Johannes Wirbelauer

Kongenitale portosystemische Shunts sind seltene Malformationen der viszeralen Venen, die in einer Vielzahl anatomischer Varianten dem Systemkreislauf portalvenöses Blut unter Umgehung der Leber zuführen. Die Erstbeschreibung erfolgte durch Abernethy im Jahr 1793. Bei einer geringen Inzidenz mit nur 136 in der Literatur beschriebenen Fällen (Kobayashi N et al. Hepatology Research 2010; 40: 585 – 593) werden für kongenitale extrahepatische portosystemische Shunts (CEPS) verschiedene anatomische Einteilungen verwendet. Diese werden mit zum Teil recht unterschiedlicher Prognose und Häufigkeit begleitender Fehlbildungen und Syndrome sowie Art und Zeitpunkt einer therapeutischen Intervention assoziiert (Blanc T et al. Ann Surg 2014; 260: 188 – 198). Wesentlich häufiger als beim Menschen werden portosystemische Shunts in der Veterinärmedizin mit einer Inzidenz bis zu 3 % bei bestimmten Hunderassen beobachtet (Tobias et al. J Am Vet Med Assoc 2003; 223: 1636 – 1639).

Introduction: Late vitamin K deficiency bleeding in young infants is a rare disorder which occurs almost exclusively in breast-fed infants who did not receive proper vitamin K prophylaxis at birth and who might additionally suffer from cholestasis. Its impact on morbidity is high since in 50% of the cases it presents with intracranial hemorrhage with a mortality rate of 20% and life-long neurologic sequelae in 30% of the affected infants. Case reports: 2 male infants were both admitted to our unit at the age of 5 weeks with subdural hematoma with midline shift due to late vitamin K deficiency bleeding. Both infants did not receive the recommended Vitamin K prophylaxis in Germany. One patient presented with cholestatic jaundice on admission as an additional risk factor. Discussion: Parents who in the apparent best interest for their children refuse the recommended and well established vitamin K prophylaxis at birth leading to the reappearance of late vitamin K deficiency bleeding. These parents also tend to refuse routine immunizations of childhood in later life, which not only have an impact on their own child but might bear a risk for the whole community. Conclusion: It is the responsibility of health-care takers to show increased awareness to the growing number of parents refusing vitamin K prophylaxis at birth and educate them properly about the devastating consequences of late vitamin K deficiency bleeding. Hintergrund: Die späte Vitamin-K-Mangelblutung tritt sehr selten auf. Betroffen sind in der Regel ausschließlich gestillte Säuglinge, die entweder keine oder nur eine unzureichende Vitamin-K-Prophylaxe bei Geburt erhalten haben und/oder bei denen zusätzlich eine Cholestase besteht. In 50% der Fälle manifestiert sich die Erkrankung mit einer Hirnblutung. Es besteht dann eine Mortalitätsrate von 20% und neurologische Defizite treten bei 30% der betroffenen Kinder auf. Patienten: 2 männliche Säuglinge wurden beide im Alter von 5 Wochen jeweils mit einem raumfordernden Subduralhämatom aufgrund einer späten Vitamin-K-Mangelblutung auf unsere Intensivstation aufgenommen. Beide Säuglinge hatten nicht die in Deutschland empfohlene Vitamin-K-Prophylaxe erhalten. Bei einem Patienten bestand bei Aufnahme als zusätzlicher Risikofaktor ein cholestatischer Ikterus. Diskussion: Eltern, die die etablierte Vitamin-K-Prophylaxe bei Geburt verweigern, lassen im Weiteren auch häufiger die Routineimpfungen des Kindesalters nicht durchführen. Dieses Vorgehen mag nicht nur eine potentiell vermeidbare Auswirkung – wie im Fall der Vitamin-K-Mangelblutung – auf das eigene Kind haben, sondern birgt auch ein Erkrankungs- und Infektionsrisiko für die Gesellschaft in sich. Zusammenfassung: Es liegt in der Verantwortung von Hebammen, Geburtshelfern und Kinderärzten, die steigende Anzahl der Eltern, die eine Vitamin-K-Prophylaxe verweigern, zu identifizieren und hinreichend aufzuklären, um ein gehäuftes Wiederauftreten der späten Vitamin-K-Mangelblutung zu vermeiden.

