Preoperative selective embolization with vascular coiling of giant sacrococcygeal teratoma.

Sacrococcygeal teratoma is one of the most common congenital tumors. Its optimal management requires interdisciplinary care by obstetricians, radiologists, pediatric surgeons, and neonatologists. Early surgery entailing complete tumor excision is the main therapy aim, but a substantial risk of life-threatening complications remains, especially uncontrollable intraoperative hemorrhage. To reduce the risk of bleeding in a female neonate with a giant sacrococcygeal teratoma, we successfully coil-embolized the tumor's main feeding arteries. Her subsequent complete surgical resection was uneventful, and the child is well with favorable reconstructive and functional status of all involved and adjacent organ systems.

[Percentile Values for the Anthropometric Dimensions of Triplet Neonates - Analysis of German Perinatal Survey Data of 2007-2011 from all States of Germany]. / Perzentilwerte für die Körpermaße neugeborener Drillinge - Ergebnisse der deutschen Perinatalerhebung der Jahre 2007-2011 unter Beteiligung aller Bundesländer.

AIM: We aimed to develop national reference values for birth weight, length, head circumference, and weight for length for newborn triplets based on data from the German perinatal survey of 2007-2011. MATERIAL AND METHODS: Perinatal survey data of 3,690 newborn triplets from all the states of Germany were kindly provided to us by the AQUA Institute in Göttingen, Germany. Data of 3,567 newborn triplets were included in the analyses. Sex-specific percentile values were calculated using cumulative frequencies. Percentile values at birth were computed for the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles for 21-36 completed weeks of gestation. RESULTS AND CONCLUSIONS: We present the first German reference values (tables and curves) for the anthropometric dimensions of triplet neonates and compare selected birth weight and length percentiles of triplets (after 32 and 34 completed weeks of gestation) to those of singletons and twins. The differences in the 50th birth weight percentiles between singletons and triplets after 32 completed weeks of gestation were 180 g for girls and 210 g for boys; after 34 weeks of gestation the differences were 320 and 325 g, respectively. The differences between twins and triplets after 32 weeks of gestation were 100 g for girls and 120 g for boys; after 34 weeks of gestation they were 130 and 135 g, respectively. The data presented here enable the classification of newborn triplets according to somatic parameters making reference to German perinatal data.

[New percentile values for the anthropometric dimensions of twin neonates: analysis of perinatal survey data of 2007-2011 from all 16 states of Germany]. / Neue Perzentilwerte für die Körpermaße neugeborener Zwillinge: Ergebnisse der deutschen Perinatalerhebung der Jahre 2007-2011 unter Beteiligung aller 16 Bundesländer.

AIM: The aim of this study was to develop new national standards for birth weight, length, head circumference, and weight for length for newborn twins based on the German perinatal survey of 2007-2011. We also assessed trends in anthropometric measurements by comparing these new percentile values with the percentile values of 1990-1994. MATERIAL AND METHODS: Perinatal survey data of 110,313 newborn twins from all the states of Germany collected in the years 2007-2011 were kindly provided by the AQUA Institute in Göttingen, Germany. Sex specific percentile values were calculated using cumulative frequencies. Percentile values at birth were computed for the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles for 21-40 completed weeks of gestation. Percentile curves and tabulated values for the years 2007-2011 were compared with the published values of 1990-1994. RESULTS AND DISCUSSION: The new percentile curves (2007-2011) closely resemble the previous ones (1990-1994). Small differences can nonetheless be found. For example, for birth weight the new values for the 10th percentile are a little higher. CONCLUSIONS: We recommend using the new percentile values instead of the old ones.

Somatostatin - therapeutic option for chylothorax in preterm neonates. Report on two patients and review of the literature.

