Specific sensitization of lymphocytes to tumor antigens by co-cultivation with peritoneal cells exposed to such antigens

Twenty-seven cases of congenital posterolateral diaphragmatic hernia past infancy are reviewed in tabular form and discussed as to presenting symptoms, physical and radiographic findings, operative treatment, and final outcome. A ten year old male treated by us is presented as a detailed case report. A great contrast is noted between the acute respiratory symptoms which threaten life in the infant hernia compared with the more chronic and recurrent gastrointestinal and respiratory symptoms in pateints past infancy. Onset of symptoms did not correlate with age or sex. Chest x-ray films and gastrointestinal contrast studies were most helpful in diagnosis. Abdominal and thoracic approaches were equally effective in reducing the herniated viscera and closing the diaphragmatic defect. We believe that long-term survival of patients with congenital posterolateral diaphragmatic hernia may be due to persistence of a confining pleuroperitoneal sac. Rupture of this sac in later life may coincide with onset of the characteristic symptoms which in turn prompt diagnostic studies. Congenital diaphragmatic hernia must be considered in the differential diagnosis of patients with both recurrent gastrointestinal and respiratory complaints. Plain radiographs of the chest and contrast studies of the gastrointestinal tract are necessary to confirm diagnosis preoperatively.     

Embryology of congenital diaphragmatic hernia

It is still generally believed that the defect in congenital diaphragmatic hernia results from failure of the so-called pleuroperitoneal canals (PPCs) to close at the end of the embryonic period (8th gestational week). Furthermore, it is assumed that gut could enter the thoracic cavity through this defect, causing compression and finally hypoplasia of the lung. However, this sequence of embryological events has never been studied, and many details even of normal diaphragmatic development are still unknown. Using scanning electron microscopy and a new animal model of congenital diaphragmatic hernia (CDH), the nitrofen rat model, the normal embryology of the diaphragm was reinvestigated and, for the first time, the crucial developmental steps of congenital diaphragmatic hernia formation were studied. The basic results were: (1) In normal development, the PPCs are never wide enough to allow herniation of gut loops. (2) The formation of the defect happens in an early embryonic period. (3) The early ingrowth of liver through the defect is of major importance for the formation of CDH. In another set of experiments, the nitrofen rat model of congenital diaphragmatic hernias was used to study the cellular mechanisms involved during epithelial and mesenchymal growth and differentiation in normal and in abnormal lungs. These results, combined with selected culture techniques (eg, branching morphogenesis and epithelio-mesenchymal interaction) probably open new ways to a better understanding of the mechanisms that finally lead to an abnormal lung in CDH.     

Laparoscopic operation of a Morgagni hernia

Morgagni hernias are the least common form of diaphragmatic hernias. Although they are congenital, most of them are not diagnosed until later in life. The indication for surgery is based on the patient's symptoms or on the radiological evidence of incarcerated tissue, and until quite recently involved a laparotomy or thoracotomy. Laparoscopy not only permits the suspected diagnosis to be confirmed--which is otherwise often difficult--but also makes it possible to close the hernia site by suturing. For improved security, the hernia site is augmented by fixing in place a non-absorbable mesh. The operative technique employed is described.     

Wandering spleen presenting as duodenal obstruction after repair of congenital diaphragmatic hernia

The most common presentation of the wandering spleen in children is torsion with infarction. Duodenal obstruction by the spleen has not been reported previously. Wandering spleen can accompany congenital diaphragmatic hernia (CDH) because of its loss of retroperitoneal fixation. If absence of normal splenic fixation is found during repair of CDH, splenopexy should be performed to eliminate the risk of torsion, infarction, or, as described here, duodenal obstruction.     

Hemoglobin sickle-lepore: an unusual case of sickle cell disease

We report the case of a newborn with a large left-sided congenital diaphragmatic hernia (CDH) who required extra corporeal membrane oxygenation (ECMO) for severe respiratory insufficiency. CDH repair had to be performed on bypass circulation. Intraoperatively, an atypical hemihepatectomy of the herniated lobe was conducted, because reposition of the liver led to a kinking of the vena cava and to a torsion of the right lobe, resulting in ischemia and compromised venous flow. The extraordinary anatomical indication and the potential danger of uncontrollable bleeding are discussed.     

