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1.
Internist (Berl) ; 61(2): 140-146, 2020 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-31938816

RESUMO

Benign liver tumors form a heterogeneous group. The most frequent forms include simple cysts, hemangiomas, focal nodular hyperplasia and hepatocellular adenomas. They are often incidentally detected during routine sonography. The diagnosis of a liver tumor not uncommonly causes anxiety and insecurity in those affected, which is why a rapid and reliable diagnostic procedure should be carried out. Because some tumors, particularly hepatocellular adenomas, are of prognostic relevance due to the potential risk of malignant transformation, a correct classification should always be strived for. The type and extent of diagnostic clarification depend on the clinical and patient-related risk factors. This article describes the most important benign space-occupying lesions. The etiology, clinical manifestations and diagnostics as well as possible necessary treatment measures are presented.


Assuntos
Cistos , Hiperplasia Nodular Focal do Fígado , Hemangioma , Hepatopatias , Neoplasias Hepáticas , Cistos/diagnóstico , Cistos/terapia , Diagnóstico Diferencial , Hiperplasia Nodular Focal do Fígado/diagnóstico , Hiperplasia Nodular Focal do Fígado/terapia , Hemangioma/diagnóstico , Hemangioma/terapia , Humanos , Hepatopatias/diagnóstico , Hepatopatias/terapia , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapia
2.
World Neurosurg ; 133: e129-e134, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31476453

RESUMO

OBJECTIVE: Choroidal hemangioma (CH) is a benign vascular tumor that induces subretinal fluid collection or exudative retinal detachment and consequent visual symptoms. Current standard treatments for CH include cryotherapy, diathermy, photocoagulation, photodynamic therapy, transpupillary thermotherapy, and radiation therapy. Stereotactic radiosurgery has recently been applied to the treatment of CH because of its characteristic stiff dose-fall-off and accuracy. We have adopted gamma knife radiosurgery (GKRS) to treat CH and have retrospectively assessed tumor volume reductions and improvements to visual acuity achieved thereby. METHODS: Fourteen patients with CHs were treated with GKRS from November 2006 to December 2017. Eight patients had circumscribed CH, and 6 exhibited diffuse CHs and were diagnosed with Sturge-Weber syndrome. The mean age of patients was 27.1 years (range: 8-68 years) and the mean duration of clinical or radiological follow-up was 40.2 months (range: 5-105 months). The mean volume of the tumors at the time of GKRS was 533.5 mm3 (range: 124-1150 mm3), and the mean prescribed marginal dose was 11.6 Gy (range: 10-16 Gy) with 50% isodose lines. RESULTS: The tumor volume decreased by the last follow-up in all patients. The visual acuity improved in 9 patients (64%) and decreased in 1 (7%). Six patients (43%) required trans-pars plana vitrectomy before or after GKRS. There were no symptomatic complications from radiation injury during the follow-up periods. CONCLUSIONS: GKRS could be an acceptable alternative treatment for symptomatic CH when standard therapy is not feasible.


Assuntos
Neoplasias da Coroide/cirurgia , Hemangioma/cirurgia , Radiocirurgia , Adolescente , Adulto , Idoso , Criança , Neoplasias da Coroide/complicações , Neoplasias da Coroide/patologia , Neoplasias da Coroide/terapia , Terapia Combinada , Feminino , Seguimentos , Hemangioma/complicações , Hemangioma/patologia , Hemangioma/terapia , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Carga Tumoral , Transtornos da Visão/etiologia , Adulto Jovem
3.
Tech Vasc Interv Radiol ; 22(4): 100634, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31864529

RESUMO

Klippel-Trenaunay syndrome or KTS is a complex vascular syndrome associated with overgrowth occurring as a result of somatic mutations in the PIK3CA gene. Patients are diagnosed on the basis of physical findings, sometimes with supportive imaging, of commonly a segmental anomaly with a cutaneous port-wine stain, lymphatic and venous malformations and overgrowth. The severity of the component vascular malformations and the degree of overgrowth varies from patient to patient which demands care given by a multi-professional team with regular follow-up in a specialist clinic. Some patients may present with acute life-threatening problems, often as a result of veno-thromboembolic events (VTEs) especially following surgical and invasive radiological procedures. Awareness of such problems is vital and prophylactic measures to reduce such risks are paramount. The interventional radiologist is vital to the care team as he/she can undertake procedures including endovascular closure of significant venous anomalies which predispose to such VTEs. Although these procedures can be lengthy and complex, they can now provide a minimally invasive means to reduce the risk from life-threatening and sometimes fatal VTEs. The results however from such interventions will require long-term studies which to date are unavailable.


