One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register.

OBJECTIVE: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. STUDY DESIGN: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. RESULTS: A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year. CONCLUSIONS: Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.

Staged laparoscopic orchiopexy of intra-abdominal testis: Spermatic vessels division versus traction? A multicentric comparative study.

BACKGROUND: Staged laparoscopic management of intra-abdominal testes using pedicular section is recognized as gold standard technique, successful in 85 % of cases for scrotal testicular position with less than 10 % testicular atrophy. Recently, Shehata proposed a new technique without pedicular division for these testes, using spermatic vessels traction, but did not provide a comparative study of the two techniques. OBJECTIVE: To evaluate the laparoscopic spermatic pedicular traction (Shehata technique, ST) for the treatment of intra-abdominal testis, as an alternative to gold standard pedicular section (2-stage Fowler-Stephens, FS). STUDY DESIGN: Intra-abdominal testes of 129 patients in two tertiary pediatric urology centers were managed laparoscopically (2011-2019) either by 2-stage FS orchidopexy or ST according to the surgeon preference. Testicular position and size were statistically compared. RESULTS: A total of 147 testes were pulled down by 80 ST and 67 FS, including 18 bilateral cases. Median (IQR) age at surgery was 24.2 (15.6-46.4) months (ST) and 18.3 (13.1-38.2) months (FS) (p = 0.094). Scrotal pulling-down of the testis was performed after a median (IQR) period of 2.3 (1.6-3.4) months (ST) and 6.1 (4.7-8.3) months (FS), respectively (p < 0.005). Although ST had collapsed in 17 cases (21.3 %), only one (1.3 %) redo procedure was required. After a median (IQR) follow-up of 22 (12-40) and 19 (8.75-37) months (p = 0.59), the testis was in the scrotum in 85 % and 81 % of ST and FS cases, respectively (p = 0.51). Testicular atrophy occurred in 10 % of ST and 13.4 % of FS (p = 0.61). Multivariate analysis using the propensity score analysis did not identify any difference between the two techniques. DISCUSSION: Our results seem to confirm that FS and ST achieve the same results regarding final testicular position and testicular atrophy rate, with a long-term follow-up. Our study supports pediatric surgeons to favor laparoscopic spermatic pedicular traction (ST) which preserves the testicular vascularization and may ensure better spermatogenesis after puberty. More details on the size and position of the testicle at the beginning of the first laparoscopy seem however essential to assess more accurately the outcomes of each surgical technique. Our outcomes will also be re-evaluated when our patients have reached puberty, from an exocrine and endocrine points of view. CONCLUSIONS: This study showed similar results after laparoscopic traction or section of spermatic vessels for intra-abdominal testis in a long-term follow-up, providing more evidence for the use of ST as a valuable alternative to FS.

Evaluating pediatric ureteropelvic junction obstruction: Dynamic magnetic resonance urography vs renal scintigraphy 99m-technetium mercaptoacetyltriglycine.

