Search

Background/purpose: The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. A recent report from CDH Study Group showed that dimension of diaphragmatic defect is the only independent risk factor of mortality. However, the influence of defect size on late morbidity is still controversial. The aim of the study was to evaluate the influence of patch repair (proxy of diaphragmatic defects size) on midterm morbidity., Methods: All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 hours of life) CDH survivors treated at our institution from 2004 to 2008 were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary, and orthopedic evaluations were performed at 6, 12, and 24 months of age. Patient outcomes were compared with respect to +/- patch repair., Results: Of 70 survivors, 61 (87%) were enrolled and prospectively evaluated in follow-up. Poorer auxological outcome, increased rate of gastroesophageal reflux, and altered pulmonary function test were observed during follow-up., Conclusions: Patch repair correlates with higher pulmonary, auxological, and gastroesophageal morbidity without increasing chest wall deformities at long-term follow-up., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Aim of the Study: Infants with congenital diaphragmatic hernia (CDH) are at high risk of sensorineural hearing loss (SNHL). Extracorporeal membrane oxygenation is known to increase this risk, but little is known about other potential causes. We evaluated the impact of several risk factors on SNHL development in CDH survivors not treated with extracorporeal membrane oxygenation., Methods: All high-risk CDH survivors consecutively treated between 1999 and 2005 were included. SNHL was diagnosed based on formal assessment with standard audiologic tests. Patients with and without SNHL were compared for patient-related and treatment-related risk factors. Subsequently, a logistic regression analysis was performed to identify independent risk factors associated with SNHL development., Main Results: Out of 87 CDH survivors, 82 had a formal audiologic evaluation and 40 (49%) had SNHL. Patients with SNHL had significantly lower gestational age (P = .045); higher prevalence of sepsis (P < .001); older age at audiologic examination (P < .001); more episodes of hypocapnia (P = .045); higher prevalence of inhaled nitric oxide use (P = .005); longer mechanical ventilation (P = .009); and longer aminoglycosides (P = .006), furosemide (P = .004), and pancuronium bromide (P = .001) treatments. On logistic regression analysis, the only variable independently associated with the development of SNHL was patient's age at audiologic follow-up (P = .012)., Conclusions: Several risk factors were associated with SNHL development at univariate analysis. After logistic regression, only age at evaluation remained independently associated with SNHL. Routine audiologic follow-up is advocated in all CDH patients. Further studies are needed to define if other (genetic) factors may be involved in the pathogenesis of SNHL in patients with CDH.

In a newborn with severe respiratory failure and abnormal elevation of the right diaphragm, congenital diaphragmatic hernia with sac was diagnosed during surgery. However, microscopic examination of the sac showed atrophic striated muscle cells, indicating eventration instead of hernia. After several extubation failures, the final diagnosis of nemaline myopathy was made by skeletal muscle biopsy. In diaphragmatic defects with sac, diaphragm microscopic analysis should be recommended in order to discriminate between hernia and eventration. Congenital myopathies may underlie such diaphragmatic defects and should be promptly recognized, given their prognostic implications.

Background: Despite improvements in clinical management, mortality of congenital diaphragmatic hernia (CDH) remains high. Early prediction of mortality risk helps in comparing strategies and/or performances of different centers. Birth weight (BW), Apgar Score at 5 minutes, and modified McGoon Index (MGI) calculated by the ratio between the diameters of pulmonary arteries and the descending aorta have been used to determine mortality of CDH., Aim: The purpose of this study is to evaluate the relationship between early detectable variables and survival in newborns with CDH intubated at birth, managed with "gentle" ventilation and delayed surgery., Methods: All medical records of patients affected by high-risk CDH and treated with a standardized protocol at Bambino Gesù Children's Hospital, Rome, Italy, between January 2002 and September 2004 were reviewed. Prenatal diagnosis, gestational age, BW, sex, side of hernia, and MGI were recorded on admission. The relationship with mortality of each variable was evaluated by univariate analysis. Subsequently, a predictive model of mortality was developed using a logistic regression: the explanatory variables, BW, and MGI were dichotomized in high (HBW and HMGI) and low (LBW and LMGI) according to the best cutoff found with receiver-operating characteristic curves., Results: Thirty-four newborns with CDH, treated with a standardized protocol, were studied. The main characteristics of the 34 patients were BW, 2886 g (1500-3620 g); gestational age, 37.7 weeks (32-42 weeks); male/female, 22/12; right/left, 8/26; prenatal diagnosis, 29; MGI, 1.31 (0.9-1.85). Only BW and MGI were significantly (P < .05) associated with mortality at the univariate analysis. The best cutoff values were 2755 g for BW (sensitivity, 70%; specificity, 74%) and 1.25 for MGI (sensitivity, 73%; specificity, 78%). Using these limits, BW and MGI resulted independently associated with mortality in the multivariate analysis. Using the 4 possible combinations, the LBW associated with the LMGI presented the highest prediction of mortality (80%)., Conclusions: Birth weight and MGI, variously combined, were predictive of mortality. Because they are not influenced by subsequent modalities of care, they can be considered as valid early severity scores in CDH and used for comparing strategies and/or performances of different centers.

