Single-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt.

OBJECTIVES: To assess the spectrum of underlying pathologies, the intrauterine course and postnatal outcome of 46 fetuses with megacystis that underwent intrauterine vesico-amniotic shunting (VAS) with the Somatex® shunt in a single center. METHODS: Retrospective analysis of 46 fetuses with megacystis that underwent VAS either up to 14 + 0 weeks (early VAS), between 14 + 1 and 17 + 0 weeks (intermediate VAS) or after 17 + 0 weeks of gestation (late VAS) in a single tertiary referral center. Intrauterine course, underlying pathology and postnatal outcome were assessed and correlated with the underlying pathology and gestational age at first VAS. RESULTS: 46 fetuses underwent VAS, 41 (89%) were male and 5 (11%) were female. 28 (61%) fetuses had isolated and 18 (39%) had complex megacystis with either aneuploidy (n = 1), anorectal malformations (n = 6), cloacal malformations (n = 3), congenital anomalies overlapping with VACTER association (n = 6) or Megacystis-Microcolon Intestinal-Hypoperistalsis Syndrome (MMIHS) (n = 2). The sonographic 'keyhole sign' significantly predicted isolated megacystis (p < 0.001). 7 pregnancies were terminated, 4 babies died in the neonatal period, 1 baby died at the age of 2.5 months and 34 (74%) infants survived until last follow-up. After exclusion of the terminated pregnancies, intention-to-treat survival rate was 87%. Mean follow-up period was 24 months (range 1-72). The underlying pathology was highly variable and included posterior urethral valve (46%), hypoplastic or atretic urethra (35%), MMIHS or prune belly syndrome (10%) and primary vesico-ureteral reflux (2%). In 7% no pathology could be detected postnatally. No sonographic marker was identified to predict the underlying pathology prenatally. 14 fetuses underwent early, 24 intermediate and 8 late VAS. In the early VAS subgroup, amnion infusion prior to VAS was significantly less often necessary (7%), shunt complications were significantly less common (29%) and immediate kidney replacement therapy postnatally became less often necessary (0%). In contrast, preterm delivery ≤ 32 + 0 weeks was more common (30%) and survival rate was lower (70%) after early VAS compared to intermediate or late VAS. Overall, 90% of liveborn babies had sufficient kidney function without need for kidney replacement therapy until last follow-up, and 95% had sufficient pulmonary function without need for mechanical respiratory support. 18% of babies with complex megacystis suffered from additional health restrictions due to their major concomitant malformations. CONCLUSIONS: Our data suggest that VAS is feasible from the first trimester onward. Early intervention has the potential to preserve neonatal kidney function in the majority of cases and enables neonatal survival in up to 87% of cases. Despite successful fetal intervention, parents should be aware of the potential of mid- or long-term kidney failure and of additional health impairments due to concomitant extra-renal anomalies that cannot be excluded at time of intervention.

[Challenges in neonatal dermatology : An introduction]. / Besonderheiten und Herausforderungen der neonatalen Dermatologie : Eine Einführung in die Problematik.

In order to support dermatologists' interest for the fascinating area of neonatal dermatology, we provide (1) an introduction to the specifics of skin barrier in premature and full-term neonates as well as their clinical implications and (2) an example of age-dependent differential diagnoses and approach to a facial vascular stain in a neonate.

Use and Cutaneous Side Effects of Skin Antiseptics in Extremely Low Birth Weight Infants - A Retrospective Survey of the German NICUs.

UNLABELLED: Background Nosocomial infections are a serious problem in the treatment of extremely low birth weight infants (ELBW, <1 000 g). In these patients, effective skin antisepsis is critical to prevent hospital-acquired infections and their sequelae. However, serious side effects of topical antiseptics have been repeatedly reported in extremely preterm infants and no agreement has been reached on the best product in this population. Therefore, we conducted a survey of the German NICUs aiming to investigate current practices and safety of topical antiseptics in ELBW neonates. METHODS: We sent anonymized questionnaires to 166 German NICUs with the highest level of care. RESULTS: Usable questionnaires were returned by 64 NICUs (39%). These NICUs had treated a total of 2130 patients with a birth weight<1 000 g in 2012. Octenidine without phenoxyethanol (OwPh) and Octenisept(®) were the predominantly used skin antiseptics for intensive-care procedures. At least one skin complication was reported by 27% (n=17) of the NICUs. In 9 cases Octenisept(®) was used, and in 6 cases octenidine was used. CONCLUSIONS: According to our knowledge, this is the first study surveying practices and safety of skin antisepsis in ELBWs in the German NICUs. Most German NICUs use octenidine, however, in different preparations. Skin complications including blistering, necrosis and scarring were seen with all octenidine products, a fact which has not been previously reported.

