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Diagram of thorax, lungs, and mediastinum in left- sided congenital diaphragmatic hernia before and after repair. Arrows represent possible changes in mechanical forces in thorax and abdomen produced by repair. Reprinted with permission from Saki et al. J Pediatr . 1987;111:432– 438.
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OBJECTIVES After completing this article, readers should be able to: 1. Delineate the studies used for prenatal diagnosis of congenital diaphragmatic hernia (CDH). 2. Describe prenatal therapies currently being investigated for the treatment of CDH. 3. Delineate the options available for ventilation of the neonate who has CDH. 4. Describe the poten...
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Aim of the Study Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation inde...
Despite advances in therapeutic modalities, congenital diaphragmatic hernia (CDH) still accounts for significant neonatal mortality. This study aimed to describe the demographic features, clinical experiences of postnatal care, and differences between non-survivors and survivors with CDH.
We retrospectively reviewed medical records of neonates with...
Many different prosthetic materials have been used for repair of large posterolateral congenital diaphragmatic hernias, which cannot be primarily repaired. Almost 50% of patch repaired diaphragmatic hernias will recur. The ideal prosthetic material for congenital diaphragmatic hernia repair has yet to be established. We report on two cases with unu...
To review outcomes of patients with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) at a level IIIC neonatal intensive care unit and to determine if pre-ECMO respiratory status can help predict mortality.
A single-center retrospective chart review was conducted on all infants with CDH treated with ECMO in...
The primary aim of this study was to determine the predictive value of prenatal ultrasound and echocardiography for prognosis in congenital heart disease (CHD) with coexisting diaphragmatic hernia (DH) in a tertiary care center. Eleven records from the database of the Department for Diagnoses and Prevention of Congenital Malformations, Polish Mothe...
Citations
... Several studies support delayed surgery after stabilization with high frequency oscillatory ventilation, surfactant replacement and EHCMO there by improves oxygenation, lung expansion and reduces risk of pulmonary hypertension. Inhalation of nitric oxide in clinical trials of infants with hypoxic respiratory failure has shown to improve oxygenation significantly and decreases the need for ECMO [10]. Cartlidge et al., in his 17 patients observed decreased mortality from 88% to 47% by allowing preoperative stabilization [11]. ...
... Ventilation with small tidal volumes and low inflating pressures are advocated to avoid contralateral pneumothorax [2,[12][13][14]. The post-surgical long term complications reported are of low pulmonary perfusion on the herniated side on V/P scan, gastroesophageal reflux, failure to thrive, herniation, chest asymmetry and significant scoliosis [10]. Our patient was also stabilized for 48 hours and we used combined technique of GA with CEA and found stable hemodynamic speri operatively. ...
... Pada hernia diafragmatika terdapat paru yang hipoplastik, tidak atelektasis vaskularisasi arteriolar yang abnormal dan hipertensi pulmonal sehingga dipertimbangkan pembedahan ditunda atau dipersiapkan dahulu. 11 Umur rata-rata untuk melakukan pembedahan adalah sekitar 72 jam. 7 ...
... Ventilasi dengan inspirasi bertekanan rendah dipilih karena menurunkan kemungkinan terjadinya pneumothorax kontralateral yang dapat meningkatkan ketidakstabilan sistem kardiorespirasi dan dekompensasi. 11 Jika dengan ventilasi mekanik konvensional ini gagal maka dipakai strategi ventilasi yang lain yaitu high-frequency oscillatory ventilation (HFOV), gentle ventilation dan intratracheal pulmonary ventilation (ITPV). Selain strategi ventilasi juga dibutuhkan terapi pendukung untuk menunjang keberhasilan pembedahan dan memperbaiki prognosis. ...
