Cases Of Congenital Diaphragmatic Hernia Research Articles

Two Cases of Congenital Diaphragmatic Hernia Manifestated with Atypical Symptoms in Newborn Infants

Two Cases of Congenital Diaphragmatic Hernia Manifestated with Atypical Symptoms in Newborn Infants

Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study

IntroductionPublished prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of...

Expanded intrathoracic space in fetal cases of isolated congenital diaphragmatic hernia contributes to disparity between percent predicted lung volume and observed to expected total lung volume.

The aim of this study was to determine whether fetal lung volume and visceral herniation are associated with changes in intrathoracic space in congenital diaphragmatic hernia(CDH). We retrospectively examined the relationship between magnetic resonance imaging-derived measurements of intrathoracic space [predicted lung volume (PLV)] and residual lung volume or visceral herniation among isolated left-sided CDH fetuses. Data from fetal magnetic resonance imaging studies of 60 isolated left-sided CDH cases were analyzed. The median PLV of the CDH fetuses was found to be much greater than the expected total lung volume (eTLV) of a normal fetus at the same gestational age. Surprisingly, liver herniation and observed TLV(oTLV) were positively correlated with the PLV. Although the PPLV was consistently less than the o/eTLV, both indices were greater in survivors than in non-survivors, whereas no significant difference was seen in the PLV/eTLV ratio in regard to survivorship. The intrathoracic domain available for lungs and viscera is expanded in CDH fetuses and positively affected by the lung volume and the presence of liver herniation, leading to the difference in the PPLV and o/eTLV. Future study of intrathoracic space as it relates to the growth of the lung and herniated viscera is needed to better characterize the relationship between these parameters.

Repair of congenital diaphragmatic hernia under high-frequency oscillatory ventilation in high-risk patients: an opportunity for earlier repair while minimizing lung injury.

BACKGROUNDRepair of congenital diaphragmatic hernia (CDH) is ideally delayed until ventilatory parameters are stabilized and patients are switched to conventional ventilation. However, in selected high-risk patients, repair can be performed earlier while they are still on high-frequency oscillatory ventilation (HFOV).DESIGN AND SETTINGSA retrospective review of all CDH cases treated in our tertiary referral center between 1997 and 2013.METHODSIn 1997, we started repairing selected high-risk CDH cases under HFOV with or without inhaled nitric oxide (iNO). All repairs were performed once the infants’ blood gas levels were acceptable. The infants were gradually weaned to conventional ventilation followed by extubation as their ventilatory parameters improved. Their records were reviewed to determine the group-wide outcomes.RESULTSBetween 1997 and 2013, 55 infants with CDH were treated in our institute; of these 12 high-risk cases were repaired under HFOV/iNO combinations and 1 was repaired without iNO. All patients had significant pulmonary hypertension and 8 had herniated livers. The mean age at repair was 9.1 (6.3) days. Two mortalities occurred at the first and tenth postoperative days. Among the remaining 11 survivors, the median ventilation and hospitalization days were 29.5 (11–84) and 45.5 (25–107), respectively, and the median duration under HFOV and conventional ventilation days were 15 (9–40) and 12 (3–47), respectively.CONCLUSIONCDH repair can be performed earlier under HFOV and iNO. The possible advantages are earlier restoration of normal anatomy and earlier start of enteral feeding while minimizing the risk of lung injury.

Prenatal diagnosis and fetal outcome of 57 cases of congenital diaphragmatic hernia

Objective To explore the prenatal diagnosis and factors influencing prognosis of congenital diaphragmatic hernia (CDH).Methods Fifty seven cases of CDH prenatally diagnosed by ultrasound in the Department of Obstetrics,Xinhua Hospital from January 1,2006 to March 31,2013 were retrospectively reviewed.Prognosis and risk factors were analyzed by ultrasound characteristics and newborn status.Statistical analysis was performed using t test,x2 test and Logistic regression.Results Forty-five (79％)of all cases were left-side CDH and the rest 12 (21％)were right-side.Fourteen fetuses were aborted.Thirty three were delivered and treated surgically,twenty-three infants survived after surgery and ten died,with an overall survival rate of 40％ (23/57).The other ten cases were delivered,but died of no treated surgically.The birth weight of the surviving neonates was heavier than that of those who died [(3 173 ±348) vs (2 846±568)g,t=2.238,P=0.033].The gcstational age at diagnosis of the aborted fetuses was younger than that of the surviving neonates [(24.0 ± 4.1) vs (30.0± 6.0) weeks,t=3.181,P=0.003].Twenty-one (91％,21/23) of the surviving neonates had a lung-to-head ratio (LHR) ≥ 1.4.A linear trend test showed a negative correlation between LHR and adverse outcome (x2=9.223,P=0.002) with a correlation coefficient-0.730 (P=0.000).LHR＜1.4 (OR=35.867,95％CI:1.861-691.341,P=0.018) and polyhydramnios (OR=9.285,95％CI:1.102-78.212,P=0.040) were risk factors for neonatal death.Right-side CDH (OR=0.158,95％CI:0.009-2.824,P=0.210),liver herniation into the thorax (OR=1.730,95％CI:0.144 20.707,P=0.665) and birth weight (OR=l.002,95％CI:0.999-1.005,P=0.135) were not risk factors.Conclusions The survival rate of CDH is still low.Gestational age on diagnosis,presence of polyhydramnios and LHR＜ 1.4 predict an adverse outcome. Key words: Hernia, diaphragmatic; Ultrasonography, prenatal; Prognosis

