European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis.

BACKGROUND: The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care. METHOD: A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations. RESULTS: The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection. RECOMMENDATIONS: The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi's approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.

Long-Term Evaluation of the Shape of the Reconstructed Diaphragm in Patients with Left-Sided Congenital Diaphragmatic Hernia Using Serial Chest Radiographs and Correlation to Further Complications.

Background: Defining risk factors for long-term comorbidities in patients after neonatal repair of congenital diaphragmatic hernia (CDH) is an important cornerstone of the implementation of targeted longitudinal follow-up programs. Methods: This study systematically assessed serial chest radiographs of 89 patients with left-sided CDH throughout a mean follow-up of 8.2 years. These geometrical variables for the left and right side were recorded: diaphragmatic angle (LDA, RDA), diaphragmatic diameter (LDD, RDD), diaphragmatic height (LDH, RDH), diaphragmatic curvature index (LDCI, RDCI), lower lung diameter (LLLD, RLLD) and thoracic area (LTA, RTA). Results: It was demonstrated that the shape of the diaphragm in patients with large defects systematically differs from that of patients with small defects. Characteristically, patients with large defects present with a smaller LDCI (5.1 vs. 8.4, p < 0.001) at 6 months of age, which increases over time (11.4 vs. 7.0 at the age of 15.5 years, p = 0.727), representing a flattening of the patch and the attached rudimentary diaphragm as the child grows. Conclusions: Multiple variables during early follow-up were significantly associated with comorbidities such as recurrence, scoliotic curves of the spine and a reduced thoracic area. Some geometrical variables may serve as surrogate parameters for disease severity, which is associated with long-term comorbidities.

Use of Temporary Double-J Stent Placement for Children With Congenital Hydronephrosis: A Long-Term Single-Center Cohort Study.

OBJECTIVE: To evaluate the utilization of double-J stents in children with congenital hydronephrosis in order to avoid or postpone more invasive surgical intervention. Numerous studies have demonstrated that congenital hydronephrosis caused by ureteropelvic junction obstruction (UPJO) or primary obstructive megaureter (POM) may require a surgical correction in up to 20% of cases. METHODS: All infants with severe hydronephrosis and/or an obstructive pattern on renal scintigraphy that received double-J stent placement between 2010 to 2015 in our center were analyzed. Children were followed regularly with ultrasound and received antibiotic metaphylaxis. Urinary tract infection (UTI) and double-J dislocation were defined as complications. Treatment success was defined as avoidance of surgery and reduction of hydronephrosis to grade 1 or 0 during the observation period. RESULTS: 29 children were included, in these, 34 (23 UPJO, 7 POM, 4 UPJO and POM) treatment attempts were performed. Stent implantation failed in six cases, resulting in 28 double-J stent treatments, of which 19 (69%) were successful within the follow-up period of 20 to 104 months. The most common complications were febrile UTI in 6 of29 cases and double-J dislocation in 3 of 29 cases. CONCLUSION: During the observation period, the success rate of temporary double-J in urodynamically relevant obstruction was relatively high. However, urinary tract infections and a complex disease course due to renal stent dislocation, as well as the need for repeated anesthesia and radiation exposure, should be taken into account. Hence, we do not recommend double-J stents placement in all children with congenital hydronephrosis; it may be useful in selected cases.

Long-Term Evaluation of Gastroesophageal Reflux in Neonates with and without Preventive Anti-reflux Surgery at the Time of Congenital Diaphragmatic Hernia Repair.

One potential comorbidity after congenital diaphragmatic hernia (CDH) is gastroesophageal reflux (GER), which can have a substantial effect on patients' quality of life, thriving, and complications later in life. Efforts have been made to reduce gastroesophageal reflux with a preventive anti-reflux procedure at the time of CDH repair. In this follow-up study of neonates participating in a primary RCT study on preventive anti-reflux surgery, symptoms of GER were assessed longitudinally. Long-term data with a median follow-up time of ten years was available in 66 patients. Thirty-one neonates received an initial fundoplication. Secondary anti-reflux surgery was necessary in 18% and only in patients with large defects. It was required significantly more often in patients with intrathoracic herniation of liver (p = 0.015) and stomach (p = 0.019) and patch repair (p = 0.03). Liver herniation was the only independent risk factor identified in multivariate regression analysis. Primary fundopexy and hemifundoplication did not reveal a protective effect regarding the occurrence of GER symptoms, the need for secondary antireflux surgery or the gain of body weight regardless of defect size neither in the short nor in the long term. Symptoms of GER must be assessed carefully especially in children with large defects, as these are prone to require secondary anti-reflux surgery in the long-term. Routine evaluation of GER including endoscopy and impedance measurement should be recommended especially for high-risk patients.

