Concordance between referral and final diagnoses of pediatric patients with vascular malformations / Concordancia entre el diagnostico de referencia y final de pacientes pediátricos con malformaciones vasculares

Abstract Background: Vascular malformations (VaM) are a heterogeneous group of disorders resulting from the dysmorphogenesis of blood vessels. Although correct classification is relevant to providing adequate treatment according to evidence-based medicine, diagnostic terminology may be misused or need clarification. Methods: We conducted a retrospective study to measure agreement and concordance between referral and final confirmed diagnoses of 435 pediatric patients with VaM newly referred to the multidisciplinary Vascular Anomalies Clinic (VAC) using Fleiss kappa (κ) concordance analysis. Results: We found fair concordance between referral and confirmed diagnoses of VaM (κ 0.306, p < 0.001). Lymphatic malformations (LM) and VaM associated with other anomalies showed moderate diagnostic concordance (κ 0.593, p < 0.001 and κ 0.469, p < 0.001, respectively). Conclusions: Continuing medical education strategies are required to improve physician knowledge and diagnostic accuracy in patients with VaM.

Resumen Introducción: Las malformaciones vasculares (MVa) son un grupo heterogéneo de trastornos resultantes de la dismorfogénesis de los vasos sanguíneos. A pesar de que la correcta clasificación es relevante para brindar un adecuado tratamiento de acuerdo con la medicina basada en la evidencia, la terminología diagnóstica podría resultar confusa o utilizarse de manera inapropiada. Métodos: En este estudio retrospectivo se midieron el acuerdo y la concordancia entre los diagnósticos de referencia (o derivación) y los diagnósticos finales confirmados de 435 pacientes pediátricos con MVa recién remitidos a la Clínica de anomalías vasculares (CAV) multidisciplinaria, mediante el análisis de concordancia kappa de Fleiss (κ). Resultados: Se encontró una buena concordancia entre los diagnósticos de referencia (o derivación) y los diagnósticos confirmados de MVa (κ 0.306, p < 0.001). Las malformaciones linfáticas (LM) y las MVa asociadas con otras anomalías presentaron concordancias diagnósticas moderadas (κ 0.593, p < 0.001 y κ 0.469, p < 0.001, respectivamente). Conclusiones: Se requiere de estrategias de educación médica continua para mejorar el conocimiento de los médicos y la precisión diagnóstica de los pacientes con MVa.

Progress in the Treatment of Topical Rapamycin Preparations / 罕见病研究

Rapamycin is a mammalian target of rapamycin(mTOR) receptor inhibitor. Advances in the understanding of the mTOR signaling pathway and its downstream effects on tumorigenesis and vascular proliferation have broadened the clinical applications of mTOR receptor inhibitors in treating many challenging diseases. Rapamycin is used orally for the treatment of kidney transplantation, lymphatic leiomyomatosis of lung, tuberous sclerosis complex(TSC), and etc. But systemic therapy using the rapamycin has significant side effects. To mitigate the side effects of systemic rapamycin for dermatologic applications, clinicians have used topical therapy. In recent years, research publications on the topical rapamycin in the treatment of a variety of diseases have increased, as on such diseases of facial angiofibroma of tuberous sclerosis complex, lymphatic malformation, Kaposi hemangioendothelioma, tufted angiomas, and etc. Topical rapamycin can be used as an effective long-term therapy while avoiding systemic side effects, providing a new treatment method for dermatologists. This paper discusses the progress in the treatment of topical rapamycin preparations.