Measurement of DNA in intervertebral disc and other autofluorescent cartilages using the dye hoechst 33258

BACKGROUND AND OBJECTIVES: Morgagni hernias are unusual congenital diaphragmatic hernias that are generally asymptomatic and discovered incidentally. Surgical treatment is indicated once the diagnosis is made. These hernias have traditionally been repaired by the open abdominal or thoracic approaches. We report a case of Morgagni hernia repaired successfully via the laparoscopic approach. METHODS AND RESULTS: The patient was noted to have a large anteromedial diaphragmatic hernia by chest radiograph and CT imaging. He underwent laparoscopy, during which the hernia was reduced and the defect repaired with mesh placement. We used intracorporeal suture placement to anchor the mesh. The patient recovered uneventfully after a short hospitalization. CONCLUSIONS: The laparoscopic approach for repair of Morgagni hernias offers diagnostic advantages as well as the potential for reduced morbidity when compared to laparotomy. We report intracorporeal knot-tying for fixation of the mesh to be a secure and satisfactory means to achieve the laparoscopic repair.     

Evidence of transmission of hepatitis B virus to spouses from sequence analysis of the viral genome

Despite advances in postnatal care, patients born with a congenital diaphragmatic hernia (CDH) suffer substantial morbidity and mortality. The present study was undertaken to determine the prognostic influence of prenatally-diagnosed liver herniation in the hemithorax in fetuses with CDH. The medical records of 48 patients evaluated for a prenatally-diagnosed left CDH were retrospectively reviewed. Patients were analysed according to the position of the liver by prenatal ultrasound; 32 fetuses had a major portion of the liver herniated into the left hemithorax ('liver up') and 16 had an intra-abdominal liver ('liver down'). Liver position was determined using colour-flow Doppler ultrasonography. There were two fetal deaths in the liver-up group and one in the liver-down group. The liver-up group more frequently required extracorporeal membrane oxygenation (ECMO) support (53 per cent) compared with the liver-down group (19 per cent). Postnatal survival was significantly less in the liver-up group (43 per cent) vs. the liver-down group (93 per cent). Fetuses with congenital diaphragmatic hernia and liver herniated into the hemithorax have a much worse prognosis than similarly afflicted fetuses without liver herniation. Prenatal ultrasonographic diagnosis of congenital diaphragmatic hernia allows for preparation for a critically ill newborn and aids in prenatal family counselling.     

Step-wise lengthening for delayed repair of avulsion of the flexor pollicis longus

Congenital abnormalities of the umbilical venous system are rare. A case of fatal right congenital diaphragmatic hernia (CDH) in association with an anomalous umbilical vein bypassing the liver and directly entering the right atrium is presented. The ductus venosus was absent. Although much of the liver was within the right hemithorax, radiographs showed an apparently normal umbilical venous catheter (UVC) course, suggesting a normally positioned liver and mitigating against the diagnosis of CDH. Aberrant umbilical drainage, yielding a falsely normal appearing UVC course, may delay the diagnosis of CDH.     

Effects of barium on stimulus-induced changes in [K+]o and field potentials in dentate gyrus and area CA1 of human epileptic hippocampus

Congenital para-oesophageal hiatal hernia (PEHH) is a rare problem in infancy, however, it constitutes a clinical entity that mandates surgical repair once the diagnosis is made. In the paediatric age group, acquired PEHH has been described as a major complication in a number of patients who were treated surgically for gastro-oesophageal reflux (GER) by Nissen fundoplication. PEHH is a frequently encountered condition in elderly patients; it accounts for 5% of diaphragmatic hiatal hernias. In both paediatric and adult patients PEHH, whether congenital or acquired in origin, is usually associated with potentially lethal complications such as gastric volvulus, incarceration, and perforation. In clinical practice true PEHH is extremely rare. The term has been expanded to include large gastric hiatal hernias where most of the stomach and the gastro-oesophageal junction are in the chest. Six infants with congenital PEHH are presented, together with an attempt to understand its possible aetiology and a review of its current surgical management.     

