Segmental Infantile Hemangiomas Research Articles

Propranolol for the treatment of ulcerated infantile hemangiomas: A prospective study

Propranolol for the treatment of ulcerated infantile hemangiomas: A prospective study

Infantile hemangioma explained in simple terms

Infantile hemangioma (IH) it is the most common benign vascular tumor in children of the first year, which is based on abnormal proliferation of endothelial cells under the influence of the main pro-angiogenic factors VEGF and FGF. It develops in the first weeks after birth, forming over 3–9 months with regression in the next 3–7 years. Three-quarters of infantile hemangiomas are nodular and are not accompanied by malformations. At the same time, segmental IH is most often associated with syndromic forms. Despite spontaneous regression (in 90% of cases), some forms and localization of IH can lead to the development of complications, local and endangering vital functions. In most cases, the diagnosis is based on anamnesis, characteristic features of the tumor, and clinical course. Additional studies (ultrasound DG, MRI/CT) are necessary in complicated forms and in doubtful clinical cases. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

Clinical Features of Segmental Infantile Hemangioma: A Prospective Study.

BackgroundInfantile hemangioma (IH) is the most common benign tumor in children. However, few studies have reported the clinical features of segmental IH. We aimed to determine the clinical characteristics of segmental IH and to identify features that may aid clinicians in managing segmental IH.MethodsIn the cross-sectional prospective study approved by the Ethics Committee of the hospital, children diagnosed with IH were recruited, and information including patient demographics, IH morphology and anatomical location, complications and treatments were recorded and analyzed.ResultsIn total, 153 patients with segmental IH and 1375 patients with nonsegmental IH were enrolled in this study. The average age on the day of the first visit in patients with segmental IH was 3.63±3.23 months. In 69 patients (45.10%), segmental IH was diagnosed at birth. Most segmental IHs (49.67%) occurred in the limbs, while only 22.04% of nonsegmental IHs occurred in the extremities (P<0.001). Thirteen patients (8.50%) with segmental IH had ulceration. Compared with patients with nonsegmental IHs, patients with segmental IHs were more likely to be treated with oral drugs (P<0.001).ConclusionSegmental IHs mainly occur in the extremities and are frequently diagnosed at birth. Segmental IHs are usually accompanied by ulceration, which are more commonly seen in the neck and perineal/perianal/genital areas than nonsegmental IHs. Oral propranolol is prescribed more often in patients with segmental IH than in those with nonsegmental IH.

Intracranial Abnormalities of Infantile Hemangiomas in the Head and Neck Regions: A Retrospective MRI Study

Abstract Objective: Few reports have described intracranial hemangiomas and structural brain and/or arterial anomalies in patients with infantile hemangiomas. This study was performed to examine the magnetic resonance imaging findings of intracranial abnormalities in a group of infants with hemangiomas in the head and neck regions. Methods: We reviewed our hemangioma treatment center database from January 2010 to July 2018 to assess the prevalence of intracranial abnormalities in infants with hemangiomas in the head and neck regions. Clinical and electronic magnetic resonance imaging data were also retrieved from the patients’ medical charts. Results: Of 436 patients with infantile hemangiomas in the head and neck regions, 23 (5%) had intracranial abnormalities, including 20 (23%) with segmental hemangiomas and 3 (1%) with focal hemangiomas. In total, 14 patients had intracranial hemangiomas located within the ventricle and cisterns or ipsilateral lesions involving the extradural space. Eight patients had intracranial structural abnormalities, such as cerebellar dysplasia, Dandy-Walker malformation, and hydrocephalus. Five patients had intracranial arterial anomalies, and three patients had arteriovenous malformations. Conclusions: These findings support the hypothesis that intracranial hemangiomas are commonly associated with segmental infantile hemangiomas in the head and neck regions.