Malformación de la vena de Galeno en un lactante menor / Malformation of the Galeno vein in a minor infant

El aneurisma de la vena de Galeno es una afectación poco frecuente que genera una elevada morbilidad y mortalidad en neonatos y lactantes. Se presenta como una fístula arteriovenosa con comunicación directa entre las arterias piales y los canales venosos con drenaje a la vena de Galeno. Su clínica es variable de acuerdo con la edad de presentación, puede manifestarse con o sin fallo cardiaco, así como otros hallazgos neurológicos y otras malformaciones vasculares cerebrales. El diagnóstico se puede realizar en etapa prenatal, a través de ecografía y del estudio Doppler, mientras que el estándar de oro para el diagnóstico posnatal es la angiografía. El tratamiento endovascular como en el caso que se presenta es el manejo adecuado a través de una embolización. El objetivo de esta investigación fue dar a conocer los elementos clínicos que permitieron el diagnóstico de esta rara malformación en un niño de 4 meses de edad(AU)

The aneurysm of the vein of Galen is a rare condition that generates high morbidity and mortality in neonates and infants. It presents as an arteriovenous fistula with direct communication between the pial arteries and venous channels with drainage to the vein of Galen. Its clinic varies according to the age of presentation, it can manifest with or without heart failure, as well as other neurological findings and other cerebral vascular malformations. The diagnosis can be made in the prenatal stage, through ultrasound and the Doppler study, while the gold standard for postnatal diagnosis is angiography. Endovascular treatment as in the case presented below is the proper management through embolization. The objective of this research was to present the clinical elements that allowed the diagnosis of this rare malformation in a 4-month-old child(AU)

Unusual Number of Phleboliths in a Lip Vascular Malformation – A Case Report

Rationale: Vascular malformation (VM) associated with jaws may cause jaw size discrepancy. Multiple phleboliths in VM are relatively rare. This case report aims to present one such case. Patient Concerns: A 33?year?old female patient sought surgical correction of her abnormally sized jaw and on examination, she was identified with VM. Diagnosis: Subsequent imaging tests revealed the presence of several phleboliths. Treatment: The patient was treated for an abnormal?sized mandible as well as partial removal of the superficial part of VM. Outcomes: The patient had satisfactory esthetics and there was less bleeding than anticipated. Take?away Lessons: VMs could cause jaw size discrepancy and the extent of the malformation could cause blood flow abnormalities leading to multiple phlebolith formation. Proper surgical planning and education of the patient are essential for successful treatment.

Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

Abstract Double-lumen aortic arch is a rare congenital anomaly related to persistence of the fifth aortic arch. It may be found alone or in association with other anatomical changes of the heart. We report a case of double-lumen aortic arch associated with coarctation of the aorta and patent ductus arteriosus in a child with a congenital malformation known as the VACTERL association (vertebral defects, imperforate anus, cardiopathy, tracheoesophageal fistula, renal abnormalities and limb anomalies).

Hemorragia intestinal por malformación vascular de difícil diagnóstico en un caso pediátrico / Intestinal bleeding due to venous vascular malformation of difficult diagnosis in a pediatric case

RESUMEN Fundamento: la hemorragia gastrointestinal aguda es una de las principales emergencias médicas en todo el mundo y responsable de un elevado número de ingresos hospitalarios anuales. Las malformaciones vasculares intestinales, en especial las del colon derecho, son causa frecuente de sangrado digestivo en adultos mayores, pero son raras en pediatría. Objetivo: mostrar la malformación venosa intestinal como una causa rara de hemorragia digestiva recurrente en el niño. Presentación del caso: escolar de seis años de edad, masculino, raza blanca, que proviene de un área rural de difícil acceso en otra provincia, con antecedentes de sangramiento digestivo a forma de melena y hematoquecia desde el primer año de edad. Es remitido a los cinco años a los servicios de Gastroenterología de esta provincia con varios estudios endoscópicos que no revelaron la causa. Es ingresado en el hospital pediátrico para diagnóstico y tratamiento. Conclusiones: ante la presencia de hemorragia digestiva aguda en niños debe considerarse la malformación vascular intestinal a pesar de lo infrecuente en edades pediátricas. Su identificación y tratamiento constituyen un desafío para el equipo médico de asistencia.

