Hemangiolinfangioma mesentérico: reporte de un caso / Mesenteric hemangiolymphangioma: case report

El hemangiolinfangioma es un tipo muy raro de malformación del sistema vascular, caracterizado histológicamente por la presencia de vasos venosos y linfáticos dilatados quísticamente, cuyas células endoteliales de revestimiento son positivas para marcadores de inmunohistoquímica como CD31, CD34 y D2-40. El compromiso extenso retroperitoneal y del tracto gastrointestinal es infrecuente. Se presenta el caso de una paciente femenina de 24 años con antecedente de dolor pélvico crónico, con exacerbación de síntomas. El diagnóstico imagenológico mostró una masa retroperitoneal multiquística. Se hizo hemicolectomía derecha y resección de la masa, encontrándose que dicha lesión estaba íntimamente adherida al mesenterio con compromiso extenso del tracto gastrointestinal, y cuyo estudio histopatológico reveló un hemangiolinfangioma, con mejoría clínica posterior a la resección quirúrgica. Aportamos a la literatura mundial, la caracterización de los hallazgos clínicos, imagenológicos e histopatológicos de este tipo de malformaciones

Hemangiolymphangioma is a very rare type of malformation of the vascular system, characterized histologically by the presence of cystically dilated venous and lymphatic vessels, whose lining endothelial cells are positive for immunohistochemical markers such as CD31, CD34 and D2-40. Extensive retroperitoneal and gastrointestinal tract involvement is uncommon. We present the case of a 24-yearold female patient with a history of chronic pelvic pain with exacerbation of symptoms. The imaging diagnosis revealed a multicystic retroperitoneal mass. A right hemicolectomy and resection of the mass was performed, finding that the lesion was intimately adherent to the mesentery with extensive involvement of the gastrointestinal tract, and whose histopathological study revealed a hemangiolymphangioma, with clinical improvement after surgical resection. We contribute to the world literature with the characterization of the clinical, imaging and histopathological findings of this type of malformations

Linfangioma cavernoso de la lengua / Cavernous lymphangioma of the tongue

Los linfangiomas son malformaciones congénitas de los vasos linfáticos. Son raros en la cavidad oral y cuando se presentan en la misma pueden provocar trastornos funcionales del sistema estomatognático. El objetivo es presentar un caso de linfangioma cavernoso del dorso de la lengua, revisando los aspectos clínicos y de tratamiento de la enfermedad, con énfasis en la ubicación en la cavidad oral, fundamentalmente en la lengua. Un paciente de 17 años de edad se presenta con una lesión en el dorso de la lengua de aproximadamente 10 años de evolución. La lesión tiene una apariencia pedregosa y una base sésil, y mide aproximadamente 3 cm. El paciente es sometido a escisión quirúrgica de la lesión, sin intercurrencias. Actualmente el paciente se encuentra en seguimiento, sin signos clínicos de recidiva del tumor después de 2 años de tratamiento. La escisión quirúrgica de la lesión parece ser el tratamiento más apropiado para los linfangiomas de la lengua, con bajas tasas de recidiva(AU)

Lymphangiomas are congenital malformations of the lymphatic vessels. They are rare in the oral cavity and when present in this area they may cause functional disturbances of the stomatognathic system. The objective is to report a case of cavernous lymphangioma of the dorsum of the tongue, reviewing the clinical and treatment features of the disease, with emphasis on the oral cavity location, mainly the tongue. A 17-year-old male patient presented with a lesion on dorsum of his tongue of approximately 10 years of evolution. The lesion had a pebbly appearance and a sessile base, and measured about 3 cm. The patient was submitted to surgical excision of the lesion, without any intercurrences. Currently, the patient is under clinical follow-up, without any clinical sign of tumor recurrence after 2 years of treatment. Surgical excision of the lesion seems to be the most adequate treatment for tongue lymphangiomas, with low rates of recurrence(AU)

Neuronavegación en exéresis de linfangioma orbitario / Neuronavigation in exeresis of orbital lymphangioma

El linfangioma es un tumor benigno raro y predominante en la infancia, debido a su crecimiento puede comprometer al órgano donde se desarrolla, se han propuesto varias opciones de trata-miento, sin embargo, la cirugía continúa siendo la primera opción. La neuronavegación permite realizar exéresis con gran precisión y de utilidad en cirugía ocular, por lo que disminuye el riesgo de secuelas después de una exéresis de linfangioma orbitario.

