Complications after Heller myotomy in children: a national multicenter study on the impact of prior endoscopic dilatation and identification of risk factors.

BACKGROUND: Although esophageal achalasia has been historically treated by Heller myotomy, endoscopic esophageal dilatations are nowadays often the first-line treatment in children. The aim was to assess whether performing an endoscopic dilatation before a Heller myotomy is associated with higher risks of esophageal perforation in children. METHODS: A retrospective multicentric study was performed, including children that underwent a Heller myotomy (2000-2022, 10 centers). Two groups were compared based on the history of previous dilatation before myotomy. Outcomes esophageal perforation (intra-operative or secondary) and post-operative complications requiring surgery (Clavien-Dindo III). Statistics Comparisons using contingency tables or Kruskal-Wallis when appropriate. Statistical significance: p-value < 0.05. RESULTS: A Heller myotomy was performed in 77 children (median age: 11.8 years), with prior endoscopic dilatation in 53% (n = 41). A laparoscopic approach was used in 90%, with associated fundoplication in 95%. Esophageal perforation occurred in 19% of children (n = 15), including 12 patients with intra-operative mucosal tear and 3 with post-operative complications related to an unnoticed esophageal perforation. Previous endoscopic dilatation did not increase the risk of esophageal perforation (22% vs 17%, OR: 1.4, 95%CI: 0.43-4.69). Post-operative complications occurred in 8% (n = 6), with similar rates regardless of prior endoscopic dilatation. Intra-operative mucosal tear was the only risk factor for post-operative complications, increasing the risk of complications from 5 to 25% (OR: 6.89, 95%CI: 1.38-31.87). CONCLUSIONS: Prior endoscopic dilatations did not increase the risk of esophageal perforation or postoperative complications of Heller myotomy in this cohort of children with achalasia. Mucosal tear was identified as a risk factor for post-operative complications.

Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia.

While sickle cell anemia (SCA) and hereditary spherocytosis (HS) share common features of increased spleen erythrophagocytosis due to increased red blood cell (RBC) turnover, SCA is specifically characterized by susceptibility to infections. In this study, histological lesions in the spleens of pediatric patients with SCA were analyzed, in close correlation with past clinical history and comparatively to HS, healthy and transfused ß-thalassemia patients (TDT). An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy-Gamna, and RBC trapping) combined into a severity score was established, as well as B-cell follicles analysis. Quantification on digitalized slides of iron deposition, RBC trapping, and red pulp fibrosis was additionally performed. Spleens from 22 children with SCA, eight with HS, eight with TDT, and three healthy controls (HC) were analyzed. Median age at splenectomy was not different between SCA and HS patients, 6.05 years (range: 4.5-16.0) versus 4.75 (range: 2.2-9.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions. Contrary to previous reports, B-cell follicles were generally preserved in SCA. While RBC trapping was significantly increased in both SCA and HS (compared to TDT and HC), quantitative fibrosis and overall red pulp severity score were significantly increased in SCA spleens compared to other conditions. Moreover, there was an inverse correlation between quantitative fibrosis and number of B-cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B-cell subpopulations defects.

Enhanced Recovery after Surgery Applied to Pediatric Laparoscopic Cholecystectomy for Simple Cholelithiasis: Feasibility and Teaching Insights.

