Beckwith-Wiedemann syndrome with multiple hepatic and cutaneous hemangiomas in a female patient of Albanian origin: Diagnostic and therapeutic considerations.

We describe a 2-month-old female infant with macroglossia, macrosomia, omphalocele, neonatal hypoglycemia, earlobe creases, low nasal bridge, midface retrusion, syndromic facies and multiple cutaneous and hepatic hemangiomas (HH). Genetic evaluation confirmed the diagnosis of Beckwith-Wiedemann Syndrome (BWS) with mosaic uniparental disomy 11 as the underlying genetic mechanism suggested by partial hypermethylation of H19/IGF2:IG-DMR and partial hypomethylation of KCNQ1OT1:TSS-DMR on chromosome 11p15.5. Pediatric endocrinology and cardiology assessments were normal. No malignant liver or renal tumors were detected during the follow-up period. Treatment with propranolol was started for the multiple HH, according to international recommendations. At 3-, 6-, and 9-month follow up, a gradual decrease in the size of the hemangiomas and AFP levels was observed, without side effects. This is the fifth case in the literature combining HH and BWS, and among these, the third case with this specific genetic defect suggesting a possible association between HH and BWS caused by 11 paternal uniparental disomy [upd(11)pat]. The case also highlights that if treatment is warranted, then oral propranolol can be used for the management of infantile HH in BWS patients similarly to non-BWS patients.

Laparoscopic liver resection or enucleation for giant hepatic hemangioma: how to choose?

BACKGROUND: Laparoscopic treatment has been increasingly adopted for giant hepatic hemangioma (HH), but the role of liver resection or enucleation remains uncertain. The aim of this study is to compare the laparoscopic resection (LR) with laparoscopic enucleation (LE) for HH, and to provide evidence on how to choose the most suitable approach for HH. METHODS: A retrospective analysis of HH patients underwent laparoscopic treatment between March 2015 and August 2022 was performed. Perioperative outcomes were compared based on the surgical approaches, and risk factors for increased blood loss was calculated by logistic regression analysis. RESULTS: A total of 127 patients in LR group and 287 patients in LE group were enrolled in this study. The median blood loss (300 vs. 200 mL, P < 0.001) was higher in LE group than that in LR group. Independent risk factors for blood loss higher than 400 mL were tumor size ≥ 10 cm, tumor adjacent to major vessels, tumor occupying right liver or caudate lobe, and the portal phase enhancement ratio (PER) ≥ 38.9%, respectively. Subgroup analysis showed that LR was associated with less blood loss (155 vs. 400 mL, P < 0.001) than LE procedure in patients with high PER value. Both LR and LE approaches exhibited similar perioperative outcomes in patients with low PER value. CONCLUSIONS: Laparoscopic treatment for HH could be feasibly and safely performed by both LE and LR. For patients with PER higher than 38.9%, the LR approach is recommended.

Clinical impact and role of major vessels involvement in laparoscopic resection for hepatic hemangioma.

BACKGROUND: Severe bleeding remains a significant concern in laparoscopic resection for hepatic hemangioma. It is rarely reported that how the degree of major vessels involvement impacts on severe bleeding. The present study primarily aimed to analyze the impacts of the number of involved major vessels (NIMV) during laparoscopic surgery for hepatic hemangioma and evaluate the risk factors associated with increased bleeding. METHODS: A database search was carried out for consecutive patients who underwent laparoscopic resection for liver hemangiomas at our department from January 2018 to December 2023. The collected data included demographics, characteristics of the hemangiomas, laboratory data, operation method, surgical and postoperative variables. RESULTS: A total of 72 patients were enrolled in the study. 42 patients were categorized into the group with NIMV < 2, while 30 patients were divided into the group with NIMV ≥ 2. The group with NIMV ≥ 2 demonstrated a significant correlation with special segments, involved multiple segments and diameter of the hemangiomas (P < 0.01). And the perioperative variables including the extent of resection, operative time, blood loss, Pringle maneuver times, postoperative stay, drainage tube duration, and postoperative liver function (ALT, AST) also showed significant differences between the two groups (P < 0.05). Notably, NIMV ≥ 2 was identified as the most important independent risk factor for intraoperative blood loss ≥ 500 ml in laparoscopic surgery for hepatic hemangioma (P = 0.011). For NIMV ≥ 2, the independent risk factor was special segments in multivariate analysis (P = 0.000). CONCLUSION: The involvement of multiple major vessels (NIMV ≥ 2) was significantly associated with special segments, resulting in increased intraoperative blood loss, operation difficulty, and delayed postoperative recovery. Moreover, it was identified as the single independent risk factor with a considerable risk for increased blood loss during laparoscopic resection for hepatic hemangioma.

