrum (Figure 2A) and duodenum (Figure 2C). Stains were negative for H. pylori, acid-fast bacilli, and fungal organisms. He remained stable after intervention, without any further bleeding episodes. Other labs performed during the hospitalization revealed vitamin D deficiency, hypoalbuminemia, elevated inflammatory markers (erythrocyte sedimentation rate (ESR) 30 mm/hr (reference range 0–15 mm/hr), C-reactive protein (CRP): 0.56 mg/dL (reference range 0.00–0.50 mg/dL), and fecal calprotectin (109µg/g (reference range <50 mcg/g). Evaluation performed by the infectious disease and rheumatology teams were reassuring against any infectious or rheumatologic process. Computed tomography (CT) at presentation showed thickening of the gastric mucosa with a focal peripherally enhancing lesion in the fundus of the stomach (Figure 3A, 3B), and wall thickening of the terminal ileum, without any significant surrounding inflammation (Figure 3C). Given the biopsy results, an ileocolonoscopy was performed, which revealed nonbleeding ulcers with edema in the left colon and ulcers with exu-dates in the terminal ileum (TI) (Figure 1C). Histological examination revealed granulomas in the left colon and TI biopsies (Figure 2B). Infliximab was started prior to discharge and the PPI was continued. He received 5 mg/kg of infliximab during hospitalization (first induction dose). However, the dose was increased to 10 mg/kg Q 4 weeks to achieve a therapeutic drug trough of >5. He has completed infliximab induction, is on infliximab maintenance infusions, and is currently in clinical remission. His inflammatory markers showed CRP has normalized since starting therapy, and ESR has slowly normalized within 2 months of starting therapy. He has not had any further episodes of bleeding.

Discussion

CD is a worldwide disease that is rapidly increasing in incidence, particularly in newly developed/industrializing nations. CD is associated with chronic intestinal inflammation and can lead to complications necessitating hospitalizations and sometimes requiring surgical interventions. Early detection of this disease has been shown to result in better outcomes, including fewer surgeries and decreased morbidity [6]. Furthermore, the relatively recent widespread adoption of biologics and other advanced medical therapies in the treatment of CD has resulted in decreased surgical resection rates among CD patients and a paradigm shift toward more conservative treatment, especially among those who do not experience significant diagnostic delays [7,8].

Early detection is especially important in children, for whom a delay in diagnosis and treatment has been associated with higher rates of strictures, intestinal fistulas, and age-adjusted growth delay [9].

Conclusions

To the best of our knowledge, this is the first report of CD with gastroduodenal and ileocolonic involvement presenting with an isolated acute upper-GI bleed in a child. Surprisingly, our patient had no other symptoms and was gaining weight and growing appropriately at the time of presentation. It is crucial for providers to be aware of atypical presentations of IBD and maintain a high index of suspicion when treating children and adolescents with GI problems.