BACKGROUND: Same-day discharge after a cholecystectomy is a common practice in the adult population and has been demonstrated as safe and viable for children as well. However, there is a lack of comprehensive teaching models for pediatric cholecystectomy. Drawing inspiration from standardized outpatient procedures, this study aimed to assess the clinical outcomes and feasibility of teaching programs and an Enhanced Recovery After Surgery (ERAS) protocol following ambulatory laparoscopic cholecystectomy in pediatric patients. METHODS: In 2015, an ERAS pathway for laparoscopic cholecystectomy (LC) was implemented, focusing on admission procedures, surgery timing, anesthetic choices, analgesia, postoperative feeding, mobilization, and pain assessment. Day-case surgery was not applicable for acute cholecystitis, choledochal lithiasis, sickle cell disease, and hereditary spherocytosis cases. The protocol was employed for a group of attending surgeons and fellows, as well as a group of residents under the supervision of experienced surgeons. A retrospective analysis was conducted to evaluate the feasibility and effectiveness of ambulatory cholecystectomy in children and its utilization in training pediatric surgical trainees. RESULTS: Between 2015 and 2020, a total of 33 patients were included from a cohort of 162 children who underwent LC, with 15 children operated on by senior surgeons and 18 by young surgeons. The primary diagnoses were symptomatic gallbladder lithiasis (n = 32) and biliary dyskinesia (n = 1). The median age at the time of surgery was 11.3 years (interquartile range (IQR) 4.9-18), and the median duration of surgery was 54 min (IQR 13-145). One intraoperative complication occurred, involving gallbladder rupture and the dissemination of lithiasis into the peritoneal cavity. Three patients (9%) required an overnight stay, while no postoperative complications or readmissions within 30 days were observed. ERAS was successfully implemented in 30 patients (91%). No significant differences in surgical outcomes were noted between senior and young surgeons. At an average follow-up of 55 months, no long-term sequelae were identified. CONCLUSIONS: These findings align with the current trend of increasing use of outpatient laparoscopic cholecystectomy and underscore its feasibility in the pediatric population. The application of a structured ERAS protocol appears viable and practical for training the next generation of pediatric surgeons. LEVEL OF EVIDENCE: Level III.

Robotic-assisted colectomy in children: a comparative study with laparoscopic surgery.

The aim of this study was to compare outcomes of laparoscopic and robotic-assisted colectomy in children. All children who underwent a colectomy with a laparoscopic (LapC) or robotic-assisted (RobC) approach in our institution (January 2010-March 2023) were included. Demographics, surgical data, and post-operative outcomes within 30 days were collected. Additional cost related to the robotic approach was calculated. Comparisons were performed using Fisher tests for categorical variables and Mann-Whitney tests for continuous variables. A total of 55 colectomies were performed: 31 LapC and 24 RobC (median age: 14.9 years). Main indications included: inflammatory bowel disease (n = 36, 65%), familial adenomatous polyposis (n = 6, 11%), sigmoid volvulus (n = 5, 9%), chronic intestinal pseudo-obstruction (n = 3, 5%). LapC included 22 right, 4 left, and 5 total colectomies. RobC included 15 right, 4 left, and 5 total colectomies. Robotic-assisted surgery was associated with increased operative time (3 h vs 2.5 h, p = 0.02), with a median increase in operative time of 36 min. There were no conversions. Post-operative complications occurred in 35% of LapC and 38% of RobC (p = 0.99). Complications requiring treatment under general anesthesia (Clavien-Dindo 3) occurred in similar rates (23% in LapC vs 13% in RobC, p = 0.49). Length of hospitalization was 10 days in LapC and 8.5 days in RobC (p = 0.39). The robotic approach was associated with a median additional cost of 2156€ per surgery. Robotic-assisted colectomy is as safe and feasible as laparoscopic colectomy in children, with similar complication rates but increased operative times and cost.

Early splenectomy in a large cohort of children with sickle cell anemia: risks and consequences.

In children with sickle cell anemia (SCA), early splenic complications can require splenectomy, but the benefit-to-risk ratio and the age at which splenectomy may be safely performed remain unclear. To address this question, we analyzed the rate of post-splenectomy events in children with SCA splenectomized between 2000-2018 at the Robert Debré University Hospital, Paris, France. A total of 188 children underwent splenectomy, including 101 (11.9%) from our newborn cohort and 87 referred to our center. Median (Q1-Q3) age at splenectomy was 4.1 years (range 2.5-7.3 years), with 123 (65.4%) and 65 (34.6%) children splenectomized at ≥3 years of age or <3 years of age, respectively. Median postsplenectomy follow-up was 5.9 years (range 2.7-9.2 years) yielding 1192.6 patient-years (PY) of observation. Indications for splenectomy were mainly acute splenic sequestration (101 [53.7%]) and hypersplenism (75 [39.9%]). All patients received penicillin prophylaxis; 98.3% received 23-valent polysaccharic pneumococcal (PPV-23) vaccination, and 91.9% a median number of 4 (range 3-4) pneumococcal conjugate vaccine shots prior to splenectomy. Overall incidence of invasive bacterial infection and thrombo-embolic events were 0.005 / PY (no pneumococcal infections) and 0.003 / PY, respectively, regardless of age at splenectomy. There was an increased proportion of children with cerebral vasculopathy in children splenectomized <3 years of age (0.037 / PY vs. 0.011 / PY; P<0.01). A significantly greater proportion of splenectomized than non-splenectomized children were treated with hydroxycarbamide (77.2% vs. 50.1%; P<0.01), suggesting a more severe phenotype in children who present spleen complications. If indicated, splenectomy should not be delayed in children, provided recommended pneumococcal prophylaxis is available. Spleen complications in childhood may serve as a marker of severity.

