Iatrogenic anterior diaphragmatic hernia in childhood

Congenital anterior diaphragmatic hernias (ADH) account for 2 - 6 % of diaphragmatic defects, whereas acquired ADH are rare. These hernias are most often the result of blunt or penetrating trauma. This is the first report of iatrogenic ADH in childhood. Two children with asymptomatic, iatrogenic ADH were diagnosed at 6 and 12 months of age, respectively. Both had undergone previous cardiac surgery requiring pericardial drainage. The ADH was seen on an incidental chest X-ray in one patient, and during a laparoscopic-assisted gastrostomy in the other. Both were successfully repaired laparoscopically. Subxiphoid pericardial drains may result in iatrogenic ADH. A high index of suspicion after cardiac surgery is necessary to avoid missing this potentially serious complication. Laparoscopy is a useful tool in both the diagnosis and repair of this condition.     

Bilateral diaphragmatic hernia

Bilateral congenital diaphragmatic hernias are extremely rare; only 12 cases have been reported in the literature. We report six further cases of this rare malformation. The condition is invariably fatal. Only 3 of the 18 cases have survived and these 3 had hernial sacs. Four of our six cases had major associated malformations. Offprint requests to: F. Rivilla     

Intrapericardial diaphragmatic hernia

Report of a case with congenital right sided intrapericardial diaphragmatic hernia. This type of a congenital diaphragmatic hernia occurs only rarely. Until 1984 30 cases are recorded in the available literature.     

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia occurs in approximately 1 in every 2500 live births and is associated with a reported mortality of almost 35% in live-born patients and a higher mortality when in utero deaths are counted. Ventilator-induced lung injury, pulmonary hypoplasia, and other associated anomalies account for the high death rate. Numerous adjunctive measures have been used to treat these patients. Inhaled vasodilators (nitric oxide), intravenous vasodilators, and fetal therapy have no proven benefit. While animal models of congenital diaphragmatic hernia are surfactant deficient, controversy remains over the use of surfactant in infants. There has been no clinical trial showing any clear benefit with the use of exogenous surfactant in these patients. Similarly, prenatal corticosteroids show some improvements in animal models, but again, there is a complete absence of supportive data to show benefit in humans. Mechanical ventilator strategies that limit ventilator-induced lung injury by avoiding hyperventilation and lung over inflation are the strategies currently in use that have been associated with improved survival. Long-term follow-up of these patients is quite important since gastroesophageal reflux, developmental delay, chronic lung disease, and chest wall deformity are all seen with increased frequency in these children.     

Congenital diaphragmatic hernia

The incidence of congenital diaphragmatic hernia (CDH) may be as high as 1 in 2000. Over the past two decades, antenatal diagnosis rates have increased, the pathophysiology of CDH has become better understood, and advances in clinical care, including foetal surgery, have occurred. However, there remains a paucity of randomised controlled trials to provide evidence-based management guidelines. Reports of improved survival rates appear to be confined to a select subset of CDH infants, surviving to surgical repair, while the overall mortality, at over 60%, appears to be unchanged, largely due to the often forgotten 'hidden mortality' of CDH. The significant long-term morbidity in surviving infants has become apparent, and the need for long-term multidisciplinary follow up established. A total of 10% of cases may present later in life, and misdiagnosis on initial chest X-ray may lead to significant morbidity.     

Congenital diaphragmatic hernia

Posterolateral congenital diaphragmatic hernia (Bochdalek's hernia, CDH) that presents in the neonatal period is often manifest as life-threatening respiratory distress due to mechanical compression of the cardiopulmonary system by herniated viscera. Most patients can be supported through surgical reduction and repair with standard neonatal techniques. Postoperatively, many develop severe, refractory persistent pulmonary hypertension of the newborn (PPHN) after a variable period of adequate oxygenation. Despite aggressive ventilatory and pharmacologic maneuvers, a significant number of these infants develop fatal hypoxia and acidosis. At our institution, extracorporeal membrane oxygenation (ECMO) has been used as a therapy of last resort in 12 moribund infants with PPHN and respiratory failure after repair of CDH. There were 7 survivors. All long-term survivors [6] were totally weaned from ventilatory support; all met the criteria for nonsurvival as defined by Bohn et al. [2]. Eight patients (4 survivors) had no PaO₂ greater than 100 torr postoperatively. ECMO can relieve the hypoxic and metabolic stimuli that aggravate pulmonary hypertension and provide cardiopulmonary support. It is the treatment of choice for infants who are deteriorating in the face of maximum conventional therapy after surgical correction. Lesions that were formerly denoted nonsurvivable can be effectively treated. It is urged that this therapy be used whenever necessary to sustain the life of an infant who has survived operation, regardless of whether the patient has demonstrated a honeymoon period, PaO₂ greater than 100 torr, or any other factor conventionally thought to delineate survivable from nonsurvivable lesions. Such practices may exclude patients with survivable lesions from life-saving therapy.     

Recurrent diaphragmatic hernia

Recurrent congenital diaphragmatic hernia (CDH) is a well-known complication, but one that has not been examined systematically. Review of the literature shows a varied incidence as well as a myriad of treatment strategies. Evaluation of the available data is made more difficult by lack of standardized repair techniques and comparable patient groups. As an increasing number of diaphragmatic hernia patients survive the newborn period, a study of the true incidence of recurrent diaphragmatic hernia, its etiologic factors, and the methods of repair is essential to prevent and treat this problem.     

Congenital diaphragmatic hernia

There is a paucity of level 1 and level 2 evidence for best practice in surgical management of CDH. Antenatal imaging and prognostication is developing. Observed to expected lung-to-head ratio on ultrasound allows better predictive value over simple lung-to-head ratio. Based on 2 randomised studies, the verdict is still out in terms the best group and indication for antenatal intervention and their outcome. Tracheal occlusion is best suited for prospective randomised studies of benefit and outcome. Only one pilot randomised controlled study of thoracoscopic repair exists, suggesting increased acidosis; blood gases and CO₂ levels should be closely monitored. Only poorly controlled retrospective studies suggest higher recurrence rates. Randomised studies on the outcome of thoracoscopic repair are needed. Careful selection, anaesthetic vigilance, monitoring and follow-up of these cases are required. There is no evidence to suggest the best patch material to decrease recurrences. Evidence suggests no benefit from routine fundoplication based on the one randomised study. Multi-disciplinary follow-up is required. This can be visits to different specialities, but may be best served by a multi-disciplinary one-stop clinic.     

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a lethal human birth defect. Hypoplastic lung development is the leading contributor to its 30-50% mortality rate. Efforts to improve survival have focused on fetal surgery, advances in intensive care and elective delivery at specialist centres following in utero diagnosis. The impact of abnormal lung development on affected infants has stimulated research into the developmental biology of CDH. Traditionally lung hypoplasia has been viewed as a secondary consequence of in utero compression of the fetal lung. Experimental evidence is emerging for a primary defect in lung development in CDH. Culture systems are providing research tools for the study of lung hypoplasia and the investigation of the role of growth factors and signalling pathways. Similarities between the lungs of premature newborns and infants with CDH may indicate a role for antenatal corticosteroids. Further advances in postnatal therapy including permissive hypercapnia and liquid ventilation hold promise. Improvements in our basic scientific understanding of lung development may hold the key to future developments in CDH care.