Postnatal treatment and evolution patterns of giant fetal hepatic hemangioma: a case series of 29 patients.

OBJECTIVES: To explore the clinical characteristics, postnatal treatment and prognosis of giant fetal hepatic hemangioma (GFHH). METHOD: Retrospective analysis was performed on children with giant fetal hepatic hemangioma (maximum tumor diameter > 40 mm) diagnosed by prenatal ultrasound and MRI from December 2016 to December 2020. These patients were observed and treated at the Children's Hospital of Fudan University after birth. The clinical data were collected to analyze the clinical characteristics, treatment, and prognosis of GFHH using independent sample t tests or Fisher's exact tests. RESULTS: Twenty-nine patients who were detected by routine ultrasound in the second and third trimester of pregnancy with giant fetal hepatic hemangiomas were included. The first prenatal ultrasound diagnosis of gestational age was 34.0 ± 4.3 weeks, ranging from 22 to 39 weeks. Of the patients, 28 had focal GFHHs and 1 had multifocal GFHHs. Surgery was performed, and the diagnosis was confirmed histopathologically in two patients. There were 8 cases with echocardiography-based evidence of pulmonary hypertension, 11 cases had a cardiothoracic ratio > 0.6, and 4 cases had hepatic arteriovenous fistula (AVF). The median follow-up time was 37 months (range: 14-70 months). During the follow-up, 12 patients received medical treatment with propranolol as the first-line therapy. The treatment group had a higher ratio of cardiothoracic ratio > 0.6 (P = 0.022) and lower albumin levels (P = 0.018). Four (14.8%) lesions showed postnatal growth before involuting. Complete response was observed in 13 (13/29) patients, and partial response was observed in 16 (16/29) patients. CONCLUSIONS: Fetal giant hepatic hemangioma is mainly localized, and its clinical outcome conforms to RICH (rapidly involuting) and PICH (partially involuting), but some fetal giant hepatic hemangiomas will continue to grow after birth and then gradually decrease. For uncomplicated giant fetal hepatic hemangioma, postnatal follow-up is the main concern, while those with complications require aggressive medical treatment. Propranolol may have no effect on the volume change of GFHH.

Spontaneous rupture of neonatal hepatic hemangioma diagnosed by ultrasound: A case report.

Hepatic hemangioma is the most prevalent benign liver tumor during the fetal and neonatal period, and its rupture poses a severe threat to newborns' lives-this article presents a case involving the spontaneous rupture of a hepatic hemangioma in a neonate. Early diagnosis through ultrasound enabled prompt treatment, resulting in the patient's timely discharge.

Hepatic Hemangioma: Review of Imaging and Therapeutic Strategies.

Hepatic hemangiomas are the most common benign liver tumors. Typically, small- to medium-sized hemangiomas are asymptomatic and discovered incidentally through the widespread use of imaging techniques. Giant hemangiomas (>5 cm) have a higher risk of complications. A variety of imaging methods are used for diagnosis. Cavernous hemangioma is the most frequent type, but radiologists must be aware of other varieties. Conservative management is often adequate, but some cases necessitate targeted interventions. Although surgery was traditionally the main treatment, the evolution of minimally invasive procedures now often recommends transarterial chemoembolization as the treatment of choice.

Differentiation of hepatic alveolar echinococcosis with a hemangioma-like pattern compared to typical liver hemangioma using contrast-enhanced ultrasound: a pilot study.

