Right-sided Defects Research Articles

Transabdominal robotic repair of a congenital right diaphragmatic hernia containing an intrathoracic kidney: a case report

Bochdalek diaphragmatic hernia is a rare condition and is typically diagnosed prior to adulthood. Furthermore, right-sided defects are also uncommon due to the location of the liver, but can contain colon, omentum, small bowel, or rarely the kidney. Minimally invasive laparoscopic and thoracoscopic diaphragmatic hernia repairs are associated with improved outcomes when compared to open approaches-recently, robotic-assisted repairs have been performed in children with no morbidity and minimal complications. We report a case of an 80-year-old female who presented with an enlarging right-sided Bochdalek hernia containing an acquired intrathoracic kidney that was repaired using a robotic-assisted laparoscopic transabdominal approach with mesh placement. In this case, the robotic platform's advantages included excellent visualization of the posterolateral defect and efficient suturing during mesh placement. This approach is a viable option for skilled minimally invasive surgeons; however, further studies are warranted to investigate its utility in the management of diaphragmatic hernia repair.

Tissue engineering strategy using mesenchymal stem cell-based chitosan scafolds in growth plate surgery: A preliminary study in rabbits

BackgroundGrowth plate injury in children could produce limb length discrepancy and angular deformity. Removal of damaged physis or bony bar and insertion of spacers produced variable results and for large defects in young children, the treatment is challenging. In this study, we used tissue-engineered mesenchymal stem cells (MSC-based chitosan scaffold) for restoration of the damaged physis. The usage of chitosan as a spacer was also investigated. Materials and methodsAn experimental model of growth arrest was created by removing lateral 50% of distal femoral physis of fourteen 4-week-olds albino rabbits. The left side growth plate defects were filled with MSC-based chitosan scaffold in 10 and scaffold alone in 4 rabbits. For all the rabbits, right-side defects were left alone as the control limb. After 3 months, femoral bones were harvested and gross inspection and radiology for measurement of angulations were done; histological study for evaluation of regeneration of physis was also done. ResultsThe hemiphyseal resection procedures were successful and all of the operated limbs showed angular deformities. There was a trend toward less angular deformity in cases in which more concentration of MSCs with chitosan scaffold was used. In cases of transfer of MSCs with concentration of less than 1.5 millions, mixed results were observed and angular deformities were not reduced. Transfer of chitosan alone yielded poor results. ConclusionIn this study, we have developed an in vitro construction of a transplantable tissue-engineered disk, using natural chitosan scaffold and MSCs. We investigated the efficacy of these disks for repairing the defect of growth plate cartilage at distal femoral physis. Our results showed that the beneficial effect of these cells on scaffold appeared in more concentration of cells. Level of evidenceLevel III. Low power comparative study.

Prenatal prediction of pulmonary arterial hypertension in congenital diaphragmatic hernia

To evaluate the role of prenatal prognostic markers obtained routinely by ultrasound examination and magnetic resonance imaging (MRI) in the prediction of development of postnatal pulmonary arterial hypertension (PAH) in isolated congenital diaphragmatic hernia (CDH). One hundred and ten cases of isolated CDH were referred to our fetal medicine unit between January 2004 and April 2013. Mortality and morbidity rates were reviewed for those presenting with postnatal PAH. The following prenatal markers were evaluated as potential predictive factors of PAH: liver position, side of the CDH defect, lung area to head circumference ratio (LHR) and observed/expected LHR (o/e-LHR), which were measured by ultrasound, and observed/expected total fetal lung volume (o/e-TFLV), which was measured by MRI. Univariable logistic regression was used to assess associations. PAH was significantly associated with perinatal mortality and morbidity (P < 0.001). The occurrence of PAH decreased significantly with an increasing LHR, o/e-LHR and o/e-TFLV and was significantly increased for cases with an intrathoracic liver, but not for those with right-sided defects. Univariable regression revealed that o/e-TFLV (odds ratio (OR), 0.9 (95% CI, 0.86-0.95); P < 0.05 for percentage unit change in o/e), LHR (OR, 0.19 (95% CI, 0.09-0.40); P < 0.05 for unit change), o/e-LHR (OR, 0.95 (95% CI, 0.93-0.98); P < 0.05 for percentage unit change in o/e) and liver position (OR, 2.82 (95% CI, 1.13-7.00); P < 0.05 for intrathoracic liver) were significant predictors of subsequent PAH. No differences were found after adjusting for gestational age at delivery. The areas under the receiver-operating characteristics curve were 0.80 and 0.75 for o/e-TFLV and o/e-LHR, respectively. In cases of CDH, PAH is associated with high rates of mortality and morbidity. Routinely obtained prenatal markers, usually used for the assessment of pulmonary hypoplasia, are also relevant for the postnatal prediction of PAH.

