Angioma de células litorales esplénico. Presentación de caso clínico / Littoral cell angioma of the spleen. Report on a clinical case / Angioma de células litoraneas do baço: apresentação de um caso clínico

El angioma de células litorales del bazo es una neoplasia vascular benigna poco frecuente, de presentación clínica, paraclínica e imagenológica inespecífica. Se realizó en nuestro paciente la esplenectomía, el tratamiento indicado y curativo, y se logró el diagnóstico a través de la histología e inmunohistoquímica de la pieza de anatomía patológica.

Littoral cell angioma of the spleen is rather an unusual benign vascular neoplasm with non-specific clinical, paraclinical and imaging presentation. Our patient underwent splenectomy, performed as the appropriate and remedial treatment. Diagnosis was made by means of histology and immunohistochemistry techniques of the pathologic anatomy of the surgical piece.

O angioma de células litoraneas do baço é uma neoplasia vascular benigna rara com apresentação clínica, laboratorial e de imagem inespecíficas. A esplenectomia foi realizada em nosso paciente, sendo esse o tratamento indicado e curativo, obtendo-se o diagnóstico através da histologia e imunohistoquímica da peça anatomopatológica.

Angiomatosis intraorbitaria / intraorbital angiomatosis

Introducción: Los angiomas cavernosos son entidades frecuentes, dentro de los tumores orbitarios, la presencia de 2 o más es infrecuente dentro de una órbita, la topografía extraconal es la más común, cuando son múltiples pueden ser parte de angiomatosis cerebral en algún momento de su evolución, por ello la necesidad de controles evolutivos. Descripción del caso: Paciente mujer, 30 años que debuta con proptosis derecha, dolor retrocular, diplopía, agudeza visual normal.Tac se visualizan dos lesiones intraorbitarias derechas, intraconales, solidas, bien definidas, retrooculares, que contacta (la más interna) el nervio óptico y provoca una rotación externa del globo ocular. RMN: las lesiones se manifiestan más difusas y heterogéneas, con signos de hemosiderina. Resultados: Video explicativo del caso, y pasos estructurados de la técnica. Se utilizó orbitomía lateral derecha, incisión ciliar con extensión hacia la porción lateral de la órbita, osteotomía del reborde lateral orbitario, luego con drill se avanza la resección de la pared lateral hasta el borde externo de la hendidura esfenoidal. Punto de tracción sobre la glándula lagrimal, permitiendo rotación del globo ocular y tensar periorbita. Se abre periorbita, identificando músculo recto externo y por su borde superior se avanza en la disección profunda reconociendo vaina del nervio óptico, localizando los angiomas cavernosos, disecándolos circunferencialmente y extrayéndolos. Reconstrucción de la pared orbitaria y reborde orbitario, cierre por planos con criterio cosmético, no hubo déficit óculo-motor. Conclusión: La exéresis quirúrgica es el gol estándar de tratamiento. La orbitotomía lateral es un abordaje mínimamente invasivo, seguro a esta patología.

Introduction: Cavernous angiomas are common entities, within orbital tumors, the presence of 2 or more than two is uncommon within an orbit, extraconal topography is the mostcommun, when they are multiple, they can be part of brain angiomatosis at some point in their evolution, therefore the need for evolutionary controls. Case Description: Pte. woman, 30 years who debuts with right proptosis, retrocular pain, diplopia, normal visual acuity. Tac two right, intracontal, solid, well-defined, retroocular intraorbital lesions are displayed that contacts (the innermost) the optic nerve and causes an external rotation of the eyeball. MRI: lesions appear more diffuse and heterogeneous, with signs of hemosiderin. Results: Explanatory video of the case, and structured steps of the technique.Right lateral orbitomia, ciliary incision extending to the lateral portion of the orbit, osteotomy of the right orbital lateral rim was used, then with drill the resection of the lateral wall is advanced. Traction points on the tear gland, allowing rotation of the eyeball and tensioning periorbita. It opens periorbita, identifying external straight muscle and by its upper edge advances in the deep dissection, recognizing sheath of the optic nerve, locating the cavernous angiomas, dissecting them circumferentially and removing them. Reconstruction of the orbital wall and orbital rim, closure by planes with cosmetic criteria, there was no oculo-motor deficit. Conclusion: Surgical resection is the standard treatment goal. The lateral orbitotomia was a minimally invasive approach, safe to this pathology.

