Congenital atresia of the portal vein and extrahepatic portocaval shunt associated with benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and atrial septal defect.

A rare case of congenital atresia of the portal vein and ductus venosus, extrahepatic portocaval shunt, benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and an atrial septal defect is reported. Twenty-two cases of congenital extrahepatic end-to-side shunts have been described before. Although additional anomalies are common in this type of shunt, hemangiomatosis has been described only once. Adrenal hyperplasia has never been reported in this anomaly.

Diaphragm pacing with a quadripolar phrenic nerve electrode: an international study.

We sought to determine the international experience with the quadripolar diaphragm pacer system and to test two hypotheses: the incidence of pacer complications would be (1) increased among pediatric as compared to adult patients; and (2) highest among active pediatric patients with idiopathic congenital central hypoventilation syndrome (CCHS). Data were collected via a questionnaire coupled with the Atrotech Registry data for a total of 64 patients (35 children and 29 adults) from 14 countries. Thoracic implantation of electrodes and bilateral pacer use each occurred in 94% of all subjects. Tetraplegic (vs pediatric CCHS) patients were more typically paced 24 hours/day (P = 0.001). Pacing duration averaged 2.0 +/- 1.0 years among children and 2.2 +/- 1.1 years among adults. Infections occurred among 2.9% of surgical procedures, all in pediatric CCHS patients (vs pediatric tetraplegic patients, P = 0.01). The incidence of mechanical trauma was 3.8%, without significant differences among patient groups. The incidence of presumed electrode and receiver failure were 3.1% and 5.9%, respectively, with internal component failure greater among pediatric CCHS than pediatric tetraplegic patients (P < 0.01). Intermittent or absent function of 0-4 electrode combinations occurred among 19% of all patients, with increased frequency among pediatric CCHS than pediatric tetraplegic patients (P < 0.03). Complication-free successful pacing occurred in 60% of pediatric and 52% of adult patients. In all, 94% of the pediatric and 86% of the adult patients paced successfully after the necessary intervention. Although pacer complications were not increased among pediatric as compared to adult patients, the incidence of complications was highest among the active pediatric patients with CCHS. Longitudinal study of these patients will provide invaluable information for modification and improvement of the quadripolar system.

[Practical experiences with home ventilation in childhood]. / Praktische Erfahrungen mit der Heimbeatmung im Kindesalter.

BACKGROUND: The introduction of portable ventilators independent of compressed air as well as non-invasive devices for assessment of blood gases, helped to facilitate a long-term mechanical ventilation in family environment in the last decade. Home care is of particular interest at the infant age, as the disruption of the mother-child relationship may induce severe developmental disorders, known as syndrome of psychosocial deprivation. PATIENTS AND METHODS: We investigated retrospectively the tendency of the ventilator parameters, the support and the daily practice of 16 families with children of all ages with long-term ventilator assistance. RESULTS: The duration of the ventilation ranged between 5 months and 14 years, on the average at 5.5 years. The average portion of the ventilation at home care was 78%. The respiratory patterns, tidal volume and positive inspiration pressure, exceeded occasionally the physiologic standard of the age group. The common reason is, that the children prefer small sized tubes, having advantages in comfort and speech abilities. Therefore results an increased tube-resistance and air leakage of the tracheostomy tubes. The long-term tendency of the ventilator parameters is stable or improving, excluding the patients with progredient primary diseases. Commonly the motivation of the family members is high, therefore the psychosocial integration of the patients satisfying. Two thirds of the families have no personal support for the domiciliary care of the ventilator assisted child. Mothers tell about an enormous distress. CONCLUSIONS: The long-term mechanical ventilation of children at home care is an adequate therapy for all ages. It combines technical support of the underlying chronic respiratory failure with the chance of an intact psychosocial development of the child. Specialized centres and a national organisation may provide competent information and support for the increasing number of home-ventilated children in Germany.

Cerebrospinal fluid penetration of cefmenoxime in children with bacterial meningitis.

Blood and cerebrospinal fluid (CSF) concentrations of cefmenoxime were determined either microbiologically or by means of HPLC in 20 children with proven or suspected bacterial meningitis. Sixteen children suffered from bacterial meningitis: causative organisms were Haemophilus influenzae type b (n = 10), Streptococcus pneumoniae (n = 4) and Neisseria meningitidis (n = 2). In these patients the cefmenoxime concentration in the CSF ranged from 0.9 to 12.2 mg/l, with a mean concentration of 4.63 mg/l 1.5-3 h after the last intravenous cefmenoxime application and 24-48 h after initiating therapy with 200 mg cefmenoxime/kg/d in four doses. In eight cases the bactericidal titers of the CSF were examined during therapy. Titers between 1:64 and 1:2,048, exceeding the minimal bactericidal concentration, were found. After five doses of cefmenoxime 50 mg/kg, two CSF cultures showed bacterial growth: one H. influenzae (bactericidal titer in CSF 1:256) and one S. pneumoniae.