Hernia diafragmatika adalah masuknya organ-organ abdomen melalui defek (lubang) pada
diafragma ke dalam rongga toraks. Secara umum terdapat tiga tipe dasar hernia
diafragmatika yaitu hernia Bochdalek (melalui defek posterolateral), hernia Morgagni
(melalui defek anterio retrosternal) dan hiatus hernia. Diagnosis ditegakkan berdasarkan
anamnesis, gejala klinik, pemeriksaan radiologik, dan laboratorium. Insiden pasti hernia
diafragma sulit diperkirakan karena separuhnya meninggal dalam kandungan atau
meninggal saat neonatus belum dibawa ke pusat rujukan atau sebelum diagnosis ditegakkan.
Insiden hernia Bochdalek dilaporkan 1 : 2000-4000 kelahiran hidup dengan perbandingan
jenis kelamin laki-laki : perempuan adalah 1,5 : 1. Hernia Bochdalek memberikan gejala
kardiopulmonal yang berat, seperti sesak nafas segera setelah lahir dengan mortalitas yang
tinggi, 40-50% sebelum pemakaian dan 30 %, setelah pemakaian Extracorporeal Membrane
Oxygenation (ECMO). Pembedahan dilaksanakan setelah kondisi bayi stabil.
... En la década de los noventa comenzaron a aplicarse nuevas estrategias ventilatorias (como la ventilación mecánica sincronizada, la ventilación de alta frecuencia oscilatoria -VAFO-, etc.) y nuevos tratamientos para la HPPN, como la administración de óxido nítrico inhalado (ONi) y la asistencia respiratoria extracorpórea (ECMO), sin lograrse tampoco una reducción significativa de la mortalidad. En la actualidad, con la llegada del nuevo siglo, además de darles la importancia que se merecen a las medidas iniciales de estabilización y reanimación del RN tras el nacimiento, se postula como cambio fundamental la ventilación «suave» y la hipercapnia permisiva, intentando evitar el daño al pulmón hipoplásico y la posibilidad de aire ectópico (neumotórax y/o enfisema intersticial) al pulmón contralateral (1,(3)(4)(5) . ...
... La variabilidad clínica observada en los últimos años en el manejo de la HDC y la existencia de aspectos claves de la misma, que aun hoy en día siguen siendo controvertidos, hacen necesaria una revisión actualizada de la evidencia científica disponible para intentar aportar luz a los puntos que más controversia han suscitado en el manejo terapéutico de los RN afectos de esta malformación congénita. Algunos aspectos polémicos en el manejo de la HDC son los siguientes (1,4,5) : • ¿Cirugía precoz o diferida? ...
... • En Índice Médico Español, utilizando el término «hernia diafragmática congénita» nos devuelve un total de 40 artículos, siendo la mayoría de los trabajos publicados en revistas pediátricas españolas con el diseño de casos clínicos o casuística, si bien algunos de ellos, publicados hace más de 5 años, indagaban sobres las controversias del manejo terapéutico (52)(53)(54) . También cabe destacar algún artículo publicado en literatura «gris», como la actualización de Bancalari en un Curso sobre Cardiología Infantil en el año 2003 (5) , y algunas revisiones del tema de relativa actualización (3,4,55) . ...
Congenital diaphragmatic hernia (CDH) is one of the high-risk diseases in neonatal surgery. The aim of this article is to make an update of the controversies about the therapeutic management (time of surgery and modalities of medical stabilization) of CDH, by means of a systematic and critical review of the best scientific evidence in bibliography.
Systematic and structured review of the articles about therapeutic management of CDH (surgery, mechanical ventilation, inhaled nitric oxide, extracorporeal membrane oxygenation, surfactant, etc) published in secondary (TRIPdatabase, systematic review in Cochrane Collaboration, clinical practice guidelines, health technology assessment database, etc) and primary (bibliographic databases, biomedical journals, books, etc) publications and critical appraisal by means of methodology of the Evidence-Based Medicine Working Group. We selected the publications with the main scientific evidence in therapeutical articles (clinical trial, systematic review, meta-analysis and clinical practice guideline).
The main secondary information is found in The Cochrane Library: 3 systematic review in the Neonatal Group (one specific about the time of surgery, and two related to the use of nitric oxide and extracorporeal membrane oxygenation in neonatal severe respiratory failure). But we found the main relevant articles in Pubmed database, mainly published in Journal Pediatric Surgery and with some clusters of investigation (Congenital Diaphragmatic Hernia Study Group in Texas University and Buffalo Institute of Fetal Therapy in New York University).