Lymphangioma is a benign tumor predominantly in childhood, due to growth that can compromise the organ where it grows. Several treatment options, have been proposed however,surgery remains the first choice. Neuronavigation allows successful excision and use in eye surgery,which decreases the risk of sequels following excision of orbital lymphangioma.

Anesthesia for ex utero intrapartum treatment: renewed insight on a rare procedure / Anestesia para tratamento ex-útero intraparto: visão renovada sobre um procedimento raro

The ex utero intrapartum treatment is a rare surgical procedure performed in cases of expected postpartum fetal airway obstruction. The technique lies on a safe establishment of a patent airway during labor in anticipation of a critical respiratory event, without interrupting maternal-fetal circulation. Anesthetic management is substantially different from that regarding standard cesarean delivery and its main goals include uterine relaxation, fetal anesthesia and preservation of placental blood flow. We present the case of an ex utero intrapartum treatment procedure performed on a fetus with a large cervical lymphangioma and prenatal evidence of airway compromise. Modifications to the classic ex utero intrapartum treatment management strategies were successfully adopted and will be discussed in the following report.

O tratamento ex-útero intraparto é um procedimento cirúrgico feito em casos raros de obstrução esperada das vias aéreas fetais no pós-parto. A técnica tem como base o estabelecimento seguro de vias aéreas permeáveis durante o trabalho de parto em antecipação a um evento respiratório crítico, sem interromper a circulação materno-fetal. O manejo anestésico é substancialmente diferente daquele destinado à cesariana padrão e tem como principais objetivos o relaxamento uterino, a anestesia fetal e a preservação do fluxo sanguíneo placentário. Apresentamos o caso de um procedimento para tratamento ex-útero intraparto feito em feto com um grande linfangioma cervical e evidência pré-natal de comprometimento das vias aéreas. As modificações das estratégias adotadas no tratamento ex-útero intraparto clássico foram feitas com sucesso e serão discutidas no relato a seguir.

Exéresis de linfangioma en encía con electrobisturí / Exeresis of gingival lymphangioma with an electric scalpel

El linfagioma es considerado por la mayoría de los autores como una neoplasia benigna de los vasos linfáticos, que se puede presentar en cavidad oral. El 75 por ciento de los casos se presentan mayormente en la cabeza y el cuello. Cuando la boca se ve afectada, la lengua es el principal órgano que muestra alteraciones clínicas. El propósito de esta presentación fue describir un caso clínico de linfangioma en tejido gingival, tratado exitosamente mediante extirpación quirúrgica con electrobisturí. Se trata de una paciente de 13 años de edad, que acudió a consulta por presentar sangrado a nivel de la encía de molares inferiores derechos. Durante la anamnesis no se detectaron antecedentes familiares ni personales de interés. Los resultados de los exámenes de laboratorio se encontraron en los rangos normales. A partir de los hallazgos clínicos y radiográficos observados, se establecieron como diagnósticos presuntivos: granuloma piógeno, hemangioma o linfangioma. Se realizó fase higiénica con posterior escisión quirúrgica de la lesión empleando electrobisturí. Se efectuó análisis histopatológico. Se estableció como diagnóstico: linfangioma en encía a nivel de órganos dentales 46 y 47. Se obtuvo una cicatrización excelente 15 días después de la intervención. La localización inusual de este tipo de lesiones en la encía, y su tratamiento exitoso con electrobisturí, lo constituyen en un caso interesante, debido a los escasos reportes que sobre él existen en la literatura científica(AU)

