Infantile Hemangiomas Research Articles

Analysis of a Case of Airway Hemangioma in a 1-Month-Old Infant with Dyspnea – A Secondary Publication

Infantile hemangiomas are the most common benign tumors of infancy. However, hemangiomas located in the respiratory tract are rare and could cause life-threatening events due to airway obstruction. To date, the best recommended treatment for infantile hemangioma is oral propranolol as it exhibits faster effects with fewer adverse effects as compared to systemic corticosteroid therapy. In this study, we reported a case of a 1-month-old girl who was presented with respiratory symptoms and hemangioma on the scalp. The hemangioma extended from the right base of the skull to the thoracic inlet, causing inspiratory stridor and dyspnea. Treatment with oral propranolol was initiated and her symptoms regressed. Imaging showed regression of the hemangioma. This was a rare case of skin hemangioma found on the scalp, in which the hemangioma extended from the base of the skull to the subglottis, precipitating respiratory symptoms from airway obstruction. Based on this encounter, the presentation of skin hemangioma on the head, coupled with respiratory symptoms, necessitates the use of imaging studies, such as computed tomography, ultrasound, and magnetic resonance imaging to ascertain the extent of hemangioma.

ASSESSMENT OF THE THERAPEUTIC EFFICACY OF PROPRANOLOL FOR THE TREATMENT OF INFANTILE HEMANGIOMAS

Infantile hemangiomas (IHs) are common vascular tumors affecting pediatric populations, often necessitating medical intervention due to their potential for complications. Propranolol has emerged as a promising therapeutic option for IHs, although optimal dosing regimens and treatment outcomes remain areas of ongoing research. This prospective observational study aimed to evaluate the therapeutic efficacy, safety profile, and clinical characteristics associated with propranolol treatment in pediatric patients with IHs. Eighty infants aged one month to 1 year were included in the study, with IH occurrence and treatment initiation predominantly observed within the first 12 months of life. Propranolol was administered orally at a starting dose of 2 mg/kg/day, titrated up to 3-4 mg/kg/day as tolerated. Assessment of therapeutic response revealed significant reductions in IH size and vascularity, with 31.3% of patients demonstrating an excellent response and 37.5% showing a good response. Adverse effects were minimal, with diarrhea (22.5%) and decreased heart rate (7.5%) being the most commonly reported side effects. Anatomical distribution analysis identified the parotid region as the most common site of lesion occurrence, highlighting the heterogeneous nature of IHs. Overall, our findings support the favorable outcomes of propranolol therapy in pediatric patients with IHs, emphasizing the need for individualized treatment approaches tailored to patient-specific characteristics.

The CHIP Score: A Valuable Method for Determining Risk of Surgical Intervention for Hemangioma Patients

Background: Infantile hemangiomas (IHs) are the most common benign tumor of infancy. These tumors may have wide-ranging effects on functional and cosmetic outcomes for patients. With variations in recommendations for surgery among providers, it can be difficult to counsel patients and their families on potential surgical treatment. Through the development of the Case Western Hemangioma Intervention Predictor (CHIP) score, this study aims to improve the consistency of counseling regarding surgery and timing of surgical referral. Methods: This was a retrospective review of all patients (179) treated for IHs at a single tertiary care center over 17 years. Patients were divided equally into 2 cohorts. Descriptive statistics and correlation plots were performed on the first cohort to evaluate which disease factors (including size, location, and complications) and patient factors significantly correlated with the decision to pursue surgical treatment (surgical risk). These factors were used to form a CHIP score, which was then validated through logistic regression with length of medical management as a covariate against the second cohort of patients. Results: After controlling for length of medical management to treat IH, lack of medical management, functional impairment, and ulceration were found to be significantly correlated with the risk of surgical intervention ( P < .05). When validated against the second half of our cohort, a CHIP score of 3 (of a maximum score of 3) was found to have a specificity of 92% and a sensitivity of 81% in predicting risk of surgical intervention. Conclusion: IHs can have clinical characteristics that may be predictors of complexity and surgical intervention. The CHIP score can assist in educating patients and families on surgery as a treatment option and guiding appropriate referrals.