Neonatal opiate withdrawal and rooming-in: a retrospective analysis of a single center experience.

AIM: To evaluate our treatment of neonatal abstinence syndrome (NAS), our experience with rooming-in of opiate-dependent mothers and to examine the influence of rooming-in on short term outcome of infants exposed to opiates in utero. METHOD: Retrospective analysis of maternal and perinatal data of newborn infants with NAS treated between 2004 and 2011 in a level 3 academic children's hospital in a German metropolis. Therapy of NAS and duration of therapy, length of hospital stay and costs were considered in particular. FINDINGS: Data of 77 newborns with NAS were analysed. 84.6% of infants were treated with tincture of opium (79.2% rooming-in, 88.7% no rooming-in). Infants with rooming-in (n=24) had a 17% shorter median duration of therapy [27.0 d (IQR 24.0-38.5), no rooming-in (n=53) 32.5 d (IQR 25.0-54.5)] and shorter median length of hospital stay [33.0 d (IQR 28.0-48.0), no rooming-in 41.5 d (IQR 30.3-54.5)]. Demographic data was comparable between newborns and mothers with or without rooming-in. Costs were median 13 457 € (IQR 8 967-17 494)/patient [rooming-in: 9 547 € (IQR 7 024-16 135), no rooming-in: 14 486 € (IQR 9 479-19 352)]. CONCLUSIONS: Rooming-in in NAS should be encouraged to shorten duration of therapy and length of hospital stay and thereby reduce costs. No major problems arose in the care of the infants with NAS when parents stayed with their infants but close monitoring of the newborn and strict instruction of parents are required.

Observations on the effects of inhaled isoflurane in long-term sedation of critically Ill children using a modified AnaConDa©-system.

Long-term intravenous sedation may present problems due to dependence and side effects. Medical records of children who were administered isoflurane were reviewed. 15 patients (9 boys, 6 girls) with a mean age of 11.8 month (+2.4) were analysed.Analgesia and sedation was given in mean 9.7+1.1 days before commencing inhalation using a modified application device (AnaConDa©). Administration was given over a period of 7.2+1.4 days. Depth of sedation was monitored by using Comfort- and Hartwig-scores. Observations included continuous monitoring of heart-rate, pulse oxymetry, blood pressure and cerebral tissue oxygenation.Within 4 h post administration of isoflurane a satisfactory increase in the depth of sedation was seen and kept till extubation. 6/15 patients received tracheostomies during the observation period. None of the patients observed suffered life-critical events of the modified application of isoflurane proceeded without complications. Ketamine and clonidine infusion rates were significantly reduced (p<0.005) as well as the use and overall infusion rate of midazolam, γ-hydroxy butyrate, fentanyl and morphine (p<0.05).Isoflurane inhalation may provide an additional option for long-term sedation in a specific group of critically ill infants but neurodegenerative toxic effects will have to be taken into account when using volatile anesthetics at any time during infancy.

Postnatal surgical treatment and complications following intrauterine vesicoamniotic shunting with the SOMATEX® intrauterine shunt. A single center experience.