Small Bowel Obstruction After Neonatal Repair of Congenital Diaphragmatic Hernia-Incidence and Risk-Factors Identified in a Large Longitudinal Cohort-Study.

Objective: In patients with a congenital diaphragmatic hernia (CDH), postoperative small bowel obstruction (SBO) is a life-threatening event. Literature reports an incidence of SBO of 20% and an association with patch repair and ECMO treatment. Adhesions develop due to peritoneal damage and underly various biochemical and cellular processes. This longitudinal cohort study is aimed at identifying the incidence of SBO and the risk factors of surgical, pre-, and postoperative treatment. Methods: We evaluated all consecutive CDH survivors born between January 2009 and December 2017 participating in our prospective long-term follow-up program with a standardized protocol. Results: A total of 337 patients were included, with a median follow-up of 4 years. SBO with various underlying causes was observed in 38 patients (11.3%) and significantly more often after open surgery (OS). The majority of SBOs required surgical intervention (92%). Adhesive SBO (ASBO) was detected as the leading cause in 17 of 28 patients, in whom surgical reports were available. Duration of chest tube insertion [odds ratio (OR) 1.22; 95% CI 1.01-1.46, p = 0.04] was identified as an independent predictor for ASBO in multivariate analysis. Beyond the cut-off value of 16 days, the incidence of serous effusion and chylothorax was higher in patients with ASBO (ASBO/non-SBO: 2/10 vs. 3/139 serous effusion, p = 0.04; 2/10 vs. 13/139 chylothorax, p = 0.27). Type of diaphragmatic reconstruction, abdominal wall closure, or ECMO treatment showed no significant association with ASBO. A protective effect of one or more re-operations has been detected (RR 0.16; 95% CI 0.02-1.17; p = 0.049). Conclusion: Thoracoscopic CDH repair significantly lowers the risk of SBO; however, not every patient is suitable for this approach. GoreTex®-patches do not seem to affect the development of ASBO, while median laparotomy might be more favorable than a subcostal incision. Neonates produce more proinflammatory cytokines and have a reduced anti-inflammatory capacity, which may contribute to the higher incidence of ASBO in patients with a longer duration of chest tube insertion, serous effusion, chylothorax, and to the protective effect of re-operations. In the future, novel therapeutic strategies based on a better understanding of the biochemical and cellular processes involved in the pathophysiology of adhesion formation might contribute to a reduction of peritoneal adhesions and their associated morbidity and mortality.

Neurocardiovascular coupling in congenital diaphragmatic hernia patients undergoing different types of surgical treatment.

BACKGROUND: The effect of peri-operative management on the neonatal brain is largely unknown. Triggers for perioperative brain injury might be revealed by studying changes in neonatal physiology peri-operatively. OBJECTIVE: To study neonatal pathophysiology and cerebral blood flow regulation peri-operatively using the neuro-cardiovascular graph. DESIGN: Observational, prospective cohort study on perioperative neuromonitoring. Neonates were included between July 2018 and April 2020. SETTING: Multicentre study in two high-volume tertiary university hospitals. PATIENTS: Neonates with congenital diaphragmatic hernia were eligible if they received surgical treatment within the first 28 days of life. Exclusion criteria were major cardiac or chromosomal anomalies, or syndromes associated with altered cerebral perfusion or major neurodevelopmental impairment. The neonates were stratified into different groups by type of peri-operative management. INTERVENTION: Each patient was monitored using near-infrared spectroscopy and EEG in addition to the routine peri-operative monitoring. Neurocardiovascular graphs were computed off-line. MAIN OUTCOME MEASURES: The primary endpoint was the difference in neurocardiovascular graph connectivity in the groups over time. RESULTS: Thirty-six patients were included. The intraoperative graph connectivity decreased in all patients operated upon in the operation room (OR) with sevoflurane-based anaesthesia ( P  < 0.001) but remained stable in all patients operated upon in the neonatal intensive care unit (NICU) with midazolam-based anaesthesia. Thoracoscopic surgery in the OR was associated with the largest median connectivity reduction (0.33 to 0.12, P  < 0.001) and a loss of baroreflex and neurovascular coupling. During open surgery in the OR, all regulation mechanisms remained intact. Open surgery in the NICU was associated with the highest neurovascular coupling values. CONCLUSION: Neurocardiovascular graphs provided more insight into the effect of the peri-operative management on the pathophysiology of neonates undergoing surgery. The neonate's clinical condition as well as the surgical and the anaesthesiological approach affected the neonatal physiology and CBF regulation mechanisms at different levels. TRIAL REGISTRATION: NL6972, URL: https://www.trialre-gister.nl/trial/6972 .