Massive spinal epidural infantile hemangioma, image findings, and treatment: a case report and review of literature

Spinal involvement of infantile hemangiomas is rare with the predilection to involve the epidural space. A proper diagnosis might be challenging due to the atypical location and variable/inconsistent use of the International Society for the Study of Vascular Anomalies (ISSVA) classification by radiologists, pathologists, and clinicians. A proper diagnosis of epidural infantile hemangioma is key due to the different aggressiveness of the treatment options with inconstant literature regarding the best available treatment. Herein, we present a case of a massive epidural infantile hemangioma successfully treated with only beta-blocker. We discuss the clinical, MRI, CT, ultrasound, and histological features of this lesion as we review the literature with the objective of addressing some of the confusion surrounding the subject.

Facial Segmental Infantile Haemangioma: A Cutaneous Mosaicism Quandary.

Mosaicism has long been considered the underlying mechanism of segmental infantile hemangiomas (SIH). This was a prospective pilot case-control study conducted with the objective to quantify the percentage overlap of silhouettes of facial SIH with those of Blaschko lines (the most well studied archetypical pattern of mosaicism on face) as compared to other mosaic disorders on face. Lesional silhouettes of 8 patients with SIH (Group A) and 6 patients with other facial dermatosis known to have blaschkoidal distribution (Group B), were overlapped on a standardized template with Blaschkoidal lines on the frontal view of face. The alignment was done via the auto align tool of Photoshop and the percentage of overlap was calculated with an online image comparison software (IMGonline.com.ua). There was a significant difference in mean overlap in Group A (72.92 ± 15.6 %) as compared to Group B (90.1 ± 4.3%; P=0.018). Hence, we concluded that facial SIH do not follow lines of Blaschko.

Construction and applications of the EOMA spheroid model of Kaposiform hemangioendothelioma

BackgroundKaposiform hemangioendothelioma (KHE) is a rare intermediate vascular tumor with unclear pathogenesis. Recently, three dimensional (3D) cell spheroids and organoids have played an indispensable role in the study of many diseases, such as infantile hemangioma and non-involuting congenital hemangiomas. However, few research on KHE are based on the 3D model. This study aims to evaluate the 3D superiority, the similarity with KHE and the ability of drug evaluation of EOMA spheroids as an in vitro 3D KHE model.ResultsAfter two days, relatively uniform morphology and high viability of EOMA spheroids were generated by the rotating cell culture system (RCCS). Through transcriptome analysis, compared with 2D EOMA cells, focal adhesion-related genes such as Itgb4, Flt1, VEGFC, TNXB, LAMA3, VWF, and VEGFD were upregulated in EOMA spheroids. Meanwhile, the EOMA spheroids injected into the subcutaneous showed more obvious KMP than 2D EOMA cells. Furthermore, EOMA spheroids possessed the similar characteristics to the KHE tissues and subcutaneous tumors, such as diagnostic markers (CD31 and LYVE-1), cell proliferation (Ki67), hypoxia (HIF-1α) and cell adhesion (E-cadherin and N-cadherin). Based on the EOMA spheroid model, we discovered that sirolimus, the first-line drug for treating KHE, could inhibit EOMA cell proliferation and downregulate the VEGFC expression. Through the extra addition of VEGFC, the effect of sirolimus on EOMA spheroid could be weakened.ConclusionWith a high degree of similarity of the KHE, 3D EOMA spheroids generated by the RCCS can be used as a in vitro model for basic researches of KHE, generating subcutaneous tumors and drug screening.

Pediatric orbital lesions: neoplastic extraocular soft-tissue lesions.

Pediatric neoplastic extraocular soft-tissue lesions in the orbit are uncommon. Early multimodality imaging work-up and recognition of the key imaging features of these lesions allow narrowing of the differential diagnoses in order to direct timely management. In this paper, the authors present a multimodality approach to the imaging work-up of these lesions and highlight the use of ocular ultrasound as a first imaging modality where appropriate. We will discuss vascular neoplasms (congenital hemangioma, infantile hemangioma), optic nerve lesions (meningioma, optic nerve glioma), and other neoplastic lesions (plexiform neurofibroma, teratoma, chloroma, rhabdomyosarcoma, infantile fibrosarcoma, schwannoma).

