Correlation between selected angiogenic markers and prognosis in pediatric adrenocortical tumors: Angiogenic markers and prognosis in pediatric ACTs.

BACKGROUND/PURPOSE: Pediatric adrenocortical tumor (ACT) remains a challenging disease. Tumor weight and disease stage are still the most used indicators to prognosis and guidance of clinical decisions. Histology has not added meaningful data for risk stratification and management. ACT is metabolically active, highly vascularized, locally invasive and has the propensity to produce distant metastasis. Our objective was to correlate the expression of vascular endothelial growth factor (VEGF) and intratumoral microvessel density (MVD) with clinical and prognostic aspects in pediatric ACT. PROCEDURE: In 27 tumors, immunohistochemical expression of VEGF, CD105 (endoglin) and CD34 was analyzed. MVD was determined by CD34 and CD105 antibodies. MVD and VEGF expression was correlated with clinical characteristics and outcome. Normal pediatric glands were used as controls. RESULTS: Endoglin MVD was significantly higher and CD34 MVD was significantly lower in ACT than control. The VEGF expression did not differ between groups. Cytoplasmic staining for endoglin was correlated with hypertension in ACT. Endoglin MVD greater than 1 mv/field, CD34 MVD less than 32 mv/field and VEGF expression levels above 4.8% were associated with clinical and biological indicators of poor prognosis. CONCLUSIONS: Endoglin and CD34 MVD values are potential histological markers to refine the histologic classification of pediatric ACT.

Renal tumor and trauma: a pitfall for conservative (correction of conversative) management.

PURPOSE: Conservative management has been largely used for renal trauma. Although this approach is safe and highly recommended, it can hide a pre-existing unknown condition, such as tumors or urinary malformations. A high index of suspicion is needed for early recognition of these conditions. We present four cases treated at the Pediatric Oncology Institute - Federal University of São Paulo, which have been initially treated conservatively for renal trauma. MATERIALS AND METHODS: We reviewed all 218 renal cases of renal tumors treated at our institution in a 22-year period, searching for associated trauma events. RESULTS: Four cases of renal tumors were initially treated conservatively for blunt renal trauma of low energy mechanism. Patients ' ages ranged from 7 to 12 years old. Two patients had no previous symptoms, one patient had hematuria and another had an abdominal mass. Computerized Axial Tomography (CT) of the abdomen revealed disparate magnitude of the renal bleeding to the low energy mechanism of trauma. All patients underwent surgical treatment. Kidney specimens showed Wilms tumor in three cases and renal carcinoma in one. CONCLUSIONS: The association between renal tumors and trauma should be suspected when renal trauma hemorrhage on abdominal CT scan does not match the low energy mechanism of blunt abdominal trauma. The key for a successful diagnosis of renal tumor or congenital malformations is the high index of suspicion for these conditions.

Totally implantable catheter embolism: two related cases / Embolia de cateter totalmente implantável: relato de dois casos

CONTEXT AND OBJECTIVE: Long-term totally implantable catheters (e.g. Port-a-Cath®) are frequently used for long-term venous access in children with cancer. The use of this type of catheter is associated with complications such as infection, extrusion, extravasation and thrombosis. Embolism of catheter fragments is a rare complication, but has potential for morbidity. The aim here was to report on two cases in which embolism of fragments of a long-term totally implantable catheter occurred. DESIGN AND SETTING: Case series study at Hospital do Servidor Público Estadual, São Paulo. METHODS: Retrospective review of catheter embolism in oncological pediatric patients with long-term totally implantable catheters. RESULTS: The first patient was a 3-year-old girl diagnosed with stage IV Wilms' tumor. Treatment was started with the introduction of a totally implantable catheter through the subclavian vein. At the time of removal, it was realized that the catheter had fractured inside the heart. An endovascular procedure was necessary to remove the fragment. The second case was a boy diagnosed with stage II Wilms' tumor at the age of two years. At the time of removal, it was noticed that the catheter had disconnected from the reservoir and an endovascular procedure was also necessary to remove the embolized catheter. CONCLUSION: Embolism of fragments of totally implantable catheters is a rare complication that needs to be recognized even in asymptomatic patients.