INTRODUCTION: Intrauterine vesicoamniotic shunting (VAS) using a Somatex® shunt was shown to significantly affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO) [Figure 1]. Data on postnatal surgical management and complications are largely lacking. OBJECTIVE: To describe the postnatal management of patients with prenatal VAS for megacystitis in suspected severe LUTO. STUDY DESIGN: All male newborns with previous intrauterine VAS using a Somatex® shunt treated in our institution were retrospectively analyzed. We evaluated the spectrum of urethral pathologies and postnatal surgical management, especially focusing on shunt removal. RESULTS: Between 2016 and 2022, 17 patients (all male) were treated postnatally in our institution after VAS for suspected severe LUTO. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in eight patients (8/17). Seven shunts could be removed without further anesthesia as a bedside procedure. Ten patients required surgical shunt removal under general anesthesia due to migration (59%). Laparoscopic shunt extraction was performed in 8/10 cases. Most frequently, dislocated shunts were located incorporated in the detrusor in eight cases and the removal required a bladder suture in 2/8 patients. In one case, the shunt was removed from the abdominal wall and in one case from the intestine wall [Figure 2]. Posterior urethral valves were found in 8/17 patients, 6/17 patients showed a urethral atresia and one patient had urethral duplication. In two patients, we identified a high grade bilateral vesicoureteral reflux without LUTO. CONCLUSION: In our observation, more than half of the newborns with megacystis in suspected LUTO require a shunt removal surgery after early VAS using a Somatex® shunt. Urethral atresia may be found more frequently in these patients. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

Surfactant in spontaneous breathing with nCPAP: neurodevelopmental outcome at early school age of infants ≤ 27 weeks.

AIM: In 2001, we implemented the 'surfactant without intubation' (SWI) method to administer surfactant during spontaneous breathing with nasal continuous positive airway pressure in preterm infants. To determine whether application of the SWI method in infants born at ≤ 27 weeks gestational age influences early school age outcome. METHODS: A historical control cohort was compared to an interventional cohort, when the SWI method had become part of primary respiratory support. According to the overall results of psychometric and neurological examinations, children were classified as showing no functional impairment, minor or major impairments. RESULTS: Out of 79 surviving children, 54 (68%, two children not completely classifiable) were assessed at a median age of 6 5/12 years [25 (32%) lost to follow-up]. Thirteen of 31 (42%, interventional cohort) vs. 8 of 21 (38%, control cohort) children had no functional impairment, 16 (52%) vs. 9 (43%) showed minor, and 2 (7%) vs. 4 (19%) major impairments. CONCLUSION: There was no statistically significant difference regarding neurodevelopmental school age outcome in children treated after implementing the SWI procedure, even though surviving infants had been more immature and survival rates had increased.

Comparison of sufentanil versus fentanyl in ventilated term neonates.

BACKGROUND: Increasingly frequent applications of opioid analgesics in neonatal intensive care require the evaluation of efficacy and side effects. PATIENTS: Mechanically ventilated term neonates were consecutively enrolled. METHODS: In a double-blind randomized trial 20 newborns received a continuous intravenous infusion of fentanyl (n=10) or sufentanil (n=10) in an assumed equipotent dose of 7:1. The analgesic dose was individually adjusted according to sedation scores. The period between cessation of analgesic medication and successful extubation (weaning time), adverse drug effects and urinary cortisol concentrations were evaluated. RESULTS: No significant difference of weaning time was seen between fentanyl and sufentanil group (mean weaning time (+/-SD) of fentanyl group 520+/-381 min, median 380 min; sufentanil group 585+/-531 min, median 405 min, p=0.78, 2-tailed U-Test, Mann and Whitney). The mean opioid dose resulted in a 10:1 ratio (fentanyl 4.11 microg/(kg x h) vs sufentanil 0.41 microg/(kg x h)). We found no marked differences in sedation levels, blood pressure, heart rate, oxygenation index, co-medication or urinary cortisol levels. In both groups similar adverse effects were assessed including respiratory depression, mild withdrawal symptoms or decrease of gastrointestinal motility. CONCLUSION: In our study sufentanil did not reduce the weaning period in ventilated term neonates when compared to fentanyl. The equipotent dose ratio for fentanyl/sufentanil was 10:1. According to sedation scores both substances provided effective pain and stress protection.

Rapid development of life-threatening complete atrioventricular block in Kearns-Sayre syndrome.

Kearns-Sayre syndrome is a rare mitochondrial disorder with defined diagnostic criteria. Knowledge of these diagnostic criteria and early diagnosis are important to ensure periodic electrocardiograms for identification of cardiac conduction disorders, which are the most important prognostic factor of the disease. We report on a 9-year-old girl with rapid development of a life-threatening complete atrioventricular block within 10 months and discuss the importance and time interval of regular electrocardiograms. Our patient survived by placing a temporary transvenous pacemaker lead followed by permanent pacemaker implantation a few days later.

Remifentanil for INSURE in preterm infants: a pilot study for evaluation of efficacy and safety aspects.