PURPOSE: Echinococcus multilocularis infects humans as a false intermediate host, primarily with intrahepatic manifestation. Incorrect diagnostic interpretation of these liver tumors, especially the hemangioma-like pattern, can lead to progressive disease. The aim of the study was to investigate the differentiation of typical hemangioma and a hemangioma-like pattern of E. multilocularis using contrast-enhanced ultrasound (CEUS). METHODS: This prospective clinical pilot study comprised patients with hemangioma (n = 14) and patients with alveolar echinococcosis (AE) and hemangioma-like pattern (n = 7). Inclusion criteria were the detection of a liver lesion according to a hemangioma-like pattern on E. multilocularis Ulm classification-ultrasound (EMUC-US) and "confirmed" or "probable" AE according to WHO case definition. The comparison group had hepatic hemangioma with typical B-scan sonographic morphology. All participants underwent conventional and contrast-enhanced ultrasonography. RESULTS: The patient group comprised five men (71.4%) and two women (28.6%) with a mean average age of 64.1 ± 11.2 years. The patient group with hemangioma comprised nine female subjects (64.3%) and five male subjects (35.7%) with a mean average age of 56.1 ± 12.0 years. Early arterial bulbous ring enhancement (p < 0.0001) and iris diaphragm phenomenon could only be visualized in the patients with hemangioma (p < 0.0001). Furthermore, the patients with hemangioma exhibited hyperenhancement in the late phase (p = 0.0003). In contrast, the patients exhibited typical early arterial rim enhancement (p < 0.0001) and, in the portal venous and late phase, complete or incomplete non-enhancement (black hole sign; p = 0.0004). CONSLUSION: The behavior of hemangioma-like AE lesions and typical liver hemangiomas is significantly different on CEUS. AE should be considered as a possible differential diagnosis, especially in high-endemic areas.

Effectiveness of microwave ablation for the treatment of hepatic hemangioma - meta-analysis and meta-regression.

BACKGROUND: The treatment of hepatic hemangioma includes surgical resection, radiofrequency ablation and Transarterial embolization. However, complications, mortality and compromised effectiveness limit their applications. Microwaves with effective heating generation and short ablation time become a promising treatment. The aim of this study is to conduct systematic review and meta-analyses to evaluate the effectiveness of Microwave Ablation (MWA) for the treatment of hepatic hemangioma. METHODS: A systematic literature review was conducted in PubMed. Main outcomes were defined as hemangioma decreases in diameters and volume changes post-MWA. Conventional random-effect meta-analysis technique was applied to analyze the pooled data, and meta-regression model was established to explore the association among factors. RESULTS: There were nine studies with a total of 501 patients retrieved. The pooled estimate of mean differences and 95% CI of hemangioma decreases after MWA treatment in diameter and in volume change (%) were 3.009 cm and (1.856, 4.161), and 53.169% and (51.274, 55.065), respectively. The pooled estimates of liver enzyme, ALT and AST, elevation were 219.905 with 95%CI (160.860, 278.949) and 315.679 with 95%CI (226.961, 404.397), respectively. Major complications were defined as acute kidney injury (AKI), pleural effusion, diaphragmatic hernia, and jaundice that needed to be treated, and the pooled incidence was 0.017 with 95% CI of (0.006, 0.029). No mortality related to MWA was reported. Meta-regression showed ablation time was associated with pre-operative lesion size (p = .001). CONCLUSION: MWA is effective and safe in treatment of hepatic hemangioma, and our study suggests that hemangioma size should be investigated in the future MWA pretreatment difficulty scoring system study.

MRI-based deep learning model for differentiation of hepatic hemangioma and hepatoblastoma in early infancy.