CONTEXTO E OBJETIVO: Os cateteres de longa permanência totalmente implantáveis (por exemplo, Port-a-Cath®) são muito utilizados para acesso venoso de longa duração em crianças com câncer. O uso deste tipo de cateter está associado a algumas complicações como infecção, extrusão, extravasamento e trombose. A embolia de fragmento de cateter é complicação rara, mas com potencial morbidade. O objetivo do estudo foi relatar dois casos em que houve embolia de fragmento de cateter de longa permanência. TIPO DE ESTUDO E LOCAL: Série de casos do Hospital do Servidor Público estadual, São Paulo. MÉTODOS: Estudo retrospectivo das embolias de cateter de longa permanência em crianças. RESULTADOS: No primeiro caso, descreve-se uma menina com diagnóstico de tumor de Wilms estádio IV aos três anos de idade. Iniciou tratamento com implante de cateter de longa permanência em veia subclávia. Três anos após diagnóstico, foi programada a retirada do cateter por término de tratamento. No momento da retirada, houve fratura intracardíaca do cateter. Houve necessidade de retirada do fragmento intracardíaco com hemodinâmica. O segundo caso tratava-se de um menino com diagnóstico de tumor de Wilms estádio II aos dois anos de idade. Teve o cateter implantado no início do tratamento. No momento da retirada por fim de tratamento, o cateter já havia desconectado e embolizado. O fragmento intracardíaco foi retirado também por procedimento de hemodinâmica. CONCLUSÃO: A embolia de fragmentos de cateter em cateteres de longa permanência é uma complicação rara, mas que necessita ser reconhecida, mesmo em pacientes assintomáticos.

Ultrasound evaluation of techniques for internal jugular vein puncture in children / Avaliação ultra-sonográfica das técnicas de punção da veia jugular interna em crianças

PURPOSE: To determine by ultrasound which access and position the child must stay to obtain the best transversal section of the right Internal Jugular Vein (RIJV) allowing a safer puncture. METHODS: Three possible accesses to the RIJV, anterior, lateral and posterior, from 57 healthy children, were analyzed through ultrasound images in a sequence of positions of the head, in supine position, with or without a roll under the scapula: head centered in neutral position with and without a roll (NPP and NP); contra lateral rotation with and without a roll (CLRP and CLR), neutral position and the patient raised in 30° in Trendelenburg position (TDG). To analyze the results it was applied one statistic method, with variation analysis to the same individuals. Basic Procedures: Ultrasound evaluation in each one of the proposed positions. RESULTS: The statistical analysis of the results observed that the lateral puncture with the patient in the neutral position, in Trendelemburg without a roll, offers a bigger area in comparison to all the other options of puncture and positioning of the patient (p<0, 0001). CONCLUSION: The safer way for the puncture of RIJV in children is obtained in neutral position in Trendelemburg by lateral puncture, without a shoulder roll.

OBJETIVO: Determinar pelo ultra-som qual o melhor acesso e posicionamento da criança com o intuito de se obter a melhor secção transversal da veia jugular interna direita (VJID), permitindo uma punção com maior segurança. MÉTODOS: Três possíveis acessos a VJID, anterior, lateral e posterior foram analisados pela ultrassonografia em uma sequência de diferentes posições da cabeça, estando o paciente em posição supina com ou sem um coxim sob a escápula; cabeça na posição neutra; (NPP E NP); rotação lateral da cabeça (CLRP e CLR), posição neutra com o paciente em posição de Trendlemburg a 30(0) (TDG). Para analisar os resultados foi aplicado um método estatístico com análise variada sobre os mesmos indivíduos. Procedimentos básicos: Avaliação ultrassonográfica em cada uma das posições propostas. RESULTADOS: Pela análise estatística dos resultados observou-se que a punção lateral estando o paciente em posição neutra, em Trendlemburg sem a colocação de coxim sob a escápula oferece uma área maior em comparação a todas as outras opções de punção e posicionamento do paciente (p<0,0001). CONCLUSÃO: A melhor técnica para a punção da VJID em crianças foi a posição neutra em Trendlemburg, por punção lateral , sem a colocação de um coxim sob a escápula.