ABSTRACT Background: acute gastrointestinal hemorrhage is a major medical emergency worldwide and responsible for a high number of annual hospital admissions. Intestinal vascular malformations, especially those of the right colon, are frequent causes of gastrointestinal bleeding in older adults, but are rare in pediatrics. Objective: to show intestinal venous malformation as a rare cause of recurrent digestive hemorrhage in the child. Case report: a six-year-old male, white-race school boy from a rural area of difficult access in another province, with a history of gastrointestinal bleeding in the form of melaena and hematoquecia since the first year of age. It is referred at the age of five years to the Gastroenterology services of this province with several endoscopic studies that did not reveal the cause. It is admitted to the pediatric hospital for diagnosis and treatment. Conclusions: in the presence of acute digestive bleeding in children, intestinal vascular malformation should be considered, despite its infrequency in pediatric ages. Its identification and treatment are a challenge for the medical assistance team.

Comparison of clinical effects of the Yu flap and the Karapandzic flap in repairing greater than 2/3 lower lip defects / 口腔疾病防治

Objective@#To compare the clinical effect of the Yu flap and the Karapandzic flap in repairing greater than 2/3 defects of the lower lip and to provide a reference for clinical application.@*Methods@#Ten patients with greater than 2/3 lower lip defects after surgical resection of lower lip tumors and vascular malformations were enrolled: 5 patients were repaired with the Yu flap (Yu flap group) and 5 patients were repaired with the Karapandzic flap (Karapandzic flap group). Follow-up for at least 1 year was conducted to evaluate the morphology (symmetry, stoma, exposure of vermilion) and function (sensory function, motor function) of the reconstructed lower lip.@*Results @#All the flaps survived, and all wounds showed primary healing. The lower lips reconstructed with the Yu flap or the Karapandzic flap obtained similar satisfactory oral function. The sensory function was essentially restored. There were no obvious obstacles in speech and expression, and no saliva leakage occurred. In the Yu flap group, only 1 patient had slight microstomia. In the Karapandzic flap group, 2 patients had slight microstomia and 3 patients had moderate microstomia. 90% (9/10) of the patients were very satisfied with the postoperative outcome, and 1 patient in the Karapandzic flap group was basically satisfied. @*Conclusion@#Both the Yu flap and the Karapandzic flap can be used to repair greater than 2/3 lower lip defects and reliable outcomes can be achieved. These two methods can achieve similar oral functions, but the effect of the Karapandzic flap is inferior to that of the Yu flap in terms of aesthetic appearance, and microstomia often occurs, while the Yu flap can generally maintain the original size of the mouth cleft.

Hemorragia digestiva como manifestación de neurofibromatosis tipo I: reporte de un caso / Gastrointestinal hemorrhage due to neurofibromatosis type 1: a case report

RESUMEN Presentamos el caso de un paciente de 40 años con diagnóstico previo de neurofibromatosis y un antecedente crónico de episodios de rectorragias intermitentes que acude al servicio de emergencia por una rectorragia asociada a hipotensión y taquicardia, y es hospitalizado. En los exámenes complementarios se evidencian hemorroides internas de grado 1 que no se correlacionaban con las características clínicas del paciente. Debido a la naturaleza intermitente del cuadro hemorrágico se sospechó de alguna malformación vascular, la cual se confirmó posteriormente mediante una angiografía abdomino-pélvica.

ABSTRACT We present the case of a 40-year-old male with a previous diagnosis of neurofibromatosis and a chronic history of intermittent rectal bleeding episodes. He visited the emergency service for frequent rectal bleeding associated with hypotension and tachycardia, and was hospitalized. Complementary tests evidenced grade 1 internal hemorrhoids not associated with the clinical features of the patient. Due to the intermittent nature of the bleeding, a vascular malformation was suspected, which was subsequently confirmed by a pelvic and abdominal angiography.

Anomalías Vasculares de la Cavidad Oral: Revisión de la Clasificación y Tratamiento Aplicado a dos Casos Clínicos / Vascular Anomalies of the Oral Cavity: Review of Classification and Treatment Applied to Two Clinical Cases

RESUMEN: Las anomalías vasculares de cabeza y cuello son un grupo de lesiones que afectan vasos sanguíneos y linfáticos donde el tratamiento sigue siendo un desafío. La clasificación actualizada de anomalías vasculares de cabeza y cuello es la clasificación de Mulliken modificada, que las subdivide en a) tumores vasculares y, b) malformaciones vasculares. En este reporte, presentamos dos casos clínicos de pacientes de sexo masculino, con diagnóstico de anomalías vasculares que afectan al labio y paladar duro, diagnosticados como malformación arteriovenosa y malformación venosa, respectivamente. Dichas lesiones remitieron completamente mediante tratamientos conservadores (agentes esclerosantes) y/o quirúrgicos (exéresis quirúrgica completa de la lesión) logrando una remisión completa. Consecutivamente, presentamos una revisión de la literatura enfocado a la clasificación actual, enfoques terapéuticos actuales y futuros.

