3.4 Management
Of the twenty-four patients, 23 (95.8%) required an intervention at
some point during their clinical course. Twenty-one, 87.5%, were
managed initially with sclerotherapy. Most interventions were performed
for abdominal symptoms before 3 years old or during adolescence. Ten
patients (41.7%) underwent laparoscopic or open surgery. Two patients
(8.3%) underwent surgery as their primary therapy. 13 of 19 patients
(68.4%) who underwent sclerotherapy did not require further invasive
intervention. Four patients, 21%, received sclerotherapy and then
underwent surgical resection for progressive disease. Six patients
(25%) were treated with sirolimus at some point during their clinical
course.