Patient Characteristics
These 30 studies reported on 1181 total patients, of which 290 patients
had a total of 315 infections. Of note, one study only listed the number
of infections without specifying whether any patient had more than one
infection, so we counted each infection in this study as a unique
patient to avoid underestimating the number of patients experiencing
infections.24 Patients in all included studies
received sirolimus. Many studies lacked sufficient detail to determine
key demographics of patients with infections. For example, age was not
specified in 237/290 patients (82%). The remainder were younger than 1
year (n=16, 5%), 2 to 17 years (n=28, 10%), or older than 18 years
(n=9, 3%). Similarly, sex was not specified for 268/290 patients
(n=92%). The remainder were male (n=14, 5%) and female (n=8, 3%).
Similarly, these studies lacked granular detail about the sites of VA
disease (not specified in 83%) and whether sirolimus was held during
infections (not specified in 81%). Patients with infections for whom
they type of VA was reported (n=121/290) had a wide variety of VAs, with
slow flow vascular malformations (n=60, 50%) and kaposiform
hemangioendothelioma (KHE)/tufted angioma (n=24, 20%) most commonly
reported. Given the limited granular reporting, 58% of participants did
not have a specific VA diagnoses listed and instead were aggregated into
the category “multiple VAs” (Table 2).