We report the use of somatostatin in two preterm neonates with chylothorax and discuss those cases in the light of current literature on the treatment of chylothorax in infants born preterm. Chylothorax, a severe complication in thorax surgery, is also a symptom of different diseases and may even occur spontaneously. Treatment is difficult, especially in preterm neonates with co-morbidities. The standard therapeutic strategy with non-invasive procedures (e.g. enteral diet free of long chain triglycerides or parenteral nutrition) is not always effective. Surgical interventions, like pleurodesis, ligation of the ductus thoracicus, or pleuroperitoneal shunt may be of considerable risk in preterm infants and must be carefully evaluated. Somatostatin is a new non-invasive therapeutic option for the treatment of chylothorax in adults and older pediatric patients. Case reports demonstrate the effectiveness of the somatostatin treatment, mostly in adult patients and in adolescents. There are only few case reports describing the use of somatostatin in preterm neonates. One VLBW (very low birth weight) and one hypotrophic ELBW (extremely low birth weight) neonate (gestational ages of 30+3/7, and 25+2/7 weeks; birth weights of 1270g, and 450g respectively) were treated for chylothorax with continuous infusion of somatostatin in addition to the dietary treatment. The chylothorax disappeared after start of somatostatin. No major side-effects of the somatostatin treatment were observed. As reported in other published pediatric cases, somatostatin seems to be a therapeutic option for the treatment of chylothorax in preterm neonates. In review of the literature we identified another eleven case reports on the treatment of persistent chylothorax with somatostatin or octreotide in preterm neonates. Further observations are needed before somatostatin can be recommended as a standard first-line treatment procedure for chylothorax in infants.

Neurodevelopmental outcome and pulmonary morbidity two years after early versus late surfactant treatment: does it really differ?

AIM: To investigate whether neurodevelopmental outcome or pulmonary morbidity at age two years might be different after early versus late surfactant treatment in intubated preterm infants with severe respiratory distress syndrome (RDS). METHODS: In 185 ex-preterm infants of 27-32 completed weeks of gestation, who were enrolled in a controlled trial of early versus late surfactant treatment (31 +/- 19 min vs. 202 +/- 80 min, respectively), a standardized follow up of medical history, pulmonary morbidity and neurodevelopmental outcome using the Griffiths scales were carried out. RESULTS: Neurobehavioural and motor development was comparable in both groups, as was medical history and actual morbidity. However, in the early treatment group a delay in the subscale 'personal social' of the Griffiths test and in one 'milestone' of motor development (rolling over from supine to prone) was noticed, and the rate of increased muscular tone was significantly higher. CONCLUSION: In terms of long-term morbidity or neurological development there is no obvious advantage of an immediate surfactant administration after intubation in preterm infants with RDS. This is in line with our results published earlier on morbidity at discharge, so improvement of gas exchange after intubation can first be awaited before surfactant is indicated.

A congenital teratoma with a cleft palate: report of a case.

Teratomas are benign tumors containing cells from ectodermal, mesodermal and endodermal layers. They occur in about 1 in every 4000 births and most commonly in the sacrococcygeal region, followed by the ovaries. Congenital epignathus teratomas are rare embryological neoplasms localised in the region of head and neck. An epignathus is found in approximately 1:35,000 to 1:200,000 live births. This accounts for 2-9% of all teratomas. Size and location of the neoplasm in the oronasopharynx is variable. Teratomas are partly undiagnosed at the time of birth. They may exist with an intracranial extension or as small polyps. Large epignathi can lead to difficult management during and after birth. The case of a newborn girl with a combination of an epignathus and a cleft palate is described. The epignathus presented as a huge mass extending out of the mouth of the infant girl. On the day of birth debulking of the extraoral portion of the tumor, followed by intraoral exstirpation, was performed. The results of the histologic examination indicated a congenital epignathus. Six months later a recurrence was found.

[Mortality and morbidity of very premature infants in Baden-Württemberg depending on hospital size. Is the current degree of regionalization adequate?]. / Mortalität und Morbidität sehr unreifer Frühgeborener in Baden-Württemberg in Abhängigkeit von der Klinikgrösse. Ist der derzeitige Grad der Regionalisierung ausreichend?

BACKGROUND: Regionalization of perinatal and neonatal care improves outcome. The aim of this study was to compare outcome in preterm infants with a gestational age (GA) < 32 weeks in relation to patient volume. METHODS: Outcome data from the state-wide neonatal quality assurance system from 2003 - 2004 from all infants treated in one of the five largest perinatal centers in Baden-Wuerttemberg were subtracted from the total dataset. Data derived from these five centers was compared with data from all other remaining NICU's in the state. RESULTS: Mortality was 33.3 % vs. 15.0 % (other NICU's vs. five perinatal centers; p < 0.001) for infants < 26 weeks GA, 11.4 % vs. 8.9 % (n. s.) for infants 26 - 27 weeks GA, and 2.5 % vs. 3.5 % (n. s.) for infants 28 - 31 weeks GA. When analyzed as one group of infants < 28 weeks GA, mortality was 20.1 vs. 12.1 % (p = 0.003). The rate of intraventricular hemorrhage degrees III-IV was 30.2 % vs. 18.6 % (p = 0.015) for infants < 26 weeks GA, 14.5 % vs. 10.2 % (n. s.) for infants 26 - 27 weeks GA, and 2.9 % vs. 2.5 % (n. s.) for infants 28 - 31 weeks GA. The rate of periventricular leukomalacia was 11.3 % vs. 6.7 % (p = 0.18) for infants < 26 weeks GA, 6.1 % vs. 2.8 % (n. s.) for infants 26 - 27 weeks GA, and 2.8 % vs. 2.3 %; (n. s.) for infants 28 - 31 weeks GA. CONCLUSION: This study supports the hypothesis, that regionalization of neonatal care for very immature infants to few perinatal centers with a large case load may improve survival of these infants, and may reduce morbidity, associated with long-term sequelae.

