Eosinophilic Gastroenteritis Accompanied with Partial Midgut Malrotation in an Adult: A Case Report

Abstract

Patient: Male, 25-year-old

Final Diagnosis: Eosinophilic gastroenteritis

Symptoms: Abdominal pain

Clinical Procedure: —

Specialty: Allergology • Gastroenterology and Hepatology

Objective: Rare disease

Background

Eosinophilic gastroenteritis (EGE) is a rare inflammatory condition characterized by eosinophilic infiltration of the gastrointestinal tract, often presenting with non-specific symptoms such as abdominal pain, diarrhea, and ascites. Midgut malrotation is a congenital anatomical anomaly typically diagnosed in infancy but may remain undetected until adulthood. The coexistence of EGE and partial midgut malrotation in an adult is extremely rare and has not been previously reported.

Case Report

We report the case of a 25-year-old man who presented with persistent epigastric cramping pain and abdominal distension. Laboratory evaluation revealed marked eosinophilia, elevated C-reactive protein, and high total IgE levels. Abdominal contrast-enhanced CT showed duodenal wall thickening, mucosal edema, and abnormal positioning of the horizontal duodenum and proximal jejunum, suggestive of partial midgut malrotation. Endoscopy revealed congested, swollen mucosa with patchy erosions and narrowing at the duodenal bulb-descending junction. Histopathology confirmed eosinophilic infiltration in the duodenal and gastric mucosa. The patient was diagnosed with eosinophilic gastroenteritis accompanied by partial midgut malrotation. He was treated with corticosteroids and supportive care, resulting in significant clinical and hematologic improvement.

Conclusions

This case highlights a rare but important diagnostic consideration involving the coexistence of inflammatory and congenital anatomical gastrointestinal disorders. Clinicians should maintain a high level of suspicion for structural anomalies in adults presenting with unexplained gastrointestinal symptoms. Early multidisciplinary evaluation is essential for accurate diagnosis and appropriate management.

Introduction

Eosinophilic gastroenteritis (EGE) has been documented in the medical literature since the mid-20^th century [1]. It is an uncommon and clinically diverse inflammatory condition marked by eosinophilic infiltration within the layers of the gastrointestinal (GI) tract, in the absence of identifiable secondary triggers such as parasitic infections, adverse drug reactions, or systemic illnesses. The clinical presentation varies widely depending on the depth of infiltration and whether it involves the mucosa, muscularis, or serosa, resulting in non-specific symptoms like abdominal discomfort, diarrhea, ascites, or even intestinal obstruction. These vague symptoms frequently contribute to delays in diagnosis or initial misinterpretation [2,3]. A thorough workup, including peripheral eosinophil counts, endoscopic findings, and histopathological confirmation, is essential for accurate identification [4–6].

Midgut malrotation, on the other hand, is a congenital anatomical defect arising from incomplete intestinal rotation and fixation during embryonic development. While typically diagnosed during infancy [7], some cases remain silent and may not be recognized until adulthood. In such adult cases, patients may experience intermittent abdominal pain, signs of subclinical obstruction, or the anomaly can be incidentally detected through imaging [8].

To our knowledge, the co-occurrence of EGE and partial midgut malrotation in an adult patient has not been previously documented. In this report, we present the case of a 25-year-old man with chronic epigastric cramping, peripheral eosinophilia, and imaging findings indicative of EGE and partial midgut malrotation. This case brings attention to the diagnostic challenges posed by overlapping inflammatory and structural GI disorders and emphasizes the necessity of multidisciplinary collaboration when managing unexplained gastrointestinal symptoms in adults.

Case Report

A 25-year-old man presented with persistent cramping pain in the epigastric region, primarily localized below the xiphoid process, accompanied by abdominal distension without nausea or vomiting. An emergency non-contrast abdominal CT revealed duodenal wall edema and torsion.