Interventional Radiology Management of Pediatric Liver Tumors

AbstractPediatric liver tumors are not only rare, they are also different from the adult liver tumors. Hepatoblastomas and hepatocellular carcinomas (HCCs) are the most common primary malignant tumors seen in children. Fibrolamellar HCC, sarcomas, cholangiocarcinoma, germ cell tumor, and rhabdoid tumor and metastatic tumors comprise the remainder of liver malignancies. Hepatic infantile hemangiomas, mesenchymal hamartoma, regenerative nodules, adenomas, and focal nodular hyperplasia are the common benign liver tumors of childhood. The Pediatric Hepatic International Tumour Trial (PHITT) is an international trial with the goal of evaluating new and existing treatments for pediatric liver tumors. Chemotherapy, surgery, and liver transplantation are well-established in the management of pediatric liver tumors. In the background of increasing incidence of pediatric tumors, and availability of newer, less invasive modalities to treat tumors, it is vital to understand the role of interventional radiology in the management of pediatric liver tumors.

Hemangioma activity score evaluation in infantile hemangioma patients: a retrospective study

Introduction: Infantile hemangioma (IH) is a benign vascular tumor commonly found in children. Generally, no special therapy is required because spontaneous resolutions occur. However, in some cases, therapy is required due to the potential of complications. During therapy, the side effects and effectiveness of therapy must be evaluated. One of the assessment tools is the Hemangioma Activity Score (HAS). HAS is a simple and objective assessment to evaluate the successful treatment of IH by observing swelling, color, and ulceration. The use of HAS in Indonesia is still minimal. This study aims to evaluate HAS before and after therapy in IH patients at Dr. Soetomo General Academic Hospital and Surabaya Skin Centre. Methods: Twenty-seven patient medical records were selected based on the inclusion and exclusion criteria. This study is an analytic cross-sectional retrospective using consecutive sampling. The collected data were tested for suitability using Cohen's Kappa. Pre- and post-tests were carried out using the Shapiro-Wilk and Wilcoxon tests afterward. Results: Among 27 IH cases occurred in females (81.4%), with the youngest age of the patient being 1 month and the oldest being 8 years. The onset of lesions in all patients appeared before 1 year of age. The most frequent location for IH lesions was the facial area (51.8%), with a clinical manifestation being plaque (37%). The most common therapy used in this study was pulse dye laser (PDL) (48.1%). Analysis of the difference in HAS before and after therapy showed p value = 0.00, a statistically significant difference in HAS before and after therapy. Conclusions: The hemangioma activity score is an effective measurement tool for measuring the severity of IH and evaluating the effectiveness of therapy.

Capillary Hemangioma of the Spinal Cord: Case Report and Systematic Review of Literature

Background and ObjectivesCapillary hemangiomas are rare vascular lesions that rarely affect the central nervous system. When they present within the spinal canal, they are typically confined intradurally, with seldom intramedullary extension. We herein present a rare case of spinal intramedullary capillary hemangioma, with a systematic review of literature. MethodsMedical records and imaging data were retrospectively reviewed using the health record software EPIC and the radiology management software system RIS/PACS (QReads). The report was written in accordance with the CARE guidelines. We also performed a systematic review of literature on all cases of intramedullary spinal capillary hemangiomas, in accordance with PRISMA guidelines. ResultsThis is a case of a 54-year-old male who presented with progressive paraplegia and sensory deficits in the lower extremities. A spinal MRI showed an intramedullary enhancing lesion centered at T11 with associated cord compression. He underwent a thoracic laminectomy and gross total resection of the lesion without complications and subsequent improvement on his neurological exam. Histological examination showed findings consistent with a capillary hemangioma. Literature review also documented 21 studies with a combined total of 38 cases of intramedullary spinal capillary hemangiomas. ConclusionPurely intramedullary capillary hemangiomas are unusual spinal lesions with only a few cases reported in the literature, that should be considered in the differential diagnosis of intramedullary tumors. Surgical management remains the first line of treatment for symptomatic patients.

Infantile Hemangiomas of the Head and Neck: A Single-Center Experience.

Background: Infantile hemangiomas (IHs) are the most common benign vascular tumors of infancy. Methods: We report our experiences with 248 patients with head and neck IHs. Results: The median admission age was 4 months, and the female/male ratio was 2.18. Among the cases, 45% were followed by no treatment. No local complications were observed in any of these patients. Propranolol was provided to all patients who received medical treatment. The median duration of treatment was 12 months (1-30 months), and the median follow-up period of all patients was 14 months (0-118 months). The treatment response was 98%. The complication rate was 17%, and children aged between 3 and 9 months accounted for 60% of the patients who developed complications. Most of the complications were local complications, such as ulceration and bleeding. Conclusions: Although most IHs regress spontaneously, complications may occur. Propranolol alone is an effective treatment option, and early treatment initiation increases the success rate.