Totally implantable catheter embolism: two related cases.

CONTEXT AND OBJECTIVE: Long-term totally implantable catheters (e.g. Port-a-Cath) are frequently used for long-term venous access in children with cancer. The use of this type of catheter is associated with complications such as infection, extrusion, extravasation and thrombosis. Embolism of catheter fragments is a rare complication, but has potential for morbidity. The aim here was to report on two cases in which embolism of fragments of a long-term totally implantable catheter occurred. DESIGN AND SETTING: Case series study at Hospital do Servidor Público Estadual, São Paulo. METHODS: Retrospective review of catheter embolism in oncological pediatric patients with long-term totally implantable catheters. RESULTS: The first patient was a 3-year-old girl diagnosed with stage IV Wilms' tumor. Treatment was started with the introduction of a totally implantable catheter through the subclavian vein. At the time of removal, it was realized that the catheter had fractured inside the heart. An endovascular procedure was necessary to remove the fragment. The second case was a boy diagnosed with stage II Wilms' tumor at the age of two years. At the time of removal, it was noticed that the catheter had disconnected from the reservoir and an endovascular procedure was also necessary to remove the embolized catheter. CONCLUSION: Embolism of fragments of totally implantable catheters is a rare complication that needs to be recognized even in asymptomatic patients.

Vertical gastric plication versus Nissen fundoplication in the treatment of gastroesophageal reflux in children with cerebral palsy.

CONTEXT AND OBJECTIVE: Association between neurological lesions and gastroesophageal reflux disease (GERD) in children is very common. When surgical treatment is indicated, the consensus favors the fundoplication technique recommended by Nissen, despite its high morbidity and relapse rates. Vertical gastric plication is a procedure that may have advantages over Nissen fundoplication, since it is less aggressive and more adequately meets anatomical principles. The authors proposed to compare the results from the Nissen and vertical gastric plication techniques. DESIGN AND SETTING: Randomized prospective study within the Postgraduate Surgery and Experimentation Program of UNIFESP-EPM, at Hospital do Servidor Público Estadual (IAMSPE) and Hospital Municipal Infantil Menino Jesus. METHODS: Fourteen consecutive children with cerebral palsy attended between November 2003 and July 2004 were randomized into two groups for surgical treatment of GERD: NF, Nissen fundoplication (n = 7); and VGP, vertical gastric plication (n = 7). These were clinically assessed by scoring for signs and symptoms, evaluation of esophageal pH measurements, duration of the operation, intra and postoperative complications, mortality and length of hospital stay. RESULTS: The mean follow-up was 5.2 months; symptoms were reduced by 42.8% (NF) (p = 0.001) and 57.1% (VGP) (p = 0.006). The Boix-Ochoa score was favorable for both groups: NF (p < 0.001) and VGP (p < 0.042). The overall mortality was 14.28% in both groups and was due to causes unrelated to the surgical treatment. CONCLUSION: The two operative procedures were shown to be efficient and efficacious for the treatment of GERD in neuropathic patients, over the study period.

Gastric hemangioma in a 5-year-old boy.

We report a rare cause of gastrointestinal bleeding in a 5-year-old boy with hematemesis and melena for a month. Upper gastrointestinal endoscopy and an abdominal magnetic resonance image suggested a large hemangioma on the gastric fundus. The patient underwent an exploratory laparotomy with partial gastrectomy limited to the gastric fundus. An intraoperative endoscopy helped to ensure a complete resection of the tumor. Histopathology showed a cavernous hemangioma of the gastric fundus.

