Clínica, auxología y calidad de vida en cincuenta niños, niñas y adolescentes con síndromes de sobrecrecimiento corporal segmentario de un único centro / Clinical and auxological characteristics and quality of life of 50 children and adolescents with segmental overgrowth syndromes at a single center

Introducción. Los síndromes de sobrecrecimiento corporal segmentario son un grupo de enfermedades poco frecuentes caracterizadas por exceso de crecimiento en una o más partes del cuerpo relacionadas, en su mayoría, con mutaciones en mosaico en la vía de señalización AKT/PI3K/mTOR y RAS-MAPK. Nuestro objetivo fue analizar las características clínicas y auxológicas, y la calidad de vida relacionada a salud (CVRS) en este grupo de pacientes en un hospital de tercer nivel de atención. Población y métodos. Estudio transversal de una cohorte en seguimiento. Se analizaron edad, sexo, datos sociodemográficos, mediciones antropométricas del segmento afectado y del contralateral, complicaciones, tratamiento, calidad de vida (PedsQL4.0) y dolor. Se calcularon medidas centrales y de dispersión. Se realizó análisis univariado entre calidad de vida y variables incluidas. Resultados. Se incluyeron 50 pacientes, 29 varones. Mediana de edad 9,95 (r 1,44-17,81) años. El diagnóstico más frecuente fue síndrome de sobrecrecimiento relacionado a PIK3CA (PROS) (37/50). Mediana de número de segmentos afectados 2 (r: 1-7) por niño. Cuarenta casos presentaron malformación vascular; 20, capilar. El dolor (24/50) fue la complicación más frecuente. Treinta y un pacientes mostraron asimetría de longitud de miembros inferiores, < 5 cm. La estatura se ubicó entre los centilos 50 y 97 en la mayoría de los niños. Menor CVRS se observó en mujeres, en pacientes con malformación vascular compleja y necesidades básicas insatisfechas (NBI). Conclusiones. PROS fue el diagnóstico más frecuente. El dolor fue una complicación frecuente. La CVRS fue menor en mujeres, pacientes con malformación vascular combinada y NBI.

Introduction. Segmental overgrowth syndromes are a group of rare diseases characterized by overgrowth in one or more parts of the body, mostly related to mosaic mutations in the AKT/PI3K/mTOR and RASMAPK signaling pathway. Our objective was to analyze the clinical and auxological characteristics and health-related quality of life (HRQoL) in this group of patients at a tertiary care hospital. Population and methods. Cross-sectional study of a follow-up cohort. Age, sex, sociodemographic data, anthropometric measurements of the affected and contralateral segments, complications, treatment, quality of life (PedsQL 4.0), and pain were analyzed. Central and dispersion measures were estimated. A univariate analysis between the quality of life and study variables was done. Results. A total of 50 patients were included; 29 were males. Median age: 9.95 (r: 1.44­17.81) years. The most common diagnosis was PIK3CA-related overgrowth spectrum (PROS) (37/50). The median number of affected segments was 2 (r: 1­7) per patient. Vascular malformations were observed in 40, and capillary malformations, in 20 patients. Pain was the most common complication (24/50). An asymmetry of the lower extremities of < 5 cm was observed in 31 patients. In most children, height was between the 50th and 97th percentiles. A lower HRQoL was observed among girls, patients with complex vascular malformations, and those with unmet basic needs (UBNs). Conclusions. PROS was the most common diagnosis. Pain was the most common complication. HRQoL was lower among girls, patients with combined vascular malformations, and those with UBNs.

An Unusual Cause of Posterior Knee Pain: Mucoid Degeneration of the Anterior Cruciate Ligament - Case Report / Uma causa incomum de dor posterior no joelho: degeneração mucóide do ligamento cruzado anterior - Relato de caso

Abstract Mucoid degeneration of the anterior cruciate ligament (ACL) is an uncommon cause of pain in the posterior region of the knee, of unknown pathophysiology and underdiagnosed. The best treatment modality is still under discussion. Resection of the lesion with partial ACL debridement has shown good results without the occurrence of instability. The authors present a case of mucoid degeneration of the ACL treated with resection of the mucoid degeneration and partial debridement of the ACL by arthroscopy.

