Congenital high airway obstruction syndrome (CHAOS): Natural history,prenatal management strategies,and outcomes at a single comprehensive fetal center

PurposeCongenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions. This report describes our experience with management and fetal interventions for CHAOS, including laser laryngotomy.MethodsWe retrospectively reviewed all patients diagnosed with CHAOS at a single academic institution between 2006 and 2017.ResultsFifteen patients were identified. Eight had obstruction at the trachea and seven at the larynx. In the laryngeal obstructions, three expired shortly after birth, and one survived after spontaneous fistulization and subsequent EXIT to tracheostomy. The remaining three underwent in-utero treatment with laser laryngotomy. One had preterm premature rupture of membranes (PPROM), delivered 3 days post-operatively, and died. Two underwent EXIT to tracheostomy with one surviving to discharge and is currently 2 years old.ConclusionOur study demonstrates the outcomes of a large series of patients diagnosed with CHAOS. While mortality remains high, options for fetal intervention are being explored to allow alterations in the prenatal natural history and improve postnatal outcomes.Type of StudyRetrospective Treatment Study.Level of EvidenceLevel IV.     

Fluorescein angiography of the newborn rat. Implications in oxygen-induced retinopathy

The current technique was developed to characterize the morphologic changes in the retinas of oxygen-reared rats, as an animal model of retinopathy of prematurity. Past studies have used ink perfusion to observe the retinal vasculature, but this method is static and requires the sacrifice of the subject. Fluorescein angiography, however, is dynamic and relatively noninvasive, and allows the survival of the animal for further study. The fundus camera cannot be used because the source of light that is focused in an annulus is too large for the pupil size of a young (approximately 14-day-old) rat. To overcome this, a Nikon inverted microscope (Diaphot-TMD) was used. Using the proper exciting and barrier filters for fluorescene, a photographic sequence was made by rapidly focusing to the plane of the retinal vessels. To our knowledge, similar photographs have not been previously published. This technique was used in newborn pigmented ratlings that were 1) exposed to 80% oxygen for the first 14 days of life; 2) exposed to 80% oxygen for the first 21 days of life; or 3) exposed for the first 14 days followed by 7 days in room air. Age-matched controls were raised simultaneously in room air and evaluated with the same technique. Differences were observed between treatments in the amount of retinal capillary loss, and in the tortuosity and diameter of the major retinal vessels. The hyaloid system also varied between treatment groups. Oxygen-exposed rats showed a persistence of the hyaloid vessels that was particularly prominent in the group returned to room air before analysis. Comparisons are made to past results obtained with other histologic techniques.     

Orbicularis oculi responses to stimulation of nerve afferents from upper and lower limbs in normal humans

A brief mechanical or electrical stimulus to peripheral nerve afferents from the upper and lower limbs elicited a small and inconsistent EMG response of the orbicularis oculi muscles. This response was facilitated when the stimuli were delivered at fixed leading time intervals, of 45–300 ms, with respect to a supraorbital nerve electrical stimulus. Also, the peripheral nerve stimulus modified the conventional blink reflex responses, inducing facilitation of R1 and inhibition of R2. These results suggest a complex processing of sensory inputs from the face and the limbs at the brainstem, where they are probably integrated in a network of interneurons influencing the excitability of facial motoneurons.     

A new nematode, Ansiruptodera scapteromi sp. nov. (Nematoda: Aspidoderidae), recovered from the Argentinean water rat Scapteromys tumidis (Waterhouse, 1837) in Uruguay

This report describes a new species of aspidoderid nematode, Ansiruptodera scapteromi sp. nov., the second species to be reported in the genus Ansiruptodera Skrjabin and Shikhobalova, 1947. The A. scapteromi sp. nov. is the first species of the genus to be recorded from a rodent host. The new species is clearly different from the only other species, A. ansirupta (Proença, 1937) Skrjabin and Shikhobalova, 1947, in that it possesses short lateral alae that terminate before the midbody; a smaller cephalic extremity; a shorter esophagus, pharynx, tail, and tail appendage; a smaller sucker; and longer spicules. The two species also differ in the numbers and arrangements of caudal papillae. A. scapteromi appears to be a parasite of capture and the water rats seem to have been infected from Edentata in Uruguay.     

Post-traumatic diffuse brain swelling: isolated or associated with cerebral axonal injury

Eighteen children with severe head injuries and diffuse brain swelling were studied. They were separated into two groups based on the computed tomography (CT) findings. Seven patients had small ventricles in the normal location and small or absent cisterns. Eleven had these signs plus small deep-seated intraparenchymal hemorrhagic foci and/or intraventricular hemorrhage. Patients in the first group were in relatively good neurological condition; their intracranial pressure was easily controlled and all had a favourable outcome. On contrast, children in the second group had a more severe clinical presentation, frequently had uncontrollable intracranial hypertension, and more than 50% died.