Hepatic hemangioma (HH) and hepatoblastoma (HBL) are common pediatric liver tumors and present with similar clinical manifestations with limited distinguishing value of serum AFP in early infancy. An accurate differentiation diagnostic tool is warranted for optimizing treatments and improving prognosis. The present study aimed to develop an innovative and cost-effective diagnostic tool to differentiate HH and HBL in early infancy using advanced deep learning (DL) techniques. One hundred forty patients ≤4 months old diagnosed as HH or HBL with histological specimens were recruited from two institutions assigned into a training set with cross-validation and a testing set for external validation, respectively. Based on MRI images, imaging diagnoses were interpreted by two radiologists, and imaging-derived radiomic features were extracted by pretrained convolutional neural networks (CNNs)-Xception extractor via DL analysis. A nomogram model was constructed integrating predictive clinical variables, radiologist-based interpretation, and DL features, evaluated comprehensively on diagnostic and calibration accuracy. The DL-based model performed an area under the receiver operating characteristic curve (AUC) of 0.966 for the training cohort and 0.864 for the testing cohort. The radiologist-interpreted differentiation model showed an AUC of 0.837 in the testing cohort. The integrated nomogram model represented an increasing performance with an AUC of 0.887, accuracy of 78.57%, sensitivity of 76.19%, and specificity of 80.95% in the testing cohort. CONCLUSION:  The MRI-based integrated model, a noninvasive preoperative diagnostic tool, yielded favorable efficacy for differentiating HH and HBL in early infancy, which might reduce the patients' costs of repetitive and unnecessary examinations or over-treatment. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05170282. WHAT IS KNOWN: • Hepatic hemangioma (HH) and hepatoblastoma (HBL) are common pediatric liver tumors and present with similar clinical manifestations with limited distinguishing value of serum AFP in early infancy. • Considering the rare incidence of infantile hepatic tumors, the distinguishing accuracy between HBL and HH for cases in early infancy is unsatisfactory for radiologists' recognition solely. WHAT IS NEW: • The MRI-based integrated model, a noninvasive preoperative diagnostic tool yielded favorable efficacy for differentiating HH and HBL in early infancy, which might reduce the patients' costs of repetitive and unnecessary examinations or over-treatment.

Mortality predictive factors in congenital hepatic hemangioma: a case-control study.

Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case-control study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival. Demographic, gestational, imaging, and laboratory data at diagnosis were collected and compared between both groups. A total of 29 patients were included (12 males; 17 females) of whom 5 died as a result of CHH evolution due to cardiac failure and coagulopathy, with a median age of 11 days until death. No differences in demographic or gestational data were reported. There were neither differences when comparing imaging tests, nor in location, number of affected liver segments, or CHH estimated volume. Upon laboratory data at diagnosis, deceased patients had a significant elevation of median liver enzymes [glutamic-oxaloacetic transaminase (359 u/L vs. 45 u/L; p < 0.01) and glutamic-pyruvic transaminase (313 u/L vs. 20 u/L; p < 0. 01)], as well as a decreased median platelet count (85,250/µL vs. 337,000/µL; p < 0.01), prothrombin activity (54% vs. 93%; p < 0.01), and fibrinogen (131 mg/dL vs. 284 mg/dL; p < 0.01), with no differences in blood count or biochemistry data. CONCLUSIONS: CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality. WHAT IS KNOWN: • Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening. WHAT IS NEW: • Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients.

Unusual presentation of a case of fetal hepatic mass: a case report.

BACKGROUND: Giant hepatic hemangiomas are rare and can cause serious complications that contribute to a high risk of perinatal mortality. The purpose of this article is to review the prenatal imaging features, treatment, pathology, and prognosis of an atypical fetal giant hepatic hemangioma and to discuss the differential diagnosis of fetal hepatic masses. CASE PRESENTATION: A gravida 9, para 0 woman at 32 gestational weeks came to our institution for prenatal ultrasound diagnosis. A complex, heterogeneous hepatic mass measuring 5.2 × 4.1 × 3.7 cm was discovered in the fetus using conventional two-dimensional ultrasound. The mass was solid and had both a high peak systolic velocity (PSV) of the feeding artery and intratumoral venous flow. Fetal magnetic resonance imaging (MRI) revealed a clear, hypointense T1-W and hyperintense T2-W solid hepatic mass. Prenatal diagnosis was very difficult due to the overlap of benign and malignant imaging features on prenatal ultrasound and MRI. Even postnatally, neither contrast-enhanced MRI nor contrast-enhanced computed tomography (CT) was useful in accurately diagnosing this hepatic mass. Due to persistently elevated Alpha-fetoprotein (AFP), a laparotomy was performed. Histopathological examination of the mass showed atypical features such as hepatic sinus dilation, hyperemia, and hepatic chordal hyperplasia. The patient was ultimately diagnosed with a giant hemangioma, and the prognosis was satisfactory. CONCLUSIONS: When a hepatic vascular mass is found in a third trimester fetus a hemangioma should be considered as a possible diagnosis. However, prenatal diagnosis of fetal hepatic hemangiomas can be challenging due to atypical histopathological findings. Imaging and histopathological assays can provide useful information for the diagnosis and treatment of fetal hepatic masses.