Resumo A degeneração mucoide do ligamento cruzado anterior (LCA) é uma causa pouco frequente de dor na região posterior do joelho, de patofisiologia desconhecida e subdiagnosticada. A melhor modalidade de tratamento ainda está em discussão. A ressecção da lesão com desbridamento parcial do LCA tem apresentado bons resultados, sem a ocorrência de instabilidade. Os autores apresentam um caso de degeneração mucoide do LCA tratado com ressecção da degeneração mucoide e desbridamento parcial do LCA por artroscopia.

Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso / Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

Comparison of pingyangmycin fibrin glue composite and pingyangmycin dexamethasone composite in the treatment of pharyngolaryngeal venous malformation / 中华耳鼻咽喉头颈外科杂志

Objective: To analyze and compare the efficacy and safety of pingyangmycin fibrin glue composite (PFG) and pingyangmycin dexamethasone composite (PD) in the treatment of pharyngolaryngeal venous malformation (VM). Methods: The clinical data of 98 patients with pharyngolaryngeal VM who underwent sclerotherapy with pingyangmycin composite in the First Affiliated Hospital of Sun Yat-sen University from June 2013 to November 2022 were retrospectively analyzed. According to their treatment, patients were divided into PFG group (n=34) and PD group (n=64), among those patients there were 54 males and 44 females, aged 1-77(37.06±18.86)years. The lesion size, total treatment times and adverse events were recorded before and after treatment. And the efficacy was divided into three grades: recovery, effective and invalid. According to the length of VM, all patients were divided into three subgroups, to compare the differences in efficacy and treatment times between each two groups.And finally the adverse events and their treatments were analyzed. SPSS 25.0 software was used for statistical analysis. Results: The efficacy of PFG group was 94.11%(32/34), the recovery rate was 85.29%(29/34).And the efficacy of PD group was 93.75%(60/64), the recovery rate was 64.06%(41/64). No serious adverse eventst occurred in subgroup comparison, there was no statistical difference between the two groups in efficacy and the times of treatments when the length was≤3 cm (Zefficacy=1.04, ttreatment times=2.18, P>0.05); when the length was 3-5 cm, there was no significant efficacy difference between the two groups(Zefficacy=1.17, P>0.05), but the treatment times of PFG were less (ttreatment times=4.87, P<0.01); when the length≥5 cm, efficacy of PFG was significantly better than PD (Zefficacy=2.94, P<0.01), and had fewer treatments times (ttreatment times=2.16, P<0.01). There were no serious adverse events in either group during treatment and follow-up. Conclusion: Both PFG and PD are safe and effective composite sclerotherapy agent for the treatment of laryngeal VM, but PFG has a higher cure rate and fewer treatment times for massive lesions.

Oral sirolimus in the treatment of adult eruptive cherry angiomas

Introduction@#Cherry angiomas are a common type of acquired vascular proliferation of the skin which manifest as single or multiple bright red spots that usually appear on the trunk and arms. They are generally asymptomatic; patients may opt to remove the lesions for cosmetic reasons and prevention of bleeding. Conventionally, most cherry angiomas are treated with curettage, laser, and electrosurgery. Herein, we report a case of multiple cherry angiomas managed alternatively with oral sirolimus. @*Case@#A 47-year-old Filipino female presented with a 10-month history of gradually enlarging multiple bright-red papules and pedunculated nodules with a propensity to spontaneously bleed on gentle manipulation involving the scalp and forehead. Clinicopathological correlation suggests a diagnosis of eruptive cherry angiomas. The patient was started on oral sirolimus, a mammalian target of rapamycin (mTOR) inhibitor.@*Conclusion@#We present a case of a patient with eruptive cherry angiomas who experienced significant decrease in size and bleeding with treatment of oral sirolimus with minimal adverse effects. For patients with eruptive cherry angiomas, especially with contraindicated comorbidities, first-line therapeutic option treatments with oral sirolimus can be beneficial.