Prognostic factors of gastroesophageal reflux disease in congenital diaphragmatic hernia: a multicenter study

Gastroesophageal reflux disease (GERD) is one of the concomitant problems in infants with congenital diaphragmatic hernia (CDH). We assessed risk factors of GERD in CDH patients. The retrospective observational study for CDH infants was conducted. Cases of CDH who were born between January 2006 and December 2010, were operated in the 9 participating institutions, and survived to discharge were included. Completion of medical therapy for GERD and incidence of surgery were primary outcomes. Kaplan-Meier survival analysis and Cox proportional hazards regression were used. In 182 cases of CDH, the medical therapies for GERD were performed in 23.8% (40/168), and were completed in 60.0% (24/40). Prenatal detection of CDH (HR 5.87, CI 1.6-18.8, p = 0.012) and tube feeding at discharge (HR 5.04, 95% CI 1.3-33.1, p = 0.016) were significantly correlated with unsuccessful weaning from medical therapy. Surgery for GERD was performed in 10.7% (18/169). Gestational age (HR 4.78, 95% CI 1.5-21.1, p = 0.006) and diaphragmatic defect of more than 75% (HR 4.3, 95% CI 1.6-12.9, p = 0.005) were significantly correlated with need for antireflux surgery. Diaphragmatic defect of more than 75% was risk factor of future need for antireflux surgery.

Delayed Presentation of Congenital Diaphragmatic Hernia as Heart Failure

Congenital diaphragmatic hernia (CDH) is rare congenital condition that primarily presents in infants. Bochdalek hernia is the most common of them. Late presentation is unusual, and in most cases is diagnosed in adolescents or early adulthood. Morgagni hernia is the one that usually presents late in adults. The finding of CDH in adults is mostly incidental. We report a case of CDH, posterolateral (Bochdalek type), with left lung hypoplasia, in a 60 year old female, who primarily presented with sudden shortness of breath.

Fetal surgery: an overview.

In utero fetal surgery interventions are currently considered in selected cases of congenital diaphragmatic hernia, cystic pulmonary abnormalities, amniotic band sequence, selected congenital heart abnormalities, myelomeningocele, sacrococcygeal teratoma, obstructive uropathy, and complications of twin pregnancy. Randomized controlled trials have demonstrated an advantage for open fetal surgery of myelomeningocele and for fetoscopic selective laser coagulation of placental vessels in twin-to-twin transfusion syndrome. The evidence for other fetal surgery interventions, such as tracheal occlusion in congenital diaphragmatic hernia, excision of lung lesions, fetal balloon cardiac valvuloplasty, and vesicoamniotic shunting for obstructive uropathy, is more limited. Conditions amenable to intrauterine surgical treatment are rare; the mother may consider termination of pregnancy as an option for many of them; treatment can be lifesaving but in itself carries risks to both the infant (preterm premature rupture of the membranes, preterm delivery) and the mother. This makes conducting prospective or randomized trials difficult and explains the relative lack of good-quality evidence in this field. Moreover, there is scanty information on long-term outcomes. It is recommended that fetal surgery procedures be performed in centers with extensive facilities and expertise. The aims of this review were to describe the main fetal surgery procedures and their evidence-based results and to provide generalist obstetricians with an overview of current indications for fetal surgery.

