1/97. 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.- - - - - - - - - - ranking = 1keywords = newborn (Clic here for more details about this article) |
2/97. 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 = 41.06384241679keywords = persistent pulmonary hypertension, pulmonary hypertension, newborn, hypertension (Clic here for more details about this article) |
3/97. 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 = 1.0936482320574keywords = newborn, circulation (Clic here for more details about this article) |
4/97. Limb anomalies among offspring of women with a septate uterus: a report of three cases.A retrospective analysis of 467 births among 255 women with uterine malformations revealed that three (0.64%) newborns had limb reduction defects. Two women had a subseptate uterus and one a complete septate uterus with a longitudinal vaginal septum. One newborn had a bilateral split hand and split foot, one had absence of left hand and wrist. One infant born without left hand, wrist and one antebrachial bone associated with omphalocele and diaphragmatic hernia died during the neonatal period. An association between severe limb reduction defects and septate uterus is possible, although the mechanism is unclear. The findings indicate a need to evaluate the uterine cavity if a newborn has this invalid defect, and a detailed ultrasound examination of fetal limbs is warranted in a case of a pregnant septate uterus.- - - - - - - - - - ranking = 0.6keywords = newborn (Clic here for more details about this article) |
5/97. cholecystokinin administration in congenital diaphragmatic hernia--a case report and review of the literature.Deterioration in the respiratory function of a newborn infant with a repaired diaphragmatic hernia and respiratory insufficiency followed administration of cholecystokinin for cholestatic jaundice. The possible mode of action is discussed and a vasoactive/bronchoactive effect is proposed.- - - - - - - - - - ranking = 0.2keywords = newborn (Clic here for more details about this article) |
6/97. Combined pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic ductal atresia: prenatal diagnostic features and review of the literature.The authors recently managed a case of combined pure esophageal, duodenal, biliary, and pancreatic ductal atresia in a fetus and newborn with trisomy 21. The authors present a case report and review of the literature, emphasizing the prenatal radiographic features of the combined lesions and the high incidence of associated anomalies including Down's syndrome. Prenatal suspicion of these anomalies warrants karyotype analysis as well as careful pre- and postnatal screening for other anomalies.- - - - - - - - - - ranking = 0.2keywords = newborn (Clic here for more details about this article) |
7/97. Congenital diaphragmatic hernia misdiagnosed as pneumothorax in a newborn.Congenital diaphragmatic hernia (CDH) is usually left sided and has a large defect allowing abdominal viscera herniated into thoracic cavity. The chest films usually show air-filled stomach and/or loops of bowel in the ipsilateral hemithorax with mediastinal shift. We report a newborn with CDH, presenting as hyperlucent hemithorax, right-shifted mediastinum, apparently normal pattern of abdominal bowel gas, with the tip of nasogastric tube below the left hemidiaphragm on the radiograph. It was initially misdiagnosed as pneumothorax, and the acute respiratory distress was temporarily relieved by needle aspiration. Hyperlucent hemithorax due to intrathoracic gastric dilatation alone is an unusual presentation of CDH in neonatal period. Absence of stomach bubble in the left upper quadrant of the abdomen, in both radiography and abdominal sonography, is an important clue to make diagnosis of CDH in this misleading condition.- - - - - - - - - - ranking = 1keywords = newborn (Clic here for more details about this article) |
8/97. Early sepsis, obstructive jaundice and right-sided diaphragmatic hernia in the newborn.A male newborn was admitted to our Unit because of early sepsis and shock. He required antimicrobial therapy and mechanical ventilation and initially did well, although he exhibited jaundice and cholestasis. During the second week he deteriorated, with radiological opacification of the right hemithorax and pleural effusion, and did poorly in spite of antibiotical therapy and drainage of the effusion. In the third week, the X-ray suggested some bowel loops in the right hemithorax. A right-sided diaphragmatic hernia was confirmed by a CT-scan, and surgery was performed with good outcome. The association of delayed-onset right-sided CDH following early sepsis and obstructive jaundice has not been published before, and illustrates a scarcely known form of presentation of this condition.- - - - - - - - - - ranking = 1keywords = newborn (Clic here for more details about this article) |
9/97. chylothorax after repair of congenital diaphragmatic hernia--a case report.chylothorax is a rarely recognised post-operative complication following repair of congenital diaphragmatic hernia. We report here a newborn infant with this condition which resolved with percutaneous chest drainage, total parenteral nutrition and enteral feeding of a formula high in medium-chain triglycerides.- - - - - - - - - - ranking = 0.2keywords = newborn (Clic here for more details about this article) |
10/97. Incarcerated diaphragmatic hernia secondary to plication for eventration of diaphragm.Diaphragmatic plication is the procedure of choice for congenital diaphragmatic eventration. In the absence of complications, most newborns with eventration do well after plication and have normal long-term respiratory functions. However complications are rare. recurrence, ipsilateral pneumothorax and rupture of the diaphragm have been reported in literature. We report a case of incarcerated diaphragmatic hernia in a two-year-old child who had undergone plication for eventration of the diaphragm at six months of age.- - - - - - - - - - ranking = 0.2keywords = newborn (Clic here for more details about this article) |
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