Xanthogranulomatous cholecystitis is a rare benign inflammatory disease of gallbladder that may be misdiagnosed as carcinoma of the gallbladder intraoperative or in pre-operative imaging. Intramural accumulation of lipid-laden macrophages and acute and chronic inflammatory cells is the hallmark of the disease. The xanthogranulomatous inflammation can be very severe and can spill over to the neighboring structures like liver, bowel and stomach resulting in dense adhesions, abscess formation, perforation, and fistulous communication with adjacent bowel [1-3]. Cholecysto-colic fistula is a rare and late complication of gallstones roughly found 1 in every 1,000 cholecystectomies.
Clinical featuresThe clinical features are variable and non-specific. Patients with cholecysto-colonic fistula often present with symptoms of acute cholecystitis and preoperative diagnostic tools often fail to show the fistula. Hence most cases it is an on table diagnosis.
ManagementTreatment involves closing the fistula and performing an open or laparoscopic cholecystectomy.
Juvenile xanthogranuloma (JXG) is a rare form of non-Langherans cell histiocytosis (non-LCH) observed almost exclusively in infants and young children. It is rarely systemic, involving extracutaneous sites, such as the liver, lungs, spleen, kidney, pancreas, bone or central nervous system. Systemic JXG may be associated with significant complications requiring aggressive medical or surgery care; especially, central nervous system lesions are difficult to treat and reported to be possibly fatal. Clinical presentation of JXG of central nervous system is not specific and is related to the involved site while magnetic resonance imaging examination remains the first choice for localizing the lesions. If no other system is involved, surgical excision could be sufficient.
K Belcadi*, Z Isfaoun, O EL-Athmani, I El-Abdallaoui, IN Ansari, M Lakhrissi, A Guindo, M El-Kababri, A Kili, L Hessissen, S Sefiani and M El-Khorassani
Published on: 15th May, 2024
Juvenile Xanthogranulomatosis (JXG) is a condition, characterized by a proliferation of histiocytes, primarily observed in infants and young children. Cutaneous manifestations appear as yellow-orange-brown papules or nodules, typically localized on the face, neck, and upper chest. While most lesions regress spontaneously, some may require intervention for aesthetic, diagnostic, or hemorrhagic reasons. A rare case of disseminated JXG in a child with hemophilia has been reported. In this patient with severe hemophilia A, cutaneous nodules appeared, some associated with bleeding requiring appropriate management. Treatment included the administration of factor VIII to prevent bleeding during surgical procedures and secondary prophylaxis, to control recurrent bleeding. The outcome was favorable with the disappearance of the cutaneous lesions without sequelae, under regular surveillance for both medical conditions. This case highlights the rare association between juvenile xanthogranulomatosis (JXG) and hemophilia, a combination that has never been documented in the medical literature. This association only impacts the management of JXG when the cutaneous lesions bleed and their excision becomes necessary.
Micaela Redivo, María del Pilar Mingheira, Federica Fernandez Long and Carlos Santiago Ruggeri*
Published on: 23rd August, 2024
Xanthogranulomatosis is an uncommon disease affecting non-Langerhans histiocytes. Laryngeal involvement is very rare.We describe the clinical case of an adult patient with disseminated xanthogranulomas affecting the supraglottic region of the larynx, leading to thickening and immobility of the epiglottis. The patient experienced inspiratory dyspnea on exertion. A transoral supraglottic epiglottectomy was performed, and a tracheostomy was subsequently required.
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