Latitudes and attitudes: A multinational study of laparoscopic pyeloplasty in children.

PURPOSE: The Anderson-Hynes technique has been the treatment of choice for primary ureteropelvic junction obstruction in children. Laparoscopic approach has shown similar outcomes to open, with advantages of shorter hospital stay and less pain. We reviewed the experience of 11 geographically diverse, tertiary pediatric urology institutions focusing on the outcomes and complications of laparoscopic pyeloplasty. MATERIALS AND METHODS: A descriptive, retrospective study was conducted evaluating patients undergoing Anderson-Hynes dismembered laparoscopic pyeloplasty. Centers from four different continents participated. Demographic data, perioperative management, results, and complications are described. RESULTS: Over a 9-year period, 744 laparoscopic pyeloplasties were performed in 743 patients. Mean follow-up was 31 months (6-120m). Mean age at surgery was 82 months (1 w-19 y). Median operative time was 177 min. An internal stent was placed in 648 patients (87%). A catheter was placed for bladder drainage in 702 patients (94%). Conversion to open pyeloplasty was necessary in seven patients. Average length of hospital stay was 2.8 days. Mean time of analgesic requirement was 3.2 days. Complications, according to Clavien-Dindo classification, were observed in 56 patients (7.5%); 10 (1%) were Clavien-Dindo IIIb. Treatment failure occurred in 35 cases with 30 requiring redo pyeloplasty (4%) and 5 cases requiring nephrectomy (0.6%). CONCLUSION: We have described the laparoscopic pyeloplasty experience of institutions with diverse cultural and economic backgrounds. They had very similar outcomes, in agreement with previously published data. Based on these findings, we conclude that laparoscopic pyeloplasty is safe and successful in diverse geographics areas of the world.

Distinguishing outcomes of neonatal intestinal volvulus: Review of our experience over the last 20 years.

AIM: There are two types of intestinal volvulus: midgut (MGV) and segmental (SV). Patients with different types of intestinal volvulus are often included in the same case series, which may affect the perception of how severe "intestinal volvuli" are. We aimed to compare both types of intestinal volvulus. METHODS: This is a retrospective observational study including all patients with MGV and SV up to 28 days of life admitted to a tertiary hospital in Spain over a 20-year-period (1999-2019). A comparison between groups and a logistic regression model for mortality were done. RESULTS: We identified 32 patients: 23 MGV and 9 SV. Malrotation was exclusive of MGV. Prenatal diagnosis, cystic fibrosis, and intestinal resection were significantly more frequent in SV. Surgery was performed at a significantly lower age in SV. The mortality observed in acute MGV with intestinal compromise (41.7%) is four times higher than the mortality of SV (11.1%). The overall mortality of all MGV patients (21.7%) is almost twice that of SV. Mortality was best predicted by the presence of hemodynamic instability (OR 27.5 95% CI 2.50-302.17; p = 0.007). CONCLUSION: SV and MGV have a different clinical presentation. Hemodynamic instability is the major risk factor for death.

Expression of Protein SOX9 in Biliary Atresia.

OBJECTIVES: Biliary atresia (BA) is still an enigmatic disease. Deeper knowledge of its pathophysiology could help develop better treatments. SOX9 regulates bile duct development, liver regeneration and fibrosis; therefore, it could be determinant in characterizing BA liver damage. Aim: To study if there is a SOX9 expression pattern in liver biopsies from BA patients. METHODS: Liver biopsies from BA patients (group BA), from age-matched infants without primary hepatic disease (group Control), and from patients with other liver conditions (group OLC) were compared. Expression of SOX9 was checked for: amount, intensity of immunoreaction, localization within ductular structures, perifibrotic epithelial cells, and lobular cells. The scores were added to create a scale from 0 to 11 that allowed group comparison. SOX9 Scale and liver survival were also looked for a correlation. RESULTS: All BA cases had a score >4, while all controls scored <4. OLC livers scored 1 to 8 (3.5 ±â€Š2.0) (P < 0.001 between all groups). A cut-off at 4 had 100% sensitivity and 88.24% specificity to differentiate BA from Controls and from OLC (area under receiver operating characteristic curve: 0.9989 (95% confidence interval: 0.9964-1.000). Strong expression of SOX9 was observed mainly in the nuclei of proliferated ductules of portal spaces and fibrotic bridges. SOX9 Scale score could not be related to liver survival in this study. CONCLUSION: In BA livers, SOX9 is mainly expressed in reactive ductular epithelium, following a pattern significantly different from that seen in non-BA patients; thus, SOX9 Scale may have a role in the diagnosis of BA.

