Intra-Abdominal Xanthogranulomatosis Secondary to Postoperative Bile Leak

Abstract

Introduction: Xanthogranuloma is a rare benign tumor, macroscopically characterized by golden yellow nodules and microscopically as multicentric, mass-like accumulation of non-Langerhans lipid-laden histiocytes. The etiopathogenesis is not fully understood, however granulomatous reaction of histiocytes to an unknown stimulus is postulated. When xanthogranuloma affects multiple organs, it is called Erdheim-Chester disease, which occurs more commonly in men than women, with a peak incidence in the sixth decade of life. Here, we discuss a rare presentation of intra-abdominal xanthogranulomatosis in a patient caused due to postoperative bile leak. Case Report: A 71-year-old female patient with past medical history of hypertension, hyperlipidemia, and hypothyroidism was referred to our facility for persistent abdominal pain for past 6 months. The pain was associated with poor appetite and progressive loss of weight. On further questioning, it was revealed that she had undergone laparoscopic cholecystectomy for acute cholecystitis prior to the onset of these symptoms. Postoperative course was complicated by bile leak which was diagnosed by HIDA scan. Subsequently, she underwent ERCP with sphincterotomy with resolution of bile leak. No stents were placed. For the persistent abdominal pain, the patient underwent a CT scan of abdomen that showed a 1.7-cm nodule in the omentum surrounding the gastric antrum. In addition, she also had multiple peritoneal nodules surrounding the liver. To further delineate these lesions, she underwent MRI of the abdomen, which showed similar lesions suspicious for peritoneal carcinomatosis. The patient underwent CT-guided biopsy of the lesions. Pathology revealed mixed inflammatory cell infiltrate composed of histiocytes, lymphocytes, plasma cells, eosinophils, and giant cells suggestive of xanthogranulomatous inflammation. The giant cells contained bile pigment, which suggested that the most likely etiology was postoperative bile leak. Further, a diagnostic laparoscopy was done. Intraoperative findings included yellowish 1-2 cm masses in omentum near the gallbladder fossa and greater curvature of stomach. Surgical pathology confirmed the diagnosis of xanthogranulomatous with bile pigment deposits. Conclusions: Intra-abdominal xanthogranulomatosis is an extremely uncommon condition, the etiopathogenesis of which is not yet known. Given the presence of bile pigments in the histology, postoperative bile leak was the inciting factor for this patient.