Survival of children born with congenital anomalies

Aim: To describe the survival to age 5 years of children born with congenital anomalies. Methods: Between 1980 and 1997, 6153 live born cases of congenital anomaly were diagnosed and registered by the population based Glasgow Register of Congenital Anomalies. They were retrospectively followed to assess their survival status from birth up to the age of 5 years. Results: The proportions of all live born infants with congenital anomalies surviving to the end of the first week, and first and fifth year were 94%, 89%, and 88%, respectively. Survival to age 5, the end point of follow up, was significantly poorer for infants with chromosomal anomalies (48%) compared to neural tube defects (72%), respiratory system anomalies (74%), congenital heart disease (75%), nervous system anomalies (77%), and Down''s syndrome (84%). Conclusion: Although almost 90% of all live born infants with congenital anomalies survive to 5 years, there are notable variations in survival between anomaly types. Our findings should be useful for both clinicians and geneticists to assess the prognosis of congenital anomalies. This information is also important for affected families and for the planning of health care needs for this high risk population.     

Survival of children born with congenital anomalies.

AIM: To describe the survival to age 5 years of children born with congenital anomalies. METHODS: Between 1980 and 1997, 6153 live born cases of congenital anomaly were diagnosed and registered by the population based Glasgow Register of Congenital Anomalies. They were retrospectively followed to assess their survival status from birth up to the age of 5 years. RESULTS: The proportions of all live born infants with congenital anomalies surviving to the end of the first week, and first and fifth year were 94%, 89%, and 88%, respectively. Survival to age 5, the end point of follow up, was significantly poorer for infants with chromosomal anomalies (48%) compared to neural tube defects (72%), respiratory system anomalies (74%), congenital heart disease (75%), nervous system anomalies (77%), and Down's syndrome (84%). CONCLUSION: Although almost 90% of all live born infants with congenital anomalies survive to 5 years, there are notable variations in survival between anomaly types. Our findings should be useful for both clinicians and geneticists to assess the prognosis of congenital anomalies. This information is also important for affected families and for the planning of health care needs for this high risk population.     

Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia?

OBJECTIVES: To determine the impact of surfactant replacement on survival, need for extracorporeal membrane oxygenation (ECMO), and chronic lung disease in term infants with prenatally diagnosed congenital diaphragmatic hernia (CDH). STUDY DESIGN: Prenatally diagnosed infants born at > or =37 weeks' gestation with immediate distress at delivery and no other major congenital anomalies, who were enrolled in the CDH Registry, were analyzed. For univariate analysis, chi 2 tests were used for categoric variables and unpaired t tests for nominal variables. Multiple logistic regression was used to calculate adjusted odds ratios. RESULTS: Eligible infants (n = 522) were identified. Demographic variables were similar between the surfactant-treated (n = 192) and nonsurfactant-treated (n = 330) groups, with the exception of race (white, 88.0% vs 71.2%; P =.0007). The use of ECMO and incidence of chronic lung disease were higher (59.8 vs 50.6, P =.04; 59.9 vs 47.6, P =.0066) and survival lower in the surfactant-treated cohort (57.3 vs 70.0, P =.0033). Adjusted logistic regression for use of ECMO, survival, and chronic lung disease resulted in odds ratios inconsistent with an improved outcome associated with surfactant use. CONCLUSIONS: This analysis shows no benefit associated with surfactant therapy for term infants with a prenatal diagnosis of isolated CDH.     

Congenital diaphragmatic hernia: two case studies with atypical presentations

Congenital diaphragmatic hernia (CDH) affects 1 in every 2,000 to 4,000 live births. Many infants with this condition are diagnosed antenatally through routine ultrasound screening. Nearly 90 percent present at delivery with severe respiratory distress requiring intubation. Many of these infants develop persistent pulmonary hypertension of the newborn due to hypoplasia of the affected lung. The survival of infants with CDH is limited by the degree of pulmonary hypoplasia and requires sophisticated medical technology such as high-frequency ventilation and inhaled nitric oxide. Some infants also require treatment with extracoporeal membrane oxygenation. This article gives details of two cases of CDH in which the presentation was atypical. The more subtle presentation is discussed, as well as the embryology and pathophysiology of CDH and the possibility of associated anomalies. Clinical management and impact on the family are outlined.     