BACKGROUND: Ureteropelvic junction obstruction (UPJO) is a common congenital urinary tract disorder in children. It can be diagnosed as early as in utero due to the presence of hydronephrosis or later in life due to symptomatic occurrence. AIM: To evaluate the discrepancy between dynamic contrast-enhanced magnetic resonance urography (dMRU) and scintigraphy 99m-technetium mercaptoacetyltriglycine (MAG-3) for the functional evaluation of UPJO. METHODS: Between 2016 and 2020, 126 patients with UPJO underwent surgery at Robert Debré Hospital. Of these, 83 received a prenatal diagnosis, and 43 were diagnosed during childhood. Four of the 126 patients underwent surgery based on the clinical situation and postnatal ultrasound findings without undergoing functional imaging evaluation. Split renal function was evaluated preoperatively using scintigraphy MAG-3 (n = 28), dMRU (n = 53), or both (n = 40). In this study, we included patients who underwent surgery for UPJO and scintigraphy MAG-3 + dMRU but excluded those who underwent only scintigraphy MAG-3 or dMRU. The patients were divided into groups A (< 10% discrepancy) and B (> 10% discrepancy). We examined the discrepancy in split renal function between the two modalities and investigated the possible risk factors. RESULTS: The split renal function between the two kidneys was compared in 40 patients (28 boys and 12 girls) using scintigraphy MAG-3 and dMRU. Differential renal function, as determined using both modalities, showed a difference of < 10% in 31 children and > 10% in 9 children. Calculation of the relative renal function using dMRU revealed an excellent correlation coefficient with renal scintigraphy MAG-3 for both kidneys. CONCLUSION: Our findings demonstrated that dMRU is equivalent to scintigraphy MAG-3 for evaluating split renal function in patients with UPJO.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication. (3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair; METHODS: This prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion. RESULTS: Of 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0-5.0). (1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p < 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not. (2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p < 0.0001), patch repair (p < 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis. (3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03). CONCLUSIONS: Children undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children. LEVEL OF EVIDENCE: II - Prospective Study.

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.

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.

Retroperitoneal laparoscopic pyeloplasty in children: long-term outcome and critical analysis of 10-year experience in a teaching center.

BACKGROUND: Laparoscopic pyeloplasty in children remains controversial and is not included in most pediatric urology centers because of technical difficulties and lack of long-term results. OBJECTIVE: To critically analyze our 10-yr experience with the retroperitoneal approach (RA), with a particular interest on the impact of the learning curve in a teaching center. DESIGN, SETTING, AND PARTICIPANTS: Patients who underwent pyeloplasty between 1999 and 2010 at our institution were reviewed (n=390). The diagnosis of ureteropelvic junction obstruction was confirmed by ultrasound and technetium Tc 99m mercaptoacetyltriglycine-3 renal scan or magnetic resonance imaging; the same criteria were used to evaluate the outcome. The lateral RA was selected in children >1 yr of age without abnormal migration or fusion of the kidney (n=104). SURGICAL PROCEDURE: Dismembered pyeloplasty and anastomosis were performed using running monofilament 5-0 or 6-0 absorbable suture. All were drained by double-J stent except 20 cases drained by external transanastomotic stent. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: We assessed intra- and postoperative morbidity and analyzed the teaching of technique and learning curve. Data are expressed as medians and interquartile range (25th, 75th percentiles) for quantitative variables. RESULTS AND LIMITATIONS: Median age was 6.2 yr (2.2-10.3). Thirty-three patients had crossing vessels. Median operative time was 185 min (160-235). Median hospital stay was 2 d (1-2). Redo pyeloplasty was needed in only two children (2%). Median follow-up was 2.1 yr (1.4-4.1). Operative time was <3 h after 35 cases. After 30 cases performed by the same surgeon, standardization of the technique was feasible, which helped in the teaching process because 50% of the final 30 cases were done by trainees. CONCLUSIONS: Retroperitoneal dismembered laparoscopic pyeloplasty is a safe, reliable, and efficient procedure with an excellent outcome in selected children according to their indications and age, and the experience of the surgical team. Even if the transmission to trainees is successful, it is still a long learning process and remains a challenging task for a teaching center.

Duhamel operation vs neonatal transanal endorectal pull-through procedure for Hirschsprung disease: which are the changes for pathologists?

