1/781. Congenital diaphragmatic hernia: report of three cases. Three cases of congenital diaphragmatic hernia (CHD) are presented with special emphasis on a neonate with an unusual combination of abnormalities. It was noted that in all three the hernias were of the Bochdaleck's type. CDH may not always be a single isolated failure of closure of the pleural peritoneal hiatus but a more complex multi organ anomaly. ( info) |
2/781. Esophageal duplication cyst coexisting with Bochdalek's hernia and polysplenia. Esophageal duplication cyst, Bochdalek's hernia and polysplenia are uncommon congenital anomalies which have not been reported to be associated with each other. We present the radiological aspects of an unusual coexistence of these three congenital anomalies in a 4-month-old girl. ( info) |
3/781. Bochdalek hernia in adulthood: a case report and review of recent literature. A 37-year-old Filipino woman presented with a post road-traffic accident fracture of dorsal spine 12. Chest radiograph revealed evidence of loops of small bowel in the left lung field. She admitted to symptoms of respiratory insufficiency since birth and treatment for tuberculosis in childhood. A pre-operative diagnosis of left traumatic diaphragmatic hernia was not confirmed at laparotomy which revealed typical left congenital Bochdalek hernia with smooth edges and herniation of small bowel and spleen into the left pleural cavity. Following reduction and repair of the hernia, the patient made an uneventful recovery. Chest radiograph remains normal till now, eight years post-operatively. ( info) |
4/781. Laparoscopic repair of a Morgagni hernia: report of a case and review of the literature. A case is reported of an 85-year-old woman with subacute intestinal obstruction due to a large Morgagni hernia containing the transverse colon. The repair was carried out laparoscopically without a mesh. The procedure lasted 45 min, and the patient was discharged after 4 days. According to the literature, mini-invasive repair of a Morgagni hernia can be performed easily and without complications. In only few cases was a mesh necessary. The Morgagni hernia must be considered a clear indication for laparoscopic surgery, which should be offered as the first approach to this disease. ( info) |
5/781. Prenatal sonographic features of spondylocostal dysostosis and diaphragmatic hernia in the first trimester. Spondylocostal dysostosis is a congenital disorder characterized by multiple malformations of the vertebrae and ribs. We describe the sonographic features of an affected fetus at 12 and 14 weeks of gestation. The fetus had thoracic scoliosis, multiple vertebral and rib malformations and a grossly dilated stomach that had herniated into the chest through a left-sided diaphragmatic hernia. The stomach spanned the whole length of the fetal trunk. ( info) |
6/781. Two forms of cutis laxa presenting in the newborn period. Two infants are described with congenital cutis laxa. They represent two distinct disorders. In the first, congenital cutis laxa is associated with a generalized disorder of elastic tissue in which there may be diaphragmatic or other hernias, diverticula of the gastrointestinal or urinary tract and infantile emphysema. The disease is fatal often within the first year. In the second, congenital cutis laxa is associated with widely patent anterior fontanel, a variety of malformations, and retarded growth and development. Recognition of these distinct syndromes in the newborn period and their recessive inheritance permit realistic discussion of the prognosis which is very different from the benign dominant forms of cutis laxa. ( info) |
| Disorders of gastric emptying are rare in healthy infants and children. Delayed gastric emptying is encountered in adults after operations on the stomach, such as vagotomy and partial gastrectomy, and is extremely rare in young patients. The authors report on a 15-year-old patient with gastroparesis after three attempts to repair a congenital diaphragmatic hernia. Medical therapeutic trials consisting of all combinations of diet regimes with various gastrokinetic drugs failed to alleviate the intractable vomiting. All the patient's symptoms resolved after subtotal gastrectomy with gastroduodenostomy (Billroth I). ( info) |
8/781. Bochdalek diaphragmatic hernia presenting with acute gastric dilatation. Congenital diaphragmatic hernia through the foramen of Bochdalek may present after infancy. A 21/2-year-old Malay girl presented with acute respiratory distress. Chest examination showed reduced chest expansion and decreased breath sounds on the left side. Chest radiograph showed a large "cyst" in the left chest, which was thought to be a lung cyst under tension. Tube thoracostomy resulted in clinical improvement. Results of a barium study showed that the cyst perforated by the thoracostomy tube was the stomach, which had herniated through a Bochdalek diaphragmatic defect. Surgical repair of the diaphragmatic defect and closure of the perforated stomach was performed successfully. Congenital diaphragmatic hernia should be included in the differential diagnosis of respiratory distress in young children. Nasogastric tube placement must be considered as an early diagnostic or therapeutic intervention when the diagnosis is suspected. ( info) |
9/781. Diaphragmatic hernia of Morgagni. Most cases of Morgagni hernia are asymptomatic and diagnosed incidentally on routine chest x-ray film, but they may occasionally become symptomatic. Symptomatic Morgagni hernias may present in many different ways, making the diagnosis challenging. We describe a patient with a Morgagni hernia, resulting in intractable nausea and vomiting, give a brief review of symptoms, note the different types of abdominal contents herniated, and describe the methods used to make the diagnosis. ( info) |
10/781. Dypiridamole, a cGMP phosphodiesterase inhibitor, transiently improves the response to inhaled nitric oxide in two newborns with congenital diaphragmatic hernia. INTRODUCTION: Congenital diaphragmatic hernia (CDH) remains a frustrating cause of respiratory failure associated with persistent pulmonary hypertension of the newborn (PPHN). Although inhaled nitric oxide (iNO) is effective in many infants with PPHN, it often fails to improve oxygenation in infants with CDH. As the increase in vascular smooth muscle cyclic guanosine monophosphate (cGMP) in response to iNO may be impeded by increased phosphodiesterase type-V (PDE-V) activity, it has been suggested that PDE-V blockade potentiates the efficiency of iNO. case reports: We used dypiridamole (Persantine), a specific PDE-V inhibitor, in two patients with CDH. prenatal diagnosis showed a left-sided CDH at 23 weeks of gestation (GA) with intrathoracic stomach and left heart underdevelopment in the one infant and a right-sided CDH at 26 weeks GA with intrathoracic liver in the other. After antenatal corticoids, planned delivery was performed by the vaginal route at 38 weeks GA. Preoperative stabilization was achieved by high frequency oscillation, iNO and inotropic support over 24 h. Both had early pneumothorax drained by a chest tube. Despite optimization of ventilatory and hemodynamic support with surfactant replacement, iNO and adrenaline, oxygenation worsened progressively. Dypiridamole was introduced intravenously at 27 and 40 h, respectively, and improved oxygenation over the next 12 h. However, oxygenation again deteriorated and both patients died. CONCLUSION: Dypiridamole enhanced the response to iNO in PPHN associated with CDH, although this effect was transient. Combined therapy of iNO with PDE-V inhibitors may improve pulmonary vasodilation in some forms of PPHN which do not respond to iNO, thereby reducing the need for extracorporeal membrane oxygenation (ECMO) and improving outcome. ( info) |