Purpose
This study aimed to report a multi-institutional retrospective case series of outcomes after robot-assisted laparoscopic pyeloplasty (RALP) in pediatric patients with complex pelvi-ureteric ...junction obstruction (PUJO).
Methods
All patients undergoing complex RALP over the last 5 years were included. RALP was defined complex in the following cases of PUJO: anatomic variations including ectopic, malrotated, horseshoe, or duplex kidney and recurrent PUJO after failed open pyeloplasty.
Results
Forty-eight patients underwent complex RALP in four European centers in the study period and included 18 girls and 30 boys with a median age of 8 years (range 5–12). The PUJO was associated with anatomic variations in 35/48 (72.9%), whereas a recurrent PUJO was present in 13/48 (27.1%). A dismembered Anderson–Hynes pyeloplasty was performed in all patients. The median operative time including docking was 178.5 min (range 117–255) and the median anastomotic time was 64.8 min (range 50–76). All patients were discharged on 2nd postoperative day (POD). The median follow-up was 18.2 months (range 14–43). The overall success rate was 95.8% (46/48). Early postoperative complications (< 30th POD) included urinary tract infections (UTIs) and stent-related irritative symptoms in 4/48 (8.3%) II Clavien, whereas late complications (> 30th POD) included recurrence of PUJO in 2/48 (4.2%), who needed re-operation IIIb Clavien.
Conclusions
RALP was safe, feasible, and with good mid-term outcome in complex PUJO. An accurate pre-operative planning, a standardized technique, and an experienced surgical robotic team represented key points to manage successfully such complex cases.
PIK3CA-related overgrowth spectrum (PROS) includes rare genetic conditions due to gain-of-function mutations in the PIK3CA gene. There is no approved medical therapy for patients with PROS, and ...alpelisib, an approved PIK3CA inhibitor in oncology, showed promising results in preclinical models and in patients. Here, we report for the first time the outcome of two infants with PROS having life-threatening conditions treated with alpelisib (25 mg) and monitored with pharmacokinetics. Patient 1 was an 8-mo-old girl with voluminous vascular malformation. Patient 2 was a 9-mo-old boy presenting with asymmetrical body overgrowth and right hemimegalencephaly with West syndrome. After 12 mo of follow-up, alpelisib treatment was associated with improvement in signs and symptoms, morphological lesions and vascular anomalies in the two patients. No adverse events were reported during the study. In this case series, pharmacological inhibition of PIK3CA with low-dose alpelisib was feasible and associated with clinical improvements, including a smaller size of associated complex tissue malformations and good tolerability.
Little is known about the effects of CO
2
insufflation (CDI) on cerebral oxygen saturation (CrSO
2
) during laparoscopy in the pediatric population. In children undergoing robotic-assisted ...laparoscopic pyeloplasty (RALP), we prospectively assessed the effects of CDI using standard monitoring and cerebral near-infrared spectroscopy (NIRS). We also explored whether a correlation existed between CrSO
2
and parameters known to affect cerebral blood flow. Between January 2021 and September 2023, a cohort of consecutive children older than 2 years underwent RALP at Necker-Enfants Malades Hospital in Paris. A ventilation protocol aimed to prevent hypercarbia was implemented. Data collected included standard monitoring parameters and CrSO
2
by NIRS. Thirty patients (16 females), mean age 5.5 ± 3.9 (2.0–9.5) years, were included. Twenty-three patients underwent a retroperitoneal approach. The mean baseline CrSO
2
value was 83.0 ± 9.8. Mean CrSO
2
decreased during progressive CDI, never below baseline values, while standard-monitoring parameters did not significantly change. No significant correlation was detected between CrSO
2
and end tidal CO
2
, or between CrSO
2
and mean arterial pressure, at any operative time. During RALP, a gradual CDI doesn’t cause pathological derangements of CrSO
2
. The lack of correlation between CrSO
2
and standard parameters affecting cerebral blood flow suggests the likely presence of cerebral autoregulation in our population.
Purpose
We have studied outcome of double-face preputial island flap (DFPIF) technique in severe types of hypospadias: penoscrotal, scrotal and perineal.
Methods
We have used DFPIF in 75 boys at a ...median age of 1.1 years (1.0–1.5). The meatus was penoscrotal, scrotal or perineal after de-gloving the penis. The inner face of the foreskin was used for urethroplasty and the outer face for ventral skin covering. Modifications were added: proximal anastomosis was protected by a spongioplasty; in case of urethral plate transection, we anastomosed on onlay proximal and distal segments of the flap (onlay-tube-onlay) and the tubularized part was sutured to corpus cavernosa. FU was scheduled at one month then every 3 months for a year then annually. At each consultation, the surgeon filled out a detailed cosmetic and functional sheet including flowmeter.
Results
Thirty-four patients had onlay preputial flap repair with urethral plate preservation. Forty-one had the onlay-tube-onlay technique. All children had a curvature, 19 had a significant residual curvature after dissection, corrected by dorsal plication (
n
= 9) and ventral lengthening (
n
= 10). Median FU was 4.2 years (2.7–6.5). 36 children (48%) had complications and needed redo surgery: 12 fistulas, 11 diverticula, 7 meatal stenosis, 3 strictures and 2 residual curvatures. All children but three voided within the normal limits for their age.
