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Objective:To investigate the treatment of preterm and low birth weight infants with congenital diaphragmatic hernia (CDH) and to share the experience.Methods:This retrospective study enrolled 117 newborns with CDH who underwent major surgery at Children's Hospital, Capital Institute of Pediatrics from May 1, 2011, to March 31, 2022. Based on gestational age and birth weight, the infants were divided into the preterm and/or low birth weight group (gestational age < 37 weeks and/or birth weight less than 2 500 g, n=41) and the control group (gestational age ≥ 37 weeks and birth weight ≥ 2 500 g, n=76). Furthermore, the preterm and/or low birth weight infants were divided into the thoracoscopic surgery subgroup ( n=31) and the open surgery subgroup ( n=10) according to the surgical approach. Statistical analysis of the data was performed using two independent sample t-tests, rank sum tests, Chi-square test, or Fisher's exact probability test. Results:Preoperative data showed that the Apgar scores at 1 min [7.0 (6.0-8.0) vs 9.0 (8.0-9.8), Z=－4.03] and 5 min [9.0 (8.0-10.0) vs 9.0 (9.0-10.0), Z=－2.13] of the preterm and/or low birth weight infants were both lower than those in the control group (both P<0.05), while the proportion of infants with moderate to severe pulmonary hypertension was higher [68.3% (28/41) vs 38.2% (29/76), χ 2=9.68, P<0.05]. There were no statistically significant differences between the two groups in terms of the proportion of thoracoscopic surgery, operation time, right diaphragmatic hernia, presence of hernia sac, grading of the defect, presence of liver herniation, and application of mesh (all P>0.05). Regarding the postoperative outcomes, the death rate in the preterm and/or low birth weiht group was higher compared to the control group [36.6% (15/41) vs 13.2% (10/76), χ 2=8.70, P<0.05]. Additionally, the time required to resume full enteral nutrition after surgery was longer in the preterm and/or low birth weight group than that in the control group [25 d (18-29 d) vs 16 d (10-25 d), Z=2.31, P<0.05]. The thoracoscopic subgroup had a lower mortality compared to the open surgery subgroup [25.8% (8/31) vs 7/10, P<0.05]. The thoracoscopic surgery subgroup had a higher Apgar score at 1 min after birth [(7.4±1.6) vs (6.0±2.2), t=2.20, P<0.05], later age at operation (hours after birth) [31.0 h (23.0-48.0 h) vs 17.0 h (4.7-24.5 h), Z=2.57, P<0.05], a lower proportion of infants operated within 24 hours after birth [32.3% (10/31) vs 8/10, P<0.05], and longer duration of operation [170.0 min (122.0-200.0 min) vs 110.0 min (87.3-120.0 min), Z=3.65, P<0.05]. Conclusions:In this study, a higher mortality in the preterm and/or low birth weight group compared to the control group was observed, which may be attributed to the higher proportion of neonates with moderate-severe pulmonary hypertension. The thoracoscopic diaphragmatic repair can be attempted for preterm and low birth weight infants who have relatively stable respiratory and circulatory functions.
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Abstract Objective Antenatal recognition of severe cases of congenital diaphragmatic hernia (CDH) by ultrasound (US) and magnetic resonance imaging (MRI) may aid decisions regarding the indication of fetal endoscopic tracheal occlusion. Methods An integrative review was performed. Searches in MEDLINE and EMBASE used terms related to CDH, diagnosis, MRI, and US. The inclusion criteria were reviews and guidelines approaching US and MRI markers of severity of CDH published in English in the past 10 years. Results The search retrieved 712 studies, out of which 17 publications were included. The US parameters were stomach and liver positions, lung-to-head ratio (LHR), observed/expected LHR (o/e LHR), and quantitative lung index. The MRI parameters were total fetal lung volume (TFLV), observed/expected TFLV, relative fetal or percent predicted lung volumes, liver intrathoracic ratio, and modified McGoon index. None of the parameters was reported to be superior to the others. Conclusion The most mentioned parameters were o/e LHR, LHR, liver position, o/e TFLV, and TFLV.