Thoracoscopic lobectomy for congenital pulmonary airway malformations before or after 5 months of age: evaluation of pulmonary function.

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is the most common pulmonary malformation. It can be managed via thoracoscopic lobectomy, which is safe and advantageous over thoracotomy. Some authors advocate the need for early resection to get an advantage over lung growth. Our study aimed to evaluate and compare the pulmonary function in patients who underwent thoracoscopic lobectomy for CPAM before and after 5 months of age. METHODS: This retrospective study was conducted between 2007 and 2014. Patients younger than 5 months were assigned to group 1 and those over 5 months of age were assigned to group 2. Pulmonary function tests (PFT) were requested for all the included patients. For patients who could not undergo full PFT, the function residual capacity (FRC) was evaluated by the helium dilution technique. The parameters evaluated in full PFT were forced expiratory volume in one second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and FEV1 to FVC ratio (FEV1/FVC). The Mann Whitney U test was used to compare both groups of patients. RESULTS: Seventy patients underwent thoracoscopic lobectomy during this period, 40 of which had CPAM. Twenty-seven patients tolerated and underwent PFT (group 1: 12 patients; group 2: 15 patients). Among them, 16 patients underwent full PFT and 11 patients had FRC measurement. FRC was similar in both groups (91% vs. 88.2%). FEV1 (83.9% vs. 86.4%), FVC (86.8% vs. 92.6%) and TLC (86.5% vs. 87.8%) were also similar between both groups. FEV1/FVC was slightly higher in group 1 (97.9% vs. 89.4%) but the difference was not statistically significant. CONCLUSIONS: PFT for patients who underwent thoracoscopic lobectomy for CPAM before or after 5 months of age is normal and comparable between both groups. Surgical resection of CPAM can be performed safely early in life without any consequences for pulmonary function or more complications when older children undergo surgery.

Scarless laparoscopic incisions in Pfannenstiel (slip): the first 50 cases using an innovative approach in pediatric robotic surgery.

Incisions in the supra-pubic region have been described robotic-assisted surgery to improve cosmetic results in adults, but seldom in children. We aimed to present an innovative trocar placement in the Pfannenstiel line, named Scarless Laparoscopic Incisions in Pfannenstiel (SLIP), and evaluate its feasibility for various intra-abdominal procedures and its cosmetic results in pediatric robotic surgery. We performed a monocentric prospective study, including children undergoing robotic-assisted surgeries using a SLIP approach (July 2019-September 2021). Data regarding demographics, surgery, and outcome were collected and reported as median (range), or number (percentage). Cosmetic results were evaluated with a questionnaire. A SLIP approach was performed in 50 children (24 cholecystectomies, 12 splenectomies, 2 cholecystectomies and splenectomies, 9 colonic resections, 2 choledochal cyst resections, and 1 pancreatic pseudocyst resection). Median age was 11 years (2-18) and median weight 35 kg (10.5-80). Conversion to laparoscopy occurred in two cases. Post-operative complications occurred in 5 patients (10%), after colectomies [intrabdominal abscess (n = 3), stoma dysfunction (n = 1), parietal abscess (n = 1)], of which 3 (6%) required reintervention (intrabdominal abscess n = 2, stoma dysfunction n = 1). Regarding scars, 68% (n = 28) of parents and patients reported the maximal score of 5/5 for global satisfaction and 63% (n = 26) had all scars hidden by underwear. SLIP approach is versatile and can be used in supra- and infra-mesocolic robotic-assisted procedures. The low complication rate shows its safety in both young children and teenagers and does not increase operative difficulties. It results in high patient satisfaction regarding scars, and a scarless abdomen.

