Turner syndrome morphology and morphometrics: Cardiac hypoplasia as a cause of midgestation death.

Background: A female fetus with massive truncal-limb hydrops and large, loculated, nuchal hygromas in midgestation is highly likely to have Turner syndrome. This phenotype is recognized to be usually lethal, with only more mildly affected fetuses surviving to term birth.

Methods: The morphology and morphometrics of 117 midgestation fetuses with phenotypic Turner syndrome were analyzed.

Fetal cardiac dextroposition in the absence of an intrathoracic mass: sign of significant right lung hypoplasia.

We reviewed our experience of fetal cardiac dextroposition in the absence of an intrathoracic mass. Ten cases were found by fetal echocardiography to have a normal cardiac axis, but the heart was shifted into the right chest and the amount of right lung tissue was reduced. At birth seven of the infants had confirmed structural heart disease (70%), including three with scimitar syndrome.

Bacterial tracheitis in children.

We examined the records of 14 patients aged 7 months to 10 1/4 years who were treated for bacterial tracheitis from May 1982 to December 1987; the management protocol for 13 of the patients included the use of nasotracheal intubation. The infection was caused by Staphylococcus aureus in seven, Haemophilus influenzae in three, Branhamella catarrhalis in one and Streptococcus pneumoniae in one. Both H.

Elective oro-tracheal intubation to diagnose sleep apnea syndrome in children with Down's syndrome and ventricular septal defect.

Three children with Down's syndrome, ventricular septal defect, pulmonary hypertension and clinical evidence of upper airway obstruction had the trachea electively intubated while pulmonary artery pressures were monitored. In two, the pulmonary pressures immediately decreased and systemic arterial oxygen saturations increased. Both of these children showed dramatic clinical improvement after tonsillectomy.

Elective orotracheal intubation and aspiration for microbiologic diagnosis in children.

Endotracheal intubation is generally performed to facilitate the treatment of respiratory failure or to control the airway during general anesthesia. We electively intubated 14 children with undiagnosed pneumonia to obtain tracheobronchial secretions for microbiologic diagnosis. Three were intubated awake, three with sedation, and eight with sedation and neuromuscular blockade.