Resumo Objetivo A identificação pré-natal de casos graves de hérnia diafragmática congênita (HDC) por ultrassonografia (US) e ressonância magnética (RM) pode ajudar a decidir sobre a indicação de oclusão traqueal endoscópica fetal. Métodos Uma revisão integrativa foi realizada pesquisando nas bases MEDLINE e EMBASE comtermos relativos a HDC, diagnóstico, RM, e US. Os critérios de inclusão foram revisões e diretrizes abordando marcadores ultrassonográficos e de ressonância para a gravidade de HDC publicados em inglês nos últimos 10 anos. Resultados Foram obtidos 712 estudos, dos quais 17 foram incluídos. Os parâmetros de US foram posições do estômago e do fígado, relação pulmão-cabeça (LHR, na sigla em inglês), LHR observada/esperada (o/e LHR), e índice pulmonar quantitativo (QLI, na sigla em inglês). Os parâmetros de RM foram volume pulmonar fetal total (TFLV, na sigla em inglês), o/e TFLV, volume pulmonar fetal relativo e porcentagem predita, razão do fígado intratorácico (LiTR, na sigla em inglês) e índice de McGoon modificado. Nenhum dos parâmetros foi mencionado como superior aos demais. Conclusão Os parâmetros mais citados foram o/e LHR, LHR, posição do fígado, o/e TFLV, e TFLV.
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Humans , Female , Pregnancy , Reference Standards , Magnetic Resonance Imaging , Patient Selection , Hernias, Diaphragmatic, Congenital/diagnostic imagingABSTRACT
Objective:To investigate the prediction value of observed to expected lung area to head circumference ratio (o/e LHR), measured at different gestational age with various methods, on indication for extracorporeal membrane oxygenation (ECMO) in fetuses with isolated left-sided congenital diaphragmatic hernia (CDH).Methods:Clinical data of 40 neonates who were diagnosed with left-sided CDH and treated in Guangzhou Women and Children's Medical Center were retrospectively collected from January 2017 to May 2021. The o/e LHRs were prenatally calculated using maximum diameter and tracing method at 22-24 and 31-33 weeks of gestation. According to whether the neonates had indications for ECMO after birth or not, they were divided into ECMO ( n=12) or non-ECMO group ( n=28). Differences in the o/e LHR and general situations between the two groups were analyzed using C hi-square test, independent sample t-test, and non-parametric Mann-Whitney U test. Binary logistic regression was used to analyze the influencing factors for ECMO requirement and receiver operating characteristic (ROC) curve was used to evaluate the value of o/e LHR in predicting the indication for ECMO. Results:Both maximum diameter and tracing method suggested that the o/e LHR at 31-33 gestational weeks was lower than that at 22-24 gestational weeks [maximun diameter method: 40.4 (32.9-51.5) vs 45.1 (36.3-53.4), Z=－2.48, P=0.013; tracing method: 38.6 (33.2-47.6) vs 44.1 (35.9-51.7), Z=－3.29, P=0.001]. There was no statistical difference in o/e LHR detected at the same gestational weeks between the two methods (both P>0.05). Binary logistic regression showed that o/e LHR measured at 31-33 gestational weeks using maximum diameter method was an independent protective factor for ECMO requirement ( OR=0.873, 95% CI: 0.790-0.965, P=0.008). ROC curve analysis showed that the area under the curve for evaluating the predictive value of o/e LHR for ECMO requirement was 0.830 with the sensitivity of 83.3% and the specificity of 71.4% when the cut-off value of o/e LHR at 31-33 gestational weeks was 38.195 measured by maximum diameter method. Conclusions:The o/e LHR measured at 31-33 weeks is lower than that at 22-24 weeks of gestation by both methods. The o/e LHR measured by maximum diameter method at 31-33 weeks of gestation may be useful for predicting the ECMO indication after birth but requiring comprehensive evaluation of clinical conditions due to its insufficient predicting power.