MR lung perfusion measurements in adolescents after congenital diaphragmatic hernia: correlation with spirometric lung function tests.

OBJECTIVES: To evaluate whether lung perfusion continues to be reduced in 10-year-old children after congenital diaphragmatic hernia (CDH) and whether lung perfusion values correlate with spirometric lung function measurements. METHODS: Fifty-four patients after CDH repair received dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI)-based lung perfusion measurements at the age of 10 years (10.2 ± 1.0 years). Additionally, a control group of 10 children has been examined according to the same protocol. Lung spirometry was additionally available in 43 patients of the CDH group. A comparison of ipsilateral and contralateral parameters was performed. RESULTS: Pulmonary blood flow (PBF) was reduced on the ipsilateral side in CDH patients (60.4 ± 23.8 vs. 93.3 ± 16.09 mL/100 mL/min; p < 0.0001). In comparison to the control group, especially the ratio of ipsilateral to contralateral, PBF was reduced in CDH patients (0.669 ± 0.152 vs. 0.975 ± 0.091; p < 0.0001). There is a positive correlation between ipsilateral pulmonary blood flow, and spirometric forced 1-s volume (r = 0.45; p = 0.0024). CONCLUSIONS: Pulmonary blood flow impairment persists during childhood and correlates with spirometric measurements. Without the need for ionizing radiation, MRI measurements seem promising as follow-up parameters after CDH. KEY POINTS: • Ten-year-old children after congenital diaphragmatic hernia continue to show reduced perfusion of ipsilateral lung. • Lung perfusion values correlate with lung function tests after congenital diaphragmatic hernia.

Computed tomography based measurements to evaluate lung density and lung growth after congenital diaphragmatic hernia.

Emphysema-like-change of lung is one aspect of lung morbidity in children after congenital diaphragmatic hernia (CDH). This study aims to evaluate if the extent of reduced lung density can be quantified through pediatric chest CT examinations, if side differences are present and if emphysema-like tissue is more prominent after CDH than in controls. Thirty-seven chest CT scans of CDH patients (mean age 4.5 ± 4.0 years) were analyzed semi-automatically and compared to an age-matched control group. Emphysema-like-change was defined as areas of lung density lower than - 950 HU in percentage (low attenuating volume, LAV). A p-value lower than 0.05 was regarded as statistically significant. Hypoattenuating lung tissue was more frequently present in the ipsilateral lung than the contralateral side (LAV 12.6% vs. 5.7%; p < 0.0001). While neither ipsilateral nor contralateral lung volume differed between CDH and control (p > 0.05), LAV in ipsilateral (p = 0.0002), but not in contralateral lung (p = 0.54), was higher in CDH than control. It is feasible to quantify emphysema-like-change in pediatric patients after CDH. In the ipsilateral lung, low-density areas are much more frequently present both in comparison to contralateral and to controls. Especially the ratio of LAV ipsilateral/contralateral seems promising as a quantitative parameter in the follow-up after CDH.

Longitudinal Follow-Up With Radiologic Screening for Recurrence and Secondary Hiatal Hernia in Neonates With Open Repair of Congenital Diaphragmatic Hernia-A Large Prospective, Observational Cohort Study at One Referral Center.