Assuntos
Malformações Arteriovenosas/terapia , Procedimentos Endovasculares , Síndrome de Klippel-Trenaunay-Weber/terapia , Tromboembolia Venosa/prevenção & controle , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/genética , Malformações Arteriovenosas/mortalidade , Classe I de Fosfatidilinositol 3-Quinases/genética , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Predisposição Genética para Doença , Hemangioma/diagnóstico , Hemangioma/genética , Hemangioma/terapia , Humanos , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/genética , Síndrome de Klippel-Trenaunay-Weber/mortalidade , Mutação , Fenótipo , Radiografia Intervencionista , Fatores de Risco , Resultado do Tratamento , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/genética , Tromboembolia Venosa/mortalidade
4.
Ophthalmic Plast Reconstr Surg ; 35(6): e154-e157, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31593044

RESUMO

Congenital hemangiomas comprise a subset of vascular tumors with clinicopathologic features that are distinct from the more common infantile hemangioma. The authors present a patient with a large congenital hemangioma involving the forehead and brow which obstructed the visual axis and created significant risk for deprivational amblyopia. Management of the congenital hemangioma involved customized headgear to clear the visual axis and early vascular embolization of feeder vessels with the subsequent successful surgical resection at 23 days of life.A large amblyogenic congenital hemangioma required a multidisciplinary approach involving early vascular embolization of feeder vessels and subsequent surgical resection at 23 days of life.


Assuntos
Ambliopia/prevenção & controle , Neoplasias Faciais/terapia , Hemangioma/terapia , Neoplasias Cutâneas/terapia , Sobrancelhas , Neoplasias Faciais/congênito , Testa , Hemangioma/congênito , Humanos , Recém-Nascido , Masculino , Neoplasias Cutâneas/congênito
5.
Pediatrics ; 144(4)2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31511312

RESUMO

Hepatic hemangioma (HH) is a common asymptomatic, self-limiting benign vascular tumor of the liver in neonates. Although complicated HHs are rare, they have significant risks of morbidity and mortality, especially during the perinatal period. Because of the high risks of complications from surgical interventions, there is an unmet need for effective medical therapy. We report 2 neonates with life-threatening HH who were evaluated for a liver transplant before being treated successfully with combined medical therapy, which included sirolimus, corticosteroids, and propranolol.


Assuntos
Hemangioma/terapia , Neoplasias Hepáticas/terapia , Metilprednisolona/uso terapêutico , Propranolol/uso terapêutico , Sirolimo/uso terapêutico , Quimioterapia Combinada , Embolização Terapêutica , Feminino , Glucocorticoides/uso terapêutico , Hemangioma/diagnóstico por imagem , Artéria Hepática , Humanos , Recém-Nascido , Neoplasias Hepáticas/diagnóstico por imagem , Masculino , Vasodilatadores/uso terapêutico
6.
Fam Cancer ; 18(4): 445-449, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31240424

