A Rare Cause of Sleep-Disordered Breathing: ROHHAD Syndrome

Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) syndrome; is a rare but crucial disorder. Sleep-disordered breathing can occur at the beginning or after of obesity. A disease-specific test for diagnosis is not yet available. Neural crest tumors (ganglioneuroma, ganglioneuroblastoma) have been reported in 40% of patients. In our study, three patients diagnosed as having ROHHAD syndrome are presented from our hospital. In the evaluation of the hypothalamic functions of the patients, one of them had growth hormone deficiency and hyperprolactinemia; recurrent hypernatremia reflecting irregular water balance was detected in another. One of the patients had abnormal pupil reflex and heart rate irregularity while another had excessive sweating as autonomic dysfunction. One of the patients was diagnosed with paravertebral ganglioma accompanying ROHHAD syndrome. Non-invasive ventilation treatment was started in all patients because there was a sleep-disorder breathing clinic diagnosis. ROHHAD syndrome deserves a multidisciplinary team approach as it can affect more than one organ system. In these patients, should be sleep-disorder breathing determined early and appropriate treatment should be initiated immediately to reduce morbidity and mortality.

Assessment of Proportion of Surgical Patients with

Obstructive sleep apnea is the most common type of sleep disorder breathing.90%of them remain undiagnosed. These patients may be associated with an increased risk of perioperative complications. Our objective was to evaluate the proportion of surgical patients with risk of undiagnosed Obstructive sleep apnea. After research ethics board approval, patient attending preanesthetic clinic were asked to fill the STOP-Bang questionnaire. Prospective observational study is used with the sample size of 70 patients and the study duration is 4 months. In 70 patients, the proportion of surgical patients with the increased(intermediate-high) of developing Obstructive sleep apnea is 78%(55 patients) This study is conducted to find out the percentage of surgical patients having undiagnosed Obstructive sleep apnea can be identified and treated to prevent perioperative complications.

The Efficacy of Posterior Turbinoplasty in Combination with Tonsillectomy and Adenoidectomy in Pediatric Sleep Disordered Breathing Patients

Background and Objectives Tonsillectomy and adenoidectomy (T&A) are effective for the treatment of pediatric sleep disorder breathing. However, in some pediatric patients, there remain unresolved snoring and mouth breathing problems after the operation. This study is aimed at evaluating the efficacy of coblation of hypertrophic posterior turbinate in combination with tonsillectomy and adenoidectomy.Subjects and Method We analyzed data from 50 children aged between 5 to 13 years old who were scheduled to undergo T&A operation from June 2018 to December in 2018. We randomly divided them into two groups. The first group consisted of 25 patients who were scheduled to perform only tonsillectomy and adenoidectomy. The second group was 21 patients who were scheduled to perform posterior turbinoplasty in combination with tonsillectomy and adenoidectomy. We evaluated preoperative and postoperative polysomnogram [Apnea-Hypopnea Index (AHI), O2 saturation], acoustic rhinometry (minimal cross section area, nasal volume), and Korean Obstructive Sleep Apnea-18 Survey (KOSA-18) score.Results After additional turbinoplasty (coblation of hypertrophic posterior turbinate), the rate of snoring and mouth breathing remaining was decreased. There were significant improvements in AHI, oxygen saturation, Visual Analog Scale and Epworth Sleepiness Scale in two groups. Symptoms (snoring and mouth breath) remaining postoperatively was much more decreased in the posterior turbinoplasty combined with T&A group than in the single T&A group in terms of KOSA-18.Conclusion When it comes to pediatric sleep disorder breathing surgery, nasal cavity evaluation is an important factor for patient’s postoperative satisfaction for sleeping and snoring. For pediatric sleep disorder breathing patients with hypertrophic posterior turbinate, who are scheduled to do tonsillectomy and adenoidectomy, an additional coblation of posterior turbinate should be considered.