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

A life‑threatening benign vascular lesion of the uterus, cavernous hemangioma: A case report / Philippine Journal of Obstetrics and Gynecology

@#The objective of this clinical report is to present the first local case of rare cavernous uterine hemangioma. This is a case of a 28‑year‑old G2P1 (1001) during her first trimester of pregnancy who was admitted to our institution for the second time due to profuse vaginal bleeding and severe anemia. The transvaginal scan revealed an embryonic demise of 8 weeks age of gestation noted at the endocervical canal. There is a posterofundal heterogeneous mass measuring 6.3 cm × 5.7 cm × 5.0 cm (volume: 94.2 ml) with multiple cystic spaces, which on Doppler studies showed abundant vascularity suggestive of uterine hemangioma. Antifibrinolytics were administered. The patient underwent emergency hysterectomy with bilateral salpingectomy due to profuse vaginal bleeding with histopathology result of cavernous hemangioma of the uterus. Cavernous uterine hemangioma is a rare vascular lesion that poses a great challenge in the diagnosis and management. There were limited published articles regarding cavernous hemangioma of the uterus. There were conservative treatment options such as uterine artery embolization, the use of hormonal oral contraception, intralesional glucocorticoid therapy, the use of interferon‑α, laser therapy, and surgical excision. Hysterectomy is the definitive treatment for intractable bleeding. It is recommended to establish an international registry for this rare case. The experts in different specialties such as obstetrician‑gynecologists, interventional radiologists, and vascular surgeons, can also formulate an algorithm for its diagnosis and treatment.

Uso del sildenafilo oral en el tratamiento del linfangioma orbitario / Use of Oral Sildenafil in the Treatment of Orbital Lymphangioma

Los linfangiomas orbitarios son malformaciones vasculares benignas, de crecimiento lento, abortivas y no funcionales, que se presentan principalmente en la primera década de la vida. Las opciones terapéuticas en estos casos presentan una resolutividad limitada, algunos tratamientos suelen ser agresivos y provocar daños del aparato visual. Se presenta un caso de una paciente femenina de 6 años de edad atendida por proptosis del ojo izquierdo a la que se le realizó el diagnóstico clínico-imagenológico de linfangioma de la órbita, con el objetivo de mostrar el resultado alcanzado en el manejo de la misma mediante el uso del sildenafilo por vía oral, modalidad terapéutica en estudio a nivel mundial en el tratamiento de estas afecciones. El tratamiento con sildenafilo en el linfangioma orbitario demostró ser eficaz en la mejoría del cuadro clínico y por imágenes. Durante el tratamiento no se reportaron reacciones adversas(AU)

Orbital lymphatic malformations are benign, slow-growing, abortive, nonfunctional vascular malformations that occur mainly in the first decade of life. Therapeutic options in these cases present limited resolution, some treatments are usually aggressive and cause damage to the visual apparatus. We present a case of a 6-year-old female patient treated for proptosis of the left eye. The clinical-imaging diagnosis of lymphangioma of the orbit was made to show the results achieved in its treatment through the use of oral sildenafil, a therapeutic modality under study worldwide in the treatment of these conditions. The treatment with sildenafil in orbital lymphangioma proved to be effective in the improvement of the clinical and imaging picture. No adverse reactions were reported during treatment(AU)

Experiencia inicial de un equipo multidisciplinario para el manejo de anomalías vasculares complejas / Initial experience of a multidisciplinary approach for the management of complex vascular anomalies