Initial laboratory tests (Table 1) showed marked elevations in white blood cell count, eosinophils (absolute and relative), C-reactive protein, and immunoglobulin E (IgE, total and allergen-specific). Dynamic contrast-enhanced CT of the upper and lower abdomen revealed a tortuous duodenum with abnormal rightward displacement of its horizontal segment, the proximal jejunum located in the right upper quadrant, and diffuse thickening of the gastrointestinal wall, most prominent in the duodenum. Submucosal edema was noted, along with blurring of the peritoneal fat planes, a small amount of ascites, bilateral pleural effusions, and multiple small lymph nodes in the peritoneal and retroperitoneal spaces (Figure 1A–1D). Esophagogastroduodenoscopy revealed congested cardia mucosa with patchy erosions and ulcers, yellowish gastric fluid, edematous antral mucosa with irregular pyloric morphology, and significant swelling with luminal narrowing at the duodenal bulb-descending junction. Biopsies were obtained from the cardia, antrum, and duodenum (Figure 2A–2C). Histopathology showed moderate chronic inflammation in the duodenum with cystic gland dilation and stromal infiltration by lymphocytes, plasma cells, and abundant eosinophils. The antrum revealed chronic non-atrophic gastritis with prominent eosinophilic infiltration (Figure 2D, 2E).

Table 1.

Aggregated hematological and biochemical parameters.

Item	Apr 15	Apr 19	Apr 21	Apr 22	Apr 25	Apr 28	Apr 30	May 04	Reference range	Unit
White blood cell count (WBC)	12.39 ↑	12.09 ↑	13.85 ↑			17.42 ↑	18.35 ↑	11.43 ↑	3.5–9.5	109/L
Lymphocyte percentage	25.8	19.5 ↓	16.1 ↓			13.5 ↓	16.5 ↓	24.7	20–50	%
Monocyte percentage	3.7	3.8	3.3			3.0	2.8 ↑	4.6	3–10	%
Eosinophil percentage	22.2 ↑	23.2 ↑	26.0 ↑			33.8 ↑	41.5 ↑	5.1	0.4–8.0	%
Eosinophil count	2.74 ↑	2.80 ↑	3.6 ↑			5.89 ↑	7.62 ↑	0.58 ↑	0.02–0.52	109/L
Basophil percentage	0.3	0.2	0.2			1.3 ↑	0.4	0.1	0–1.0	%
Basophil count	0.04	0.02	0.03			0.23 ↑	0.07	0.01	0–0.06	109/L
Neutrophil percentage	48.0	53.3	54.4			48.4	38.8 ↓	65.5	40–75	%
Neutrophil count	5.93	6.45 ↑	7.53 ↑			8.43 ↑	7.12 ↑	7.49 ↑	1.8–6.3	109/L
Platelet count	336	363 ↑	367 ↑			373 ↑	371 ↑	367 ↑	125–350	109/L
Platelet crit (PCT)	0.33 ↑	0.33 ↑	0.37 ↑			0.41 ↑	0.39 ↑	0.39 ↑	0.09–0.30	%
CRP	1.96	18.31 ↑	7.89 ↑			12.62 ↑	5.87 ↑		0–5	mg/L
Amylase	60.0								30–110	U/L
D-dimer	2.65 ↑			2.46 ↑		5.78 ↑			0–0.5	μg/ml
Total bilirubin		18.00							3–22	μmol/L
Direct bilirubin		5.70 ↑							0–5	μmol/L
Indirect bilirubin		12.30							0–19	μmol/L
Aspartate aminotransferase (AST)		16.00 ↓							17–59	U/L
Alanine aminotransferase (ALT)		12.00							0–50	U/L
Erythrocyte sedimentation rate (ESR)					2.0				0–15	mm/1h
Immunoglobulin A (IgA)						1.14			1.00–4.20	g/L
Immunoglobulin G (IgG)						7.28 ↓			8.60–17.40	g/L
Immunoglobulin M (IgM)						0.76			0.30–2.20	g/L
Immunoglobulin E (IgE)						1102.00 ↑			0–100	g/L
Procalcitonin (PCT)						0.07 ↑			<0.05	μg/L
Total allergen-specific IgE						744.23 ↑			0–100	KU/L
Dermatophagoides farinae d2-specific IgE						0.53 ↑			<0.35	KU/L
Cat dander (e1) allergen-specific IgE						0.19			<0.35	KU/L
Dog dander (e5) allergen-specific IgE						0.16			<0.35	KU/L
Aspergillus fumigatus (m3) allergen-specific IgE						0.13			<0.35	KU/L
Egg white (f1) allergen-specific IgE						0.23			<0.35	KU/L
Cow’s milk (f2) allergen-specific IgE						0.13			<0.35	KU/L
Peanut (f13) allergen-specific IgE						0.14			<0.35	KU/L
Fish (Cod) (f3) allergen-specific IgE						0.11			<0.35	KU/L
Shrimp (f24) allergen-specific IgE						0.22			<0.35	KU/L

