Adenotonsillar hypertrophy and frequency of Vaso-occlusive crisis in patients with sickle cell disease at Mulago Hospital

Abstract

Sickle cell disease is a multisystem disease with episodes of acute illnesses associated with progressive organ damage. The commonest manifestation of sickle cell disease is a vaso-occlusive painful crisis. Complications of adenotonsillar hypertrophy such as obstructive sleep apnea and recurrent infections are more prevalent in children with sickle cell disease compared to the general paediatric population. However, there is limited information regarding the effect of tonsillar hypertrophy on the frequency of vaso-occlusive crises in patients with sickle cell disease. This study sought to determine the factors associated with adenotonsillar hypertrophy and its variation with the frequency of vaso-occlusive painful crises among patients with sickle cell disease. Objectives: To determine the factors associated with adenotonsillar hypertrophy, and to compare the frequency of vaso-occlusive crisis among sickle cell disease patients with and without adenotonsillar hypertrophy at Mulago Hospital. Methods: This was an unmatched case-control study conducted at Mulago National referral Hospital Sickle Cell Clinic. Of the 401 children and adolescents aged 2-17 years enrolled in the study, 123(30.7%) cases of adenotonsillar hypertrophy were compared to 278(69.3%) controls. Tonsillar hypertrophy was diagnosed using direct visual examination using illumination light and, a post-nasal space x-ray was used to diagnose adenoid hypertrophy. An interviewer-administered questionnaire was used to obtain social demographic information and medical history. Data were collected and managed using an open data kit (ODK) and analyzed using STATA version 16.Results: There was a significant difference in the mean frequency of painful vaso-occlusive crises suffered in the previous 6 months between cases and controls (p value= 0.018). Age of 15-18 years (OR: p=0.047, females (OR: 2.35; p=0.003), Snoring (OR: p< 0.0001), and children who predominately breathed through the mouth (OR=5.14, p=0.001) were all associated with increased odds of adenotonsillar hypertrophy. Among disease-modifying factors, patients who were taking hydroxyurea (OR: 2.33; p=0.027) had higher odds of adenotonsillar hypertrophy. Conclusion. Children with sickle cell disease and adenotonsillar hypertrophy are at risk of suffering more pain vaso-occlusive crises compared to their counterparts. The presence of obstructive adenotonsillar hypertrophy in children with sickle cell disease increases sickle cell morbidity, more attention to these children through routine screening, diagnosis, and timely therapeutic intervention could reduce the morbidity.