Three months later, the kidney graft volume partly decreased (17 cm, 770 cc) but ascites reappeared. Discussion We describe two cases of kidney graft lymphangiectasia responsible for persistent ascites and symptomatic nephromegaly revealed by atypical chronic pelvic pain and local mechanical complications: STING ligand-1 inguinoscrotal hydrocele (patient STING ligand-1 1) and bladder compression (patient 2). Renal lymphangiectasia is an extremely rare pathology. reported in the literature, exceptionally after kidney transplantation, 7 and there is currently only sparse data on the clinical presentation, pathophysiology, and therapeutic options in kidney transplant recipients. We present here two cases of kidney transplant lymphangiectasia revealed by persistent ascites and atypical abdominal pain, without STING ligand-1 kidney graft dysfunction. Case 1 A 34-year-old man was referred to our department for refractory ascites 10 years after first kidney transplantation for end-stage kidney disease secondary to idiopathic focal and segmental glomerulosclerosis. He had experienced a biopsy-proven acute subclinical humoral rejection revealed by the occurrence of donor-specific antigens 4 years after transplantation. Rejection was treated with corticosteroids, intravenous immunoglobulins, and immunoadsorption (10 sessions). The serum creatinine level remained stable at 0.9 mg/dl, as well as urinary albumin-to-creatinine ratio at 50 mg/g. He was then maintained on a combination of tacrolimus, mofetil mycophenolate, and steroids. Ascites gradually appeared 8 years after transplantation, leading to an increasing discomfort. There was no associated sign of peripheral edema. Cardiac function was normal and there was no nephrotic syndrome. Ascites was transudative, non-chylous, and without evidence for infectious or malignant disease. Abdominal MRI did not show liver structural abnormality and positron emission tomography scan did not find any evidence for cancer or lymphomatous disease. There was also no sign of retroperitoneal lymph nodes or fibrosis. A liver biopsy was also performed and did not reveal any significant abnormality. Given the hypothesis of mycophenolate-induced regenerative nodular hyperplasia, which may not be detected on biopsy findings, a conversion to mammalian target of rapamycin (mTOR) inhibitor (mTORi) was performed. Magnetic resonance urography (MR-urography) showed significant nephromegaly (13.7 cm, 603 cc) with multiple peripheral plurilobular fluid collections at the upper and lower poles of the kidney graft as well as extensive ascites responsible for inguinoscrotal hydrocele (Figures?1a and ?and1b).1b). A kidney graft biopsy was performed and revealed chronic active humoral rejection and significant interstitial edema, but no argument for post-transplantation lymphoproliferative disorder. Retrospectively, the transplanted kidney had normal appearance at the time of procurement with no cyst detected. However, 4 years before the recent episode of ascites (6 years after transplantation), an ultrasound scan was performed because of intermittent pain in the graft area: its size had already increased (13 cm) and a significant amount of fluid (4-mm-thick) outlined the graft, which argued for perirenal lymphangiectasia (Figure?2). This led us conclude to cortical and perirenal lymphangiomatosis. One year after mTORi conversion, the patient experienced better control of ascites and pain relief, but the kidney graft volume was still moderately increasing (14.3 cm, 654 cc). Regarding graft function, the serum creatinine level remained stable at 1.1 mg/dl. A prosthetic parietal surgery under the Lichtenstein procedure was also proposed for the treatment of inguinoscrotal hydrocele, but the patient preferred to decline the proposition. Open in a separate window STING ligand-1 Figure?1 MR-urography findings regarding patient 1 and patient?2. (a,b) Patient 1. (c,d) Patient 2. Both examinations revealed major nephromegaly with multiple plurilobular fluid collections of different morphological patterns: peripheral development at the upper and lower poles of the graft (patient 1: red arrows); parapyelic, juxtacapsular, and perihilar (patient 2: yellow arrows). The magnetic resonance urographies also showed local mechanical complications of kidney graft lymphangiectasia: large inguinoscrotal hydrocele (red star, b) and bladder compression (yellow stars, c and d). Open in another window Amount?2 Renal ultrasound. Significant nephromegaly is normally proven (13 cm) and a slim perirenal hypoechoic level (4.2 mm), suggestive of peripyelic lymphangiectasia and cysts. Case 2 STING ligand-1 A 35-year-old guy was described our Rabbit Polyclonal to NCAPG2 section for chronic pelvic discomfort connected with urinary symptoms 8 years after initial kidney transplantation for end-stage kidney disease supplementary to typical youth hemolytic and uremic symptoms. Five years after transplantation, he experienced a biopsy-proven subclinical severe humoral rejection (graft biopsy performed due to donor-specific antigen). As a result, he underwent cure.