RESUMO
Objective:To investigate the clinical features and outcomes of giant fetal hepatic hemangioma (GFHH).Methods:In this retrospective study, patients with GFHH from Guangzhou Women and Children's Medical Centre who received conservative management or medical intervention with a minimum of one year's follow-up after birth from August 2012 until March 2020 were enrolled. Prenatal and postnatal data were collected to analyze the pathological (size, location, and lesion type) and clinical manifestations, treatment, and prognosis of GFHH using independent sample t-test or Chi-square (or Fisher's exact) test. Results:(1) A total of 22 subjects were enrolled, including one preterm infant (4.5%), with an average gestational age of (38.9±1.5) weeks at birth, ranging from 33 +4 to 40 +3 weeks. Among them, 17 were male (77.3%), and five were female (22.7%). The gestational age at the first diagnosis was (35.0±2.8) weeks, ranging from 30 to 40 +1 weeks. Fifteen babies (68.2%) were born vaginally and seven (31.8%) by cesarean section due to fetal distress. Focal type accounted for 21 of the 22 (95.5%) patients, and multifocal type in one patient (4.5%). All the patients received B-ultrasonography on an average of 2.5 times (2-4 times) prenatally, which showed that the diameter of hepatic hemangioma increased with gestational age and reached its maximum at birth [56 mm (42-99 mm)]. Nine fetuses (40.9%) were diagnosed with GFHH with prenatal ultrasonography, and 12 with MRI. Among them, six fetuses were diagnosed with both ultrasonography and MRI. The other cases showed space-occupying lesions. (2) Ten babies received conservative management (conservative group), and 12 received drug therapy (treatment group). There was no significant difference between the two groups in gestational age at diagnosis or birth, birth weight, the maximum diameter of the tumor before birth, and location and classification of the lesions (all P>0.05). The incidence of thrombocytopenia in the treatment group was significantly lower than that in the conservative group (7/12 vs 0/10, Fisher's exact test, P=0.014). No abnormality of heart function or pulmonary hypertension was found in the conservative group, while two patients were complicated with pulmonary hypertension in the treatment group. The outcomes between the two groups were similar ( P>0.05). (3) During a median followed-up of 3.3 years (1.2-7.0 years), all patients survived, and none of the children in the conservative group received drug therapy. One of the ten patients in the conservative group had a complete tumor involution at two years old without recurrence, and the other nine had the tumor regressed in size. Besides oral propranolol, three of 12 cases in the treatment group also had dexamethasone at the early stage, two had rapamycin, and two received invasive therapy due to progressive enlargement of lesions at the early stage, with the lesions completely involuted in two cases at 1.7 and 5.5 years of age and regressed in the other ten cases. Conclusions:The diameter of GFHH increases with gestational age. Conservative management conservative is recommended for asymptomatic GFHH cases. For those affected with dyspnea and abdominal distention resulting in repeated vomiting, drugs therapy can be considered regardless of the test results, and invasive therapy is an option when drugs are ineffective. Some fetuses may have unsignificant imaging manifestations. Most children prenatally detected with giant hepatic hemangiomas can survive, and the tumor begins to regress after birth with a good long-term prognosis.