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Fetal anomalies are detected in approximately 2% of all fetuses and, among these, genitourinary tract abnormalities account for 30% to 50% of all structural anomalies present at birth. Although ultrasound remains the first line diagnostic modality, fetal MRI provides important additional structural and functional information, especially with the development of faster sequences and the use of functional sequences. The added value of MRI-based imaging is three-fold: (a) improvement of diagnostic accuracy by adequate morphological examination, (b) detection of additional anomalies, and (c) in addition, MRI has the potential to provide information regarding renal function. In this review, we describe the role of fetal MRI in the anatomical evaluation of renal and urogenital tract anomalies, and we also touch upon the contribution of functional MRI to the diagnostic workup of these conditions.

Purpose To evaluate feasibility and outcomes of minimally invasive surgery (MIS) in Wilms tumor (WT). Methods International multicenter review of MIS total nephrectomies for WT between 2006 and 2018. Medical records of confirmed WT were retrospectively assessed for demographic, imaging, treatment, pathology, and oncological outcome data. Results Fifty patients, with a median age of 38 months (6-181), were included in 10 centers. All patients received neoadjuvant chemotherapy, as per SIOP protocol. Median tumor volume post-chemotherapy was 673 mL (18-3331), 16 tumors crossed the lateral border of the spine, and three crossed the midline. Six patients with tumors that crossed the lateral border of the spine (tumor volumes 1560 mL [299-2480]) were converted to an open approach. There was no intraoperative tumor rupture. Overall, MIS was completed in 19% of the 195 nephrectomies for WT presenting during the study period. Tumor was stage I in 29, II in 16, and III in 5, and histology was reported as low in three, intermediate in 42, and high risk in five. Three patients had positive tumor margins. After a median follow-up of 34 months (2-138), there were two local recurrences (both stage I, intermediate risk, 7 and 9 months after surgery) and one metastatic relapse (stage III, high risk, four months after surgery). The three-year event-free survival was 94%. Conclusion MIS is feasible in 20% of WT, with oncological outcomes comparable with open surgery, no intraoperative rupture, and a low rate of local relapse. Ongoing surveillance is, however, needed to evaluate this technique as it becomes widespread.

OBJECTIVES To compare the prognostic value of fetal serum biochemistry and fetal urine biochemistry in predicting renal outcome in lower urinary tract obstruction (LUTO). METHODS We retrospectively studied renal outcome following a prenatal diagnosis of LUTO in cases for which both fetal blood and fetal urine were sampled. We classified the renal outcome as either "favorable," when postnatal renal function was normal, or "adverse," in the case of postnatal chronic renal failure or when renal histological lesions were present at autopsy in the case of termination of pregnancy. A prognostic model was constructed for urine and serum separately. β2-Microglobulin was the only remaining independent predictor in fetal urine. β2-Microglobulin in serum and urine were compared by using receiver operating characteristic curves. RESULTS In the 50 cases included, the rate of adverse outcome was 34 of 50(68%): autopsy confirmed severity of renal disease in all 27 cases who underwent termination of pregnancy, and among the 23 live born children, 7 developed renal failure. Fetal serum and urine markers were all significantly associated with renal outcome (P

We reviewed data from a cohort of fetuses with ureterocele diagnosed and operated prenatally in four fetal therapy centers. Inclusion criteria were: (1)ureterocele confirmed on detailed fetal ultrasound examination, (2)absence of additional fetal malformations (3)fetal intervention to decompress the ureterocele with local institutional review boards’ approval. Data on sonographic follow-up, obstetrical, neonatal outcome and postnatal evaluation were collected. Ten cases of prenatally treated ureterocele are described. Six cases benefited from a fetoscopy for laser incision and decompression, two cases had an ultrasound guided puncture before resorting to a fetoscopy with laser incision, one case had a balloon catheterization under ultrasound guidance and one case had an ultrasound-guided opening of the ureterocele with a laser fiber passed through a 20G needle. Mean GA at diagnosis was 21.6GW. Two cases underwent TOP. The remaining eight cases recovered normal amniotic fluid volume, and delivered a live-born child at a mean GA of 38.6GW with normal creatinine levels during the first week of life. Prenatal incision provided complete treatment of severely obstructive ureteroceles in 80% of the cases and allowed improvement of urinary electrolytes, renal size and echogenicity, bladder filling in all survivors and recollection of normal amniotic fluid volume, in case of oligoanhydramnios.

