Orthognathic Correction in Prader-Willi Syndrome: Occlusion and Sleep Restored.

Children with Prader-Willi Syndrome (PWS) may present with a malocclusion and have a high propensity of developing obstructive sleep apnea (OSA). Obstructive sleep apnea is associated with short- and long-term adverse effects that negatively impact children with PWS. A case of a 15-year-old male with PWS, OSA, and a debilitating malocclusion is presented who underwent a combination of Le Fort 1 osteotomy, genioplasty, and tongue reduction to successfully treat his OSA and malocclusion. In select cases, orthognathic correction and other surgical therapies should be considered in patients with PWS.

Acute Respiratory Distress Syndrome in a Patient with Acute Epstein-Barr Viral Infection and Eosinophilic Pneumonia.

We report a case of acute respiratory distress syndrome associated with eosinophilic pneumonia in an adolescent with acute Epstein-Barr viral infection.

Respiratory disease associated with Bordetella bronchiseptica in a Hoffmann's two-toed sloth (Choloepus hoffmanni).

A 2-yr-old female captive-born Hoffmann's two-toed sloth (Choloepus hoffmanni) presented with respiratory disease. A severe inspiratory dyspnea with nasal congestion was observed with open-mouthed breathing and bilateral mucopurulent nasal exudate. Despite initial treatment with broad-spectrum antimicrobial therapy and anti-inflammatory and supportive care, the dyspnea persisted. The animal was anesthetized for bronchoscopy to obtain a deep tracheal sample. Based on culture of Bordetella bronchiseptica and sensitivity, a combination of systemic enrofloxacin, dexamethasone, and coupage with nebulization of saline, gentamicin, and albuterol as well as supportive care resulted in full recovery after 6 weeks of treatment.

The effect of maternal smoking on respiratory and arousal patterns in preterm infants during sleep.

Prenatal exposure to cigarette smoke is associated with an increased risk of sudden infant death syndrome. The effect of maternal smoking on apnea and arousal patterns in preterm infants is currently unknown. Multichannel polysomnographic studies were performed in preterm infants. Thirty infants were enrolled into the study: 16 exposed prenatally to cigarette smoke (S) and 14 control infants (C). There was no difference in the gestational and postconceptional ages at the time of study. Maternal smoking was associated with a significant increase in the apneic index in these infants (28.6 +/- 6.4/hour [S] vs. 13.2 +/- 3.9 [C]; p<0.05), and the difference was noted for obstructive events and only during active sleep. The arousal index was significantly decreased in the maternal smoking group (34.5 +/- 2.3/hour [S] vs. 46.3 +/- 5.6/hour [C]; p<0.05), with a specific decrease in percentage of arousal after respiratory events (10.7 +/- 2.1% [S] vs. 29.4 +/- 5.4% [C]; p<0.05). In conclusion, preterm infants exposed prenatally to cigarette smoke have increased respiratory events during active sleep, predominantly due to obstructive apnea, and possibly a higher arousal threshold during apneic events. These alterations in respiratory and arousal patterns in preterm infants born to smoking mothers may lead to significant vulnerability in this population.

Effect of supplemental oxygen on sleep architecture and cardiorespiratory events in preterm infants.

OBJECTIVE: To investigate the effect of low-flow supplemental oxygen (SupOx) on sleep architecture and cardiorespiratory events in asymptomatic preterm infants. METHODS: An overnight polysomnographic evaluation was conducted prospectively in 23 premature infants who were born at 30.0 +/- 3.2 (standard deviation) weeks' gestational age and studied at 38.1 +/- 4.4 weeks' postconceptional age. Infants were free of any adverse events, including cardiorespiratory monitor alarms in the nursery for at least 1 week before the study. Infants received room air (RA) or SupOx via nasal cannula at 0.25 L/min. RESULTS: Quiet sleep density was increased during SupOx (33.3 +/- 10.8% vs 26.6 +/- 10.0% total sleep time [TST] in RA), and a reciprocal decrease in active sleep density was observed (61.5 +/- 11.1% vs 68.4 +/- 9.9% TST in RA). No differences in sleep efficiency emerged (69.7 +/- 10.6% SupOx vs 69.7 +/- 8.8% RA). SupOx elicited significant decreases in apnea index (3.8 +/- 2.4 events/h vs 11.1 +/- 6.4 events/h in RA) and in the percentage of time spent in periodic breathing (1.8 +/- 2.9% vs 6.7 +/- 8.9% in RA). In addition, SupOx decreased the frequency of bradycardic events (0.3 +/- 0.8 events vs 2.5 +/- 0.03 events in RA) and improved overall oxygen saturation (98.7 +/- 1.1% vs 96.4 +/- 2.2%). No changes in alveolar ventilation, as derived from end-tidal CO2 measurements, was detected (38.6 +/- 5.8 mm Hg in SupOx vs 38.4 +/- 5.4 mm Hg in RA). CONCLUSIONS: Asymptomatic preterm infants exhibit frequent and potentially clinically adverse cardiorespiratory events when assessed in the sleep laboratory. Administration of SupOx to these infants is associated with an increase in the overall duration and percentage TST spent in quiet sleep with reciprocal changes in active sleep. In addition, improvement in respiratory stability is observed with the use of low-flow SupOx, as evidenced by a decrease in apnea, periodic breathing, and bradycardia, without adverse effects on alveolar ventilation.