Características clínico-epidemiológicas y consideraciones terapéuticas de los hemangiomas congénitos e infantiles de un hospital de tercer nivel durante un periodo de 3 años: estudio comparativo y revisión de la literatura / Congenital and Infantile Hemangiomas: Epidemiological, Clinical, and Treatment Characteristics Based on 3 Years’ Experience at a Tertiary Care Hospital — A Retrospective Case Comparison and Review of the Literature

El diagnóstico diferencial clínico entre los hemangiomas congénitos (HC) y los infantiles (HI) es complicado pero esencial para el tratamiento. El marcador inmunohistoquímico GLUT-1 ayuda a distinguirlos, sin embargo, la biopsia no es habitual. Se diseñó un estudio retrospectivo incluyendo los HI y a los HC diagnosticados en un hospital terciario en un periodo de 3 años, con el objetivo de describir y comparar los principales aspectos clínicos, epidemiológicos y terapéuticos. Se incluyeron un total de 107 hemangiomas, 34 HC (NICH/PICH/RICH), 70 HI y 3 pendientes de clasificar. El HI superficial de cabeza y cuello fue el tumor más frecuente. El tronco fue la localización más frecuente de los HC. Los factores de riesgo estudiados fueron más frecuentes en el grupo de los HI. Para los HI, el tipo de respuesta obtenida fue independiente de las variables (sexo, fecundación in vitro, profundidad, localización y tipo de tratamiento) (AU)

Distinguishing between congenital and infantile hemangiomas is challenging, but essential for appropriate treatment. The immunohistochemical marker glucose transporter type 1 is helpful, but biopsies are uncommon in this setting. The aim of this retrospective study was to describe and compare epidemiological, clinical, and treatment characteristics of congenital and infantile hemangiomas diagnosed at a tertiary care hospital over 3 years. We studied 107 hemangiomas: 34 congenital hemangiomas (rapidly involuting, partially involuting, and noninvoluting), 70 infantile hemangiomas, and 3 hemangiomas pending classification. Superficial infantile hemangiomas of the head and neck were the most prevalent tumors. Congenital hemangiomas were most often located on the trunk. Studied risk factors were more common in patients with infantile hemangiomas. In this group of patients, treatment response was independent of sex, in vitro fertilization, lesion depth and location, and type of treatment (AU)

[Translated article] Congenital and Infantile Hemangiomas: Epidemiological, Clinical, and Treatment Characteristics Based on 3 Years’ Experience at a Tertiary Care Hospital – A Retrospective Case Comparison and Review of the Literature / Características clínico-epidemiológicas y consideraciones terapéuticas de los hemangiomas congénitos e infantiles de un hospital de tercer nivel durante un periodo de tres años: estudio comparativo y revisión de la literatura

Distinguishing between congenital and infantile hemangiomas is challenging, but essential for appropriate treatment. The immunohistochemical marker glucose transporter type 1 is helpful, but biopsies are uncommon in this setting. The aim of this retrospective study was to describe and compare epidemiological, clinical, and treatment characteristics of congenital and infantile hemangiomas diagnosed at a tertiary care hospital over 3 years. We studied 107 hemangiomas: 34 congenital hemangiomas (rapidly involuting, partially involuting, and noninvoluting), 70 infantile hemangiomas, and 3 hemangiomas pending classification. Superficial infantile hemangiomas of the head and neck were the most prevalent tumors. Congenital hemangiomas were most often located on the trunk. Studied risk factors were more common in patients with infantile hemangiomas. In this group of patients, treatment response was independent of sex, in vitro fertilization, lesion depth and location, and type of treatment (AU)

El diagnóstico diferencial clínico entre los hemangiomas congénitos (HC) y los infantiles (HI) es complicado pero esencial para el tratamiento. El marcador inmunohistoquímico GLUT-1 ayuda a distinguirlos, sin embargo, la biopsia no es habitual. Se diseñó un estudio retrospectivo incluyendo los HI y a los HC diagnosticados en un hospital terciario en un periodo de 3 años, con el objetivo de describir y comparar los principales aspectos clínicos, epidemiológicos y terapéuticos. Se incluyeron un total de 107 hemangiomas, 34 HC (NICH/PICH/RICH), 70 HI y 3 pendientes de clasificar. El HI superficial de cabeza y cuello fue el tumor más frecuente. El tronco fue la localización más frecuente de los HC. Los factores de riesgo estudiados fueron más frecuentes en el grupo de los HI. Para los HI, el tipo de respuesta obtenida fue independiente de las variables (sexo, fecundación in vitro, profundidad, localización y tipo de tratamiento) (AU)

Percutaneous Cryoablation for Symptomatic Peripheral Congenital Hemangioma.

