Kristy L Rialon1, Rudy Murillo1, Rebecca D Fevurly1, Ann M Kulungowski1, Emily R Christison-Lagay1, David Zurakowski2, Harry P W Kozakewich3, Ahmad I Alomari4, Steven J Fishman5. 1. Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA; Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA. 2. Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA. 3. Department of Pathology, Boston Children's Hospital, Boston, MA 02115, USA; Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA. 4. Department of Radiology, Boston Children's Hospital, Boston, MA 02115, USA; Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA. 5. Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA; Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA. Electronic address: steven.fishman@childrens.harvard.edu.
Abstract
PURPOSE: Multifocal and diffuse hepatic hemangiomas are true infantile hemangiomas, which likely exist in a continuum. We reviewed our hepatic hemangioma registry to identify prognostic indicators for mortality. METHODS: Registry records entered between 1995 and 2012 were reviewed. Clinical characteristics were evaluated for prognostic significance using the multivariable Cox proportional hazards model. Survival data were analyzed using the Kaplan-Meier product-limit method. RESULTS: We identified 123 patients with multifocal (n=91) and diffuse (n=32) hepatic hemangiomas. Mortality was 16% (n=20); 40% (n=8) had multifocal and 60% (n=12) had diffuse lesions. A diagnosis of diffuse disease (hazard ratio: 9.9, 95% CI: 2.0-50.8, P=.002) and congestive heart failure (CHF) (hazard ratio: 3.9, 95% CI: 1.3-14.2, P=.031) were significant risk factors for mortality across the continuum; age at presentation, cardiomegaly, presence of shunts, and hypothyroidism were not statistically significant independent risk factors. Among patients with diffuse lesions, eight (67%) who died had abdominal compartment syndrome, which was also associated with mortality (P=.002). CONCLUSIONS: Hepatic hemangioma patients with CHF or diffuse disease are at higher risk for mortality. Patients with multifocal lesions without CHF may go undetected until lesions become diffuse. Aggressive treatment of symptomatic patients and close follow-up of asymptomatic patients may improve mortality.
PURPOSE: Multifocal and diffuse hepatic hemangiomas are true infantile hemangiomas, which likely exist in a continuum. We reviewed our hepatic hemangioma registry to identify prognostic indicators for mortality. METHODS: Registry records entered between 1995 and 2012 were reviewed. Clinical characteristics were evaluated for prognostic significance using the multivariable Cox proportional hazards model. Survival data were analyzed using the Kaplan-Meier product-limit method. RESULTS: We identified 123 patients with multifocal (n=91) and diffuse (n=32) hepatic hemangiomas. Mortality was 16% (n=20); 40% (n=8) had multifocal and 60% (n=12) had diffuse lesions. A diagnosis of diffuse disease (hazard ratio: 9.9, 95% CI: 2.0-50.8, P=.002) and congestive heart failure (CHF) (hazard ratio: 3.9, 95% CI: 1.3-14.2, P=.031) were significant risk factors for mortality across the continuum; age at presentation, cardiomegaly, presence of shunts, and hypothyroidism were not statistically significant independent risk factors. Among patients with diffuse lesions, eight (67%) who died had abdominal compartment syndrome, which was also associated with mortality (P=.002). CONCLUSIONS:Hepatic hemangiomapatients with CHF or diffuse disease are at higher risk for mortality. Patients with multifocal lesions without CHF may go undetected until lesions become diffuse. Aggressive treatment of symptomatic patients and close follow-up of asymptomatic patients may improve mortality.
Authors: Maria Gnarra; Gerald Behr; Alison Kitajewski; June K Wu; Sudha A Anupindi; Carrie J Shawber; Nick Zavras; Dimitrios Schizas; Chris Salakos; Konstantinos P Economopoulos Journal: World J Clin Pediatr Date: 2016-08-08