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Objective:To investigate the influencing factors of the survival in infants with congenital diaphragmatic hernia (CDH) and to develop a prediction model for CDH.Methods:Clinical data of 252 infants with a prenatal diagnosis of CDH in Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine from January 2010 to December 2018 were retrospectively analyzed. Adverse outcomes were defined as neonatal death on discharge. Chi-square and t test were used for univariate analysis of 16 perinatal and five postnatal risk factors. Regression analysis was used to determine the independent predictors of survival. The receiver operating characteristics (ROC) curves of the risk factors for predicting the survival of CDH were drawn. A prediction model based on the combination of risk factors for predicting adverse outcomes of CDH was established using the cut-off value. ROC curves of the model were drawn and the area under curve (AUC), sensitivity, and specificity were calculated. Results:Out of the 252 patients, 173(68.7%) survived on discharge. Univariate analysis showed that lung-to-head ratio (LHR), polyhydramnios, right diaphragmatic hernia, liver herniation, intrathoracic stomach, premature birth, low birth weight, 5 min Apgar score <7, and amniotic fluid index (AFI) were significantly associated with the survival of CDH. Regression analysis showed that polyhydramnios ( OR=11.19,95% CI:2.83-45.33), liver herniation ( OR=2.81,95% CI:1.32-11.92), intrathoracic stomach ( OR=5.02, 95% CI:1.29-17.13), low birth weight ( OR=8.58,95% CI:1.59-45.01) and AFI ( OR=3.68, 95% CI:1.37-14.72) were independent risk factors for survival at discharge in children with CDH, while LHR ( OR=0.36, 95% CI:0.01-0.69) were protective factors. The predictive cut-off values of LHR, polyhydramnios, liver herniation, intrathoracic stomach, low birth weight, and AFI were 1.6, 1.0, 1.0, 1.0, 1.0, and 232.5 mm, respectively. The model based on the combination of the above indicators for predicting CDH adverse outcomes was shown with an AUC value of 0.904, predictive sensitivity of 0.747, and specificity of 0.896. Conclusions:In this study, LHR, liver herniation, intrathoracic stomach, polyhydramnios, low birth weight, and AFI are independent risk factors for CDH survival. The combination of prenatal and postnatal indicators is noted for a higher accuracy for predicting CDH survival.
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Symptoms and prognosis of infants with congenital diaphragmatic hernia (CDH) are various, as all mild cases might survive while those severe ones have higher mortality. Therefore, an applicable tool that can screen out high-risk CDH children may help clinicians develop an individualized treatment plan and increase the survival rate. A growing number of international studies for CDH postnatal prediction tools, including single factor index and five score models, have been conducted in recent years, whereas few in China. We hereby summarized the advances in postnatal evaluation tools to predict the outcomes of CDH.
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Symptoms and prognosis of infants with congenital diaphragmatic hernia (CDH) are various,as all mild cases might survive while those severe ones have higher mortality.Therefore,an applicable tool that can screen out high-risk CDH children may help clinicians develop an individualized treatment plan and increase the survival rate.A growing number of international studies for CDH postnatal prediction tools,including single factor index and five score models,have been conducted in recent years,whereas few in China.We hereby summarized the advances in postnatal evaluation tools to predict the outcomes of CDH.
