Katrin Zahn

Congenital diaphragmatic hernia: a modern day approach.

Centralization of all complicated congenital diaphragmatic hernias (CDH) was organized in Germany from 1998, collecting 325 consecutive patients with striking increasing survival rates. This series report 244 patients from 2002 to 2007. Today, large defects are detected early in pregnancy by ultrasound and magnetic resonance imaging (MRI). In extracorporeal membrane oxygenation (ECMO) patients, prenatal lung head ratio (LHR) was 1.2 (median) at the 34th week of gestation or less than 25 ml lung tissue in MRI. This means that all patients below LHR of 1.4 should be transferred prenatally in a tertiary center. High risk group for survival was defined as LHR below 0.9, ie, 10 ml in MRI planimetry. Inborn patients show better results than outborns. In algorithm therapy, gentle ventilation plays an important role in preventing damage to the lung tissue and avoiding long term ventilation. When PaCO(2) was more than 75 mmHg, ventilation was changed to high frequency oscillatory ventilation (HFOV). Indication for ECMO was seen in preductal PaO(2) less than 50 mmHg over 2-4 h or less than 40 mmHg over 2 h. ECMO related risks included intracerebral bleeding (9%), intrapulmonary bleeding (14%), and convulsions (16%). Surgically, a longitudinal midline incision for exposure of the defect, the duodenal kinking, and probably for abdominal patching was perfect. A cone formed goretex patch provided more abdominal space and reduced abundant intrathoracical cavity. No drain was used. Postoperative complications were described. Overall survival in 244 consecutive patients was 86.5% for all patients born alive. All those who needed ECMO survived in 71%, underlining ECMO as a treatment of last choice. Follow-up for quality of life after CDH is described.

Prediction of chronic lung disease, survival and need for ECMO therapy in infants with congenital diaphragmatic hernia: Additional value of fetal MRI measurements?

INTRODUCTIONnThe lung-to-head ratio (LHR), measured by ultrasound, and the fetal lung volume (FLV), measured by MRI, are both used to predict survival and need for extra corporeal membrane oxygenation (ECMO) in infants with congenital diaphragmatic hernia (CDH). The aim of this study is to determine whether MRI measurements of the FLV, in addition to standard ultrasound measurements of the LHR, give better prediction of chronic lung disease, mortality by day 28 and need for ECMO.nnnMATERIALS AND METHODSnPatients with unilateral isolated CDH born between January 2002 and December 2008 were eligible for inclusion. LHR and FLV were expressed as observed-to-expected values (O/E LHR and O/E FLV). Univariate and multivariate analyses were performed. Receiver operating characteristic curves were constructed and areas under the curve (AUC) were calculated to determine predictive values.nnnRESULTSn90 patients were included in the analysis. Combined measurement of the O/E LHR and O/E FLV gave a slightly better prediction of chronic lung disease (AUC=0.83 and AUC=0.87) and need for ECMO therapy (AUC=0.77 and AUC=0.81) than standard ultrasound measurements of the O/E LHR alone. Combined measurement of the O/E LHR and O/E FLV did not improve prediction of early mortality (AUC=0.90) compared to measurement of the O/E LHR alone (AUC=0.89). An intrathoracal position of the liver was independently associated with a higher risk of early mortality (p<0.001), chronic lung disease (p=0.007) and need for ECMO therapy (p=0.001).nnnDISCUSSIONnChronic lung disease and need for ECMO therapy are slightly better predicted by combined measurement of the O/E LHR and the O/E FLV. Early mortality is very well predicted by measurement of the O/E LHR alone.nnnCONCLUSIONnClinical relevance of additional MRI measurements may be debated.

Preventive antireflux surgery in neonates with congenital diaphragmatic hernia: a single-blinded prospective study

OBJECTIVEnCongenital diaphragmatic hernia (CDH) is known to be a predisposing factor in gastroesophageal reflux (GER) leading to pulmonary and nutritional problems. The aim of this prospective, randomized, patient-blinded study was to evaluate the benefit of antireflux surgery at the time of CDH repair.nnnMETHODSnFrom 2003 to 2009, 79 neonates with left-sided CDH were included. Forty-three had regular hernia closure. Thirty-six patients additionally had fundoplication at hernia repair. Follow-up was at 6, 12, and 24 months after birth with a standardized questionnaire and a thorax radiograph. Patients with clinical signs for GER were evaluated with upper gastrointestinal series and 24-hour pH-metry.nnnRESULTSnSeventy-nine of 263 patients participated in this prospective trial. Survival rate was 88.61%. The GER symptoms were almost significantly more frequent in the group without concomitant fundoplication at the age of 6 months. At 24 months, the difference between both groups was not significant anymore. Development of body weight in the first 2 years of life was similar in both groups. No complications related to initial antireflux surgery were noted.nnnCONCLUSIONnPatients profit from fundoplication at CDH repair only within the first year of life. At the present point of this study, simultaneous fundoplication at the time of primary CDH repair cannot be recommended as a standard procedure in all patients with left-sided CDH.