Reduction in ventilator-induced lung injury improves outcome in congenital diaphragmatic hernia?

The purpose of this historical study was to compare the outcome for two treatment strategies, for neonates with congenital diaphragmatic hernia (CDH). The records of 65 infants born between 1991 and 2005 with CDH from a single tertiary care perinatal centre in the United Kingdom were retrospectively reviewed. Conventional mechanical ventilation (CMV) and systemic vasodilators were used from 1991 to 1995 (era 1). High frequency oscillatory ventilation (HFOV) and nitric oxide (NO) were used between 1996 and 2005 (era 2). Main outcome measures were survival and incidence of chronic lung disease. The results showed that the survival rate was 38% (8/21) in era 1 and 73% (32/44) in era 2, 95% CI for difference −59 to −10%. The incidence of chronic lung disease in survivors was 45% (5/11) in era 1 and 30% (9/30) in era 2, 95% CI for difference −18 to 49%. These data show significantly improved survival with elective use of HFOV and NO compared to CMV and systemic vasodilators. The survival results for CDH at St George’s Hospital are comparable to those published from other institutions. The results may reflect a reduction in ventilator-induced lung injury with HFOV compared to CMV.     

Extracorporeal membrane oxygenation and the management of congenital diaphragmatic hernia

Uniform survival in infants with congenital diaphragmatic hernia (CDH) remains an elusive goal. Those that qualify for extracorporeal membrane oxygenation (ECMO) represent a subgroup that has a predicted mortality of 90%. This report summarizes an experience over 5 years of offering ECMO to all CDH infants with an oxygen index > 40 for 2 h. No infants were excluded on the basis of a minimum PaO₂ or other criteria. Thirty-eight infants were treated and 24 survived (63%). Patients were grouped into survivors and nonsurvivors. There was no difference in the average worst pre-ECMO PaO₂ or PaCO₂, while the pH values were less in those that died (P <.01). The average best pre-ECMO PaO₂ of 113 mm Hg in survivors was significantly different from that of 44 mm Hg observed in nonsurvivors (P <.05). Of 24 survivors, 14 would have died as a result of exclusion if a minimum pre-ECMO PaO₂ of 50 mm Hg had been required. Pre-ECMO CDH repair was performed in 15 patients with 3 deaths; repair was performed during ECMO in 17 patients with 6 deaths. In all patients except 2, ECMO could be discontinued within several hours of repair. No improvement in lung function was seen in 5 patients at 10 to 14 days and bypass was discontinued without repair. Dynamic lung compliance and end-tidal CO₂ as well as the ability to be weaned from ECMO were used as measures of lung function. A heparin/Carmeda bonded circuit and reduced heparin were used successfully in 3 patients to treat bleeding complications. It is concluded that all infants should be resuscitated with ECMO. Survival in this series was signficantly increased over historical controls treated without ECMO (P <.01). The timing of CDH repari did not appear to affect survival. With over 37% of patients still dying from this disease, future investigation is necessary to define the role fo therapies such as fetal surgery and neonatal lung transplantation as well as to improve current therapy.     

Familial severe congenital diaphragmatic hernia: Left herniation in one sibling and bilateral herniation in another

Familial congenital diaphragmatic hernia (CDH) is extremely rare; it comprises about 2% of all CDH cases. The empirical risk is about 2%, increasing to 10% in a family with two affected children. This report describes severe CDH in two siblings who had been diagnosed prenatally. The female newborn diagnosed with left CDH prenatally was born at 38 weeks of gestation. Despite surgical repair and intensive treatment, she died 10 days after birth. Her younger brother was born at 39 weeks of gestation after being diagnosed with bilateral CDH prenatally, and died 75 min after birth. Both infants had neither other congenital anomaly nor chromosomal abnormalities. Their parents are healthy without consanguinity. Their first daughter and the fourth child have no congenital anomalies.     

Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis

This retrospective study reviews the medical records of 77 fetuses and babies with congenital diaphragmatic hernia (CDH) referred to two hospitals in Detroit from 1986 through 2000. The aims were to examine the effects on outcome of multiple variables, especially the type of CDH, associated anomalies, and ultrasound prognostic parameters. Ultrasound measurements of head (HC), chest (CC), and abdominal circumferences (AC) were obtained from videotapes. ANOVA and chi-square analysis were used to determine statistical significance between groups and proportions. Eighty-nine percent (65/73) of pregnancies resulted in live births, and 54% (35/65) of patients survived past 30 days. Liveborn patients with low APGAR scores were less likely to survive. Forty-three percent (30/70) had major associated anomalies, with cardiac anomalies constituting about 52% (33/64) of the major associated anomalies. Seventy percent of patients with isolated CDH survived versus 36% of patients with both CDH and cardiac anomalies. Sixty-seven percent (8/12) of fetuses antenatally diagnosed before 25 weeks of gestation survived past 30 days of birth. The survival rate of right-sided CDH with liver herniation was 80% (8/10), compared with 29% (4/14) for left-sided CDH with liver herniation (p=0.088). There was a significant linear relationship (r=0.603, p =0.029) between CC/AC and CC/HC among patients with CDH; survivors had higher CC/AC and CC/HC values than nonsurvivors. These results support the utility of CC/AC and CC/HC measurements and the presence of liver herniation as important prognostic factors that can be used in antenatal counseling and in planning clinical trials.     

Abnormal circulatory stress responses of preterm graduates

Preterm birth and chronic lung disease may increase the risk of hypertension and cardiovascular disease in infancy and adolescence. Here we looked for evidence of early circulatory dysfunction associated with these perinatal complications. We compared infants born at term (n = 12) with those born preterm with an uncomplicated neonatal course (n = 12) or diagnosed with bronchopulmonary dysplasia (BPD) (n = 10). We measured blood pressure (BP) (Finometer), and heart rate (HR) responses to 4 min of breathing 4% CO2 during quiet sleep. Hypercapnia accelerated HR and increased BP of term infants. Preterm infants either (i) had an exaggerated pressor but little or no HR response to CO2 (healthy or mild-moderate BPD) or (ii) had a diminished pressor response and accompanying decrease in HR (severe BPD). Short-term reflex cardiovascular control was consequently altered by premature birth, with potentially more serious aberrations associated with severe BPD. Most anomalies had not resolved by the time infants born preterm reached term age; some may be early signs of emerging long-term cardiovascular dysfunction.     

Outcome of low birth weight infants in a West African village

Low birthweight (LBW) infants are at risk of increased mortality, impaired growth, and increased morbidity. We studied the progress of all LBW infants (less than 2.5 kg) born in a West African village where there is a resident pediatrician and midwife. There were 426 live births during the 8 years studied of which 42 (10 per cent) were of LBW. There were 17 infant deaths (IMR = 40/1000). Mean birthweight and gestation of infants dying in the first year were significantly lower than those of all live birth (2.33 kg v. 2.99 kg, P less than 0.001; 36.9 w v. 38.9 w, P less than 0.05). The mean weights of the 32 surviving LBW infants (15 preterm, 17 full term, small for gestational age) were compared with those of 32 full term, appropriate for gestational age matched controls over the first year. Growth velocities of paired groups were identical. The significant differences in weight at 1 year (P less than 0.005) reflected those at birth (P less than 0.001). There were no significant differences in clinic attendance rate or incidence of diarrhoea and respiratory disease between the four groups. We conclude that LBW is a strong risk factor for infant mortality, but not for diarrhoeal and respiratory morbidity in a community with ready access to obstetric and pediatric care.     

A clinical analysis of prognostic parameters of survival in children with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) still has a high mortality because of accompanying lung hypoplasia and persistent pulmonary hypertension. Although prognostic parameters based on perinatal measurements have been proposed, our ability to accurately predict the surgical results remains insufficient. METHODS: We treated 55 infants with CDH from 1981 to 2004. Among them, 46 patients presented respiratory distress within the first 24 hours of life. Results of surgical treatment in the 46 infants were retrospectively correlated with gender, birth weight, gestational age at diagnosis, laterality, cardiac anomalies, diaphragmatic defect area, contents of herniated viscera, and the great vessel diameters measured by echocardiography. RESULTS: Out of 46 CDH neonates, 27 (58.7 %) survived and 19 (41.3 %) died aged 3 to 17 days. Non survivors had a significantly larger diaphragmatic defect and more frequent liver herniation. Out of possible predictive parameters studied, an index of the main pulmonary artery (cross-sectional area/diaphragmatic defect area ratio) most closely correlated with the surgical outcomes. CONCLUSIONS: The postoperative prognosis of CDH infants does not depend only on pulmonary hypoplasia, but also on other factors including the magnitude of abdominal visceral herniation. In this series of patients, the most reliable prognostic predictor was a clinical index reflecting the degree of both pulmonary hypoplasia and diaphragmatic maldevelopment.     

