Mandibular growth in infants with Robin sequence treated with the Tübingen palatal plate.

Background: Robin sequence (RS) is characterized by mandibular retrognathia, glossoptosis and upper airway obstruction. Whether mandibular catch-up growth may occur in RS is yet controversial. Our functional and less invasive treatment including the Tübingen Palatal Plate (TPP), early oral feeding and orofacial stimulation may promote mandibular catch-up growth.

Less invasive treatment of sleep-disordered breathing in children with syndromic craniosynostosis.

Background: Infants and children with syndromic craniosynostosis (SCS), such as Apert-, Crouzon- or Pfeiffer syndrome, are prone to sleep disordered breathing (SDB) including obstructive sleep apnea and upper airway resistance syndrome (OSAS, UARS), potentially leading to tracheostomy. We modified the Tübingen Palatal Plate (TPP), an oral appliance with a velar extension effectively treating airway obstruction in Robin sequence, by attaching a tube to its velar extension to bridge the narrow pharyngeal airway in SCS patients. Here, we evaluated this treatment concept.

Treatment of infants with Syndromic Robin sequence with modified palatal plates: a minimally invasive treatment option.

Background: Infants with Robin sequence (RS) suffer from upper airway obstruction (UAO) and feeding problems. We developed an oral appliance with a velar extension in combination with functional treatment and appropriate feeding techniques, which was proven effective in isolated RS. As the above problems are particularly challenging in syndromic RS, we set out to evaluate our treatment concept also in these patients.

Multicenter study on the effectiveness of the pre-epiglottic baton plate for airway obstruction and feeding problems in Robin sequence.

Background: Treatment of Robin sequence is often either invasive or of unproven effectiveness. The pre-epiglottic baton plate (PEBP) is a well-studied alternative, yet is not widely applied internationally. We report on a prospective 3-center cohort study investigating this treatment.

Functional treatment of airway obstruction and feeding problems in infants with Robin sequence.

Background: Robin sequence (RS), characterised by micrognathia/retrognathia and glossoptosis with or without cleft palate, presents with intermittent upper airway obstruction and feeding difficulties. Active intervention is required to reduce the risk of brain damage or sudden death. Most treatment options are poorly studied and/or invasive.

A newborn with grouped facial skin lesions and subsequent seizures.

Background: Congenital grouped skin lesions are alarming signs of a variety of threatening diagnoses of quite different origin. The present case report shows an impressive clinical pattern of a neonate and illustrates the difficulty in differential diagnosis of mixed connective tissue disease and neonatal lupus erythematosus in newborns. This reported case is to our knowledge the first description of an unrecognized mixed connective tissue disease in the mother with an unusual clinical manifestation in the newborn, comprising skin lesions, neurological damage and non-typical antibody constellation.

Treatment of obstructive sleep apnea in infants with trisomy 21 using oral appliances.

Objective : To perform a retrospective study to evaluate the effect of oral appliances, aimed at increasing the pharyngeal space, on obstructive sleep apnea (OSA) in infants with trisomy 21 (TS21). Design and Setting : Retrospective study in a tertiary referral center. Intervention : We analyzed data from 51 consecutive infants (mean age, 2.

[Pierre Robin Sequence: interdisciplinary treatment after prenatal diagnosis].

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.

[Treatment of infants with Pierre Robin sequence].

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.

An oral appliance with velar extension for treatment of obstructive sleep apnea in infants with Pierre Robin sequence.

Objective: A new oral appliance to treat obstructive sleep apnea in infants with Pierre Robin sequence has recently been shown to be superior to a sham procedure. We now investigate safety and long-term effects of this appliance on obstructive sleep apnea in infants with Pierre Robin sequence.

Design: Case series with repetitive follow-up examinations.

A randomized clinical trial of a new orthodontic appliance to improve upper airway obstruction in infants with Pierre Robin sequence.

Objective: To test the hypothesis that a new orthodontic appliance with a velar extension that shifts the tongue anteriorly would reduce upper airway obstruction in infants with Pierre Robin sequence (PRS).

Study Design: Eleven infants with PRS (median age, 3 days) and an apnea index (AI) >3 were studied. The effect of the new appliance on the AI was compared with that of a conventional appliance without a velar extension by using a crossover study design with random allocation.

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.

Autosomal dominant precalcaneal congenital fibrolipomatous hamartoma.

Precalcaneal congenital fibrolipomatous hamartoma is a rare minor malformation previously reported only as sporadic. We report this finding in three family members from two generations, suggesting autosomal dominant inheritance.

[The Tübingen palatal plate--an innovative therapeutic concept in Pierre-Robin sequence].

Infants with Pierre-Robin sequence (PRS) may suffer severe upper airway obstruction resulting in hypoxemia that is difficult to treat. We are currently evaluating a new therapeutic approach involving an oral appliance that widens the pharynx by pulling the base of the tongue forward using a preepiglottic baton. Here we present a patient treated with this device who showed a decrease in his desaturation index from 50 to < 1.

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.

[Serum concentration of gentamicin in newborn infants].

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.

[Treatment of post-hemorrhagic hydrocephalus in premature infants].

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.

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.

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).

[Effect of short-term aerogenic hypoxia on the behavior of heart rate and blood pressure in newborn swine].

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.