Opções terapêuticas no manejo do nevus congênito gigante, experiência do Serviço de Cirurgia Plástica e Reaparadora HC-UFPR / Therapeutic options in the management of giant congenital nevus: Experience of the Plastic and Reconstructing Service at Hospital de Clínicas, Federal University of Paraná

INTRODUÇÃO: O nevus congênito gigante (NCG) possui diversas modalidades de tratamento. A abordagem cirúrgica inclui a ressecção parcial seriada ou excisão total. Objetivo: Demonstrar as principais modalidades terapêuticas utilizadas para o tratamento do NCG e avaliar a incidência da localização, idade e tamanho destas lesões no Serviço de Cirurgia Plástica e Reparadora do Hospital de Clínicas da Universidade Federal do Paraná. MÉTODOS: Estudo retrospectivo dos pacientes submetidos a tratamento cirúrgico do NCG no período de janeiro de 2004 a janeiro de 2010. Foram coletados dados como: idade, gênero, tratamento realizado, número de cirurgias realizadas, evolução e complicações. RESULTADOS: Foram avaliados 11 pacientes, sendo 8 mulheres e 3 homens. A média de idade foi de 12,4 anos (3 a 25 anos). O subtipo mais encontrado foi o nevus melanocítico intradérmico correspondendo a 90,9% dos casos e 9,1% com nevus melanocítico composto. Não foram identificados casos de melanoma. A localização mais comum foi a região da cabeça e pescoço. O diâmetro médio das lesões foi de 9,1 cm.As técnicas utilizadas para reconstrução foram: sutura primária, retalhos locais, enxerto de pele e uso de expansores. No seguimento, 63,6% dos pacientes apresentavam ainda nevus residual, 27,3% apresentaram ressecção completa e 9,1% perderam seguimento. CONCLUSÃO: Foi observada maior incidência de NCG na faixa etária de 3 a 25 anos, sendo a localização mais comum na face e com tamanho médio de 9,1cm. O principal tratamento instituído para os pacientes com NCG foi a ressecção parcelada, com bons resultados.

INTRODUCTION: Several modalities are available for the treatment of giant congenital nevus (GCN). The surgical approach includes partial serial resection or total excision. Objective: To demonstrate the main therapeutic modalities in the treatment of GCN and to assess the incidence of location, age, and size of this lesion at the Plastic and Reconstructive Surgery Service of the Hospital de Clínicas, Federal University of Paraná. METHODS: This retrospective study included patients who had undergone surgical treatment for GCN between January 2004 and January 2010. We collected data such as age, sex, treatment performed, number of surgeries carried out, evolution, and complications. RESULTS: We evaluated 11 patients (8 female and 3 male). The average age was 12.4 years (range, 3-25 years). The GCN subtype most commonly found was intradermal melanocytic nevus, which accounted for 90.9% of cases, with melanocytic nevus accounting for the remaining 9.1%. No cases of melanoma were identified. The most common location was the head and neck. The average diameter of the lesions was 9.1 cm. The techniques used for the reconstruction included primary suture, local flaps, skin graft, and the use of expanders. In the follow-up period, 63.6% of the patients still presented a residual nevus, 27.3% underwent complete resection, and 9.1% were not monitored. CONCLUSION: A higher incidence of GCN was observed in patients aged 3-25 years. The most common location was the face, and the average size was 9.1 cm. The main treatment of patients with GCN was splitting resection, which resulted in satisfactory outcomes.