ABSTRACT: Vascular anomalies of the head and neck are a group of lesions that affect blood and lymph vessels where treatment remains a challenge. The updated classification of head and neck vascular anomalies is the modified Mulliken classification, which subdivides them into a) vascular tumors and b) vascular malformations. In this report, we present two clinical cases of male patients, with diagnosis of vascular anomalies affecting the lip and hard palate, diagnosed as arteriovenous malformation and venous malformation, respectively. These lesions were completely treated with conservative (sclerosing agents) and/or surgical (complete surgical exeresis of the lesion) treatments, achieving a complete remission. Consequently, we present a review of the literature focused on the current classification, current and future therapeutic approaches.

Vascular Malformation of Lower LimbA Diagnostic Dilemma

Intramuscular venous malformations are rare presentations and when occur they are most commonly foundin the head and neck and extremities but are relatively rare in the trunk, they are well localized to a singlemuscle or adjacent muscle groups. We present here a case report of patient who presented with venousmalformation confined to the lower extremity which was successfully treated with surgical excision.

Conservative management of knee arthropathy in a patient with Klippel Trenaunay syndrome / Manejo conservador da artropatia do joelho em paciente com síndrome de Klippel-Trenaunay

Abstract Klippel-Trenaunay syndrome (KTS) is a rare vascular malformation characterized by capillary malformation, venous malformations, and soft tissue or bone hypertrophy that affect the extremities in most cases. Knee or hip arthropathy are common associated conditions and cause serious disability. We present the case of a patient with a diagnosis of KTS and severe knee arthropathy. A 34-year-old man with KTS was referred to our hospital with severe knee arthropathy, with the joint fixed in a 90° position. CT Angiography and MRI of the left leg showed important varicose development of the superficial venous system with intraarticular vessels. After discussion of the case by a multidisciplinary committee, the patient was enrolled on a physiotherapy program and had achieved significant improvements in movement and quality of life at 12-month follow-up. Treatment of KTS is primarily conservative and a multidisciplinary approach is necessary.

Resumo A síndrome de Klippel-Trenaunay (SKT) é uma malformação vascular rara caracterizada por malformação capilar, malformações venosas e hipertrofia de tecidos moles ou ósseos que afetam as extremidades na maioria dos casos. A artropatia do joelho ou do quadril é uma condição comumente associada e causa sérias deficiências. Apresentamos o caso de um paciente com diagnóstico de SKT e artropatia grave do joelho. Um homem de 34 anos com SKT foi encaminhado ao nosso hospital com artropatia grave do joelho com articulação fixa na posição de 90 °. A angiotomografia e a ressonância magnética da perna esquerda mostraram importante desenvolvimento varicoso do sistema venoso superficial com vasos intra-articulares. Após o caso ser discutido em um comitê multidisciplinar, o paciente foi incluído em um programa de fisioterapia, obtendo uma melhora significativa nos movimentos e na qualidade de vida após 12 meses de acompanhamento. O tratamento da SKT é principalmente conservador e exige uma abordagem multidisciplinar.

