Abstract • It is difficult to recognize and treat the factors that exacerbate the vascular crises of sickle cell disease. We describe a 12-year-old black girl with sickle cell anemia who, since the age of 9 years, has been repeatedly hospitalized for pain associated with vaso-occlusive disease. A diagnosis of obstructive sleep apnea was suggested by the history and physical examination and was confirmed by polysomnography. Following preoperative transfusion, the patient underwent elective tonsillectomy and adenoidectomy. She has been free of vaso-occlusive pain and crises for over two years and has not been hospitalized since her surgery. Postoperative polysomnography has shown no evidence of obstructive sleep apnea. It is our hypothesis that repeated oxygen desaturation during periods of obstructive sleep apnea was the cause of this patient's frequent vaso-occlusive crises. She not only obtained complete relief of her symptoms, but objective sleep study measurements were normal following surgery. Obstructive sleep apnea is a recent diagnosis, and its pathologic effects are only beginning to be known. Physicians taking care of patients with hemoglobinopathies need to be aware of possible contributing factors to their patients' disease, and an aggressive approach to their diagnosis and possible relief should be sought. (Arch Otolaryngol Head Neck Surg 1988;114:916-917) References 1. Bunn HF: Disorders of hemoglobin , in Braunwald E, Isselbacher KJ, Petersdorf RG, et al (eds): Harrison's Principles of Internal Medicine . New York, McGraw-Hill International Book Co, 1987, pp 1518-1527. 2. Potsic WP, Pasquariello PS, Baranak CC, et al: Relief of upper airway obstruction by adenotonsillectomy . Otolaryngol Head Neck Surg 1986;4:476-480. 3. Grundfast KM, Wittich DJ: Adenotonsillar hypertrophy and upper airway obstruction in evolutionary perspective . Laryngoscope 1982; 92:650-656.Crossref 4. Coker NJ, Milner PF: Elective surgery in patients with sickle cell anemia . Arch Otolaryngol Head Neck Surg 1982;108:574-576.Crossref 5. Janik J, Seeler RA: Perioperative management of children with sickle hemoglobinopathy . J Pediatr Surg 1980;15:117-120.Crossref 6. Feinberg AN, Shabino CL: Acute pulmonary edema complicating tonsillectomy and adenoidectomy . Pediatrics 1985;75:112-114.
Archives of Otolaryngology - Head & Neck Surgery – American Medical Association
Published: Aug 1, 1988