AIM: To evaluate intubating conditions, extubation times and outcome in preterm infants receiving remifentanil as induction agent for the INSURE procedure. METHODS: In twenty-one preterm infants of 29 to 32 weeks gestation and signs of respiratory distress, we utilized remifentanil as induction agent for the INSURE procedure. Following intubation and surfactant application, the infants were mechanically ventilated until respiratory drive was judged to be satisfactory for continuing CPAP therapy. Intubating conditions were classified by our own scoring system by rating limb movements, coughing and breathing. Heart rate, blood pressure and oxygen saturation were recorded during the entire INSURE procedure. RESULTS: Remifentanil provided excellent or good intubating conditions in all patients. We observed no serious side effects after remifentanil infusion, in particular, no thorax rigidity, clinically significant bradycardia or arterial hypotension. Average extubation time after surfactant administration was 16.9 min (1-45 min); none of the infants had to be reintubated. Following extubation, the infants required only 3.3 days (1-8 days) of CPAP therapy. None exhibited serious complications of prematurity like periventricular leucomalacia, intraventricular haemorrhage >I degree, necrotizing enterocolitis or retinopathy. CONCLUSION: In this pilot study, INSURE with remifentanil was associated with good intubating conditions and early extubation resulting in an excellent neonatal outcome.

[Primary ciliary dyskinesia causing neonatal respiratory distress]. / Primäre Ziliendyskinesie als Ursache neonataler Atemnot.

BACKGROUND: Primary ciliary dyskinesia (PCD) is a hereditary disorder of structure and function of the cilia of respiratory epithelium of the upper and lower airways. Prevalence is estimated with 1:15 000 to 1:30 000 births. We present a newborn infant with respiratory distress caused by PCD. PATIENT: On the first day of life, the male newborn developed dyspnoe and cyanosis, so that CPAP and short term ventilation was necessary. Varying atelectasis impressed on the chest radiographs and the diagnosis of PCD was made by nasal brush biopsies. Causative is a lack of the inner dynein arms of the cilia. The clinical features of newborns with the diagnoses of PCD are listed and compared with the own case. CONCLUSION: PCD is a rare cause of neonatal respiratory distress and should be considered in term infants with unknown and prolonged course even if Situs inversus is lacking.

Recombinant activated factor seven in acute life-threatening bleeding in neonates: report on three cases and review of literature.

INTRODUCTION: Acute bleeding of different genesis can be a severe, life-threatening problem in neonatology. Recombinant factor seven (rFVIIa) is known to have unique hemostatic properties in adults and older children. CASE PRESENTATION: Three cases of acute life-threatening peri- and postnatal hemorrhage were successfully controlled after the application of fFVIIa. All infants were first treated with vitamin K, fresh-frozen plasma and platelet transfusion. CONCLUSION: The cases substantiate other reports that rFVIIa is an effective treatment for acute, refractory and life-threatening bleeding in neonates and premature infants.

Severe primary cutaneous aspergillosis refractory to amphotericin B and the successful treatment with systemic voriconazole in two premature infants with extremely low birth weight.

Primary cutaneous aspergillosis is a rare, life-threatening, infectious complication in premature infants that may result in fulminant sepsis and subsequent multi-organ failure. In the past decade, the incidence of primary aspergillosis has increased significantly, whereas the high morbidity and mortality of invasive aspergillosis remains unaltered. In vitro studies reveal that more and more Aspergillus species seem to be refractory to the classical treatment with fluconazole or amphotericin B. This case report presents two extremely low birth weight infants (ELBW) with primary cutaneous aspergillosis, which was refractory to amphotericin B. Both patients were successfully treated with systemic voriconazole, an extended-spectrum triazole antifungal, supported by topical care. This paper provides the clinical manifestation, diagnostics and pharmacotherapy of primary cutaneous aspergillosis, as well as pharmacokinetic aspects of voriconazole in ELBW infants.

Mothers seeing their VLBW infants within 3 h after birth are more likely to establish a secure attachment behavior: evidence of a sensitive period with preterm infants?