Congenital hemangiomas (CHs) are rare, benign vascular tumors that are present at birth. They are divided into 3 major subtypes: rapidly involuting CHs, partially involuting CHs, and noninvoluting CHs (NICHs). There is a paucity of data in the literature regarding NICH and its management. Most symptomatic patients with NICH are managed with surgery. However, endovascular embolization, direct percutaneous sclerotherapy, or laser treatment can be used on a case-by-case basis. In this report, 3 cases with peripheral symptomatic NICH treated with percutaneous cryoablation are presented.

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.

Pediatric Penile Non-Involuting Congenital Hemangioma With an Associated Pyogenic Granuloma: Surgical Management of a Rare Vascular Anomaly.

Vascular anomalies include both tumors and malformations. Infantile hemangiomas are the most common benign vascular tumor of infancy that proliferate after birth and eventually involute. By contrast, congenital hemangiomas are formed at birth and are categorized into three groups: rapidly involuting, partially-involuting, and non-involuting congenital hemangiomas (NICH). NICH do not regress and grow with age. Pyogenic granulomas, another acquired vascular tumor, develop over vascular lesions and cause bleeding. Primary treatment options for NICH and pyogenic granulomas are surgical resection. Here, we report a case of a NICH with a co-existing pyogenic granuloma involving the penile shaft and scrotum treated surgically.

Reconstrucción del labio inferior con colgajo miomucoso del buccinador por hemangioma congénito: a propósito de un caso / Reconstruction of the lower lip with myomucosal buccinator flap due to congenital hemangioma: a case report

Los labios son repliegues músculo membranosos móviles que se encuentran en la entrada de la cavidad oral, y tienen como funcionalidad proporcionar competencia para la cavidad oral durante la masticación y el reposo. Bozzola y cols., en 1989, describieron la arteria bucal como el principal pedículo para el músculo buccinador. El objetivo de este trabajo es demostrar la efectividad del colgajo miomucoso del buccinador para reconstrucción del labio inferior en un paciente con hemangioma congénito. Este es un estudio longitudinal de tipo retrospectivo, con resultado descriptivo, que se le realizó a un paciente masculino de 32 años, para cubrimiento de defecto de lesión en labio inferior secundario a hemangioma congénito utilizando la reconstrucción con colgajo miomucoso del buccinador. El colgajo miomucoso del buccinador o colgajo yugal se considera uno de los mejores colgajos locales para la reconstrucción del labio inferior, sea cual fuere la causa, por su fácil disección y versatilidad debido a que tiene una tasa de sobrevida muy elevada con mínimas complicaciones en el posoperatorio. Nuestros resultados obtenidos concuerdan con los resultados ya publicados por diferentes autores, que llegan a las mismas conclusiones; aunque existen varias técnicas con colgajos locales para la reconstrucción del labio inferior, el colgajo del músculo buccinador o yugal se considera como una de las principales alternativas, ya que puede lograrse un labio funcional y estético.

The lips are mobile muscle membranous folds, found at the entrance of the oral cavity, whose function is to provide competition for the oral cavity during chewing and rest. Bozzola et al. in 1989, they described the buccal artery as the main pedicle for the Buccinator Muscle. The objective of this work is to demonstrate the effectiveness of the buccinator myomucosal flap for reconstruction of the lower lip in a patient with congenital hemangioma. This is a retrospective longitudinal study, with descriptive results, which was performed in a 32-year-old male patient to cover a lower lip lesion defect secondary to congenital hemangioma using buccinator myomucosal flap reconstruction. The buccinator myomucosal flap or jugal flap is considered one of the best local flaps for the reconstruction of the lower lip whatever the cause, due to its easy dissection and versatility due to the fact that it has a very high survival rate with minimal complications in the postoperative period. Our obtained results agree with the results already published by different authors, reaching the same conclusions; Although there are several techniques with local flaps for the reconstruction of the lower lip, the buccinator or jugal muscle flap is considered one of the main alternatives, since a functional and aesthetic lip can be achieved.

Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico / Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach-Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach-Merritt, con excelente respuesta y tolerancia al propranolol

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach-Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

A case series of tardive expansion congenital hemangioma: A variation of noninvoluting congenital hemangioma or a new hemangiomatous entity?

BACKGROUND: Congenital hemangiomas present fully grown at birth and share a remarkably similar lack of disproportionate or accelerated postnatal proliferation. OBJECTIVE: We report a series of unusual congenital hemangiomas that arise prenatally and initially exhibit a proportional growth pattern similar to that of noninvoluting congenital hemangioma. However, a tardive expansion of the lesion, similar to the proliferation phase of infantile hemangioma, occurs later during childhood. METHODS: A total of 11 unusual congenital hemangiomas were reviewed in regard to clinical presentation, imaging, and pathologic characteristics. RESULTS: The infants included 9 boys and 2 girls. The tumors were located in the head and neck (n=10) and abdominal wall (n=1). Spontaneous expansion began at the age of 12 months to 61 months, as determined from clinic notes and paired follow-up photographs. Uniform parenchymal masses and fast-flow vessels were confirmed by imaging examination. There are both histopathological overlap and distinction between these lesions and other congenital hemangiomas. LIMITATIONS: Only a small number of cases were identified. CONCLUSION: We propose that these lesions be denominated "tardive expansion congenital hemangioma (TECH)" to indicate their specific clinical and histological distinctiveness. Recognition of these distinct lesions will contribute to a better understanding of congenital hemangiomas.