BACKGROUND: The aim of this study was to evaluate whether performing definitive surgery for Hirschsprung disease (HD) in neonatal period with a transanal endorectal pull-through (TEPT) procedure had modified our diagnostic relevance, particularly during intraoperative frozen sections (IOFS), compared to classic Duhamel (DH) surgery performed in older children. METHODS: We collected pathologic data for 47 children who underwent surgery for neonatal nontotal HD over a 5-year period. RESULTS: Twenty-nine patients underwent TEPT and 18 the DH operation. Mean age at operation was 19 days for TEPT and 4 months for DH operation. The mean number of IOFS was 2.6 for TEPT and 2.4 for DH operation. Gross examination could be fully completed in all TEPT cases, but was incomplete in 5 DH cases. The average total lengths of bowel, and aganglionic, transitional, and ganglionic segments were 12.3, 7.3, 3, and 2 cm for TEPT, and 17.6, 9.3, 3.5, and 4.8 cm for DH operation, respectively. Discordance between IOFS and paraffin-section analysis occurred in 5 cases (3 TEPT and 2 DH operation). CONCLUSION: When TEPT was used, the gross examination and sampling was more accurate, leading to a clearer pathology report. The TEPT procedure facilitates the work of the pathologist without modifying the results of IOFS, if some precautions are taken.

Cytomegalovirus infection as a possible underlying factor in neonatal surgical conditions.

BACKGROUND: The seroprevalence of cytomegalovirus (CMV) in France is about 38%. Fetal contamination during pregnancy is 40%. We report a series of gastrointestinal conditions associated with CMV in neonates. PATIENTS AND METHOD: This is a retrospective study on neonates operated on for gastrointestinal conditions in our institution between January 1998 and December 2002. Only children with suspected CMV infection (characteristic nuclear inclusions) on pathologic examination were reviewed. We analyzed the age of onset, the clinical and radiologic presentation, and the serologic findings in mother and child. RESULTS: The study included 3 boys and 2 girls. The average gestational age was 34.4 weeks (range, 28-39 weeks) and the average birth weight was 2364 g (range, 790-3580 g). Two infants had necrotizing enterocolitis, 2 had pathologic condition related to Meckel's diverticulum (volvulus and perforation, respectively), and 1 had distal ileal atresia. All surgical specimens showed characteristic CMV nuclear inclusions. Maternal serologic studies were positive for CMV in only 1 case with CMV IgM detected in serum. Two babies had a positive serological finding for CMV with detection of CMV IgM, and 2 had CMV isolated on urinary viral culture (one had both). CONCLUSION: Cytomegalovirus is a viral agent that may be involved in surgical pathology in neonates. To confirm the causal relationship between CMV and these pathologic findings, a large-scale longitudinal prospective screening of CMV in neonates with intestinal conditions should be performed.

Retroperitoneal laparoscopic versus open pyeloplasty in children.

PURPOSE: The indications for laparoscopy in pediatric urology are expanding and yet the advantages over open surgery remain unclear. We compared the results of retroperitoneal laparoscopic vs open pyeloplasty for pyeloureteral junction obstruction in children. MATERIALS AND METHODS: A total of 22 children with a mean age of 88 months (range 25 to 192) underwent laparoscopic dismembered pyeloplasty via the retroperitoneal approach. An additional 17 children with a mean age of 103 months (range 37 to 206) underwent similar procedures via open surgery through a flank incision. We retrospectively analyzed and compared operative time, the use of analgesics (acetaminophen or morphine derivatives) and hospital stay. RESULTS: The 2 groups were similar in mean age and weight at surgery. Mean operative time was significantly shorter in the open surgery vs the laparoscopy group (96 minutes, range 50 to 150 vs 219, range 140 to 310, p <0.0001). Mean postoperative use of acetaminophen (1.9 vs 3.22 days, p = 0.03) and morphine derivatives (1.9 vs 3.06 days, p not significant) was less in the laparoscopy group. Mean hospital stay was shorter in the laparoscopy group than in the open surgery group (2.4 days, range 1 to 5 vs 5, range 3 to 7, p <0.0001). Mean followup was 21 (range 12 to 51) and 24 months (range 12 to 60) in the open and laparoscopy groups, respectively. CONCLUSIONS: The operative time of laparoscopic pyeloplasty remains significantly longer than that of the open procedure in children. The main advantage of the laparoscopic approach is that it significantly decreases hospital stay compared with that after an open procedure. Although in our study analgesic use was less after laparoscopy, our results should be confirmed by a prospective, randomized study.