Aim We present the preliminary results of robotic-assisted laparoscopic (RAL) total and partial nephrectomy for renal malignant tumors in children. Methods This is a prospective study of patients operated with RAL between December 2016 and September 2018. Patients with Wilms tumors were treated according to the SIOP-2001 protocol. Patient and tumor characteristics, type of surgery, surgical-related morbidity, and oncologic outcomes were recorded. Results were compared with a series of patients with similar age- and tumor-related characteristics operated during the same period by an open surgical approach. Results Ten children underwent RAL nephrectomy with a mean age of five years (3.2-14.1 years). Total nephrectomy was done in six cases for Wilms tumor and in one case for renal sarcoma; three cases were converted. Complete removal of tumor without rupture was achieved in all cases. Postoperative course was uneventful, and patients were discharged between days 2 and 7. Neither recurrence nor medium-term complications occurred. Nine patients are alive with a median follow-up of 16 months (6-27 months) and one female died from complications of central nervous system metastases one year after surgery. When compared with the open surgical approach group, median tumor volume was smaller (P = 0.005), hospital stay was shorter (P = 0.01), and operative time was similar (P = 0.20). Conclusions RAL total and partial nephrectomy procedure for renal tumor in children may be an option in carefully selected cases. Indication should be discussed at tumor boards and surgery performed while adhering strictly to oncological surgical rules.

INTRODUCTION: Prognosis of dumbbell neuroblastoma (NBL) is mainly determined by the sequelae induced by the tumor itself and the neurosurgical approach. However, after primary chemotherapy, surgical management of the residual tumor, especially the spinal canal component, remains controversial. METHODS: We conducted a single-center retrospective cohort study over the last 15 years (2002-2017) including patients treated for NBL with spinal canal extension focusing on timing and type of surgery, complications, and functional and oncological follow-up. RESULTS: Thirty-two children (14 M, 18 F) were managed for NBL, with the majority (26) presenting with NBL stroma poor while four had ganglioneuroblastoma intermixed, one nodular, and one ganglioneuroma. All but two patients received neoadjuvant chemotherapy. Upfront laminotomy for spinal cord decompression was performed in two patients; nine patients had extraspinal surgery with a follow-up neurosurgical procedure in seven cases; eight patients had initial neurosurgery followed by an extraspinal procedure, while six patients underwent a combined multidisciplinary approach. With a median follow up of 3.6 years (0.1-14.9), 29 patients (90.6) are alive and two out of three (19, 65.5%) have functional sequelae. CONCLUSION: Patients with NBL with persistent spinal canal extension of the tumor after neoadjuvant chemotherapy treated at our center had outcomes that compare favorably with the literature. This is likely due to the multidisciplinary approach to optimal surgical strategy and continuous evaluation of the respective risks of tumor progression. Neurological disability results from initial spinal cord compression or the radicular sacrifice required for tumor resection. ispartof: PEDIATRIC BLOOD & CANCER vol:66 ispartof: location:United States status: published

Objective The aim of the study was to evaluate a biochemical analysis of fluid sampled in utero from fetal abdominal cystic masses. Methods A retrospective study of 42 intra-abdominal cystic masses [ovarian cysts (n = 22), cloaca (n = 8), urodigestive fistulae (n = 4), and urogenital sinuses (n = 8)] was carried out. The control group (n = 30) consisted of fetal urine. Seven biochemical markers were assayed: sodium, estradiol, β2-microglobulin, total proteins and digestive enzymes, gamma-glutamyl transpeptidase, aminopeptidase M, and intestinal alkaline phosphatase isoenzyme. Outcome of pregnancies and final diagnosis of intra-abdominal mass were known in all cases. Results Biochemical patterns allowed to demonstrate (1) an ovarian origin based on elevated level of estradiol and of total proteins (100% specificity, 100% sensitivity); (2) urodigestive fistula based on the presence of high levels of digestive enzymes (cloaca in female fetuses or recto-urethral fistula in male fetuses); and (3) a renal origin (urinary pattern with low total proteins and absence of digestive enzymes); however, a biochemical pattern could not differentiate between fetal urogenital sinus and megacystis. Conclusion Etiological diagnosis of a fetal cystic abdominal mass could impact both prenatal and postnatal management providing adapted prenatal counseling by a pediatric surgeon in surgically correctable congenital anomalies. © 2012 John Wiley & Sons, Ltd.