Numerical flow experiment for assessing predictors for cerebrovascular accidents in patients with PHACES syndrome

There is an increased risk of cerebrovascular accidents (CVA) in individuals with PHACES, yet the precise causes are not well understood. In this analysis, we aimed to examine the role of arteriopathy in PHACES syndrome as a potential contributor to CVA. We analyzed clinical and radiological data from 282 patients with suspected PHACES syndrome. We analyzed clinical features, including the presence of infantile hemangioma and radiological features based on magnetic resonance angiography or computed tomography angiography, in individuals with PHACES syndrome according to the Garzon criteria. To analyze intravascular blood flow, we conducted a simulation based on the Fluid–Structure Interaction (FSI) method, utilizing radiological data. The collected data underwent statistical analysis. Twenty patients with PHACES syndrome were included. CVAs were noted in 6 cases. Hypoplasia (p = 0.03), severe tortuosity (p < 0.01), absence of at least one main cerebral artery (p < 0.01), and presence of persistent arteries (p = 0.01) were associated with CVAs, with severe tortuosity being the strongest predictor. The in-silico analysis showed that the combination of hypoplasia and severe tortuosity resulted in a strongly thrombogenic environment. Severe tortuosity, combined with hypoplasia, is sufficient to create a hemodynamic environment conducive to thrombus formation and should be considered high-risk for cerebrovascular accidents (CVAs) in PHACES patients.

Association of Attention Deficit Hyperactivity Disorder with the Use of Oral Propranolol in Infantile Haemangiomas: An Ambispective Comparative Study.

Previous studies have raised concerns about the effects of oral propranolol on the central nervous system in infants, the exact measure and mechanism and the long-term follow-up of which is less well studied. This was an ambispective comparative study of children with infantile haemangioma (IH) followed by a repeat visit 4-10 years after completion of propranolol therapy. Parents were asked about psychologic functioning along with an initial screening examination. All patients were evaluated by a paediatric psychiatrist. After evaluation by the Strength and Difficulties Questionnaire, and subsequently by the paediatric psychiatrist, 2 of 12 patients (16.67%) showed features of attention deficit hyperactivity disorder in comparison to 0 of 40 subjects in the control group (0.0498; α = 0.05). These results indicate an increased risk of neuropsychiatric illnesses such as attention deficit hyperactivity disorder (ADHD) in patients given propranolol for IH, as supporting evidence to previous claims.

Color Doppler Ultrasound Indices as Predictors of Propranolol Response in Infantile Hemangioma: A Prospective Study

Color Doppler Ultrasound Indices as Predictors of Propranolol Response in Infantile Hemangioma: A Prospective Study

Kaposiform haemangioendothelioma: A radiological dilemma

Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular tumours diagnosed in infancy or early childhood. KHE has a worse prognosis than infantile or congenital haemangioma due to its infiltrative nature and risk of developing Kasabach-Merritt Syndrome (KMS). This case report is about a 4-month-old girl who presented with progressive left neck swelling. She was diagnosed with KHE and thrombocytopenia suggestive of Kasabach-Merritt Syndrome (KMS). We describe the imaging appearance of KHE on ultrasound and magnetic resonance imaging. We also highlight the importance of integrating patient’s clinical history with the physical, laboratory and imaging findings for early diagnosis to prevent disease-related life-threatening complications.

Rare complication – skin atrophy – after systemic conservative therapy of infantile hemangioma

BackgroundHemangiomas, also called infantile hemangiomas (IH) or hemangiomas of infancy are the most frequently seen benign vascular tumors of infancy. Different types of hemangiomas are described in the literature. The current approach is to assess the risk and, if needed, first line treatment is to initiate systemic propranolol.Case presentationA 3-month-old Caucasian female patient was brought as an outpatient. The main complaint was an infantile hemangioma in the facial area, which as per the parents’ story appeared within a week of birth like a small reddish line and it rapidly grew. Systemic propranolol was proposed as a first-line treatment and the adverse effects were explained. The parents, afraid of the side effects, wanted to explore other possibilities such as topical timolol, however, since it had no effect, propranolol was initiated in the end. Hemangioma was completely reduced in size; however, a skin defect was detected. As per the dermatologist’s counsel, topical cream was initiated. The skin defect was reduced but not fully healed. The child is still being monitored periodically.ConclusionAfter successful treatment of hemangioma, we identified a skin defect, which was very similar to steroid-induced skin atrophy. However, we cannot attribute this to a single factor. The only thing that can be concluded is that the subject needs a thorough studying, since rate of infantile hemangioma is high, and pediatricians need a clear management strategy of how to approach skin atrophy after successfully treating the hemangioma itself.