Complete thrombotic obliteration of the ascending aorta and the aortic arch as a cause of acute heart failure in a newborn.

Thrombosis of the great vessels, and especially of the aorta, is rare in neonates. We report a case with thrombosis of the ascending aorta, aortic arch, brachiocephalic trunk and subclavian artery. Clinically, severe heart failure occurred on the 1st day of life and the diagnosis was confirmed by echocardiography and cardiac catheterization, including angiocardiography. Left ventricular function was found to be extremely depressed. An infusion with prostaglandin E1 was initiated in order to improve the systemic circulation by dilating the arterial duct. The infant died of neurological complications prior to surgery.

[Control of breathing in the central alveolar hypoventilation syndrome with and without a phrenic pacemaker]. / Untersuchungen der Atemregulation bei zentralem alveolärem Hypoventilations-Syndrom ohne und mit Zwerchfellschrittmacher.

Primary central alveolar hypoventilation (CAHV) is a rare disorder described in newborns, children, and adults. We report a 2 9/12 year old child with CAHV of unknown etiology. The evaluation of her ventilatory control system showed abnormalities awake and in the different sleep states. Hypoventilation was found to be more severe during non-REM sleep than during REM sleep and awake state. She had central apnea, an irregular respiratory rhythm in the non-REM sleep too, and diminished ventilatory response to inhaled 5%-6% CO2 in both REM and non-REM sleep. Her ventilation decreased when she was breathing 50% and 100% oxygen. During breathing 15% oxygen she did not arouse in spite a transcutaneous pO2 of 10 mmHg. She was first treated with mechanical ventilation during sleep and has now received bilateral simultaneous phrenic pacemaker support during quiet sleep for about one year. With the phrenic pacemaker she has normal minute volume and transcutaneous blood gases during sleep. During a respiratory infection she needed again mechanical ventilation via her tracheostoma 24 hours a day for one week. This case of a CAHV demonstrates a dysfunction of the central and partially also of the peripheral chemoreceptors. The abnormalities of the ventilation were demonstrable not only in the non-REM sleep but also in the REM sleep and awake state.

[Experience using transcutaneous pO2 measurement (tcpO2 measurement) during the transport of premature and newborn infants with artificial respiration]. / Erfahrungen mit der transkutanen pO2-Messung (tcpO2-Messung) beim Transport beatmeter Früh- und Neugeborener.

In 55 high-risk neonates of different birth-weight and gestational age, transcutaneous pO2 (tcpO2) was continuously monitored during transports from the Obstetric Units to the Neonatal Intensive Care Units. We were able to demonstrate that the need for oxygen was overestimated in most of the children and thus FiO2 could be reduced in nearly all cases. In this way, FiO2 can be adapted to the real need of the children and hypoxemias can be avoided as far as possible frequency and duration of hyperoxemias can be reduced. Moreover, valuable knowledge about further management of mechanical ventilation may be obtained. Complications, as e.g. obstruction of endotracheal tube, are more rapidly recognized than by ECG-monitoring alone.

Early diagnosis and medical treatment of the persistent ductus arteriosus in infants.

Diagnosis of an isolated patent ductus arteriosus (PDA) is usually straightforward. If at later age it is associated with pulmonary hypertension, however, the symptoms may be variable and more difficult to assess. In the age group primarily discussed here, pulmonary hypertension is frequently present, so diagnosis cannot be based on physical findings alone. Echocardiography and Doppler echocardiography are essential diagnostic procedures; however, cardiac catheterization and angiography may still be needed especially in cases with associated heart defects. At present, surgical closure of the PDA is the therapy of choice in infants. In preterm newborns, an attempt by medical treatment is indicated and often promising. Unfortunately, there are no interventional techniques available at present for duct closure in this age group, whereas in some centers catheter closure of a PDA is successfully employed in older children.

Non-invasive oscillometric measurement of systolic, mean and diastolic blood pressure in infants with congenital heart defects after operation. A comparison with direct blood pressure measurements.