Conclusion
DFPIF remains a good option for a one-stage repair of severe hypospadias. After a median of 1.8 procedures, the final success rate was 96%. The healthy well-vascularized ventral skin allows safe redo surgery when needed.
Abstract
BACKGROUND AND AIMS
Graft artery stenosis can have a significant short- and long-term negative impact on kidney graft function. We previously reported an unusual number of graft-arterial ...anomalies following kidney transplantation (KTx) in children during the first coronavirus disease (COVID-19) pandemic wave (Berteloot et al.) 1. We report herein the 1-year follow-up of these patients.
METHOD
In this retrospective study, we included all children who received a KTx at our centre from February to July 2020. We compared their outcome to that of paediatric recipients who were transplanted at our centre from 2015 to 2019 and presented an allograft vascular complication (‘Historic’ group) by querying our local data warehouse.
RESULTS
Among the 9 children who received a KTx at our centre between February and July 2020 8 boys, median age 10 years (3–17), 8 presented Doppler features suggesting arterial stenosis, with an unusual extensive pattern (Figure 1) after a median delay of 13 days (8–113). For comparison, persistent spectral Doppler arterial anomalies were observed in only 5% of children following KTx at our centre over the previous 5-year period and were all focal anastomotic stenoses. In addition, five children had lymphoceles, which required surgical management as compared to only one patient in the 5 previous years (1%). We retrospectively diagnosed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-20 infection in 6/8 children with arterial stenosis on serologies performed at D0, including one boy with a history of positive real time reverse transcription-polymerase chain reaction (RT-PCR) 120 days before KTx. None of the patients had reported any symptom suggestive of COVID-19. The remaining two patients had received a graft from an asymptomatic deceased adolescent donor with a positive serology at D0. These data led us to suspect immune post-viral graft vasculitis, triggered by SARS-CoV-2.
At 1-year post-transplantation, the outcome was favourable in the 8 isolated KTx recipients. A total of 4/8 children had normal blood pressure and 4 had controlled high blood pressure on mono or bi-therapy. Doppler anomalies had resolved in 5/8 and persisted in 3/8 with a trend for improvement of peak systolic velocities and no severe consequences on kidney function and histology. Indeed, the median glomerular filtration rate (GFR) was 91 mL/min/1.73 m² (65–129), with unspecific and mild lesions on 4/8 protocol kidney biopsies (IFTA 1 or Cpt 1). One liver-kidney graft recipient had persistent hypertension and diffuse irregular inflammatory parietal thickening of the whole vascular graft associated with a parietal thrombus upstream of the birth of the two hepatic arteries (Figure 2); treated with anti-aggregation and prednisone 10 mg/d.
CONCLUSION
Our case series suggests a risk of post-viral kidney graft vasculitis in children with recent SARS-CoV-2 infection in the recipient or donor. Pre-transplant vaccination against COVID-19 is mandatory in children > 5 years and their kidney donor candidates at our centre. We also strongly recommend vaccination of all people aged > 5 years in the household.
Abstract Aims Guidelines regarding voiding cystourethrogram (VCUG) indications following a paediatric kidney abscess are lacking. This study evaluates vesicoureteral reflux (VUR) prevalence and ...outcome after a first kidney abscess. Methods This retrospective study included all children presenting to a tertiary paediatric reference centre with de‐novo kidney abscesses from 2011 to 2022, diagnosed through imaging (ultrasonography or computed tomography). VCUG's clinical utility was assessed by exploring outcomes related to interventions. Results Among the 17 patients (median age 9 months, IQR; 6 months–6 years), VCUG identified VUR in 7 (41%; 95% CI: 18–65%), including two with grade IV–V. Median abscess size was 19 mm (IQR; 14–27). 7/8 (88%) children with DMSA scan presented scars, including 4 with hypofunctioning (20%–44%), and one with a non‐functioning kidney. Scarring on the DMSA scan was similar regardless of identified VUR. Six children had subsequent pyelonephritis. Three of the remaining 11 had grade I–III and two IV–V VUR. Surgery was required in four children overall: three for recurrent pyelonephritis and one for high‐grade VUR and scars. Conclusion Among initial kidney abscess cases, 41% had VUR, similar to children experiencing their first uncomplicated pyelonephritis. VCUG results guided antibiotic prophylaxis but not surgical decisions. We suggest considering VCUG following recurrent pyelonephritis/kidney abscess and/or kidney scarring.
Kidney function is crucially dependent on the complex three-dimensional structure of nephrons. Any distortion of their shape may lead to kidney dysfunction. Traditional histological methods present ...major limitations for three-dimensional tissue reconstruction. Here, we combined tissue clearing, multi-photon microscopy and digital tracing for the reconstruction of single nephrons under physiological and pathological conditions. Sets of nephrons differing in location, shape and size according to their function were identified. Interestingly, nephrons tend to lie in planes. When this technique was applied to a model of cystic kidney disease, cysts were found to develop only in specific nephron segments. Along the same segment, cysts are contiguous within normal non-dilated tubules. Moreover, the shapes of cysts varied according to the nephron segment. Thus, our findings provide a valuable strategy for visualizing the complex structure of kidneys at the single nephron level and, more importantly, provide a basis for understanding pathological processes such as cystogenesis.
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