Quantitative pulmonary perfusion imaging at 3.0 T of 2-year-old children after congenital diaphragmatic hernia repair: initial results

AbstractObjectiveTo investigate whether dynamic contrast-enhanced MR imaging of the lung following congenital diaphragmatic hernia repair is feasible at 3.0xa0T in 2-year-old children and whether associated lung hypoplasia (reflected in reduced pulmonary microcirculation) can be demonstrated in MRI.MethodsTwelve children with a mean age 2.0u2009±u20090.2xa0years after hernia repair underwent DCE-MRI at 3.0xa0T using a time-resolved angiography with stochastic trajectories sequence. Quantification of lung perfusion was performed using a pixel-by-pixel deconvolution approach. Six regions of interest were placed (upper, middle and lower parts of right and left lung) to assess differences in pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) while avoiding the inclusion of larger pulmonary arteries and veins.ResultsThe difference in PBF and PBV between ipsilateral and contralateral lung was significant (Pu2009<u20090.5). No significant differences could be detected for the MTT (Pu2009=u20090.5).ConclusionDCE-MRI in 2-year-old patients is feasible at 3.0xa0T. Reduced perfusion in the ipsilateral lung is reflected by significantly lower PBF values compared with the contralateral lung. DCE-MRI of the lung in congenital diaphragmatic hernia can help to characterise lung hypoplasia initially and in the long-term follow-up of children after diaphragmatic repair.Key Points• Congenital diaphragmatic hernia often leads to lung hypoplasia and secondary pulmonary hypertension.• Dynamic contrast-enhanced 3-T magnetic resonance can assess these complications in 2-year-olds.• The affected ipsilateral lung shows reduced perfusion and lower pulmonary blood flow.n • Thoracic DCE-MRI helps characterise lung hypoplasia in children after hernia repair.

Candidiasis caused by Candida kefyr in a neonate: Case report

BackgroundSystemic Candidia infections are of major concern in neonates, especially in those with risk factors such as longer use of broad spectrum antibiotics. Recent studies showed that also term babies with underlying gastrointestinal or urinary tract abnormalities are much more prone to systemic Candida infection. We report a very rare case of candidiasis caused by Candida kefyr in a term neonate.Case PresentationRenal agenesis on the left side was diagnosed antenatally and anal atresia postnatally. Moreover, a vesico-ureteral-reflux (VUR) grade V was detected by cystography. The first surgical procedure, creating a protective colostoma, was uneventful. Afterwards our patient developed urosepsis caused by Enterococcus faecalis and was treated with piperacillin. The child improved initially, but deteriorated again. A further urine analysis revealed Candida kefyr in a significant number. As antibiotic resistance data about this non-albicans Candida species are limited, we started liposomal amphotericin B (AMB), but later changed to fluconazole after receiving the antibiogram. Candiduria persisted and abdominal imaging showed a Candida pyelonephritis. Since high grade reflux was prevalent we instilled AMB into the childs bladder as a therapeutic approach. While undergoing surgery (creating a neo-rectum) a recto-vesical fistula could be shown and subsequently was resected. The child recovered completely under systemic fluconazole therapy over 3 months.ConclusionsCandidiasis is still of major concern in neonates with accompanying risk factors. As clinicians are confronted with an increasing number of non-albicans Candida species, knowledge about these pathogens and their sensitivities is of major importance.

High temporal versus high spatial resolution in MR quantitative pulmonary perfusion imaging of two-year old children after congenital diaphragmatic hernia repair.

