The hidden burden of Pediatric urology in Sub-Saharan Africa: an analysis of hospital admission data from three East African Health Centres.

Specialist facilities for children are still unavailable in some Sub-Saharan African contexts. It is the case of pediatric urology, whose recent advances are still largely unshared. Prenatal diagnosis of urinary abnormalities (CAKUT) is largely unknown. Early recognition and referral of Undescended testis (UDT), Hypospadia, bladder exstrophy epispadias complex, ambiguous genitalia, stone disease, and tumours are uncommon in rural areas. Missed diagnosis is not uncommon and delayed management is associated with poor outcomes. We present a cross-sectional, descriptive study about the epidemiology of Pediatric urological admissions to three sub-Saharan East African Hospitals. All the urological cases between 0-18 years referred to three distinct East African Hospitals over 124 weeks were considered. Prevalence of different groups of diseases, age, and mode of presentation were reported. We found 351 cases (M/F 127/24) out of 2543 surgical referrals (13%). Seventy percent of cases were Hypospadias and UDT. Fifty percent of UDT were beyond 6, and most Hypospadias were between 4 and 7 yrs. CAKUT had a very low prevalence (4.84%), and about 50% of Wilms Tumours came too late to be resectable. In many African contexts, urology is still a tiny portion of the pediatric surgical workload compared to the 25% of European and American reports. There are also differences in the epidemiology of genitourinary conditions. A hidden burden of diseases may be presumed, remaining undiagnosed due to the shortage of specialist facilities.

Meta-Analysis on Long-Term Outcomes of Pediatric Renal Cancer Survivors Following COG and SIOP Protocols.

BACKGROUND: Pediatric renal cancer survivors have higher rate of chronic renal disease and hypertension. These patients have similar survival rates when treated according to either Children's Oncology Group (COG) or International Society of Pediatric Oncology (SIOP) protocols. We aimed to compare the late outcome of these two approaches. METHODS: We performed a meta-analysis of all studies from 2000 to 2021; database search using keywords: long-term outcomes OR late effects, nephrectomy, pediatric renal cancer. For each protocol, data were collected, and the "pooled" outcomes were compared. Continuous and dichotomous variables were obtained with a 95% odds ratio. RESULTS: Sixteen studies with a total of 715 pediatric renal cancer survivors were analyzed. The mean follow-up time was 17.4 (standard deviation 5.6) years. Reduced renal function and hypertension were the most encountered long-term complications. The mean estimated glomerular filtration rate was similar in both protocols (101.62 vs. 101.70 mL/min/1.73 m2), while the prevalence of hypertension was 23% in COG and 10% in SIOP. The prevalence of secondary malignancy was 1.1% in COG and 6.7% in SIOP (1.1% vs. 6.7%, p ≤ 0.001). Chronic kidney disease was similar in both groups. CONCLUSION: A high prevalence of hypertension was observed among pediatric renal cancer survivors, as well as an increased risk of a secondary tumor. These results emphasize the importance of long-term follow-up into adulthood, to promptly diagnose any long-term side effects of the treatment. Thanks to the increased overall survival, future protocols will pay attention to the reduction of long-term sequelae.

Elaboration and development of a realistic 3D printed model for training in ultrasound-guided placement of peripheral central venous catheter in children.

BACKGROUND: Simulation for training is becoming a trend topic worldwide, even if its applications are commonly limited to adulthood. Ultrasound-guided procedures require practice and experience-especially in the pediatric field, where the small size of the involved anatomical structures poses major problems. In this context, a realistic 3D printed pediatric phantom for training of the ultrasound-guided placement of peripheral central venous catheters in children was developed. MATERIALS AND METHODS: Starting from Computed Tomography scans of an 8 years-old girl, her left arm was virtually reconstructed-including bones, arteries, and veins-through a semi-automatic segmentation process. According to preliminary results, the most suitable 3D printing technologies to reproduce the different anatomical structures of interest were selected, considering both direct and indirect 3D printing techniques. Experienced operators were asked to evaluate the efficacy of the final model through a dedicated questionnaire. RESULTS: Vessels produced through indirect 3D printing latex dipping technique exhibited the best echogenicity, thickness, and mechanical properties to mimic real children's venous vessels, while arteries-not treated and/or punctured during the procedure-were directly 3D printed through Material Jetting technology. An external mold-mimicking the arm skin-was 3D printed and a silicone-based mixture was poured to reproduce real patient's soft tissues. Twenty expert specialists were asked to perform the final model's validation. The phantom was rated as highly realistic in terms of morphology and functionality for the overall simulation, especially for what concerns vessels and soft tissues' response to puncturing. On the other hand, the involved structures' US appearance showed the lower score. CONCLUSIONS: The present work shows the feasibility of a patient-specific 3D printed phantom for simulation and training in pediatric ultrasound-guided procedures.

