Background Infantile hemangioma (IH) is the most common benign tumor of infancy. For children with IH who require treatment, propranolol and other beta blockers have been shown to be safe and effective. Although consensus guidelines for propranolol have been published, anecdotal experience suggests that there remain variations in management. This study was performed to document these variations amongst providers and to identify areas for future research. Methods We conducted an internet-based survey of clinicians who treat patients with IH. Characteristics of respondents were collected. Hypothetical cases and management scenarios were presented and respondents were ask to comment on dosing, monitoring, frequency of follow-up, duration of therapy, whether to taper or abruptly discontinue mediation, and which patients should get additional evaluation. Results Twenty-nine respondents participated in the survey: pediatric hematologists/oncologists (n= 15), pediatric cardiologists (n= 10), dermatologists (n = 2), an ophthalmologist (n = 1), and a neonatologist (n = 1). Most respondents use generic propranolol in infants with growing IH of the head and neck, with a goal dose of 2 mg/kg/day, until approximately one year of age. A variety of management strategies were documented including which patients should be treated, optimal dose and duration of therapy, how patients should be monitored, which patients should get additional work up, how propranolol should best be discontinued, and how often to see patients in follow-up. Conclusions This study demonstrates wide practice variations in managing patients with infantile hemangioma. Further research is indicated to address these variations and develop additional/updated evidence-based guidelines.

A document by Denise Adams. Click on the document to view its contents.

Background: While rare in children, chylothorax is a significant cause of respiratory morbidity and can lead to malnutrition and immunodeficiency. Historically, the traditional pharmacological treatment has been octreotide. There are several treatments that have been utilized in the past few years including sirolimus, however data regarding their efficacy and outcomes is limited. Furthermore, sirolimus has proven efficacy in complex vascular malformations, and hence, its utility/efficacy in pediatric chylous effusions warrants further investigation. Methods: In this retrospective study at Texas Children’s Hospital, data were extracted for all patients with chylothorax who were treated with sirolimus between 2009 and 2020. Details regarding underlying diagnosis, co-morbidities and number of days from sirolimus initiation to resolution of effusion were collected. Descriptive statistics were used to analyze the study cohort. Results: Initially a total of twelve infants were identified. Among them, seven patients had complete data and were included in the study. The mean duration of sirolimus treatment needed for chest tube removal was 16 days, with a median of 19 days and range of 7- 22 days. Chest tube output corresponded with sirolimus serum trough levels and trended down prior to chest tube removal. Conclusion: With close monitoring, sirolimus is a safe and effective therapy for pediatric lymphatic effusions even in critically-ill infants. The study also demonstrates shorter duration of chest tube requirement after initiation of sirolimus compared to previous studies. Our conclusion is based on a small case series due to the rare incidence of the condition.

Complex lymphatic anomalies are congenital diseases of the lymphatic circulation system that are associated with significant morbidity and early mortality. While guidelines for the comprehensive evaluation of the complex lymphatic anomalies were recently published, the diagnostic approach and medical management are not standardized. The current manuscript presents the clinical features of 4 complex lymphatic anomalies: Gorham-Stout disease, generalized lymphatic anomaly, kaposiform lymphangiomatosis, and central collecting lymphatic anomaly. We also offer three cases from the authors’ practice and our views on diagnostic testing and disease management including supportive care, medical therapies and other interventions.