Síndrome de Proteus: relato de casos / Proteus Syndrome: case reports

INTRODUÇÃO: A síndrome de Proteus é uma doença complexa e rara, classificada nos grupo das hamartoses. Foi primeiramente descrita em dois pacientes, em 1979, por Cohen e Hayden. Existe dificuldade no diagnóstico, sendo comum a confusão com síndromes de Klippel-Trenaunay-Weber, neurofibromatose ou Stuge-Weber. Apresentamos dois casos tratados no Serviço de Cirurgia Plástica e Reparadora da Universidade Federal do Paraná. MÉTODO: Paciente masculino (caso 1), que chegou ao serviço aos 6 anos de idade, tendo como principais apresentações lipomatoses e assimetrias. A segunda paciente (caso 2) deu entrada no serviço com 20 anos de idade e diagnóstico de síndrome de Klippel-Trenaunay-Weber, que posteriormente mostrou se tratar de síndrome de Proteus. CONCLUSÃO: A hipótese etiológica mais aceita para a doença é genética. Acredita-se que exista mosaicismo somático e que a doença seja letal no estado não mosaico. Morte prematura é bastante frequente. Entretanto, a sequela mais comum é a ocorrência de tumores incomuns. O cuidado dos pacientes portadores da síndrome é um desafio devido às suas consequências médicas e psicossociais.

INTRODUCTION: Proteus syndrome is a complex and rare disorder classified as a hamartomatous disease. It was first described in two patients in 1979, by Cohen and Hayden. Proteus syndrome is difficult to diagnose, and is often confused with Klippel-Trenaunay-Weber syndrome, neurofibromatosis, or Sturge-Weber syndrome. In this study we describe two patients who were treated at the Plastic and Reconstructive Surgery Service of the Federal University of Paraná. METHOD: A 6-year-old male patient (case 1) presented to the Service with lipomatosis and asymmetry, as the primary findings. A 20-year-old (case 2) was admitted to the Service with a diagnosis of Klippel-Trenaunay-Weber syndrome, which later was shown to be Proteus syndrome. CONCLUSION: The etiological hypothesis that is most accepted for this disease is genetic. It is believed that somatic mosaicism may occur during pathogenesis, which can be lethal in the mosaic state. Premature death is common. However, the most common sequelae are the occurrence of unusual tumors. The care of patients with this syndrome is a challenge due to medical and psychosocial consequences.

An alternative experimental model for training in microsurgery.

OBJECTIVE: to describe a new model of training in microsurgery with pig spleen after splenectomy performed by undergraduate students of the Discipline of Operative Technique of the UFPR Medical School. METHODS: after the completion of splenectomy we performed dissection of the vascular pedicle, distal and proximal to the ligation performed for removal of the spleen. After complete dissection of the splenic artery and vein with microscope, clamps were placed and the vessels were cut. We then made the anastomosis of the vessels with 9.0 nylon. RESULT: the microsurgical training with a well-defined routine, qualified supervision and using low cost experimental materials proved to be effective in the practice of initial microvascular surgery. CONCLUSION: the use of pig spleen, which would be discarded after splenectomy, is an excellent model for microsurgical training, since besides having the consistency and sensitivity of a real model, it saves the sacrifice of a new animal model in the initial learning phase of this technique.

An alternative experimental model for training in microsurgery / Modelo experimental alternativo para treinamento em microcirurgia

OBJECTIVE: to describe a new model of training in microsurgery with pig spleen after splenectomy performed by undergraduate students of the Discipline of Operative Technique of the UFPR Medical School. METHODS: after the completion of splenectomy we performed dissection of the vascular pedicle, distal and proximal to the ligation performed for removal of the spleen. After complete dissection of the splenic artery and vein with microscope, clamps were placed and the vessels were cut. We then made the anastomosis of the vessels with 9.0 nylon. RESULT: the microsurgical training with a well-defined routine, qualified supervision and using low cost experimental materials proved to be effective in the practice of initial microvascular surgery. CONCLUSION: the use of pig spleen, which would be discarded after splenectomy, is an excellent model for microsurgical training, since besides having the consistency and sensitivity of a real model, it saves the sacrifice of a new animal model in the initial learning phase of this technique. .