Malformaciones cavernosas cerebrales, presentación de 14 casos y revisión de literatura / Brain cavernous malformations, presentation of 14 cases and literature review

Objetivo: Presentar los resultados de tratamiento quirúrgico obtenidos en una serie de 14 casos de malformación cavernosa, situadas en diferentes localizaciones encefálicas, además de realizar una revisión bibliográfica sobre el tema. Material y métodos: En el periodo de los años 2014-2019, se diagnosticaron y protocolizaron 14 pacientes por medio de la consulta externa de neurocirugía del Hospital Juárez de México. Todos menos 2, fueron intervenidos quirúrgicamente. Resultados: En 12 de los 14 casos que recibieron tratamiento quirúrgico, se documentó mejoría neurológica posterior a la resección total en 10 pacientes, 1 paciente de cavernoma gigante temporal se hizo resección subtotal, en 1 paciente con lesión de localización protuberancial se le realizó únicamente drenaje de hematoma. El déficit preoperatorio tendió a mejorar progresivamente en las lesiones de mayor tamaño y en ningún caso se documentaron complicaciones, las crisis convulsivas se controlaron disminuyendo progresivamente la dosis de fármacos anticonvulsivantes en el periodo postquirúrgico de este grupo de pacientes. Y dos pacientes, uno con lesión mesencefálica y el otro con cavernomatosis solo se sometieron a observación. Conclusiones: La cirugía es el método de elección hoy en día para el tratamiento de las malformaciones cavernosas, siendo los mejores resultados a menor tamaño de la lesión y con localizaciones más superficiales. Los resultados quirúrgicos de nuestros pacientes son similares a lo reportado en la literatura mundial.

Objectives: To present the surgical outcomes obtained in a series of 14 cases of cavernous malformation, located in different brain locations, in addition to conducting a literature review on the subject. Method: Between the years, 2014 and 2019, 14 cases were diagnosed and protocolized in neurosurgery department of Hospital Juárez of México. All patients except two, were surgically treated. Results: In 12 of the 14 cases received surgical treatment, neurological improvement was documented after the total resection in ten patients, one patient with giant temporal cavernoma performed a subtotal resection, other case with a lesion in the pontine location a hematoma drainage was performed. All surgical patients the preoperative clinical deficit tended to improve progressively in larger lesions and no complications were documented. Seizures were controlled by gradually decreasing the dose of anticonvulsant drugs in the post-surgical period of this group of patients. And two patients, one with mesencephalic lesion and another with cavernomatosis, were only observe. Conclusion: Surgery is the method of choice today for the treatment of cavernous malformations, with the best outcome being the smallest size of the lesion and with more superficial locations. The surgical outcomes in our patients are similar to those reported in the world literature

Síndrome Sturge-Weber. Revisión de la literatura a propósito de un caso / Sturge-Weber syndrome. Literature review about a case / Síndrome de Sturge-Weber. Revisão de literatura sobre um caso

RESUMEN Introducción: Entre las hamartoblastosis se reconoce el síndrome de Sturge-Weber (SSW), cuyo diagnóstico es inusual y complejo su tratamiento. Objetivo: Socializar la experiencia en la atención a un paciente pediátrico con síndrome de Sturge-Weber en el Hospital Pediátrico Docente "General Pedro Agustín Pérez" de Guantánamo. Método: Se presentó un paciente preescolar, sin antecedentes familiares de interés. Al mes de nacido fue operado de glaucoma congénito. Durante el primer año de vida presentó retardo del desarrollo psicomotor y movimientos involuntarios considerados convulsiones tónico-clónicas. Se revisó la literatura para transmitir a la comunidad médica, de modo particular, a estudiantes de medicina y médicos generales, información para lograr un diagnóstico y un seguimiento adecuado de esta afección. Resultados: Según las manifestaciones clínicas, examen físico mediante el método clínico y los resultados de los exámenes complementarios, se planteó el diagnóstico de síndrome neurocutáneo, de modo particular un SSW. Se aplicó tratamiento con diazepam y al persistir la crisis, fenitoína. La primera clasificación de los síndromes neurocutáneos la realizó Jan Van der Hoeve, que acuñó el término facomatosis. Es un trastorno neurológico congénito, no hereditario aunque hay descrito casos familiares, poco común pero frecuente respecto a otros síndromes neurocutáneos. Se estima una incidencia de 5 x 100,000 nacidos vivos. Afecta a todos los grupos étnicos y ambos sexos. Conclusiones: No siempre hay relación entre la gravedad de las manifestaciones cutáneas, neurológicas y oculares del SSW con las alteraciones cerebrales. Es importante el examen físico para establecer el diagnóstico oportuno y evitar futuras secuelas y complicaciones.