Introducción: Las anomalías vasculares (AV) están constituidas por un amplio espectro de alteraciones en los vasos sanguíneos y linfáticos, incluidos los tumores y las malformaciones vasculares. El objetivo del presente estudio fue describir la experiencia inicial de un grupo multidisciplinario para el manejo de las anomalías vasculares complejas en pacientes pediátricos en un hospital de tercer nivel. Metodología: El presente estudio observacional de pacientes pediátricos, presentados y analizados en un grupo de anomalías vasculares en Ecuador, desde noviembre de 2019 hasta mayo de 2022. Se recopilaron los diagnósticos según la clasificación de la Sociedad Internacional para el Estudio de las Anomalías Vasculares, localización de la lesión, trata-miento ofrecido, complicaciones y evolución; se excluyeron los pacientes con hemangioma infantil no complicado y hemangioma congénito. Resultados: Se incluyeron 45 pacientes con anomalías vasculares de los cuales, 53% fueron femenino y 47% masculino. La edad promedio al momento de la presentación fue de 5.9 años, un 78% tenían malformaciones vasculares y 22% tenían tumores vasculares. Las mal-formaciones vasculares de tipo venosa fueron las más frecuentes con un 22%. La malformación más frecuente fue venosa en cabeza/cara (29%) y linfáticas (16%). El 89% de los pacientes presentaron por lo menos un síntoma y el tratamiento más usado fue la escleroterapia en un 36.36%. Conclusión: Es el primer reporte realizado en Ecuador de un equipo multidisciplinario que atiende AV complejas en niños según la bibliografía revisada, afirmando que las AV son un gran reto debido al amplio espectro de patologías que las conforman. El equipo multidisciplinario garantiza el manejo completo en los casos de AV complejo logrando un adecuado diagnóstico, para así ofrecer las mejores opciones terapéuticas para el paciente pediátrico.

Introduction: Vascular Anomalies (VA) are constituted by a wide spectrum of alterations, including tumors and vascular malformations, pediatric patients with vascular anomalies will usually benefit from the contributions made by a multidisciplinary team approach. The aim of this study was to describe the initial experience of a multidisciplinary approach group for the management of complex vascular anomalies in pediatric patients. Methodology: This observational study of pediatric patients (< 18 years), presented and analyzed in a group of vascular anomalies in Ecuador, from November 2019 to May 2022. Diagnoses were compiled according to the classification of the Society International for the Study of Vascular Anomalies, location of the lesion, treatment offered, complications and evolution; patients with uncomplicated infantile hemangioma and congenital hemangioma were excluded. Results: A total of 45 patients with vascular anomalies were included, of which 53% were female and 47% male. The mean age at presentation was 5.93 years, 78% had vascular malformations, and 22% had vascular tumors. Venous vascular malformations were the most frequent with 22% and lymphatic malformations 16%. The most frequent location was head/face affected by venous-type vascular malformations with 29%. At least one symptom was present in 89% of the patients, the most used treat-ment was sclerotherapy in 36.36%. Conclusión: It is the first report made in Ecuador of a multidisciplinary team that treats complex VA in children according to the reviewed bibliography, affirming that VA are a great challenge due to the wide spectrum of pathologies that comprise them. The multidisciplinary team guarantees complete management in complex VA cases, achieving an adequate diagnosis, in order to offer the best therapeutic options for the pediatric patient.

Escleroterapia no tratamento de lesões vasculares orais / Escleroterapia en el tratamiento de las lesiones vasculares orales / Sclerotherapy in the treatment of oral vascular injuries

Objetivo: apresentar um caso de lesão vascular em paciente idoso tratado pela técnica da escleroterapia. Relato do caso: paciente masculino, 67 anos, vítima de acidente vascular cerebral, buscou atendimento odontológico por apresentar raízes residuais. Durante o exame físico foi identificada lesão exofítica, de coloração violácea, base séssil, com aproximadamente dois centímetros, localizada em comissura labial esquerda. Para confirmar a origem da alteração foi realizada manobra semiotécnica (diascopia) que revelou tratar-se de lesão vascular. Por ser o paciente idoso, hipertenso, com histórico de acidente vascular cerebral, foi feita a opção por tratamento conservador, sendo realizada a escleroterapia com oleato de monoetanolamina 5%, numa única sessão. No retorno de sete dias, foi observada regressão parcial da lesão e com 30 dias a região se mostrou completamente cicatrizada, sem vestígios da alteração. O oleato de monoetanolamina provoca uma reação inflamatória estéril, aguda, dose-dependente, no endotélio vascular e nos tecidos extravasculares que resulta em fibrose e obliteração dos vasos sanguíneos, induzindo a regressão das lesões. Conclusão: Com base no caso apresentado e nos registros da literatura é possível afirmar que a escleroterapia é uma alternativa terapêutica minimamente invasiva, eficaz, de baixo custo e com resultado estético favorável no tratamento de lesões vasculares orais... (AU)