The epidural route is still considered the gold standard for labour analgesia, although it is not without serious consequences when incorrect placement goes unrecognized, e.g. in case of intravascular, intrathecal and subdural placements. Until now there has not been a viable alternative to epidural analgesia especially in view of the neonatal outcome and the need for respiratory support when long-acting opioids are used via the parenteral route. Pethidine and meptazinol are far from ideal having been described as providing rather sedation than analgesia, affecting the cardiotocograph (CTG), causing fetal acidosis and having active metabolites with prolonged half-lives especially in the neonate. Despite these obvious shortcomings, intramuscular and intravenously administered pethidine and comparable substances are still frequently used in delivery units. Since the end of the 90ths remifentanil administered in a patient-controlled mode (PCA) had been reported as a useful alternative for labour analgesia in those women who either don’t want, can’t have or don’t need epidural analgesia. In view of the need for conversion to central neuraxial blocks and the analgesic effect remifentanil has been demonstrated to be superior to pethidine. Despite being less effective in terms of the resulting pain scores, clinical studies suggest that the satisfaction with analgesia may be comparable to that obtained with epidural analgesia. Owing to this fact, remifentanil has gained a place in modern labour analgesia in many institutions. However, the fact that remifentanil may cause harm should not be forgotten when the use of this potent mu-agonist is considered for the use in labouring women. In the setting of one-to-one midwifery care, appropriate monitoring and providing that enough experience exists with this potent opioid and the treatment of potential complications, remifentanil PCA is a useful option in addition to epidural analgesia and other central neuraxial blocks. Already described serious consequences should remind us not refer to remifentanil PCA as a “poor man’s epidural” and to safely administer remifentanil with an appropriate indication. Therefore, the authors conclude that economic considerations and potential cost-savings in conjunction with remifentanil PCA may not be appropriate main endpoints when studying this valuable method for labour analgesia.

Background. Pulmonary capillary hemangiomatosis (PCH) is a rare disease, especially in infancy. Four infants have been reported up to the age of 12 months. So far, no familial patients are observed at this age.Patients. We report three siblings, two female newborns and a foetus of 15-week gestation of unrelated, healthy parents suffering from histologically proven PCH. The first girl presented with increased O₂requirements shortly after birth and patentductus arteriosus(PDA). She subsequently developed progressive respiratory failure and pulmonary hypertension and died at the age of five months. The second girl presented with clinical signs of bronchial obstruction at the age of three months. The work-up showed a PDA—which was surgically closed—pulmonary hypertension, and bronchial wall instability with stenosis of the left main bronchus. Transient oxygen therapy was required with viral infections. The girl is now six years old and clinically stable without additional O₂requirements. Failure to thrive during infancy and a somewhat delayed development may be the consequence of the disease itself but also could be attributed to repeated episodes of respiratory failure and a long-term systemic steroid therapy. The third pregnancy ended as spontaneous abortion. The foetus showed histological signs of PCH.Conclusion. Despite the differences in clinical course, the trias of PCH, PDA, and pulmonary hypertension in the two life born girls suggests a genetic background.

Plastic bronchitis is characterized by the presence of large, thick, mucofibrinous plugs filling the bronchopulmonary tree. This rare condition has been described in children with univentricular palliation of cardiac defects [Verghese S et al., Anesth Analg 2008;107:1446–1447] and in patients with chronic pulmonary diseases such as bronchial asthma and cystic fi­brosis. A potentially life-threatening com­plication of the disease is obstruction of airways, particularly of the trachea.

Objectives. To test the hypothesis if very immature preterm infants exposed to chorioamnionitis would exhibit increased numbers of leukocytes, neutrophils, and nucleated red blood cells (NRBC) in peripheral blood. Study design. Preterm infants with birth weight Results. Birth weight, gestational age, and sex did not differ between the groups (n = 71). Seventeen infants who were exposed to chorioamnionitis had significantly higher counts of leukocytes, neutrophils, and immature neutrophils after birth. However, there was no difference in the number of circulating NRBCs between both groups. In contrast, there was a tendency towards an increased NRBC count in the control group. Conclusion. Preterm infants exposed to chorioamnionitis elicited a strong inflammatory response as reflected by increased numbers of leukocytes and neutrophils. However, chorioamnionitis did not induce an increase in numbers of NRBC.

It is unclear if very immature preterm infants who are born small for gestational age (SGA) have similar leukocyte counts as infants who are born appropriate for gestational age (AGA). Our study included 49 preterm infants with a gestational age ≤32 weeks and without exposure to chorioamnionitis and funisitis. Blood cells were counted in the first 2 hours of life. Eighteen SGA preterm infants were compared with 31 AGA preterm infants. Gestational age, sex, rate of caesarean section, and prenatal administration of corticosteroids did not differ between the groups. Median birth weight was 583 g in the SGA group versus 1100 g in the AGA group. Infants in the SGA group had significantly lower counts of leukocytes, total neutrophils, immature neutrophils, lymphocytes, and monocytes. These findings were not affected by maternal preeclampsia. No significant difference for nucleated red blood cell counts was found. Prenatal growth retardation is an independent factor for lower counts of different leukocytes in very immature preterm infants. It is not clear if these low leukocyte counts are associated with a higher risk of neonatal infections or if lower numbers of inflammatory cells protect the lung and brain of very immature SGA infants by reducing inflammatory events postnatally.