ABSTRACT Introduction: Sturge-Weber syndrome (SSW) is recognized among hamartoblastosis, whose diagnosis is unusual and complex treatment. Objective: To socialize the experience in the care of a pediatric patient with Sturge-Weber syndrome at the "General Pedro Agustín Pérez" Pediatric Teaching Hospital in Guantanamo. Method: A preschool patient was presented, with no family history of interest. One month after birth, he underwent congenital glaucoma surgery. During the first year of life he presented retardation of psychomotor development and involuntary movements considered tonic-clonic seizures. Literature was reviewed to transmit to the medical community, in particular, to medical students and general practitioners, information to achieve a diagnosis and adequate follow-up of this condition. Results: According to the clinical manifestations, physical examination by means of the clinical method and the results of the complementary examinations, the diagnosis of neurocutaneous syndrome was proposed, in particular a SSW. Treatment with diazepam was applied and when the crisis persisted, phenytoin. The first classification of neurocutaneous syndromes was carried out by Jan Van der Hoeve, who coined the term phacomatosis. It is a congenital neurological disorder, not inherited although there have been family cases described, uncommon but frequent compared to other neurocutaneous syndromes. An incidence of 5 x 100,000 live births is estimated. It affects all ethnic groups and both sexes. Conclusions: There is not always a relationship between the severity of cutaneous, neurological and ocular manifestations of SSW with brain disorders. The physical examination is important to establish the timely diagnosis and avoid future sequelae and complications.

RESUMO Introdução: A síndrome de Sturge-Weber (SSW) é reconhecida entre as hamartoblastose, cujo diagnóstico é tratamento incomum e complexo. Objetivo: Socializar a experiência no atendimento de um paciente pediátrico com síndrome de Sturge-Weber no Hospital Pediátrico "General Pedro Agustín Pérez" em Guantánamo. Método: Foi apresentado um paciente pré-escolar, sem histórico familiar de interesse. Um mês após o nascimento, ele foi submetido a uma cirurgia de glaucoma congênito. Durante o primeiro ano de vida, apresentou retardo no desenvolvimento psicomotor e movimentos involuntários considerados convulsões tônico-clônicas. A literatura foi revisada para transmitir à comunidade médica, em particular aos estudantes de medicina e clínicos gerais, informações para obter um diagnóstico e acompanhamento adequado dessa condição. Resultados: De acordo com as manifestações clínicas, o exame físico por meio do método clínico e os resultados dos exames complementares, foi proposto o diagnóstico de síndrome neurocutânea, em particular um SSW. O tratamento com diazepam foi aplicado e, quando a crise persistiu, fenitoína. A primeira classificação das síndromes neurocutâneas foi realizada por Jan Van der Hoeve, que cunhou o termo facomatose. É um distúrbio neurológico congênito, não herdado, embora haja casos familiares descritos, incomum, mas frequente em comparação com outras síndromes neurocutâneas. É estimada uma incidência de 5 x 100.000 nascidos vivos. Afeta todos os grupos étnicos e ambos os sexos. Conclusões: Nem sempre existe relação entre a gravidade das manifestações cutâneas, neurológicas e oculares da SSW com distúrbios cerebrais. O exame físico é importante para estabelecer o diagnóstico oportuno e evitar futuras sequelas e complicações.

A capillary angioma of the lips: an extremely rare situation in the oral cavity / Angioma capilar de los labios: una situación extremadamente rara en la cavidad oral

Hemangiomas are tumors identified by rapid endothelial cell proliferation in early infancy, followed mostly by involution over the time. However, 10-12 % of true hemangiomas don't involute and require surgical treatment. Hemangiomas are classified on the basis of their histological appearance as capillary, mixed and cavernous. We report a case of capillary angioma in a 13-year old boy who presented with a budding into the lower lip. The lesion was excised and histopathological report confirmed the diagnosis.

Los hemangiomas son tumores identificados por la rápida proliferación de células endoteliales en la primera infancia, seguidos principalmente por involución a lo largo del tiempo. Sin embargo, entre el 10 y el 12 % de los hemangiomas no intervienen y no requieren tratamiento quirúrgico. Los hemangiomas se clasifican en función de su aspecto histológico como capilares, mixtos y cavernosos. Reportamos un caso de angioma capilar en un niño de 13 años que presentó un brote en el labio inferior. La lesión fue extirpada y el informe histopatológico confirmó el diagnóstico.