^*The administration of methylprednisolone was initiated on April 28^th.

Figure 1.

Enhanced CT of the upper abdomen.

(A) Diffuse and significant thickening of the gastric wall at the cardia with mucosal edema; (B) Diffuse and significant thickening of the gastric wall at the antrum near the pylorus, with mucosal edema; (C) The superior mesenteric artery (red arrow) and superior mesenteric vein (blue arrow) are nearly perpendicular in orientation. The horizontal portion of the duodenum is twisted toward the right side, and the duodenojejunal junction is located to the right of the midline, suggestive of midgut malrotation; (D) The duodenum is tortuous with marked wall thickening and surrounding blurred fat planes, indicating possible exudation.

Figure 2.

Esophagogastroduodenoscopy (EGD) and pathological findings of biopsy.

(A) Congested mucosa at the gastric cardia with patchy erosions and ulcerative lesions; (B) Roughened, congested, and edematous mucosa in the antrum with pyloric deformity; (C) Edematous mucosa in the duodenum, with marked swelling at the bulb-descending junction and luminal narrowing; (D) Eosinophilic infiltration in the gastric antrum; (E) Eosinophilic infiltration in the duodenum.

Based on clinical, radiological, and pathological findings, a diagnosis of EGE with partial midgut malrotation was made. After informed consent, the patient received methylprednisolone 40 mg (Qd) along with acid suppression, mucosal protection, and calcium supplementation. After 7 days of treatment, a follow-up complete blood count showed a decrease in eosinophil count, and the patient’s abdominal pain and distension were significantly relieved. Intravenous methylprednisolone was then discontinued, and the patient was discharged with oral medications including methylprednisolone (40 mg/d), glutamine capsules, calcium carbonate with vitamin D3 tablets, rabeprazole enteric-coated capsules, and ebastine tablets, with a follow-up scheduled in 14 days.

Discussion

This case describes an adult patient with the rare co-occurrence of EGE and partial midgut malrotation. Based on the clinical examination results, EGE was considered the primary cause of the patient’s series of clinical manifestations, while the partial midgut malrotation was an incidental finding. The significant symptom relief after methylprednisolone treatment supports this conclusion. However, the midgut malrotation contributed to the presence of highly distinct clinical and radiological features. To our knowledge, there has been no previous report of the coexistence of these 2 conditions in an adult, making this the first documented case of its kind. This rare association has important clinical implications and research value.

EGE is a rare, chronic inflammatory disorder of the GI tract characterized by eosinophilic infiltration of 1 or more layers of the GI wall. The etiology remains unclear but may involve allergic mechanisms, autoimmune responses, or impairment of the intestinal mucosal barrier [9,10]. The clinical manifestations of EGE are often non-specific, including abdominal pain, bloating, nausea, and ascites, and can mimic other GI diseases, which often leads to diagnostic delay. In this case, the patient presented with diffuse thickening and mucosal edema of the stomach and duodenum, along with abdominal pain, distension, and ascites. Laboratory testing revealed peripheral eosinophilia, and histopathology from endoscopic biopsies confirmed eosinophilic infiltration of the GI mucosa, establishing the diagnosis. Unfortunately, due to limitations in clinical resources and the patient’s financial constraints, no further analysis of the ascites or lymphadenopathy was performed, making it impossible to fully determine the subtype. Since the edema was mainly confined to the stomach and duodenum, with minimal involvement of the colon, colonoscopy was not conducted. The patient’s case information therefore requires further completion.