OBJETIVO: descrever um novo modelo de treinamento em microcirurgia com baço de suínos após esplenectomia realizada pelos alunos de graduação da disciplina de técnica operatória do curso de medicina da UFPR. MÉTODOS: após a realização da esplenectomia realizamos dissecção do pedículo vascular distal e proximal a ligadura realizada para a retirada do baço. Após a dissecção completa da artéria e veia esplênica ,com microscópio, são colocados os clampes e o vaso é seccionado. É então realizada a anastomose dos vasos com mononylon 9,0. RESULTADO: o treinamento microcirúrgico, com uma rotina bem definida, supervisão qualificada e utilizando materiais experimentais de baixo custo, mostrou-se efetivo na prática de cirurgia microvascular inicial. CONCLUSÃO: a utilização do baço suíno, que seria desprezado após esplenectomia, é um excelente modelo para treinamento microcirúrgico, pois além de ter a consistência e delicadeza de um modelo real poupa o sacrifício de um novo modelo animal, na fase inicial de aprendizado desta técnica. .

Fraturas nasoetmoideorbitais: experiências de 37 anos do serviço de cirurgia craniofacial do hospital de Cajuru e hospital do trabalhador / Naso-orbitoethmoid fractures: 37-year experience of the craniofacial surgery service of the hospital de Cajuru and hospital do trabalhador

Fractures of the naso-orbitoethmoid complex (NOE) remain one of the most challenging tasks in facial reconstruction and account for 2.1% of facial trauma cases. Clinical analyses of NOE fractures showed that they usually affect the telecanthus and cause deformities that would then require retropositioning of the nasal pyramid. Therefore, computed tomographyis an essential technique for further assessment and to identify bone dislocations and fistulas. Treatment involves reconstruction of the intercanthal distance, nasal projection, and internal orbital structures.

A fratura do complexo nasoetmoideorbital (NEO) permanece como uma das tarefas mais desafiadoras no trauma facial. Corresponde a 2,1% dos casos de trauma de face. Achados clínicos clássicos das fraturas NEOs são telecanto e deformidade com retroposicionamento da pirâmide nasal. O estudo com tomografia computadorizada é imprescindível para determinar detalhes e procurar localizar deslocamentos ósseos e fístulas. O tratamento é direcionado à reconstrução da relação intercantal, da projeção nasal e das estruturas internas da órbita.

The impact of the model for end-stage liver disease (MELD) on liver transplantation in one center in Brazil.

CONTEXT: Presently the MELD score is used as the waiting list criterion for liver transplantation in Brazil. In this method more critical patients are considered priority to transplantation. OBJECTIVE: To compare the results of liver transplantation when the chronologic waiting list was the criterion for organ allocation (pre-MELD era) with MELD score period (MELD era) in one liver transplantation unit in Brazil. METHODS: The charts of the patients subjected to liver transplantation at the Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, PR, Brazil, were reviewed from January of 2001 to August of 2008. Patients were divided into two groups: pre-MELD era and MELD era. They were compared in relation to demographics of donors and receptors, etiology of cirrhosis, cold and warm ischemia time, presence of hepatocellular carcinoma, MELD score and Child-Pugh score and classification at the time of transplantation, units of red blood cells transfused during the transplantation, intensive care unit stay, total hospital stay and 3 month and 1 year survival. RESULTS: Initially, 205 liver transplantations were analyzed. Ninety four were excluded and 111 were included: 71 on the pre-MELD era and 40 on the MELD era. The two groups were comparable in relation to donors and receptors age and sex, etiology of cirrhosis and cold and warm ischemia time. The receptors of the MELD era had more hepatocellular carcinoma than those of the pre-MELD era (37.5% vs 16.9%). Patients with hepatocellular carcinoma had less advanced cirrhosis on both eras. The MELD score was the same on both eras. Excluding the cases of hepatocellular carcinoma, MELD era score was higher than pre-MELD score (18.2 vs 15.8). There were an increased number of transplants on Child-Pugh A and C and a decreased number on Child-Pugh B receptors on MELD era. Both eras had the same need of red blood cells transfusion, intensive care unit stay and hospital stay. Also, 3 month and 1 year survival were the same: 76% and 74.6% on pre-MELD era and 75% and 70.9% on MELD era. CONCLUSION: In our center, after the introduction of MELD score as the priority criterion for liver transplantation there were an increased number of transplants with hepatocellular carcinoma. Excluding these patients, the receptors were operated upon with more advanced cirrhosis. Nevertheless the patients had the same need for red blood cells transfusion, intensive care unit and hospital stay and 3 months and 1 year survival.