The effects of text reminders on the use of family planning services: evidence from a randomised controlled trial in urban Mozambique.

INTRODUCTION: Reduction of unmet need for contraception is associated with enhanced health outcomes. We conducted a randomised controlled trial in Mozambique analysing the effects of text messages encouraging use of family planning services. METHODS: This trial was conducted within a sample of women served by the Integrated Family Planning Program implemented by Population Services International, in which community health workers provide clinic referrals for family planning services. The evaluation enrolled 5370 women between 20 January and 18 December 2020 who received a referral, reported access to a mobile phone and provided consent. Women were randomly assigned to a treatment group that received a series of text message reminders encouraging them to visit a clinic or to a control arm. An intention-to-treat analysis was conducted to analyse the effect of reminders on the probability of a clinic visit and contraceptive uptake. The final analysis includes 3623 women; 1747 women were lost to follow-up. RESULTS: Women assigned to receive the text reminders are weakly more likely to visit a clinic (risk difference 2.3 percentage points, p=0.081) and to receive a contraceptive method at a clinic (2.2 percentage points, p=0.091), relative to a base rate of 48.0% and 46.9%, respectively. The effect on clinic visits is larger and statistically significant in the prespecified subsample of women enrolled prior to the COVID-19-related state of emergency (3.2 percentage points, p=0.042). CONCLUSION: Evidence from this trial suggests that text message reminders are a promising nudge that increases the probability that women receive contraception. TRIAL REGISTRATION NUMBER: AEARCTR-0005383.

Enhancing recovery after minimally invasive surgery in children: A systematic review of the literature and meta-analysis.

OBJECTIVE: Enhanced recovery after surgery (ERAS) has been widely implemented after minimally invasive surgeries (MIS) in adults. The aim of this study was to evaluate the current evidence available on ERAS after MIS in children. METHODS: Using a defined search strategy (PubMed, Cochrane, Scopus), we performed a systematic review of the literature, searching for studies reporting on ERAS after MIS (thoracoscopy, laparoscopy, retroperitoneoscopy) in children (1975-2019). This study was registered with PROSPERO-international prospective register of systematic reviews. A meta-analysis was conducted using comparative studies for length of stay (LOS), complication rates, and readmission rates. RESULTS: Of 180 abstracts screened, 20 full-text articles were analyzed, and 9 were included in our systematic review (1 randomized controlled trial, 3 prospective, and 5 retrospective studies), involving a total number of 531 patients. ERAS has been applied to laparoscopy for digestive (n = 7 studies) or urologic surgeries (n = 1), as well as thoracoscopy (n = 1). Mean LOS was decreased in ERAS children compared to controls (6 studies, -1.12 days, 95%IC: -1.5 to -0.82, p < 0.00001). There was no difference in complication rates between ERAS children and control children (5 studies, 13% vs 14%, OR = 0.84, 95%CI: 0.49-1.44, p = 0.52). The 30-day readmission rate was decreased in ERAS children compared to controls (6 studies, 4% vs 10%, OR = 0.34, 95%CI: 0.18-0.66, p = 0.001). CONCLUSIONS: Although the evidence regarding ERAS in MIS is scarce, these protocols seem safe and effective, by decreasing LOS and 30-day readmission rate, without increasing post-operative complication rates.

Short-term effects of the COVID-19 state of emergency on contraceptive access and utilization in Mozambique.

The COVID-19 pandemic has increasingly disrupted the global delivery of preventive health care services, as a large number of governments have issued state of emergency orders halting service delivery. However, there is limited evidence on the realized effects of the pandemic and associated emergency orders on access to services in low-income country contexts to date. To address this gap, this paper analyzes administrative data on utilization of contraceptive health services by women referred via community health promoters in two large urban and peri-urban areas of Mozambique. We focus on the period immediately surrounding the national state of emergency declaration linked to the COVID-19 pandemic on March 31, 2020. Data reported for 109,129 women served by 132 unique promoters and 192 unique public health facilities is analyzed using logistic regression, interrupted time series analysis and hazard analysis. The results demonstrate that the imposition of the state of emergency is associated with a modest short-term drop in both service provision and utilization, followed by a relatively rapid rebound. We conclude that in this context, the accessibility of reproductive health services was not dramatically reduced during the first phase of the pandemic-related emergency.