Right-sided congenital diaphragmatic hernia: the challenge remains.

Fetal surgery is without a doubt one of the greatest achievements in fetal medicine over the last three decades, addressing a number of lethal or otherwise debilitating congenital conditions and pregnancy complications. Fetal endotracheal occlusion (FETO) for the treatment of pulmonary hypoplasia secondary to congenital diaphragmatic hernia (CDH), although always ‘high on the fetal surgery agenda’, has seen many waves of ‘hope and despair’ (Deprest JA et al., Fetal surgery for congenital diaphragmatic hernia is back from never gone, Fetal Diagn Ther 2011;29:6–17). Indeed, despite sound physiological theories and animal experiments suggesting its potential benefits, randomised controlled trials comparing FETO to expectant management have not consistently shown the expected success (Harrison MR et al., A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia, N Engl J Med 2003;349:1916– 24). Notwithstanding the low incidence of CDH, a continued challenge surrounding FETO has been the difficulty in accurately determining fetal prognostic factors which may prompt intervention. These concerns appear to be greater in right-sided CDH (RCDH), given that its incidence is 70–80% lower than leftsided defects, and that liver herniation, a major prognostic factor in contralateral cases, is almost universally present (Hedrick HL et al., Right congenital diaphragmatic hernia: prenatal assessment and outcome, J Pediatr Surg 2004;39:319– 23). In their study, DeKoninck et al. correctly point out that most CDH studies address isolated left-sided defects, and that perhaps RCDH may be a different pathology altogether, worthy of its own evaluation. To describe the specific characteristics and outcomes of RCDH, they conducted a retrospective review of cases with isolated RCDH and liver herniation subjected to FETO at two centres over 10 years, and find that fetuses with a lung-to-head ratio ≤45% have a tendency towards increased survival at 42% relative to 17% among expectantly managed cases (P = 0.09), without a significant increase in preterm birth. Akin to left-sided defects, they re-iterate that initial lung size predicts survival following FETO in right-sided defects. These results, although not statistically significant, point to the value that FETO may have in severe RCDH. Or do they? The issue with retrospectively analysing rare pathologies using observational data that is collected outside the realm of a controlled trial, in addition to not offering a power calculation, is that facing non-statistically significant results such as those provided in this study, one cannot attribute the differences, or lack thereof, between subjects and controls either to a lack of statistical power or to the absence of a true relationship between intervention and outcome. The nuanced interpretation in conclusions such as ‘Though the current data do not show benefit, in poor prognosis cases FETO may seem to be justifiable’ needs to be therefore regarded with the utmost caution. Indeed, the same way in which a trend towards positive outcomes, such as increased survival, may prompt intervention, the risk of rendering a non-statistically significant negative outcome such as preterm birth significant, increases. All in all, it would seem from these results that at best, FETO

An eye movement study on the role of the visual field defect in pure alexia.

Pure alexia is a severe impairment of word reading which is usually accompanied by a right-sided visual field defect. Patients with pure alexia exhibit better preserved writing and a considerable word length effect, claimed to result from a serial letter processing strategy. Two experiments compared the eye movements of four patients with pure alexia to controls with simulated visual field defects (sVFD) when reading single words. Besides differences in response times and differential effects of word length on word reading in both groups, fixation durations and the occurrence of a serial, letter-by-letter fixation strategy were investigated. The analyses revealed quantitative and qualitative differences between pure alexic patients and unimpaired individuals reading with sVFD. The patients with pure alexia read words slower and exhibited more fixations. The serial, letter-by-letter fixation strategy was observed only in the patients but not in the controls with sVFD. It is argued that the VFD does not cause pure alexic reading.

