Guía mexicana para el diagnóstico y el tratamiento del hemangioma infantil.

Infantile hemangioma is a benign vascular tumor, the most common in childhood, whose natural evolution is the disappearance of the lesion in the pediatric age and which has effective and safe treatments that limit its growth and favor its disappearance at younger ages. Infantile hemangioma continues to be a reason for attention to complications, due to erroneous diagnoses, lack of knowledge of the condition, late referral or fear of the effects of the medications used for its treatment. Furthermore, its presence is normalized without taking into account that it can cause uncertainty, anxiety, feelings of guilt and, as a consequence, a significant impact on the quality of life, mainly in the parents or caregivers of the child. The need for a clinical practice guideline in our country arises from the high presentation of late-remitted complications in infantile hemangioma even with the availability of adequate treatments, the continuous evolution of medicine and the appearance of new evidence. Throughout the guide you will find recommendations regarding the diagnosis, treatment and follow-up of patients with infantile hemangioma, taking into account the paraclinical tests that can be performed, topical or systemic management options, as well as adjuvant therapies. For the first time, objective tools for patient follow-up are included in a guide for the management of infantile hemangioma, as well as to help the first contact doctor in timely referral.

El hemangioma infantil es un tumor vascular benigno, el más frecuente de la infancia, cuya evolución natural favorece la desaparición de la lesión en la misma edad pediátrica y que cuenta con tratamientos eficaces y seguros que limitan su crecimiento y favorecen su desaparición a edades más tempranas. Continúa siendo motivo de atención de complicaciones, debido a diagnósticos erróneos, desconocimiento del padecimiento, referencia tardía o temor de los efectos de los fármacos utilizados para su tratamiento. Además, se normaliza su presencia sin tomar en cuenta que puede llegar a causar incertidumbre, ansiedad, sentimientos de culpa y, como consecuencia, importante afectación de la calidad de vida, principalmente en los padres o cuidadores del niño. La necesidad de una guía de práctica clínica en nuestro país surge ante la alta presentación de complicaciones del hemangioma infantil referidas de manera tardía aun con la disponibilidad de tratamientos adecuados, la evolución continua de la medicina y la aparición de nueva evidencia. A lo largo de la guía se encontrarán recomendaciones en relación con el diagnóstico, el tratamiento y el seguimiento de los pacientes con hemangioma infantil, tomando en cuenta los paraclínicos que pueden realizarse, las opciones de manejo tópico o sistémico, y las terapias adyuvantes. Por primera vez se incluyen en una guía para el manejo del hemangioma infantil herramientas objetivas para el seguimiento de los pacientes, así como para ayudar al médico de primer contacto en su referencia oportuna.

Bilateral sciatic artery persistence in a patient with infrarenal abdominal aortic aneurysm.

A persistent sciatic artery (PSA) is a rare congenital vascular anomaly, with an estimated prevalence ranging from 0.01% to 0.05%. This condition can cause ischemic events in the lower limbs and sciatic artery aneurysms but can also be asymptomatic. A PSA can complicate the treatment of other coexistent vascular diseases due to the thin caliber of the external iliac and femoral arteries. We report a case of a patient with bilateral PSAs and an infrarenal aortic aneurysm. The aneurysm was successfully treated by the endovascular approach. An ultra-low profile endograft associated with access incisions slightly above the usual position was used to overcome this challenging access.

Frequency of the prominent transverse nasal root vein in children.

BACKGROUND: The presence of a vascular, blue linear discoloration on the nasal root of infants and young children is a frequent incidental feature, rarely reported in the medical literature. It is related to the trajectory of the transverse nasal root vein (TNRV). OBJECTIVE: To study the frequency and clinical characteristics of the vascular discoloration of the nasal root in children. METHODS: A prospective study was performed to address the presence or absence of vascular discoloration of the nasal root in all children under 6 years of age attending a pediatric dermatology clinic from November 2022 to November 2023. Data on age and skin phototype (Fitzpatrick classification I-VI) were also collected. RESULTS: Of 701 patients examined, 345 (49.2%) presented with a vascular discoloration of the nasal root. This was present in 97 of 193 (50.3%), 127 of 261 (48.7%), and 121 of 247 (49.0%) patients for the age groups 0-1, 1-3, and 3-6 years, respectively. The presence of vascular discoloration of the nasal root was more frequent in patients with lighter Fitzpatrick skin phototypes: 49 of 69 (71.0%) phototype II, 157 of 290 (54.1%) phototype III, and 137 of 337 (40.7%) phototype IV. CONCLUSIONS: A vascular discoloration of the nasal root is a frequent skin feature in infants and children, persisting at least until the age of 6. It does not constitute any medical problem aside from cosmetic concern and parents can be reassured of its benign nature. We propose the medical term "prominent TNRV" to describe this condition.