RESUMO

A 51-year old presented with a 6-month history of increasing pelvic/lower back pain with nocturnal waking and episodes of anorexia and vomiting. Examination revealed right torticollis and Horner's syndrome, and a large abdominal mass arising from the pelvis. Magnetic resonance and positron emission tomography imaging revealed (A) a 14 cm heterogeneous enhancing mass, abutting the left kidney with standardised uptake value max = 2.9, (B) a large heterogeneous enhancing pelvic mass (C) mesenteric adenopathy standardised uptake value max = 10.3 and (D) 6 cm right lung apex mass standardised uptake value max = 4.3. Computerised tomography-guided biopsy of lesion A was reported as neurofibroma with occasional atypia, lesion B a benign uterine leiomyoma and lesion C follicular lymphoma world health organisation Grade 2. Although she had been given the diagnosis of Neurofibromatosis Type-1 (NF1) 25-years previously following removal of an intradural extramedullary schwannoma she had no cutaneous stigmata of NF1. Genetic analysis of blood lymphocyte DNA identified a pathogenic variant in SMARCB1 confirming a diagnosis of schwannomatosis. Following 6-months chemotherapy for lymphoma, surgery was performed to remove lesion A. Histology revealed a malignant peripheral nerve sheath tumour with areas of low and high-grade change. An incidental, well-differentiated small bowel neuroendocrine carcinoma was also excised. Close surveillance continues with no recurrence after 6 years. This case study describes a novel finding of three separate synchronous primary malignancies in a patient with schwannomatosis and a proven SMARCB1 pathogenic variant.


Assuntos
Hemangioma/genética , Neoplasias Primárias Múltiplas/genética , Neurilemoma/genética , Neurofibromatoses/genética , Neurofibrossarcoma/genética , Proteína SMARCB1/genética , Neoplasias Cutâneas/genética , Feminino , Hemangioma/terapia , Síndrome de Horner/diagnóstico por imagem , Humanos , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/complicações , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/terapia , Neurilemoma/complicações , Neurilemoma/patologia , Neurilemoma/terapia , Neurofibromatoses/complicações , Neurofibromatoses/terapia , Neurofibrossarcoma/patologia , Neurofibrossarcoma/terapia , Neoplasias Retroperitoneais/genética , Neoplasias Retroperitoneais/terapia , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/terapia
7.
BMC Cancer ; 19(1): 550, 2019 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-31174503

RESUMO

BACKGROUND: To evaluate the value of a three dimension (3D)visualization operative planning system in ultrasound-guided percutaneous microwave ablation (US-PMWA) for large hepatic hemangiomas (LHHs). METHODS: Fifty-eight patients with LHHs were divided into 3D and 2D groups. The therapeutic efficacy was assessed by contrast-enhanced imaging during follow-up. Hepatic and renal function were examined. The complete ablation, tumor volume shrinkage, and complication rates were analyzed. RESULTS: The ablation time and energy of the 3D group were lower than those of the 2D group (1152.0 ± 403.9 s vs. 1379.7 ± 375.8 s and 87,407.2.9 ± 50,387.0 J vs. 117,775.8 ± 46,245.6 J, P = 0.031 and 0.021, respectively). The 3D group had a higher complete ablation rate than the 2D group (97.7 ± 2.4% vs. 94.5 ± 3.7%, P < 0.001). The incidence of hemoglobinuria after ablation in the 3D group was lower than that in the 2D group (32.0% vs. 57.6%, P = 0.047). The levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), and creatinine (Cre) after ablation in the 3D group were lower than those in the 2D group (126.7 ± 56.4 U/L vs. 210.9 ± 96.2 U/L, P < 0.001; 141.0 ± 60.8 U/L vs. 211.4 ± 90.0 U/L, P = 0.001; 57.3 ± 17.6 U/L vs. 80.8 ± 41.9 U/L, P = 0.010; and 66.6 ± 16.6 mmol/L vs. 84.5 ± 39.6 mmol/L, P = 0.037, respectively). There were no significant differences in antenna insertion and the volume reduction rate between the groups. One patient developed acute kidney injury shortly after ablation in the 2D group and recovered after hemodialysis. No other severe complications occurred during the follow-up period. CONCLUSIONS: The 3D visualization operative planning system has a relatively high clinical application value in providing scientific, reasonable, quantifiable, and individualized therapy for LHHs by US-PMWA.