Comparison between mini-laparoscopy, conventional laparoscopy and open approach for ureteropelvic junction obstruction treatment in children.

INTRODUCTION: The aim of the study was to compare 3 mm mini-laparoscopy (mini LP), standard 5 mm laparoscopy (LP) and open surgery for pediatric pyeloplasty in a single center. METHODS: Patients who underwent pyeloplasty from 1997 to 2017 at Hospital Sant Joan de Déu were prospectively collected. Demographic data, clinical, surgical and radiological variables were assessed. A multivariate logistic regression analysis was performed in order to identify risks for surgical complications, urinary leak and need for redo-surgery. RESULTS: 340 pyeloplasties were performed in this period: 197 open, 30 LP and 113 mini LP. Independent risk factors for surgical complications in a multivariate logistic regression model were: LP (vs mini LP, OR = 3.95; 95% CI: 1.13-13.8), higher differential renal function (each point more increases the risk 6%; 95% CI: 1-11%), older children (every year increases the risk 1.11 times; 95% CI: 1.002-1.225). Open surgery, pelvis diameter or the use of different stents were not risk factors. This model had an 80% PPV and a 92% NPV. LP (OR = 4.65; 95% CI: 1.08-19.96) and longer surgical time (OR = 1.014; 95% CI: 1.003-1.025) were independent risk factors for urinary leak. Higher pelvis diameter (OR = 0.93; 95% CI: 0.87-0.99) and the use of external stents were independent protective risk factors for urinary leak (OR = 0.09; 95% CI: 0.01-0.72). We have not found independent risk factors for redo-surgery in a multivariate logistic regression model. CONCLUSION: mini LP can be safely and effectively used to perform pyeloplasty in pediatric patients of all ages.

Porcine dermal patches as a risk factor for recurrence after congenital diaphragmatic hernia repair.

PURPOSE: Recurrence of congenital diaphragmatic hernia (CDH) is a treatment-related morbidity which can be preventable. There is no consensus about the ideal material for diaphragmatic substitution. The aim of our study is to identify if the use of porcine dermis patches increases the risk of CDH recurrence. METHODS: Retrospective review of medical records of CDH patients treated between 2013 and 2017 in our center was carried out. Demographic, clinical and surgical variables were collected. Regression analysis was performed to identify which factors increase the risk of recurrence. RESULTS: 50 patients entered the study. 94% of the patients had a left CDH, mean observed/expected lung-to-head ratio was 46%. 17 patients underwent a primary closure, the rest a patch closure: 25 Gore-Tex® and 8 porcine dermis patches were used. Seven patients presented recurrence (14%). Median follow-up time was 3.5 years (1.2-6.2). Univariate analysis revealed that the use of a porcine dermis patch (75%) increased the risk of recurrence compared with Gore-Tex® patch (4%) and primary closure (0%) p < 0.001 (HR 58.7; IC 95%: 6.9-501.2; p < 0.001). CONCLUSION: The main risk factor for CDH recurrence is the use of a porcine dermis patch. We do not recommend the use of these patches for CDH repair.

Bilateral hydrosalpinx in patients with Hirschsprung's disease.

PURPOSE: Hirschsprung's disease (HD) is uncommon in females. There are very few reports on the patients' obstetric and gynecological outcome. Hydrosalpinx causes pain and infertility. It is rare in nonsexually active teenagers. It may be because of an intrinsic disease of the fallopian tubes or secondary to surgery. AIM: to describe the relationship between hydrosalpinx and HD or its surgical approach; to report the impact of bilateral hydrosalpinx on fertility in HD. METHODS: The records of all females with HD since 1980 were reviewed. Only patients who reached menarche were included. Prevalence of hydrosalpinx and hydrosalpinx-free survival were compared after abdominoperineal (A) or transanal (T) surgery. Treatment for hydrosalpinx was reviewed. RESULTS: Seventeen out of 27 patients had reached menarche (Group A: 13 patients; Group T: 4 patients). Five patients in group A and none in group T presented bilateral hydrosalpinx (p=0.261). There were no statistical differences in hydrosalpinx-free survival between groups (p=0.344). Hydrosalpinx treatment: two bilateral and one unilateral salpingectomy, one pyosalpinx evacuation and one untreated. Three patients had conception desire: one has children; two are on IVF program. CONCLUSION: An association between hydrosalpinx and HD was observed. The development of hydrosalpinx was not associated with surgical approach in our study. Females with HD should have a gynecological follow-up for the development of hydrosalpinx, which can impair fertility. LEVEL OF EVIDENCE: Level III, retrospective comparative study.