Background: Cardiorespiratory stabilization is recommended before surgical repair of congenital diaphragmatic hernia (CDH) because surgery may induce a transitory deterioration of chest compliance and gas exchange. It is not known if surgical intervention can affect cerebral circulation and oxygenation., Aim: The aim of the study was to assess noninvasively, by near-infrared spectroscopy, the possible changes in cerebral hemodynamics and oxygenation associated with surgical repair of CDH., Subjects: Twenty-five newborns with severe CDH (birth weight, 3057 +/- 354 g; gestational age, 37.8 +/- 1.8 weeks; male/female newborns, 15/10; left/right CDH, 19/6) were sedated, paralyzed, and mechanically ventilated by conventional gentle ventilation and surgically corrected at a median age of 2.7 days (min-max, 2-14 days) after cardiorespiratory stabilization., Methods: Heart rate (HR [beats per minute]), preductal transcutaneous oxygen saturation (tcSaO2 [%]), carbon dioxide tension (tcPCO2 [Torr]), and mean arterial blood pressure (mm Hg) were continuously monitored. Inspired fractional oxygen concentration (FIO2) was adjusted to maintain and preductal tcSaO2 of greater than 80%, whereas the ventilator's settings were kept unchanged throughout the surgical procedure. Cerebral hemodynamics was assessed by near-infrared spectroscopy (NIRO 300, Hamamatsu Photonics, Japan), recording continuously and noninvasively the relative changes in concentration of oxygenated (DeltaO2Hb [micromol/L]), deoxygenated (DeltaHHb [micromol/L]), and total (DeltatHb [micromol/L]) hemoglobin; the tissue oxygenation index (TOI [%]) was also calculated (TOI = O2Hb/O2Hb + HHb). Total hemoglobin concentration is considered to be representative of cerebral blood volume. Arterial blood gases were also measured at the beginning (T1) and at the end of surgery (T2). For all measurements, results at T1 and at T2, as well as the differences between T1 and T2, have been expressed as means or medians and SDs or 95% confidence intervals or ranges. The differences between T1 and T2 were considered statistically significant for a P value of less than .05 by the Student t test for paired values., Results: At T1, mean tcSaO2% was 94.1 % (SD, 4.6) with a FIO2 of 0.25 (SD, 0.1); at T2, to obtain similar values of tcSaO2 (93.4%; SD, 4.4), it was necessary to increase the FIO2 to 0.37 (SD, 0.14; P < .001). Mean HR at T1 was 149.5 beats per minute (SD, 9.1) and increased significantly (P < .05) at T2 (165.2 beats per minute; SD, 14.2). Mean arterial blood pressure was 54.7 mm Hg (SD, 7.7) at T1 and did not change appreciably at T2 (55.6 mm Hg; SD, 8.1). Moreover, tcPCO2 did not change significantly during the procedure (mean tcPCO2 = 49.9 Torr [SD, 12.8] at T1 and 57.3 mm Hg [SD, 17.9] at T2). O2Hb and tHb decreased (P < .001 and <.005) and HHb increased (P < .05) significantly during the surgical procedure (mean Delta [SD]: DeltaO2Hb= -10.9 micromol/L [9.7], DeltatHb = -7.5 micromol/L [11.7], and DeltaHHb = -3.5 micromol/L [6.8]). Mean TOI was 70% at T1 (normal values >60%) and decreased significantly at T2 (mean DeltaTOI = -6.1% [SD, 10.6]). In all infants, the greatest changes occurred when the viscera were positioned into the abdomen., Conclusions: Notwithstanding the initial cardiorespiratory stabilization, surgical repair of CDH was associated with a rise in HR and oxygen requirement and a drop in cerebral tHb and O2Hb, suggesting a reduction in cerebral blood volume and oxygenation. These events were probably due to the combined effects of an increase in right to left shunting (as indicated by the increased oxygen requirement) and a decrease in venous return (possibly due to compression of the inferior vena cava by the viscera positioned into the abdomen). These preliminary results reinforce the importance of achieving a good cardiorespiratory stability before undertaking surgical correction of CDH to minimize the possible interference of the procedure with cerebral circulation and oxygenation.