Congenital Posterolateral Diaphragmatic Hernia Research Articles

Congenital diaphragmatic (Bochdalek) hernia of the fetus

Congenital diaphragmatic hernia (CDH) is a developmental malformation characterized by a defect in the diaphragm that allows abdominal viscera to move into the chest cavity. A routine ultrasound screening performed during pregnancy detects over half of all cases. This paper describes two women who were prenatally diagnosed to have a fetus with isolated posterolateral CDH. Both mothers underwent medical abortion. In the first case, a postmortem examination of the fetus revealed a large right-sided CDH, in which a part of the liver and small intestine were propagated into the chest. In the second one, a huge left-sided CDH was identified, in which the entire stomach, a large proportion of the small intestine and part of the left lobe of the liver were pushed up into the thoracic cavity. CDH is a diagnostically challenging birth defect with a marked variation in severity and corresponding survival. In utero-diagnosed cases usually represent more severe and prognostically unfavorable conditions than those that were postnatally diagnosed. Early prenatal detection of CDH may help improve the clinical outcome as the mother can be referred to a specialist center before the onset of labor for optimal perinatal management. A legal medical abortion should be the last option after all the others have been exhausted.

Management of right posterolateral congenital diaphragmatic hernia in a resource-limited country. case report and review

Congenital right diaphragmatic hernia is rare and often has a guarded prognosis when the liver is intrathoracic. The clinical case presented is that of a right posterolateral diaphragmatic hernia with the liver up, which highlights the importance of a chest X-ray in the face of neonatal respiratory distress and early management as soon as clinical stabilization is achieved.

Supraventricular Tachycardia in Infants With Congenital Diaphragmatic Hernia: Prevalence, Associations, and Outcomes.

To characterize the prevalence, associations, management, and outcomes of supraventricular tachycardia (SVT) in neonates with congenital diaphragmatic hernia (CDH). Retrospective chart and cardiology code review within a cohort of patients with CDH was used to define a subpopulation with atrial arrhythmia. SVT mechanisms were confirmed by electrocardiogram analysis. Cox proportional hazard regression identified risk factors for SVT and association with clinical outcomes. Medical Surgical ICU in a single, tertiary center, Boston Children's Hospital. Eligible patients included neonates presenting with classic Bochdalek posterolateral CDH between 2005 and 2017, excluding newborns with Morgagni hernia or late diagnoses of CDH (>28 d). None. SVT arose in 25 of 232 neonates with CDH, (11%); 14 of 25 infants (56%) had recurrent SVT; atrioventricular node-dependent tachycardia was the most frequent mechanism (32%). The majority (71%) of SVT episodes received intervention. Nine patients (36%) received preventative antiarrhythmic medications. SVT was associated with lower Apgar score at 1 min, structural heart disease, larger defect size, extracorporeal membrane oxygenation (ECMO) support, and prostaglandin therapy for ductal patency as well as hospital stay greater than or equal to 8 weeks and use of supplemental oxygen at discharge. SVT can occur in neonates with CDH and frequently requires treatment. Odds of occurrence are increased with greater CDH disease severity, ECMO, and prostaglandin use. In unadjusted logistic regression analysis, SVT was associated with adverse hospital outcomes, underscoring the importance of recognition and management in this vulnerable population.

Congenital Diaphragmatic Hernia Repair at the Bedside or Operating Theater.

For critically ill congenital diaphragmatic hernia (CDH) patients on high frequency oscillatory ventilation (HFOV), extracorporeal membrane oxygenation (ECMO), and/or inhaled nitric oxide (iNO), operative repair in the neonatal intensive care unit (NICU) has been proposed to avoid complications during transport to an operating room (OR). This study compared neonates with CDH who received herniorrhaphy in the NICU or OR, with a subgroup analysis considering only patients supported with ECMO. Patients admitted to the NICU in the first 2weeks of life at a free-standing children's hospital between July 2004 and September 2021 were examined. Patients were categorized according to location of CDH repair, and impact on operative complications and survival was compared. 185 patients were admitted to the NICU with posterolateral CDH and received operative repair. 48 cases were operated on at the bedside in the NICU and 137 in the OR. Patients repaired in the NICU had higher use of HFOV, pulmonary vasodilators, and ECMO (all P < .001). Children repaired in the NICU experienced significantly higher in-hospital death and overall mortality (P < .001). However, in multivariate analysis, repair location was not a significant predictor of survival to discharge in patients receiving ECMO. No significant difference in surgical site infection was detected for operative location (P = .773). Congenital diaphragmatic hernia repair in the NICU occurred more frequently among higher risk patients who experienced worse survival. The rate of surgical site infection appeared similar overall and across subgroups suggesting adequate sterility and technique for bedside procedures, when necessary, despite restricted access to advanced operative equipment.