Assuntos
Técnicas de Ablação , Hemangioma/diagnóstico , Hemangioma/terapia , Imagem Tridimensional , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapia , Cirurgia Assistida por Computador , Ultrassonografia , Adulto , Feminino , Humanos , Testes de Função Renal , Testes de Função Hepática , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Cirurgia Assistida por Computador/métodos , Resultado do Tratamento , Ultrassonografia/métodos
8.
World Neurosurg ; 128: e283-e288, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31028979

RESUMO

OBJECTIVE: Vertebral hemangiomas (VHs) are the most common incidental lesions of vertebral body, but they are very challenging to treat if they become symptomatic. Several treatments have been proposed but none was superior to others. The aim of this study is to analyze blood loss and long-term clinical and neurological results of aggressive VHs treated with arterial embolization the day before operation, followed by vertebroplasty, posterior decompression, and short segment stabilization. METHODS: Ten patients (4 males and 6 females) were treated for aggressive VHs with polyvinyl alcohol microparticles embolization, posterior short segment stabilization, and poly methyl methacrylate. Clinical and neurological outcomes were assessed with visual analog, Nurick, and American Spinal Injury Association (ASIA) scales. RESULTS: At last follow-up mean, visual analog scale was 1.8 ± 1.3, with a significant difference with preoperative values (P = 0.00018). Neurological deficits persisted in 4 patients (ASIA scale: C in 1 patient [10%], D in 3 patients [30%]), but they improved from baseline in all cases. Also, Nurick scale rating improved in all patients (0 in 3 patients [30%], 1 in 4 patients [40%], 2 in 2 patients [20%], and 3 in the last one [10%]). A statistically significant difference between pre- and postoperative values was observed for both scores (ASIA, P = 0.0102; Nurick, P = 0.026). Relapse of pathology was recorded in 2 patients. CONCLUSIONS: Polyvinyl alcohol microparticles embolization, short segment fixation, and vertebroplasty is an effective treatment option for aggressive VHs, with a fast surgical time, poor blood loss, and improvement of preoperative clinical and neurological outcomes.


Assuntos
Cimentos para Ossos/uso terapêutico , Embolização Terapêutica/métodos , Hemangioma/terapia , Polimetil Metacrilato/uso terapêutico , Neoplasias da Coluna Vertebral/terapia , Vertebroplastia/métodos , Adulto , Idoso , Perda Sanguínea Cirúrgica , Descompressão Cirúrgica/métodos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Parafusos Pediculares , Álcool de Polivinil/uso terapêutico , Adulto Jovem
9.
Presse Med ; 48(4): 388-397, 2019 Apr.
Artigo em Francês | MEDLINE | ID: mdl-31006564

RESUMO

Beta-blockers are efficient for treating complicated infantile hemangiomas; propranolol is currently the first-line treatment. Superficial vascular malformations have to be managed by multidisciplinary teams. T2 FAT-SAT MRI is the most interesting sequence to explore superficial vascular malformations. Recent advances in molecular biology allow exploring new genetic mutations which could be involved in vascular malformations and be the target of new drugs. Mammalian target of rapamycin (mTOR) inhibitors are promising drugs for slow-flow malformations. Arteriovenous malformations are aggressive lesions with very few treatment options.


Assuntos
Hemangioma/terapia , Dermatopatias Vasculares/terapia , Neoplasias Cutâneas/terapia , Malformações Vasculares/terapia , Feminino , Humanos , Masculino
10.
Dermatol Ther ; 32(3): e12919, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30977938

RESUMO

Maffucci syndrome is a rare genetic disease due to somatic mutation of IDH1 gene. Currently there is no medical treatment available for spindle cell hemangioma associated with this disorder. Here we report successful management of these hemangiomas using sirolimus in combination with surgery.


Assuntos
Encondromatose/complicações , Hemangioma/terapia , Adulto , Feminino , Humanos , Sirolimo/uso terapêutico
11.
Pediatr Clin North Am ; 66(2): 437-459, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30819347

RESUMO

Infantile hemangiomas (IH) are a common benign tumor of infancy, most being uncomplicated and not requiring therapy. Some IH may require treatment; the pediatric provider must be familiar with morphology, distribution, natural history, and associations of IH. Several treatment options are available for IH: current standard of care, oral propranolol. Other therapies include wound care; topical beta-blocker therapy for small, superficial, and uncomplicated IH; treatment of IH residua. In addition to functional compromise and other complications, potential for permanent deformity and eventual psychosocial stigmatization are important when considering the need for treatment of IH in a neonate or infant.