Objective: to present a case of vascular injury in an elderly patient treated by the sclerotherapy technique. Case report: male patient, 67 years old, victim of a stroke, sought dental care due to residual dental roots. During the physical examination, an exophytic lesion, violet in color, sessile base, approximately two centimeters, located in the left labial commissure, was identified. Diascopy was performed to confirm the origin of the alteration, which revealed that it was a vascular le sion. As the patient was elderly, hypertensive, with a history of stroke, conservative treatment was chosen, with sclerotherapy with 5% mon oethanolamine oleate in a single session. On return after seven days, partial regression of the lesion was observed and, after 30 days, the region was completely healed, with no traces of the alteration. Mon oethanolamine oleate causes a sterile, acute, dose-dependent inflam matory reaction in the vascular endothelium and extravascular tissues that results in fibrosis and obliteration of blood vessels, inducing re gression of the lesions. Conclusion: Based on the case presented and on the literature records, it is possible to affirm that sclerotherapy is a minimally invasive, effective, low-cost therapeutic alternative with a favorable aesthetic result in the treatment of oral vascular lesions... (AU)

Objetivo: presentar un caso de lesión vascular en un paciente de edad avanzada, tratado mediante la técnica de escleroterapia. Reporte de caso: paciente masculino, 67 años, víctima de un derrame cerebral, buscó atención odontológica por raíces dentarias residuales. Durante el examen físico se identificó una lesión exofítica, de color violeta, de base sésil, de aproximadamente dos centímetros, ubicada en la comisura labial izquierda. Se realizó diascopia para confirmar el origen de la alteración, que reveló que se trataba de una lesión vascular. Como el paciente era anciano, hipertenso, con antecedentes de ictus, se optó por tratamiento conservador, con escleroterapia con oleato de monoetanolamina al 5% en una sola sesión. Al regreso a los siete días se observó una regresión parcial de la lesión y, a los 30 días, la región estaba completamente curada, sin rastros de la alteración. El oleato de monoetanolamina provoca una reacción inflamatoria estéril, aguda y dependiente de la dosis en el endotelio vascular y los tejidos extravasculares que produce fibrosis y obliteración de los vasos sanguíneos, lo que induce la regresión de las lesiones. Conclusión: Con base en el caso presentado y en los registros de la literatura, es posible afirmar que la escleroterapia es una alternativa terapéutica mínimamente invasiva, efectiva, de bajo costo y con resultado estético favorable en el tratamiento de las lesiones vasculares orales... (AU)

Asociación del complejo ring-sling con pig bronchus: a propósito de un caso pediátrico / Ring-sling complex association with pig bronchus: a pediatric case report

El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

Caliber-persistent labial artery: report of 3 cases

Abstract The caliber-persistent labial artery is a vascular anomaly in which a primary arterial branch penetrates into the submucosal tissue without reduction in diameter. Most lesions are benign and do not require treatment, except for complications and/or on patient demands. In this way, noninvasive diagnostic tools are preferred such as high-resolution and color Doppler ultrasonography which allow direct observation of the lesion, assessing its exact location and diameter at every axis, as well as the blood flow velocity. An excisional biopsy of these lesions or even their surgical extirpation could have a fatal outcome with profuse bleeding.

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.

Malformación venosa del útero / Uterine venous malformation

Resumen Las malformaciones venosas son lesiones vasculares benignas infrecuentes que se presentan en el útero. Están conformadas por venas anormales, de diferentes tamaños y proporciones, con configuración espongiforme y disposición al azar. En la literatura, han sido previamente reportados algunos casos, usando el término "hemangioma cavernoso", pero según los cambios recientes en la terminología, aprobados por Sociedad Internacional para el Estudio de las Anormalidades Vasculares (ISSVA), se desaconseja el uso de este término y se sugiere el de "Malformación venosa", si se cumplen los hallazgos histopatológicos al momento de hacer el diagnóstico. Presentamos el caso de una mujer de 44 años, con cuadro de hemorragia vaginal anormal y diagnóstico clínico de miomatosis y mioma abortado por el orificio cervical interno, el estudio histopatológico reveló la presencia de una malformación venosa que comprometía el miometrio y endometrio, con formación subsecuente de un pólipo.

