Hemangioma segmentario de la barba / Segmental Beard Hemangioma

Los hemangiomas infantiles son el resultado de la proliferación de células del endotelio vascular y representan los tumores benignos más frecuentes en la infancia, con una incidencia estimada del 4-10% en bebés caucásicos. Se clasifican según el número, la profundidad y la distribución. Dentro de esta última clasificación se encuentran aquellos denominados segmentarios, que se caracterizan por su distribución extensa en áreas de prolongaciones mesodérmicas embrionarias. Se comunica el caso de una paciente evaluada al mes y medio de vida, con un hemangioma extenso del área mandibular y cuello anterior (hemangioma segmentario de la barba). Se describe la importancia de los estudios complementarios para evaluar el compromiso de órganos subyacentes, para detectar síndromes asociados y definir el tratamiento sobre la base de estos resultados. (AU)

Infantile hemangiomas arise from the proliferation of vascular endothelial cells and represent the most common benign tumors in infancy, with an estimated incidence of 4-10% in Caucasian infants. They vary according to their number, depth, and distribution. Within the latter classification are the so-called segmental ones, which feature an extensive distribution in areas of embryonic mesodermal extensions. We report the case of a patient evaluated at one and a half months of life with an extensive hemangioma of the mandibular area and anterior neck (segmental hemangioma of the beard). We describe the importance of complementary studies for evaluating the involvement of underlying organs, detecting associated syndromes, and defining the treatment based on these findings. (AU)

Asociación de hemangioma en zona cérvico facial y hemangioma subglótico en un neonato / Association of cervical-facial and subglottic hemangioma in neonates / Associação de hemangioma cervicofacial e subglótico em neonatos

Se presenta un neonato con hemangioma cérvico facial y posterior diagnóstico de hemangioma subglótico. Los hemangiomas en el período neonatal y los primeros meses de vida requieren una atención cuidadosa. Debido a su patrón de crecimiento y la futura aparición de nuevas lesiones, son considerados imprevisibles en esta etapa. Se encontró una fuerte asociación entre los hemangiomas difusos de localización cérvico facial y los hemangiomas sintomáticos de la vía aérea alta. El riesgo está relacionado con el grado de extensión de la afectación cutánea en un área que incluye la piel de la región mandibular, el mentón, el labio inferior y la parte anterior del cuello. Los hemangiomas infantiles requieren tratamiento cuando presentan riesgo vital y alteraciones funcionales, como compromiso de la vía aérea.

This is the case of a newborn with cervical hemangioma and a subsequent diagnosis of subglottic hemangioma. Hemangiomas in neonates and infants require careful attention. Due to their growth pattern and the potential appearance of new lesions, they are considered unpredictable at this stage. A strong link was found between diffuse cervical-facial and symptomatic upper airway hemangiomas. The risk is related to the extent of skin involvement in a given area, which might include the jaw, chin, lower lip, and front of the neck skin. Infant hemangiomas require treatment when they present life-threatening and functional alterations, such as airway compromise.

Relatamos o caso de um recém-nascido com hemangioma cervical com diagnóstico posterior de hemangioma subglótico. Hemangiomas em recém-nascidos e lactentes requerem atenção cuidadosa. Devido ao seu padrão de crescimento e ao potencial aparecimento de novas lesões, são considerados imprevisíveis nessa fase. Uma forte associação foi encontrada entre hemangiomas cervicofaciais difusos e hemangiomas sintomáticos das vias aéreas superiores. O risco está relacionado à extensão do envolvimento da pele da mandíbula, o queixo, o lábio inferior e a pele da frente do pescoço. Os hemangiomas infantis necessitam de tratamento quando apresentam alterações funcionais ou risco de vida, como comprometimento das vias aéreas.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

Cutaneous leiomyosarcoma on the face

Abstract: Leiomyosarcoma is a rare skin tumor, most common in white men in the fifth to eighth decades of life. Primary tumors are classified in dermal or subcutaneous, that differ by clinical and prognostic features. They may appear on any site of the body, but are rare on the face. A 54-year-old female was admitted with a 5cm exophytic nodular lesion of 8 months duration on the right cheek, site of previous chronic radiodermatitis. Histopathology revealed spindle-shaped cell neoplasia, positive for smooth muscle actin on immunohistochemistry. Cutaneous leiomyosarcomas on the face are rare and may occur in previously irradiated areas. Immunohistochemistry is mandatory for an accurate diagnosis. Its similarity with other tumors may complicate the diagnosis, with delay expansion of the tumor.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Abstract: Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Malformación linfática facial mixta: Caso clínico / Mixed facial lymphatic malformation: Case report

