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.

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

This article has no abstract

PURPOSE:

To evaluate the most used approach to treat traumatic diaphragmatic ruptures, and in which one the requirement to assess the second cavity is more frequent.

METHODS:

Systematic review, observational studies. Outcomes: moment of approach, most commonly via addressed and the requirement to open the other cavity. Bases searched: Lilacs, Pubmed, Embase, Clinicaltrials.gov and Web of Science. Statistical analysis: StatsDirect 3.0.121 software.

RESULTS:

Sixty eight studies (2023 participants) were included. Approach in acute phase was performed four times more than in chronic phase. Approach: abdominal 65% (IC 95% 63-67%), thoracic 23% (IC 95% 21-24%), abdominal in the acute phase 75% (IC 95% 71-78%), and chronic 24% (IC 95% 19-29%), thoracic in the acute phase 12% (IC 95% 10-14%) and chronic 69% (IC 95% 63-74%). Thorax opening in the abdominal approach: 10% (95% CI 8-14%). Abdomen opening in the thoracic approach: 15% (95% CI 7-24%).

CONCLUSIONS:

The most common approach was the abdominal. The approach in the acute phase was more common. In the acute phase the abdominal approach is more frequent than the thoracic approach. In the chronic phase the thoracic approach is more frequent than the abdominal one. The requirement to open the second cavity was similar in both approaches.

BACKGROUND:

ADH is a rare and potentially fatal complication following LT. In this study, a systematic review was completed to identify risk factors which may contribute to ADH.

METHODS:

Transplant databases at three LT programs were reviewed. Four pediatric and zero adult cases were identified. Next, a systematic review was completed. Fourteen studies describing 41 patients with ADH were identified. Patient demographics, transplant characteristics, and features of ADH diagnosis were examined.

RESULTS:

The majority (90.2%) of ADH were in children. In pediatric LT, 95.1% received a segmental allograft. ADH occurred in the right P diaphragm 92.7% of the time, and 87.8% were repaired primarily. Patient demographics, post-transplant complications, and immunosuppression regimens were broad and failed to predict ADH. Most patients presented with either respiratory or gastrointestinal symptoms. There were two pediatric deaths related to undiagnosed ADH. The combined worldwide incidence of ADH in pediatric LT is 1.5% (34/2319 patients).

CONCLUSION:

ADH is a rare complication post-LT that primarily occurs in pediatric recipients. When diagnosed early, ADH can be repaired primarily with good outcomes.

Diaphragmatic hernia may be asymptomatic and may have a delayed clinical manifestation. We describe a 78-year-old man who developed colonic obstruction as a complication to a diaphragmatic hernia. Unlike most diaphragmatic hernias, this case was not associated with a trauma. Normally, the diagnosis is made clinically by means of thoracic X-ray, computer tomography or magnetic resonance imagining of the abdomen. In this patient, barium enema showed a tumor-like stenosis of the left colonic flexure, but intraoperative findings demonstrated a defect in the left diaphragm with herniation of the left colonic flexure and the greater omentum.

At 32 weeks of gestation, delivery of a female fetus was induced because of severe malformations seen on ultrasonogram: congenital diaphragmatic hernia and cerebellar hypoplasia. The diagnosis of Coffin-Siris syndrome was based on the physical examination: coarse face with low-set ears, low nuchal hairline, scalp hypotrichosis and hypoplasia of the nails of fingers and toes with absence of the right fifth fingernail. Autopsy confirmed the prenatally diagnosed major associated abnormalities: hypoplasia of the cerebellum and congenital diaphragmatic hernia. Various clinical entities are included in the differential diagnosis.

Congenital diaphragmatic hernia (CDH) is a severe congenital malformation, related to a developmental defect of the diaphragm. The incidence of CDH is approximated at 1 in 3,000 live births. Although advances in surgery and neonatal intensive care have improved the prognosis, mortality remains high, around 30-50% related to severe lung hypoplasia and persistent pulmonary hypertension. Prenatal evaluation with observed/expected Lung over Head Ratio (o/e LHR), liver position and total lung volume measured by magnetic resonance, have been shown to correlate with neonatal mortality . However, the preponderant factor of persistent pulmonary hypertension remains difficult to predict prenatally. In patients with isolated diaphragmatic hernia (without associated malformations or karyotype abnormalities), prognosis is evaluated indirectly on pulmonary development from pulmonary volume measurements. Apart from the most caricatural cases with extremely good or very pejorative values, for a large proportion of fetuses with diaphragmatic dome hernia the prognosis remains uncertain.

The aim of the proposal is to investigate whether the analysis of the proteom of the amniotic fluid of the fetuses with CDH could give information of a prognostic character. The objective of the study is to identify, from the proteomic profile of the amniotic fluid of mothers whose fetus has CDH, prognostic markers candidates for death at 2 months of the infant. The first step is to carry out an exploratory and non-interventional study on a small sample (n = 10) of the target population. This is a preliminary step before considering, if the results are encouraging, a large-scale study from a biological collection to determine candidate proteins (new biomarkers) which relative expression levels could be used as surrogate marker of pulmonary hypoplasia.