To most authors, lymphangioma is a benign neoplasm of lymphatic vessels which may occur in the oral cavity. In 75 percent of the cases it presents in the head and neck. When the mouth is affected, the tongue is the main organ showing clinical alterations. The purpose of the study was to describe a clinical case of lymphangioma of gingival tissue successfully treated by surgical removal with an electric scalpel. A 13-year-old female patient attended consultation with bleeding from the gingival tissue next to the lower right molars. No personal or family antecedents of interest were detected during history taking. Laboratory results were within normal ranges. Based on clinical and radiographic findings, the following diagnoses were tentatively established: pyogenic granuloma, hemangioma or lymphangioma. Upon cleaning up the area, surgical removal of the lesion was performed using an electric scalpel. Histopathological examination was conducted. The following diagnosis was established: lymphangioma of the gingiva at the level of dental organs 46 and 47. Excellent scarring was obtained 15 days after the intervention. The singularity of this case lies in the unusual location of this type of gingival lesion, its successful treatment with an electric scalpel, and the fact that very few reports about the subject can be found in the literature(AU)

Vulvar Lymphangioma Circumscriptum.

Lymphangioma circumscriptum (LC) is a rare benign disorder of lymphatic channels in deep dermal and subcutaneous layers. Typically fluid filled vesicles that contain lymphatic fluid with frogspawn-like appearance are seen over any part of body. Vulvar LC is seen as congenital condition or might develop secondary to radiotherapy or surgery. LC involving the vulva is very rare; only 33 cases of vulvar LC have been reported. Surgical treatment is the most commonly used method to treat vulvar LC. In this report we present a case with LC secondary to radiotherapy and lesions regressed with cryotherapy.

E X I T (ex-utero intrapartum therapy) en linfangioma cervical fetal / EXIT procedure (ex-utero intra-partum therapy) for fetal neck lymphangioma

Se presenta un caso clínico de una embarazada primigesta de 17 años, con un feto con gran masa cervical a las 20 semanas, se diagnostica como linfangioma cervical. La evaluación prenatal concluye que existe gran riesgo de asfixia perinatal por obstrucción de la vía aérea superior, se resuelve el parto mediante procedimiento EXIT (ex-utero intrapartum therapy) a las 37 semanas. Se logra realizar intubación con larin-goscopia directa, con un tiempo de by-pass uteroplacentario de 7 minutos. Se obtiene un recién nacido de 3300 g, al segundo día se opera del tumor con buenos resultados. Se revisa el protocolo del procedimiento EXIT en sus aspectos anestésicos, obstétricos, quirúrgicos y neonatológicos. Se concluye que el EXIT debe ser planteado en todo caso en que se sospeche obstrucción de la vía aérea superior y puede ser realizado en hospitales que cuenten con equipamiento habitual y un equipo médico multidisciplinario.

We report a case of primigravida patient, 17 years old, with a fetus showing a large cervical mass at 20 weeks of gestation and was diagnosed as a cervical lymphangioma. The prenatal evaluation concludes that there exists a great risk of perinatal asphyxia due to obstruction of the upper airway and therefore it is decided to perform a cesarean section at 37 weeks of gestation, using an EXIT procedure (ex-utero intra-partum therapy). We perform intubation with a semi- rigid tube having a by-pass time utero-placental of 7 minutes, obtaining a newborn of 3300 g at birth. The newborn is operated two days after birth removing the cervical tumor with good results. We review the protocol of the EXIT procedure concerning aspects related to anesthesia, obstetrics, surgery and neonatal care. We conclude that EXIT should be considered in all cases in which obstruction of the upper airway is suspected, and can be performed in hospitals that have basic surgical facilities and a multidisciplinary team.

Tonsilla Lymphangioma.

Lymphagiomas are uncommon benign tumours of tonsils. Lymphangiomas are benign lymphatic tumours typically composed of dialated lymphatic channels which often occur subcutaneously in the head and neck region. Tonsillar lymphangiomas have been reported rarely in the literature which presents as a mass lesion. Histological confirmation is necessary for diagnosis. We report a case of lymphangioma of the tonsil in an otherwise well 17 year old female patient and review the clinical and histological features of this tumour. These lesions are usually cured by simple surgical excision.