Objective: After neonatal repair of congenital diaphragmatic hernia (CDH) recurrence is the most severe surgical complication and reported in up to 50% after patch implantation. Previous studies are difficult to compare due to differences in surgical techniques and retrospective study design and lack of standardized follow-up or radiologic imaging. The aim was to reliably detect complication rates by radiologic screening during longitudinal follow-up after neonatal open repair of CDH and to determine possible risk factors. Methods: At our referral center with standardized treatment algorithm and follow-up program, consecutive neonates were screened for recurrence by radiologic imaging at defined intervals during a 12-year period. Results: 326 neonates with open CDH repair completed follow-up of a minimum of 2 years. 68 patients (21%) received a primary repair, 251 (77%) a broad cone-shaped patch, and 7 a flat patch (2%). Recurrence occurred in 3 patients (0.7%) until discharge and diaphragmatic complications in 28 (8.6%) thereafter. Overall, 38 recurrences and/or secondary hiatal hernias were diagnosed (9% after primary repair, 12.7% after cone-shaped patch; p = 0.53). Diaphragmatic complications were significantly associated with initial defect size (r = 0.26). In multivariate analysis left-sided CDH, an abdominal wall patch and age below 4 years were identified as independent risk factors. Accordingly, relative risks (RRs) were significantly increased [left-sided CDH: 8.5 (p = 0.03); abdominal wall patch: 3.2 (p < 0.001); age ≤4 years: 6.5 (p < 0.002)]. 97% of patients with diaphragmatic complications showed no or nonspecific symptoms and 45% occurred beyond 1 year of age. Conclusions: The long-term complication rate after CDH repair highly depends on surgical technique: a comparatively low recurrence rate seems to be achievable in large defects by implantation of a broad cone-shaped, non-absorbable patch. Longitudinal follow-up with regular radiologic imaging until adolescence is essential to reliably detecting recurrence to prevent acute incarceration and chronic gastrointestinal morbidity with their impact on prognosis. Based on our findings and literature review, a risk-stratified approach to diaphragmatic complications is proposed.

Cerebral Oxygenation and Activity During Surgical Repair of Neonates With Congenital Diaphragmatic Hernia: A Center Comparison Analysis.

Background and aim: Neonatal brain monitoring is increasingly used due to reports of brain injury perioperatively. Little is known about the effect of sedatives (midazolam) and anesthetics (sevoflurane) on cerebral oxygenation (rScO2) and cerebral activity. This study aims to determine these effects in the perioperative period. Methods: This is an observational, prospective study in two tertiary pediatric surgical centers. All neonates with a congenital diaphragmatic hernia received perioperative cerebral oxygenation and activity measurements. Patients were stratified based on intraoperatively administrated medication: the sevoflurane group (continuous sevoflurane, bolus fentanyl, bolus rocuronium) and the midazolam group (continuous midazolam, continuous fentanyl, and continuous vecuronium). Results: Intraoperatively, rScO2 was higher in the sevoflurane compared to the midazolam group (84%, IQR 77-95 vs. 65%, IQR 59-76, p = < 0.001), fractional tissue oxygen extraction was lower (14%, IQR 5-21 vs. 31%, IQR 29-40, p = < 0.001), the duration of hypoxia was shorter (2%, IQR 0.4-9.6 vs. 38.6%, IQR 4.9-70, p = 0.023), and cerebral activity decreased more: slow delta: 2.16 vs. 4.35 µV 2 (p = 0.0049), fast delta: 0.73 vs. 1.37 µV 2 (p = < 0.001). In the first 30 min of the surgical procedure, a 3-fold increase in fast delta (10.48-31.22 µV 2) and a 5-fold increase in gamma (1.42-7.58 µV 2) were observed in the midazolam group. Conclusion: Sevoflurane-based anesthesia resulted in increased cerebral oxygenation and decreased cerebral activity, suggesting adequate anesthesia. Midazolam-based anesthesia in neonates with a more severe CDH led to alarmingly low rScO2 values, below hypoxia threshold, and increased values of EEG power during the first 30 min of surgery. This might indicate conscious experience of pain. Integrating population-pharmacokinetic models and multimodal neuromonitoring are needed for personalized pharmacotherapy in these vulnerable patients. Trial Registration: https://www.trialregister.nl/trial/6972, identifier: NL6972.

The Chest Radiographic Thoracic Area Can Serve as a Prediction Marker for Morbidity and Mortality in Infants With Congenital Diaphragmatic Hernia.

