以雙側嚴重腎積水表現的IgG4相關性後腹腔纖維化

鄧子麒、林友翔、楊佩珊、侯鎮邦、陳建綸、崔克宏、張慧朗

林口長庚醫院 外科部 泌尿科，長庚大學

A Igg4-related retroperitoneal fibrosis presenting with bilateral severe hydronephrosis

Tzu-Chi Teng, Yu-Hsiang Lin, Pei-Shan Yang, Chen-Pang Hou, Chien-Lun Chen, Ke-Hung Tsui, Phei-Lang Chang

Division of Urology, Department of Surgery, Chang Gung Memorial Hospital,

Chang Gung University, Linkou, Taiwan

Abstract:

A 67-year-old gentleman with chronic voiding difficulties was initially presented with acute urine retention. The patient denied having any headache, flank pain, dizziness, nausea, vomiting, diarrhea, constipation or abrupt body weight loss symptoms. The patient went to local medical doctor for first aid. Transrectal ultrasound of the prostate and renal ultrasound showed a severely enlarged prostate and bilateral hydronephrosis. Lab data showed impaired renal function. These results initially led local medical doctors to believe the patient was experiencing obstructive uropathy caused by an enlarged prostate. However, a fever developed with decreased urine output after Foley insertion. Due to this, the patient was transferred to our hospital for further evaluation. On digital examination, an elastic, enlarged prostate without palpable nodule was noted. Lab data revealed leukocytosis with elevated CRP levels and acute deterioration of renal function. Urinalysis showed bacteriuria. Urosepsis with undetermined obstructive uropathy was impressed. Non-contrast abdominal computed tomography was then arranged, which showed multiple paraaortic lymphadenopathies had externally compressed bilateral middle ureters, causing bilateral severe hydroureter and hydronephrosis. Lymphoma or metastases lymphadenopathies was impressed. Effective antibiotics and bilateral percutaneous nephrostomy insertion were arranged, which improved renal functions. Computed tomography guided biopsy for paraaortic lymphadenopathies was performed. Surprisingly, the pathology report showed IgG4-related retroperitoneal fibrosis. Oral steroid was prescribed and bilateral percutaneous nephrostomy was maintained. Laser prostatectomy was done for sustained urinary retention. Follow-up anterograde pyelography showed the bilateral middle ureter obstruction improving 2 months later. Afterwards, the bilateral percutaneous nephrostomy was then removed.