Congenital diaphragmatic hernia and intrathoracic intestinal volvulus

The main symptom of the congenital diaphragmatic hernia (CDH) is the respiratory distress due to the pulmonary hypoplasia and the persistence of foetal pulmonary circulation. Sometimes the CDH appears outside of the neonatal period with respiratory symptoms, abdominal pain and seldom with intestinal obstruction. A case of CDH presenting with intrathoracic volvulus is reported. This case shows that tendency of delayed repair until the newborn has been recovered, require more attention because the good condition of the patient can be changed not only by the pulmonary hypoplasia and the persistence of the foetal pulmonary circulation but also by the symptoms of the intrathoracic gut complications.     

Concurrent right diaphragmatic hernia and esophageal atresia

The occurrence of a coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula is extremely rare and is considered highly lethal. Only 19 cases of CDH with EA have been reported in the world literature to date. This is a very challenging clinical problem, and the neonate is likely to deteriorate rapidly. Such a case is reported with a successful outcome, probably the first survivor with a right CDH and EA. Management guidelines for such a case are discussed.     

Contemporary controversies in the management of congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) has remained the most frustrating of the major birth defects to manage successfully. Despite the earlier detection of severe diaphragmatic defects by prenatal ultrasound, and the early recognition of CDH as a cause for respiratory distress presenting at birth, current mortality has improved little from the original series presented in 1940 by Ladd and Gross. This article discusses the difficulty in defining population with congenital diaphragmatic hernia, current controversies in the medical and surgical management of these patients, appropriate timing and usage of ECMO, a review of current, experimental therapies, and short-term and long-term outcomes of these patients.     

A comparative evaluation of two day hospitals. Goal attainment scaling of behavior therapy vs. milieu therapy

A retrospective anatomical, family, and epidemiological study was made of 143 patients (81 female and 62 male) with diaphragmatic hernia who were born in the south-west of England between 1943 and 1974. Thirty-nine cases were stillborn. Seventy-five per cent of patients had a left-sided diaphragmatic defect, 22% had a right-sided defect, and 3% had a bilateral defect. Fifty per cent of the patients had other congenital malformations, most frequently of the nervous system. No maternal age or birth order effect was noted. Cases of diaphragmatic hernia without other malformations had in general a normal fetal growth rate. Eight per cent of the cases were illegitimate. There were two pairs of twins discordant for diaphragmatic hernia, one pair being dizygotic and the other monozygotic. In no case of diaphragmatic hernia was there a relative affected with a diaphragmatic hernia. The most common type of diaphragmatic defect was a posterolateral hernia (92%), followed in frequency by an eventration of the diaphragm (5%), the least common defect being a retrocostosternal hernia (2%). Diaphragmatic hernia appears to be aetiologically as well as anatomically heterogeneous. In this series there were two cases of trisomy 18, one case of trisomy 21, one case trisomic for a small part of chromosome 20, and two cases with the Pierre Robin syndrome. It seems likely that diaphragmatic hernia is a non-specific consequence of several teratological processes.     

Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group

BACKGROUND: Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult. METHODS: The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients. RESULTS: Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours). CONCLUSIONS: The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.     

Ultrasound diagnosis of fetal diaphragmatic hernia and complex congenital heart disease at 12 weeks' gestation--a case report

Congenital diaphragmatic hernia is commonly associated with congenital heart disease. Their coexistence indicates a poor prognosis. Prenatal diagnosis of these conditions in early pregnancy allows the option of pregnancy termination. We present a case of left-sided fetal diaphragmatic hernia and complex congenital heart disease diagnosed by ultrasound examination at 12 weeks' gestation.     