Right posterolateral congenital diaphragmatic hernia without liver herniation: presentation of a case with successful treatment

La hernia diafragmática congénita es un defecto en la formación del diafragma con una alta mortalidad y morbilidad para el recién nacido. La ubicación en el lado derecho corresponde a solo el 10% de todos los casos y se asocia con una menor supervivencia, especialmente cuando hay una herniación hepática. No se conoce con precisión el porcentaje de casos de hernia diafragmática congénita derecha que no implican una herniación hepática. Aun no hay certeza del tiempo estimado para obtener el mayor beneficio de la corrección quirúrgica, en nuestro caso realizado con éxito a los 2 días de vida, logrando previamente una adecuada estabilidad hemodinámica y ventilatoria. El manejo perioperatorio en unidad de cuidado intensivo neonatal, es fundamental para reducir la morbimortalidad asociada. Se presenta un caso de hernia diafragmática congénita derecha tratado con éxito y se exponen los aspectos más relevantes del manejo médicoquirúrgico de esta patología. MÉD.UIS.2021;34(3): 71-7.

Patch repair of congenital diaphragmatic hernia is not at risk of poor outcomes

PurposeRecurrence of congenital diaphragmatic hernia (CDH) was retrospectively evaluated after correction with or without a patch in an institution where tension-free repair is advocated. MethodsDemographics and outcomes of patients with a postero-lateral CDH repaired (2000–2016) were analyzed (univariate tests and binary logistic regression adjusting for time since start of study, gender, defect side, liver herniation, patch, surgical approach, absence of postero-lateral rim and length of follow-up). ResultsOf 203 patients, 107 received a patch (P), and 96 were not patched (NP). Groups were not different for gestational age birthweight, gender, defect side and minimally invasive approach rate. Preoperative ECMO incidence (P:29.9% vs. NP:2.1%, p < 0.01), liver herniation (P:57.0% vs. NP:22.9%, p < 0.01) and absence of a postero-lateral rim (P:61.7% vs. NP:8.3%, p < 0.01) were higher in the P group. The mortality rate was 10.8% (P:15.0% vs. NP:6.2%, p = 0.07). Recurrence was not different (P:9.3% vs. NP:4.2%, p = 0.15). Multivariate analysis showed that recurrence was higher after thoracoscopy compared to open (OR = 12.2 [2.2–68], p < 0.01); neither the use of patch (OR = 2.3, [0.5–10.4], p = 0.28) nor any other factors were associated with recurrence. ConclusionIn this single centre series where tension-free repair was advocated, patch repair of CDH was not associated with higher recurrence, though access route was. Type of StudyCohort Study. Level of EvidenceLevel III.

Rare combination of left-sided congenital diaphragmatic hernia and omphalocele

We reported a rare case of left-sided posterolateral congenital diaphragmatic hernia (CDH) and omphalocele, which is not associated with chromosomal abnormalities or other syndromes. Omphalocele was detected antenatally (CDH was...

Thoracoscopic diagnosis and repair of central congenital diaphragmatic hernia in a neonate: A case report of a rare entity

Abstract Introduction Central congenital diaphragmatic hernia that involves a central tendon defect is a very rare form of diaphragmatic hernias. Experience in the thoracoscopic repair of congenital diaphragmatic hernia has expanded in recent years, but this expansion was mainly for the most common type; the posterolateral. We report a case of central congenital diaphragmatic hernia, which is supposed to be the case number seventeen of those reported in literature. And we report the first use of thoracoscopic repair in such rare entity. Case presentation We present a case report of a Bengali neonate who had a prenatal diagnosis of left congenital diaphragmatic hernia, which was supposed to be the usual posterolateral type. As we usually approach such cases by thoracoscopy, the patient had an intraoperative diagnosis of central congenital diaphragmatic hernia. The defect was amenable to a successful thoracoscopic repair. Conclusion The rare neonatal central congenital diaphragmatic hernia could present as a left sided herniation that clinically resembles the usual posterolateral congenital diaphragmatic hernia. Thoracoscopic approach offers a tremendous tool for diagnosis as well as the management of this kind of presentation of a central congenital diaphragmatic hernia.