Fetal Cardiac Tamponade in a Case of Right-Side Congenital Diaphragmatic Hernia

Intrafetal fluid collection is a rare ultrasound finding in fetuses with right-side congenital diaphragmatic hernia. Our patient had a fetus with a large right-side congenital diaphragmatic hernia with a significant amount of the fetal liver herniated into the chest. At 31 weeks of gestation, the fetus had significant ascites and high-pressure intrathoracic fluid accumulation, hydrops fetalis, deviation of the mediastinum, and tamponade-like physiology that compromised cardiac function. Ultrasound-guided fetal thoracoamniotic and peritoneal amniotic shunt placement markedly improved the fetal cardiac function and resolved the hydrops. The patient delivered at 34 weeks of gestation because of preterm labor and the neonate had an uncomplicated diaphragmatic hernia repair 3 days after delivery. Fetal thoracoamniotic shunting in selected cases of congenital diaphragmatic hernia with hydrops may improve the prognosis.

Fetoscopic endoluminal tracheal occlusion (FETO) for congenital diaphragmatic hernia in Australia and New Zealand: Are we willing, able, both or neither?

An estimated 140 pregnancies are diagnosed with congenital diaphragmatic hernia (CDH) in Australia and New Zealand each year, with these fetuses having a less than even chance of 1-year survival. Fetoscopic endoluminal tracheal occlusion (FETO) is a relatively new technique that offers a prenatal interventional strategy for selective cases of CDH. This is not routinely offered in Australia or New Zealand. The aim of this systematic review is to critically appraise controlled clinical trials investigating the role of FETO in moderate and severe isolated CDH and explore whether this treatment is justified within our region. A systematic literature search of multiple electronic databases was undertaken, with restrictions to human subjects and controlled clinical trials. Nine relevant studies were identified. No current evidence was found in favour of FETO for moderate severity CDH. For severe CDH, the most recent evidence demonstrates significantly improved survival following FETO performed using contemporary percutaneous minimally invasive techniques. Optimum timing for balloon insertion, removal and occlusion duration remains conjectural. Substantial variation in survival rates observed among control groups highlights the impact of post-natal care in prenatally diagnosed CDH. Until recently, evidence to support a role for FETO in prenatal CDH management was weak. Recently reported and ongoing controlled trials give cause for optimism, with improved FETO safety and increased survival reported for severe CDH cases. Should Australasia embrace FETO for selected CDH cases, a co-ordinated, evidence-informed service should be established under the guidance of experienced international partnerships.

Antenatally diagnosed congenital diaphragmatic hernia in Singapore: a five-year series

INTRODUCTION We aimed to investigate the epidemiology, diagnosis, prognostication, follow-up care and outcomes of antenatally diagnosed congenital diaphragmatic hernia (CDH) in KK Women's and Children's Hospital (KKH), Singapore. The objective of this study was to identify trends in current practice, and evaluate and improve the management of CDH. METHODS We retrospectively reviewed cases of antenatally diagnosed CDH from 2006 to 2010. RESULTS A total of 22 cases of CDH were found, bringing its incidence in KKH to 3.6 per 10,000 live births. The mean gestational age at diagnosis was 22.7 weeks, with 14 (63.6%) cases diagnosed at < 22 weeks and 8 (36.4%) diagnosed at ≥ 22 weeks. All cases were left-sided - 15 (68.2%) were isolated CDH and 7 (31.8%) were associated with other anomalies. Of the 22 cases, counselling about the prognosis of pregnancy based on the lung-to-head ratio was provided in 9 (40.9%). Overall, 10 (45.5%) cases resulted in termination of pregnancy (TOP), 9 (40.9%) resulted in live birth and 1 (4.5%) in stillbirth; 2 (9.1%) cases were lost to follow-up prior to delivery. Of the 9 live births, 3 (33.3%) resulted in neonatal death. In the 10 births, 5 (50.0%) were delivered by normal vaginal delivery, 4 (40.0%) by emergency Caesarean section and 1 (10.0%) by elective Caesarean section. CONCLUSION CDH is a challenging perinatal problem with a low overall survival rate. Almost half of the cases opt for TOP. The data in this study can help clinicians better undertake the task of adequately counselling parents with qualitative and quantitative prognostic factors, using an evidence-based approach.