Congenital hepatic hemangioma: an unusual case report of pulmonary hypertension.

BACKGROUND: Pulmonary hypertension (PH) in newborns is a rare but serious condition that often requires immediate intervention and quick diagnosis of the correct etiology to prevent mortality. Congenital hepatic hemangioma (CHH) is an example of an extrathoracic etiology of PH. CASE PRESENTATION: Herein, we report the case of a newborn with a giant liver hemangioma, who presented with an early onset of PH and was successfully treated with intra-arterial embolization. CONCLUSIONS: This case illustrates the importance of suspicion and prompt evaluation of CHH and related systemic arteriovenous shunts among infants with unexplained PH.

Risk factors analysis of surgical complications of hepatic hemangioma: a modified Clavien-Dindo classification-based study.

PURPOSE: There are few studies on the risk factors of postoperative complications after surgical treatment of hepatic hemangioma (HH). This study aims to provide a more scientific reference for clinical treatment. METHODS: The clinical characteristics and operation data of HH patients undergoing surgical treatment in the First Affiliated Hospital of Air Force Medical University from January 2011 to December 2020 were retrospectively collected. All enrolled patients were divided into two groups based on the modified Clavien-Dindo classification: Major group (Grade II/III/IV/V) and Minor group (Grade I and no complications). Univariate and multivariate regression analysis was used to explore the risk factors for massive intraoperative blood loss (IBL) and postoperative Grade II and above complications. RESULTS: A total of 596 patients were enrolled, with a median age of 46.0 years (range, 22-75 years). Patients with Grade II/III/IV/V complications were included in the Major group (n = 119, 20%), and patients with Grade I and no complications were included in the Minor group (n = 477, 80%). The results of multivariate analysis of Grade II/III/IV/V complications showed that operative duration, IBL, and tumor size increased the risk of Grade II/III/IV/V complications. Conversely, serum creatinine (sCRE) decreased the risk. The results of multivariate analysis of IBL showed that tumor size, surgical method, and operative duration increased the risk of IBL. CONCLUSIONS: Operative duration, IBL, tumor size, and surgical method are independent risk factors that should be paid attention to in HH surgery. In addition, as an independent protective factor for HH surgery, sCRE should attract more attention from scholars.

Clinical and Pathological Features of Congenital Hepatic Hemangioma in Children: A Retrospective Analysis.

Objective: We reviewed our experience with congenital hepatic hemangiomas (CHH) to assess the effectiveness of our treatment strategies. Methods: Clinical and pathologic features of children with CHH were reviewed. Results: Twenty-two cases of CHH were collected, 17 were resected and 5 were followed until resolution. In 17 with alpha-feto-protein (AFP) levels, 9 were elevated with 5 decreasing to normal before surgery. In six with tumors under 3 cm, five regressed between 1 and 13 months, one required removal 5 years after initial diagnosis. Postoperative histopathology of 17 cases showed abnormal vascular hyperplasia without lobular architecture. CD34 was expressed in all tumors, glucose transporter 1(Glut1) was negative. Conclusion: If the tumor is less than 3 cm, AFP is normal and there are no obvious complications, the lesion can be followed with regular assessment imaging. Surgical resection is an option in tumors less than 3 cm which have not regressed over time.

