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Obstructive sleep apnea syndrome in sickle cell disease
B R Maddern1, H T Reed, K Ohene-Frempong
1Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine, New Orleans, LA 70112.
The Annals of Otology, Rhinology, and Laryngology
|March 1, 1989
Summary
Children with sickle cell disease (SCD) and obstructive sleep apnea syndrome may benefit from adenotonsillectomy. This procedure, while risky in SCD patients, can resolve symptoms when managed with a specific protocol.
Area of Science:
- Pediatric Otolaryngology
- Pediatric Hematology
- Sleep Medicine
Background:
- Obstructive sleep apnea syndrome (OSAS) and sickle cell disease (SCD) can co-occur in children.
- Adenotonsillar hypertrophy (ATH) may be more prevalent in children with SCD.
- The management of adenotonsillectomy in SCD patients presents unique challenges and risks.
Purpose of the Study:
- To describe the outcomes of adenotonsillectomy in children with OSAS and SCD.
- To present a management protocol for adenotonsillectomy in this high-risk population.
- To identify predictors of disease severity in pediatric SCD patients with OSAS.
Main Methods:
- Retrospective review of 21 children with OSAS and SCD.
- Analysis of polysomnography (sleep studies) data.
- Evaluation of pre- and post-operative management, including hydration and transfusion therapy.
Main Results:
- Thirteen patients underwent adenotonsillectomy, with symptom resolution and improved alveolar hypoventilation.
- Increased end-tidal carbon dioxide during sleep was a significant predictor of OSAS severity.
- Oxygen saturation monitoring was found to be unreliable for assessing disease severity.
Conclusions:
- Adenotonsillectomy can be effective for OSAS in children with SCD when managed appropriately.
- A specialized protocol is crucial for mitigating surgical risks in these patients.
- ATH in SCD may represent a compensatory lymphoid enlargement, not solely linked to infection.