
In a case report, a 35-year-old male with Erdheim-Chester disease (ECD), a rare multisystem disorder, underwent bilateral robotic upper tract reconstruction, marking the first such documented instance. The patient, initially presenting with renal complications and extra-genitourinary manifestations, opted for definitive repair using percutaneous nephrostomy tubes and trametinib. To bridge the gap, the challenging procedure involved ureteral dissection, partial nephrectomy, and ileal interposition. Despite postoperative complications, the patient's renal function improved, showcasing the viability of robotic ureterolysis and ureterocalycostomy for ECD-related upper tract reconstruction.
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