Malformações vasculares pulmonares congênitas: análise de 24 casos / Congenital vascular anomalies of the lungs: analysis of 24 cases

Objetivo: Avaliar a casuística de malformações vasculares do serviço de pneumologia de um hospital público referência estadual para o atendimento pediátrico e analisar os achados clínicos predominantes associados a estas malformações. Métodos: Foi realizado um levantamento de todos os casos de malformações vasculares pulmonares diagnosticadas no serviço, no período de agosto de 1986 a dezembro de 2003. Por meio da análise de prontuários destes pacientes, as seguintes variáveis foram avaliadas: alteração estrutural pulmonar, as manifestações clínicas, a idade média ao diagnóstico, o sexo do paciente e evolução clínica. Resultados: As patologias encontradas foram: sequestro pulmonar - 6 casos (25%), agenesia e/ou hipoplasia pulmonar - 7 casos (30%), fístulas artériovenosas - 4 casos (16%), anel vascular ­ 6 casos (25%) e agenesia da valva pulmonar - 1 caso (4%). Nove pacientes pertenciam ao sexo masculino e 13 ao sexo feminino. A idade ao diagnóstico variou de 1 mês a 58 anos A sintomatologia variou de acordo com a patologia: infecção respiratória de repetição, estridor, cianose e dispneia foram os achados mais frequentes. Dos 22 pacientes estudados, 11 (50%) casos foram passíveis de correção cirúrgica. Conclusão: As malformações vasculares pulmonares são patologias raras, porém o pediatra deve tê-las em mente para se obter um diagnóstico precoce, propedêutica correta e se possível correção cirúrgica dessas patologias. (AU)

Objective: To evaluate the casuistry of vascular malformations in our division and to analyze the predominant clinical findings of these malformations. Methods: A survey was carried out searching for all cases of pulmonary vascular malformations diagnosed, from August 1986 to December 2003. Through the analysis of the medical records of these patients, the following variables were evaluated: structural pulmonary alteration, clinical presentation, mean age at diagnosis, gender and clinical evolution. Results: The pathologies found were: pulmonary sequestration - 6 cases (25%), agenesis and / or pulmonary hypoplasia - 7 cases (30%), arteriovenous fistulas - 4 cases (16%), vascular ring - 6 cases (25%) and pulmonary valve agenesis - 1 case (4%). Nine patients were male and 13 were female. Age at diagnosis ranged from 1 month to 58 years. The symptoms varied according to the pathology. The most frequent were recurrent respiratory infection, stridor, cyanosis, dyspnea. Of the 22 patients studied, 11 (50%) cases were amenable to surgical correction Conclusion: Pulmonary vascular malformations are rare pathologies, but pediatricians should keep them in mind to obtain an early diagnosis, correct propaedeutics and, if possible, surgical correction of these pathologies. (AU)

Facomatosis tipo Cesioflammea: Reporte de caso de neonato con Manchas Mongólicas y Vino oporto. Una presentación simple de una condición dermatológica compleja.

La facomatosis pigmentovascular es un conjunto de signos y síntomas caracterizado por la coexistencia de malformaciones vasculares capilares con una lesión pigmentaria cutánea congénita (nevo epidérmico, nevo spilus o melanosis dérmica) asociada o no, a manifestaciones sistémicas. Existen varios grupos de facomatosis pigmentovasculares según el tipo de nevo asociado a la lesión capilar vascular. Puede existir solo la afectación cutánea o tener manifestaciones sistémicas, entre ellas, traumatológicas, neurológicas, oftalmológicas, inmunológicas, renales, vasculares, linfáticas, entre otras. Exponemos el caso de un neonato masculino, quien presenta manchas vino oporto facial bilateral, cuello y tórax anterior y posterior, junto a melanocitosis dérmicas en espalda y ambos miembros inferiores, asociado a glaucoma congénito del ojo derecho. Debido a las manifestaciones clínicas cutáneas y sistémicas, se diagnostica facomatosis pigmentovascular, realizándose evaluación multidisciplinaria por radiología, dermatología, oftalmología, genética y neurología, para descartar afectación extracutánea. Los estudios complementarios son de vital importancia para detectar alteraciones extracutáneas, como se presentaron en el paciente.