Objective: Valid postnatal prediction parameters for neonates with congenital diaphragmatic hernia (CDH) are lacking, but recently, the chest radiographic thoracic area (CRTA) was proposed to predict survival with high sensitivity. Here, we evaluated whether the CRTA correlated with morbidity and mortality in neonates with CDH and was able to predict these with higher sensitivity and specificity than prenatal observed-to-expected (O/E) lung-to-head ratio (LHR). Methods: In this retrospective cohort study, all neonates with CDH admitted to our institution between 2013 and 2019 were included. The CRTA was measured using the software Horos (V. 3.3.5) and compared with O/E LHR diagnosed by fetal ultrasonography in relation to outcome parameters including survival, extracorporeal membrane oxygenation (ECMO) support, and chronic lung disease (CLD). Results: In this study 255 neonates were included with a survival to discharge of 84%, ECMO support in 46%, and 56% developing a CLD. Multiple regression analysis demonstrated that the CRTA correlates significantly with survival (p = 0.001), ECMO support (p < 0.0001), and development of CLD (p = 0.0193). The CRTA displayed a higher prognostic validity for survival [area under the curve (AUC) = 0.822], ECMO support (AUC = 0.802), and developing a CLD (AUC = 0.855) compared with the O/E LHR. Conclusions: Our data suggest that the postnatal CRTA might be a better prognostic parameter for morbidity and mortality than the prenatal O/E LHR.

Congenital Y-urethra - A diagnostic and therapeutic challenge.

BACKGROUND: For the Y-subtype of urethral duplications expression and nomenclature vary, as treatment recommendations do. OBJECTIVE: To raise awareness of the variety and variable terminology of Y-type duplication of the urethra, and to discuss the diagnostic work-up in light of options for or against surgical reconstruction. MATERIALS AND METHODS: Five patients with congenital Y-urethra were treated in four institutions within 15 years (2004-2019). While patients were managed in our respective institution with some exchange of experience, all available data were shared and evaluated for this review. RESULTS: The age at initial presentation was 1 day-6 months. In three patients the Y-urethra was found together with an anorectal malformation (ARM). With the focus on reconstruction rather than suprapubic diversion the orthotopic urethra was restored in the majority of patients using either single-step or staged approaches while the accessory urethral limb was incorporated. This was successful despite additional procedures aiming at ARM reconstruction. The patients void spontaneously and do control urine and bowels. One patient underwent kidney transplantation as a consequence of associated renal anomalies while reconstructive attempts regarding his urethral anomaly failed. In two patients, uncertainty in recognizing the pathology delayed a purposive treatment. DISCUSSION: For this rare anomaly the terminology in the literature merges and suggestions for the treatment differ. Success in four out five patients supports an approach which makes use of the accessory ventral track to restore the orthotopic urethra. Since some patients present as a neonatal emergency with concomitant problems such as ARM, a basic understanding of the variable pathology is required. CONCLUSION: The cases of Y-urethra reported herein demonstrate that correct allocation at the initial presentation or at least prior to first surgical steps will preserve the chance for physiologic micturition and urinary continence. Timing of surgery has to be done in the context of associated malformations and is not an emergency as long as proper bladder drainage is ensured. However, relocation of the accessory track requires several procedures bearing risks of complications short and long-term. This may be an argument to consider any therapeutic strategy against other options such as postponed treatment or permanent suprapubic diversion. Evidence-based guidelines are lacking.

[Scrotal orchidopexy for palpable undescended testis]. / Operative Zugangswege beim Gleithoden und tiefen Leistenhoden ­ skrotaler Zugang.

Undescended testis is the most common genitourinary disorder during childhood. The traditional inguinal approach still represents the standard technique to correct undescended testis. However, over the last decades a large number of studies have shown that the scrotal approach has a lower morbidity rate and significantly shorter operation times and offers a good cosmetic result as well as an equal surgical treatment result, which makes it a good alternative to the traditional inguinal approach in gliding and low palpable testis.

[Pelviureteric junction obstruction and crossing vessels: pro "vascular hitch"]. / Subpelvine Stenose & kreuzende Gefäße ­ Pro "vascular hitch".