Redo Laparoscopic Pyeloplasty in Infants and Children: Feasible and Effective.

Purpose: To determine the feasibility and effectiveness of redo laparoscopic pyeloplasty among patients with failed previous pyeloplasty, specifically examining rates of success and complications. Materials and Methods: We retrospectively reviewed the charts of all patients, who underwent redo laparoscopic pyeloplasty from 2006 to 2017. This included patients who underwent primary pyeloplasty at our institution and those referred for failures. Analysis included demographics, operative time, complications, length of hospital stay, complications, and success. Success was defined as improvement of symptoms and hydronephrosis and/or improvement in drainage demonstrated by diuretic renogram, especially in those with persistent hydronephrosis. Descriptive statistics are presented. Results: We identified 22 patients who underwent redo laparoscopic pyeloplasty. All had Anderson-Hynes technique except two cases in which ureterocalicostomy was performed. Median (IQR) follow-up was 29 (2-120) months, median time between primary pyeloplasty and redo laparoscopic pyeloplasty was 12 (7-49) months. The median operative time was 200 (50-250) min, and median length of hospital stay was 3 (2-10) days. The procedure was feasible in all cases without conversion. During follow-up, all but two patients demonstrated an improvement in the symptoms and the degree of hydronephrosis. Ninety-one percent of patients experienced success and no major complications were noted. Conclusions: Redo laparoscopic pyeloplasty is feasible and effective with a high success rate and low complication rate.

Thoracoscopy Versus Thoracotomy in the Repair of Esophageal Atresia with Distal Tracheoesophageal Fistula.

Background/Objective: Thoracoscopic repair of esophageal atresia (EA) is gaining popularity, but it is a highly technically demanding procedure. The aim of our study is to evaluate our outcomes in the management of type C EA comparing the thoracoscopic and the open (thoracotomy) approaches. Methods: This is a retrospective bicentric study of two major pediatric surgery centers, reviewing all the patients operated for EA with distal tracheoesophageal fistula. Only patients who underwent primary anastomosis were included. From 2008 to 2018, 187 patients were included. Results: Forty-seven patients were operated thoracoscopically (TS group) and 140 by the open approach (TT group). Mean gestational age was 38 ± 2.4 weeks in TS group and 36.4 ± 3.3 weeks in TT group (P = .005) with a mean birth weight of 2785 ± 654 g and 2404.9 ± 651 g in TS and TT groups, respectively (P = .003). The mean operative time was 127.6 ± 35 minutes in TS group and 105.7 ± 23 minutes in TT group (P = .0005). The mean postoperative ventilation time and the mean length of stay were significantly shorter in the thoracoscopic group (P = .004 and P < .0001, respectively). The incidence of anastomotic leak was 8.9% in TS group versus 16.4% in TT group (P = .33). Anastomotic stenosis occurred in 33.3% of TS group and in 22.4% of TT group (P = .17). Conclusions: Surgical outcome of thoracoscopic repair of EA is comparable to the open repair with no higher complication rate with the expected skeletal and cosmetic benefits. However, possible bias regarding prematurity, weight at surgery, and associated anomalies must be taken into consideration.

The role of a pediatric tertiary care center in avoiding collateral damage for children with acute appendicitis during the COVID-19 outbreak.