Vertical gastric plication versus Nissen fundoplication in the treatment of gastroesophageal reflux in children with cerebral palsy

CONTEXT AND OBJECTIVE: Association between neurological lesions and gastroesophageal reflux disease (GERD) in children is very common. When surgical treatment is indicated, the consensus favors the fundoplication technique recommended by Nissen, despite its high morbidity and relapse rates. Vertical gastric plication is a procedure that may have advantages over Nissen fundoplication, since it is less aggressive and more adequately meets anatomical principles. The authors proposed to compare the results from the Nissen and vertical gastric plication techniques. DESIGN AND SETTING: Randomized prospective study within the Postgraduate Surgery and Experimentation Program of Unifesp-EPM, at Hospital do Servidor Público Estadual (IAMSPE) and Hospital Municipal Infantil Menino Jesus. METHODS: Fourteen consecutive children with cerebral palsy attended between November 2003 and July 2004 were randomized into two groups for surgical treatment of GERD: NF, Nissen fundoplication (n = 7); and VGP, vertical gastric plication (n = 7). These were clinically assessed by scoring for signs and symptoms, evaluation of esophageal pH measurements, duration of the operation, intra and postoperative complications, mortality and length of hospital stay. RESULTS: The mean follow-up was 5.2 months; symptoms were reduced by 42.8 percent (NF) (p = 0.001) and 57.1 percent (VGP) (p = 0.006). The Boix-Ochoa score was favorable for both groups: NF (p < 0.001) and VGP (p < 0.042). The overall mortality was 14.28 percent in both groups and was due to causes unrelated to the surgical treatment. CONCLUSION: The two operative procedures were shown to be efficient and efficacious for the treatment of GERD in neuropathic patients, over the study period.

CONTEXTO E OBJETIVO: A associação de crianças portadoras de lesão neurológica com doença do refluxo gastroesofágico (DRGE) é muito freqüente. Quando indicado o tratamento operatório, o consenso é pela técnica de fundoplicatura preconizada por Nissen, porém com alta taxa de morbidade e recidiva. A técnica de plicatura gástrica vertical é um procedimento que tem eventuais vantagens sobre a técnica consensual por ser menos agressiva e atender mais adequadamente aos princípios anatômicos. Os autores se propõem a comparar os resultados da técnica à Nissen com a de plicatura gástrica vertical. TIPO DE ESTUDO E LOCAL: Estudo prospectivo randomizado do Programa de Pós-graduação em Cirurgia e Experimentação da Universidade Federal de São Paulo (UNIFESP-EPM), Hospital do Servidor Público Estadual-Iamspe e Hospital Municipal Infantil Menino Jesus. MÉTODOS: 14 crianças consecutivamente internadas com paralisia cerebral no período de novembro de 2003 a julho de 2004 foram randomizadas em dois grupos para o tratamento cirúrgico da DRGE: Grupo FN (n = 7), fundoplicatura a Nissen, e Grupo PGV (n = 7), plicatura gástrica vertical. Foram avaliados clinicamente através de um escore de sinais e sintomas, a avaliação da pHmetria esofágica, o tempo operatório, complicações intra e pós-operatórias, mortalidade e a permanência hospitalar. RESULTADOS: O acompanhamento médio foi de 5,2 meses; redução dos sintomas em 42,8 por cento (G-FN) (p = 0,001) e 57,1 por cento (G-PGV) (p = 0,006). Escore de Boix-Ochoa foi favorável nos dois grupos G-FN (p < 0,001) e G-PGV (p < 0,042). A mortalidade global foi de 14,28 por cento nos dois grupos e deveu-se a causas estranhas ao tratamento cirúrgico. CONCLUSÃO: Os dois procedimentos operatórios mostraram-se efetivos no tratamento da DRGE em pacientes neuropatas, no período de tempo estudado.

Cavectomy for the treatment of Wilms tumor with vascular extension.