Síndrome De Sturge-Weber: Reporte De Un Caso Y Revisión De La Literatura / Sturge-Weber Syndrome: A Case Report And Literature Review

Resumen El Síndrome de Sturge-Weber es un trastorno poco común del desarrollo neuroectodérmico, caracterizado por un angioma facial tipo nevus flammeus y una angiomatosis leptomeníngea, con frecuencia ipsilateral al nevus. Este síndrome predispone a calcificaciones, atrofia cerebral y convulsiones refractarias. Propósito: En este artículo se realiza una revisión de la literatura sobre el Síndrome de Sturge-Weber y se reporta el caso de un paciente de 18 meses de edad diagnosticado con esta patología que ingresa a urgencias por presentar cuadro febril de tres días y comienzo de convulsiones tónico clónicas localizadas en hemicuerpo derecho refractarias al tratamiento convencional; en esta revisión se resalta la importancia del diagnóstico y manejo oportuno al igual que un adecuado seguimiento. Desarrollo: se realizaron búsquedas en las bases de datos PubMed, Science Direct y Scielo, confirmando que aún se desconocen algunos aspectos de esta patología, sin embargo, con el descubrimiento de la mutación somática de GNAQ hay un amplio campo para próximas investigaciones. Hallazgos y conclusiones: Es importante en el ejercicio médico no pasar de alto las lesiones angiomatosas que posean una ubicación trigeminal en los recién nacidos, con el fin de establecer un diagnóstico oportuno e intentar conseguir un mejor desarrollo a futuro.

Abstract Sturge-Weber Syndrome is a rare developmental neuroectodermical disorder. It is characterized by a facial port-wine stain and a leptomeningeal angiomata, frequently localized ipsilateral to the facial port-wine stain. This syndrome predisposes either to brain atrophy, calcifications and refractory seizures. In this paper a Sturge-Weber Syndrome literature review was made and a 18 month aged child case with this diagnosis is reported. He was admitted to the emergency department of a local hospital with a history of three days of fever and tonic-clonic seizures localized on the right side and refractory to conventional treatment. This review highlights the importance of an early diagnosis and an appropriate follow up. To carry out this review a search in PubMed, Science Direct and Scielo databases was done, confirming that there are some issues about this disorder that are still unknown. However, with the GNAQ somatic mutation discovery, there is an open field for new researches. It is very important in medical practice not to understimate a facial port-wine stain over trigeminal territory in newborns in order to make an early diagnosis and try to achieve a better future neurodevelopment.

Síndrome de Klippel-Trenaunay en un recién nacido / Klippel-Trenaunay Syndrome in a newborn

Se describe el caso clínico de un recién nacido, asistido en el Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, sin antecedentes patológicos maternos ni familiares, quien presentaba macrocefalia, asimetría de los miembros inferiores, marcada hipertrofia en el miembro inferior izquierdo y macrodactilia, así como angiomas cutáneos en el área inferior del tronco, en el abdomen, la región glútea y las piernas. Luego de una evaluación detallada por parte de un equipo multidisciplinario, que se basó en el cuadro clínico y el estudio radiográfico, se diagnosticó el síndrome de Klippel-Trenaunay. El paciente ha mantenido una evolución favorable hasta el momento actual y se continúa un estricto seguimiento médico.

The case report of a newborn, assisted in Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba is described, without maternal or family pathological history who presented macrocephalic asymmetry of the lower members, marked hypertrophy in the left low member and macrodactilia, as well as cutaneous angiomas in the lower area of the trunk, abdomen, buttocks region and legs. After a detailed evaluation by a multidisciplinary team which was based on the clinical pattern and radiographical study, the Klippel-Trenaunay syndrome was diagnosed. The patient has maintained a favorable clinical course up to now and a strict medical follow-up is carried out.