A notable finding in this case was the concurrent presence of partial midgut malrotation, with imaging showing the horizontal part of the duodenum abnormally rotated toward the right abdomen and the proximal jejunum located in the right upper quadrant. The junction between the duodenum and jejunum was displaced to the right of the midline, consistent with malposition of the bowel due to abnormal intestinal rotation and fixation. While midgut malrotation is typically diagnosed in infancy, adult cases are rare and usually present with recurrent abdominal pain, partial obstruction, or other non-specific GI symptoms [8,11]. This case highlights the importance of considering congenital anatomical abnormalities in adult patients presenting with unexplained obstructive symptoms. Furthermore, the intestinal wall edema caused by EGE may exacerbate functional narrowing of already abnormally positioned bowel segments, potentially aggravating obstructive symptoms. A synergistic pathophysiological interaction between the 2 conditions may thus exist.

Regarding complications, EGE may be associated with ascites, bowel obstruction, GI bleeding, or protein-losing enteropathy. Severe cases can lead to perforation or malnutrition. On the other hand, midgut malrotation may result in intermittent subacute obstruction, volvulus, or compromised mesenteric blood flow. The coexistence of these 2 disorders may contribute to more complex clinical presentations and management challenges.

In terms of treatment, corticosteroids remain the first-line therapy for EGE [12], especially in moderate to severe cases, and can produce a rapid clinical response. In this case, the patient showed significant symptom relief following steroid treatment, further supporting the EGE diagnosis. Concurrently, an elimination diet is equally important in managing this condition. In this case, while allergen-specific IgE testing for multiple foods showed no significant abnormalities, the potential for certain foods to exacerbate symptoms could not be definitively ruled out. Therefore, the patient was placed on a liquid diet and advised to empirically eliminate dairy, seafood, nuts, and other potentially aggravating foods, in addition to avoiding irritating and greasy foods. Surgical intervention is generally recommended for midgut malrotation if volvulus or obstruction is severe. If the patient develops signs of peritonitis, systemic toxicity, or shock, imaging findings suggestive of intestinal ischemic necrosis (such as pneumatosis of the bowel wall, decreased or absent bowel wall enhancement), or hemoperitoneum, or if prolonged conservative treatment proves ineffective, surgical intervention should be considered. In this case, the patient did not present with any indications for surgical intervention, and his general condition improved significantly after glucocorticoid therapy; therefore, no surgical treatment was performed.

Regrettably, a review of the patient’ s diagnostic course revealed delays in reaching a definitive diagnosis of EGE. He initially presented to the emergency department with abdominal pain and underwent CT imaging, which suggested duodenal malposition and raised the concern for bowel torsion. As a result, he was transferred to surgery. It was only after more than 10 days of evaluation and management that the diagnosis of EGE was established by gastroenterologists. This underscores the diagnostic complexity of rare diseases like EGE and the crucial need for interdisciplinary collaboration across gastroenterology, radiology, pathology, and surgery in similar cases.

Conclusions

We report a case with a unique presentation of EGE coexisting with partial midgut malrotation in an adult patient. It highlights the necessity of considering inflammatory and structural abnormalities in patients with unexplained GI symptoms. The case also underscores the importance of multidisciplinary teamwork in improving diagnostic accuracy and patient outcomes in rare and complex presentations.

Abbreviations

EGE

eosinophilic gastroenteritis

EGD

esophagogastroduodenoscopy

GI

gastrointestinal