Diffuse hyperplasticperilobarnephroblastomatosis (DHPLN) is a rare precursor lesion of Wilms tumor (WT). Because of the increased risk to develop WT in either kidney, current management algorithms of DHPLN meritnephron-sparing strategies, beginning with chemotherapy and close radiographic monitoring into late childhood. After resolution of DHPLN, subsequent detection of a renal nodule mandates resection to exclude WT. Here, we report the case of a 4 year-old girl who developed two synchronous nodules in the right kidney more than two years after completion of therapy for DHPLN. Because of the early detection and peripheral location of these two nodules, laparoscopic nephron-sparing resection of each was performed using ultrasonic dissection. Both nodules were determined on pathology to be favorable histology WT with negative surgical margins. The child was placed onvincristine and actinomycin-D therapy for 18 weeks. KeywordsWilms tumor; nephroblastomatosis; laparoscopy; nephron-sparing surgery Diffuse hyperplasticperilobarnephroblastomatosis (DHPLN) is a rare precursor lesion of Wilms tumor (WT)and may be considered an intermediate, pre-neoplastic stage in the sequence of Wilms tumorigenesis(1-5).Because the vast majority of DHPLN cases occur with bilateral renal involvement, current treatment algorithms emphasize nephron-sparing strategies(4).At time of diagnosis, DHPLN patients are recommended to receive 18 weeks of vincristine and actinomycin-D and may continue on extended therapy until all lesions resolve(5,6). Refractory, recurrent or new expanding nodules prompt tissue diagnosis with nephron-sparing resection to exclude WT initiation. Notably, in the seminal report outlining the natural history of DHPLN and the risk to develop WT, 24 of 52 (46.2%) patients who Address correspondence to: Harold "Bo" N. Lovvorn, III, MD, FACS, FAAP, Assistant Professor of Pediatric Surgery, Vanderbilt University Children's Hospital, Doctor's Office Tower, Suite 7102, 2200 Children's Way, Nashville, TN 37232-9780, Office Phone: 615-936-1050, Facsimile: 615-936-1046.lovvorn@vanderbilt.edu. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. were followed for a minimum of five years developed WT, and 11 of these children developed two or more WT(4). Because of this risk to develop multiple WT in either kidney, conservative surgical strategies need to be followed to minimize late renal insufficiency, dialysis dependence, and need for renal transplantation(7). NIH Public AccessNephron-sparing strategies have been described for children presenting with bilateral WT(7-9).However, no des...
It has been reported that up to half of renal stones and associated urine specimens have been positive on culture, and that up to 50% of such stones contain magnesium ammonium phosphate. In a prospective study using infrared and wet chemical analysis, we found positive cultures in only 7 of 132 renal, 5 of 105 ureteral and 6 of 21 bladder stones obtained surgically and handled with sterility. Of the culture positive calculi only 43% from the kidney, none from the ureter and 50% from the bladder contained detectable magnesium ammonium phosphate. However, magnesium ammonium phosphate was detectable in 20% of renal, 2% of ureteral and 27% of bladder stones with negative cultures. Of the culture positive renal and ureteral calculi 42% were predominantly calcium phosphate and 17% were predominantly calcium oxalate. For culture negative stones 25% and 51% from the kidney, and 15% and 82% from the ureter were composed of predominantly calcium phosphate and calcium oxalate, respectively. Among the culture positive stones, related positive urine cultures were noted in 100% of the renal, 20% of the ureteral and 50% of the bladder cases, compared to 26%, 10% and 27%, respectively, of culture negative calculi. The same organism was found in the stone and urine in only 38% of the cases. The lower frequency of positive urine cultures, of stones with magnesium ammonium phosphate, and especially of culture positive renal and ureteral stones (5%) than in previous reports suggests that stone culture may be of less value than indicated previously, except for bladder calculi and large renal stones, such as the branched type.
Background The Children's Oncology Group recommends upfront resection of Wilms tumor (WT), however, unique scenarios warrant neoadjuvant chemotherapy and delayed resection. We hypothesized that in the context of neoadjuvant chemotherapy, minimally invasive surgery (MIS) to resect WT achieves equivalent oncologic fidelity and better maintains therapy schedules. Methods A retrospective analysis of WT treated between 2010-2021 at a free-standing children's hospital was performed. Patient and disease specific characteristics were collected, and pre-resection tumor volumes (TV) were calculated. Impact of MIS or open resection on oncologic fidelity and time to resume chemotherapy was analyzed. Results For the study period, 62 patients were treated for 65 WT, and 14 patients (22.6%) received neoadjuvant chemotherapy to treat 17 WT (26.2%): 7 Stage I (all predisposition syndromes), 2 stage III, 7 stage IV, and 1 stage V (bilateral). MIS was utilized to resect 6 WT from 5 patients. For partial nephrectomy, pre-resection TV was 0.38 ml if MIS and 10.38 ml if open ( P = .025). For radical nephrectomy, pre-resection TV was 31.58 ml if MIS and 175.00 ml if open ( P = .101). No significant differences between surgical approach were detected regarding pathologic variables or survival. Epidural use was significantly greater with open procedures ( P = .001). Length of stay was 2.00 days after MIS compared to 6.00 for open resection ( P = .004). Time to resume chemotherapy was 7.00 days after MIS versus 27.00 for open ( P = .004). Conclusion After neoadjuvant chemotherapy for WT, MIS partial and radical nephrectomies achieved equivalent oncologic fidelity, reduced epidural use and post-operative stays, and better maintained adjuvant therapy timelines when compared to open resections.
Purpose Given evolving imaging technologies, we noted significant variation in the diagnostic evaluation of pediatric choledochal cysts (CDC). To streamline the diagnostic approach to CDC, and minimize associated expenses, we compared typing accuracy and costs of ultrasound (US), intraoperative cholangiography (IOC), and magnetic resonance cholangiopancreatography (MRCP). Methods Records of 30 consecutive pediatric CDC patients were reviewed. Blinded to all clinical data, two pediatric radiologists reviewed all US, MRCPs, and IOCs to type CDCs according to the Todani classification. When compared with pathologic findings, the concordance between and accuracy of each diagnostic test were determined. Inflation-adjusted procedure charges and collections for imaging modalities were analyzed. Results Mean typing accuracy overlapped for US, IOC, and MRCP. Inter-rater reliability was 87 % for US (κ = 0.77), 80 % for IOC (κ = 0.62), and 60 % for MRCP (κ = 0.37). MRCP procedure charges ($1204.69) and collections ($420.85) exceeded IOC and US combined ($264.80 charges, p = 0.0002; $93.40 collections, p = 0.0021). Conclusion Our data support the use of US alone in the diagnosis of pediatric CDC when no intrahepatic biliary ductal dilatation is visualized. However, when dilated intrahepatic ducts are encountered on US, MRCP should be utilized to distinguish a type I from a type IV CDC, which may alter the operative approach.
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