Anteromedial Approach to Assess the Right Heart on Echocardiography

n today’s ultrasound community, sonographers sometimes forget about the extra steps we can take to improve a patient’s end result while visiting our lab. How many times have we called a patient back to reassess a valve lesion or to look at pressures that were uninterpretable? Let us not be afraid to manipulate our technical ability to provide the absolute best in imaging. The right heart is difficult to image with echocardiography because of the location, size, and available windows (Figure 1). The traditional apical 4-chamber view allows for a direct, parallel image of the left ventricle and atrium. However, trying to maneuver the probe from this particular area may only bring in more lung artifacts or decrease the appropriate angle we were trying to achieve. Right ventricular inflow patterns, tissue Doppler imaging, tricuspid annular plane systolic excursion, and tricuspid regurgitation interrogation can be of important use in determining a patient’s care plan, given the appropriate angles. We must ask ourselves, are we doing everything we can to provide the necessary information to the reading physician? As a veteran cardiac sonographer, I am compelled to pay close attention to the left ventricle, as it is the “main” pumping chamber of the heart. In this case, we will go out of our way, in difficult cases, to give the reading physician the best we have so the physician might see the endocardium a little clearer. Sonographers must give the physician this information to assess numerous pathologic questions. The reading physician should be given the same opportunity on the right side of the heart. Congestive heart failure, hypertension, valve lesions, and congenital heart defects affect the right side as well. How many patients have had complications because a rightsided congenital defect was overlooked? In most cases, the sonographer can easily slide the probe medially from the traditional 4-chamber view. Looking at the monitor, the atria will be seen moving to the left of the screen, while the apex stays somewhat midline. To improve the parallel flow angle, move anteriorly one rib space if needed (Figure 2). Traditionally, foreshortening the 4-chamber view is frowned upon; however, this movement improves the visualization of this particular area of the heart. Cardiologists are looking to assess right ventricular hypertrophy, tricuspid valve lesions, new findings of congenital defects or ones that might have been missed, pacemaker/defibrillator leads (including infection of the leads), and more.1 One of the most common mistakes made in the field today is underesAddress correspondence to James Michael Rampoldi, RDCS, RVT, The Heart Hospital of Baylor Plano, Center for Advanced Cardiovascular Care, 4716 Alliance Blvd, Pavilion II, Suite 300, Plano, TX 75093 USA. E-mail: james.rampoldi@baylorhealth.edu I

Abdominal wall closure in neonates after congenital diaphragmatic hernia repair

PurposeRepair of Congenital Diaphragmatic Hernia (CDH) abruptly increases intra-abdominal pressure. This study sought to characterize the incidence and significance of ACS and delayed fascial closure (DFC) after CDH repair. MethodsWe reviewed the CAPSNet database from 2006 to 2011, identifying the subset of patients that developed ACS or required DFC. Prenatal and demographic information, operative and physiologic details, and outcomes were investigated. ResultsOf 349 patients with CDH, 3 (0.8%) were diagnosed with ACS, while 43 patients (12%) had DFC at the time of CDH repair. Patients more often had right-sided defects (26% vs 13%, p=0.04) and trended toward requiring a patch repair (41% vs 31.2%, p=0.23) and having a liver lobe above the diaphragmatic rim (47% vs 32.7, p=0.09).Patients with ACS or DFC had increased length of stay (47.5 vs 33.9, p=0.01), days fasting (8.2 vs 5.8, p=0.01), days on parenteral nutrition (23.6 vs 15.5, p=0.003), and days on mechanical ventilation (16.3 vs 9.0, p=0.001). ConclusionsWhile ACS in neonates after CDH repair is rare (<1%), DFC is required relatively commonly (>10%) and is associated with right-sided diaphragmatic hernias. Inability to close abdominal fascia is associated with increased morbidity. Clinicians caring for neonates with CDH should be facile with strategies to manage delayed abdominal fascia closure.

Bone regeneration in a canine cranial model using allogeneic adipose derived stem cells and coral scaffold