Lymphangiomas are a common form of vascular malformation of the lymphatic vessels, mainly in the head and neck region. Most cases are progressive evolution and require a multidisciplinary approach. Currently, the first therapeutic option is sclerotherapy, leaving surgery for the treatment of remaining lesions. Objective: To present a case of facial lymphatic malformation (LM) treated with sclerotherapy, surgery and orthodontics in a 15-year follow up. Case report: A one-year-old female patient who consulted health professionals due to a progressive volume increase of the soft parts of her right cheek. The imaging study confirmed the diagnosis of microcystic lymphatic malformation. It was managed with OK-432 sclerotherapy and Bleomycin. At 2 years of age, the patient response was considered adequate; an intralesional submandibular surgical excision was then performed, with partial resection of the lesion. The biopsy confirmed the diagnosis of microcystic LM. Six months after, a re-resection was planned using the same approach and removing the remaining lesion, with favorable development until the age of 9 years when the patient required surgery and orthodontic management due to intraoral recurrence. No major developments until the age of 13 when a new orthodontic surgery and handling are planned to perform right oral commissure suspension. Conclusion: LM management by sclerotherapy, surgery, and orthodontics has shown the advantages of a multidisciplinary long-term treatment in this case.

El linfangioma corresponde a una malformación vascular de los vasos linfáticos, preferentemente de la región de cabeza y cuello. La mayoría de los casos son de evolución progresiva y requieren un manejo multidisciplinario. Actualmente la primera opción terapéutica es la esclerosis, reservando la cirugía para el tratamiento de las lesiones remanentes. Objetivo: Presentar un caso de malformación linfática (ML) facial, tratado con escleroterapia, cirugía y ortodoncia en un seguimiento a 15 años. Caso clínico: Paciente de sexo femenino que consulta al año de edad por aumento de volumen progresivo de partes blandas en su mejilla derecha. El estudio de imágenes confirmó el diagnóstico de Malformación Linfática microquística. Se manejó con esclerosis seriada con OK-432 y Bleomicina. A los 2 años de edad se consideró que la respuesta era adecuada, y se procedió a realizar extirpación quirúrgica intralesional submandibular, con resección parcial de la lesión. La biopsia confirmó el diagnóstico de ML microquística. Seis meses después se planificó nueva resección utilizando el mismo abordaje y extirpando lesión remanente, con evolución favorable hasta la edad de 9 años en que requiere cirugía y manejo por ortodoncia, por recidiva de lesión a nivel intraoral. Evolución favorable hasta que a la edad de 13 años se planifica nueva cirugía y manejo por ortodoncia para suspender la comisura bucal derecha. Conclusión: El manejo de la ML mediante escleroterapia, cirugía, y ortodoncia muestra en este caso las ventajas de un tratamiento multidisciplinarion a largo plazo.

Higroma cístico de face com involução após infecção local / Involution of a cystic hygroma of the face following local infection

Relatamos um caso de higroma cístico na face de uma criança de quatro meses de idade. Havia história de aumento de volume congênito na hemiface direita que involuiu consideravelmente após sinais de infecção. O higroma cístico ou linfangioma é uma malformação congênita rara do sistema linfático, presente ao nascimento em 50 por cento dos casos. Localiza-se preferencialmente na região cervical e na face. Geralmente evolui com crescimento lento e progressivo, podendo comprimir e infiltrar estruturas adjacentes. Sua regressão espontânea ocorre em apenas 6 por cento dos casos.

This report describes the case of a cystic hygroma on the face of a four-month old child. There was a history of congenital swelling of the right hemiface that decreased considerably following signs of infection. The cystic hygroma or lymphangioma is a rare congenital malformation of the lymphatic system that is present at birth in 50 percent of cases. It is usually located on the neck or face. It generally grows slowly and progressively and may compress and infiltrate adjacent structures. Its spontaneous regression occurs in only 6 percent of cases.

Lesões múltiplas de osteoma cutis na face: terapêutica minimamente invasiva em pacientes com sequela de acne - relato de casos / Multiple injuries of osteoma skin in the face: therapeutical least invasive in patients with acne sequela - case report

Osteoma cutis é a formação óssea no interior da pele, podendo ser primária ou secundária. Única ou múltipla, de tamanhos variados e acometendo ambos os sexos, é uma lesão cutânea rara, de etiopatogenia e classificação ainda discutidas. Nosso objetivo foi relatar o diagnóstico e a terapêutica minimamente invasiva de lesões múltiplas de osteoma cutis na face em pacientes com sequelas de acne. Fizemos a retirada dos osteomas com agulhas BD 0,70 x 25 22G1, sem anestésicos tópicos ou injetáveis no local. As pequenas incisões foram deixadas expostas, com pomada cicatrizante. Obteve-se um excelente resultado estético em 15 dias.

Osteoma cutis is a bone formation in the dermis can to be primary or secondary forms. Only, multiples, many forms, occurring on either sex, they are a rare cutaneous disease. The pathogenesis and classification remains unclear. Our objective was the diagnostic and small invasive surgery treatment of the osteoma cutis multiple of the face, in patients as a sequel of acne. To remove the osteoma we used needle BD 0,70x25 22G1, without anesthetic topic or inject able site. The small wounds were exposed with scarring balsam. We got an excellent esthetic result after 15 days.