PURPOSE: To evaluate the impact of the COVID-19 pandemic-related lockdown on management and outcomes of children with acute appendicitis. METHODS: A retrospective cohort study was conducted, including children treated for acute appendicitis (January 20th-May 11th, 2020). The data regarding the severity of appendicitis and outcome were collected and compared for two time periods, before and after the nationwide lockdown (March 17th, 2020). RESULTS: The number of cases of acute appendicitis increased by 77% during the lockdown (n = 39 vs. n = 69, p = 0.03). During the lockdown, children treated for appendicitis were older (11.1 vs. 8.9 years, p = 0.003), and were more likely to live more than 5 km away from our institution (77% vs. 52%, p = 0.017). Less children had previously consulted a general practitioner (15% vs. 33%, p = 0.028), whereas more children were transferred from other hospitals (52% vs. 31%, p = 0.043). There was no difference in terms of length of hospital stay, rate of postoperative intra-abdominal abscess, ER visits, and readmissions between both periods. Three children (4%) were diagnosed with COVID-19 and appendicitis. CONCLUSIONS: Despite an increase in the number of children with appendicitis managed at our hospital during the COVID-19-related lockdown, management, and outcome remained similar. Although our pediatric center was strongly affected by this pandemic, maintaining our prior practice strategies for acute appendicitis avoided the occurrence of collateral damage for those children.

Mowat Wilson syndrome and Hirschsprung disease: a retrospective study on functional outcomes.

AIM OF THE STUDY: Mowat Wilson syndrome (MWS) is a complex genetic disorder due to mutation or deletion of the ZEB2 gene (ZFHX1B), including multiple clinical features. Hirschsprung disease is associated with this syndrome with a prevalence between 43 and 57%. The aim of this study was to demonstrate the severe outcomes and the high complication rates in children with MWS, focusing on their complicated follow-up. METHODS: A retrospective comparative study was conducted on patients referred to Robert-Debré Children's Hospital for MWS from 2003 to 2018. Multidisciplinary follow-up was carried out by surgeons, geneticists, gastroenterologists, and neurologists. Data regarding patient characteristics, surgical management, postoperative complications, and functional outcomes were collected. RESULTS: Over this period of 15 years, 23 patients were diagnosed with MWS. Hirschsprung disease was associated with 10 of them (43%). Of these cases, two patients had recto-sigmoïd aganglionosis (20%), three had aganglionic segment extension to the left colic angle (30%), two to the right colic angle (20%), and three to the whole colon (30%). The median follow-up was 8.5 years (2 months-15 years). All patients had seizures and intellectual disability. Six children (60%) presented with cardiac defects. At the last follow-up, three patients still had a stoma diversion and 7 (70%) were fed orally. One patient died during the first months. Eight (80%) of these children required a second surgery due to complications. At the last follow-up, three patients reported episodes of abdominal bloating (42%), one recurrent treated constipation (14.3%), and one soiling (14.3%). Genetic analysis identified three patients with heterozygous deletions, three with codon mutations, and three with frameshift mutations. CONCLUSIONS: MWS associated with Hirschsprung disease has a high rate of immediate surgical complications but some patients may achieve bowel function comparable with non-syndromic HD patients. A multidisciplinary follow-up is required for these patients. LEVEL OF EVIDENCE: Retrospective observational single cohort study, Level 3.

Association of Maternal First Trimester Serum Levels of Free Beta Human Chorionic Gonadotropin and Hypospadias: A Population Based Study.

PURPOSE: Human chorionic gonadotropin stimulates fetal testosterone production and contributes to normal development of male genitalia. Using population based data we hypothesized that differences in maternal free beta human chorionic gonadotropin may be associated with hypospadias. MATERIALS AND METHODS: Data were obtained from the Paris Registry of Congenital Malformations (REMAPAR) (2011 to 2016). The initial study population included 3,172 pregnant women who gave birth to a singleton live born male infant with a congenital malformation. After exclusion of cases with unknown beta human chorionic gonadotropin and those with chromosomal or genetic abnormalities, the study population included 194 boys with isolated hypospadias and 1,075 controls. For cases with operative notes (125) we obtained data on type (proximal/distal) of hypospadias. Using quantile regression we compared median values of multiple of median beta human chorionic gonadotropin measured for first trimester Down syndrome screening (10th to 13th gestational weeks) for overall as well as by type of hypospadias vs controls. We also considered possible effects of placental dysfunction (maternal age, intrauterine growth retardation and preterm births) as potential confounding factors. RESULTS: Overall the median beta human chorionic gonadotropin multiple of median was comparable for women who had an infant with hypospadias vs controls (0.99 vs 0.95, p=0.3). However, proximal hypospadias was associated with a statistically significant higher median multiple of median than distal hypospadias or unspecified (1.49 vs 0.92 vs 1.05, p=0.02). The estimates were comparable after adjustment for placental dysfunction. CONCLUSIONS: Our findings support the hypothesis that an alteration in maternal beta human chorionic gonadotropin levels is associated with hypospadias. However, this association appears to be limited to proximal hypospadias.