Adipose tissue derived stem cells (ASCs) based therapies for the repair and regeneration of various tissues have been widely investigated recently because of their multilineage potential and self-renewal capability. Our previous study demonstrated that autologous ASCs loaded onto natural coral scaffolds could repair cranial critical-sized defects (CSDs) in a canine model. The objective of this study was to determine whether the use of allogeneic ASCs could heal the same defect without the use of immunosuppressive therapy. The pedigree mismatch, mixed lymphocyte reaction assays (MLRs) and allogeneic skin graft experiments were performed to confirm unrelated ASC donors and recipients. A total of 12 adult Beagle dogs were enrolled in this study and divided into two groups. Bilateral cranial CSDs were created in each animal. The right-side defect was treated with allogeneic ASCs delivered onto a coral scaffold, and the left defect was either filled with an autologous ASC/coral composite (Group 1, n = 5) or with one coral scaffold alone (Group 2, n = 5). The systematic immune response and bone healing were evaluated postoperatively. The results showed that allogeneic ASC transplantation did not induce a systemic immune response by the hosts, and allogeneic ASCs could repair the cranial CSDs in an analogous way to that of the autologous cells. Moreover, both the green fluorescently labeled allogeneic and autologous ASCs were detected within the lacunae of newly formed bone in the defect site at 24 weeks, illustrating that the grafted ASCs contributed directly to bone regeneration in vivo. Thus, we concluded that allogeneic ASCs have the capacity to regenerate bone within craniofacial defects, providing an alternative source of seed cells for bone tissue engineering.

A Comparison of the Healing Capabilities of Various Grafting Materials in Critical-Size Defects in Guinea Pig Calvaria

To estimate the efficacy of various bone grafting materials in the healing of 8-mm-diameter critical-size defects (CSD) in guinea pig calvaria. In this randomized trial study, critical-size defects were created in the calvaria of 36 guinea pigs 6 months of age. Animals were assigned into three groups and each received one of three experimental protocols. In protocol A, the right-side defect was filled with macroporous biphasic calcium phosphate (MBCP Gel) and the left side was left empty as a control. In protocol B, the right-side defect was filled with demineralized freeze-dried bone allograft (DFDBA) and the left side was filled with Stypro. In protocol C, the right-side defect was filled with Bio-Oss and the left side was filled with autogenous bone. The percentage of new bone formation was evaluated histomorphometrically after 8 weeks. The mean bone formation was 68.19% for autogenous bone, 66.96% for MBCP Gel, 57.28% for Bio-Oss, 50.19% for DFDBA, 18.79% for Stypro, and 10.61% for the empty control. Except for the MBCP Gel and autogenous bone groups (P = .6), the differences between the other groups were statistically significant (P < .05). The results of this study indicated that all the testing materials had different capacities to produce new bone in CSD of guinea pig calvaria. MBCP Gel showed promising results in producing new bone proportionate to the autogenous bone graft group.

Pelvic floor muscle injuries 6 weeks post partum—an intra‐ and inter‐rater study

To evaluate intra- and inter-rater reliability when diagnosing major defects, and inter-rater reliability of diagnosing minor defects and muscle thickness of the pubovisceral muscle in primiparous women 6 weeks after vaginal delivery, using 3D/4D transperineal ultrasound. Forty primiparous women were assessed using 3D/4D transperineal ultrasound. Volumes were acquired at maximal pelvic floor muscle (PFM) contraction, and diagnosis of muscle defects were done using tomographic ultrasound imaging (TUI) of the axial plane. Thickness was measured in three central levels of TUI. The stored volumes were analyzed offline by two investigators blinded to each others' results and the women's clinical data. Cohen's kappa (κ) and percentual agreement were calculated for defects, intraclass correlations coefficient (ICC) with 95% confidence intervals were calculated for thickness. Excellent intra-rater values were found for all major defects. Inter-rater values for bilateral and right-sided defects were excellent, and good for left-sided. Agreement for minor defects was poor. Measuring thickness ICC of 0.72 was found for the left side and 0.48 for the right side, although up to half of the cases had to be excluded owing to poor demarcation of the muscle. Tomographic ultrasound imaging of the axial plane using three central slices seems to be a reliable tool for detecting major pubovisceral muscle defects shortly after childbirth. Minor defects showed low reliability. Muscle thickness measurements showed moderate reliability, but too many cases had to be excluded for this to be a useful method for determining muscle thinning 6 weeks after delivery.