Linfangiomatosis mesentérica como causa de abdomen agudo en la tercera edad / Mesenteric lymphangiomathosis presenting as an acute abdomen in an older subject

We report a 71 years old male who consulted in the emergency room for abdominal pain lasting 12 hours. On physical examination there was pain on abdominal palpation and signs of peritoneal irritation. An abdominal CT scan showed a thickening of the medial and distal ileum, multiple adjacent collections and signs of peritonitis. The patient was operated, observing multiple white tumors in the mesentery and serosa of small bowel, one of these lesions, adhered to the bowel wall, had a hemorrhagic infarct. One of the lesions was punctured, obtaining a milky fluid. Biopsies were obtained and the infarcted lesion was excised. The pathological study reported a Mesenteric Multilocular Lymphangioma. The patient had an uneventful postoperative evolution.

Presentamos el caso clínico de un hombre de 71 años, sin antecedentes mórbidos, salvo apendicectomía y colecistectomía hace más de 20 años. Consulta en servicio de urgencias por dolor abdominal de 12 horas de evolución, sin otros síntomas asociados. Al examen físico destacaba dolor abdominal e irritación peritoneal a la palpación de hipocondrio izquierdo. Exámenes destacan aumento de parámetros inflamatorios, Ia tomografía computada de abdomen y pelvis revela engrosamiento de íleon medio-distal, con múltiples colecciones adyacentes y signos de peritonitis. Se realiza laparotomía exploradora, evidenciándose múltiples tumoraciones blanquecinas en mesenterio y serosa de todo el intestino delgado, una de las cuales se aprecia con infarto hemorrágico adherida a Ia pared abdominal. Punción de lesiones da salida a líquido lechoso. Se toman biopsias y se reseca lesión infartada. Paciente evoluciona favorablemente, dado de alta al tercer día. La histología reveló un Linfangioma Quístico Multilocular Mesentérico. Pese a ser una patología infrecuente, debe ser considerada dentro de los diagnósticos diferenciales de abdomen agudo, siendo extremadamente rara su presentación en Ia tercera edad.

Linfangioma mesentérico / Mesentery lymphangioma

El linfangioma representa el 6 por ciento de los tumores benignos, y su localización más frecuente es la región cervical, seguida de la axila en el 95 por ciento de los casos. Solo un 5 por ciento tiene localización en otro sitio. Dentro de estos, los abdominales representan entre el 2 y el 5 por ciento , y los más frecuentes son los del mesenterio. Se presenta el caso de una paciente de 45 años con el diagnóstico de metrorragia disfuncional, que a pesar del tratamiento hormonal, aumentó en intensidad. Se le practicó un legrado diagnóstico, cuyo resultado fue hiperplasia adenomatosa con ligera atipia, y se le indicó una histerectomía abdominal, que se realizó sin dificultad. En el transcurso de esta intervención se llevó a cabo la extirpación de un tumor del mesenterio del yeyuno, cuyo diagnóstico histológico definitivo fue linfangioma mesentérico(AU)

The lymphangioma accounts for the 6 percent of the benign tumors and its more frequent location is the cervical region, followed by the axilla in the 95 percent of cases. Only the 5 percent is located in other site including the abdominal ones accounting for the 2 and the 5 percent and the more frequent are those of mesentery. This is the case of a patient aged 45 diagnosed with dysfunctional metrorrhagia that despite the hormonal treatment increased its intensity. A diagnostic curettage was made whose result was an adenomatous hyperplasia with a slight atypia prescribing an abdominal hysterectomy carried out without problem. During this intervention a yeyunal mesenteric tumor was removed with a definitive histological diagnosis of mesenteric lymphangioma(AU)

Malformación linfangiomatosa intestinal con tejido pancreático ectópico: a propósito de un caso y revisión de la literatura / Intestinal lymphangiomatosis with ectopic pancreatic tissue: a case report and literature review

Intestinal lymphangiomatosis is a rare malformation typically affecting children. Its etiology is unknown, although several hypotheses have been proposed. We present a case of intestinal lymphangiomatosis with ectopic pancreatic tissue of the mesentery, an association not previously described in literature. We emphasize the fact that this entity has sometimes been associated with other intra-abdominal malformations, even conditions undescribed in scientific literature. It must be considered the first differential diagnosis for pediatric patients with radiological evidence of mesenteric cystic lesions, subsequently confirmed by pathological examination.