AbstractObjectivesCongenital diaphragmatic hernia (CDH) leads to lung hypoplasia. Using dynamic contrast-enhanced (DCE) MR imaging, lung perfusion can be quantified. As MR perfusion values depend on temporal resolution, we compared two protocols to investigate whether ipsilateral lung perfusion is impaired after CDH, whether there are protocol-dependent differences, and which protocol is preferred.MethodsDCE-MRI was performed in 36 2-year old children after CDH on a 3xa0T MRI system; protocol A (nu2009=u200918) based on a high spatial (3.0xa0s; voxel: 1.25xa0mm3) and protocol B (nu2009=u200918) on a high temporal resolution (1.5xa0s; voxel: 2xa0mm3). Pulmonary blood flow (PBF), pulmonary blood volume (PBV), mean transit time (MTT), and peak-contrast-to-noise-ratio (PCNR)xa0were quantified.ResultsPBF was reduced ipsilaterally, with ipsilateral PBF of 45u2009±u200926xa0ml/100xa0ml/min to contralateral PBF of 63u2009±u200928xa0ml/100xa0ml/min (pu2009=u20090.0016) for protocol A; and for protocol B, side differences were equivalent (ipsilateral PBFu2009=u200962u2009±u200924 vs. contralateral PBFu2009=u200985u2009±u200930xa0ml/100xa0ml/min; pu2009=u20090.0034). PCNR was higher for protocol B (30u2009±u200918 vs. 20u2009±u20099; pu2009=u20090.0294). Protocol B showed higher values of PBF in comparison to protocol A (p always <0.05).ConclusionsIpsilateral lung perfusion is reduced in 2-year old children following CDH repair. Higher temporal resolution and increased voxel size show a gain in PCNR and lead to higher perfusion values. Protocol B is therefore preferred.Key Points• Quantitative lung perfusion parameters depend on temporal and spatial resolution.n • Reduction of lung perfusionxa0in CDH can be measured with different MR protocols.n • Temporal resolution of 1.5xa0s with spatial resolution of 2xa0mm3is suitable.

Lung Perfusion MRI After Congenital Diaphragmatic Hernia Repair in 2-Year-Old Children With and Without Extracorporeal Membrane Oxygenation Therapy

OBJECTIVEnIn severe cases of congenital diaphragmatic hernia (CDH), extracorporeal membrane oxygenation (ECMO) therapy improves survival. Later on, lung morbidity mainly defines development. The purpose of this study was to investigate whether 2-year-old children who need ECMO therapy after delivery have reduced perfusion MRI values as a sign of more severe lung hypoplasia than do children who do not need ECMO.nnnMATERIALS AND METHODSnAfter CDH repair, 38 children underwent dynamic contrast-enhanced MRI with a 3D time-resolved angiography with stochastic trajectories sequence. Fifteen (39%) of the children had received ECMO therapy in the neonatal period. Pulmonary blood flow (PBF), pulmonary blood volume (PBV), and mean transit time were calculated for both lungs. In addition, the ratio of ipsilateral to contralateral lung was calculated for all parameters.nnnRESULTSnIn all children, those with and those without ECMO requirement, PBF and PBV were significantly reduced on the ipsilateral side (p < 0.05). Children who had received ECMO therapy had significantly reduced PBF and PBV values on the ipsilateral side (p < 0.05) compared with children who had not needed ECMO therapy. The ratios of ipsilateral to contralateral lung for PBF and PBV were also significantly reduced after ECMO.nnnCONCLUSIONnTwo-year-old children undergoing CDH repair who had needed neonatal ECMO had significantly reduced perfusion MRI values in the ipsilateral lung in comparison with children who had not needed ECMO. Perfusion MRI measurements are associated with the severity of lung hypoplasia and may therefore be helpful in follow-up investigations.

Semi-automatic lung segmentation of DCE-MRI data sets of 2-year old children after congenital diaphragmatic hernia repair: Initial results

In congenital diaphragmatic hernia (CDH), lung hypoplasia and secondary pulmonary hypertension are the major causes of death and severe disability. Based on new therapeutic strategies survival rates could be improved to up to 80%. However, after surgical repair of CDH, long-term follow-up of these pediatric patients is necessary. In this, dynamic contrast enhanced magnetic resonance imaging (DCE-MRI) provides insights into the pulmonary microcirculation and might become a tool within the routine follow-up program of CDH patients. However, whole lung segmentation from DCE-MRI scans is tedious and automated procedures are warranted. Therefore, in this study, an approach to semi-automated lung segmentation is presented. Segmentation of the lung is obtained by calculating the cross correlation and the area under curve between all voxels in the data set and a reference region-of-interest (ROI), here the arterial input function (AIF). By applying an upper and lower threshold to the obtained maps and intersecting these, a final segmentation is reached. This approach was tested on twelve DCE-MRI data sets of 2-year old children after CDH repair. Segmentation accuracy was evaluated by comparing obtained automatic segmentations to manual delineations using the Dice overlap measure. Optimal thresholds for the cross correlation were 0.5/0.95 and 0.1/0.5 for the area under curve, respectively. The ipsilateral (left) lung showed reduced segmentation accuracy compared to the contralateral (right) lung. Average processing time was about 1.4s per data set. Average Dice score was 0.7±0.1 for the whole lung. In conclusion, initial results are promising. By our approach, whole lung segmentation is possible and a rapid evaluation of whole lung perfusion becomes possible. This might allow for a more detailed analysis of lung hypoplasia of children after CDH.