Effect of acellular dermal matrix as a delivery carrier of adipose‐derived mesenchymal stem cells on bone regeneration

The purpose of this study is to evaluate the effect of acellular dermal matrix (ADM) as a delivery carrier of adipose-derived mesenchymal stem cells (ASCs) on bone regeneration in athymic murine calvarial bone defect. Paired-critical size defects in nude rat skull were made. The right-side defects received ASCs/ADM or only ADM, whereas the left-side defect was not treated. In 3D images, new bone formation in the ASCs/ADM group was apparent at 4 wk, but in the ADM group at 8 wk. At 4 and 8 wk, bone mineral density and tissue volume in rats that received ASCs/ADM were significantly greater than rats that received ADM and control groups. Histological examination revealed that the defect was repaired by bone in the ASCs/ADM group, whereas only minimal bone island with fibrous connection was observed in the control group. In histomorphometric analysis, the total healing score in the ASCs/ADM group at 4 wk was significantly higher than the ADM and negative control group, whereas the score of 8 wk was similar between the ASCs/ADM and ADM group. ASCs/ADM implants promote new bone formation more rapidly than ADM only or no treatment. ADM seeded with ASCs may be potentially useful as a future biomaterial option in bone implants.

Comparison of the osteogenic potentials of autologous cultured osteoblasts and mesenchymal stem cells loaded onto allogeneic cancellous bone granules

We compared the bone regeneration potentials of autologous cultured osteoblasts and of bone-marrow-derived autologous MSCs in combination with allogeneic cancellous bone granules in a rabbit radial defect model. Radial shaft defects over 15mm were made in 26 New Zealand white rabbits. The animals underwent insertion of allogeneic cancellous bone granules containing autologous osteoblasts into right-side defects (the experimental group) and of allogeneic cancellous bone granules with autologous MSCs into left-side defects (the control group). To quantitatively assess bone regeneration, radiographic evaluations as well as BMD and BMC measurements were performed 3, 6, 9 and 12weeks post-implantation and histology as well as micro-CT image analysis were performed at 6 and 12weeks. Radiographic evaluations 3 weeks post-implantation showed that the experimental group had a higher mean bone quantity index (p < 0.05) and micro-CT image analysis showed that experimental sides had a greater mean total regenerated bone volume and surface area than the control sides (p < 0.05). Histologic evaluations obtained at 6 and 12weeks revealed distinctly greater granule resorption and new bone formation in the experimental group. This in vivo study demonstrates that a combination of autologous osteoblasts and small-sized, allogeneic cancellous bone granules leads to more rapid bone regeneration than autologous MSCs and small-sized, allogeneic cancellous bone granules.

Ultrasound in the diagnosis of small bowel obstruction secondary to an incarcerated jejunal loop in Morison’s pouch

Abstract A few authors have published articles on unusual cases of internal hernias; however, there are no reports in the literature of a hernia arising from a right-sided retroperitoneal defect resulting in the incarceration of small bowel in Morison’s pouch. This case illustrates how point-of-care ultrasound was used to diagnose a small bowel obstruction in an atypical location.

1245 Congenital Diaphragmatic Hernia: 15 Year-Experience in a Pediatric Intensive Care Unit

Background and aims: congenital diaphragmatic hernia (CDH) is a rare malformation with a high mortality rate. Prenatal diagnosis is essential in defining the optimal perinatal therapeutic strategy. All cases of Portugal's Central Region have been referred to PICU of Coimbra's Pediatric Hospital (CPH). The aims were to determine the incidence of CDH, to characterize the newborns (NB) with CDH admitted to the PICU, to evaluate the mortality and its evolutionary pattern.Methods: a retrospective, study analysis made up of children with CDH was undertaken in a 15 year period. Based on the admission' year, two groups were formed: group A (1995-2001) and group B (2002-2009), where the newborns' characteristics, therapeutic strategies, prognostic factors and mortality were compared.Results: the mean annual incidence was 2.2 per 10.000 live-newborn. Of the 60 NB admitted to the PICU-CPH, 48.7% were prenatally diagnosed, 38% had associated malformations and 23.3% had a right-sided defect. 71.7% were born in perinatal tertiary referral centre. Regarding prognostic factors, newborns' characteristics and underlying therapeutic strategies, no statistically significant differences were observed between the two study groups. Prenatal diagnosis was made in 37.5% in the group A and 52.8% in the group B cases (p=0.369). The mortality in group A (29%) was significantly higher than in group B (5%; p=0.032).Conclusions: a significant decrease in mortality was observed in the two periods of the study, and that can be partially explained by prenatal diagnosis, which led to an in perinatal care.