[Neonatal septicemia in the G-DRG system]. / Die Neugeborenensepsis in DRG-System.

INTRODUCTION: The introduction of Diagnosis Related Groups in Germany (G-DRG) has brought forward the obligation for physicians to take into account an intricate system of medical, economical and legal implementations. Mistakes in the process of encoding the principal diagnosis or procedures may have financial consequences. Problems to determine the correct ICD-code will be most prominent for diseases with poorly defined or even inconsistent diagnostic criteria as is the case for neonatal septicemia. We decided to evaluate whether the introduction of G-DRG resulted in a change of frequency of the diagnosis "neonatal septicemia". METHODS: We analysed data derived from the quality assurance program "Neonatalerhebung" in the state of Baden-Württemberg during the years of 2001 through 2004, i. e., 2 years before and 2 years during the introduction of G-DRG. During this period an annual number of 12,316 up to 13,172 newborns were admitted to the participating hospitals. RESULTS: The mean number of diagnoses per patient increased from 2.2 to 3.8. The frequency of the diagnosis of septicemia remained constant. The percentage of newborns receiving antibiotic therapy did not change. The ratio of cases with "septicemia yes" over "antibiotics yes" did not change. Although it is difficult to determine the diagnosis of neonatal septicemia and in spite of the economic implications of this diagnosis, no change in the frequency of this diagnosis occurred during the introduction of DRG. CONCLUSIONS: Assuming that the participating hospitals used an identical database for the quality assurance program "Neonatalerhebung" and for accounting, we conclude that the DRG system is stable with respect to neonatal septicemia.

Hypercontractile heart failure caused by catecholamine therapy in premature neonates.

UNLABELLED: Catecholamine treatment for hypotension is common practice in neonatal intensive care units. In the presence of left ventricular hypertrophy, the positive inotropic action of catecholamines with mainly beta-receptor specificity can lead to excessive hypercontractility and paradoxical hypotension. This reports presents two cases of hypercontractile heart failure during beta-agonist treatment in very low birthweight (VLBW) infants. Both patients (27 wk, 5 d; and 26 wk, 6 d of gestation) underwent surgical arterial duct ligation and coarctation repair. After operation they developed arterial hypotension that failed to respond to epinephrine (adrenaline) or dobutamine treatment. Echocardiography demonstrated a complete systolic obliteration of the left ventricular cavity. Epinephrine and dobutamine were stopped, and norepinephrine (noradrenaline) was successfully used to control the hypotension. CONCLUSION: Treatment of hypotension with beta-agonists in VLBW infants with left ventricular hypertrophy can lead to hypercontractility and left ventricular obliteration with paradoxical hypotension. Careful echocardiographic monitoring is indicated in such cases.

Vaginal delivery and neonatal outcome in extremely-low-birth-weight infants below 26 weeks of gestational age.