OBJECTIVE: Close contact of mother and child in the first hours after birth is essential for the establishment of a secure attachment behavior in term infants. To date, studies investigating whether a 'sensitive period' also exists for very low birth weight (VLBW) preterm infants are lacking. STUDY DESIGN: Attachment patterns of 62 VLBW infants were assessed using the 'strange situation' setting and correlated with the time mothers saw their child for the first time. Furthermore, maternal and infant covariates possibly influencing the attachment behavior were analyzed. As maternal factors the mother's age, social status and pregnancy history were recorded and at three time points (time 1, 2 and 3 (t-1, t-2 and t-3)), a semi-structured interview, a depression and a social support questionnaire were performed. As infant factors neonatal basic data, ventilation time and length of hospital stay were recorded. Disease severity was scored using the clinical risk index for babies, score for neonatal acute physiology (SNAP), SNAP perinatal extension and nursery neurobiological risk score. At time points t-2 and t-3, the infants were examined using the second edition of Bayley scales of infant development. RESULTS: In all, 53.2% of the children showed a secure, 33.9% an insecure-avoidant, 3.2% an insecure-ambivalent and 9.7% an insecure-disorganized attachment behavior. Preterm infants whose mothers had seen them within 3 h after birth had a higher rate of secure attachment than preterm infants with no early contact (76 versus 41%, P=0.009). Firstborns showed a significantly higher rate of insecure attachment behavior (93 versus 67%, P=0.01). No influence on attachment behavior was shown for any other maternal or infant factor. CONCLUSIONS: Our results support the hypothesis that the first hours after birth are a 'sensitive period' for the development of attachment behavior in VLBW infants. When a mother is enabled to see her infant shortly after birth, the 'sensitive period' right after birth may be used to help forming an important basis for the secure attachment of the preterm infant.

Polymorphisms in the Renin-Angiotensin system and outcome of very-low-birthweight infants.

BACKGROUND: The insertion/deletion polymorphism of the angiotensin-converting enzyme (ACE-ins/del) and the angiotensin II type 1 receptor 1166A/C polymorphism (ATR1166A/C) were reported to be associated with several unfavorable outcome parameters in preterm infants like bronchopulmonary dysplasia, persistent ductus arteriosus and impaired insulin sensitivity. OBJECTIVE: To confirm the above-mentioned associations in a large cohort of very-low-birthweight (VLBW) infants. METHOD: Clinical data of VLBW infants were prospectively recorded. The ACE-ins/del polymorphism and the ATR1166A/C polymorphism were determined by polymerase chain reaction in 1,209 and 1,168 infants, respectively. RESULTS: There was no significant association between ACE-ins/del or ATR1166A/C genotype and outcome parameters (death, intraventricular hemorrhage, sepsis, bronchopulmonary dysplasia, ventilation, supplemental oxygen at discharge, postnatal treatment with insulin, surgery for intestinal perforation/necrotizing enterocolitis/retinopathy of prematurity/persistent ductus arteriosus. CONCLUSION: Both known functional polymorphisms of the renin-angiotensin system do not seem to be associated with the outcome of VLBW infants.

Aerosolized iloprost in the treatment of pulmonary hypertension in extremely preterm infants: a pilot study.

BACKGROUND: In premature infants with preterm prolonged rupture of membranes, death after birth is often due to persistent pulmonary hyper-tension. PATIENTS: Aerosolized iloprost was used to treat pulmonary hypertension due to prolonged preterm rupture of fetal membranes (7-56 days) in four extremely low-birthweight neonates (23-25 weeks' gestation, weight 448-645 g) under spontaneous breathing supported by nasal continuous positive airway pressure. METHOD: Inhalation dose was 2 microg/kg b.w. and between 44 and 65 inhalations were performed in each patient starting within the first hour of life over a total of several days. Single inhalations lasted 5 min and were not repeated until 60 min had elapsed. RESULTS: After the first inhalation, the PaO2/FiO2 mean ratio increased from 65 (range 35-114) to 194 (148-250) mmHg and oxygenation requirements decreased within the next 7 days. Echocardiography similarly showed reduction in pulmonary resistance. We observed no severe side effects on blood pressure or prolonged bleeding time during inhalation. CONCLUSIONS: Iloprost inhalation might therefore be an additional treatment for improving oxygenation in cases of persistent pulmonary hypertension in extremely low-birthweight infants under spontaneous breathing. Further randomized clinical studies are required to establish the role of iloprost in this setting.