The impact of the model for end-stage liver disease (MELD) on liver transplantation in one center in Brazil / O impacto do MELD no transplante de fígado em um centro no Brasil

CONTEXT: Presently the MELD score is used as the waiting list criterion for liver transplantation in Brazil. In this method more critical patients are considered priority to transplantation. OBJECTIVE: To compare the results of liver transplantation when the chronologic waiting list was the criterion for organ allocation (pre-MELD era) with MELD score period (MELD era) in one liver transplantation unit in Brazil. METHODS: The charts of the patients subjected to liver transplantation at the Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, PR, Brazil, were reviewed from January of 2001 to August of 2008. Patients were divided into two groups: pre-MELD era and MELD era. They were compared in relation to demographics of donors and receptors, etiology of cirrhosis, cold and warm ischemia time, presence of hepatocellular carcinoma, MELD score and Child-Pugh score and classification at the time of transplantation, units of red blood cells transfused during the transplantation, intensive care unit stay, total hospital stay and 3 month and 1 year survival. RESULTS: Initially, 205 liver transplantations were analyzed. Ninety four were excluded and 111 were included: 71 on the pre-MELD era and 40 on the MELD era. The two groups were comparable in relation to donors and receptors age and sex, etiology of cirrhosis and cold and warm ischemia time. The receptors of the MELD era had more hepatocellular carcinoma than those of the pre-MELD era (37.5 percent vs 16.9 percent). Patients with hepatocellular carcinoma had less advanced cirrhosis on both eras. The MELD score was the same on both eras. Excluding the cases of hepatocellular carcinoma, MELD era score was higher than pre-MELD score (18.2 vs 15.8). There were an increased number of transplants on Child-Pugh A and C and a decreased number on Child-Pugh B receptors on MELD era. Both eras had the same need of red blood cells transfusion, intensive care unit stay and hospital stay. Also, 3 month and 1 year survival were the same: 76 percent and 74.6 percent on pre-MELD era and 75 percent and 70.9 percent on MELD era. CONCLUSION: In our center, after the introduction of MELD score as the priority criterion for liver transplantation there were an increased number of transplants with hepatocellular carcinoma. Excluding these patients, the receptors were operated upon with more advanced cirrhosis. Nevertheless the patients had the same need for red blood cells transfusion, intensive care unit and hospital stay and 3 months and 1 year survival.