Clinical outcomes of congenital diaphragmatic hernia without extracorporeal membrane oxygenation

Background

Neonatal extracorporeal membrane oxygenation (ECMO) is not available in Hong Kong.

Objectives

To document the survival of neonates with symptomatic congenital diaphragmatic hernia at birth, but without access to ECMO.

Methods

Twenty-two patients diagnosed to have CDH within a ten year period (1999–2009) at Prince of Wales Hospital were systematically reviewed. CDH patients who presented after the neonatal period were excluded.

Results

There were 17 neonates with symptomatic CDH at birth and the overall survival, including infants with multiple anomalies, was 14/17 (82%). 6 of 17 (35%) infants met the ECMO criteria and the survival rate for these serious cases was 4/6 (67%).

Conclusions

Our results are comparable with centers which provide ECMO and suggest that there may be only marginal benefit for using ECMO to improve survival. A territory-wide registry for documenting risk factors and outcomes would be important, especially in light of improving neonatal intensive care and survival.     

Low birthweight in Hausa infants

As infants with low birth weights (LBWs) constitute a group in need of specialized care, the problems of LBW among the Hausas of Nigeria were evaluated. The case records of all liveborn infants of Hausa parents, born at the Maternity Hospital in Katsina, Nigeria between January 1, 1974 and December 31, 1977, were selected for study. The LBW infants i.e., those weighing 2500 g or below were separated, their characteristics studied, and possible etiological factors identified. A comparison of this group was then made with those neonates who weighed more than 2500 g. The duration of gestation was determined from the menstrual history and by appropriate clinical examination before delivery. As data were not considered to be very reliable, no attempt was made to correlate the birth weights with duration of pregnancy. Infants born before the 37th week of pregnancy were labeled as premature. During the study period, 3890 live Hausa infants (2111 males and 1779 females) were born to 3780 mothers. Of these 3890 infants, 408 males and 420 females weighed 2500 g or less. These 828 infants were born to 774 mothers and included 91 sets of twins and 5 sets of triplets though the outcome of all multiple pregnancies were not live births. The maternal age ranged from 13-45 years. The parity ranged from 0-14 but there were more primigravida compared to other parities. All mothers belonged to lower and middle socioeconomic classes. 70% were urban and 30% were rural. The incidence of LBW was 213/1000 live births or 21.3%. 71.1% of these babies weighed between 2000 g and 2500 g; only 1.6% weighed less than 1000 g. The percentage of females among LBW infants was higher (50.7%) as compared to that of males (49.3%). The incidence of LBW was 19.3% among males and 23.6% among females. The monthly and seasonal incidence of LBW was uniform and no seasonal variation could be found. The incidence of LBW was 18.4% among urban women and 23.4% among rural women. The difference was highly significant. The highest percentage of deaths in the present study occurred among infants weighing less than 1000 g and the immediate neonatal death rate declined in each successive higher weight group, exhibiting a strong relationship between LBW and immediate neonatal mortality. Only 1 child died out of 589 who weighed between 2001-2500 g.     

Congenital diaphragmatic hernia - the neonatal period (part I)

AIM: The aim of the study was to review our experience in the management of newborns with congenital diaphragmatic hernia (CDH). METHODS: A retrospective study including all infants with CDH at the Hospital de S?o Jo?o, a center that does not provide ECMO support, for the period from 1997 to 2006. Since 2003, a new treatment protocol has been used. RESULTS: 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. CONCLUSIONS: 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.     

Sudden infant death syndrome among twins.