Adrenal medullary hyperplasia with coexistent cerebral angiomas

Sporadic adrenal medullary hyperplasia (AMH) is a rare entity and mimics pheochromocytoma clinically as well as pharmacologically. It is characterized by increase in adrenal medullary cells with the expansion of cells into areas normally not seen. A 59-year-old male presented with chronic hypertension and raised 24-h urinary normetanephrine levels. Radiological and clinical possibility of pheochromocytoma led to left transperitoneal laparoscopic adrenalectomy. Histopathology, however, showed increase in adrenal medullary to cortical ratio, further confirmed by immunohistochemistry. The absence of any well-defined lesion led to the diagnosis of AMH. Furthermore, on routine imaging, two asymptomatic cavernous hemangiomas were seen. We present this case to reiterate that AMH is an entity which should be considered as a differential for pheochromocytoma. Furthermore, the presence of asymptomatic cavernous hemangiomas in the cerebrum, in this case, makes it rarer since this sporadic association is seldom seen.

Optical coherence tomography angiography of fleeting macroaneurysm in retinal racemose angioma

We present a rare case of retinal racemose angioma complicated with fleeting macroaneurysm (MA). A 50-year-old female presented with diminution of vision in her right eye for 6 years. Fundus examination showed a racemose angioma with hemorrhagic MA temporal to the fovea in the right eye. On subsequent follow-ups, spontaneous thrombosis of MA was noted with the development of new MA inferior to the fovea, with intraretinal hemorrhage extending into the fovea. Focal laser to MA resulted in resolution of MA with improvement in vision. We report optical coherence tomography angiographic features of the fleeting MA in a case of racemose angioma.

Surgical Treatment of Cavernous Angiomas in the Pineal Gland ­ Case Report

Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.

Síndrome de Millard-Gubler secundario a angioma cavernoso: reporte de un caso / Millard-Gubler syndrome secondary to cavernous angioma: report of a case

RESUMEN El síndrome de Millard-Gubler es considerado un síndrome protuberancial debido a una lesión pontina inferior y caracterizado, desde el punto de vista clínico, por parálisis facial y del VI par, ipsilateral a la lesión y parálisis braquiocrural contralateral; frecuentemente es de causa vascular, menos frecuente de causa traumática o por efecto de masa secundario a un tumor. Se realiza reporte de un caso de un paciente de 45 años de edad que acude al servicio hospitalario de la Fundación Centro Colombiano y Enfermedades Neurológicas FIRE con cuadro clínico caracterizado en pérdida de la fuerza muscular en hemicuerpo izquierdo, y déficit motor de la hemicara derecha, disartria y deterioro del estado de consciencia. La TAC de cráneo simple al ingreso evidenció sangrado extenso del tallo cerebral. De acuerdo con los hallazgos clínicos se concluye que el paciente presentó un síndrome de Millard-Gubler.

SUMMARY The Millard Gubler syndrome is considered a protuberant syndrome due to a lower pontine lesion and characterized, from the clinical point of view, by facial paralysis and VI pares, ipsilateral to the lesion and contralateral brachiocrural palsy; frequently it is of vascular cause, less frequent of traumatic cause or by effect of mass secondary to a tumor. A case report is presented of a 45-year-old patient who attends the hospital service of the Fundación Centro Colombiano y Neurológicas FIRE with a clinical picture characterized by loss of muscle strength in the left side of the body, and motor deficit of the right side of the face, dysarthria and deterioration of the state of consciousness. The simple skull CT on admission showed extensive brainstem bleeding. According to the clinical findings, it is concluded that the patient presented a Millard Gubler syndrome.

Clinical Outcome of Nonfistulous Cerebral Varices: the Analysis of 39 Lesions

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases. METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well. RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively. CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.

Clinical Outcome of Nonfistulous Cerebral Varices: the Analysis of 39 Lesions

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases.METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well.RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively.CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.

Recent advances in the diagnosis and treatment of Kasabach-Merritt phenomenon / 国际儿科学杂志

Kasabach-Merritt phenomenon (KMP),also known as Kasabach-Merritt syndrome,is a rare syndrome associated with Kaposiform haemangioendothelioma or tufted angioma,and characterized by thrombocytopenia and consumptive coagulopathy.KMP onsets early and progresses quickly.If diagnosis and treatment delayed,it can be life-threatening.In this paper,the progress of clinical manifestations,pathogenesis,diagnosis and treatment of KMP are summarized.

