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IgG4-Related Disease Presenting as Gastric Outlet Obstruction in a 12-Year-Old: A Case Report and Literature Review
Sarra Aggoun, Nour Eddine Bouhadiba, Mansour Ali, Guy Brisseau, Saleem Mamoo
Sidra Medicine, Qatar
Introduction:
IgG4-related disease (IgG4-RD) is a rare, systemic fibroinflammatory condition characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells into various organs. While it affects middle-aged males, pediatric cases are increasingly recognized. Gastric outlet obstruction (GOO) due to IgG4-RD is exceptionally uncommon in children, posing significant diagnostic challenges.
Case presentation:
We report a 12-year-old male with a history of inguinal hernia and a family history of testicular cancer. He presented with a two-year history of progressive abdominal pain, loss of appetite, weight loss, chronic vomiting, malnutrition, and anemia, leading to failure to thrive. Imaging studies, including abdominal CT, revealed a significant duodenal mass causing GOO. Initial upper gastrointestinal endoscopy (OGD) was unsuccessful due to the mass's size. Subsequently, laparoscopy and laparotomy were performed; however, only a biopsy was obtained due to the mass's high vascularity. Intraoperative findings included duodenal perforation, prompting the decision to perform gastrojejunostomy with pyloric exclusion.
Histopathological examination of the biopsy specimen revealed fibrosis, plasma cell infiltration, and a characteristic "cartwheeling" pattern of IgG4-positive plasma cells, confirming the diagnosis of IgG4-RD. Postoperatively, the patient developed significantly elevated liver enzymes, and imaging revealed severe intrahepatic and extrahepatic bile duct dilatation. An external biliary drain was placed by interventional radiology, leading to subsequent improvement in liver function tests.
The rheumatology team initiated treatment with corticosteroids and Rituximab to manage the disease and minimize steroid use. As of the latest follow-up, the patient remains on steroids and Rituximab, with decreasing biliary output and plan is to remove the drain.
Conclusion:
This case underscores the importance of considering IgG4-RD in the differential diagnosis of pediatric Gastric Outlet Obstruction . Early recognition and appropriate steroids and immunosuppressive therapy can prevent irreversible organ damage and obviate the need for invasive procedures.