[Pierre Robin Sequence: interdisciplinary treatment after prenatal diagnosis]. / Pierre Robin Sequenz: Pränatale Diagnostik und interdisziplinäre Therapie.

BACKGROUND: Pierre Robin Sequence (PRS) is characterised by mandibular micrognathia and/or retrognathia, glossoptosis and upper airway obstruction (UAO). In severe cases, UAO and cyanosis occur immediately after birth and endoscopic intubation may become necessary. Therefore, prenatal diagnosis with referral to a specialized department is important. METHOD: A non-invasive interdisciplinary treatment protocol is presented. The postnatal adjustment of the preepiglottic baton plate (PEBP) as early as possible is essential in this concept. EVIDENCE: In a randomised cross-over trial with 11 patients with isolated PRS, the PEBP was found to reduce the apnoea index significantly and to be superior to a conventional palatal plate. An uncontrolled longitudinal study indicated that the UAO had normalised 3 months after discharge; all infants showed adequate weight gain with bottle feeding. In a comparative study with 34 healthy children and 34 children with isolated PRS, no significant differences in cognitive outcome was found. CONCLUSIONS: Interdisciplinary co-operation between prenatal care, neonatology and orthodontics is a pre-requisite for optimal postnatal therapy. Complications of UAO can be avoided by early and adequate treatment, resulting in good results for feeding, speech and facial profile. Invasive surgical treatment options like tongue-lip-adhesion, mandibular extension or distraction should be obsolete.

[Treatment of infants with Pierre Robin sequence]. / Therapeutisches Vorgehen bei Pierre-Robin-Sequenz.

The Pierre Robin sequence (PRS) characterized by mandibular micro- or retrognathia and glossoptosis with or without cleft palate, presents clinically with intermittent upper airway obstruction (UAO). It is associated with other malformations in about half the cases. The incidence is about 1:8,500. Isolated PRS without other malformations does not, by itself, appear to affect neurodevelopment. Active intervention may therefore be required to reduce the risk for neurocognitive impairment resulting from UAO. Current treatment options for UAO range from prone positioning, use of a nasopharyngeal tube, glossopexy via tongue lip adhesion, mandibular distraction to tracheostomy. An effective, non-invasive treatment protocol which includes implementation of an intraoral orthodontic appliance with velar extension (the pre-epiglottic baton plate; PEBP) is presented in this article.

Cystic colon duplication as differential diagnosis to ovarian cyst.

OBJECTIVE: We report the case of a female fetus with a single spherical anechoic cyst on the right side of the lower abdomen first diagnosed at 22 weeks of pregnancy. METHODS: Serial ultrasound monitoring and needle aspiration of the cyst were performed. RESULTS: The cyst grew during pregnancy up to 8 cm diameter. Needle aspiration was performed at 33 and 36 weeks. At 38 weeks labor was induced and a girl was delivered spontaneously. Postnatally the child showed abdominal distention, vomiting, and an obstruction of venous return of the right leg. To improve venous circulation, another needle aspiration of the cyst was performed. Laparoscopy revealed cystic colon duplication. CONCLUSIONS: Prenatal differentiation of a single round anechoic cyst in the pelvis of a female fetus can be difficult. As a rare abnormality cystic colon duplication has to be considered.

Interaction between systemic circulation and brain injuries in newborns.

In several studies different pathogenetic mechanisms of metabolic and/or cardiovascular origin were shown depending on the type of hypoxic or hypoxic/hypercapnic stress and conditions of animals (normal developed or intrauterine growth retarded newborn piglets). Thus, in normal-weight piglets the prevention of compensatory hyperventilation owing to severe hypoxia (artificially ventilated animals) leads to critical, i.e. lethal acidosis. In intrauterine growth retarded piglets, a severe hypoxia cannot be vitally compensated because of the restricted circulatory centralization. Moreover, a limited cerebrovascular dilatation during moderate hypoxia/hypercapnia produces a critical state of the metabolic supply in those animals.

[Serum concentration of gentamicin in newborn infants]. / Untersuchungen zur Serumkonzentration von Gentamicin bei Frühgeborenen.