PURPOSE: Vascular extension to the vena cava occurs in 4% of Wilms tumor cases and can reach the right atrium in up to 1%. When this happens the thrombus is usually not adherent to the vessel wall, and there is blood flow around it. Preoperative chemotherapy can cause thrombus regression and even resolution. If the thrombus persists after chemotherapy, surgery will be a challenge. On the other hand, if the thrombus invades the vessel wall, its removal may not be feasible. In this situation cavectomy is a good surgical strategy because it provides complete resection. The prerequisite for cavectomy is the absence of blood flow in the vena cava on preoperative Doppler ultrasonography. We report 3 cases of Wilms tumor with vena caval invasion in which cavectomy was performed, and discuss the principles, indications and operative technique. MATERIALS AND METHODS: A total of 171 patients with Wilms tumor were treated at our institution between 1984 and 2004. Of these patients 6 with intravascular extension of thrombus within the right atrium were treated with extracorporeal circulation, cardiac arrest and profound hypothermia, and 3 were treated with cavectomy. RESULTS: There were no instances of surgical complications or postoperative renal failure in our patients who underwent cavectomy. All remain well and free of disease. CONCLUSIONS: Cavectomy is a safe procedure for treating pediatric patients with Wilms tumor when there is extension and invasion of the vena cava wall without blood flow.

Prehospital pediatric trauma classification (PHPTC) as a tool for optimizing trauma care resources in the city of São Paulo, Brazil

OBJETIVO: Avaliar o atendimento pré-hospitalar de crianças e adolescentes em São Paulo, avaliar o banco de dados das Unidades de Suporte Básico (UR) e Avançado (USA) e propor um método simples e eficaz para a avaliação da gravidade do trauma pediátrico na fase pré-hospitalar. MÉTODOS: Uma única central do Corpo de Bombeiros (COBOM) coordena todo o atendimento pré-hospitalar em São Paulo. Dois bancos de dados foram analisados para crianças de 0 a 18 anos de idade, entre 1998 e 2001: um das Unidades de Suporte Básico de Vida (UR- bombeiros) e outra de Unidades de Suporte Avançado (USA - médico e bombeiros). Neste período, o Serviço de Atendimento Médico de Urgência do Estado de São Paulo (SAMU) forneceu relatórios médicos de 604 vítimas, enquanto os bombeiros forneceram relatórios de 12.761 vitimas (UR+USA). A classificação do trauma pré-hospitalar pediátrico é baseada na condição fisiológica, mecanismo de trauma e lesões anatômicas das vítimas. A classificação do trauma pré-hospitalar pediátrico foi comparada à Escala de Coma de Glasgow (GCS) e ao Escore de Trauma Revisado (RTS). RESULTADOS: Houve predominância do sexo masculino em ambos bancos de dados. O mecanismo de trauma mais freqüente foi relacionado a transporte, seguido de quedas. A mortalidade foi 1,6% nas Unidades Básicas e 9,6% no Suporte Avançado. Houve associação entre a classificação do trauma pré-hospitalar pediátrico, Escala de Coma de Glasgow (GCS) e ao Escore de Trauma Revisado (RTS) GCS e RTS (p<0,0001). CONCLUSAO: A classificação do trauma pré-hospitalar pediátrico é um método simples e confiável para a avaliação, triagem e recrutamento de recursos para o atendimento pré-hospitalar do trauma pediátrico.

Hormonal cryptorchidism therapy: systematic review with metanalysis of randomized clinical trials.

The importance of cryptorchidism treatment concerns the possibility of diminishing risk of malignant degeneration and improving fertility. Success rates of hormonal treatment vary: 0-55% with human chorionic gonadotropin (hCG) and 9-78% with gonadotropin-releasing hormone (GnRH). Due to uncertainties regarding the effectiveness of this treatment, a systematic review and meta-analysis of randomized controlled trials (RCTs) on hormonal cryptorchidism treatment was done using the methodology of Cochrane Collaboration. Two studies compared hCG with GnRH, with a testicular descent rate of 25% vs. 18%, respectively. Nine trials compared intranasal LHRH with placebo, with complete testicular descent rates of 19% vs. 5%. Two other studies comparing doses and administration intervals could not be pooled together due to heterogeneity. With the information analyzed until the present, the evidence for the use of hCG vs. GnRH shows advantages for hCG, and this review also shows that there is evidence that luteinizing hormone releasing hormone (LHRH) is more effective than placebo. But because this evidence is based on few trials, with small sample sizes and moderated risk of bias, this treatment cannot be recommended for everyone, and there is no evidence that supports hCG's use in larger doses and larger intervals. Results from this systematic review are important for developing better RCTs that may decrease the uncertainty of cryptorchidism treatment.