Outcomes of extremely-low-birth-weight infants (ELBW) with gestational age below 26 weeks based on mode of delivery (vaginal versus cesarean delivery) were retrospectively compared. During the observation period (1997 to 2000) 48 ELBW infants, below 26 weeks of gestational age, had been admitted to the Neonatal Intensive Care Unit (NICU) of the Department of Pediatrics, University of Freiburg, Germany. Twenty-seven (56%) patients were born vaginally and 21 (44%) by cesarean section. Birth weight, umbilical artery pH, and rectal temperature were significantly lower in the cesarean than in the vaginal group. Clinical Risk Index for Babies (CRIB) score showed significantly (p < 0.005) higher values in the cesarean group compared with the vaginal group. Hypothermia (rectal temperature below 36.2 degrees C after birth) was more common in the cesarean group (48%) than in the vaginal group (33%). Eighty-five percent of the fetuses in the vaginal group received antenatal corticosteroids and 88% in the cesarean group. Survival rate was significantly (p < 0.05) higher in infants born vaginally (78%) than in the cesarean group (43%). Several complications occurred less frequently after vaginal birth than after cesarean section: intraventricular hemorrhage grade III to IV (18 versus 33%); periventricular leukomalacia (4 versus 14%); and neonatal septicemia (33 versus 52%), but not statistical significant. In our study group, extremely immature preterm infants had a more favorable outcome if they were born vaginally when compared with infants delivered by cesarean section.

Sequential changes in compliance and resistance after bolus administration or slow infusion of surfactant in preterm infants.

OBJECTIVE: As bolus instillation of surfactant can lead to acute pulmonary, hemodynamic and cerebral side effects, we tested whether pulmonary mechanics and gas exchange differ between slow surfactant infusion and bolus administration. DESIGN AND SETTING: Prospective, randomized pilot study in a tertiary care university hospital. PATIENTS AND METHODS: Of 20 consecutive preterm infants (27-35 weeks' gestation) with severe respiratory distress syndrome) who were enrolled 14 with bovine surfactant finally were analyzed. INTERVENTIONS: Six treatments were administered by slow endotracheal surfactant infusion and eight as a bolus. Static compliance (C(stat)) and resistance (R(rs)) were measured every 3 min. RESULTS: C(stat) first decreased and then increased in both groups. In the infusion group C(stat) after 90 min was significantly higher than after bolus treatment but not after 15 or 45 min. R(rs) increased about threefold, with large fluctuations in the bolus group. After 90 min PaO(2)/FIO(2) had increased from 111+/-44 to 254+/-69 in the bolus group and from 86+/-40 to 238+/-102 in the infusion group, but early FIO(2) reduction and increase in PaO(2)/FIO(2) seemed delayed in the infusion group. CONCLUSIONS: Very slow infusion of natural surfactant is at least as effective as bolus instillation in terms of improvement in C(stat) and oxygenation after 90 min. However, until 90 min the course of C(stat) and indices of gas exchange seem superior after bolus therapy. Because R(rs) is substantially increased, long expiratory times are required to yield complete exhalation.

[Catheter-related thrombosis and its prevention in infancy]. / Katheterthrombosen im Kindesalter und ihre Prävention.

Vascular catheters are the most important cause of thromboembolism in neonates. Additionally, a concomittant genetic predisposition with the well-known mutations is often present. In order to understand the pathology, knowledge about the specific condition of the newborn is important, especially concerning the altered hemostatic balance. For indications the specific situation of the very small infant is to be considered, e. g. the increased risk of cerebral bleeding in the first days of life of prematures. Therefore, careful consideration of indication and contraindications is mandatory. To avoid catheter-related thrombosis different schedules of prophylaxis are well-tried, especially low dose heparinization (sometimes as continuous infusion) is recommended for different types of vascular access. In neonates specific organ-related complications of umbilical catheters are to be considered. For the early diagnosis of catheter-related thrombosis, attention should be given to its first signs. Thrombolytic therapy is worth to be considered. However, the state of the patient and the dynamic of thrombus growth must be taken into account.

Airway compression following thymus hyperplasia in an infant with Pierre Robin sequence and congenital diaphragmatic hernia.

A female infant with respiratory embarrassment due to external compression of the tracheobronchial tree by the thymus is presented. After successful intrauterine drainage of pleural effusions, she was born with a diaphragmatic hernia and Pierre Robin sequence, which required long-term mechanical ventilation and several surgical interventions, including tracheostomy, until she breathed spontaneously. At age 7 months, she was rehospitalized in respiratory failure because of pneumonia. At this time, thymus hyperplasia was first diagnosed, which had caused tracheobronchomalacia and displacement of the tracheobronchial tree. Weaning from the ventilator was unsuccessful for 8 weeks. She underwent thymectomy, following which impairment of breathing resolved immediately. Different manifestations of thymus enlargement and their relation to impaired breathing are discussed.

Cord blood levels of interleukin-6 and interleukin-8 for the immediate diagnosis of early-onset infection in premature infants.