Serum levels of gentamicin were determined in 15 premature infants (birth weight 1,120-2,250 g, gestational age 27-36 weeks) by agar immunoassay. The intramuscular applied doses was 6 mg/kg/day, the doses interval 12 hours. We found an increase of the gentamicin serum level during the first 72 hours after the beginning of therapy. At the second day of treatment the serum levels in 46.5% of all patients were determined below the therapeutic concentration, but a toxic level was found in more than 60% of all cases during the period of steady state. The serum levels were higher in preterm newborns of less than 1,500 g birth weight, then in more mature infants. We recommend: 1. To double the first doses of gentamicin 2. To modify the gentamicin level by expanded intervals of therapy and/or reduced doses under monitoring, especially during the first week of life.

[Treatment of post-hemorrhagic hydrocephalus in premature infants]. / Zur Problematik der Behandlung des posthämorrhagischen Hydrozephalus Frühgeborener.

Our experiences in the course of illness of very low birth weight infants suffered from posthemorrhagic hydrocephalus are described. Clinical proceedings for performing of the hydrocephalus are explained, advantages and disadvantages of treatment by serial lumbal punctures, influences of drugs on the dilatation of the ventricles and the insertion of ventriculo-peritoneal shunts are discussed. The outcome was unfavourable in hydrocephalus following Grad III ore IV hemorrhage. Therefore the prevention of severe hydrocephalus must be the common sense of obstetricians and neonatologists.

Restricted cardiovascular and cerebral performance of intra-uterine growth retarded newborn piglets during severe hypoxia.

In 7 term born two-day-old intra-uterine growth retarded (IUGR) piglets cardio- and cerebrovascular, metabolic and EEG reactions were studied at rest and during severe hypoxia in comparison to 17 normal-weight piglets. In IUGR piglets even in control conditions a distinct cerebral blood flow (CBF) elevation, lower cerebrovascular resistance and oxygen consumption in the higher perfused brain regions at diminished arterial blood pressure were observed. Also the arterial glucose concentration was in the borderline hypoglycemic range, however, cerebral glucose delivery was nearly maintained. IUGR piglets survived a 1-h hypoxic period (paO2 = 25-30 mmHg) less frequently. The main reason was the distinctly restricted emergency reaction with a lack of arterial blood pressure increase and blood glucose elevation as well as a reduced (higher perfused brain regions) or absent CBF increase (lower perfused brain regions). Thereby, even in the early stage of hypoxia cerebral oxygen delivery in both brain compartments and oxygen consumption in the latter were decreased followed by a further decrease of the arterial blood pressure. The latter factor was essential for acute cardio-vascular-cerebral insufficiency.

Vascular resistance, metabolism and EEG within cerebral grey and white matter during hypoxia in neonatal piglets.

To study unsolved problems of the causal chain of neonatal hypoxic brain damage in 31 hypoxic newborn non-anesthetized piglets cerebro-vascular, metabolic and EEG reactions were investigated (FiO2: 0.06-0.10, 1 h). Only in artificially ventilated newborn piglets this acute hypoxic hypoxia provoked a vital decompensation by critical depression of mean arterial blood pressure (less than or equal to 4.67 kPa [35 mmHg]) and/or a critical increase of vascular resistance (Rc) in the cerebral white matter (greater than 50%). Spontaneously breathing piglets survived always showing hyperventilation, higher pHa and an increase of cerebral O2-consumption in cerebral grey matter, partly also in white matter. This critical increase of Rc was related to a critical decrease of O2-consumption in the white matter and an insufficient decrease of Rc of the grey matter. The observed strong metabolic and hemodynamic differences between these two brain compartments can explain the evaluated special morphological vulnerability of cerebral white matter in ventilated animals.

[Effect of short-term aerogenic hypoxia on the behavior of heart rate and blood pressure in newborn swine]. / Untersuchungen zum Einfluss einer kurzzeitigen aerogenen Hypoxie auf das Verhalten von Herzfrequenz und Blutdruck bei neugeborenen Ferkeln.

The adaption of the cardiovascular system to hypoxia has been examined in piglets aged 3 to 81 hour. The reaction of the piglets to a shorttime normobaric hypoxia was very different: In 50% there was an inadequate reaction with decrease of blood pressure, heart rate, pulse pressure and therefore of stroke volume, in 30% there was in compliance with the adult mechanism a compensatory increase of blood pressure, heart rate, pulse pressure and stroke volume. Only in 2 cases a compensatory increase of blood pressure could be observed in combination with a simultaneous decrease of heart rate. The results refer once more to the fact, that the reactions of the cardiovascular system of the neonate to hypoxia can be very variable and require an individual therapeutic regime provided that they are transferable to the human newborn and premature baby.