Assuntos
Hemangioma/diagnóstico , Antagonistas Adrenérgicos beta/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Hemangioma/complicações , Hemangioma/terapia , Humanos , Lactente , Recém-Nascido , Terapia a Laser/métodos , Masculino , Fatores de Risco
13.
J Dermatol ; 46(5): 371-375, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30888067

RESUMO

Although infantile hemangiomas (IH) spontaneously involute, some leave more volumetric sequelae than others. Identifying predictors of such sequelae would provide a management reference for IH lesions and help achieve favorable outcomes. Our objective is to explore the correlation between volumetric residue remaining after IH involution and its location and to identify other potential outcome predictors. All IH patients examined at our hospital from 2008 to 2011 were reviewed, and both the degree of volumetric residue after involution and the quantified degree of sequelae were obtained by comparing follow-up photographs with photographs of lesions at their maximum size. We discovered that for children with a defined age at the initial visit, lesions of the perifacial area achieved significantly more volumetric regression than lesions of the central facial (P < 0.0001) and chest areas (P = 0.0002). Perifacial lesions had a significantly higher aesthetic score than those on the central facial area (P < 0.0001), chest (P < 0.0001) and trunk/extremities (P = 0.0226) when the age at initial visit and lesion type were consistent. Our study reveals that lesion location and age at initial visit are contributing factors to volumetric sequelae and the aesthetics of the lesion after involution. The results of this study provide a reference to guide treatment decisions with the aim of achieving a favorable outcome.


Assuntos
Estética , Hemangioma/patologia , Regressão Neoplásica Espontânea , Neoplasias Cutâneas/patologia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Tomada de Decisão Clínica , Face , Feminino , Seguimentos , Hemangioma/diagnóstico por imagem , Hemangioma/terapia , Humanos , Lactente , Recém-Nascido , Masculino , Neoplasia Residual/diagnóstico por imagem , Neoplasia Residual/patologia , Neoplasia Residual/prevenção & controle , Fotografação , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/terapia , Tórax , Resultado do Tratamento , Carga Tumoral
14.
Ann Otol Rhinol Laryngol ; 128(6): 575-580, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30781970

RESUMO

OBJECTIVES: The treatment of hemangiomas in the hypopharynx and larynx can be challenging and stressful because of the high tumor recurrence rate. The objective of this study was to investigate the therapeutic effect of the combination of intratumor injection of bleomycin and electroresection/electrocautery on the hemangiomas in the hypopharynx and larynx through suspension laryngoscopy. METHODS: With patients under general anesthesia, the hemangiomas were fully exposed through suspension laryngoscopy. After intratumor injection of bleomycin, in some patients, the hemangiomas were completely resected along the bottom of the tumor pedicle by polypus-forceps electroscalpel; for other patients, the hemangiomas were pinched and held, and then the whole-tumor tissues were cauterized and coagulated by the electroscalpel. Prior to commencing the study, all participants signed informed consents, and all procedures were approved by the hospital ethical committee. RESULTS: There was almost no bleeding during the operations, no postoperative dyspnea, and no hemorrhage. The patients were followed up for 3 years; the 3-year cure rate was 97%. CONCLUSION: The hemangioma in the hypopharynx and larynx can be cured by a single-session treatment, using the combination of intratumor injection of bleomycin and electroresection/electrocautery through suspension laryngoscopy. Our method is reliable, affordable, and effective, and it could be widely applied in other hospitals.


