Rapidly evolving narcolepsy-like syndrome coinciding with severe OSA following pharyngoplasty in Prader-Willi syndrome.

Our patient with Prader-Willi syndrome (PWS) not only displayed many typical syndromic features but also presented several unique challenges, with gross velopharyngeal insufficiency necessitating repair and severe obstructive sleep apnea developing thereafter, requiring ongoing non-invasive ventilation. This coincided with development of a narcolepsy-like syndrome, treated with dexamphetamine. Cataplexy, hypnogogic/hypnopompic hallucinations, sleep paralysis were absent and HLA-DQB1*06:02 was negative. Growth hormone (GH) therapy was commenced at 8 months of age and, as recommended, regular polysomnograms were conducted. Adenotonsillar growth on GH therapy is reported as well as several reports of sudden death in PWS patients on GH. Despite GH, lifestyle measures with regular dietician review, and an exercise program, there was progressive excessive weight gain. Our patient also developed moderate tonsil hypertrophy. To our knowledge, this is the first case report of severe obstructive sleep apnea secondary to sphincter pharyngoplasty coinciding with rapidly evolving narcolepsy-like syndrome.

Parental perceptions of posterior pharyngeal wall augmentation using autologous fat for treating velopharyngeal dysfunction.

Posterior pharyngeal wall augmentation using autologous fat to treat velopharyngeal dysfunction (VPD) is an alternative surgical procedure to more commonly used invasive procedures such as the pharyngeal flap. However, limited research exists on this technique. The aim of this study was to qualitatively investigate parental perceptions of posterior pharyngeal wall augmentation using autologous fat when treating velopharyngeal dysfunction. Furthermore, this research aimed to examine parent's perspectives of their child's speech and quality-of-life following this procedure. A qualitative collective case study methodology was used in the form of semi-structured interviews with seven participants. These were then analysed using constant comparative analysis. Four distinct themes emerged: post-surgical outcomes; speech-language pathology, not just medicine; factors for successful post-operative speech and resonance; and long-term sustainability and worthiness of the procedure. Six out of seven participants expressed positive post-operative speech and resonance results. Five further expressed long-term satisfaction up to 6 years post-operatively. Overall the majority of participants were satisfied that this procedure provided their child with long-term successful speech outcomes. The participants also discussed the importance of receiving speech-language pathology services alongside surgery and the positive impact of the procedure on their child's quality-of-life including social interactions, confidence, friendships, as well as eating and drinking.

Prevention of hyponatremia during maintenance intravenous fluid administration: a prospective randomized study of fluid type versus fluid rate.

OBJECTIVES: To determine the importance of sodium content versus administration rate of intravenous fluids in the development of hyponatremia in postoperative children. STUDY DESIGN: In this prospective, randomized, nonblinded study, 124 children admitted for surgery received 0.9% (NS) or 0.45% (N/2) saline solution at 100% or 50% maintenance rates. Plasma electrolytes, osmolality, and ADH at induction of anesthesia were compared with values 8 hours (T(8)), and 24 hours (T(24); n = 67) after surgery. Blood glucose and ketones were measured every 4 hours. Electrolytes and osmolality were measured in urine samples. RESULTS: Plasma sodium concentrations fell in both N/2 groups at T(8) (100%: -1.5 +/- 2.3 mmol/L 50%: -1.9 +/- 2.0 mmol/L; P < .01) with hyponatremia more common than in the NS groups at T(8) (30% vs 10%; P = .02) but not T(24). Median plasma antidiuretic hormone concentrations increased 2- to 4-fold during surgery (P < or = .001) and only reattained levels at induction of anesthesia by T(24) in the N/2 100% group. On multiple linear regression analysis, fluid type, not rate determined risk of hyponatremia (P < .04). Two children on 100% developed SIADH (1NS). Fourteen (23%; 7NS) on 50% maintenance were assessed as dehydrated. Dextrose content was increased in 18 for hypoglycemia or ketosis. CONCLUSIONS: The risk of hyponatremia was decreased by isotonic saline solution but not fluid restriction.