When the disease goes beyond the skin, changes in the skin can be a sign of systemic disease

Infantile hemangioma may be accompanied by malformations of internal organs and blood vessels. In 1996 PHACE syndrome was defined as a disease which is characterized by the association of segmental infantile hemangioma with localization in the head/neck region and the presence of malformations in the posterior cranial fossa, abnormalities of arterial blood vessels including coarctation of the aorta, heart defects, as well as malformations of the eyes and central nervous system. This article presents a clinical case of a child who was diagnosed this syndrome at the age of 1.5 months based on the presence of segmental hemangioma, as well as large and small criteria specific for this disease. In addition to the main symptoms, the child had an accompanying pathology: Wolff–Parkinson–White syndrome. Therapy with non-selective b-blockers and polychemotherapy allowed stopping already developed and prevent possible complications associated with this syndrome. Parents gave their consent to use information about the child, including fotos, in the article.

Hemangioma-related syndromes.

There is a growing understanding of complications and anomalies associated with infantile hemangiomas. The current review will discuss recent clinical advances in syndromes associated with segmental hemangiomas, including PHACE and LUMBAR syndrome. In addition, the importance of recognizing visceral hemangiomatosis is highlighted. Ongoing longitudinal studies of PHACE and LUMBAR syndromes associated with segmental infantile hemangiomas have led to improved diagnosis and recommendations for screening for associated anomalies. Characterization of a growing spectrum of associated anomalies as well as better classification of at-risk patients will improve diagnosis and outcomes. In addition, visceral hemangiomatosis recognition and understanding of the potential association with consumptive hypothyroidism will improve initiation of appropriate screening. Clinicians should be aware of infantile hemangiomas associated with potential syndromic complications and recognize the need to initiate appropriate work-up. Segmental hemangiomas of the head and neck region may indicate a risk of PHACE syndrome and associated developmental anomalies. Although LUMBAR syndrome is the association of lower body segmental hemangioma with developmental anomalies. Visceral hemangiomas most commonly affect the liver and may be associated with complications such as consumptive hypothyroidism and heart failure.

An ulcerated giant segmental hemangioma resulting in contracture of the left elbow.

The majority of infantile hemangiomas are benign and will resolve on their own. We report a 4-month-old infant with an ulcerated giant segmental infantile hemangioma involving the left upper limb who developed a contracture of the left elbow despite treatment with oral propranolol, proper wound care, and regular intense physiotherapy. To our knowledge, contracture resulting from an infantile hemangioma has not been reported previously.

Frey syndrome-like developmental dysautonomia in a child with PHACE syndrome.

PHACE syndrome classically presents with a large, segmental facial infantile hemangioma (IH) associated with structural and vascular abnormalities involving the head and neck, heart, and eyes. We evaluated an infant who presented with ptosis caused by a clinically subtle, deep right-sided periorbital IH identified on MRI that also incidentally revealed hypoplasia of the right common carotid and right internal carotid arteries, supporting a diagnosis of PHACE syndrome. She subsequently developed acute-onset, transient right-sided facial erythema without anisocoria, triggered by feeding and emotional stress. We believe this represents a Frey syndrome-like developmental dysautonomia, previously unreported in association with PHACE syndrome, suggesting an associated defect in neurovascular embryogenesis.

Characteristic patterns of segmental infantile hemangiomas in Indian children: A descriptive study.

Approximately 18% of infantile hemangiomas are segmental. These are larger than other infantile hemangiomas, associated with higher rate of complications and developmental anomalies, and often require treatment. They follow nonrandom patterns on the head and neck as well as extremities which are probably related to embryologic development. Our study aimed to describe segmental patterns of infantile hemangiomas in Indian children, with associated anatomical abnormalities if any. Over a 9-year period, 59 infants presenting with lesions classified as segmental infantile hemangiomas were evaluated and analyzed. Associated developmental anomalies were assessed and recorded. In addition, patterns of "indeterminate" infantile hemangiomas in another 43 patients were analyzed. There were 14 male and 45 female infants with an average birth weight of 2.7 ± 0.726 kg in our study; the average age at onset was 1 ± 1.25 months with most (50.8%) lesions localized to the head and neck area. Mapping of lesions showed that the most common facial segments involved were mandibular (33%) and maxillary (30%). However, additional repetitive patterns not previously described (such as an "inverted comma" pattern on the chest, bilateral neck involvement and unilateral labium involvement) were seen in our patients. Common local complications were ulceration (27%), amblyopia and nasal obstruction (3% each). Mapping of the additional 43 patients with indeterminate infantile hemangiomas also showed repetitive though incomplete patterns. Relatively small number of patients. Segmental infantile hemangiomas present as large, distinctively patterned lesions, even on the trunk and genitalia. These patterns are probably based on embryologic developmental patterns. In addition, indeterminate lesions also showed distinctive repetitive patterns. Our study suggests that additional segments may need to be defined, particularly on the trunk and genital area.