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Objective To investigate the clinical value of abnormal course of fetal superior mesenteric arteries (SMA) detected by ultrasound during the first trimester (11-13+6 weeks) in predicting congenital diaphragmatic hernia (CDH). Methods This study enrolled women who underwent fetal nuchal translucency (NT) screening during the first trimester in Central Hospital of Panyu District from March to December 2017. Low-speed high-definition flow imaging was used to observe the course of fetal SMA, and it was regarded as abnormal when the angle between SMA and abdominal aorta >90°. Once abnormal course of the SMA was suspected, the position of fetal thoracic cavity and abdominal organs would be scanned carefully. Furthermore, ultrasound examinations would be repeated at 16-18, 20-24, 28-32 and 37-40 weeks of gestation. Fetus diagnosed as CDH by ultrasound would be scheduled for MRI or autopsy to confirm the diagnosis. Pregnancy outcomes of all cases were followed up by telephone. Descriptive statistical analysis was used in this study. Results A total of 6 899 gravidas (6 964 fetuses) underwent NT scan during the first trimester were enrolled and the SMA of all fetuses were successfully displayed. Three cases with abnormal course of the SMA were identified. Two of them were diagnosed with left CDH at 17+ and 23+ weeks of gestation, which was confirmed by autopsy after termination of pregnancy, and the other one terminated pregnancy in first trimester due to a large omphalocele. Among the 6 961 fetuses with normal SMA, the pregnancy outcomes of 6 120 were successfully followed up, only one of which was found to have left CDH at 32 gestational weeks by ultrasound examination, and was later confirmed by neonatal MRI after delivery. No other fetal CDH was detected. Conclusions Abnormal course of the SMA identified in early pregnancy may be a simple and effective indicator for CDH that allow early intervention and treatment.
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Abstract Purpose: To evaluate the vascular ventilatory response in different stages of lung development and to compare them to the neonates with congenital diaphragmatic hernia (CDH) in a rabbit model. Methods: New Zealand rabbits were divided into 8 groups (n=5): E25, E27, E30, and CDH. All groups were ventilated on a FlexiVent (Scireq, Montreal, QC, Canada), compounding the other 4 groups. The CDH surgery was performed at E25 and the harvest at E30. Dynamic compliance (CRS), dynamic elastance (ERS) and dynamic resistance (RRS) were measured every 4 min/24 min. Median wall thickness (MWT) and airspace were measured. ANOVA Bonferroni tests were used to perform statistical analysis. Significance was considered when p<0.05. Results: CRS was higher in E30 compared to all other groups (p<0.05). CRS and RRS of CDH and E27 were similar and were higher in E25 (p<0.05). MWT was decreased according to the gestational age, was increased in E27V and E30V (p<0.05) and decreased in CDHV (p<0.05), airspace was decreased in E25 and increased in all ventilated groups (p<0.05). Conclusions: The ventilation response of congenital diaphragmatic hernia is like the pseudoglandular stage of the lung development. These findings add information about the physiology of pulmonary ventilation in CDH.
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Animals , Rabbits , Respiratory Mechanics/physiology , Hernias, Diaphragmatic, Congenital/physiopathology , Lung/growth & development , Respiratory Function Tests , Diaphragm/surgery , Total Lung Capacity , Airway Resistance , Disease Models, Animal , Hernias, Diaphragmatic, Congenital/etiology , Lung/physiopathology , Lung/blood supply , Animals, NewbornABSTRACT
Objective To evaluate the diagnostic value of MRI-measured fetal percent predicted lung volume (PPLV) for the prognosis of left congenital diaphragmatic hernia (CDH) in fetus. Methods Clinical data of 32 children who were admitted to Women and Children's Medical Center of Guangzhou from September 2012 to December 2017 for prenatally diagnosed left CDH were retrospectively analyzed. These children were divided into two groups, the survival group (n=24) and the death group (n=8), according to the postoperative outcomes at 30 days after CDH repair. Moreover, they were also divided into non-pulmonary hypertension (non-PH) group (n=20) and PH group (n=12), based on whether they suffered from PH or not. Clinical data such as gestational age, birth weight, Apgar score and PPLV values in different