From the evidence-based analysis, the results of CDH management between immediate versus delayed surgery were unclear, but delayed surgical (with pre-operative stabilization) has become preferred approach in many centers, and foetal surgery is not better than neonatal one. Opinion regarding the time of surgery has gradually shifted from early repair to a policy of stabilization and delayed repair. Because of associated persistent pulmonary hypertension and/or pulmonary hypoplasia in CDH, medical therapy is focused toward optimizing oxygenation while avoiding barotrauma, using gentle ventilation and permissive hypercarbia. High frequency oscillatory ventilation, inhaled nitric oxide and extracorporeal membrane oxygenation are used in severe cases, but these treatments do not clearly improve the outcome in neonates with CDH. The usefulness of surfactant and partial liquid ventilation are based in animal model experimentation, because the clinical trials in newborns are little and non-conclusive. Challenges for the future in this thematic area include the need for bigger and better trials of therapy in this field, with long-term outcomes among surviving children.
... CDH occurs in about 1 out of 3,000 live births 1 and shows no gender differences. 2 The most common form of CDH is the Bochdalek type. This type of hernia occurs in the posterolateral segment of the diaphragm, more often on the left than on the right side. ...
Fung Hin Tat, FRCSEd, FHKCEM, FHKAM(Emergency Medicine) Kam Chak Wah, MRCP(UK), FRCSEd, FHKAM(Emergency Medicine) Case A four-year-old Pakistan boy presented to the Accident and Emergency Department because of cough and shortness of breath for two days. He had past history of microcephaly and developmental delay, with regular follow-up in the Department of Paediatrics. No history of injury or recent travel was reported. He was afebrile with SpO 2 of 95% on oxygen supplement (by nasal cannula at 2 litres per minute). His pulse rate was 130 beats per minute. Examination of his chest revealed equal air entry on both sides with mild crepitations. Chest radiography was performed (Figure 1). Questions 1. What is the X-ray diagnosis? 2. What physical findings would suppor t the diagnosis? 3. If the patient develops increased respiratory distress, what to do and what not to do? (Answers on the next page) Figure 1.
The revised and updated second edition covers practical approaches to caring for healthy and high-risk infants.
https://shop.aap.org/neonatalogy-for-primary-care-2nd-edition-paperback/
Congenital diaphragmatic hernia (CDH) is an emergency case in neonates. The prevalence of CDH is 1 in 2500 births and occurs 4–8 times more commonly on left side than right side. CDH is usually worsened by pulmonary hypoplasia and pulmonary hypertension. It is characterized by respiratory failure and bowel sound in the chest area and mostly requires surgical procedure. This case report discusses a neonate with congenital left diaphragmatic hernia who underwent surgical procedure to close the defect. Patients present with cyanosis and shortness of breath, are early intubated, and are mechanically ventilated in neonatal intensive care unit (NICU). In the present case, the surgical procedure lasted approximately 2 h with general anesthesia, with hemodynamic stability during the surgery. The patient was thereafter intubated and treated at the NICU for close monitoring.
Congenital lung abnormalities are common in pediatric patients. While these lung malformations may be incidental findings in some children, they can also result in various symptoms and imaging findings depending on their size, location, as well as connection to and mass effect upon the adjacent thoracic structures. Imaging plays an important role for early and correct diagnosis, which in turn can improve pediatric patient care by guiding the appropriate next step in management. In this chapter, we discuss clinical presentation and imaging findings of common congenital lung abnormalities in the pediatric population. In addition, rare but important other congenital malformations such as horseshoe lung, hepatopulmonary fusion, bronchobiliary fistula (BBF), and cast bronchitis are reviewed. Familiarity with the characteristic clinical and imaging findings of congenital lung abnormalities can avoid delay in diagnosis and optimize pediatric patient care.
