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Fine clinical differences between patients with multifocal and diffuse hepatic hemangiomas.
Journal of Pediatric Surgery 2016 December
BACKGROUND: It has been reported that multifocal and diffuse hepatic hemangiomas are true infantile hemangiomas for which a continuum probably exists. We determined the similarities and fine differences between the two types of hemangioma and identified the multifocal subgroup of type, which needs timely treatment.
MATERIAL AND METHODS: Twenty-four patients (4 males and 20 females; age 114±142days) with multifocal or diffuse hepatic hemangiomas who were treated between January 2000 and June 2015 were studied. For the multifocal type, patients were divided into countable (MC) and uncountable (MU) subgroups. The medical data were analyzed retrospectively.
RESULTS: The clinical presentations included hepatomegaly (n=11), dyspnea (n=7), heart failure (n=9), hypothyroidism (n=6), and anemia (n=1). There were 19 and 5 patients with multifocal and diffuse types, and 6 and 13 patients in the MC and MU groups, respectively. There were significant differences between the multifocal and diffuse types with respect to hepatomegaly, heart failure, dyspnea, and hypothyroidism. However, there was no difference between the MU group and the diffuse type except for hypothyroidism. Observation was commonly recommended for patients with the multifocal type, and their survival rate was clearly higher than for patients with the diffuse type. Both the MU and diffuse-type groups needed treatment, unlike MC patients. The complete remission rate was higher in the MC group than in the MU and diffuse-type groups. Patients with the diffuse type were more likely to die.
CONCLUSIONS: Patients with diffuse-type hepatic hemangioma are at high risk and need active treatment. The MU group for the multifocal type is unique and has high similarities to the diffuse type with respect to clinical presentation and treatment, which suggests that a continuum of the disease phenotypes exists.
LEVEL OF EVIDENCE: Case-control study, level III.
MATERIAL AND METHODS: Twenty-four patients (4 males and 20 females; age 114±142days) with multifocal or diffuse hepatic hemangiomas who were treated between January 2000 and June 2015 were studied. For the multifocal type, patients were divided into countable (MC) and uncountable (MU) subgroups. The medical data were analyzed retrospectively.
RESULTS: The clinical presentations included hepatomegaly (n=11), dyspnea (n=7), heart failure (n=9), hypothyroidism (n=6), and anemia (n=1). There were 19 and 5 patients with multifocal and diffuse types, and 6 and 13 patients in the MC and MU groups, respectively. There were significant differences between the multifocal and diffuse types with respect to hepatomegaly, heart failure, dyspnea, and hypothyroidism. However, there was no difference between the MU group and the diffuse type except for hypothyroidism. Observation was commonly recommended for patients with the multifocal type, and their survival rate was clearly higher than for patients with the diffuse type. Both the MU and diffuse-type groups needed treatment, unlike MC patients. The complete remission rate was higher in the MC group than in the MU and diffuse-type groups. Patients with the diffuse type were more likely to die.
CONCLUSIONS: Patients with diffuse-type hepatic hemangioma are at high risk and need active treatment. The MU group for the multifocal type is unique and has high similarities to the diffuse type with respect to clinical presentation and treatment, which suggests that a continuum of the disease phenotypes exists.
LEVEL OF EVIDENCE: Case-control study, level III.
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