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The three-day-old female infant was admitted to the hospital due to respiratory distress after birth. She was born premature at 36+2 weeks gestational age. Prenatal ultrasound suggested abnormal development of the fetal liver vessels, and she had dyspnea that required respiratory support after birth. Chest X-ray indicated an enlarged cardiac silhouette, and cardiac ultrasound revealed enlargement of the right atrium and right ventricle. Diagnosis of hepatic hemangioma with arteriovenous fistula was confirmed through liver ultrasound and abdominal enhanced CT. At 19 days old, she underwent ligation of the hepatic artery under general anesthesia, which led to an improvement in cardiac function and she was subsequently discharged. Genetic testing revealed a mutation in the ACVRL1 gene, which was inherited from the mother. The article primarily introduces a case of neonatal heart failure caused by hepatic hemangioma with arteriovenous fistula, and multi-disciplinary diagnosis and treatment of this disease.

Advantages of laparoscopic hepatic hemangioma surgery in quality of life: a prospective study.

BACKGROUND: Surgical intervention is the most accurate method for the treatment of hepatic hemangioma. The advantages of laparoscopic surgery on quality of life should be clarified by prospective studies. METHODS: The sample sizes of the laparoscopic and open surgery groups were calculated based on previous retrospective literature. Intraoperative and postoperative parameters were prospectively collected and analyzed. Quality of life in both groups was predicted by a mixed linear model. RESULTS: Sixty patients were enrolled in the laparoscopic surgery group and open surgery group. The laparoscopic group had a longer operation time (P = 0.040) and more hospitalization expenses (P = 0.001); however, the Clavien-Dindo classification and comprehensive complication index suggested a lower incidence of surgical complications in the laparoscopic group, with P values of 0.049 and 0.002, respectively. After mixed linear model prediction, between-group analysis indicated that the laparoscopic group had little impact on role-physical functioning and role-emotional functioning; in addition, within-group analysis showed a rapid recovery time on role-physical functioning and role-emotional functioning in the laparoscopic group. Quality of life in both groups recovered to the preoperative level within 1 year after the operation. CONCLUSION: The advantages of laparoscopic hepatectomy for hepatic hemangioma were fewer postoperative complications, lower impact on quality of life and faster recovery from affected quality of life.

Safety and effectiveness of laparoscopic intratumoral resection facilitated by coagulation of giant hepatic hemangioma: a matched case-control study and literature review.

BACKGROUND: To report the safety and effectiveness of laparoscopic intratumoral resection facilitated by coagulation (LIRC) compared with laparoscopic hepatectomy (LH) in treating giant hepatic hemangioma. METHODS: From 2017 to 2020, 19 consecutive patients with giant hepatic hemangioma (≥ 10 cm) received LIRC in one center. We selected a subgroup of 103 patients treated by LH in other four centers who well matched the 19 consecutive patients treated with LIRC, in a 1:1 fashion based on the tumor location, tumor size, and body mass index. Furthermore, the differences in technical success, operative time, operative blood loss, change of laboratory indexes, hospital stays, complication and clinical responds are compared between the two groups. RESULTS: Technical success was achieved in all 38 patients. Patients in the LIRC group had a relative shorter operative time (P < 0.001) and less operative blood loss (P = 0.003). The serum levels of C-reactive protein (CRP), total bilirubin (TBil), alanine aminotransferase (ALT), and aspartate transaminase (AST) were elevated significantly (P < 0.05) 1 day after the resection and returned to normal within 7 days in both groups; however, relatively lower serum levels of those indexes were observed in the LIRC group (P < 0.05). The total complication rate was relatively lower in the LIRC group compared with the LH group (P = 0.029). Patients in the LIRC group had shorter hospital stays than those in the LH group (P = 0.010). The clinical response was similar in the two groups. CONCLUSIONS: LIRC is safe and effective for treating giant hepatic hemangioma.

Spontaneous evolution patterns of focal congenital hepatic hemangiomas: a case series of 25 patients.