Abdome agudo no lactente e na criança / Acute abdomen in the child and newborn

O autor discorre sobre as principais causas de abdome agudo no lactente e na criança, tanto de natureza clínica como cirurgica, estudando em cada caso o quadro clínico como cirúrgica, estudando em cada caso o quadro clínico, diagnóstico e diagnóstico diferencial, complicaçöes e a conduta terapêutica, seja ela conservadora ou intervencionista.

Etiopatogenia das complicaçöes pós-operatórias na apendicite aguda em crianças / Etiopathogeny of postoperative complications in acute appendiciis in children

Este estudo foi baseado nos prontuários de 377 crianças internadas por apendicite aguda, de setembro de 1982 a novembro de 1992. As idades variaram de cinco meses a 13 anos, sendo a média de sete anos e seis meses. O objetivo do estudo, no qual foram comparados dois grupos de pacientes portadores de apendicite aguda com e sem complicaçoes pós-operatórias, foi verificar os possíveis fatores de efeito significativo na ocorrência de complicaçoes, diferenciando os dois grupos. Dentre as 322 crianças em cujas descriçoes cirúrgicas havia relatos sobre o estado macroscópico do apêndice, 183 (56,8 por cento) apresentavam perfuraçao do mesmo, com pus na cavidade abdominal. A mortalidade foi de 0,27 por cento e 55 (14,6 por cento) crianças evoluíram com complicaçoes pós-operatórias. Verificamos que o tempo de evoluçao da apendicite e o fato de haver pus na cavidade abdominal sao fatores prognósticos relevantes, com diferença significante entre os grupos (p < 0,01). Para as outras variáveis estudadas o teste do qui-quadrado nao mostrou diferença entre os grupos.

Modelo de mecanismo valvular anti-refluxo na esofagoplastia por tubo gástrico / Valvular anti-reflux mechanism model in gastric tube esophagoplasty

O refluxo gástrico é uma complicaçäo muito comum nas esofagoplastias por tubo gástrico. No entanto, apesar de sua freqüência e importância clínica, pouco se tem feito para solucionar este problema. No presente trabalho, os autores apresentam um estudo experimental em cadáveres de crianças, onde se criou um mecanismo valvular anti-refluxo pregueando-se a parede anterior do estômago sobre a base do tubo gástrico isoperistáltico. Através de infusäo líquida e medida de pressöes, verificou-se a criaçäo de um gradiente pressórico entre o tubo e o estômago remanescente. A presença do mecanismo valvular näo dificultou a mobilizaçäo do tubo à regiäo cervical pelo espaço retroesternal

Baço móvel na criança: relato de 4 casos / Wandering spleen in children: report of 4 cases

A ocorrência do baço distópico móvel (wandering spleen) é raramente referida na literatura cirúrgica pediátrica. Nos últimos três anos tratamos quatro pacientes portadores desta afecçäo, sendo que em um verificou-se quadro abdominal agudo secundário a torçäo do pedículo esplênico; o outro caso apresentou síndrome ictérica obstrutiva associada a tumoraçäo abdominal. Verificou-se que a síndrome ictérica era devida a um cisto de colédoco e a tumoraçäo abdominal um baço móvel. Em ambos os pacientes foi feito o diagnóstico pré-opeatório. A terceira paciente era portadora de osteogênese imperfecta, tendo sido submetida a cirurgia com diagnóstico de tumor de ovário com torçäo de seu pedículo, e a quarta paciente foi operada com o diagnóstico pré-operatório de massa abdominal a esclarecer. Os principais dados que podem conduzir ao diagnóstico desta entidade säo: presença de massa tumoral no abdome móvel em todas as direçöes e caracterizaçäo da ausência do baço em sua localizaçäo normal pela ultra-sonografia. Quanto ao tratamento, dada a importância imunológica do baço, especialmente em crianças, deve-se, se possível, conservá-lo fixando-se. É descrita a técnica da esplenopexia na loja esplênica, que foi empregada nos dois primeiros pacientes