Malformação venolinfática em borda lateral de língua: relato de caso / Venolymphatic malformation in lateral edge of the tongue: case report

Resumo As malformações vasculares são anomalias que podem acometer veias, vasos linfáticos e artérias de forma isolada ou mista. Quando se apresentam de forma mista, com componentes venosos e linfáticos, são denominadas malformação venolinfática ou linfático-venosa, de acordo com sua constituição predominante. Embora seja um distúrbio benigno de bom prognóstico, é localmente invasivo, podendo levar a deformidade e havendo, ainda, a propensão de recorrência local. O presente artigo traz um caso de malformação venolinfática com localização incomum em borda lateral de língua, abordando-se a conduta clínica e o referencial teórico vigente.

Abstract Vascular malformations are vascular anomalies that can affect veins, lymphatic vessels, and/or arteries in isolated or mixed form. When they present in the mixed form with venous and lymphatic involvement, they are called venolymphatic or lymphatic-venous malformations, depending on their predominant component. Although these are benign disorders with good prognosis, they are locally invasive and may lead to deformity, while there is also a propensity for local recurrence. This article presents a case of venolymphatic malformation with unusual localization on the lateral border of the tongue, addressing the clinical conduct and the current theoretical framework.

Liver transplantation in the setting of a spontaneous shunt between superior mesenteric vein and right renal vein.

Shunts between the superior mesenteric vein (SMV) and the right renal vein (RRV) are very rare. Here, we describe and depict the rare case of a liver transplant (LT) in the setting of shunt between SMV and RRV. A 67-year-old white man presenting with Child C cirrhosis secondary to hemochromatosis and persistent encephalopathy was listed for LT. Preoperative abdominal angiotomography revealed the presence of a large spontaneous shunt between the SMV and the RRV. The patient underwent LT by receiving a liver from a 17-year-old brain-dead deceased donor victim of trauma. A large shunt between the SMV and the RRV was confirmed intraoperatively. Although there was no portal vein (PV) thrombosis, the PV was atrophic and had a reduced flow. PV pressure was 22mmHg (an arterial line was inserted inside the PV stump, and this line was connected to a common pressure transducer, the pressure readings was expressed in the anesthesia monitor). After shunt ligation PV pressure increased to 32mmHg. There were no post-transplant vascular complications, and the patient was discharged home in good health. Preoperative study of all LT candidates with angio CT scan is mandatory. Whenever there is PV thrombosis, an attempt to remove the entire thrombus is warranted. After thrombectomy or whenever there is not PV thrombosis, all large shunts should be ligated. PV pressure and flow should be measured before and after shunt ligation. In the absence of PV thrombosis, ligation of the shunt should enable an increase in PV flow and pressure, as reported herein.

Efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas / Effectiveness of propranolol in treatment of cutaneous vascular malformations

Se realizó un estudio cuasi-experimental de series temporales para evaluar la efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas en 48 pacientes que asistieron a la consulta del Servicio de Dermatología del Hospital Central Universitario Dr. Antonio María Pineda durante el período febrero-julio 2018. Los resultados muestran que existen diferencias estadísticamente significativas (p <0.05; p0,0001) antes y después del primer mes de tratamiento con propranolol, las cuales se mantiene hasta los seis meses, con respecto al tamaño, color, consistencia y temperatura. Se espera que los resultados sirvan para proponer el uso de propranolol como una opción terapéutica no invasiva en el tratamiento de las malformaciones vasculares cutáneas(AU)

A quasi-experimental study of time series was carried out to evaluate the effectiveness of propranolol in the treatment of cutaneous vascular malformations in 48 patients attending the Dermatology Service of the Hospital Central Universitario Dr. Antonio Maria Pineda during the period February - July 2018. The results show that there are statistically significant differences (p <0.05; p0,0001) before and after treatment with propranolol starting one month post-treatment which are kept until six months, related to size, color, consistency and temperature of lesions. We hope that these results will encourage the use of propranolol as a non-invasive therapeutic option in the treatment of cutaneous vascular malformations(AU)

Symptomatic, unilateral, isolated, complete persistent sciatic vein.

Persistent sciatic vein is a rare anomaly of mesodermal development. When found, it is often associated with varicosities in abnormal distributions, pain, limb congestion, and hypertrophy. In this report, we describe a case of an isolated, complete persistent sciatic vein with an atypical anatomy and onset.

Abernethy malformation with multiple aneurysms: incidentally found in an adult woman with Caroli's disease.

Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. We present the case of a 28-year-old woman who was incidentally diagnosed with type II Abernethy malformation and multiple aneurysms during an investigation for nonspecific abdominal pain and fever. The patient had been diagnosed with Caroli's disease at the age of 10 and liver cirrhosis, portal hypertension a few years before. To the best of our knowledge, this is the first case reported with all such congenital anomalies associated together. Ultrasound, computed tomography, including three-dimensional reconstruction, and magnetic resonance imaging were performed which revealed a side-to-side shunt between the extrahepatic portal vein and the inferior vena cava, multiple aneurismal cystic dilation of the spleen artery and left renal artery, and extensive intrahepatic bile duct cysitic dilation with calculus formation. Etiology, clinical significance and management strategies with regard to these abnormalities are discussed.

Malformaciones Vasculares / Vascular malformation

Las malformaciones vasculares son anomalías presentes siempre desde el nacimiento que, al contrario de los hemangiomas, nunca desaparecen; pueden crecer durante toda la vida por hipertrofia. Según la clasificación de la ISSVA, las malformaciones vasculares se dividen en función del vaso afectado en capilares o venulares, venosas, linfáticas, arterio-venosas y combinadas o complejas. Cada una de ellas, con unas peculiaridades clínicas y hemodinámicas definitorias.