Impact of congenital cutaneous hemangiomas on newborn care in the United States.

Little is known regarding the characteristics of newborns with congenital cutaneous hemangioma (CH) and the burden of CH on newborn care. The objective of this study is to describe the burden of CH on newborn inpatient stays in the United States. Specific aims include characterizing newborns with CH, assessing factors predictive of CH and procedures performed during hospitalization, determining characteristics associated with increased cost of care and length of stay in newborns with CH, and investigating trends in prevalence, length of stay, and cost of care. This is a nationally representative retrospective cohort study (National Inpatient Sample, 2009-2015). Sociodemographic factors associated with CH and risk factors for increased cost of care/length of stay were evaluated using weighted multivariable regression models. Overall prevalence of CH is 17.0 per 10,000 newborns. Cost of care and length of stay for newborns with CH are increasing over time. Controlling for all covariates, white (aOR 1.69), female (aOR 1.52) newborns from higher income families (aOR 1.44) were more likely to be born with CH (p < 0.001). Newborns with CH who were premature (aOR 3.88), underwent more procedures (aOR 8.81), and born in urban teaching hospitals (aOR 2.66) had the greatest cost of care (p < 0.001). Premature (aOR 3.74) newborns with CH in urban teaching hospitals (aOR 1.31) had the longest hospital stays (p < 0.001). The burden of CH in newborns is substantial and increasing over time. Understanding contributors to costly hospital stays is critical in developing evidence-based guidelines to reduce the growing impact of CH on newborn care.

Secondary Cleft Lip Induced by Congenital Hemangioma: Report of a Rare Case.

Infant hemangioma is a relatively rare congenital disease. Ulcer and infection may occur in some cases. A few cases may develop into deformity and defect. Herein, we present a case of secondary cleft lip induced by congenital hemangioma and also our sequential treatment.

Infantile and congenital hemangiomas.

Infantile hemangiomas (IHs) are the most common benign tumors of infancy. They typically appear after birth and undergo a period of rapid growth, followed by a gradual period of involution. Although the majority of IHs do not requirement treatment, oral propranolol is the first-line therapy for lesions that are at risk for life-threatening complications, functional impairment, ulceration, or permanent disfigurement. Rarely, IHs can be associated with structural anomalies. Congenital hemangiomas (CHs) are a distinct clinical entity, caused by a point mutation in GNAQ or GNA11. These lesions are typically present at birth and display a wide spectrum of clinical presentations. CHs can be distinguished from IHs by their unique histologic and radiographic features. Given the high-flow vascularity of CHs, surgical excision may be indicated due to the high risk of bleeding.

Congenital liver hemangioma revealed by cholestasis syndrome: report of a rare case.

Our paper reports a case of hepatic angioma revealed by neonatal cholestasis, thing that has never been reported in the literature to our knowledge. A newborn boy of 25 days of life had cholestatic jaundice since his fifth day of life. During its health assessment, the angioscan detected the presence of multiple hepatic agiomas. The rest of the etiological report returned without any anomaly. Beta-blockers were started with a very good clinical and ultrasonographic evolution after 12 months of treatment.

El pediatre davant un lactant amb hemangioma infantil / El pediatra ante un lactante con hemangioma infantil / The pediatrician and the infant with hemangioma

FONAMENT: L'hemangioma infantil (HI) és el tumor benigne més freqüent en la infància, de manera que el pediatre d'atenció primària ha d'estar familiaritzat amb les seves principals característiques distintives, ja que és el primer que tindrà l'oportunitat de ferne el diagnòstic. OBJECTIU: Descriure el diagnòstic, l'evolució, la derivació precoç a dermatologia pediàtrica I el tractament oportú de lactants amb HI. MÈTODE:. Descripció de quatre casos d'infants amb HI que tenen lloc en primera instància a la consulta d'atenció primària del pediatre, detallant el procés de diagnòstic, evolució, tractament I seguiment, a partir de les últimes revisions bibliogràfiques I les guies clíniques disponibles. RESULTATS: Els casos presentats són un bon exemple del maneig actual del lactant amb HI. CONCLUSIONS: Disposar d'un diagnòstic és el primer pas, que ha de continuar amb uns altres dos: fer un control evolutiu proper del lactant, que ens permetrà en els casos necessaris derivar l'infant a dermatologia pediàtrica, I la contenció familiar, ja que l'impacte psicològic de l'HI en l'infant I la família no és extrapolable entre pacients amb el mateix tumor. El pediatre ha de conèixer els diferents camins que cal seguir després del diagnòstic: si només necessita fer un seguiment evolutiu, en quines circumstàncies ha de sol·licitar exàmens complementaris, o si ha de derivar de manera precoç a dermatologia pediàtrica pel risc de complicacions o seqüeles. La valoració individualitzada de la localització, la mida I la presència de factors de risc de l'HI influiran en el moment d'inici I la modalitat de tractament