Surgical intervention for congenital diaphragmatic hernia: open versus thoracoscopic surgery

To compare open repair (OR) with thoracoscopic repair (TR) for congenital diaphragmatic hernia (CDH) in neonates. Twenty-four neonatal CDH cases diagnosed prenatally or within 6 h of birth at our institute from 2002 to 2012 with mild pulmonary hypertension managed without inhaled nitric oxide, were studied. OR was routine until 2006 (n = 14; L:R = 12:2) and TR became routine in 2007 (n = 10; L:R = 10:0). All subjects had identical management. Gestational age at birth: OR 37.4 ± 1.6 vs. TR: 38.3 ± 1.1 weeks (p = 0.10); birth weight 2,636 ± 490 vs. 2,887 ± 429 grams (p = 0.20); preoperative A-aDO(2): 308 ± 200 vs. 331 ± 195 mmHg (p = 0.79); and operating time (min): 161 ± 42 vs. 194 ± 76 (p = 0.27). In TR, intraoperative cardiopulmonary status was stable, intraoperative hemorrhage was significantly less (4.8 ± 6.0 vs. 1.1 ± 0.1 grams; p = 0.038), wound cosmesis was excellent without surgical site infections, while, maximum postoperative CRP (2.0 ± 1.5 vs. 1.5 ± 1.1 mg/dL; p = 0.30), commencement of enteral feeding (6.9 ± 2.8 vs. 5.6 ± 2.8 days; p = 0.27), and length of postoperative hospitalization (36 ± 22 vs. 34 ± 12 days; p = 0.66) were improved, but not significantly. One OR case required surgery for intestinal obstruction. Thoracoscopic repair appears to be as effective as OR for treating selected cases of CDH in neonates, with excellent wound cosmesis.

Antenatally diagnosed congenital diaphragmatic hernia in Singapore: a five-year series

We aimed to investigate the epidemiology, diagnosis, prognostication, follow-up care and outcomes of antenatally diagnosed congenital diaphragmatic hernia (CDH) in KK Women's and Children's Hospital (KKH), Singapore. The objective of this study was to identify trends in current practice, and evaluate and improve the management of CDH. We retrospectively reviewed cases of antenatally diagnosed CDH from 2006 to 2010. A total of 22 cases of CDH were found, bringing its incidence in KKH to 3.6 per 10,000 live births. The mean gestational age at diagnosis was 22.7 weeks, with 14 (63.6%) cases diagnosed at < 22 weeks and 8 (36.4%) diagnosed at ≥ 22 weeks. All cases were left-sided - 15 (68.2%) were isolated CDH and 7 (31.8%) were associated with other anomalies. Of the 22 cases, counselling about the prognosis of pregnancy based on the lung-to-head ratio was provided in 9 (40.9%). Overall, 10 (45.5%) cases resulted in termination of pregnancy (TOP), 9 (40.9%) resulted in live birth and 1 (4.5%) in stillbirth; 2 (9.1%) cases were lost to follow-up prior to delivery. Of the 9 live births, 3 (33.3%) resulted in neonatal death. In the 10 births, 5 (50.0%) were delivered by normal vaginal delivery, 4 (40.0%) by emergency Caesarean section and 1 (10.0%) by elective Caesarean section. CDH is a challenging perinatal problem with a low overall survival rate. Almost half of the cases opt for TOP. The data in this study can help clinicians better undertake the task of adequately counselling parents with qualitative and quantitative prognostic factors, using an evidence-based approach.

Strangulated bowel as an unusual complication of neonatal congenital diaphragmatic hernia: A case report and literature review

Strangulation of bowel in utero causing infarction has been rarely reported in neonates. We report a case of congenital diaphragmatic hernia with bowel incarceration, requiring substantial resection of necrotic bowel. Despite a prolonged hospitalization, the patient had a good outcome and was discharged home nippling full enteral feeds. Three previous cases with a similar diagnosis were identified through a literature review and suggest a similar pattern of initial patient presentation.

Effect of hospital volume on the mortality of congenital diaphragmatic hernia in Japan

During the last decade, new supportive modalities and new therapeutic strategies to treat congenital diaphragmatic hernia (CDH) have been introduced. In Japan, the large number of hospitals prevents centralizing infants with CDH in tertiary centers. The aim of this study was to evaluate the correlations between the number of CDH patients, survival rates, and the current strategies employed to treat CDH at the individual hospitals. Eighty-three hospitals with 674 CDH cases were analyzed using questionnaires. We classified the hospitals into three groups according to the number of CDH patients treated: Group 1 (G1; more than 21 patients), Group 2 (G2; 11-20 patients), and Group 3 (G3; fewer than 10 patients). The median number of CDH patients in G1, G2, and G3 were 28, 14, and 4, respectively. The overall survival rate was 74.5%. When only the isolated CDH cases with a prenatal diagnosis were included, the overall survival rate was 79.3%. The survival rate of isolated CDH cases with a prenatal diagnosis was significantly higher in G1 than that in G2 or G3 (87.2% vs 75.2% vs 74.3%; P < 0.001). There were no differences in perinatal therapeutic strategies among the three groups. We concluded that it might therefore be important to centralize infants with CDH, especially those with isolated CDH with a prenatal diagnosis, to tertiary centers in Japan in order to improve the survival rates.