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress; and may hypertrophy during lifetime. According to the ISSVA classification, vascular malformations are divided, depending on the affected vessel, into capillary or venular, venous, lymphatic, arteriovenous and combined or complex; each with certain defining clinical and haemodynamic peculiarities.

Isolated absence of the right pulmonary artery as a cause of massive hemoptysis.

Isolated absence of a pulmonary artery is a rare cause of massive hemoptysis. We report a case of unilateral absence of the right pulmonary artery in an adult without any other cardiovascular anomalies. The patient presented massive hemoptysis, which was successfully treated with a right pneumonectomy.

Multiple vascular anomalies in the abdomen: a gross anatomical study / Múltiples anomalías vasculares en el abdomen: un estudio anatómico macroscópico

Knowledge of the branching pattern of the abdominal aorta is clinically important for any abdominal surgeon operating on parts of the gut or neighboring structures like the suprarenals, spleen, pancreas, liver, kidneys and ureter. The presence of abnormal inferior phrenic artery associated with aberrant branch from the celiac trunk supplying the pancreas and duodenum is a rare anomaly. In the present case, we observed four branches of the celiac artery i.e. (a) left gastric artery (b) common hepatic artery (c) splenic artery and (d) an aberrant branch, which took a course inferiorly towards the pancreas. The aberrant artery supplied the body of the pancreas and gave a branch which supplied the horizontal part of the duodenum and then entered the transverse mesocolon to supply the hepatic flexure and some portions of the ascending and the transverse colon. The inferior phrenic artery was absent on the left side. Concomitant anomalies of such type are to be kept in mind by the surgeon, while operating cases of carcinoma head of pancreas and performing kidney transplantations.

El conocimiento del patrón de ramificación de la aorta abdominal es clínicamente importante para cualquier cirujano abdominal que opere en partes del intestino o estructuras vecinas, como glándulas suprarenales, bazo, páncreas, hígado, riñones y uréteres. La presencia anormal de la arteria frénica inferior asociada con una rama aberrante originada del tronco celiaco, supliendo el páncreas y duodeno, es una variación anatómica rara. En el presente caso, se observaron cuatro ramas de la arteria celiaca: (a) arteria gástrica izquierda (b) arteria hepática común (c) arteria esplénica y (d) una rama aberrante, que tuvo un curso inferior hacia el páncreas. La arteria aberrante suministraba irrigación al cuerpo del páncreas y daba una rama para la parte horizontal del duodeno para luego entrar en el mesocolon transverso para irrigar la flexura hepática y algunas partes del colon ascendente y transverso. La arteria frénica inferior estaba ausente en el lado izquierdo. Anomalías concomitantes de este tipo deben ser consideradas por el cirujano, en casos de operación de carcinoma de cabeza de páncreas y la realización de trasplante renal.

Phacomatosis pigmentokeratotica: a follow-up report documenting additional cutaneous and extracutaneous anomalies.

This is a follow-up report on a boy with phacomatosis pigmentokeratotica. At the age of 10 years he had, in addition to a sebaceous nevus and a speckled lentiginous nevus, multiple lesions of a collagen nevus localized on the chin and in the lumbar area. On the left shoulder, a small telangiectatic spot was present within the area of the speckled lentiginous nevus. Moreover, hemiatrophy of the left-hand side of the body and hyperhidrosis of the left lumbar area were noted. At the age of 16, the lesions of his collagen nevus had considerably enlarged and showed an arrangement along Blaschko lines. Additional pinhead-sized vascular lesions were noted, with preponderance within the area of the speckled lentiginous nevus in the left scapular region and on his left leg. Moreover, the boy had developed severe arterial hypertension since the age of 13. Angiographic examination showed an aortic stenosis that reached from the aortic arch down to the origin of the renal arteries, necessitating a surgical intervention. From this follow-up report we conclude that phacomatosis pigmentokeratotica may be associated with other cutaneous abnormalities such as linear connective tissue nevus of the collagen type and multiple pinhead-sized angioma-like lesions superimposed on the speckled lentiginous nevus. The associated defects of the large vessels may belong to the component of Schimmelpenning syndrome representing one "half" of phacomatosis pigmentokeratotica, rather than being part of the speckled lentiginous syndrome that forms the other "half" of this twin-spot phenotype.

Effectiveness of low flow vascular lesions sclerosis with monoethanolamine: report of six cases.

Vascular malformations or even hemangiomas need therapeutic intervention if they start to cause clinical symptoms or personal discomfort. Different therapeutic modalities, including cryotherapy, corticosteroids, laser therapy, sclerotherapy, surgery, and/or embolization, can be performed successfully. Sclerotherapy with monoethanolamine is a relatively simple and effective method to treat low flow vascular lesions. We presented a report of six cases of vascular malformations treated with monoethanolamine. There were 3 male and 3 female patients, with an age range of 20 to 68 years. The patients were submitted to applications according to clinical response and/or tolerability. In all cases, low-flow vascular lesions were recorded and submitted to infiltration with 2.5% monoethanolamine, directly into the lesions. The volume applied was approximately the middle of affected area. Vascular lesions were characterized as low-flow due to absence of arterial pulsation and flat consistence. The sclerosis with 2.5% monoethanolamine resulted in complete or partial involution, without severe complications.