La malformación linfangiomatosa intestinal es una entidad poco frecuente, típica de la edad pediátrica, cuya etiopatogenia es desconocida, aunque se barajan varias hipótesis. Presentamos un caso de linfangiomatosis intestinal con tejido pancreático ectópico mesentérico, una asociación no descrita anteriormente en la literatura. Destacamos la importancia de conocer que esta entidad, en ocasiones, se asocia a otro tipo de malformaciones intrabdominales, incluso no descritas previamente en la literatura. Se debe plantear como el primer diagnóstico diferencial durante la edad pediátrica, ante el hallazgo radiológico de lesiones quísticas mesentéricas, confirmando posteriormente mediante un estudio anatomopatológico de las lesiones.

Hepatectomia por linfangioma em situs inversus totalis : relato de caso / Hepatectomy of lymphangioma in situs inversus totalis : case report

We present a case of hepatectomy in a patient with liver's lymphangioma associated with Situs Inversus Totalis. The first case of medical literature.

Linfangioma orbitário: relato de caso / Orbital lymphangioma: case report

Descrevemos um caso de linfangioma orbitário em uma paciente de nove anos de idade que apresentava proptose à direita (Hertel= 29 mm), acompanhada de restrição da motilidade ocular, dor e perda visual decorrente de neuropatia óptica compressiva. A ressonância magnética demonstrou a lesão expansiva, localizada na órbita direita, de aspecto cístico, não infiltrativa, extraconal e com sinais sugestivos de hemorragia intralesional. Não houve melhora com corticoterapia oral. Foi, então, realizada punção via transconjuntival, com aspiração de 35 ml delíquido "cor-de-chocolate" (confirmado como hemorrágico pela citologia). Ocorreram exacerbações do quadro clínico, manifestadas por dor e piora da proptose, devido à hemorragia intralesional, optando-sepela exérese dos cistos orbitários, usando o acesso orbitário lateral (cantólise e retirada de parede orbitária lateral, a qual não foi recolocada para efeito de descompressão) e inferior (transconjuntival inferior), com resolução do quadro. CONCLUSÃO: Foi relatado um caso de linfangioma, uma doença desafiadora, de difícil tratamento, com potenciais complicações visuais e estéticas, além da possibilidade de recidivas frequentes.

A case of orbital lymphangioma in a 9 year-old female, with proptosis (Hertel= 29 mm), ocular motility restriction, pain and visual loss due to compressive optic neuropathy is described. A magnetic resonance imaging (MRI) exam showed an expansive cystic lesion in the extraconal space of the right orbit, non-infiltrating with intralesional hemorrhage. The patient had no improvement with steroid treatment. A transconjunctival puncture with 35 ml aspiration of a "chocolate color" fluid (which was confirmed by cytologic study as a hemorrhagic fluid) was performed. Exacerbations occurred after an intralesional hemorrhage (pain and increase of proptosis). The lesion was excised via lateral cutaneous approach (a lateral canthotomy and removal of the right orbital wall was performed done for decompression) and lower transconjunctival approach. CONCLUSION: Lynphangioma is a challenging disease and difficult to treat, with potential visual and cosmetic complications and the possibility of frequent recurrences.