The current profile and outcome of congenital diaphragmatic hernia: A nationwide survey in Japan

Background/PurposeFew nationwide surveys have been reported regarding the perinatal status, clinical course and postnatal outcome of cases with congenital diaphragmatic hernia (CDH). The aim of this study was to review the current profile and the outcomes of a large cohort of CDH cases in Japan. MethodsA nationwide retrospective cohort study was conducted on neonates diagnosed to have CDH between January 2006 and December 2010. The questionnaires were sent to 159 representative regional institutions and 109 (68.6%) institutions responded to the preliminary survey which had 674 cases. Eleven institutions which had 60 CDH neonates did not respond to the second questionnaire, and 26 institutions had no cases. Finally, 614 CDH neonates from 72 institutions had been collected and were used in the detailed survey. The perinatal status, clinical course and the postnatal outcome were reviewed. Survival was defined as infants alive at hospital discharge, at the time of transfer or still in the hospital at the time of questionnaire, which was confirmed during the period from July 2011 to November 2011 by the investigators. ResultsFour hundred sixty-three (75.4%) of 614 CDH neonates survived in this study. The overall survival rate of neonates with isolated CDH was 84.0%. A total of 444 (72.0%) patients were prenatally diagnosed, and had a survival rate of 70.8%. Four hundred thirty-three (70.9%) patients were treated with high-frequency oscillatory ventilation (HFOV) as the initial ventilation, 344 (56.0%) patients received inhaled nitric oxide (iNO) and 43 (7.0%) required extracorporeal membrane oxygenation (ECMO). The overall survival rates of the CDH neonates who had been treated using HFOV, iNO and ECMO were 74.3%, 68.3% and 37.2%, respectively. ConclusionsThis study demonstrated that the current status for CDH treatment in Japan and the overall survival rate were comparable to those of recent reports from other countries.

Prognostic value of a hernia sac in congenital diaphragmatic hernia

To investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH). Our database was searched to identify all consecutive cases of CDH referred to our fetal medicine unit between January 2004 and August 2011. Presence or absence of a hernia sac was assessed in liveborn cases using surgery or postnatal autopsy reports. We studied the correlation between the presence of a hernia sac and prenatal findings and perinatal morbidity and mortality. Over the study period, there were 70 cases with isolated CDH born alive in which either a surgery or autopsy report was available. Neonatal death, either preoperative or postoperative, occurred in 1/18 (5.6%) infants with a hernia sac and in 17/52 (32.7%) cases without a hernia sac (P = 0.03). Patients with a hernia sac had a significantly higher observed to expected pulmonary volume on prenatal magnetic resonance imaging (51.9 vs 39.3%, P = 0.01). Neonatal morbidity in surviving infants was lower in the group with a hernia sac, although not significantly. The presence of a hernia sac is associated with a higher pulmonary volume and a better overall prognosis for CDH.

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.

Anesthetic management of a case of congenital diaphragmatic hernia; delayed diagnosis

Congenital diaphragmatic hernia results from the anomalous closure of pericardioperitoneal canal. There are various challenges faced by anesthesiologists during management of such cases which include intraoperative complications including hypoxia and hypercarbia, which leads to pulmonary hypertension and right to left shunt. Mortality remains high because of associated pulmonary hypoplasia and pulmonary hypertension. Here, we describe anesthetic management of a 2-month-old female child with difficulty in breathing since birth following feeding, due to congenital diaphragmatic hernia.

Amniotic lamellar body count and congenital diaphragmatic hernia in humans and in a rat model

We examined the extent of fetal lung hypoplasia and lung maturation using the amniotic lamellar body count (LBC) in congenital diaphragmatic hernia (CDH). We obtained 30 amniotic fluid samples from human CDH cases during cesarean section. We assessed LBC, magnetic resonance imaging (MRI), and ultrasound findings for predicting the prognosis of CDH. We collected newborn amniotic fluid and lung tissue at embryonic day (E)21 from normal and nitrofen-induced CDH rats (administered 100 mg orally at E9). Amniotic LBCs in rats were measured using light microscopy. In human CDH, LBC was significantly higher in the surviving than in the deceased group (P < 0.01). A significant positive correlation was observed between LBC and percentage of fetal lung volume on MRI (P < 0.001; r = 0.716). In rats, LBC was significantly higher in controls than in CDH rats (P < 0.01) and correlated with fetal lung weight. We conclude that LBC is useful for predicting lung hypoplasia in human CDH after 35 gestational weeks and in a rat model of nitrofen-induced CDH.