Angiomatosis encefalotrigeminal o síndrome de Sturge-Weber. A propósito de un caso / Encephalotrigeminal angiomatosis or Sturge-Weber syndrome. A propos of a case

El síndrome de Sturge-Weber consiste, en su forma completa, en la asociación de anomalías cerebrales, cutáneas y oculares. Desde el punto de vista clínico, se caracteriza por una mancha color vino en la cara, epilepsia, retraso mental, otras manifestaciones neurológicas deficitarias y glaucoma. Se presentó el caso de un paciente de 9 años de edad, masculino, piel negra, producto de parto eutócico, con peso de 4 200 g y antecedentes patológicos perinatales de cianosis, constatándose desde el nacimiento varios hemangiomas faciales. Al examen físico se confirma en cara y cráneo, una frente huidiza con presencia del hemangioma facial o hemangioma coroideo, glaucoma unilateral izquierdo, micrognatia. Se precisó mala oclusión dentaria, y en las extremidades superiores una clinodactilia del quinto dedo. Además, tiene el tronco asimétrico, retraso mental severo y aparecen cuadros convulsivos tónico-clónicos que repiten frecuentemente. Teniendo en cuenta todos estos elementos se comienza a sospechar el posible diagnóstico de un síndrome de Sturge-Weber (AU).

The Sturge-Weber syndrome is, in its complete form, the association of ocular, cutaneous and cerebral anomalies. From the clinical point of view, it is characterized by a wine-color spot on the face, epilepsy, mental retardation, other neurological deficit manifestations and glaucoma. We presented the case of a male, black patient, aged 9 years, who was born by eutocic delivery, with 4 200 g weight, and perinatal symptoms of cyanosis, finding several facial hemangioma from the moment of birth. At the physical examination, it was confirmed on the face and crania, evasive forehead with facial hemangioma or choroidal hemangioma, left unilateral glaucoma, micrognathia. Dental malocclusion was stated and clinodactyly of the fifth finger. Besides that, he has an asymmetric trunk, severe mental retardation and tonic-clonic convulsive episodes frequently repeated. Taking into account all these elements we began to suspect the possible diagnosis of a Sturge-Weber syndrome (AU).

Angioma en penacho: A propósito de un caso clínico / Tufted angioma: A case report

Se presenta el caso de una paciente de un año de edad, con una lesión tumoral congénita en dorso, eritemato-violácea. Se evidencia al cabo de un año durante el control evolutivo, aumento del volumen y cambio de coloración, por lo que se decide su exéresis completa. El examen histopatológico de la pieza concluyó con el diagnóstico de angioma en penacho (AP). El AP es un tumor vascular benigno, poco frecuente. Aparece sin predilección racial y es igual en ambos sexos. Puede ser congénito o adquirido en la primera infancia, ocasionalmente se presenta en la edad adulta. Su patogenia está poco dilucidada.

Presented the case of a patient of one year old with a congenital, erythematous-violaceous tumoral lesion on back. During the control evolutionary is evidence after a year increase of the volume and change of coloration by what is decides his removal complete. The histopathological examination of the piece ended with a diagnosis of tufted angioma (TA). The TA is a rare, benign vascular tumor. Appears no predilection racial and is equal in both sexes. It can be congenital or acquired in early childhood, it occurs occasionally in adulthood. Its pathogenesis is shortly to become.

13 cases of littoral cell angioma in spleens / 北京大学学报(医学版)

Objective: To investigate the clinicopathological features, morphological characteristics, immunophenotypes of littoral cell angioma (LCA) in spleen, and to provide new evidence for making diagnosis and avoiding misdiagnosis.Methods: Clinicopathological data, histological characteristics of 13 cases of LCA were retrospectively studied and immunohistochemical staining was imposed on the paraffi-nembedded specimens, and 5 cases of cavernous hemangioma, 4 cases of normal littoral cells of spleens were used as control groups, simultaneously.Results: All the 13 LCA patients included 7 males and 6 females, aged from 39 to 70 years with an average of 54.2 years and a median age of 55 years.Among these tumor patients, 6 cases were accompanied by malignances, benign tumors or inflammation states at abdominal cavities, and 7 cases were accidentally discovered by physical examinations.Grossly, spleens contained solitary or multiple gray red nodules ,which ranged from 0.5 to 6.2 cm in diameter.Histologically, tumors were composed by anastomosing vascular spaces which were lining by plump, rounded to cuboidal littoral cells that extended into vascular lumens.Usually, papillary frameworks that were covered by these cells were also seen extending into the lumens in some areas.Other types of histiocytoid cells were identified in lumens and the sizes were larger than the littoral cells.Both types of cells absented cytologic atypia.Immunohistochemical study demonstrated that the littoral cells in all cases were positive for vascular endothelial and histiocyte markers, such as CD21, CD31, CD68, polyclone FⅧRAg and ERG, while these cells were negative for CD8, CD34, and WT-1.These findings manifested that immunophenotype of littoral cell in LCA distinctive from that in controls.Conclusion: LCA is a benign lesion, which frequently occurs in the elderly.Its etiology remains confusion, however, immune dysregulation may associate with it because of the concomitance with other tumor or inflammation in some cases.The littoral cells in LCA show a hybrid endothelial-histiocytic phenotype on immunohistochemistry, therefore these cells may have features that intermediate between those of endotheliocytes and histiocytes.Emphasizing the histological findings and immunophenotypes is significant for diagnosis and differential diagnosis.