BACKGROUND: Sudden infant death syndrome (SIDS) is a major contributor to infant mortality. Previous studies have suggested that infants born of twin pregnancies are at greater risk for SIDS and that a twin who survives after a co-twin dies is at increased risk for SIDS. OBJECTIVE: To attempt to confirm the increased risk of SIDS among and within twin pairs through the use of US vital statistics data. METHODS: We analyzed data from the US-linked birth and infant death certificate tapes for the years 1987 through 1991 to determine the risk of SIDS in twin births compared with singleton births and to describe the characteristics of twin pairs in whom SIDS occurred. The analysis was limited to live births with weights of 500 g or more and gestational ages of 24 weeks or more. We used an algorithm to match co-twins (infants within a twin pair) to measure sex and birth weight concordancy, to identify twin pairs, in which one or both twins died of SIDS; and to examine, when both twins died, whether they died on the same day. RESULTS: There were 23464 singleton SIDS deaths and 1056 twin SIDS deaths during the 5-year period. The crude relative risk for SIDS among twins compared with singleton births was 2.06 (95% confidence interval, 1.94-2.19). The adjusted relative risk independent of birth weight and sociodemographic variables was 1.13 (95% confidence interval, 0.97-1.31). We successfully matched the co-twins of 172029 twin pregnancies. Of these, 767 were twin pregnancies in which one or both twins died of SIDS. Among the 767 twin pregnancies in which one or both twins experienced SIDS, there were only 7 in which both twins died of SIDS (rate ratio, 4.0 per 100000 twin pregnancies). In only 1 of these 7 did both twins die on the same day (rate ratio, 0.58 per 100000 twin pregnancies). The relative risk for a second twin dying of SIDS was 8.17 (90% confidence interval, 1.18-56.67). CONCLUSIONS: Independent of birth weight, twins do not appear to be at greater risk for SIDS compared with singleton births. In addition, the occurrence of both twins dying of SIDS is uncommon, and the occurrence of both twins dying on the same day is extremely uncommon.     

Prognosis following prenatal diagnosis of heart malformations

Our objective was to document the prognosis of cases with fetal heart malformations (FHM). Forty-two fetuses assessed both in a regional fetal medicine and paediatric cardiology unit were classified prenatally into isolated FHM or those associated with extra-cardiac structural or karyotypic anomalies (ECA) and this classification was not changed subsequently (analogous to an intention to treat analysis). The end points studied included chromosomal abnormality, pregnancy outcome and follow-up at one year of age.FHM were isolated in 16 (38%) and associated with ECA in 26 (62%) of cases. The karyotypic abnormality rate was 8/42 (19%) overall and 8/26 (31%) in ECA cases. The pregnancy outcome included termination of pregnancy in 19 (45%), intrauterine death of two (5%) and live birth in 21 (50%). 12/16 (75%) of isolated FHM cases were live born compared with 9/26 (35%) of ECA cases (P<0.03). Of the isolated FHM live born babies, 8/12 (67%) were alive at the end of the first year and seven of these were growing normally and did not require cardiac medication. However, a considerable proportion of their first year was spent in hospital (median 8%, interquartile range 5–10). Only one of nine ECA live born cases was alive but with poor growth and dependence on cardiac drugs at one year.These data confirm previous findings in prenatal diagnosis series that the prognosis for FHM is worse than that reported in studies of congenital heart disease at birth and is strongly dependent upon the presence of ECA. In their absence, outcome is better than previously published. Counselling must take place only after full fetal medicine assessment and should be based upon prenatal data.     

Congenital defects in the newborn--difficulties in prenatal diagnosis

The aim of this work is an analysis of developmental defects in newborn infants, born in the Clinical Department of Pregnancy Pathology and Reproduction in Zabrze, during the 19 months of its existence. Difficulties in prenatal diagnosis of developmental defects are presented and their structure is discussed. The study included 1524 live born infants with birth weight over 500 g and 12 stillborn. In the live born group developmental defects were found in 71 (4.6%) infants. Among the 12 stillborn infants developmental defects were present in one child (8.3%). The most common developmental defects, in our material, were those of the skeletal system and heart defects. Prenatally, developmental defects were diagnosed in 8% of the examined foetuses. The quality of health care in pregnant mothers of the investigated newborn infants was assessed, with relation to medical examination, ultrasonographic scan and biochemical screening tests. In many cases, modern standards of management were not maintained.     