Ocular Complications in PHACE Syndrome: A True Association or a Coincidence?

To characterize the risk for ocular complications in patients with PHACE syndrome. This study included consecutive patients with PHACE syndrome who were seen at Lurie Children's Hospital of Chicago from January 2000 through May 2017. A complete ophthalmic examination was performed in all patients, with extra attention for findings typically associated with PHACE syndrome. Thirty patients (67% female, median age of onset 0.08 months) were included: 38 (93%) demonstrated a segmental infantile hemangioma distribution. Twenty-one (70%) cases had a periocular involvement, and 47% had an infantile hemangioma with a deep component. Among 21 patients with periocular distribution, 9 had ocular complications secondary to the periocular location (mainly ptosis, nasolacrimal duct obstruction, and refractive errors), and one had an ocular complication specifically associated with PHACE syndrome (Horner syndrome). None of the patients without periocular distribution had an ocular complication. In patients with PHACE syndrome who have a periocular infantile hemangioma, a complete eye examination is recommended. Although specific ocular anomalies related to PHACE syndrome are rare, serious ocular complications secondary to the location of the hemangioma may be present. Eye examination in patients with PHACE syndrome without a periocular infantile hemangioma distribution is likely of low yield.

Infantile hemangioma with minimal or arrested growth as the skin manifestation of PHACE syndrome.

Infantile hemangiomas with minimal or arrested growth are vascular tumors with a proliferative component involving <25% of their total surface area. They are commonly described as localized lesions and are mainly located on the lower body. Little has been described about segmental forms on the face and their associations with PHACE syndrome. We carried out a multicenter, retrospective, case-series study involving 5 hospitals in Spain. Information was collected on cases of PHACE syndrome featuring infantile hemangiomas with minimal or arrested growth. The frontotemporal and maxillary areas were the most frequently involved sites in our series. The upper eyelid and upper lip were the 2 locations most frequently associated with proliferation and ulceration. Four patients experienced spontaneous resolution, and the rest had a very good cosmetic outcome with oral treatment. Cerebral and cervical arterial anomalies were the most frequent extracutaneous findings associated with PHACE, followed by cerebral and ocular anomalies. Some unique associated disorders were fructose intolerance and retinoblastoma. We present the largest case series of segmental facial infantile hemangiomas with minimal or arrested growth in PHACE syndrome and emphasize the importance of recognizing these lesions in early infancy, because they can indicate PHACE syndrome. The data presented suggest that infantile hemangiomas with minimal or arrested growth-associated PHACE syndrome does not seem to differ significantly from PHACE syndrome with classic infantile hemangiomas, and thus the same recommendations for diagnosis, management, and therapy should be followed. Future studies with more patients could contribute to enlighten this specific subset.

PHACE syndrome--clinical features, aetiology and management.

PHACE syndrome comprises a spectrum of anomalies including posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye anomalies. PHACE should be considered in any patient with a large facial segmental infantile haemangioma (IH), and multidisciplinary management is crucial. Low-dose propranolol is effectively for the treatment of IH associated with PHACE syndrome. Recent evidence suggests IH is comprised of mesoderm-derived haemogenic endothelium. The embryonic developmental anomaly nature of IH provides an insight into the origin of PHACE syndrome.

Ventral midline blanching in the setting of segmental infantile hemangiomas: clinical observations and pathogenetic implications.

Areas of blanched skin in children may be seen as an independent finding or in association with vascular birthmarks. We performed a retrospective chart review to identify and describe infants with areas of ventral midline blanching in the presence of segmental infantile hemangiomas. We identified nine full-term infants with partial or full segmental hemangiomas and areas of midline ventral blanching. Additional ventral wall defects were seen in five patients. Six had cardiac anomalies and six had intracranial anomalies. Five were diagnosed with definite PHACE (posterior fossa, hemangioma, arterial, cardiac, and eye abnormalities) syndrome and three had possible PHACE syndrome. Eight were complicated by ulceration. Treatment varied according to the case. Ventral blanching, even in the absence of overt midline defects, can be seen in infants with segmental hemangiomas at risk for PHACE syndrome. We hypothesize that midline blanching may represent a minor manifestation of a developmental ventral defect.