Abstract Venous malformations are benign vascular lesions that rarely appear in the uterus. They are made up of abnormal veins, of different sizes and proportions, with spongiform configuration and random disposition. In the literature, some cases have been previously reported, using the term "cavernous hemangioma", but according to recent changes in terminology, approved by the International Society for the Study of Vascular Abnormalities (ISSVA), the use of this term is discouraged, and the diagnosis of Venous malformation is suggested, if the histopathological findings are met. We present the case of a 44-year-old woman, with abnormal vaginal bleeding and a clinical diagnosis of myomatosis and myoma aborted by the internal cervical orifice, in whom the histopathological study revealed the presence of a venous malformation that compromised the myometrium and endometrium, with subsequent formation of a polyp.

Manifestaciones radiológicas del síndrome del nevus azul. Presentación de caso / Manifestaciones radiológicas del síndrome del nevus azul. Presentación de caso

El síndrome del nevus azul es una entidad infrecuente que consiste en múltiples malformaciones venosas que comprometen la piel y el resto de los órganos. Los pacientes pueden ser asintomáticos o pueden cursar con diversas manifestaciones dependiendo del lugar donde se encuentren estas alteraciones vasculares. Las distintas modalidades diagnósticas permiten identificar claramente las malformaciones vasculares; sin embargo, usualmente son consideradas un hallazgo incidental y rara vez son el motivo de la realización del estudio. A continuación, se describe el caso de un paciente de 65 años de edad con diagnóstico de síndrome del nevus azul, con malformaciones venosas evidentes, en modalidades como tomografía computarizada, ultrasonido y resonancia magnética.

The blue rubber bleb nevus syndrome is a rare vascular disorder entity that consists of multiple venous malformations that involve the skin, gastrointestinal tract and viscera. Patients may be asymptomatic or may present with various manifestations depending on the site of these vascular alterations and organs involved. The different diagnostic modalities allow for clear identification of vascular malformations; however, they are usually considered an incidental finding and are rarely the reason for the study to be performed. We describe the case of a 65-year-old patient with a diagnosis of blue nevus syndrome with evident venous malformations in computed tomography, ultrasound and magnetic resonance imaging.

Malformación arteriovenosa de alto flujo en un adulto joven: reporte de caso / High flow arteriovenous malformation in a young adult: case report / Malformação arteriovenosa de alto fluxo em um adulto jovem: relato de caso

Introducción. Las malformaciones arteriovenosas son lesiones relativamente raras e infrecuentes. Se caracterizan por presentar un aumento anormal en el número de vasos sanguíneos como consecuencia de un defecto en el desarrollo vascular. Constituyen un desafío diagnóstico y terapéutico para el médico tratante. Su incidencia es de alrededor el 1.5% de la población general. Dentro de las opciones terapéuticas se incluye la embolización selectiva, la resección quirúrgica o ambas. El objetivo del presente artículo es reportar un caso de una patología poco frecuente y hacer una revisión literaria del tema para arrojar luz sobre su diagnóstico. Reporte de caso. Se presenta el caso de un paciente adulto joven que consulta por presentar una masa en glúteo derecho de 6 años de evolución. Esta es diagnosticada erróneamente como lipoma, por lo que se lleva al paciente a cirugía sin la realización de imágenes diagnósticas previas. En la cirugía, el paciente presenta choque hipovolémico. Posteriormente, se documenta la masa como malformación arteriovenosa profunda. Discusión. Es poco usual la ubicación de dichas malformaciones en los miembros inferiores, como en el paciente del actual caso. El diagnóstico de estas lesiones puede ser clínico, pero requiere del conocimiento o sospecha de esta entidad, ya que pueden ser lesiones clínicamente no visibles, lo que lleva a que pasen inadvertidas o se diagnostiquen de forma errónea. Conclusión. Aunque se trata de una patología poco frecuente, esta puede generar repercusiones clínicas, físicas, psicológicas y estéticas importantes, por lo que es indispensable realizar adecuados métodos por imágenes que permitan establecer su correcto diagnóstico y manejo. Cómo citar. Rodriguez-Londoño NH. Malformación arteriovenosa de alto flujo en un adulto joven. MedUNAB. 2021;24(1): 72-79. doi: https://doi.org/10.29375/01237047.3785