Prenatal MR imaging of congenital diaphragmatic hernias: association of MR fetal lung volume with the need for postnatal prosthetic patch repair

AbstractObjectiveTo assess whether the need for postnatal prosthetic patch repair of the diaphragmatic defect in neonates with a congenital diaphragmatic hernia (CDH) is associated with the antenatalxa0measured observed-to-expected magnetic resonance fetal lung volume (o/e MR-FLV).MethodsThe o/e MR-FLV was calculated in 247 fetuses with isolated CDH. Logistic regression analysis was used to assess the prognostic value of the individual o/e MR-FLV for association with the need for postnatal patch repair.ResultsSeventy-seven percent (77xa0%) of patients with a CDH (190/247) required prosthetic patchxa0repair and the defect was closed primarily in 23xa0% (57/247). Patients requiring a patch had a significantly lower o/e MR-FLV (27.7u2009±u200910.2xa0%) than patients with primary repair (40.8u2009±u200913.8xa0%, pu2009<u20090.001, AUCu2009=u20090.786). With an o/e MR-FLV of 20xa0%, 92xa0% of the patients required patch repair, compared to only 24xa0% with an o/e MR-FLV of 60xa0%. The need for axa0prosthetic patch was further influenced by the fetal liver position (herniation/no herniation) as determined by magnetic resonance imaging (MRI; pu2009<u20090.001). Fetal liver position, in addition to the o/e MR-FLV, improves prognostic accuracy (AUCu2009=u20090.827).ConclusionLogistic regression analysis based on the o/e MR-FLV is useful for prenatal estimation of the prosthetic patch requirement in patients with a CDH. In addition to the o/e MR-FLV,xa0the position of the liver as determined by fetal MRI helps improve prognostic accuracy.Key Points• The o/e MR-FLV is associated with the need for postnatal patch repair in CDH.n • The need for a patch is associated with a significantly lower o/e MR-FLV (pu2009<u20090.001).n • The patch requirement is also influenced by fetal liver position.n • Fetal liver position, in addition to the o/e MR-FLV, improves prognostic accuracy.n • CDH-fetuses with a high probability for patch requirement can be identified prenatally.

Region of interest-based versus whole-lung segmentation-based approach for MR lung perfusion quantification in 2-year-old children after congenital diaphragmatic hernia repair

ObjectiveWith a region of interest (ROI)-based approach 2-year-old children after congenital diaphragmatic hernia (CDH) show reduced MR lung perfusion values on the ipsilateral side compared to the contralateral. This study evaluates whether results can be reproduced by segmentation of whole-lung and whether there are differences between the ROI-based and whole-lung measurements.MethodsUsing dynamic contrast-enhanced (DCE) MRI, pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) were quantified in 30 children after CDH repair. Quantification results of an ROI-based (six cylindrical ROIs generated of five adjacent slices per lung-side) and a whole-lung segmentation approach were compared.ResultsIn both approaches PBF and PBV were significantly reduced on the ipsilateral side (p always <0.0001). In ipsilateral lungs, PBF of the ROI-based and the whole-lung segmentation-based approach was equal (p=0.50). In contralateral lungs, the ROI-based approach significantly overestimated PBF in comparison to the whole-lung segmentation approach by approximately 9.5 % (p=0.0013).ConclusionsMR lung perfusion in 2-year-old children after CDH is significantly reduced ipsilaterally. In the contralateral lung, the ROI-based approach significantly overestimates perfusion, which can be explained by exclusion of the most ventral parts of the lung. Therefore whole-lung segmentation should be preferred.Key Points• Ipsilaterally, absolute lung perfusion after CDH is reduced in whole-lung analysis.• Ipsilaterally, the ROI- and whole-lung-based approaches generate identical results.• Contralaterally, the ROI-based approach significantly overestimates perfusion results.• Whole lung should be analysed in MR lung perfusion imaging.• MR lung perfusion measurement is a radiation-free parameter of lung function.