The “biker-glove” pattern of segmental infantile hemangiomas on the hands and feet

Infantile hemangiomas (IH) on the extremities have not been systematically studied. We sought to describe the clinical characteristics and distribution patterns of IH affecting acral surfaces and to explore the relationship among these patterns, limb development, and IH pathogenesis. This was a retrospective multicenter cohort study. Photographic archives from 4 tertiary pediatric dermatology referral centers were searched for patients with IH larger than 1 cm and involving 1 or more digit. Hemangioma location, distribution, and morphologic subtype were recorded. Medical records were reviewed for demographic and clinical data. In all, 73 patients were identified. The most common IH pattern resembled that of a "biker glove" (73%), followed by localized IH on the distal digits (14%), segmental IH extending over the distal digits (8%), and intermediate patterns (5%). Overall, 63% of acral IH were segmental, 26% indeterminate, and 11% localized. Five patients had associated structural anomalies. Complications were noted in 33% of cases. Limitations were retrospective study design; selection bias based on recall and photography; documentation and follow-up were not standardized across institutions; and treatment information may not reflect current approaches. Acral IH display specific patterns and are associated with a relatively high risk of ulceration.

Hémangiomes infantiles : actualités dans le traitement

Infantile hemangioma (IH) is not strictly speaking a tumor, but the result of anarchic postnatal vasculogenesis. Hypoxia seems to play an important role as a predisposing factor. IHs can present three clinical morphologies: superficial, deep, or mixed. Localized IHs are oval or round, circumscribed lesions, whereas segmental IHs extend across a large anatomic area with a geographic shape. Localized IHs are often benign, except when they are located near a noble structure such as the airways or the orbital area. Segmental IH may be associated with birth defects (PHACES syndrome and SACRAL syndrome). Clinical follow-up of infants with IH should be very careful in the first weeks of life since 80% of all IHs have reached their final size at 5 months of age. The main indications for treatment of IHs are: life-threatening conditions (heart failure, respiratory distress), functional risks (amblyopia, swallowing disorders, etc.), aesthetic risks (especially IH of the face localized on the nose, lips, etc.), and painful ulcerated IH. Beta-blockers, namely propranolol, have quickly become the first-line therapy of complicated IH. The treatment should be given as soon as possible to avoid sequelae.

Treatment of Infantile Hemangiomas with Sirolimus in a Patient with PHACE Syndrome

Infantile hemangiomas (IHs) are common benign tumors of childhood. IHs often regress satisfactorily without intervention, but a subset of IHs may lead to functional or cosmetic morbidity necessitating therapy. PHACE syndrome is characterized by a variety of neurocutaneous and vascular anomalies that typically include segmental hemangiomas. We present an infant with PHACE syndrome and segmental IH that failed conventional first-line therapies. Treatment with sirolimus provided benefit with regression of the cutaneous IH. As an inhibitor of the mammalian target of rapamycin (mTOR) pathway, the effective use of sirolimus may shed light on the emerging role of mTOR signaling in the development and pathogenesis of IHs.

Infantile Hemangiomas: An Update on Pathogenesis and Therapy

Infantile hemangiomas (IHs) are the most common vascular tumors of childhood, affecting ~5% of all infants. Although most lesions proliferate and then involute with minimal consequence, a significant minority can be disfiguring, functionally significant, or, rarely, life-threatening. Recent discoveries concerning hemangioma pathogenesis provide both an improved understanding and more optimal approach to workup and management. Important detrimental associations can be seen with IH, such as significant structural anomalies associated with segmental IH. Standards of care have dramatically changed evaluation and management of hemangiomas. The goal of timely recognition and therapy is to minimize or eliminate long-term sequelae. New modalities, such as oral propranolol, provide the caregiver with better therapeutic options, which can prevent or minimize medical risk or scarring, but the side effect profile and risk-benefit ratio of such interventions must always be evaluated before instituting therapy.