Introduction. Arteriovenous malformations are relative rare and infrequent injuries. Their main characteristic is an abnormal increase in the number of blood vessels as a result of defective vascular development. They represent a diagnostic and therapeutic challenge for the treating physician. Their incidence in the general population is around 1.5%. Some therapeutic options include selective embolization, surgical resection, or both. The purpose of this article is to report a case of an infrequent pathology and to perform a literature review on the topic to shed light on its diagnosis. Case report. The case involves a young adult patient who inquired about the presence of a mass in the right buttock with six years of evolution. It was erroneously diagnosed as a lipoma, as a result of which the patient was taken to surgery without performing preliminary diagnostic images. During surgery, the patient went into hypovolemic shock. Afterwards, the mass was documented as a profound arteriovenous malformation. Discussion. Such malformations are rarely found in the lower limbs, as in this case. These injuries may be clinically diagnosed, but knowledge or suspicion on the existence of this entity is required, because such injuries might not be clinically visible, which implies that they may go unnoticed or be erroneously diagnosed. Conclusion. Even though it is an infrequent pathology, it may have substantial clinical, physical, psychological and aesthetic implications, which implies that it is indispensable to perform adequate imaging-based procedures to enable its adequate diagnosis and management. Cómo citar. Rodriguez-Londoño NH. Malformación arteriovenosa de alto flujo en un adulto joven. MedUNAB. 2021;24(1): 72-79. doi: https://doi.org/10.29375/01237047.3785

Introdução. As malformações arteriovenosas são lesões relativamente raras e infrequentes. São caracterizadas por apresentarem um aumento anormal do número de vasos sanguíneos como consequência de um defeito no desenvolvimento vascular. Constituem um desafio diagnóstico e terapêutico para o médico que trata. Sua incidência gira em torno de 1.5% da população geral. As opções de tratamento incluem embolização seletiva, ressecção cirúrgica ou ambas. O objetivo deste artigo é relatar um caso de patologia pouco frequente e fazer uma revisão bibliográfica sobre o assunto para lançar luz sobre seu diagnóstico. Relato de caso. Apresentamos o caso de um paciente adulto jovem que consultou por apresentar uma massa na nádega direita, de 6 anos de evolução. Isso é diagnosticado erroneamente como um lipoma, então o paciente é levado para cirurgia sem imagens diagnósticas prévias. Na cirurgia, o paciente apresenta um choque hipovolêmico. Posteriormente, a massa é documentada como uma malformação arteriovenosa profunda. Discussão. A localização dessas malformações nos membros inferiores é incomum, como no caso deste paciente. O diagnóstico dessas lesões pode ser clínico, mas requer conhecimento ou suspeita dessa entidade, pois podem ser lesões clinicamente invisíveis, o que as leva a passar despercebidas ou mal diagnosticadas. Conclusão. Embora seja uma patologia pouco frequente, pode gerar importantes repercussões clínicas, físicas, psicológicas e estéticas, pelo que é imprescindível a realização de métodos de imagem adequados para estabelecer seu correto diagnóstico e tratamento. Cómo citar. Rodriguez-Londoño NH. Malformación arteriovenosa de alto flujo en un adulto joven. MedUNAB. 2021;24(1): 72-79. doi: https://doi.org/10.29375/01237047.3785

Rare case of concomitant juxtarenal aortic aneurysm and type 1 Inferior Vena Cava duplication: -intraoperative challenges to avoid catastrophe / Caso raro de aneurisma de aorta justarrenal e duplicação do tipo 1 concomitantes na veia cava inferior - desafios intraoperatórios para evitar catástrofes

Abstract Duplication of the inferior vena cava is a rare congenital anomaly, with an incidence of 0.2-3%. Despite being asymptomatic, anomalies of IVC are important in aortoiliac and retroperitoneal surgeries. Preoperative CT imaging is essential to identify any IVC anomaly and to prevent unexpected hemorrhage during surgery. Here, we report a case of a juxtarenal abdominal aortic aneurysm in which we encountered a type I IVC duplication anomaly intraoperatively while performing transperitoneal aneurysmorrhaphy and took precautions to avoid any iatrogenic injuries to either of the two trunks or the pre-aortic trunk of the anomalous duplicate IVC.