Prevalence and distribution of children with congenital heart diseases in the central Anatolian region, Turkey

Congenital heart diseases (CHD) are the most frequent malformation at birth. The aims of this study were to assess the prevalence of congenital heart disease, their different types, and the detection rate among children in the central Anatolian region in Turkey. The study was conducted during an eight-year period (March 1995-December 2002). The prevalence of CHD in a large tertiary care hospital in the central Anatolian region in Turkey was studied. The diagnosis of a structural defect was based on echocardiographic study. The following age groups were considered: neonates, infants and toddlers, preschool children, schoolchildren, and adolescents. In the study period, 1,693 children were found to have CHD; 1253 patients were neonates and infants. Total prevalence of CHD over the study period was 7.77 per 1000 live-born. The prevalence increased from 6.35 to 9.65 per 1000 live births between 1995 and 2002 (p < 0.05). The average age at diagnosis was 2.2 +/- 3.64 years (1 day to 18 years, median 5 months). There were 863 (51%) boys and 830 (49%) girls, with a male/female ratio of 1:1. Isolated ventricular septal defect (32.6%) was the most frequent acyanotic anomaly, and tetralogy of Fallot (5.8%) was the most frequent cyanotic anomaly. The commonest non-cardiac anomalies with CHD were musculoskeletal anomalies. Down syndrome was determined in 83 patients (78.3%) from all syndromic CHD cases. Congenital heart disease is a very significant health problem. It requires urgent measures in terms of organization of early diagnosis and proper management. The prevalence rate is comparable to that of similar developed countries. Increasing incidence of CHD might be attributed to more diagnoses with new technologic development or it may indicate a real increase in the defects.     

Outcome after preterm delivery of infants antenatally diagnosed with congenital heart disease

OBJECTIVE: To determine outcome of delivery before 36 weeks gestation in babies diagnosed antenatally with serious congenital heart disease (CHD). STUDY DESIGN: A retrospective database review at 2 tertiary care fetal cardiology centers. Details of neonatal course and outcome were obtained for those antenatally diagnosed with serious CHD who were live born before 36 weeks gestation. RESULTS: Between January 1998 and December 2002, 9918 women were referred for fetal echocardiography. Serious CHD was diagnosed in 1191 fetuses (12%), of which 46 (4%) delivered prematurely. Median gestation was 33 (range 24-35) weeks, and median birth weight 1.56 (0.50-3.59) kg. Extracardiac/karyotypic anomalies occurred in 23 (50%). Twenty-six babies (57%) underwent neonatal surgery: 16 a cardiac procedure, 5 a general surgical procedure, and 5 both. Eight died during or after operation (31%). Two babies underwent interventional heart catheterization; both died. The overall mortality rate was 72%. Extracardiac/karyotypic anomalies increased the relative risk of death by a factor of 1.36. Mean hospital stay for those surviving to initial discharge was 46 (2-137) days. CONCLUSIONS: There is a very high morbidity and mortality rate in this group, particularly for those with extracardiac/karyotypic anomalies. This should be reflected in decisions over elective preterm delivery and when counseling parents.     

Prenatal sonographic chest and lung measurements for predicting severe pulmonary hypoplasia in left-sided congenital diaphragmatic hernia

To assess the prenatal evaluation of lung hypoplasia in congenital diaphragmatic hernia (CDH), we attempted to measure the right lung area/thorax area ratio (rLT ratio) in normal fetuses and in seven cases of left-sided CDH. In addition, we analyzed early neonatal blood gas data, which were compared with the prenatal evaluation. The rLT ratio was significantly (p<0.05) higher in normal fetuses (0.27+/-0.02) than in CDH (0.14+/-0.18). The values of PaO(2), arterial-alveolar oxygen difference (A-aDO(2)) and oxygenation index (OI) showed no significant relationship with the rLT ratio in CDH at the early neonatal period. Three infants with CDH survived and showed significant higher values of rLT ratio compared with those in nonsurvived infants (p<0.05). All of the rLT ratios in nonsurvived infants were <0.11. PaO(2) at the early neonatal period was significantly (p<0.05) higher, and both A-aDO(2) and OI were significantly (p<0.05) lower in survived infants than in nonsurvived infants. These results indicated that prenatal evaluation of rLT ratio is useful to predict the severity of lung hypoplasia in infants with left-sided CDH, and blood gas analysis at early neonatal period is also useful to predict the neonatal outcome.