UEMS Section and Board of Paediatric Surgery-A Historical Perspective.

The European Union of Medical Specialists (UEMS) Section and Board of Pediatric surgery was founded more than 40 years ago. Since then major activities have been related to the improvement of quality of care of pediatric surgery in Europe. Remarkable success was achieved in the development of pediatric surgery as an independent specialty all over Europe. Other major successful activities of the UEMS Section and Board of Pediatric Surgery consisted of the development of a high-quality European examination and delineating a minimal common standard in pediatric surgery training in the form of European training requirements. Recommendations drawn from examination experience support that candidates who achieve weaker passes in part 1 may wish to consider more practice before attempting part 2 due to the weak correlation between the two scores. It may be helpful for candidates to consider having some experience working in an English-speaking clinical setting, if not truly fluent in English, to improve their chances of being successful in the part 2 examination. Other achievements of the Section were accreditation of the training centers in Europe and European Census in pediatric surgery project. All the aforementioned activities led to standardization and harmonization of pediatric surgery, as well as contributed to increasing quality of pediatric surgical service throughout Europe.

Intracavitary electrocardiography-guided positioning of central vascular access device can spare unnecessary ionizing radiation exposure in pediatric patients.

BACKGROUND: Most hospital protocols-including those of our own institute-require the use of radiography to validate tip position in every central vascular access device placement. This study evaluated whether unnecessary ionizing radiation exposure could be spared in the pediatric population when intracavitary electrocardiography is used to guide catheter placement. MATERIAL AND METHODS: Retrospective study of intracavitary electrocardiography-guided central vascular access device placements in our pediatric surgery department between 2013 and 2018. We evaluated the operating time, success in positioning the catheter, and accuracy of final tip position. We also assayed the effects of catheter type and of catheter access point on operating time, success, accuracy, and complications. We applied the chi-square test for statistical analysis. RESULTS: In total, 622 interventions of central vascular access device placements were evaluated; 340 intracavitary electrocardiography-guided central vascular access device placements were included in the study. The electrocardiography method successfully positioned the tip of the catheter in 316/340 (92.94%) of placements. Where intracavitary electrocardiography placement was successful, radiography confirmed accuracy of tip position in 314/316 (99.41%) of placements. CONCLUSION: When electrocardiography-guided positioning is uneventful and a valid P-Wave pattern is seen, postprocedure radiograph imaging for verification is unnecessary. Any effort should be made to upgrade hospital policies according to evidences and newest guidelines to spare pediatric patients harmful exposure to radiation by limiting the use of radiography only to selected cases.

Epidemiology of Pediatric Surgical Conditions Observed in a First-Level Hospital in Burundi.

Background: Little is known about the surgical conditions affecting the pediatric population in low-income countries. In this article we describe the epidemiology of pediatric surgical diseases observed in Mutoyi hospital, a first-level hospital in Burundi. Methods and Findings: We retrospectively reviewed the records of all children (0-14 years) admitted to the Surgery ward from January 2017 to December 2017. We also reviewed the records of all the patients admitted to the Neonatology ward in 2017 and among them we selected the ones in which a surgical diagnosis was present. Five hundred twenty-eight children were admitted to the surgical ward during the study period. The most common conditions requiring hospitalization were abscesses (29.09%), fractures (13.59%), osteomyelitis (9.76%), burns (5.40%) and head injuries (4.36%). The average length of stay was 16 days. Fifty-six newborns were admitted to the Neonatology ward for a surgical condition; 29% of them had an abscess. Conclusions: Conditions requiring surgical care are frequent in Burundian children and have a completely different spectrum from the western ones. This is due on one side to an under-diagnosis of certain conditions caused by the lack of diagnostic tools and on the other to the living conditions of the population. This difference should lead to intervention plans tailored on the actual necessities of the country and not on the western ones.

