BACKGROUND:
The association between Moyamoya disease and sickle cell disease is well recognized in the literature. However, there is limited data on inpatient admission of concurrent sickle cell disease and Moyamoya disease. We sought to determine the trend in incidence of admissions of concurrent Moyamoya and sickle cell disease as well as the most common presentation of these admissions.
METHODS:
We reviewed the Healthcare Cost and Utilization Project's Nationwide Inpatient Sample (NIS) database from 2000-2011 for concurrent Moyamoya and sickle cell admissions using the ICD 9-CM codes We obtained data on gender, clinical presentation, procedures, co-morbidities and patient outcomes
RESULTS:
From 2000 to 2011, an estimated patients 756 (weighted (n)=3692) with co-existing Moyamoya disease and sickle cell disease were admitted. The incidence of admission for concurrent disease increased significantly from 0.04/100,000 admissions in 2000 to 0.21/100,000 admissions in 2011(figure 1). This was very significant using the Cochrane Armitage trend test(p<0.001). The most likely reasons for admissions were ischemic stroke(7.2)% followed by hemorrhagic stroke(2.8 and transient ischemic attack(1.2%) (p = 0.0116). The most commonly performed treatment procedures included packed cell transfusion(33.7%) followed by exchange transfusion (8.8%). However there was no significant change in mortality from 2000-2011.
CONCLUSION:
The number of hospitalizations due to concurrent Moyamoya and sickle cell disease has increased significantly over the last decade and are likely to present with a cerebrovascular accident. Thus, it is important to maintain a high degree of suspicion for Moyamoya disease in sickle cell disease patients presenting with neurological symptoms since this could potentially impact their management.
Response to Letter to the Editor: Impact of Bariatric Surgery on Outcomes of Patients with Sickle Cell Disease: a Nationwide Inpatient Sample Analysis, 2004–2014