Síndrome Klippel-Trenaunay en el embarazo. Reporte de un caso y revisión de la literatura / Klippel-Trenaunay syndrome in pregnancy. Case report and literature review

Resumen Antecedes: El síndrome de Klippel-Trenaunay (KTS) es un síndrome de malformación vascular que comprende una afectación variable de capilares cutáneos, venas y linfáticos con hipertrofia de tejidos blandos y huesos de la extremidad afectada. Durante el embarazo estas malformaciones se incrementan, con afectación pélvica e intraabdominal. Caso clínico: Paciente de 15 años, primigesta, con síndrome Klippel-Trenaunay diagnosticado al nacimiento, con embarazo a término, referida por iniciar con trabajo de parto para finalización del embarazo en un hospital de tercer nivel. No cuenta con control obstétrico, estudios prenatales ni ultrasonidos obstétricos. Se realiza una ecografía pélvica en donde se descartóla presencia de varices pélvicas y un doppler que evidenció un sistema venoso conservado. Se realiza terminación del embarazo vía abdominal obteniendo un recién nacido vivo masculino, peso de 3045 gramos, APGAR 9 y 9 al minuto y a los 5 minutos. Resultados: El embarazo en pacientes con síndrome de Klippel-Trenaunay tiene alto riesgo de tromboembolismo y complicaciones hemorrágicas. La valoración debe ser por un equipo multidisciplinario capacitado para anticiparse a las potenciales complicaciones. Limitaciones del estudio o implicaciones: La principal limitación es la baja incidencia de esta patología. Se puede concluir que el diagnostico de SKT no es indicación de interrupción del embarazo. El éxito del manejo de estas pacientes requiere la participación de un equipo multidisciplinario. Originalidad o valor: Este caso clínico es de primordial relevancia ya que en la bibliografía internacional están reportados menos de 100 casos de embarazos complicados con este síndrome.

Abstract: Background: Klippel-Trenaunay syndrome (KTS) Klippel- Trenaunay syndrome (KTS) is a vascular malformation síndrome that includes variable involvement of skin capillaries, veins and lymphatics with hypertrophy of soft tissues and bones of the affected limb. During pregnancy, these malformations increase, with pelvic and intra-abdominal involvement. Clinical case: 15-year-old patient, gravida 0, with Klippel- Trenaunay syndrome diagnosed at birth, with full-term pregnancy, referred for initiating labor for resolution of pregnancy in a third level hospital. Without obstetric control, without prenatal studies or obstetric ultrasounds. A pelvic ultrasound was performed, which ruled out the presence of pelvic varices and a Doppler that showed a preserved venous system. The pregnancy was terminated by abdominal route, obtaining a male newborn, weighting 3045 grams, APGAR 9 and 9 after 1 and 5 minutes. Results: Pregnancy in patients with Klippel-Trenaunay síndrome has a high risk of thromboembolism and bleeding complications. They should be evaluated by a trained multidisciplinary team to anticipate possible complications. Study limitations or implications: The main limitation is the low incidence of this pathology. It can be concluded thatthe diagnosis of SKT is not an indication for termination of pregnancy. Successful management of these patients requires the participation of a multidisciplinary team. Originality or value: This clinical case is of primary relevance since fewer than 100 cases of complicated pregnancies with this syndrome are reported in the international literature.

Aberrant right subclavian artery: case report and literature review.

The aberrant right subclavian artery, also known as the arteria lusoria, is the most common aortic arch anomaly, occurring in 0.5 to 1% of the population. There is a higher prevalence in women and it is usually associated with other anatomical variations, such as the non-recurrent laryngeal nerve, present in 86.7% of cases. In the majority of cases, the aberrant right subclavian artery causes no symptoms. We describe this anomaly in an 82-year-old, hypertensive, and asymptomatic patient who had undergone a thoracoabdominal angiography to investigate a chronic DeBakey type III aortic dissection with dilation of the descending aorta. The aberrant right subclavian artery followed a retroesophageal course and was associated with a Kommerell diverticulum. In view of its rarity, we conducted an integrative bibliographic review of literature from the last 6 years indexed on the Medline, UpToDate, Lilacs, Scielo, and Portal Capes databases and discuss the most frequent anatomical changes, symptomatology, and therapeutic management adopted.