Surgical treatment of oral lymphangiomas with CO2 laser: report of two uncommon cases

This paper reports the treatment of oral lymphangiomas with carbon dioxide CO2 Laser. Lymphangiomas are rare congenital lymphatic malformations. These lesions are most frequently diagnosed during childhood, are most commonly located in the head and neck region, and are extremely rare in the oral cavity. Oral lymphangiomas are of complex treatment due to the difficulty in performing a complete excision. CO2 laser is the most often used laser in the oral cavity due to its affinity with water and high absorption by the oral mucosa. Several benefits of the use of CO2 laser have been reported for surgical oral procedures. The cases reported herein were biopsy-proven lymphangiomas of the oral cavity. The surgical procedures were carried out under local anesthesia and a focused CO2 laser beam (l10.600 nm, f ~2 mm, CW/RSP) was used. At the end of the surgery, the laser beam was used on a defocused mode to promote better hemostasis. Neither sutures nor dressings were used after surgery. No medication and only mouthwashes were prescribed to all patients on the postoperative period. There were no postsurgical complaints from the patients and no relapses of the conditions were observed after follow-up periods of 12 and 18 months. The use of CO2 laser was practical, easy to carry out and effective on the treatment of oral lymphangiomas, with no lesion recurrence.

O objetivo deste trabalho é relatar o tratamento de linfangiomas orais com o laser de CO2. Os linfangiomas são raras malformações linfáticas congênitas que geralmente são diagnosticados na infância. São localizados preferencialmente na região de cabeça e pescoço, mas são extremamente raros na cavidade oral. As lesões da cavidade oral são de tratamento complexo, devido à dificuldade em exercer uma completa remoção. O laser de CO2 é o laser mais usado na cavidade oral devido à sua afinidade com a água e alta absorção pela mucosa oral. Diversos benefícios da utilização deste aparelho são relatados na literatura sobre a relação de procedimentos cirúrgicos realizados na cavidade oral. Os casos relatados são de linfangiomas comprovados por biópsia prévia. Os procedimentos cirúrgicos foram realizados sob anestesia local com um feixe de laser de CO2 no modo focado (20C Sharplan Laser Indústrias Israel, l10.600 nm, f ~2 mm, CW/RSP). Ao final da cirurgia foi utilizado um feixe de laser no modo desfocado, para promover uma melhor hemostasia. Nem suturas e curativos foram realizados após a cirurgia. Nenhuma medicação foi utilizada, somente anti-sépticos bucais foram prescritos para os pacientes no período pós-operatório. Não houve queixas no pós-operatório dos pacientes e nem recidivas após acompanhamentos de 12 e 18 meses. A utilização do laser de CO2 é um método prático, fácil e eficaz no tratamento de linfangiomas orais, sem recidiva nos períodos de acompanhamento.

Cystic lymphangiomatous hamartoma masquerading as massive ascites.

We report a 4-year-old boy presenting with a tense massive ascites and large hydrocele. History and physical examination were unremarkable. Routine laboratory studies were normal. Abdominal ultrasonography revealed massive ascites. Contrast CT was suggestive of a large cyst covering the entire peritoneal cavity. At laparotomy, a large cystic tumor was found extending into the scrotum through the left inguinal ring. Histopathologic examination diagnosed the tumor as a cystic lymphangiomatous hemartoma. Although abdominal lymphangiomas are seen in children, but presenting as massive ascites with hydrocele is very rare.

Abordaje de la vía aérea en una lactante con linfangioma cervical / Access to the aerial tract in a breastfed child with cervical lymphangioma

Las grandes tumoraciones de cuello se han asociado con dificultades en la intubación. Se realizó la valoración preoperatoria de la vía aérea a través de la historia, el examen físico y los complementarios. Y se consideró una vía aérea difícil y se efectuó inducción con halotano logrando la intubación de la tráquea sin complicaciones. La inducción inhalatoria permitió la intubación endotraqueal en una paciente considerada como una vía aérea difícil.

The big tumors in the neck have being associated with intubation difficulties. We made the presurgery evaluation of the aerial tract through the medical records, physical and complementary examinations. We considered a difficult aerial tract and made the induction with halothane, achieving tracheal intubation without complications. The inhalant intubation allowed the endotracheal intubation in a patient considered as a difficult aerial tract.