Assuntos
Bleomicina/administração & dosagem , Procedimentos Cirúrgicos de Citorredução , Eletrocoagulação , Hemangioma/terapia , Neoplasias Hipofaríngeas/terapia , Neoplasias Laríngeas/terapia , Laringoscopia/métodos , Soluções Esclerosantes/administração & dosagem , Adolescente , Adulto , Idoso , Antibióticos Antineoplásicos/administração & dosagem , Terapia Combinada , Feminino , Seguimentos , Humanos , Injeções Intralesionais , Masculino , Pessoa de Meia-Idade , Adulto Jovem
15.
Cardiovasc Intervent Radiol ; 42(6): 800-811, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30783780

RESUMO

BACKGROUND: Transarterial (chemo-)embolization/lipiodolization (TAE/TAL) might be an attractive minimally invasive alternative to surgery in the treatment of symptomatic hepatic haemangioma. This review assesses the efficacy and safety of TAE/TAL as primary treatment for symptomatic hepatic haemangioma. METHODS: A systematic search of the literature was performed by two reviewers following the PRISMA guidelines. Cohort studies and case reports were identified; outcomes of cohort studies were reported. The primary efficacy outcome was tumour size before and after TAE/TAL. Improvement of symptoms and quality of life (QoL) were secondary outcomes; the primary safety outcome was complications. The Downs and Black statement was used for quality assessment. RESULTS: Eighteen cohort studies were identified, including 1284 patients. TAE/TAL led to a decrease in tumour size in 1100/1223 (89.9%) patients and to improvement or disappearance of symptoms in 1080/1096 (98.5%) patients. A significant decrease in tumour size from 9.79 ± 0.79 cm to 4.00 ± 1.36 cm (p < 0.001) was shown. Grade 3 complications occurred in 37/1284 (2.9%) patients. Surgical treatment was necessary in 35/1284 (2.7%) of patients. CONCLUSION: TAE/TAL appears to be a promising and safe treatment to decrease tumour size of hepatic haemangioma. The technique might also provide partial relief of symptoms, although no randomized clinical trials or prospective studies using validated QoL questionnaires are available. TAE/TAL may be considered as a viable alternative to resection.


Assuntos
Quimioembolização Terapêutica/métodos , Hemangioma/terapia , Neoplasias Hepáticas/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
16.
Pediatr Dermatol ; 36(3): 402-403, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30762240

RESUMO

A chart review was performed of all patients diagnosed with infantile hemangioma in the pediatric dermatology clinic to determine whether distance traveled by the patient affected adherence to follow-up and treatment plans. An increase in distance was associated with an increase in likelihood of nonadherence to treatment or follow-up plans. Compared to patients who lived within 10 miles of a pediatric dermatologist, families who lived farther away were less likely to adhere to treatment plans. Families who traveled over 20 miles to clinic were 7.38 times more likely to be nonadherent to the treatment plan than those who traveled 0-10 miles.


Assuntos
Acesso aos Serviços de Saúde , Hemangioma/terapia , Cooperação do Paciente , Neoplasias Cutâneas/terapia , Criança , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Estudos Retrospectivos
17.
Medisan ; 23(1)ene.-feb. 2019. ilus
Artigo em Espanhol | CUMED | ID: cum-74726

RESUMO

Se describe el caso clínico de un recién nacido, asistido en el Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, sin antecedentes patológicos maternos ni familiares, quien presentaba macrocefalia, asimetría de los miembros inferiores, marcada hipertrofia en el miembro inferior izquierdo y macrodactilia, así como angiomas cutáneos en el área inferior del tronco, en el abdomen, la región glútea y las piernas. Luego de una evaluación detallada por parte de un equipo multidisciplinario, que se basó en el cuadro clínico y el estudio radiográfico, se diagnosticó el síndrome de Klippel-Trenaunay. El paciente ha mantenido una evolución favorable hasta el momento actual y se continúa un estricto seguimiento médico(AU)


The case report of a newborn, assisted in Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba is described, without maternal or family pathological history who presented macrocephalic asymmetry of the lower members, marked hypertrophy in the left low member and macrodactilia, as well as cutaneous angiomas in the lower area of the trunk, abdomen, buttocks region and legs. After a detailed evaluation by a multidisciplinary team which was based on the clinical pattern and radiographical study, the Klippel-Trenaunay syndrome was diagnosed. The patient has maintained a favorable clinical course up to now and a strict medical follow-up is carried out(AU)


Assuntos
Humanos , Masculino , Recém-Nascido , Humanos , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Hemangioma/terapia , Hiperostose , Pesquisa Interdisciplinar
18.
Medisan ; 23(1)ene.-feb. 2019. ilus
Artigo em Espanhol | LILACS | ID: biblio-990184