Gastrointestinal Bleeding in Infantile Hemangioma: A Complication of Segmental, Rather than Multifocal, Infantile Hemangiomas

To highlight an association of facial segmental hemangiomas with gastrointestinal bleeding in infants with infantile hemangiomas. We conducted a multicenter retrospective case series study. Ten female patients met study inclusion criteria; 8 were Caucasian, 9 had a facial segmental hemangioma, and 9 cases met the diagnostic criteria for definitive posterior fossa malformations, hemangioma, arterial lesions, cardiac anomalies/coarctation of the aorta and eye abnormalities syndrome with abnormalities of the aorta and cerebral arteriopathy. Severe gastrointestinal bleeding requiring blood transfusion occurred in 9 cases, with age at presentation of gastrointestinal bleeding ranging from 8 days to 6 months. When detected, the location of the hemangioma in the small intestine was in the distribution of the superior mesenteric artery. More than one agent was required to control the gastrointestinal bleeding, including oral or intravenous steroids, vincristine, oral propranolol, interferon, and resection of the small intestine. All cases needed ongoing support care with red blood cell transfusions. Gastrointestinal bleeding is a rare complication of true infantile hemangioma. The segmental pattern of the cutaneous hemangioma associated with gastrointestinal bleeding should suggest a segmental infantile hemangioma of the lower gastrointestinal tract.

A Randomized Controlled Trial of Propranolol for Infantile Hemangiomas

Propranolol hydrochloride is a safe and effective medication for treating infantile hemangiomas (IHs), with decreases in IH volume, color, and elevation. Forty children between the ages of 9 weeks and 5 years with facial IHs or IHs in sites with the potential for disfigurement were randomly assigned to receive propranolol or placebo oral solution 2 mg/kg per day divided 3 times daily for 6 months. Baseline electrocardiogram, echocardiogram, and laboratory evaluations were performed. Monitoring of heart rate, blood pressure, and blood glucose was performed at each visit. Children younger than 6 months were admitted to the hospital for monitoring after their first dose at weeks 1 and 2. Efficacy was assessed by performing blinded volume measurements at weeks 0, 4, 8, 12, 16, 20, and 24 and blinded investigator scoring of photographs at weeks 0, 12, and 24. IH growth stopped by week 4 in the propranolol group. Significant differences in the percent change in volume were seen between groups, with the largest difference at week 12. Significant decrease in IH redness and elevation occurred in the propranolol group at weeks 12 and 24 (P = .01 and .001, respectively). No significant hypoglycemia, hypotension, or bradycardia occurred. One child discontinued the study because of an upper respiratory tract infection. Other adverse events included bronchiolitis, gastroenteritis, streptococcal infection, cool extremities, dental caries, and sleep disturbance. Propranolol hydrochloride administered orally at 2 mg/kg per day reduced the volume, color, and elevation of focal and segmental IH in infants younger than 6 months and children up to 5 years of age.

PHACE syndrome: MRI of intracerebral vascular anomalies and clinical findings in a series of 12 patients

PHACE (posterior fossa defects, haemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities) syndrome describes a constellation of abnormalities that can occur in association with segmental craniofacial infantile haemangioma. To report the spectrum of clinical and imaging abnormalities seen in a cohort of children. A retrospective review of the clinical and imaging records of all patients diagnosed with PHACE syndrome between 1998 and 2009 was performed. Information sought included patient demographics, craniofacial segments involved, imaging findings and other extracutaneous abnormalities. Twelve patients were diagnosed with PHACE syndrome over 11 years. All patients had a segmental craniofacial haemangioma. Involved facial segments, in order of frequency, were frontotemporal (12), maxillary (8), mandibular (5) and frontonasal (1). The most common extracutaneous abnormalities were neurovascular anomalies (10), with many patients having multiple anomalies. The spectrum of arterial anomalies ranged from hypoplasia (9) to ectasia (3), anomalous origin/course (2) and persistent fetal anastomosis (2). Other anomalies found included cardiac anomalies (3), coarctation of the aorta (2), posterior fossa malformations (1) and sternal region anomalies (1). Intracranial anomalies are the most common extracutaneous feature of PHACE syndrome. The contribution of the radiologist in the recognition of such anomalies is important for the diagnosis of PHACE syndrome.