Factors Affecting Short Term Outcome in Isolated Congenital Diaphragmatic Hernia: A Cohort Study from A Tertiary Surgical Center

Background: The mortality rate of Congenital Diaphragmatic Hernia (CDH) is still high in many centers. Most important factors influencing the outcome are pulmonary hypoplasia and associated anomalies. In this study, postnatal factors affecting short term outcome of patients with isolated CDH will be studied in an educational children hospital.Methods : This is a historical cohort study, on newborns with isolated posterolateral congenital diaphragmatic hernia from 2005 to 2015. Relative risks of variables for the short term outcome (Discharged alive or expired) were calculated. We also analyzed the highest PaO2, which was measured during the course of the management in the patients. Location of the liver and stomach were detected, too.Results: Fifty-three patients were studied, including 28(52.8%) male and 25(47.2%) female. Mean Gestational Age (GA) was 38 weeks (Range: 33-40) and mean body weight was 3001.22 (Range: 1300 – 4200) grams. Forty-two (79.2%) presented with left sided hernia, and 10(18.9%) had right sided defect. Thirty-two (60.4 %) patients discharged alive, and 21(39.6%) expired. Mortality rate for right sided hernias was 70% (7/10), and for left sided ones was 31% (13/42). Sex, weight, GA, stomach and liver herniation had no significant effect on survival. Side of involvement, time of presentation (Onset of symptoms), first measured PaO2, and first measured PaCO2, had significant influences on survival.Conclusion: According to our analysis, side of hernia, time of onset of symptoms, and initial PaO2 and PaCO2 are factors predicting survival in children with congenital diaphragmatic hernia. Although, we may predict the early outcome of the patients, more studies are needed in this regard.Chatt Maa Shi Hosp Med Coll J; Vol.15 (2); Jul 2016; Page 30-32

Predictors of prognosis in neonates with congenital diaphragmatic hernia: experience of 12 years

Introduction: Congenital diaphragmatic hernia (CDH) is a severe malformation, displaying relevant mortality and morbidity rates in newborns.Aim: To characterize clinically and demographically all neonatal cases of CDH admitted to a level III Neonatal Intensive Care Unit during a 12-year period and to evaluate the predictive value of baseline characteristics on mortality and morbidity at discharge.Methods: Maternal/infant clinical and electronic records were ret-rospectively reviewed. All neonates with posterolateral CDH admitted between January 2003 and December 2014 were included.Results: Fifty-three newborns were included. Overall mortality during hospitalization was 22/53 (41.5%). Clinical characteristics associated with mortality were the presence of intrathoracic liver (p = 0.005), intrathoracic stomach (p = 0.015), elevated arterial pCO2 or lower pH values at admission (respectively, p = 0.001 and p < 0.001), pre-ductal oxygen saturation < 85% at admission (p = 0.012) and surgical repair with prosthetic patch (p = 0.041). Morbidity at discharge was reported in 7 (22.6%) survivors. Stomach herniation and sepsis were associated with higher morbidity (respectively, p = 0.012 and p = 0.029). In a logistic regression, patch repair was the only variable with predictive value for death during hospitalization, with an odds ratio (OR) of 15 (95% CI 0.98-228.9), and intrathoracic stomach was a predictor of morbidity at discharge (OR = 15.7, 95% CI 1.4-174.2).Conclusion: Structural characteristics, namely defect size and presence of intrathoracic stomach, remain the primary determinants of neonatal prognosis in CDH. Although post-natal approaches have progressively proven their value in increasing survival and improving management of high-risk cases, future researches should continue focusing on the definition of foetal anatomical markers of severity and prenatal treatment of CDH.

