The mortality rate in cases of antenatally diagnosed congenital diaphragmatic hernia (CDH) remains high: 80% to 100% mortality was reported in early series and 73% in a more recent series. In addition, the early reports described 100% mortality for cases diagnosed before 24 weeks gestational age. To determine the mortality rate in patients at their institution (a specialized tertiary-care hospital), the authors reviewed 11 cases of CDH diagnosed from 1987 to 1990, for all of which antenatal ultrasonography (US) had been performed at the hospital. Overall, 7 of the 11 patients survived. In seven of the cases the condition had been diagnosed antenatally. Of these patients, four survived, including two of the three in whom the diagnosis had been made before 21 weeks gestational age. All four false-negative cases involved a right-side hernia, and in three of these the prenatal US examinations had been performed before 16 weeks gestational age. Three of these four patients survived. This study suggests that the prognosis in antenatally diagnosed CDH is improving and confirms that this condition is not uniformly fatal in cases diagnosed before 24 weeks gestational age.

From 1969 to 1975, 33 cases of congenital diaphragmatic hernia (CDH) were treated at the National Hospital of Norway with a "visible" or operative mortality of 30%. At least 37 additional infants with CDH who died soon after birth and did not come to the attention of a major referral center were identified retrospectively from a comprehensive survey of neonatal deaths. CDH occurred at least once in every 5455 live births and the "true" mortality was 66%. More than half of the infants born with CDH during this 6-yr period died before they could be treated, contributing to a substantial "hidden" mortality.

This article has no abstract

Critically ill infants with congenital diaphragmatic hernia were treated by either early surgery or delayed surgery after preoperative stabilisation. The preoperative stabilisation was aimed at correcting acidosis and hypoxia, thereby reducing the severity of persistent fetal circulation. Survival improved from 12.5% after early surgery to 52.9% after delayed surgery.

BACKGROUND:

Congenital diaphragmatic hernia (CDH) is a rare and life-threatening anomaly that needs surgical therapy after clinical stabilization of the neonate. Given an existing volume-outcome relationship for other high-risk, low volume procedures, we aimed at examining the relationship between hospital or surgeon volume and outcomes for surgery on CDH.

METHODS:

We conducted a systematic search in multiple databases in September 2019 and searched for additional literature. We assessed risk of bias of included studies using ROBINS-I and synthesized results in a structured narrative way using GRADE.

RESULTS:

We included 5 cohort studies on hospital volume. Results for in-hospital mortality, one-year mortality and length of stay are inconclusive. The certainty of the evidence was very low for all outcomes, due to risk of bias, inconsistency and imprecision. We did not identify any study on surgeon volume.

CONCLUSION:

Due to the very low certainty of the evidence it is uncertain whether higher hospital volume is associated with favorable outcomes for neonates undergoing surgery for CDH. There is no evidence on the relationship between surgeon volume and outcomes. Future studies should use more rigorous methodology and analyze additional outcomes to allow for more meaningful inferences.

LEVEL OF EVIDENCE:

III SYSTEMATIC REVIEW REGISTRATION:

PROSPERO (CRD42018090231).

The purpose of this study is to demonstrate the ability to prenatally deflate and to evaluate the safety of the Smart-TO device for fetoscopic endoluminal tracheal occlusion (FETO) in fetuses with congenital diaphragmatic hernia and moderate to severe pulmonary hypoplasia.

The study has been designed to investigate the effect of 12 weeks of using virtual reality based exercises on pulmonary functions, exercise capacity, functional performance, and quality of life in children with surgically-repaired congenital diaphragmatic hernia.

The purpose of this study is to test if sildenafil is effective in the treatment of infants with severe congenital diaphragmatic hernia (determined by the presence of prolonged pulmonary hypertension or prolonged oxygen supplementation on mechanical ventilation), as measured by the estimated pulmonary artery systolic pressure following treatment.