FUNDAMENTO: El hemangioma infantil (HI) es el tumor benigno más frecuente en la infancia, por lo que el pediatra de atención primaria debe estar familiarizado con sus principales características distintivas, pues es el primero que tendrá la oportunidad de realizar su diagnóstico. OBJETIVO: Describir el diagnóstico, la evolución, la derivación precoz a dermatología pediátrica y el tratamiento oportuno de lactantes con HI. MÉTODO: Descripción de cuatro casos de niños con HI que tienen lugar en primera instancia en la consulta de atención primaria del pediatra, detallando el proceso de diagnóstico, evolución, tratamiento y seguimiento, a partir de las últimas revisiones bibliográficas y las guías clínicas disponibles. RESULTADOS: Los casos presentados ejemplifican el manejo actual del lactante con HI. CONCLUSIONES: Disponer de un diagnóstico es el primer paso, el cual debe continuarse con otros dos: realizar un control evolutivo cercano del lactante, que nos permitirá en los casos necesarios derivar al niño a dermatología pediátrica, y la contención familiar, pues el impacto psicológico del HI en el niño y la familia no es extrapolable entre pacientes con el mismo tumor. El pediatra debe conocer los diferentes caminos a seguir tras el diagnóstico: si solo necesita realizar un seguimiento evolutivo, en qué circunstancias solicitar exámenes complementarios, o si debe derivar de forma precoz a dermatología pediátrica por el riesgo de complicaciones o secuelas. La valoración individualizada de la localización, el tamaño y la presencia de factores de riesgo del HI influirán en el momento de inicio y la modalidad de tratamiento

BACKGROUND: Infantile hemangioma (IH) is the most common benign tumor of children, so the primary care pediatrician should be familiar with its main features, as it is the first one that will have the opportunity to make its diagnosis. OBJECTIVE: To describe the diagnosis, evolution, early referral to the pediatric dermatologist and timely treatment of infants with IH. METHOD: Description of four cases of children with IH that take place in the first instance in the pediatrician's primary care office, detailing the process of diagnosis, evolution, treatment and follow-up, based on the latest bibliographic reviews and clinical guides available. RESULTS: The cases presented exemplify the current management of the infant with IH. CONCLUSIONS: Diagnosis is the first step, which must be continued with two others: carry out a close follow-up of the infant and prompt referral to a pediatric dermatologist when required, and family support, as the psychological impact of IH in children and families cannot be extrapolated among patients with the same tumor. The pediatrician must know the different paths to follow after the diagnosis: close follow-up, when to request complementary evaluations, and when to refer to the pediatric dermatologist due to the risk of complications or sequelae. Individual assessment of the location, size and presence of IH risk factors will influence the time of onset and the treatment modality

Congenital hepatic hemangiomas: Clinical, histologic, and genetic correlation.

BACKGROUND: The guide for monitoring and treatment of congenital hepatic hemangiomas (CHH) will depend on the subtype and the postnatal clinical behavior. Our aim is to present a series of CHH and characterize its clinical, histologic and genetic correlation, compared to cutaneous congenital hemangiomas (CCH). MATERIAL AND METHODS: A retrospective review of CHH patients diagnosed between 1991 and 2018 was performed. Clinical, morphological and histological data were analyzed and deep high-throughput sequencing was performed. MAIN RESULTS: Sixteen patients with CHH were included. Five patients were followed up with serial ultrasounds while pharmacological treatment (corticosteroids and propranolol) was decided in five. Surgical resection was performed in five owing to hemorrhage and suspicion of malignancy, and the last patient underwent embolization. Histologic analysis was available in 7 patients and confirmed CHH, showing two different histological patterns that could be associated with the presence of somatic pathogenic variants in GNAQ and/or PIK3CA detected in the genetic testing. Review of 7 samples of CCH revealed some histologic differences compared to CHH. CONCLUSION: CHH resemble its cutaneous homonym with similar clinical behavior. Histologic analysis can differentiate two subgroups while genetic testing can confirm mutations in GNAQ and in PIK3CA in a subset of CHH. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: IV.