Juvenile Granulosa Cell Tumor of the Testis: Prenatal Diagnosis and Management.

Prepubertal primary testicular tumors account for ∼1% of all pediatric solid tumors. We report a new case of prenatal diagnosis of juvenile-type granulosa cell tumor (JGCT). A fetal ultrasound performed at the 38th week of gestation for suspected nonvertex presentation identified a left multilocular septated cystic testicular mass, suggestive for JGCT. At birth, a painless left scrotal mass was detected. Ultrasound re-evaluation excluded torsion of the testis. Tumor markers and abdominal ultrasound were normal for age. Inguinal exploration revealed a cystic mass beneath the tunica albuginea that had replaced all the normal parenchyma. Since organ-sparing surgery was thus not feasible, an orchiectomy was performed and diagnosis of JGCT was confirmed. At 7-year follow-up, the child presented an uneventful outcome. Our case shows that neonatal JGCT, which has an intrauterine genesis, can be diagnosed prenatally by ultrasound in the last weeks of pregnancy.

European Census on Pediatric Surgery.

BACKGROUND: Detailed data on the distribution of pediatric surgical institutions in Europe are sparse. Therefore, the Section and Board of Pediatric Surgery of the Union of European Medical Specialists (UEMS) and the European Pediatric Surgeons' Association (EUPSA) jointly organized the first official census of the European centers of pediatric surgery (PS). MATERIALS AND METHODS: After obtaining a list of pediatric surgical centers in Europe, a specialized questionnaire was created and made available on the Internet. General, workload, staff, and ancillary data were collected for the centers. RESULTS: Total 215 out of 431 centers answered. PS center density is 1 in every 177,000 children or 1 in every 1,142,000 inhabitants. Approximately 77% of the centers (167 out of 215) are training centers in PS. Every center has an average inpatients procedures/year equal to 1,588, and 92.6 neonatal procedures/year. There are 3.9 surgeons/100,000 children, and 1,662 neonates for every surgeon. In average every EU surgeon performs 202 procedures/year, of which 11 procedures are on neonates. Trainees represent the 38% of the total workforce, and there are 2.3 trainees for every staff aged > 60 years. CONCLUSION: The paper aims to offer a more grounded basis on which the future of PS in our Continent should be planned. Presented data will provide an invaluable help to all our colleagues, as well as national policy makers, to press for and to make better informed and well-grounded sound political choices in the field of PS.

WAGR syndrome: is the 'R' always justified?

Although mild-to-moderate intellectual disability is usually considered part of WAGR syndrome (Wilms' tumour (WT), Aniridia, Genital abnormalities, and metal Retardation, due to 11p13 deletion) the neuropsychological profile of the syndrome is little reported in the literature. We report about a 12-year-old boy presenting with WAGR syndrome (WT, right complete aniridia, bilateral cryptorchidism, interstitial deletion involving band 11p13) but with no mental retardation. An in-depth clinical evaluation revealed no behavioural or social problems and the child's neuropsychological profile was found to be within the normal range for all abilities and functions investigated (with the exception of an impulsive cognitive style and some difficulties in academic skills). This case underlines the importance of in-depth neuropsychological evaluation that includes not only IQ measurement, but also examination of attention and academic skills, in order to establish the complete phenotypical profile of WAGR patients, rather than labelling them as learning disabled (i.e. mental retardation).

A comparison of renal function outcomes after nephron-sparing surgery and radical nephrectomy for nonsyndromic unilateral Wilms tumor.