Risk Factors for Chronic Lung Disease and Mortality in Newborns with Congenital Diaphragmatic Hernia

Background: Congenital diaphragmatic hernia (CDH) is associated with a mortality rate of 10–35% in live-born infants. Moreover, CDH survivors have a substantial risk of developing long-term pulmonary sequelae, such as bronchopulmonary dysplasia (BPD). Objectives: This study aims to evaluate risk factors associated with BPD and mortality in neonates with CDH, with particular focus on the initial ventilation mode. Methods: Eligible for inclusion were live-born infants with CDH born from 2001 through 2006 at the centers participating in the CDH Study Group. BPD (defined as oxygen dependency at day 30) and/or mortality by day 30 served as the primary endpoint. Results: A total of 2,078 neonates were included in the analysis. At day 30, 56% of the patients had either died or met the criteria for BPD. In infants who survived until day 30, the prevalence of BPD was 41%. The overall mortality rate was 31%. High-frequency oscillatory ventilation as initial ventilation mode, a right-sided defect, a prenatal diagnosis, a lower Apgar score at 5 min, a cardiac anomaly, a chromosomal anomaly and a lower gestational age were all associated with BPD and/or mortality by day 30. Conclusions: Despite improvements in neonatal care, the rates of BPD and early mortality in newborns with CDH are still considerable. Several important risk factors for a worse outcome are reported in this nonrandomized prospective observational study.

Unexpected findings at diagnostic laparoscopy: caecal incarceration with concurrent appendicitis in a patient with bilateral broad ligament defects

Internal herniations through broad ligament defects are very rare. We present the first report of the triad of broad ligament defect, internal herniation of the caecum and appendicitis. A 36-year-old woman with phocomelia presented with right iliac fossa pain and vomiting. The patient had no previous history of trauma or surgery. Abdominal ultrasound showed a small amount of free fluid. At laparoscopy, bilateral broad ligament defects were found, with herniation of the caecum and an inflamed appendix through the right-sided defect. A laparoscopic salpingo-oophorectomy was required for reduction of the herniated bowel, and an appendicectomy was performed. Broad ligament defects may be congenital or acquired. In this case, in light of the limb abnormality and absence of previous surgery, a congenital aetiology is more likely. Ultrasound scan is not reliable and, although computed tomography may be of help, a diagnostic laparoscopy is the best investigation.

Autosomal dominant inheritance of cardiac valves anomalies in two families: Extended spectrum of left‐ventricular outflow tract obstruction

Only a limited number of families with clear monogenic inheritance of nonsyndromic forms of congenital valve defects have been described. We describe two multiplex pedigrees with a similar nonsyndromic form of heart valve anomalies that segregate as an autosomal dominant condition. The first family is a three-generation pedigree with 10 family members affected with congenital defects of the cardiac valves, including six patients with aortic stenosis and/or aortic regurgitation. Pulmonary and/or tricuspid valve abnormalities were present in three patients, and ventricular septal defect (VSD) was present in two patients. The second family consists of 11 patients in three generations with aortic valve stenosis in seven patients, defects of the pulmonary valves in two patients, and atrial septal defect (ASD) in two patients. Incomplete penetrance was observed in both families. Although left-ventricular outflow tract obstruction was present in most family members, the co-occurrence with pulmonary valve abnormalities and septal defects in both families is uncommon. These families provide evidence that left-sided obstructive defects and thoracic aortic aneurysm may be accompanied by right-sided defects, and even septal defects. These families might be instrumental in identifying genes involved in cardiac valve morphogenesis and malformation.

Congenital Diaphragmatic Hernia: Predictive Value of MRI Relative Lung-to-Head Ratio Compared with MRI Fetal Lung Volume and Sonographic Lung-to-Head Ratio