Endoscopic Surveillance for Congenital Diaphragmatic Hernia: Unexpected Prevalence of Silent Esophagitis.

Introduction Gastroesophageal reflux disease (GERD) is a frequent comorbidity after congenital diaphragmatic hernia (CDH) repair. Our aim was to evaluate the clinical and endoscopic prevalence of esophagitis in a long-term follow-up of CDH patients. Materials and Methods Patients operated on for posterolateral CDH and undergoing general anesthesia for concomitant pathologies between January and October 2013 were included in the study. GERD was investigated both clinically (Manterola questionnaire) and endoscopically. The severity of esophagitis was evaluated according to the Hetzel-Dent classification and multiple biopsies were performed. The correlation between clinical score and severity of esophagitis was evaluated. Results Twelve patients were included in the study (mean age: 14.5 years; range, 9-18 years). Only three children (25%) had a pathological questionnaire. At endoscopy, three children (25%) were affected by grade 1 esophagitis, six (50%) by grade 2, two (17%) by grade 3, and one (8%) by grade 4. One of the children presented Barrett esophagus. A moderate negative correlation was found between clinical data and endoscopic findings (r: -0.54 and p: 0.067). Conclusion Even in the absence of symptoms, esophagitis revealed to have a high prevalence in CDH patients. A long-term clinical and instrumental follow-up is mandatory to early diagnose and treat GERD.

Giant congenital diaphragmatic hernia in an adult

Bochdalek hernia is the most common type of congenital diaphragmatic hernia. It appears frequently in infants but rarely in adults. We present the case of a 50-year-old female han patient with tremendous left-sided congenital posterolateral diaphragmatic hernia (Bochdalek hernia) who also has a pair of supernumerary breasts and pulmonary hypoplasia of the lower-left lobe. The patient had an experience of misdiagnosis and she was treated for bronchitis for one year until being admitted to our hospital. This case study emphasizes the rare presentation of Bochdalek hernia in adults and the necessity of high clinical attention to similar cases.

Pathogenesis of congenital diaphragmatic hernia: Additional clues regarding the involvement of the endothelin system

Congenital diaphragmatic hernia (CDH) has an incidence of around 1/3,000 births. The pathogenesis of this developmental anomaly remains largely unknown and the description of small chromosomal imbalances in cases of CDH is of major interest for the identification of candidate genes. We report on a tandem 4q31.23 triplication encompassing the EDNRA gene identified by array-CGH in a male presenting an isolated left postero-lateral CDH. This copy number variation was inherited from the asymptomatic father, carrier of a size-identical duplication. We demonstrate that EDNRA mRNA is over-expressed in the proband in blood tissue. Consistent with the expression of EDNRA in the developing diaphragm and the observation that the endothelin system is up-regulated in human and animal models of CDH, we conclude that the EDNRA triplication may be the cause of CDH in our patient.

Shifting From Laparotomy to Thoracoscopic Repair of Congenital Diaphragmatic Hernia in Neonates: Early Experience

Thoracoscopic repair of congenital diaphragmatic hernia (CDH) is now widely performed by pediatric surgeons. We compare our experience with this operation versus the results for a group of CDH patients treated by laparotomy at the same center over the same period. From January 2008 to December 2011, we reviewed the medical records of 50 neonates who underwent surgery for posterolateral CDH (34 by laparotomy, 16 by thoracoscopy). Two thoracoscopic operations (12.5 %) were converted to thoracotomy. Eight patients treated with extracorporeal membrane oxygenation (ECMO) underwent laparotomy. There were no significant differences in demographic characteristics between the two groups. Both nitric oxide (p = 0.13) and high-frequency oscillatory ventilation (p = 0.06) tended to be required more frequently in the laparotomy group than in the thoracoscopy group. A patch was applied to nine patients (five laparotomy, four thoracoscopy, p = 0.42). Mean operation time was longer for the thoracoscopy group, but not significantly so (p = 0.06). Times to feeding and lengths of hospital stays were similar, as were days of postoperative ventilator use and of total ventilator use. CDH recurred in two patients, both in the thoracoscopy group (p = 0.08), and intestinal obstruction occurred in five patients in the laparotomy group. Of eight patients who received ECMO treatment, only three survived. Except in patients receiving ECMO treatment, thoracoscopic repair is useful and feasible regardless of defect size or need for patching. Operation time is longer, but cosmetic appearance is better and intestinal obstruction uppers to be less frequent. However, intensive training is needed to prevent the recurrence.