CONTEXTO: Atualmente o MELD é utilizado no Brasil como critério de seleção de receptores na lista de espera para transplante hepático. Esse sistema prioriza para o transplante os pacientes com cirrose hepática mais avançada. OBJETIVO: comparar os resultados do transplante hepático quando o tempo em lista de espera era o critério de alocação de órgãos (era pré-MELD) em relação ao período em que se utiliza o MELD (era MELD). MÉTODOS: Foram revisados os prontuários dos pacientes submetidos a transplante hepático no Hospital de Clínicas da Universidade Federal do Paraná no período de janeiro de 2001 até agosto de 2008. Os pacientes foram divididos em dois grupos: era pré-MELD e era MELD. Foram comparados em relação aos dados demográficos dos doadores e dos receptores, à etiologia da cirrose, ao tempo de isquemia morna e fria, à presença de carcinoma hepatocelular, ao escore do MELD e ao escore e à classificação de Child-Pugh no momento do transplante, às unidades de concentrado de plaquetas transfundidas durante o transplante, ao tempo de permanência na UTI, ao tempo de permanência hospitalar e à sobrevida do paciente em 3 meses e em 1 ano. RESULTADOS: Inicialmente 205 transplantes foram avaliados. Noventa e quatro foram excluídos e 111 foram incluídos: 71 na era pré-MELD e 40 na era MELD. Os dois grupos foram semelhantes em relação à idade e ao sexo dos doadores e receptores, à etiologia da cirrose e ao tempo de isquemia morna e fria. Os receptores da era MELD apresentaram maior número de pacientes com carcinoma hepatocelular em relação à era pré-MELD (37,5 por cento vs 16,9 por cento). Os doentes com carcinoma hepatocelular apresentaram cirrose hepática menos avançada em ambas as eras. O escore do MELD foi igual em ambas as eras. Excluindo aqueles com carcinoma hepatocelular, o escore foi maior na era MELD em relação à era pré-MELD (18,2 vs 15,8). Na era MELD foi observado aumento no número de transplantes realizados em pacientes com cirrose hepática classes A e C de Child-Pugh e redução nos da classe B. As duas eras apresentaram resultados iguais em relação à transfusão de hemácias e tempo de permanência na UTI e permanência hospitalar. A sobrevida em 3 meses e em 1 ano também foi igual: 76 por cento e 74,6 por cento na era pré-MELD e 75 por cento e 70,9 por cento na era MELD. CONCLUSÃO: No centro deste estudo, após a introdução do MELD como critério de seleção de receptores para transplante hepático houve incremento no número de procedimentos em doentes com carcinoma hepatocelular. Excluindo-se esses pacientes, os receptores foram operados em estágios mais avançados da cirrose. Apesar disso, apresentaram a mesma necessidade de transfusão de hemácias, permanência na UTI e permanência hospitalar, e sobrevida em 3 meses e sobrevida em 1 ano.

Stapes surgery in residency: the UFPR clinical hospital experience.

UNLABELLED: Surgery of the stapedius remains the established treatment for otosclerosis. Recent publications have showed that success in surgeries done by residents have decreased and hearing results are worse than those obtained by experienced otologic surgeons. AIM: To evaluate the experience of the otorhinolaryngology unit, Parana University, relative to stapes surgery done in the residency training program. MATERIAL AND METHOD: A retrospective study of 114 stapes surgeries done in the past 9 years in 96 patients. Audiometric results were analysed according to the Committee on Hearing and Equilibrium guidelines and the Amsterdam Hearing Evaluation Plots. The improvement of the airway postoperative gap and thresholds were taken into account. RESULTS: 96 patients were included, most of them female adults (67.7%) and white (93.7%). Stapedectomy was done in 50.9% of cases, mostly under local anesthesia and sedation (96.5%), using mostly the Teflon prosthesis (37.7%). The surgical success rate was 50.88%, there was an 11.4% complication rate. CONCLUSION: Postoperative hearing gains considered as surgical success were inferior to published results in the literature, done by experienced surgeons.

Cirurgia do estapédio na residência: experiência do Hospital de Clínicas/UFPR / Stapes surgery in residency: the ufpr clinical hospital experience

A cirurgia do estapédio permanece como tratamento consagrado para a otosclerose. Recentes publicações têm demonstrado que o sucesso cirúrgico nas cirurgias realizadas por médicos residentes tem diminuído e que os resultados audiológicos tem sido piores que os obtidos por cirurgiões experientes. OBJETIVOS: Avaliar a experiência do serviço de otorrinolaringologia do Hospital de Clínicas/UFPR na realização de cirurgias do estapédio no programa de residência médica. MATERIAL E MÉTODO: Estudo retrospectivo de 114 cirurgias do estapédio realizadas nos últimos 9 anos, em 96 pacientes. Os resultados audiométricos foram analisados conforme orientação do Committee on Hearing and Equilibrium e através do Amsterdam Hearing Evaluation Plots, considerando a melhora do gap pós-operatório e de limiares da via aérea. RESULTADOS: Foram incluídos 96 pacientes, na maioria adultos, do sexo feminino (67,7 por cento) e caucasianos (93,7 por cento). Em 50,9 por cento dos casos foi realizada estapedectomia, sendo a grande maioria sob anestesia local e sedação (96,5 por cento) e utilizando principalmente a prótese de Teflon (37,7 por cento). A taxa de sucesso cirúrgico foi de 50,88 por cento, com 11,4 por cento de complicações. CONCLUSÃO: Os ganhos de audição pós-operatórios considerados como sucesso cirúrgico foram inferiores aos publicados na literatura por cirurgiões experientes.