The purpose of this study was to evaluate the prognostic accuracy of a new MRI-based relative lung-to-head ratio in regard to neonatal survival and need for extracorporeal membrane oxygenation (ECMO) in the care of fetuses with congenital diaphragmatic hernia (CDH) and to compare it with the previously described sonographic relative lung-to-head ratio and relative fetal lung volume assessed at antenatal MRI. Sonographic lung-to-head ratio and MRI fetal lung volume were measured in 90 fetuses (mean gestational age, 31.4+/-4.1 weeks) with CDH. Sonographic relative lung-to-head ratio and MRI relative fetal lung volume were assessed by expressing the observed sonographic lung-to-head ratio and MRI fetal lung volume as a percentage of the expected parameter value. The new MRI relative lung-to-head ratio was assessed as a percentage of the expected MRI lung-to-head ratio based on MRI fetal lung volume and MRI head circumference measurements. Measurements for survival and ECMO requirement were determined with the area under the curve (AUC). Data were analyzed for left-sided defects, right-sided defects, and associated liver herniation. Among fetuses with left-sided CDH, all parameters were excellent in determining neonatal survival and need for ECMO therapy (p <or= 0.0027). Prognostic accuracy was best for the newly devised MRI relative lung-to-head ratio (AUC, 0.816 and 0.807) and lowest for sonographic relative lung-to-head ratio (AUC 0.783 and 0.703). Among fetuses with right-sided defects, the predictive value was lower for all parameters (AUC, 0.788-0.560). All neonates without liver herniation survived. Among fetuses with left-sided CDH, assessment of pulmonary hypoplasia based on MRI relative fetal lung volume and MRI relative lung-to-head ratio is excellent in prediction of neonatal survival and ECMO requirement. The prognostic accuracy is slightly better than that of sonographic relative lung-to-head ratio. Among fetuses with right-sided CDH, the prognostic value of all parameters is lower than those among fetuses with left-sided defects.

Congenital Diaphragmatic Hernia. The Post-Neonatal Period (Part II)

The aim of the study was to review our experience in the management of newborns with congenital diaphragmatic hernia (CDH). A retrospective study including all infants with CDH at the Hospital de São João, a center that does not provide ECMO support, for the period from 1997 to 2006. Since 2003, a new treatment protocol has been used. There were 61 newborns (30 male/31 female) with a birth weight of 2800 g (880 - 3770), and a gestational age of 38 weeks (28 - 41); 46 (75 %) were inborn and 42 (69 %) had a prenatal diagnosis of CDH. There were 2 (3 %) chromosomal anomalies, 3 (5 %) with other congenital anomalies and 1 (2 %) with nonimmune hydrops fetalis. The diaphragmatic defect was left sided in 55 (90 %) cases. Corrective surgery was performed in 43 (70 %) patients. New therapies were used: HFOV 13 % (n = 8); inhaled nitric oxide 13 % (n = 8); and sildenafil 7 % (n = 4). We found that systemic arterial hypotension (p = 0.001), the severity of pulmonary hypertension (p = 0.001), prenatal diagnosis (p = 0.006), birth weight (p = 0.022), female gender (p = 0.029), inborn birth (p = 0.030), arterial pH < 7.35 at admission (p = 0.030), right-sided defect (p = 0.033) and pneumothorax (p = 0.033) to be predictive of mortality. The overall survival rate was 43 % (n = 26), and since 2003 this rate has improved to 61 % for term neonates without other congenital or chromosomal anomalies. Our survival rate for infants with CDH has improved over the last ten years, and this improvement is associated with the use of new therapies such as HFOV, inhaled nitric oxide and sildenafil.

Congenital Diaphragmatic Hernia – The Neonatal Period (Part I)

The aim of the study was to review our experience in the management of newborns with congenital diaphragmatic hernia (CDH). A retrospective study including all infants with CDH at the Hospital de São João, a center that does not provide ECMO support, for the period from 1997 to 2006. Since 2003, a new treatment protocol has been used. There were 61 newborns (30 male/31 female) with a birth weight of 2800 g (880 - 3770), and a gestational age of 38 weeks (28 - 41); 46 (75 %) were inborn and 42 (69 %) had a prenatal diagnosis of CDH. There were 2 (3 %) chromosomal anomalies, 3 (5 %) with other congenital anomalies and 1 (2 %) with nonimmune hydrops fetalis. The diaphragmatic defect was left sided in 55 (90 %) cases. Corrective surgery was performed in 43 (70 %) patients. New therapies were used: HFOV 13 % (n = 8); inhaled nitric oxide 13 % (n = 8); and sildenafil 7 % (n = 4). We found that systemic arterial hypotension (p = 0.001), the severity of pulmonary hypertension (p = 0.001), prenatal diagnosis (p = 0.006), birth weight (p = 0.022), female gender (p = 0.029), inborn birth (p = 0.030), arterial pH < 7.35 at admission (p = 0.030), right-sided defect (p = 0.033) and pneumothorax (p = 0.033) to be predictive of mortality. The overall survival rate was 43 % (n = 26), and since 2003 this rate has improved to 61 % for term neonates without other congenital or chromosomal anomalies. Our survival rate for infants with CDH has improved over the last ten years, and this improvement is associated with the use of new therapies such as HFOV, inhaled nitric oxide and sildenafil.