Prediction of outcome in congenital diaphragmatic hernia

The case records of 59 patients with congenital diaphragmatic hernia (CDH) who presented between 1984 and 1997 were studied retrospectively. Included in the study were infants born with CDH who required respiratory support within the first 6 h of life. Twenty-three were excluded from the study for various reasons; 36 were enrolled in the study; the male-to-female ratio was 18:18. Twenty-nine hernias were left-sided and 7 were right-sided. All patients were ventilated using conventional techniques. Arterial blood gases were measured on average 1.76 h following admission and the initial period of resuscitation (range 1-4 h). Three formulae were applied in an attempt to predict outcome: ventilation index against PCO2, alveolar-arterial oxygen gradient, and a newly derived formula from this institution (Red Cross formula) that comprises respiratory rate x PCO2 x FiO2 x mean airway pressure/PaO2 x 6000. Seventeen patients (47.2%) survived and 19 died (52.8%); 21 became stable enough to undergo surgery. The average time from presentation until surgery was 1.98 days (range 6 h to 4 days). The Red Cross formula, with a 100% predictive value for mortality, 85% predictive value for survival, and an overall predictive value of 91.6%, appeared to be superior to the other formulae studied. The availability of a highly accurate predictive formula may facilitate management of this frequently lethal disease.     

Congenital anomalies of the diaphragm. Personal experience with thirty-five consecutive cases

A personal experience of thirty-five consecutive patients with congenital anomalies of the diaphragm is reported. The anomalies included twenty-five Bochdalek hernias, seven diaphragmatic eventrations, and three esophageal hiatal hernias. Except for the patients with esophageal hiatal hernias, virtually all patients presented with respiratory embarrassment, twenty-seven of the thirty-five within 24 hours of birth. An especially high mortality is associated with this early onset of symptoms. The treatment was surgical with a thoracic approach used for right Bochdalek defects and eventrations, and an abdominal approach used for left Bochdalek hernias. The importance of proper preoperative and postoperative management in a well equipped neonatal intensive care unit is emphasized.     

Neonatal detection of developmental dysplasia of the hip (DDH)

Congenital diaphragmatic hernia (CDH) may be associated with other anomalies, most frequently cardiovascular in nature. Despite fetal echocardiography, diagnosis of an accompanying cardiac malformation often is not made until after birth and sometimes not until after extracorporeal membrane oxygenation (ECMO) has been instituted. Aortic coarctation associated with CDH may occur as an isolated, surgically correctable malformation or it may be a component of the usually fatal left heart "hypoplasia" or "smallness" syndrome. The authors present two cases of aortic coarctation associated with CDH requiring ECMO that illustrate the management challenges of these coincident diagnosis. In one case, the accompanying coarctation was suspected and required precannulation angiography for confirmation, whereas in the other case, the diagnosis of coarctation was not made until after ECMO cannulation. Depending on its anatomic location and severity, an aortic coarctation associated with life-threatening CDH may limit the physiological efficacy of venoarterial ECMO. Furthermore, arterial cannulation for extracorporeal support requires that flow through the remaining carotid artery be maintained during aortic reconstruction, which may prove difficult for lesions best treated by subclavian flap angioplasty. When the diagnosis of coincident aortic coarctation and CDH is suspected or proven before institution of extracorporeal support, serious consideration should be given to venovenous bypass, because this may provide better postductal oxygenation and facilitate aortic repair with the option of left carotid artery inflow occlusion.     

Congenital diaphragmatic hernia. I. Simple defect of the diaphragm or anomaly of the pulmonary mesenchyme?

Described for the first time in 1848 by Bochdalek, congenital diaphragmatic hernia is still a hot topic. How can it be that a simple defect of the diaphragm still has a mortality rate reaching 50% in 1997, and this despite continuous progress in neonatal intensive care? If some problems remain unsolved, experimental studies over the past 30 years have raised some questions concerning the pathogenesis, and have shed some light into the pathophysiology of congenital diaphragmatic hernia. This article reviews the recent knowledge about the aetiology, pathogenesis and pathophysiology of this complex malformation.