A Successful Renal Transplant in a Pediatric Patient With Glanzmann Thrombasthenia and Hyperimmunization.

We report the case of a patient with type 2 Glanzmann thrombasthenia who underwent successful kidney transplant with his mother's kidney. He started dialysis at 13 months. The patient had been diagnosed with Glanzmann thrombasthenia at 9 years old, after hemorrhagic shock, during which multiple transfusions were required and hyperimmunization had developed. At 12 years old, he received a kidney transplant. Before transplant, ABO- and HLA-compatible platelet donors were identified and convened to donate forthe surgery and in case of emergency. Bleeding was prevented withprophylacticHLA-matched platelet transfusion and tranexamic acid. After transplant, diuresis started immediately with excellent graft function and no severe bleeding. However, after week 5, several episodes of macroscopic hematuria occurred, with obstruction and anuria. The double J ureteric stent was replaced 4 times in 2 months. Finally, the ureteric stent was removed 9 months later. At 22 months after kidney transplant, the patient has a normal graft function and no further bleeding has occurred, underlying the importance of multidisciplinary management.

Reflective Practice About Retroperitoneal Laparoscopy in Comparison to Open Surgery for Ureteropelvic Junction Obstruction Repair in Children Less Than 1 Year of Age.

Introduction: The interest in laparoscopy in the treatment of ureteropelvic junction obstruction (UPJO) in children under 12 months of age remains controversial. The aim of this study is to evaluate feasibility and benefits of retroperitoneal laparoscopy (RL) compared to open surgery in this age group. Materials and Methods: Between January 2012 and May 2017, we performed 222 pyeloplasties: 144 by laparoscopy and 78 by open surgery. From 2012, the choice of operative technique was decided according to the laparoscopic experience of the surgeon; two surgeons operated laparoscopically on all children <12 months of age, while others operated using posterior lumbotomy (PL). The RL is standardized and performed by 3 trocars (5, 3, 3). Pre, per and postoperative parameters were analyzed retrospectively. Statistical tests: Pearson, Fisher, Student and Mann-Whitney. Results: During this 5-year period, 24 RL and 53 PL were included with a median follow-up of 27 months (5-63). In the LR group, postoperative drainage was performed by JJ (13 cases) and external stent (11 cases). No conversion has been listed in this group. In each group there was one failure that needed redo pyeloplasty. Duration of hospitalization and intravenous acetaminophen use were significantly lower in the RL group (2.8 vs. 2.3 days, p = 0.02, respectively) while operating time was significantly longer (163 vs. 85.8 min, p = 0.001). The postoperative complication rate was statistically identical in each group (urinary tract infection, wall hematoma, hematuria…). Conclusion: RL is feasible in children under 1 year of age in the hands of well-experienced surgeons with longer operative time but without added morbidity. Subject to the retrospective nature of our study, the RL seems to offer a benefit regarding duration of hospitalization and analgesics consumption.

Voiding cystography: an unusual route of induced hypothyroidism by iodine overdose in two newborns with chronic kidney disease.

BACKGROUND: Iatrogenic induced hypothyroidism had been described in newborns and more particularly in preterm infants after cutaneous or intravenous exposure to iodine. CASE-DIAGNOSIS : We reported a new risk of iodine intoxication with the cases of two newborns who developed hypothyroidism after intra vesical iodine injection during a cystography, which was performed to confirm antenatal diagnosis of posterior urethral valves (PUV). The newborns both developed transient hypothyroidism due to an iodine overdose. CONCLUSIONS: These two observations suggest that voiding cystourethrography (VCUG) should be carefully considered in newborns with severe uropathy, particularly in the case of renal insufficiency. If indicated, thyroid function should be monitored in the following weeks, and in case of hypothyroidism treatment should be started.