groups were compared with t- or Fisher's exact test. The receiver operating characteristic (ROC) curve of the MRI-measured fetal PPLV values of the 32 children was plotted. Results (1) Comparing with the death group, fetal PPLV was significantly higher [(39.5±2.5)% vs (20.4±2.1)%, t=4.27], the gestations on initial diagnosis of CDH was later [(31.6±4.2) vs (25.4±4.6) gestational weeks, t=3.40], Apgar score of the neonates at 5 min was higher (8.7±1.5 vs 5.7±3.8, t=3.26), and fewer cases of PH were reported in the survival group [16.7% (4/24) and 8/8], all P<0.01. The area under the ROC curve of PPLV values for mortality prediction was 0.930 (95%CI: 0.843-1.016, P<0.01). When the PPLV value was 28.55%, its sensitivity and specificity for death prediction in children with left CDH were 100% and 79%, respectively. (2) Comparing with the PH group, fetal PPLV was significantly higher [(41.7±2.6)% vs (23.0±2.0)%, t=4.98], the gestations on initial diagnosis of CDH was later [(32.3±3.4) vs (26.3±5.2) gestational weeks, t=3.81], neonatal Apgar score at 5 min was higher (8.6±1.4 vs 6.8±2.5, t=2.62) and death rate was lower [0(0/20) vs 8/12] in the non-PH group (all P<0.01). The area under the ROC curve of the PPLV values for predicting PH was 0.902 1 (95%CI : 0.800-1.004, P＜0.01). When the PPLV value was 33.67%, its sensitivity and specificity for PH prediction was 100% and 75%, respectively. Conclusions Prenatal MRI measurement of PPLV can be used to predict death or as a warning sign of PH in children with left CDH, which may provide evidence for prenatal evaluation and rational clinical decision-making.
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Congenital diaphragmatic hernia (CDH) is caused by unilateral or bilateral diaphragmatic hypoplasia allowing abdominal organs to move into the thoracic,resulting in a series of pathophysiological changes,high mortality and poor prognosis.Therefore,earlier diagnosis is conducive to prenatal intervention and postnatal treatment.At present,prenatal ultrasonography is the most common used examination for CDH due to its convenience and low cost.MRI is complementary to ultrasound examination as it can evaluate both pulmonary development and maturity.Fetal echocardiography is a newly developed method in recent years which helps to indicate the prognosis through observation of hemodynamics and pulmonary vessels development.
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Objective To investigate the integrated perinatal management and prognosis of prenatally diagnosed congenital diaphragmatic hernia (CDH).Methods Clinical data of 32 cases of neonatal CDH that were diagnosed and treated in the Third Affiliated Hospital of Zhengzhou University from June 2015 to June 2017 were collected and retrospectively analyzed.All cases were classified into mild,moderate and severe groups based on prenatal lung-to-head ratio (LHR),observed to expected LHR and observed to expected total fetal lung volume.The integrated perinatal management included prenatal management (prenatal diagnosis and consultation),intrapartum management (delivery) and postnatal management (postnatal diagnosis,neonatal treatment and surgical treatment).Neonatal outcomes were analyzed.Statistical analysis was performed using Chi-square test and t-test.Results All of the 32 cases were diagnosed by prenatal ultrasonography and assessed by MRI.There were 18 mild,nine moderate and five severe CDH cases,respectively.Left-sided and right-sided CDH were respectively found in 27 and five cases.Thirty neonates underwent surgery when the diagnosis had been confirmed after birth and the conditions were stable after comprehensive treatment.The total survival rate was 81％ (26/32).The neonatal survival rates of severe and mild groups were 1/5 and 18/18,respectively.The more severe the CDH,the lower the survival rate (x2=16.538,P＜0.001).Among the 18 neonates with mild CDH,five neonates underwent minimally invasive laparoscopic surgery had shorter fasting time,duration of postoperative antibiotic administration and hospital stay than those received open surgery (n=13)[(2.4±0.5) vs (4.6±1.2) d,t=-3.939;(7.6±1.2) vs (9.8±1.4) d,t=-3.144;(14.4±1.1) vs (19.7±2.8) d,t=-4.064;all P＜0.01].Neonates who were discharged alive received a three months to two years postoperative treatment and follow-up and one received a second operation due to recurrence and all recovered.Conclusions Prognosis of neonatal CDH are closely related to prenatal conditions.Integrated management is of great significance in CDH.