OBSTRUCTIVE SLEEP APNEA SYNDROME IN A PRADER WILLI PATIENT: CASE REPORT

• DOI: https://doi.org/10.22533/at.ed.1594882426096

• Palavras-chave: apnea, polysomnography, hyperphagia

• Keywords: apnea, polysomnography, hyperphagia

• Abstract:

  INTRODUCTION: 
  Prader willi is a rare disease, affecting one in thirty thousand live births, and can be the cause of obstructive sleep apnea

  OBJECTIVES: 
  To describe a case of prader willi and its otorhinolaryngological manifestations in an adolescent from the state of pará, demonstrating the importance of proper investigation, correct diagnosis and a multi-professional treatment plan. 

  CASE REPORT:
  A 17-year-old male patient with prader willi syndrome was admitted to the pediatric otorhinolaryngology outpatient clinic at bettina ferro de sousa university hospital (hubfs) complaining of mouth breathing, snoring, restless sleep, orthopnea and excessive daytime sleepiness. The patient's father says that he often sleeps on the sofa while tying his shoelaces. Polysomnography showed a moderately increased apnea and hypopnea index, reduced rem sleep, increased waso. Fibronasolaryngoscopy was also performed, which showed grade iii tonsils, hypertrophy of lymphoid tissue occupying 70% of the cavum, retropalatal Muller maneuver with 90% circumferential reduction of the airway. Despite this, the syndrome promotes hyperphagia, and the patient has increased cervical and abdominal circumference. Referred to the sleep clinic, a multi-professional approach was chosen, with therapeutic and nutritional help and uvulopalatopharyngoplasty. The patient is awaiting surgery 

  CONCLUSION:
  The case reported and the publications collected shed light on a complex situation such as Prader Willi syndrome, which requires clinical and surgical otorhinolaryngological approaches, as well as monitoring by a multidisciplinary team.