Artéria subclávia direita aberrante: relato de caso e revisão de literatura / Aberrant right subclavian artery: case report and literature review

Resumo A artéria subclávia direita aberrante, também conhecida como artéria lusória, é a anomalia do arco aórtico mais comum, ocorrendo entre 0,5 e 1% da população. Possui prevalência em mulheres e normalmente está associada a outras variações anatômicas, como o nervo laríngeo não recorrente, presente em 86,7% dos casos. Em sua maioria, a artéria subclávia direita aberrante não apresenta sintomas. Descrevemos essa alteração em uma paciente de 82 anos, hipertensa e assintomática, que havia sido submetida a uma angiotomografia toracoabdominal para a avaliação de uma dissecção crônica tipo III (DeBakey) associada à dilatação de aorta descendente. No achado, a artéria subclávia direita aberrante apresentava percurso retroesofágico associado a um divertículo de Kommerell. Devido à raridade, realizamos revisão bibliográfica integrativa das bases de dados MEDLINE, UpToDate, LILACS, SciELO e Portal CAPES dos últimos 6 anos e discutimos as alterações anatômicas mais frequentes, a sintomatologia e as condutas terapêuticas adotadas.

Abstract The aberrant right subclavian artery, also known as the arteria lusoria, is the most common aortic arch anomaly, occurring in 0.5 to 1% of the population. There is a higher prevalence in women and it is usually associated with other anatomical variations, such as the non-recurrent laryngeal nerve, present in 86.7% of cases. In the majority of cases, the aberrant right subclavian artery causes no symptoms. We describe this anomaly in an 82-year-old, hypertensive, and asymptomatic patient who had undergone a thoracoabdominal angiography to investigate a chronic DeBakey type III aortic dissection with dilation of the descending aorta. The aberrant right subclavian artery followed a retroesophageal course and was associated with a Kommerell diverticulum. In view of its rarity, we conducted an integrative bibliographic review of literature from the last 6 years indexed on the Medline, UpToDate, Lilacs, Scielo, and Portal Capes databases and discuss the most frequent anatomical changes, symptomatology, and therapeutic management adopted.

Doble cuarto arco aórtico persistente en un perro (Canis familiaris): reporte de caso

El doble arco aórtico persistente es una patología caracterizada por anomalías embrionarias en la vascularización, que pueden afectar de manera indirecta a otros sistemas, como el digestivo y el respiratorio. El objetivo de este documento es reportar un caso de doble arco aórtico, persistente en un cachorro Bull terrier, de seis meses de edad. El paciente ingresó a consulta por motivo de regurgitaciones frecuentes y pérdida ponderal. En el estudio radiográfico, se evidenció dilatación esofágica craneal a la base del corazón y en la toracotomía, se confirmó un doble arco aórtico persistente. Se realizó manejo nutricional y posteriormente, corrección quirúrgica de la anomalía vascular. Este es el primer caso de una anomalía de este tipo en Colombia. Se concluye, que un manejo quirúrgico enfocado a liberar el anillo estenosante y a recuperar la función esofágica, son la base terapéutica de este tipo de alteraciones.

Persistent double aortic arch is a pathology characterized by embryonic vascularization anomalies, which can indirectly affect other systems such as the digestive and respiratory systems. The objective of this document is to report a case of persistent double aortic arch in a six-month-old Bull Terrier puppy. The patient was admitted for consultation due to frequent regurgitation and weight loss. The radiographic study revealed cranial esophageal dilation at the base of the heart, and a thoracotomy confirmed a persistent double aortic arch. Nutritional management was performed and subsequently, surgical correction of the vascular anomaly. This is the first case of an anomaly of this type in Colombia. It is concluded that surgical management focused on releasing the stenosing ring and recovering esophageal function are the therapeutic basis for this type of alteration.