Repair of congenital posterolateral diaphragmatic hernia under laparoscopy in 10 infants [J

Objective To summarize the clinical experience of repair of congenital posterolateral diaphragmatic hernia under lapaoroscopy. Methods Retrospectively analyzed the clinical data of 10 infants with congenital posterolateral diaphragmatic hernia treated under laparoscopy. And summarize the surgical experience. Results The repairs were successful it1 all infants. The operation time was 90-150 minutes; bleeding volume was 2-3 ml. 1 newborn postoperatively died of respiratory failure. Recurrence occurred in 1 newborn. The infants were fed 3-4 days after the surgery. A 3-6 months＇ follow-up showed no complication or recurrence. Conclusions Repair of congenital posterolateral diaphragmatic hernia under laparoscopy in infants is safe and feasible. Key words: Laparoscopy; Congenital posterolateral diaphragmatic hernia

Delayed Presentation Of Congenital Diaphragmatic Hernia With Splenic And Caecal Herniation

The vast majority of posterolateral congenital diaphragmatic hernia (CDH) diagnosed on prenatal ultrasound screening after 24 weeks of gestation present with respiratory distress at birth. It has been observed that late presenting CDH has better outcome and is not associated with pulmonary hypoplasia. We report a case of posterolateral CDH with herniated spleen presented first in a four year old male child with chest infection. This case report highlights the fact that posterolateral CDH which presents late is not associated with pulmonary hypoplasia, but may cause respiratory symptoms.

The Use of High-Frequency Oscillating Ventilation to Facilitate Stability During Neonatal Thoracoscopic Operations

Thoracoscopy has become a surgical option for the repair of esophageal atresia (EA) and congenital diaphragmatic hernia (CDH). Insufflation of carbon dioxide combined with one lung ventilation creates an anesthetic challenge to control acidosis and maintain oxygenation while allowing optimal operative exposure. We have overcome these issues by utilizing the high-frequency oscillating ventilator (HFOV) and report our early experience. A retrospective review from 2007 to 2010 on neonates who underwent thoracoscopic operation utilizing HFOV. Patient demographics and intraoperative course were reviewed. Seventeen neonates were identified, 12 with EA and 5 with posterolateral CDH. The median age at operation was 4 days (range 1-166 days), with a median weight of 2.9 ± 1.0 kg. Median gestational age was 38 ± 3 weeks. Before surgery, 6 patients (35%) were on conventional mechanical ventilation, and no patient was on high-frequency oscillating ventilation. Median American Society of Anesthesiologist's score was 3 (range 3-4). There were no intraoperative complications and median operative time was 208 ± 72 minutes. Review of the operative reports identified no significant difficulties with exposure of the operative field in all patients. Median ventilator manipulations needed were 3 per case. Mean intraoperative pulse oximetry was 97% ± 2%. Sixteen patients had intraoperative arterial blood gases; 1 had venous sampling. Patients with arterial blood gases had a mean pH of 7.36 (range 7.18-7.47), mean pCO2 was 41 mmHg (range 25-63 mmHg), and mean pO2 was 156 mm Hg (range 41-426 mmHg). Usage of HFOV allows for good intraoperative exposure with excellent oxygenation and elimination of carbon dioxide to prevent acidosis.