Dangers and therapeutic difficulties of intracranial hemangioma in infants: A CARE case report

IntroductionDescription of neurological complications induced by intracranial hemangioma in infants and by the initiation of beta-blocker treatment (propranolol). ObservationA 2-month-old infant was referred for grade 5 non-congenital unilateral peripheral facial palsy. Work-up revealed ipsilateral profound hearing loss and two intracranial hemangiomas: one in the ipsilateral internal auditory canal (IAC), the other in the cerebellum opposite the nodule of vermis. Initial treatment with a beta-blocker (propranolol 1mg/kg/day for 1month, then 3mg/kg/day) resulted in disappearance of symptoms and regression of lesions within 8weeks. At 20months after introduction of maintenance therapy (propranolol 3mg/kg/day), two asthma attacks occurred, leading to initiation of fluticasone and continuation of the beta-blocker. Thirty months after discontinuation of treatment, no further progression was noted. DiscussionUnilateral facial palsy in an infant suggests a number of diagnoses. MRI revealed IAC hemangioma. The choice of dosage and duration of treatment was based on a review of the literature and a strategy defined in multidisciplinary consultation.

A Case of Arteriovenous Malformation in the External Auditory Canal

Benign vascular tumors are relatively common in the head and neck region, but are rarely found in the ear. Among vascular tumors, infantile hemangioma stands as the prevailing entity, characterized by a rapid initial proliferation of endothelial cells, succeeded by a gradual regression over the course of several years. In contrast, arteriovenous malformation (AVM) is a less common phenomenon, originating from peripheral vascular anomalies during embryogenesis, and it does not undergo spontaneous regression. Specifically, the occurrence of AVM within the external auditory canal are exceedingly uncommon. Here, we describe a case of a 49-year-old male patient who had a mass incidentally discovered in the external auditory canal, but refused initial treatment. He later returned as the mass enlarged. This case could have been clinically misdiagnosed as a hemangioma, but was ultimately diagnosed pathologically as an AVM.

Fallopian Tube Hemangioma Discovered on Follow-up for Uterine Leiomyoma.

Hemangioma in female reproductive organs, particularly in the fallopian tube (FT), is a sporadic disease. In this report, we describe a case of hidden capillary hemangioma in FT in a 39-year-old woman who suffered from uterine leiomyoma. During the preoperative stage, pelvic sonography, computed tomography, and diagnostic laparoscopy revealed a subserous leiomyomatous nodule located along the posterior wall of the uterus. Despite this, intraoperatively, a benign vascular neoplasm was diagnosed. Histologically, it is characterized by multiple thin-walled vascular spaces lined with a single layer of endothelial cells, in which single mitoses were observed. The diagnosis was then confirmed immunohistochemically by CD31 and CD34 expression in the endothelial cells lining the inner surface of the spaces and the low mitotic activity of the tumor cells. It is virtually impossible to diagnose this asymptomatic neoplasm before and during surgery, which can result in an inadequate number of surgeries. Incorrect interpretation of a benign tumor at a young age can lead to unnecessary radical surgery with a resulting loss of fertility, and an unrevealed malignant process can threaten life.

Large ulcerated hemangioma treated with topical timolol drops

Infantile hemangiomas (IH) are the most common benign tumors of infancy and undergo spontaneous remission by about four to five years of age. Usually, only superficial and uncomplicated hemangiomas are treated by topical timolol while larger and complicated hemangiomas require oral propranolol or corticosteroids. Uncommonly reported in the literature is that even larger complicated IHs on low-risk sites can be treated with timolol drops locally. Our case is unique, as we treated a large ulcerated IH with oral and topical antibiotics and only topical timolol drops with rapid and complete resolution of symptoms.