1/109. 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.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/109. intubation of newborn during delivery with intact umbilical cord--a case report.A 24-year-old gravida 2, para 1 woman at 38th week gestation was scheduled for elective cesarean section (C/S) because of a previous C/S and prenatal diagnosis of congenital diaphragmatic hernia. We decided to intubate the newborn during delivery before the umbilical cord was cut. After delivery of the fetal head and part of the shoulders, the mouth of the fetus was cleared and the trachea was intubated orally with a 2.5 mm internal diameter (I.D.) endotracheal tube under sterile conditions while the uteroplacental circulation was still intact. The patient had to be repeatedly resuscitated due to bradycardia in intensive care unit. No surgical correction of the hernia was attempted because of the poor condition of the baby, which died 3.5 hours after birth. Although our case ended up in mortality despite successful perinatal intubation, we recommend that in case where airway or ventilatory problems are anticipated, tracheal intubation is done during delivery before the umbilical cord is clamped. When the fetus is sharing the maternal circulation, it will allow physicians to have more time and safety to perform corrective measures.- - - - - - - - - - ranking = 5.8682187699711keywords = circulation, liver (Clic here for more details about this article) |
3/109. Intrapulmonary ectopic liver.A case of intrapulmonary ectopic liver in a patient who had undergone right-sided diaphragmatic hernia is described. The intrapulmonary ectopic liver was found incidentally in chest x-ray at the age of 6 years. The final diagnosis was established at thoracotomy. Fewer than 10 cases of intrathoracic ectopic liver have been reported previously, but an intrapulmonary ectopic liver like the present case has not been described previously.- - - - - - - - - - ranking = 4keywords = liver (Clic here for more details about this article) |
4/109. Congenital diaphragmatic hernia: atypical presentation.We report an unusual case of the left sided diaphragmatic hernia in which the left lobe of the liver blocked the herniation of the intestinal contents into the chest and the clinical symptoms were not apparent at birth. The patient was a full term infant born to a mother whose antenatal care included apparently normal sonographic evaluation. She developed increasing tachypnea within two hours period after birth. auscultation of the chest revealed bilateral normal aeration. No cardiac murmurs were heard. An X-ray of the chest revealed a left sided haziness without any mediastinal shift. Progressive decrease in the breath sounds was noted over the left base. A CT examination demonstrated a left sided diaphragmatic hernia, with herniation of the left lobe of the liver causing compression of the lung. No loops of the bowel were observed in the thoracic cavity. She underwent laparotomy and repair of diaphragmatic hernia. This case history illustrates that large left sided diaphragmatic hernias may be missed on antenatal sonogram and may not present with clinical signs until several hours after birth. Both physical examination and routine chest X-ray may prove inconclusive and a CT scan is necessary to make a definitive diagnosis.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
5/109. adult right-sided Bochdalek hernia facilitated by coexistent hepatic hypoplasia.An adult case of right-sided Bochdalek hernia complicating intrathoracic intestinal strangulation is presented. Formation of this rare right-sided diaphragmatic hernia was facilitated by coexistent hypoplasia of the medial segment of left hepatic lobe. Computed tomography showed characteristic findings such as dilated small intestine with convergence of mesenteric folds in the thorax, which was highly diagnostic of intestinal strangulation due to diaphragmatic hernia.- - - - - - - - - - ranking = 0.78251831831961keywords = hepatic (Clic here for more details about this article) |
6/109. Unexpected life-threatening complications in Kabuki syndrome.Kabuki syndrome is a rare multiple congenital anomalies/mental retardation syndrome comprising a distinct facial appearance and fetal fingertip pads. We observed two patients with Kabuki syndrome and describe unusual life-threatening complications, including stenosis of the central airways (not previously reported), extrahepatic biliary atresia, and congenital diaphragmatic hernia.- - - - - - - - - - ranking = 0.15650366366392keywords = hepatic (Clic here for more details about this article) |
7/109. Bilateral congenital diaphragmatic hernia: diagnostic clues at fetal sonography.Bilateral congenital diaphragmatic hernia is a rare, life-threatening malformation. We describe a case of bilateral Bochdalek hernia diagnosed prenatally. The sonographic clues to the diagnosis were anterior displacement of the heart with relatively minimal lateral shift. The definitive diagnosis was made by demonstrating the liver in the right thorax and bowel loop and stomach in the left thorax. color and power Doppler demonstrated the hepatic vessels embracing both sides of the heart from behind.- - - - - - - - - - ranking = 0.65650366366392keywords = liver, hepatic (Clic here for more details about this article) |
8/109. An unusual anterior mediastinal mass in a child with B-thalassemia major.This case report describes the delayed appearance of Morgagni's hernia in a 5 year old child with B-thalassemia major to present as an anterior mediastinal mass. The progressive enlargement of the liver resulted in herniation of the left lobe of the liver through the already congenitally present Morgagni's hernia leading to its enlargement. The report also emphasizes the fact that a previously normal chest x-ray should not preclude the diagnosis of Morgagni's hernia. Morgagni's hernia should also be included in the differential diagnosis of anterior mediastinal masses in children.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
9/109. Accessory liver in an infant with congenital diaphragmatic hernia.incidence of congenital diaphragmatic hernia (CDH) ranges from 1 in 2,400 to one in 5,000 live births. Associated anomalies in CDH are approximately 30% to 35%. The authors report on an infant with CDH and an accessory supradiaphragmatic liver with separate vascularization from the thoracic aorta. The pathogenesis of this malformation is discussed. The postoperative course and the follow-up of the child over 5 years was completely uneventful.- - - - - - - - - - ranking = 2.5keywords = liver (Clic here for more details about this article) |
10/109. Bilateral diaphragmatic hernia followed by fetal ultrasonography. A report of two cases.Two cases of bilateral congenital diaphragmatic hernia (CDH) followed by fetal ultrasonography were described. Although many cases of CDH are diagnosed by fetal ultrasonography, it is difficult to diagnose bilateral CDH in utero, which is a relatively rare and fatal condition. Two fetuses were diagnosed as having left CDH associated with severe anomalies. However, a retrospective review of fetal ultrasonography indicated elevation of the liver in the right posterior chest. Both patients died shortly after surgical repair for left CDH despite the use of extracorporeal membrane oxygenation. Diagnosis of bilateral CDH by fetal ultrasonography and the evaluation of its prognosis were discussed.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
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