Multimodal anesthesia associated with regional block in a dog who underwent corrective surgery for persistent right aortic arch: case report / Anestesia multimodal associada a bloqueio loco-regional em cão submetido a cirurgia corretiva para persistência de arco aórtico direito: relato de caso

The persistence of the fourth right aortic arch (PRAA) is a congenital malformation that affects the heart base's main vessels. Surgical treatment is recommended and should be advocated as a matter of urgency. In this context, efficient anesthesia planning is necessary, with satisfactory analgesia, associating multimodal techniques with regional blocks. The present work aims to report the anesthetic procedure during corrective surgery for PRAA in a dog. Neuroleptanalgesia was intramuscularly performed, using acepromazine (0.015 mg.kg-1) and methadone (0.3 mg.kg-1) in pre-anesthetic medication. Ketamine (1 mg.kg-1) and propofol (3 mg.kg-1) were administered at induction, both intravenously, followed by maintenance using total intravenous anesthesia with propofol (initial rate of 0.4 mg.kg-1 .minute) and remifentanil, (0.2 mcg.kg.-1.minute). In addition, ultrasound-guided regional intercostal block was performed, with 5% bupivacaine without vasoconstrictor (0.05ml.kg-1). Ketamine infusion was postoperatively maintained for one hour. The instituted protocol proved to be satisfactory in controlling trans and postoperative pain, maintaining all parameters stable during and after the procedure, without any intercurrence. Thus, the protocol provided quality recovery to the patient.(AU)

A persistência do quarto arco aórtico direito é uma má formação congênita, afetando os principais vasos da base cardíaca. O tratamento cirúrgico é recomendado e preconiza-se um planejamento anestésico eficiente, associando-se técnicas multimodais a bloqueios regionais. O presente trabalho objetiva relatar a anestesia durante cirurgia corretiva de PDA em cão. Na medicação pré-anestésica, instituiu-se neuroleptonalgesia, utilizando-se acepromazina (0,015 mg.kg-1) e metadona (0,3 mg.kg-1), por via intramuscular. Na indução, foi administrada cetamina (1mg.kg -1) e propofol (3 mg.kg-1). Para manutenção, utilizou--se propofol (taxa inicial de 0,4 mg.kg-1.minuto) e remifentanil, (0,2 mcg.kg-1.minuto). Além disso, foi realizado bloqueio regional intercostal guiado por ultrassom com bupivacaína sem vasoconstritor a 5% (0,05ml.kg-1). O paciente permaneceu em infusão analgésica de cetamina por uma hora, no pós operatório. O protocolo estabelecido demonstrou ser satisfatório no controle de dor trans e pós-operatória, mantendo todos os parâmetros estáveis, sem nenhuma intercorrência, proporcionando qualidade de recuperação ao paciente.(AU)

Aberrant right subclavian artery in a dog - case report / [Ectopia de artéria subclávia direita em cão - relato de caso]

The brachiocephalic trunk and the left subclavian artery originate from the aortic arch, and both supply blood to the head, neck, and thoracic limbs. Anatomical variations, such as an aberrant right subclavian artery, are congenital conditions rarely observed in dogs, Thus, the objective of the present report was to describe a case of aberrant right subclavian artery in a 9-year-old Dalmatian. However, this anomaly was a finding in which the patient was asymptomatic during its 9 years of life and only at this age did he exhibit signs including sialorrhea, vomiting, hyporexia, and noisy deglutition. Blood count, biochemical profile, and thoracic radiography led to a diagnosis of megaesophagus and aspiration pneumonia. Despite the recommended treatment, the patient did not respond well; as such, the owner elected to euthanize the animal. On necropsy, the right subclavian artery originated directly from the aortic arch, followed a route from left to right dorsally to the esophagus, and then formed an impression of the vascular path over the muscular wall of the esophagus. The esophagus, in turn, exhibited a flaccid wall and dilation in the caudal portion to the vascular path made by the ectopic position of the right subclavian artery.(AU)

