Twenty-two patients with bulky, metastatic germ cell tumors were treated with cyclophosphamide, vinblastine, dactinomycin, bleomycin, and cisplatin (VAB) alternating with VP-16 and vincristine (VV). The overall complete response rate after chemotherapy with or without surgical excision of residual tissue was 63% (14 of 22 patients) with a median survival of 46 months. Five patients are dead of disease progression and three are alive beyond 3 years with only residual radiographic abnormalities. A retrospective analysis of prognostic variables (numbers of sites of disease, tumor marker levels) using two previously published prognosis formulas demonstrated a good correlation between prognostic variables (numbers of sites of disease, tumor marker levels) and the clinical assessment of prognosis based on tumor bulk and tumor marker level. Nevertheless, individual patients with other, unknown prognostic features may be incorrectly evaluated using either established formulas or clinical assessment of tumor bulk.
Our approach to management of the prune belly syndrome entails a program of comprehensive early reconstruction, including abdominoplasty, bilateral orchiopexy, reduction cystoplasty, and selective ureteral tailoring and reimplantation. Of 20 patients with the prune belly syndrome treated at our institution during the last 18 years 15 underwent comprehensive reconstruction. Abdominoplasty was performed in 16 patients. Results were evaluated as to cosmetic appearance, functional performance, and preoperative and postoperative electromyography. Immediate good results were obtained in 9 patients. Of 16 patients 6 required more than 1 abdominal wall tailoring and 3 had minor complications that did not compromise the ultimate result. Transabdominal orchiopexy was performed on 32 testes; 28 (88 per cent) are in the scrotum and growing. Of the 4 failures 3 occurred in patients greater than 2 years old at repair. Selective urinary tract reconstruction has been performed; 13 patients underwent tapering and reimplantation of 1 or both ureters. Reduction cystoplasty was done in all 15 patients. Functional assessment of the upper urinary tract by improved radiological techniques and the necessity for long-term antibiotic prophylaxis have led to a more conservative approach in 5 recent patients. Renal function as measured by serum creatinine and/or glomerular filtration rate has remained stable in 12 of 15 patients (92 per cent) undergoing comprehensive reconstruction. Aggressive surgical management of patients with the prune belly syndrome provides improved abdominal wall function and appearance, and offers excellent testicular salvage. Surgical reconstruction of deformed ureters should be selective and decided on longitudinal studies of renal function.
We performed a retrospective review to evaluate the results of a nonsurgical approach to the management of primary vesicoureteral reflux during a 10-year period (1976 to 1986). During that interval patients with reflux were studied initially with a standard voiding cystourethrogram and either an excretory urogram or a renal scan with glomerular filtration rate and/or differential renal function determination. Height, weight, blood pressure, urine cultures and serum creatinine measurements also were obtained. Isotope cystography was used for followup examinations. A single, negative isotope cystogram was the radiological criterion for cessation of reflux. The charts of 545 children (55 per cent had bilateral reflux) with 844 refluxing ureters were reviewed. Based upon the international classification vesicoureteral reflux was grade I in 6.6 per cent of the cases, grade II in 54.2 per cent, grade III in 31.6 per cent, grade IV in 5.7 per cent and grade V in 1.9 per cent. All children were kept on long-term continuous prophylactic antibiotics and they were re-evaluated annually with isotope cystography. The followup rate for the entire group was 88 per cent. During the observation period spontaneous resolution of reflux was noted in 36 per cent of the patients and 39 per cent of the total refluxing ureters. Only 13 per cent of the entire group underwent surgical correction of reflux. Presently, 39 per cent (215) of the patients continue to be followed with reflux. Of the total group 66 patients (12 per cent) were lost to followup. In the 194 patients with spontaneous resolution of reflux the mean duration of reflux was 1.69 years, with 30 to 35 per cent resolving each year. Based on Student's t test there was a significant difference in duration of reflux in patients with grade II compared to grade III reflux (1.56 versus 1.97 years, p less than 0.04). When age at presentation was compared with duration of reflux there was a significantly shorter duration of reflux only in those patients presenting from age 0 to 12 months, compared to those 13 months and older (1.44 versus 1.85 years, p less than 0.02). Renal function was evaluated by serum creatinine, calculated glomerular filtration rate or differential diethylenetriaminepentaacetic acid scan results.(ABSTRACT TRUNCATED AT 400 WORDS)