BACKGROUND: Cytokine plasma levels are suggested to be sensitive indicators of neonatal sepsis, but conventional assays are time consuming. This study aimed at evaluating the significance of cord blood levels of interleukin (IL)-6 and IL-8 determined by a fully automated random access assay within 90 min of admission to predict systemic bacterial infection. PATIENTS AND METHODS: Cord blood levels of IL-6 and IL-8 were determined in 71 mature and 100 premature infants by a chemiluminescence assay (Immulite). Patients were divided into four groups according to a clinical and laboratory scoring system. Group A: documented early-onset infection; group B: infection possible; group C: infection unlikely, and group D: healthy newborns. RESULTS: Median IL-6 levels in the subgroup of premature newborns were as follows: group A, 1,920 pg/ml (5-95% confidence interval 308-4,660 pg/ml); group B, 50 (15-102) pg/ml; group C, 21 (12-71) pg/ml, and group D, 8 (6-11) pg/ml. For IL-8, median levels for groups A-D were 289 (226-514) pg/ml, 87 (40-107) pg/ml, 44 (33-98) pg/ml and 21 (16-25) pg/ml, respectively. The difference between group A and the other groups was highly significant (IL-6 p < 0.0001, IL-8 p < 0.001). At a cut-off of 80 pg/ml, the sensitivity of IL-6 for the diagnosis of sepsis was 96% (specificity 95%). For IL-8 (cut-off 90 pg/ml), the sensitivity was 87% (specificity 94%). CONCLUSION: In premature infants, the diagnosis of an early-onset infection can be established or ruled out with a high level of confidence by measuring IL-6 or IL-8 levels from cord blood using a random access chemiluminescence assay.

Effect of caffeine on oxygen consumption and metabolic rate in very low birth weight infants with idiopathic apnea.

OBJECTIVE: Methylxanthines are among the most commonly prescribed drugs in neonatal intensive care. This study evaluates the effect of caffeine on oxygen consumption and metabolic rate in premature infants with idiopathic apnea. METHODS: Eighteen preterm infants at gestational ages from 28 to 33 weeks and birth weights of 890 to 1680 g were enrolled in the study. Nine preterm infants received caffeine therapy, and 9 served as a control group. Oxygen consumption and energy expenditure were examined before, during, and after caffeine treatment. RESULTS: Oxygen consumption increased significantly from 7.0 +/- 0.9 before caffeine to 8.8 +/- 0.7 mL/kg/min after 48 hours of caffeine therapy, and energy expenditure increased from 2.1 +/- 0.3 to 3.0 +/- 0.2 kcal/kg/hour. During the observation period of 4 weeks of caffeine treatment, oxygen consumption increased significantly in the caffeine group compared with the control patients. In the caffeine group, a lower environmental temperature was sufficient to maintain a normal body temperature. With similar caloric intake in both groups during the study period, daily weight gain in the control group was significantly higher (21 +/- 4 vs 42 +/- 2 g/d). None of the other parameters recorded changed during caffeine therapy. CONCLUSION: Long-term administration of caffeine in preterm infants is associated with an increase in oxygen consumption and with a reduction of weight gain. This may have implications for clinical practice as nutritional regimens need to be adjusted during this therapy.

Physical effects of heliox versus oxygen on measurements of functional residual capacity by the nitrogen washout technique in small lung volumes: a model study.

Measurements of functional residual capacity (FRC) by the nitrogen (N(2)) washout technique yield low N(2) signals in neonates and preterm infants, especially when they are on high fractions of inspired oxygen (FiO(2)). Thus, recorded values often lie in the low range of detectability. We hypothesized that using heliox instead of oxygen as a washout gas would affect the electric discharge conditions of N(2) molecules in a standard UV analyzer and thus yield higher N(2) signals. We performed three laboratory experiments using conditions similar to those in neonates with pulmonary disease, reproducing different initial FiO(2) values and very small lung volumes. Standard calibration procedures with physical models between 13.5-87 mL using a calibration syringe and purpose-built small calibration cylinders were carried out, and washout gas was either pure oxygen (as is general practice) or heliox at different ratios. We observed that the calibration line with heliox was shifted upwards and the slope was increased, depending on helium concentration and initial FiO(2). Since this effect was dose-dependent with respect to the proportion of helium in the washout gas, this strongly suggests a physical process elicited by the noble gas helium. We conclude that the heliox wash-out modification may help to increase the accuracy of FRC measurements and thus may enable studies of smaller patients or patients on high inspired FiO(2).