The vascular hitch procedure for pelviureteric junction obstruction caused by crossing lower-pole vessels is a controversial treatment option. Since this minimally-invasive technique has been introduced in patients with aberrant lower-pole vessels, multiple publications have reported successful short, intermediate and long-term outcomes. Success rates of > 90 % are similar to those of Anderson-Hynes pyeloplasty. In general, an associated intrinsic stenosis seems to be rare. Histological evidence of muscular hypertrophy may simply indicate a compensatory mechanism and may be reversible to a certain degree. To differentiate between those patients who are eligible for a vascular hitch procedure and those who should receive an initial Anderson-Hynes pyeloplasty, an intraoperative diuretic test should be performed (volume bolus plus intravenous administration of furosemide). An associated intrinsic stenosis seems to be unlikely in a funnel-like and otherwise normal-looking pelviureteric junction, decreasing hydronephrosis after mobilisation of renal pelvis and crossing vessels and effective peristalsis of the renal pelvis after intraoperative diuretic testing. The vascular hitch technique is less demanding than laparoscopic Anderson-Hynes pyeloplasty and less time-consuming with regard to the duration of the surgical procedure and anaesthesia. Further advantages are: no risk of urinary leakage or anastomotic stricture and no need for intra-luminal stenting. Therefore, in a selected group of patients with solely extrinsic pelviureteric junction obstruction, the vascular hitch procedure is a valuable alternative to classic Anderson-Hynes pyeloplasty with seemingly long-term efficiency.

[Pediatric and adolescent varicocele - When, why and how to treat?] / Varikozele im Kindes- und Jugendalter.

The treatment of a varicocele in childhood and adolescence is considered very controversial. In the past varicocele was often diagnosed during the medical examination for military purposes, but today varicocele is common in the J1 exam and is found rather frequently. However, we are still lacking evidence-based recommendations. This means that urologists and paediatric urologists are increasingly confronted with the question of whether intervention is necessary or not.The literature on this is very contradictory. Systematic reviews and meta-analyses have recently shown that there are only modest benefits of intervention in terms of testicular size and spermiogram quality. Solid data on the impact on paternity rates are still lacking. However, it has been shown that if an intervention is to be made, the lymphatic sparing techniques have a distinct advantage.Possible indications for intervention may be the persistent difference in size of the testes (> 20 % for at least 6 months), a symptomatic varicocele (very subjective criterion), a pathological spermiogram (better 2), and possibly the presence of additional fertility-limiting factors.

Current Indications and Techniques for the Use of Bowel Segments in Pediatric Urinary Tract Reconstruction.

Today, there are few indications for the use of bowel in pediatric urology. This is in large extent due to the successful conservative therapy in patients with neurogenic bladder and the improved success of primary reconstruction in patients with the bladder exstrophy-epispadias complex. Only after the failure of the maximum of conservative therapy or after failure of primary reconstruction, bladder augmentation, or urinary diversion should be considered. Malignant tumors of the lower urinary tract (e.g., rhabdomyosarcomas of the bladder/prostate) are other rare indications for urinary diversion. Replacement or reconstruction of the ureter with a bowel segment is also a quite rarely performed procedure. In this review, the advantages and disadvantages of the different options for the use of bowel segments for bladder augmentation, bladder substitution, urinary diversion, or ureter replacement during childhood and adolescence are discussed.

Histogram based analysis of lung perfusion of children after congenital diaphragmatic hernia repair.

OBJECTIVES: To investigate a histogram based approach to characterize the distribution of perfusion in the whole left and right lung by descriptive statistics and to show how histograms could be used to visually explore perfusion defects in two year old children after Congenital Diaphragmatic Hernia (CDH) repair. METHODS: 28 children (age of 24.2±1.7months; all left sided hernia; 9 after extracorporeal membrane oxygenation therapy) underwent quantitative DCE-MRI of the lung. Segmentations of left and right lung were manually drawn to mask the calculated pulmonary blood flow maps and then to derive histograms for each lung side. Individual and group wise analysis of histograms of left and right lung was performed. RESULTS: Ipsilateral and contralateral lung show significant difference in shape and descriptive statistics derived from the histogram (Wilcoxon signed-rank test, p<0.05) on group wise and individual level. Subgroup analysis (patients with vs without ECMO therapy) showed no significant differences using histogram derived parameters. CONCLUSIONS: Histogram analysis can be a valuable tool to characterize and visualize whole lung perfusion of children after CDH repair. It allows for several possibilities to analyze the data, either describing the perfusion differences between the right and left lung but also to explore and visualize localized perfusion patterns in the 3D lung volume. Subgroup analysis will be possible given sufficient sample sizes.