O tronco braquiocefálico e a artéria subclávia esquerda emergem do arco aórtico e são responsáveis por fazerem o suprimento sanguíneo para cabeça, pescoço e membros torácicos. Variações anatômicas, como a ectopia da artéria subclávia direita, são alterações congênitas raramente encontradas em cães, cujas alterações do sistema digestivo acontecem em pacientes recém-desmamados e não em adultos. Assim, o objetivo deste relato é descrever um caso de ectopia da artéria subclávia direita em uma cadela, Dálmata, de nove anos de idade. No entanto, essa anomalia foi um achado do qual o paciente foi assintomático durante os nove anos de vida e somente com essa idade apresentou sinais como sialorreia, vômito, hiporexia e deglutição ruidosa. O hemograma e os perfis bioquímicos, associados à radiografia torácica, levaram a um diagnóstico de megaesôfago e pneumonia aspirativa. Mesmo seguindo o tratamento recomendado, houve piora clínica do quadro e o animal foi submetido à eutanásia. À macroscopia, a artéria subclávia direita originava-se direto do arco aórtico, fazia um percurso da esquerda para a direita dorsalmente ao esôfago e, então, formava uma impressão do trajeto vascular sobre a parede muscular do esôfago. O esôfago, por sua vez, apresentava parede flácida e dilatação na porção caudal ao trajeto vascular feito pela posição ectópica da artéria subclávia direita.(AU)

Aberrant right subclavian artery (arteria lusoria) aneurysm with a Kommerell's diverticulum.

The treatment options for aberrant right subclavian artery vary depending on the presence of Kommerell's diverticulum. Because there is a tendency not to report mortalities of these rare cases in the literature, it is hard to reach a conclusion on treatments from the limited data on post-interventional results in these patients. We report our experience with a 67-year old patient with an aberrant right subclavian aneurysm with Kommerell's diverticulum, diagnosed by chance.

As opções de tratamento para artéria subclávia direita aberrante variam dependendo da presença de divertículo de Kommerell. Como há uma tendência a não relatar mortalidade nos raros casos descritos na literatura, é difícil chegar a uma conclusão sobre tratamentos tendo em vista os dados limitados sobre resultados pós-intervenção nesses pacientes. Relatamos aqui nossa experiência com um paciente de 67 anos de idade com aneurisma de artéria subclávia aberrante direita com divertículo de Kommerell diagnosticado ao acaso.

Aberrant right subclavian artery (arteria lusoria) aneurysm with a Kommerell's diverticulum / Aneurisma de artéria subclávia direita aberrante (artéria lusória) com divertículo de Kommerell

The treatment options for aberrant right subclavian artery vary depending on the presence of Kommerell's diverticulum. Because there is a tendency not to report mortalities of these rare cases in the literature, it is hard to reach a conclusion on treatments from the limited data on post-interventional results in these patients. We report our experience with a 67-year old patient with an aberrant right subclavian aneurysm with Kommerell's diverticulum, diagnosed by chance

As opções de tratamento para artéria subclávia direita aberrante variam dependendo da presença de divertículo de Kommerell. Como há uma tendência a não relatar mortalidade nos raros casos descritos na literatura, é difícil chegar a uma conclusão sobre tratamentos tendo em vista os dados limitados sobre resultados pós-intervenção nesses pacientes. Relatamos aqui nossa experiência com um paciente de 67 anos de idade com aneurisma de artéria subclávia aberrante direita com divertículo de Kommerell diagnosticado ao acaso

Persistent sciatic artery found incidentally on hip MRI: report of 4 cases.

The persistent sciatic artery is a rare anatomical variant, representing the persistence of the sciatic artery in adult life that is responsible for the major blood supply to the lower limb in early embryologic development. Such persistence may be bilateral and can remain asymptomatic for many years. However, aneurysmal degeneration has been described as a complication of the persistent sciatic artery, which may cause critical limb ischemia resulting from thrombosis or embolization of aneurysm thrombus. Digital subtraction angiography, Doppler ultrasound, computed tomography angiography and magnetic resonance angiography are the most frequently used diagnostic tools to detect, classify and determine the presence of complications of a PSA. Early detection of this vascular abnormality on imaging studies can avoid life-threatening complications. We describe 4 patients with PSA that were diagnosed as an incidental finding in magnetic resonance imaging of the hip and demonstrate its characteristic imaging appearance.

Carotid artery occlusion in Kabuki syndrome: Case report and literature review.

BACKGROUND: Kabuki syndrome is a rare multiple congenital anomaly syndrome whose main diagnostic findings are craniofacial phenotypic changes and mental retardation. Organic structural lesions in the central nervous system are rare, although have been described already. Systemic vascular changes have also been reported rarely. CASE DESCRIPTION: We report the case of a young patient with Kabuki syndrome who had a transient ischemic attack due to dissection of the internal carotid artery and a likely gliosis area on the white matter. CONCLUSION: Association of cervical arterial disease with this syndrome has never been described, and its pathophysiology is not yet established; however, it can direct future research and maybe treatment.