Phakomatosis pigmentovascularis is set of signs and symptoms characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis) associated or not to systemic malformations. There are different types of phakomatosis pigmentovascularis according to the pigmentary nevus associated with the vascular malformation. There may be only the cutaneous condition or have systemic manifestations such as, trauma, neurological, ophthalmological, inmunological, renal, vascular, lymphatic, among others disorders. We present the case of a male newborn who had a port wine stain located bilaterally on the face, anterior and posterior thorax and neck, together with dermal melanocitosis in the back and lower extremities, associated with congenital glaucoma of the right eye. Due the clinical manifestations, the diagnosis of phakomatosis pigmentovascularis was made and multidisciplinary evaluation is requested by radiology, dermatology, ophthalmology, genetics and neurology to rule out extracutaneous involvement. Complementary studies are essential to detect extracutaneous alterations as presented in the patient.

Pleomorphic hyalinizing angiectatic tumor of the mesorectum: a rare case and review of literature

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of uncertain tissue origin. Although it has been classified as a benign tumor under the WHO classification, it is locally aggressive, and multiple recurrences have been reported. PHAT commonly involves the lower extremities; however, various unusual sites of origin have been reported. We present the case of a 30-year-old female with dysmenorrhea, who presented a presacral mass on imaging. The core biopsy confirmed the diagnosis of PHAT. She underwent laparotomy and excision. Histopathology confirmed the presence of a tumor comprised of aggregates of ectatic vessels with perivascular hyalinization. An immunohistochemical study showed diffuse CD34 positivity, but S100, MDM2, and smooth muscle actin negativity. After surgical procedures, the patient is disease free as at the 12-month follow-up. Only 120 cases have been published in the English literature to date. Our study is only the third case of PHAT arising from the pelvis to be reported. Though considered to be a rare condition, the diagnosis of PHAT should always be considered in the differential diagnosis of well-defined hypervascular soft tissue mass in the pelvis. The typical histopathological findings along with immunohistochemistry should clinch the diagnosis.

Vascular malformations: a hospital based study

Background: Vascular anomalies comprise a widely heterogeneous group of lesions. Diagnosis and management of these lesions present challenges to the surgeons, radiologists and histopathologists. Accurate classification of these lesions results in appropriate therapy. Aim of the study is to study the role of histopathology and histochemical stain in the diagnosis of vascular malformationMethods: The present study is a hospital based observational study on vascular malformations over a period of three years from 2016 to 2018 done in department of Pathology and Plastic Surgery at tertiary care centre, Visakhapatnam.Results: Out of 107 specimens of vascular anomalies received, 72 cases were vascular neoplasms, 35 were vascular malformations. Majority of the vascular malformations were seen in the cervicofacial region (43%) followed by extremities (37.1%). Most common malformation in the present study was arterio venous malformations (60%) followed by venous malformations (22.8%). Verhoeff’s VanGieson stain demonstrated discontinuity of internal elastic lamina in cases of arteriovenous malformation and helped in differentiating the lesions from capillary malformation and vascular tumors.Conclusions: Histopathology, special histochemical stains along with imaging features can be used to reduce diagnostic difficulties and in helping proper management of vascular malformations.

Vascular malformations – Treatment modalities

Background: Vascular malformation is a general term that includes congenital vascular anomalies of veins, lymph vessels, both veins and lymph vessels, or both arteries and veins vascular malformation, is a blood vessel abnormality. These are all present at birth and become apparent at different ages. Most are known to occur during development of the arteries, veins, and/or lymph vessels, but without specific cause. Most are congenital and few appear late in life. Aim: It was using different treatment modalities to reform, reshape, and reorganize to obtain near normal appearance and function. Materials and methods: An observational study done in VIMS - Visakha Institute of Medical Sciences, a superspeciality hospital, 20 cases of vascular malformations are taken from period January 2017 –August 2019. Consent was taken from all the patients, and counseling was done regarding the procedures we do, about surgical/ non-surgical methods, stages required and follow up. Results: Statistical analysis was done, results were tabulated and displayed in pie diagrams. Satisfaction scale was shown by VAS (visual analogue scale). Conclusion: Vascular malformations are mesenchymal lesions may be congenital/ acquired and may present late in life. Sometimes few congenital hemangiomas spontaneously resolve by growth of child, some if large may require steroids for suppression and to prevent recurrences. They are known for recurrences or residual lesions and surgery may be done in stages to obtain optimal results. Nonsurgical methods are done by intralesional sclerosants.

