Journal of Pediatric Urology最新文献

Purpose: Historically, gonadectomy was uniformly recommended for patients with Androgen Insensitivity Syndrome (AIS) due to the perceived malignancy risk. However, this practice has been questioned given the benefits of hormone production, low prepubertal tumor risk, and unclear long-term malignancy risk. An ideal screening strategy would detect premalignant/malignant changes at an early stage and improve the safety of leaving gonads in situ. This review summarizes existing screening protocols and the evidence supporting their effectiveness.

Methods: A systematic review was conducted per PRISMA-P guidelines to examine malignancy screening in AIS. We searched PubMed, CINAHL, Web of Science, Cochrane, Scopus, Google Scholar, BioRxiv, and MedRxiv using terms related to AIS, gonadal tumors, and screening. We abstracted data from included studies focusing on three major questions: 1. What modality/frequency for screening recommendations are mentioned for AIS patients, 2. What evidence is there for the sensitivity and/or specificity of imaging, and 3. What evidence is there for the effectiveness of any screening protocol to detect neoplasia?

Results: After reviewing 538 studies, 28 met the inclusion criteria. Most recommendations were based on expert opinion. Modalities included biopsy (recommended in 14 studies), physical exams (8 studies), serum tumor markers (4 studies), ultrasound (14 studies), and MRI (6 studies). Biopsy and ultrasound were the most frequently cited, though protocols varied. Only 3 studies had indirect data regarding screening modality effectiveness, and imaging prior to gonadectomy showed limitations in identifying pre-malignant lesions. Three cohort studies included screening protocols but lacked consistency or strong evidence supporting specific strategies.

Conclusions: There is a lack of standardized screening strategies for detecting malignancy in patients with Androgen Insensitivity Syndrome. Current screening methods have limitations and are mostly based on expert opinion rather than clinical evidence. No method has proven effective, and the variability in protocols reflects the uncertainty. Patients retaining their gonads should be informed of the potential risks, as there is limited evidence supporting existing screening strategies.

Background: The surgical management of pediatric unilateral Wilms' tumor (uWT) continues to be a subject of ongoing debate, particularly regarding the criteria for lymph node (LN) sampling. To address this concern, we implemented a modified radical nephrectomy (MRN) technique that includes ipsilateral perivascular regional LN dissection following nephrectomy to enhance the surgical approach. This study evaluates the safety and feasibility of MRN in treating uWT.

Methods: We conducted a retrospective analysis of 105 uWT cases treated with MRN from January 2016 to June 2023. Clinical characteristics, treatment regimens, and patient outcomes were evaluated.

Results: All 105 patients underwent MRN successfully. Among these, 48 received upfront surgery, while 57 were treated with neoadjuvant chemotherapy. After nephrectomy, regional LN dissection was performed, with a median of 9 nodes excised. LN metastasis was found in 6 patients (5.7 %). Tumor spillage was reported in 6 cases (5.7 %), mainly during upfront surgery. Surgical complications occurred in 6 patients (5.7 %), consisting of 4 cases of chylous ascites and 2 cases of intestinal obstruction. All were successfully treated. Recurrence was seen in 8 patients (7.6 %), including two cases of local recurrence. The 3-year event-free survival and overall survival rates were 91.3 % and 95.6 %, respectively.

Conclusion: The MRN demonstrated safety and feasibility in the surgical treatment of pediatric uWT. It enables LN sampling from all relevant draining stations and avoids the limitations of random sampling. However, these findings require validation in larger, prospective, multicenter studies to determine whether this approach is superior to current practice in improving staging accuracy and patient outcomes.

Introduction: Robot-assisted laparoscopic extravesical ureteral reimplantation (RALUR-EV) is a well-established surgical approach in girls with high-grade vesicoureteral reflux (VUR). Adequate exposure of the distal ureter and ureterovesical junction is crucial to achieve good outcomes.

Methods: We present a novel modification, the medial tubo-ovarian deflection (MTOD), which improves exposure of the distal ureter by dissecting the round ligament and separating the proximal peritoneum along the white line, close to the bladder.

Results: Nine girls (median age 25 months) underwent MTOD during RALUR-EV. Median console time was 105 min, and no intraoperative complications were reported. All patients were discharged the day after surgery. The short-term complications included two postoperative febrile UTIs, none of them had reflux on VCUG and one case of transient urinary retention. No ovary-related complications were reported. Median follow-up was 20 months.

Conclusion: Performing MTOD during RALUR-EV is safe and reproducible, with good outcomes in short-term follow-ups.

Introduction: Sacrococcygeal teratomas (SCT), which are the most common germ cell tumours in the neonatal period, have frequent long-term complications including gastrointestinal and urological problems due to the anatomical region of origin. This study evaluates the lower urinary tract and bowel functions in patients who underwent surgery for SCT.