The accuracy of indirect oscillometric blood pressure measurements has been evaluated in 32 infants up to 11 months of age undergoing heart surgery. In a number of 1029 simultaneous measurements the indirect blood pressure was compared with the direct value obtained from a radial artery catheter. Cuffs of different sizes were applied. The main results were as follows: Regarding the ratio of cuff width to arm circumference, the best correlation between oscillometric and direct blood pressure measurements was obtained with a ratio of 0.38-0.41. The value of indirect blood pressure measurements depends on the absolute height of the blood pressure. In low blood pressure there is a tendency to underestimate and in high blood pressure there is a tendency to overestimate by the oscillometric blood pressure measurement. By applying an appropriate cuff size and by using our diagrams it should be possible to derive a direct value for the blood pressure on the basis of indirect oscillometric blood pressure measurements.

Urapidil therapy for acute hypertensive crises in infants and children.

In 19 infants and children with acute and severe hypertension following a cardiovascular operation urapidil infusion was started for treatment of the hypertensive crisis. In all patients blood pressure was effectively reduced within 15 min. The drop in systemic blood pressure was combined with a reduction of central venous pressure. Heart rate and urine volume remained unaltered. Serum electrolytes after 12-24 h therapy showed a slight but significant decrease in serum sodium and an increase in serum potassium concentration. In one case urapidil treatment had to be interrupted because of hypotension. In this case the urapidil therapy was tolerated later in lower doses. Serious side effects were not observed. In our experience urapidil can be recommended for the treatment of hypertensive crises in children.

[Can the indications for the presence of the neonatal emergency service be determined before the onset of labor?]. / Lässt sich die Indikation für den Abruf des Neugeborenen-Notarzt-Dienstes (NNAD) vor der Geburt stellen?

The purpose of this study was to recommend guidelines for the consultation of the Neonatal Emergency Service when birth risks appear. Data from 39.479 births in 20 obstetric clinics and 1.723 emergency transports to the same clinics during the period 1979-1981 were analysed. The criteria applied for neonatal distress were necessity of resuscitation measures in the delivery room and perinatal mortality. It is recommended that the Neonatal Emergency Service should be called in before birth in the following cases: all premature births up to 34 weeks of gestation; from 35 to 37 weeks in case of additional risk factors; from 38th week onward in case of intrapartum bleeding, umbilical cord prolapse or tear, multiple births, transverse or face presentations and continuous heart rate deceleration.

Phrenic nerve pacing for the treatment of central hypoventilation syndrome - state of the art and case report.

The application of phrenic nerve pacing is an effective method of respiratory support in adults with certain forms of acquired failure of automatic ventilation. The experience with one infant suffering from congenital central hypoventilation (Ondine's syndrome) is summarized in this report. Stimulation electrodes were implanted on the thoracic portion of each phrenic nerve. After a cumbersome period of intensive medical care and conditioning of the phrenic nerve-diaphragm-system, the patient could be weaned off the respirator. Severe upper airway obstruction, however, required tracheostomy. Bilateral simultaneous pacing has been effective now for more than 2 years providing the desired normal ventilation during sleep. The state of the art of phrenic nerve pacing is described.

[Children with implanted pacemakers. Recommendations for ambulatory follow-up and emergency therapy in case of pacemaker failure]. / Kinder mit implantierten Schrittmachern. Empfehlungen für die ambulante Uberwachung und für die Notfall-Therapie des Schrittmacher-Versagens.

Permanent pacemaker implantation in children is a necessary therapy in case of life-threatening bradycardias, which cannot be treated satisfactorily by drugs. Because of the relative high frequency of complications in comparison with the pacemaker therapy in adults there is need for a consequent follow-up by a pacemaker-center, the family-doctor and the parents. Recommendations are given for the outpatient follow-up and for the emergency therapy in case of pacemaker-failure.

[PFC syndrome (persistent fetal circulation). Central cyanosis in a full-term newborn infant without congenital heart defect]. / PFC-Syndrom (Persistenz der fetalen Zirkulation). Zentrale Zyanose eines reifen Neugeborenen ohne angeborene Herzmissbildung.

In a case report the main features of the PFC-syndrome (persistent fetal circulation) are demonstrated: 1. general central cyanosis, 2. pulmonary hypertension, 3. right-to-left shunting via foramen ovale and (or) ductus arteriosus. At postmortem analysis in some of these patients the muscular thickness of the pulmonary vessels was found to be increased: primary PFC-syndrome. Hypoxia, shock, and many pulmonary disturbances of the newborn can produce the clinical picture of "persistent fetal circulation": secondary PFC-syndrome. Under adequate therapy with O2-insufflation, vasodilatory drugs, and if necessary mechanical ventilation the prognosis in the majority of cases is good.