Tufted Angioma Presenting with Polycyclic Features Mimicking Tinea Faciale / 대한피부과학회지

Tufted angioma is a rare benign vascular neoplasm that usually occurs in children. It appears as an erythematous, purplish, indurated papule or plaque on the trunk or neck. Although it can have variable clinical manifestations, the annular or serpiginous configuration of lesions resembling tinea infections has rarely been reported. A 47-year-old woman presented with an erythematous plaque enlarging by peripheral extension to form polycyclic or serpiginous figures on her left chin and buccal cheek for 7 months without any subjective symptoms. Tinea faciale was considered as the initial clinical diagnosis, but repeated KOH tests were all negative; therefore, skin biopsy was performed. Histopathology revealed discrete circumscribed foci of capillaries scattered throughout the dermis showing a cannon ball appearance, compatible with tufted angioma. Clinical features showed considerable improvement after 2 sessions of intense pulsed light and pulsed-dye laser (5 repetitions) without any complications. Herein, we report a rare case of polycyclic variant of tufted angioma, which developed in a woman in her 5th decade.

Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain

PURPOSE: To characterize the development of glaucoma, age of glaucoma onset, and treatments for patients with a facial port-wine stain (PWS). METHODS: We performed a retrospective analysis of the medical records of 58 patients (116 eyes) with facial PWS between January 2000 and August 2016. We noted patients' age at the initial examination, cup-to-disc ratio, corneal diameter, occurrence of ocular hypertension, development of glaucoma, age of glaucoma onset, and treatments. We compared the clinical features of eyes that developed glaucoma with those that did not develop glaucoma. Among those eyes with glaucoma, we investigated the differences between eyes that underwent surgery and those that did not undergo surgery. RESULTS: Among the 58 patients with a facial PWS (116 eyes), glaucoma was diagnosed in 38 patients (46 eyes; 39.66%). Of these, 26 patients (27 eyes; 58.69%) underwent glaucoma surgery. PWS-associated glaucoma usually developed by the age of 2 years (85.61%). In all patients, glaucoma developed on the same side of the face as the PWS. Of the 58 patients, 19 (32.76%) showed neurological symptoms, including seizures, developmental delays, intellectual disabilities, or hemiplegia, and 32 (55.17%) were diagnosed with Sturge-Weber syndrome. The mean number of glaucoma surgeries was 1.55 ± 0.93. The initial surgery included trabeculectomy (7 eyes), trabeculotomy (5 eyes), combined trabeculotomy/trabeculectomy (13 eyes), and aqueous drainage device insertion (2 eyes). The mean age at the first surgery was 35.14 ± 50.91 months. In 18 of 27 eyes (66.67%), the postoperative intraocular pressure (IOP) was controlled to below 21 mmHg, but 9 eyes (33.33%) showed elevated IOP and required a reoperation. CONCLUSIONS: PWS can be accompanied by ocular hypertension or glaucoma, so patients require regular ophthalmic examinations. When glaucoma occurs, it often does not respond to medication, making it difficult in some cases to control the IOP, so appropriate glaucoma surgery is necessary.

Angioma en penacho congénito: Presentación de un caso de aspecto típico / Congenital tuft angioma: A typical presentation

El angioma en penacho es un raro tumor vascular benigno de comportamiento localmente agresivo que aparece en la infancia temprana; puede complicarse con el fenómeno de Kasabach-Merritt. Presentamos el caso de un paciente masculino de 2 años de edad con la lesión clínica característica.

Tufted angioma is a rare benign vascular tumor with a locally aggressive behavior that appears in early childhood; it may be complicated by the Kasabach - Merritt phenomenon. We report a 2 year old boy with a characteristic clinical lesion.