Patients and methods: Fifteen patients who underwent sacrococcygeal teratoma surgery between 2007 and 2021 were retrospectively evaluated for lower urinary tract and bowel functions. Bladder function was assessed using patient interviews, urinalysis, voiding diaries, uroflowmetry, ultrasonography, and pressure-flow studies. In the bowel function evaluation, constipation and continence were assessed. Descriptive statistics were used to present the results as mean, standard deviation, median, frequency, and percentage.

Results: Consequently, 15 patients were included, with a mean follow-up period of 8.5 years. The majority of patients were female, with a female-to-male ratio of 2.75:1. Based on the Altman classification, 73.3 % of the patients had type I SCT, 20 % had type II, and 6.6 % had type IV. The mean follow-up period of the patients was 8.5 years (2 years-15 years). Of the 15 patients whose lower urinary tract functions were assessed, 13 achieved full urinary continence and exhibited normal voiding volumes and frequency for their age. Ultrasonography findings were normal in all evaluated patients, with no residual urine or bladder abnormalities detected. Urodynamic tests showed normal detrusor activity and bladder capacities in most cases, though one patient exhibited a higher-than-expected bladder capacity during the filling phase. Voiding phase assessments revealed normal patterns in most patients, except for an 8-year-old case with Altman type IV SCT showing staccato voiding pattern and pelvic floor electromyography (EMG) activity. Anorectal examinations revealed normal anal anatomy, though fecalomas were detected in two cases. (Altman type II and IV SCT). Constipation, based on Rome IV criteria, was identified in 3 patients (1 Altman type I, 1 type II, and 1 type IV SCT). Based on the Holschneider scale, 11 patients achieved normal scores, while 3 (2 Altman type I, 1 Altman type II SCT). demonstrated good scores, reflecting overall satisfactory bowel control.

Conclusion: Bladder function was uneffected in Altman type I and II cases, except for dysfunction in one type IV case. Constipation was noted in one case each of types I, II, and IV, with no other bowel abnormalities detected. Non-invasive investigations should be prioritized for type I and II cases, while invasive methods may be necessary for those with intrapelvic extension.

Introduction: Children with primary vesicoureteral reflux (VUR) may be placed on continuous antibiotic prophylaxis (CAP) to reduce the risk of urinary tract infections (UTI). Despite the use of CAP, up to 36 % of children with VUR still develop febrile breakthrough UTI (BT-UTI). It has been suggested that ureteral diameter ratio (UDR) may predict febrile BT-UTI in children with primary VUR. We sought to validate this finding in a large cohort of children with primary VUR on CAP.

Methods: We reviewed a prospectively maintained database of children with primary VUR at a single children's hospital from October 2015 to May 2023. Children were treated at clinician discretion. We included children with primary VUR on CAP with available voiding cystourethrogram (VCUG) imaging. UDR was calculated on VCUG by measuring the largest distal ureteral diameter within the false pelvis and dividing by the distance between the bottom of the L1 vertebral body and the top of the L3 vertebral body, as previously described. In children with bilateral VUR, the higher-grade reflux and the larger UDR were used. Children with ureteroceles, duplicated systems, and secondary VUR were excluded. The primary endpoint was febrile BT-UTI. Univariate and multivariate statistical analyses were conducted.

Results: Of the 257 children in the VUR database, 132 met inclusion criteria. The median age at VCUG was 1.1 years (IQR 0.2-4.9). Females constituted 54.5 % of the cohort. VUR grades included grade 1 (9.8 %), grade 2 (15.9 %), grade 3 (31.8 %), grade 4 (25.0 %), and grade 5 (17.4 %). The average UDR was 0.312 (range: 0-0.951). For each 0.1 increase in UDR, there was a significant increase in the risk of breakthrough fUTI (OR = 1.43, 95 % CI 1.06-1.91; p = 0.016). With a median follow-up time of 2.4 years (IQR 1.2-4.6), 21 (15.9 %) children developed a breakthrough fUTI while on CAP. The area under the receiver operating characteristic curve (AUC) was 0.72 (p = 0.0006). UDR risk-stratification of febrile BT-UTI included low-risk UDR (<0.250; BT-UTI: 12.1 %), intermediate-risk UDR (0.250-0.500; BT-UTI: 17.1 %), and high-risk UDR (>0.500; BT-UTI: 24.0 %).

Conclusion: UDR is an independent risk factor to predict febrile BT-UTI in children with primary VUR on CAP. To our knowledge, this is the first and largest study to validate this finding. We propose a UDR risk stratification that may be useful in individualizing care among children with primary VUR.