Ocular lymphatic vascular malformation combined intracystic hemorrhage of surgical treatment / 中华整形外科杂志

Objective@#To discuss the radical treatment of lymphatic vascular malformation combined intracystic hemorrhage in Children.@*Methods@#66 cases with lymphatic vascular malformation combined intracystic hemorrhage were retrospectively analyzed. The longest diameter of all the lesions was nearly 4 cm.All the 66 patients received surgical treatment. Arc-shaped incision was made to separate and expose the eyes from the hair trace. The lesions were separated from the frontal and temporal parts to the eyes along the dermal fat layer, and deep separation revealed orbicularis oculi muscle and levator upper eyelid muscle.@*Results@#All the 66 cases were completely resected, and the patients were followed up for 0.5-3.0 years after the surgery. The postoperative appearance was satisfactory in 58 cases. The wound scar was healed in 8 cases, and the patients had satisfactory postoperative appearance after elective scar resection and cosmetic repair. Postoperative complications included long-term exudation of incision lymph in 6 cases. Among them, 4 cases were complicated with incision dehiscence and 2 cases were infected.@*Conclusions@#The method of complete resection of tumor as early as possible can radical cure the ocular lymphangioma with intracapsular hemorrhage, which prevent the disease from deteriorating, and meeting the aesthetic requirements of satisfactory appearance.

Symptomatic Sinus Pericranii with Adult Onset Headache: A Case Report with Pathologic Perspective

Sinus pericranii (SP) is a rare vascular anomaly of the scalp that consists of an abnormal pericranial venous channel connected to adjacent dural venous sinuses. Most SP are asymptomatic and are found in the pediatric age group. We aim to report a case of symptomatic SP in adult and describe the clinical, radiological, and pathohistological findings to help understand and differentiate this lesion from other scalp lesions. A 40-year-old man with a scalp mass was admitted to our hospital complaining of headache. The lesion enlarged when the patient was in a recumbent position or during Valsalva maneuver. The radiologic imaging suggested its diagnosis as an accessory type of SP with bone erosion. Surgical resection and cranioplasty were successfully performed, and the related headache also gradually subsided. At the 3-year follow-up, there was no recurrence on magnetic resonance imaging.

Relationship between Abnormal Hyperintensity on T2-Weighted Images Around Developmental Venous Anomalies and Magnetic Susceptibility of Their Collecting Veins: In-Vivo Quantitative Susceptibility Mapping Study

OBJECTIVE: A developmental venous anomaly (DVA) is a vascular malformation of ambiguous clinical significance. We aimed to quantify the susceptibility of draining veins (χvein) in DVA and determine its significance with respect to oxygen metabolism using quantitative susceptibility mapping (QSM). MATERIALS AND METHODS: Brain magnetic resonance imaging of 27 consecutive patients with incidentally detected DVAs were retrospectively reviewed. Based on the presence of abnormal hyperintensity on T2-weighted images (T2WI) in the brain parenchyma adjacent to DVA, the patients were grouped into edema (E+, n = 9) and non-edema (E−, n = 18) groups. A 3T MR scanner was used to obtain fully flow-compensated gradient echo images for susceptibility-weighted imaging with source images used for QSM processing. The χvein was measured semi-automatically using QSM. The normalized χvein was also estimated. Clinical and MR measurements were compared between the E+ and E− groups using Student's t-test or Mann-Whitney U test. Correlations between the χvein and area of hyperintensity on T2WI and between χvein and diameter of the collecting veins were assessed. The correlation coefficient was also calculated using normalized veins. RESULTS: The DVAs of the E+ group had significantly higher χvein (196.5 ± 27.9 vs. 167.7 ± 33.6, p = 0.036) and larger diameter of the draining veins (p = 0.006), and patients were older (p = 0.006) than those in the E− group. The χvein was also linearly correlated with the hyperintense area on T2WI (r = 0.633, 95% confidence interval 0.333–0.817, p < 0.001). CONCLUSION: DVAs with abnormal hyperintensity on T2WI have higher susceptibility values for draining veins, indicating an increased oxygen extraction fraction that might be associated with venous congestion.