P30.18: Prenatal diagnosis of left posterolateral congenital diaphragmatic hernia with ileal loops—antepartum management and postpartum therapy

A 35 year old woman with a previous child with trisomy 21 was admitted at 22 weeks for an US scan at which the fetus appeared normal excepting for dextrocardia and dyshomogeneous left hemithorax. For the relatively high risk (5–15%) for trisomy 18 and 21 of a possible CDH we performed amniocentesis. The kariotype was normal, 46XY. The next scan at 28 weeks showed again dextrocardia, normal stomach, diaphragm with loosed hypoechoic contour on the left side, the left hemithorax with scattered small hypoechoic areas and apparent movements. This excluded the cystic adenomatoid malformation of the lung and confirms the CDH. A thorough scan was conducted in order to exclude the non-chromosomal syndromes associated with CDH (Fryns, Beckwith-Wiedemann, Pallister) and the result was normal. For left sided congenital diaphragmatic hernias the lung-to-head ratio has proved to be an indicator with good prognostic value. In our case LHR was 1.4, that means a high survival rate, and this value was maintained throughout the rest of the pregnancy. The prognosis is dependent on the degree of pulmonary hypoplasia and the subsequent pulmonary hypertension. At 40 weeks the patient delivered by caesarean section a 2900 g boy that was immediately gentle ventilated and transferred to a neonatal surgery unit where was performed at 48 hours after birth a surgical repair of the diaphragmatic defect by tissue approximation with excellent result. For left sided CDH survival rates are around 50–60% with good quality of life. Supporting information can be found in the online version of this abstract. Dextrocardia and ileal loops. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Thoracoscopic repair in congenital diaphragmatic hernia: patching is safe and reduces the recurrence rate

Purpose Congenital diaphragmatic hernia (CDH) has traditionally been repaired via a laparotomy. More and more reports on thoracoscopic repair are being published. The aim of this study was to evaluate our series of thoracoscopic CDH repair and compare this group to an open repair group treated during the same period in the same institute. Patients and Methods Between June 2006 and December 2008, 49 children with posterolateral CDH were admitted, of whom 23 (47%) were operated thoracoscopically and 23 (47%) using an open repair, depending on the discretion of the attending surgeon and the clinical condition of the patient. Three patients (6%) with CDH were not treated because of associated anomalies (twice Cornelia de Lange syndrome and once hypoplastic left heart syndrome). Six thoracoscopic operations (26%) were converted to open surgery. Nine defects (39%) were closed thoracoscopically without a patch. In 8 (35%) patients, a patch was used. We used a patch in 20 open procedures (87%). Results Three (33%) of the 9 thoracoscopic repairs without patch and 1 (12%) of the 8 with a patch developed a recurrence. All these recurrences were repaired thoracoscopically. The 3 recurrences from the thoracoscopic primary repair were repaired using a patch. In the open group, 3 patients (13%) developed a recurrence, of whom 2 were repaired thoracoscopically. Mean operative time was significantly longer in the thoracoscopic patch repair group (158 minutes), when compared to the open repair group (125 minutes). Conclusion As in open repair, it seems wise to use large patches liberally, not only to reconstruct the dome of the diaphragm but also to avoid undue tension on the repair and prevent recurrences. The thoracoscopic approach is also considered feasible in case of a recurrence from either a thoracoscopic or open repair.

Negative impacts of unreported COPD exacerbations on health-related quality of life at 1 year

Unreported chronic obstructive pulmonary disease (COPD) exacerbations are common, but their intermediate-to-long-term impacts on health-related quality of life (HRQoL) are unknown. The aim of the present study was to examine the impact of unreported exacerbations on HRQoL at 1 yr. A multicentric prospective cohort study in 491 COPD patients was conducted in China. HRQoL was measured using the St George's Respiratory Questionnaire (SGRQ). Other measurements included sociodemographic, clinical, psychosocial and treatment profiles. Patients were monitored monthly for 12 months to document exacerbations (at least one symptom worsening for > or =48 h). Patients were categorised into six groups: no exacerbation, one unreported exacerbation only, more than one unreported exacerbation only, one reported exacerbation only, more than one reported exacerbation only, and both unreported and reported exacerbations. Generalised estimating equations were used to estimate the adjusted associations between exacerbations and HRQoL change. A total of 466 unreported and 410 reported exacerbations were recorded. Compared with patients with no exacerbations, the change in SGRQ total score was similar amongst patients with one unreported exacerbation (adjusted mean change 1.22 points (95% CI -4.05-6.48)), but significantly worse among patients with more than one unreported exacerbation (4.61 (95% CI 0.09-9.13)). Development and evaluation of self-management programmes emphasising early recognition of exacerbations and consequent action appear to be warranted.