Pilomatricoma: a dermal analog of calcifying odontogenic cyst.

Pilomatricoma, also known as "Calcifying Epithelioma of Malherbe" is an uncommon, benign, cutaneous tumor originating from the hair matrix. Most often, its precise preoperative diagnosis is not possible. Rarity of this lesion and its histological resemblance to the calcifying odontogenic cyst (the Gorlin's cyst) make it relevant, both to the maxillofacial surgeons and the pathologists. Here, we describe a case of pilomatricoma of the periorbital region in a young Indian female patient. We believe that its inclusion is essential in the differential diagnosis of hard masses of the maxillofacial and paraoral regions.

Prótesis nasal de uso nocturno: presentacion de un caso / Night use nasal prosthesis: case report

Después de la cirugía oncológica, el paciente portador de tumor facial encuentra un desafío tan difícil como la propia enfermedad. Tal problema tiene que ver con la reducción de autoestima provocada por su nueva imagen corporal. Siendo así, el presente artículo describe una técnica de simple confección que puede solucionar problemas vividos por el paciente portador de prótesis nasal, sujetada mecánicamente por las gafas, pues por la noche el paciente se ve imposibilitado de usar ese aparato, por existir la posibilidad de estropearse. Para estos pacientes, la prótesis nasal de uso nocturno, a pesar de ser un aparato no estético se vuelve extremamente satisfactorio proporcionando así una mejoría significativa en la cualidad de vida de estos pacientes

After an oncological surgery, a patient who has a facial tumor encounters a challenge as hard as his own illness. This problem is related to the reduction of his self-esteem, caused by his new body image. Therefore, the following article describes a simple technique that may solve problems experienced by the patient that wears nasal prosthesis mechanically retained to glasses. During his rest, the patient is unable to normally wear his prosthesis because of the risk of breaking them up. For those patients, the night-wear prosthesis, in spite of not being an esthetical prosthesis, becomes extremely satisfying, thus providing a improvement in his quality of life

Síndrome de PHACES / PHACES syndrome

El hemangioma es el tumor vascular benigno más frecuente de la infancia y su asociación con otras malformaciones es rara. Se ha descrito recientemente asociado al síndrome de PHACES, que corresponde a una alteración del desarrollo en la gestación precoz. Este síndrome se caracteriza por la presencia de un gran Hemangioma facial (H) asociado a otras anomalías como: malformaciones cerebrales de la fosa Posterior (P), alteraciones de las arterias cervicocraneales y Coartación de la aorta (C), defectos oculares (E) y esternón hendido y/o rafe supraumbilical (S). Afecta de preferencia al sexo femenino y la mayoría tiene sólo una manifestación extracutánea.

The hemangioma is the most common benign vascular tumor in childhood and is rarely related to other systemic malformations. It has been recently described in association with PHACES syndrome, which is an alteration in gestational development. This syndrome presents a large facial Hemangioma (H), with other anomalies like: Posterior fossa brain malformation (P), cervicocraneal arterial anomalies and coarctation of the aorta (C), ophthalmologic disorders (E), and midline abdominal and sternal defects (S). Females are more frequently affected and there is usually only one extracutaneous manifestation.

CT and MR Imaging of the Buccal Space: Normal Anatomy and Abnormalities

The buccal space is an anatomical compartment lying anterior to the masticator space and lateral to the buccinator muscle. Since the major purpose of imaging is to define the likely anatomic origin and also the extent of a given lesion, thorough knowledge of the normal anatomy of the buccal space is essential, and this knowledge can aid the physician in narrowing down the list of possible maladies on the differential diagnosis. We illustrate here in this paper the important anatomic landmarks and typical pathologic conditions of the buccal space such as the developmental lesions and the neoplastic lesions. Knowledge of the expected pathologic conditions is useful for the radiologist when interpreting facial CT and MR images.

Leiomioma facial: presentación de un caso / Facial leiomyoma: a case presentation

Se presenta un caso de leiomioma facial. Hasta el 2002 se habían reportado aproximadamente 70 casos en la región bucal y maxilofacial. Teniendo en cuenta la escasa frecuencia de esta entidad, se consideró de interés aportar un nuevo caso, comentando los aspectos clínicos e histopatológicos. Se realizó una revisión del tema. Se presenta una paciente femenina con diagnóstico de leiomioma facial diagnosticada clínica e histológicamente en el Hospital Provincial Clinicoquirúrgico Docente "Manuel Ascunce Domenech" de Camagüey y que fue tratada quirúrgicamente en dicha institución(AU)

A case of facial leiomyoma is reported in this paper. Approximately 70 cases had been reported in the oral and maxillofacial regions until the year 200e. Taking into account the causistry of leiomyoma, we decided to present a new case making comments on clinical and histopathological aspects and to make a literature review in this regard. Here is the case of a female patient diagnosed with facial leiomyoma by clinical and histological means at "Manuel Ascunce" teaching clinical-surgical hospital in Camaguey province where she was also surgically treated(AU)