BACKGROUND: Hepatic hemangiomas are the most common benign liver tumors of infancy. They are termed congenital if fully developed at birth or infantile if they appear in the first weeks of life. Previous studies suggested that most focal hepatic hemangiomas are congenital in nature, exhibit no postnatal growth and have an evolution that parallels their cutaneous counterparts. They are subdivided by pattern of involution, whether rapidly involuting (RICH), partially involuting (PICH) or non-involuting (NICH) congenital hemangiomas. In our experience, some focal hepatic hemangiomas show postnatal growth, behaving like infantile forms. OBJECTIVES: To analyze the spontaneous evolution of focal congenital hepatic hemangiomas with quantification of tumor volume changes over time and to identify initial postnatal ultrasound (US) imaging biomarkers predictive of their evolution pattern. MATERIALS AND METHODS: A retrospective review of clinical, imaging and pathology data of children diagnosed with focal congenital hepatic hemangioma (prenatal diagnosis or age at diagnosis <7 days and/or glucose transporter protein 1 [GLUT1]-negative tumor) diagnosed between 2000 and 2018 was performed with analysis of tumor volume changes over time. Exclusion criteria were treatment inducing a tumor volume change (hepatic artery embolization, propranolol, or corticosteroids), imaging follow-up less than 1 month or fewer than two US examinations. Volumetric analysis was based on US and cross-sectional imaging. Lesion volumes were estimated using the standard ellipsoid formula. A 35% margin of error was assumed for tumor volume variation to account for variability in measurements. Imaging studies, including US, computed tomography, and magnetic resonance imaging, were reviewed and initial postnatal US features were correlated with evolution pattern. RESULTS: Twenty-five patients with focal congenital hepatic hemangiomas were included. The median follow-up time was 46.5 months (range: 4-144 months). Eight (32%) lesions showed postnatal growth before involuting, without signs of intralesional hemorrhage, as do cutaneous infantile hemangiomas. The other 17 (68%) lesions exhibited a strict decrease in volume with age, of which 15 underwent complete involution (8 before age 18 months and 7 after age 18 months) and 2 underwent partial involution. The different evolution patterns of focal congenital hepatic hemangiomas showed overlapping imaging features and we found no initial US feature to be significantly associated with postnatal growth. However, large vascular spaces with marked vascularity at US were noted in three of the eight rapidly involuting lesions. CONCLUSION: Focal congenital hepatic hemangiomas are not the equivalent of cutaneous RICH, as some may increase in size and tumor regression may be rapid or slow. The different evolution patterns of focal congenital hepatic hemangiomas show overlapping US features.

Efficacy of intravenous propranolol for life-threatening diffuse neonatal hemangiomatosis.

Oral propranolol is recommended as the first-line agent for infantile hemangioma requiring systemic treatment. Life-threatening complications such as obstructive infantile hemangioma of the airway or infantile hepatic hemangioma associated with high-output congestive heart failure are major indications for the consideration of early treatment. We present the case of an infant with life-threatening diffuse neonatal hemangiomatosis, including airway obstruction due to subglottic hemangioma, heart failure due to multiple hepatic hemangiomas with portohepatic venous shunts, and severe anemia due to continuous gastrointestinal bleeding, in which treatment with intravenous propranolol proved successful.

Clinical evaluation of transcatheter arterial embolization combined with propranolol orally treatment of infantile hepatic hemangioma.

PURPOSE: To assess the efficacy of transcatheter arterial embolization (TAE) plus propranolol treatment for infantile hepatic hemangioma (IHH). METHODS: A retrospective study of symptomatic IHH and hemodynamic changes in IHH was conducted between 2016 and 2019. RESULTS: There were five boys and seven girls with diffuse lesions (n = 7) and multifocal lesions (n = 5). Hepatomegaly and abdominal distension (n = 6) were the predominant clinical presentations. Seven patients (58.3%) had multiple cutaneous hemangiomas. Pulmonary arterial hypertension, heart failure (n = 4), and hypothyroidism (n = 4) were observed. A total of 17 TAE procedures were performed in 12 IHH cases, with a technical success rate of 100%. All patients received standard propranolol orally, and one patient was orally administered metacortandracin. Two patients died of heart failure and multiple organ dysfunction caused by an enlarged liver. In addition, one patient was not reexamined after discharge. Of the remaining nine children, the average follow-up time was 10.78 months (range 2-28 months), and they all responded well to TAE combined with oral propranolol. CONCLUSION: TAE combined with propranolol is safe and effective for the treatment of IHH, demonstrating low complication rates.