OBJECTIVE: To better understand the impact of nephron-sparing surgery (NSS) on renal function in patients with nonsyndromic unilateral Wilms tumor (uWT), a group of such patients treated with NSS were compared with a stage-matched cohort managed with radical nephrectomy (RN). The recommended management of nonsyndromic uWT is RN. However, NSS may decrease the risk of long-term renal disease and associated comorbidities. MATERIALS AND METHODS: An international, multi-institutional review was conducted of nonsyndromic uWT cases managed with NSS and compared with a stage-matched RN cohort. Data were collected on demographics, oncologic characteristics and outcomes, serum creatinine, and estimated glomerular filtration rate (eGFR) calculated via Schwartz formula. RESULTS: Fifteen patients who underwent NSS (6 females and 9 males) met study criteria and were diagnosed at a median age of 2.5 years (range, 0.2-8.2 years) and followed for a median of 8.4 years (range, 0.5-31.8 years). The stage-matched RN cohort consisted of 15 patients (8 females and 7 males) diagnosed at a median age of 3.7 years (0.3-7.4) and followed for a median of 2.1 years (0.6-10.5 years). The median preoperative eGFR was 91.7 (39.4-237.7) and 149.9 (93.8-215.9) for NSS and RN, respectively, P=.026. The median eGFR at last follow-up was 135.3 (57.5-185.8) and 131.0 (98.6-161.2) for NSS and RN, respectively, P=.95. The median change in eGFR during the study period was a gain of 28.6 (-51.9 to 83.0) for the NSS cohort vs a loss of 19.1 (-54.7 to 25.2) for the RN cohort, P=.007. CONCLUSION: In a highly selected patient population with nonsyndromic uWT, NSS provides excellent renal function preservation when compared with RN. These data require validation via prospective investigation on a larger scale.

Grade 3 anal intraepithelial neoplasia in an HIV-infected African girl.

Women with HIV infection are at increased risk of anogenital dysplasia and cancer, related to human papillomavirus infection. These neoplasms are rare in perinatally HIV-infected girls before onset of sexual activity. We report a case of high-grade anal dysplasia in a 10-year-old African girl with untreated perinatal HIV infection.

Isolated colon ischemia with norovirus infection in preterm babies: a case series.

INTRODUCTION: Norovirus infection with necrotizing enterocolitis has so far been reported as a specific tropism of the small bowel in premature newborns. CASE PRESENTATION: Three cases of premature newborns presenting with extensive isolated colonic ischemia due to norovirus infection are reported.Patient 1 was a Caucasian girl with a gestational age of 29+2 weeks. She had sudden onset of abdominal distension on the 30th day of life. Radiological signs of colonic pneumatosis were present 48 hours before perforation and stool analysis was positive for norovirus. On the 34th day, free air was detected on plain abdominal X-ray. At laparotomy, stenosis, necrosis and perforations involved the whole colon. The patient underwent ileostomy. A large colon resection and ileosigmoid anastomosis were done 3 months later.Patient 2 was a Caucasian boy with a gestational age of 28+3 weeks. On the 19th day, bloody stools with abdominal distension appeared. Stool analysis resulted positive for norovirus. A plain abdominal X-ray showed distended bowel loops. Antibiotic treatment was started. On the 32nd day due to the progressive deterioration of clinical conditions and the appearance of colic pneumatosis, a laparotomy was performed. Severe damage of the transverse colon and multiple areas of necrosis were found. Terminal ileostomy was performed. Six months later surgery consisted of mid-transverse colon resection as far as the splenic flexure, colocolic anastomosis and closure of ileostomy.Patient 3 was a Caucasian boy with a gestational age of 30 weeks. On the 44th day bloody-mucous stools appeared and stool analysis was positive for norovirus infection. Even with institution of antibiotic therapy clinical abdominal radiologic signs of colonic pneumatosis of the upper right quadrant were found. At the 70th day an explorative laparotomy showed dilated bowel loops and stenotic right colon and ileostomy was mandatory. Partial colectomy was later necessary and ileocolic anastomosis was performed. CONCLUSION: We hypothesize that norovirus infection may be responsible for severe, distinctive colonic lesions, even in premature newborn infants.

Bladder papilloma in a 12-year-old girl.

Epithelial bladder tumours are rare in childhood. A case of bladder papilloma in a 12-year-old girl is reported. Emphasis is given to the important role of ultrasound in diagnosis. Endoscopic treatment was successful and no evidence of recurrence was found at 5-year follow-up. Exposure to a chemical environmental factor (volatile shoe adhesives) is suggested as a possible cause of the tumour.