A Hérnia Diafragmática Congênita (HDC) é resultante de um defeito na formação do músculo diafragma com pequena incidência, mas alto índice de mortalidade. Objetivo: Descrever as principais complicações, o prognóstico, o tratamento e os tipos de ventilação mecânica utilizadas nas crianças com Hérnia Diafragmática Congênita (HDC), a fim de direcionar a conduta da fisioterapia respiratória. Metodologia: Revisão crítica da literatura sobre HDC entre os anos de 1999 e 2008. Conclusão: Diversos estudos foram realizados no intuito de encontrar os procedimentos ideais para crianças com HDC, melhorar o seu prognóstico e a taxa de sobrevivência, mas ainda não foi possível definir qual(is) o(s) melhor(es) tratamento(s) e o momento correto para ser utilizado nestas crianças.Palavras-chaves: hérnia diafragmática congênita, ventilação mecânica, recém-nascido.
The preterm infant, defined as birth before 37 weeks gestation, provides unique medical and surgical challenges to health care providers due to a myriad of anatomical underdevelopments and physiologic derangements. The physiology of prematurity as it relates to anesthesia is of particular importance when preparing for surgery. Anatomic differences of the premature airway and altered respiratory mechanics, such as a smaller airway diameter, increased oxygen consumption, bronchopulmonary dysplasia and apnea place these infants at risk for rapid and profound desaturation and hypoventilation during anesthesia. The immature heart has not had time to develop sufficient muscle fibers for optimal contractility. In addition, persistent pulmonary hypertension of the newborn may develop when right-to-left shunting of blood occurs through a patent ductus arteriosus and/or a patent foramen ovale due to failure of the pulmonary vascular resistance to drop at birth. These factors may combine and lead to significant cardiovascular collapse during surgery. Premature infants are susceptible to metabolic derangements such as hypoglycemia and hypocalcemia due to improper storage and loss of maternal-fetal transfer during gestation. The liver and kidneys are underdeveloped, leading to altered drug metabolism, thus, anesthetic drugs must be tailored accordingly. Neonatal surgical emergencies, such as congenital diaphragmatic hernia, hypertrophic pyloric stenosis, necrotizing enterocolitis and gastroschisis, can present at any time and be life-threatening. Immediate surgical intervention is not always necessary and there is often time for medical optimization. Advances in neonatal care have improved the morbidity and mortality of critically ill newborns.
Background:
Congenital diaphragmatic hernia (CDH) has a high mortality rate, representing a therapeutic challenge. Prenatal diagnosis (PND) is essential in defining optimal perinatal strategy, particularly delivery planning. Hospital Pediátrico de Coimbra is the referral centre for all neonatal surgery, particularly CDH, for the central region of Portugal. The aim was to evaluate clinical management and outcome of newborns with CDH.
Methods:
An exploratory retrospective study made up of newborns admitted to PICU with CDH was undertaken between January 1995 and December 2014. Two groups were formed based on their year of admission: group A (1995- 2004) and group B (2005- 2014) and were compared.
Results:
The mean birth weight of the 69 newborns admitted was 2.762 ± 696 g; the median of the gestational age was 38 weeks. Associated malformations were observed in 28 (40.5%) and 15 (21.7%) had a right-sided diaphragm defect. The global mortality was 13.0%; in group A was significantly higher than in group B (22.2 vs 3.0%; p = 0.029). A reduction in mortality throughout the years was confirmed after adjusting for POS score (OR = 0.77; 95% CI: 0.62- 0.96, p = 0.021). PND was made in 30.6% of cases in the group A and 66.7% in the group B (p = 0.03). Tertiary perinatal hospital birth was achieved in 60% of newborns in the group A versus 84.8% in group B (p = 0.022). Maximum fraction of inspired oxygen showed a statistically significant difference between the two study groups (60% vs 40%; p = 0.009).
Conclusions:
A significant decrease in mortality was observed throughout the study. The authors highlight the increase in prenatal diagnosis and an improvement in perinatal care with planning delivery as important contributors to these results.