Surgery of the stapedius remains the established treatment for otosclerosis. Recent publications have showed that success in surgeries done by residents have decreased and hearing results are worse than those obtained by experienced otologic surgeons. AIM: To evaluate the experience of the otorhinolaryngology unit, Parana University, relative to stapes surgery done in the residency training program. MATERIAL AND METHOD: A retrospective study of 114 stapes surgeries done in the past 9 years in 96 patients. Audiometric results were analysed according to the Commitee on Hearing and Equilibrium guidelines and the Amsterdam Hearing Evaluation Plots. The improvement of the airway postoperative gap and thresholds were taken into account. RESULTS: 96 patients were included, most of them female adults (67.7 percent) and white (93.7 percent). Stapedectomy was done in 50.9 percent of cases, mostly under local anesthesia and sedation (96.5 percent), using mostly the Teflon prothesis (37.7 percent). The surgical success rate was 50.88 percent, there was an 11.4 percent complication rate. CONCLUSION: Postoperative hearing gains considered as surgical success were inferior to published results in the literature, done by experienced surgeons.

Linfocintigrafia pré-operatória em gama-câmara associada ao azul patente para biópsia do linfonodo sentinela no câncer de mama / Gamma-camera lymphoscintigrahy and blue dye for sentinel node biopsy in breast cancer

Apesar do gama-probe ser a técnica que possui os melhores resultados na identificação do linfonodo sentinela (LS), a sua maior difusão nos países em desenvolvimento ainda é limitada em razão de seu custo elevado. Assim, o propósito deste estudo, realizado entre outubro de 1999 e agosto de 2004, foi determinar a eficácia da associação da linfocintigrafia pré-operatória na gama-câmara com o azul patente para biópsia do LS, sem o uso do gama-probe. Caso o linfonodo não estivesse corado com o azul patente, então era realizada amostragem do lifonodo com a projeção marcada na pele pela gama-câmara. Foram incluídas 146 pacientes com carcinomas invasivos T1-2 N0M0. As primeiras 50 pacientes foram submetidas ao esvaziamento axilar, para verificar o índice de falso-negativo da técnica, que resultou em 5,9%. A média de idade foi de 58,1 anos e a média do tamanho tumoral foi de 20,7 mm. O LS foi identificado em 98,6% das pacientes. Em 6 (4,1%), o LS foi identificado apenas pela gama-câmara e, em 2 pacientes, apenas o azul patente identificou o LS. Esses resultados demonstraram que essa técnica é uma alternativa viável para biópsia do LS no nosso meio.

Gamma-probe is the most reliable technique for sentinel node (SN) biopsy, but is remains cost-limiting for its widespread use in development countries. Then, the purpose of this study, performed from October 1999 to August 2004, was to determine the efficacy of the association of preoperative gamma camera lymphoscintigraphy and blue dye for SN biopsy, without the use of the gamma- probe. If the blue node were not find at surgery, then axillary sampling should be done in SN projection designated by gamma-camera.146 T1-2, N0, M0, invasive breast cancer patients were enrolled in this trial. The first 50 patients were submitted to axillary dissection, in order to find the false-negative rate. It was 5.9%. The mean age was 58.1 years and the mean tumor size was 20.7 mm. SN was identified in 98.6% of patients. In 6 (4.1%)patients. SN was identification was supported only by the gamma-camera, and the blue dye identified the SN IN 2 (1,4%) gamma-camera failure. These data support it as a reliable and low cost technique for SN biopsy.