Resumo A duplicação da veia cava inferior (VCI) é uma anomalia congênita rara com incidência de 0,2 a 3%. Apesar de assintomáticas, anomalias da VCI são importantes em cirurgias aortoilíacas e retroperitoneais. A imagem da tomografia pré-operatória é essencial para identificar qualquer anomalia de VCI e para evitar hemorragia inesperada durante a cirurgia. Relatamos um caso de aneurisma de aorta abdominal justarrenal, no qual encontramos uma anomalia de duplicação de VCI do tipo 1 intraoperatório enquanto realizávamos correção cirúrgica de aneurisma transperitoneal. Por isso, tomamos a precaução para evitar qualquer lesão iatrogênica nos dois troncos e no tronco pré-aórtico de VCI duplicada anômala.

Agenesia de segmento infra-hepático de veia cava inferior associada a trombose venosa profunda de repetição: relato de caso / Agenesis of the infra-hepatic segment of the inferior vena cava associated with recurrent deep venous thrombosis: case report

Resumo A agenesia de veia cava inferior é descrita em menos de 1% da população, de ocorrência rara e devido a alterações embrionárias. Sua correlação com a trombose venosa profunda certamente é subestimada, visto que tal alteração é de difícil detecção pela ultrassonografia. O objetivo deste artigo foi relatar o caso de uma paciente de 41 anos com dor e edema até a raiz de coxa direita após cirurgia plástica. Foi realizado dúplex venoso de membros inferiores e evidenciada trombose venosa profunda ilíaco-femoro-poplíteo e distal bilateralmente. Solicitado angiotomografia venosa devido a não visualização de veia cava inferior no ultrassom, evidenciando trombose de plexo lombar direito e segmento ilíaco-femoral bilateral, além de agenesia de segmento infra-hepático de veia cava inferior, com ectasia e tortuosidade compensatória de veias paravertebrais, sistema ázigos e hemiázigos, com varizes pélvicas bilateralmente. Foi realizada anticoagulação sistêmica e oral, com boa evolução clínica.

Abstract Agenesis of the inferior vena cava (IVC) has been described in less than 1% of the population; a rare occurrence caused by embryonic abnormalities. Its correlation with deep vein thrombosis (DVT) is certainly underestimated, since this change is hard to detect using ultrasound. The aim of the article is to report the case of a 41-year-old female patient with pain and edema up to the top of the right thigh after plastic surgery. Bilateral venous duplex ultrasound revealed bilateral DVT involving iliac-femoral-popliteal and distal segments. Venous angiotomography was requested because the IVC was not visible on ultrasound, revealing thrombosis of the right lumbar plexus and iliofemoral segment bilaterally and agenesis of the infrahepatic segment of the inferior vena cava, with ectasia and compensatory tortuosity of paravertebral veins and the azygos-hemiazygos system, and bilateral pelvic varices. Systemic and oral anticoagulation were administered, with a satisfactory clinical response.

Artéria trigeminal e aneurisma segmentar da artéria carótida associadas a hemorragia subaracnóide / Trigeminal artery and segmental aneurysm of the carotid artery associated with subarachnoid hemorrhage

A persistência e a falha da involução das comunicações embrionárias entre a circulação cerebral anterior e posterior contribuem para anomalias vasculares em adultos. Esta variação é comumente detectada de forma incidental, causando sintomas muito raramente. Sua proximidade com os nervos oculomotor, troclear e abducente podem levar a paresia muscular ocular. Em alguns casos pode causar tinnitus pulsátil e ao espasmo de hemiface. Muitos casos de artéria trigeminal foram relatados como associados a aneurismas intracranianos.

The persistence and failure of the involution of embryonic communications between the anterior and posterior cerebral circulation contribute to vascular anomalies in adults. This variation is commonly detected incidentally, rarely causing symptoms. Its proximity to the oculomotor, trochlear, and abducent nerves can lead to ocular muscle paresis. Sometimes it can cause pulsatile tinnitus and hemiface spasm. Many cases of trigeminal artery have been reported to be associated with intracranial aneurysms.