RESUMO

Se describe el caso clínico de un recién nacido, asistido en el Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, sin antecedentes patológicos maternos ni familiares, quien presentaba macrocefalia, asimetría de los miembros inferiores, marcada hipertrofia en el miembro inferior izquierdo y macrodactilia, así como angiomas cutáneos en el área inferior del tronco, en el abdomen, la región glútea y las piernas. Luego de una evaluación detallada por parte de un equipo multidisciplinario, que se basó en el cuadro clínico y el estudio radiográfico, se diagnosticó el síndrome de Klippel-Trenaunay. El paciente ha mantenido una evolución favorable hasta el momento actual y se continúa un estricto seguimiento médico.


The case report of a newborn, assisted in Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba is described, without maternal or family pathological history who presented macrocephalic asymmetry of the lower members, marked hypertrophy in the left low member and macrodactilia, as well as cutaneous angiomas in the lower area of the trunk, abdomen, buttocks region and legs. After a detailed evaluation by a multidisciplinary team which was based on the clinical pattern and radiographical study, the Klippel-Trenaunay syndrome was diagnosed. The patient has maintained a favorable clinical course up to now and a strict medical follow-up is carried out.


Assuntos
Humanos , Masculino , Recém-Nascido , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Hemangioma/terapia , Hiperostose , Pesquisa Interdisciplinar
20.
Spine J ; 19(1): 131-143, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29890263

RESUMO

BACKGROUND: Vertebral body hemangiomas with myelopathy are difficult to manage. OBJECTIVE: The objective of this study was to evaluate the role of intraoperative ethanol embolization, surgical decompression, and instrumented short-segment fusion in vertebral hemangioma (VH) with myelopathy and long-term outcome (>24 months). CLINICAL MATERIALS AND METHODS: This prospective study included symptomatic VH with cord compression with myelopathy. Pathologic fractures and deformity or multilevel pathologies were excluded from the study. Surgery consisted of intraoperative bilateral pedicular absolute alcohol (<1% hydrated ethyl alcohol) injection, laminectomy, and cord decompression at the level of pathology followed by a short-segment instrumented fusion using pedicle screws. RESULTS: The study included 33 patients (mean 26.9±13.2, range: 10-68 years, 18 females). The clinical features of the study were myelopathy in all patients (5 paraplegic), sphincter involvement (13), and mid back or lower back pain (7). The preoperative American Spinal Injury Association (ASIA) scores were A (7), B (11), C (6), D (8), and E (1). Majority of the patients had single vertebral involvement (30) and three patients had multiple-level involvement. Six patients underwent surgery earlier (one underwent alcohol embolization). The mean surgical time was 124±39 minutes, and the average blood loss was 274±80 cc. The mean amount of absolute alcohol injected was 14.6±5.7 cc (two patients required 20 and 25 cc). Immediate embolization was achieved in all patients, allowing laminectomy and easy removal of soft-tissue hemangioma. Post surgery, one patient had transient deterioration, and the condition of the rest of the patients improved (sphincters improved in nine patients) at a follow-up ranging 28-103 months (mean 47.6±22.3). Follow-up ASIA scores were E (26), D (4), B (2), and C (1). All patients showed evidence of bone sclerosis and relief of cord compression on follow-up imaging. CONCLUSIONS: This is the largest study in literature showing excellent improvement, low reoperation rates after ethanol embolization, and short-segment fixation.


Assuntos
Descompressão Cirúrgica/métodos , Embolização Terapêutica/métodos , Etanol/uso terapêutico , Hemangioma/cirurgia , Laminectomia/métodos , Complicações Pós-Operatórias/epidemiologia , Adolescente , Adulto , Idoso , Criança , Descompressão Cirúrgica/efeitos adversos , Embolização Terapêutica/efeitos adversos , Etanol/administração & dosagem , Feminino , Hemangioma/terapia , Humanos , Injeções/efeitos adversos , Laminectomia/efeitos adversos , Masculino , Pessoa de Meia-Idade , Parafusos Pediculares
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