Congenital diaphragmatic hernia (CDH) is a severe neonatal anomaly. The aim of this study was to evaluate the frequency and types of malformations associated with CDH. The outcome was compared with that in newborns with CDH alone. The study included 362 fetuses and newborns at a single national center for CDH. Associated malformations and chromosomal aberrations were noted prenatally and postnatally. The neonatal outcome was assessed relative to the use of extracorporeal membrane oxygenation (ECMO) and the mortality rate. At least one associated malformation was diagnosed in 143 cases (39.5%). Altogether, 272 associated malformations were found. Only 50 (18.4%) anomalies were diagnosed antenatally. In 62 (17.1%) cases, 102 major malformations were found along with CDH, with a prenatal detection rate of 35.3%. The associated malformations were very heterogeneous, but cardiovascular malformations were the most common. Newborns with major anomalies, chromosomal aberrations, or syndromes additional to CDH had a significantly lower survival rate than newborns with an isolated CDH. Associated malformations did not affect the rate of ECMO treatment. Associated malformations in CDH are frequent and heterogeneous, and diligent and experienced antenatal and postnatal care is important.

The incidence of congenital diaphragmatic hernia (CDH) is about 4.8/10,000 live births. Its typical clinical presentation is respiratory distress occurring immediately after birth or in the first few hours or days of a child's life. It is characterized by a high mortality rate. Exceptionally, CDH can occur at an older age, its symptoms then frequently reflecting gastrointestinal obstruction or mild respiratory symptoms. In such cases CDH presents a far more complex diagnostic problem. The paper presents the cases of two girls without typical symptomatology, aged 5.5 and 10 years, in whom CDH was detected incidentally upon thorough physical examination and chest x-rays. Further radiographic evaluation, which included barium contrast study and spiral computed tomography, confirmed the suspicion of a left-sided posterolateral diaphragmatic hernia with associated intestinal malrotation. Surgical intervention conclusively confirmed a diaphragmatic defect at the site of Bochdalek's foramen in both cases. The vital capacity of the older girl, which was low before the surgery (VC 1.66 L; 69% of predicted), was significantly increased a month after the surgical treatment (VC 2.25 L; 92% of predicted). The generally expressed view that the clinical onset of CDH is rare after the neonatal period seems to be erroneous. Some papers report on the clinical presentation of CDH after the neonatal period in as many as 13%-14% of infants and young children suffering from CDH. Infants and young children with a delayed clinical occurrence of CDH can present with respiratory or gastrointestinal symptomatology. Children presenting with gastrointestinal symptoms have been shown to be significantly older than those presenting with respiratory symptoms. In older children and adolescents, the symptoms and signs of CDH, which include acute hernial incarceration, nausea, recurrent vomiting, diarrhea, obstipation, acute gastric dilatation, subcostal pain, failure to thrive and recurrent chest infections, habitually present a significant diagnostic problem. Diagnostic errors are mainly due to the fact that the possibility of CDH in that age is totally neglected. The most recurrent diagnostic misinterpretations in such cases are pneumonia or massive pleuropneumonia, empyema, pneumothorax, lung cysts and bullae, and gastric volvulus. Thus, whenever a child presents with uncommon respiratory or gastrointestinal symptoms and an anomalous chest x-ray, a differential diagnosis of CDH should be considered. Otherwise, an accurate diagnosis in both young and older children will most probably be only reached at autopsy. In conclusion, the presented cases corroborate the finding that CDH in older children may present with scarce symptoms, mostly gastrointestinal, or may be altogether asymptomatic and unrecognized until as late as adolescence. However, when a diagnosis of CDH has been established, albeit asymptomatic, it must be promptly treated surgically in order to prevent complications, such as strangulation or bowel perforation, and thus avert a potentially fatal outcome. The size itself of the herniac foramen is unlikely to be a determining factor at the time of clinical presentation of CDH. Surgical occlusion of CDH may in older children result in an improved vital capacity, as such cases are rarely associated with major pulmonary hypoplasia. Complications resulting from surgical treatment of CDH in older children are more likely to occur in the gastrointestinal system, as a consequence of the associated bowel malrotation and inadequate bowel fixation. Finally, these two cases corroborate the diagnostic value of accurate history taking and thorough physical examination.