Gastrinoma in childhood: case report and update on diagnosis and treatment.

We report a case of gastrinoma in a child and review the literature on gastrinoma in childhood, particularly with regard to the clinical, laboratory, radiological and scintigraphic characteristics, therapeutic approaches and prognosis. An eleven-year-old boy with gastrinoma underwent surgical resection. One year later resection of the left lateral lobe of the liver was performed due to a liver metastasis. The boy has been followed up for four years since this last procedure. The outcome was satisfactory for one year after the primary resection and then for a further four years after resection of a liver metastasis. Based on our data and that of the literature, it is our opinion that gastrinoma should be considered as a potentially malignant disease. The best approach is an early diagnosis and surgical treatment through resection of the primary tumor, followed by periodic postoperative clinical, laboratory, radiological and scintigraphic evaluations, because of the risk of metastatic disease. Such metastases must be resected as soon as possible.

Ultrasound evaluations of internal jugular vein punction techniques in children: the easiest method to reach the target area.

The objective of this study is to determine the best way to access and the position in which the patients must remain in order to obtain the best transversal section of the right internal jugular vein (RIJV) section during the catheterization by ultrasound, allowing a safer and precise access. The three possible ways to access the RIJV, anterior, lateral and posterior, from 57 healthy children, were examined by ultrasound in one similar sequence of positions: horizontal dorsal decubitus with the head centered in neutral position with and without the use of a pillow; horizontal dorsal decubitus with contralateral rotation of the head with and without the use of a pillow; horizontal dorsal decubitus with the head centered in neutral position and the patient in the Trendelenburg position without the use of a pillow. The relation between the different positions and punction regions in RIJV were established using analysis of variance. As a result, the lateral punction with the patient in the Trendelemburg position offered a largest area of the RIJV transversal section in comparison to all the other options (P<0.0001). In conclusion, this study demonstrated that the safer and precise way for the RIJV catheterization in pediatric patients is obtained in Trendelenburg position with lateral access and without a pillow.

Management of Wilms' tumor with intracardiac extension.

We review our experience and the literature in treating 4 patients with Wilms' tumor (WT) with intracardiac extension among 92 patients with this neoplasm. Cardiopulmonary bypass with circulatory arrest and profound hypothermia was used. There were 3 boys (3 years, 4 years 5 months, and 15 years) and 1 girl (6 years). The follow-up periods were 8 months, 3 years, 2 years 6 months, and 15 years, respectively. We had no surgical complications and conclude that the preoperative diagnosis is extremely important. These patients must be transferred to institutions where concomitant cardiac procedures can be performed. In treating patients with WT, Doppler ultrasound must be used preoperatively in all cases, not only those in which clinical and radiologic signs of intravascular involvement are found. We propose that preoperative chemotherapy should be used, as it shrinks the thrombus and causes desirable adherence of the thrombus to the venous wall, reducing the probability of thromboembolism during the surgical procedure. We also find this method safer than in our 1st case, where neither cardiac arrest nor hypothermia was used. Our results agree with the literature that intracardiac extension of WT does not worsen its prognosis when a rational surgical approach is used.

Avaliaçäo do papel do cirurgiäo no tratamento do tumor de Wilms: análise de um estudo cooperativo / The role of surgery in the treatment of Wilms tumor: analysis of cooperative study

Objetivo. Detectar os efeitos do tratamento e estadiamento cirúrgicos nos resultados obtidos e verificar a possível relação entre tais resultados e o cumprimento das normas do protocolo cirúrgico preestabelecido (segundo as normas do NWTS). Casuística e Método. A casuística é composta de 166 pacientes de 1986 e dezembro de 1988, com dados atualizados até fevereiro de 1992. O período mínimo de acompanhamento foi de 24 meses para 147 pacientes e de três a 18 meses para 19 pacientes, sendo o período médio de acompanhamento de 36 meses. A todas as instituições que se integraram ao estudo cooperativo do GCBTTW foram fornecidos os protocolos, visando à uniformização dos procedimentos clínicos e cirúrgicos. Resultados. Após submeter os dados obtidos à análise estatística, verificamos que: 1) Não existe interferência da ligadura prévia do pedículo nos resultados (recidivas e mortalidade); 2) A ruptura tumoral intra-operatória com contaminação da cavidade peritoneal interfere desfavoravelmente no índice de mortalidade. 3) Existe discordância significante entre a opinião do cirurgião e a análise histopatológica quanto à invasão da adrenal e da gordura peri-renal. 4) Os tumores com peso superior a 500 g têm pior prognóstico. 5) O desempenho dos cirurgiões foi considerado inadequado em relação ao inventário ganglionar (56,6 por cento dos pacientes não tiveram gânglios analisados), o que pode ter contribuído para: menor índice de recidivas nos pacientes com gânglios acometidos; maior incidência de metástases pulmonares nos pacientes sem gânglios analisados; pacientes no Estádio II com melhor prognóstico em relação ao Estádio I, e pacientes em estádios I e III com índices de recidivas semelhantes.

MYCN gene amplification. Identification of cell populations containing double minutes and homogeneously staining regions in neuroblastoma tumors.

Neuroblastoma is the second most common solid tumor occurring in children. Amplification of the MYCN oncogene is associated with poor prognosis. To identify neuroblastoma tumors with MYCN amplification, we studied the number of copies of MYCN in interphase cells by fluorescence in situ hybridization in 20 neuroblastoma patients. MYCN amplification appeared in 7 tumor specimens. Interphase and metaphase studies showed a tumor cell population with both forms of amplification, double minutes and homogeneously staining regions, in two patients. These patients showed a smaller tumor cell subpopulation with the presence of more than one homogeneously staining region, suggesting that gene amplification was undergoing karyotype evolution.

[The role of surgery in the treatment of Wilms tumor: analysis of cooperative study]. / Avaliação do papel do cirurgião no tratamento do tumor de Wilms: análise de um estudo cooperativo.

OBJECTIVES: To detect the effects of the surgical treatment and staging on the obtained results and to check the possible relationship between these results and the compliance, or not, with the preset surgical approach protocol (in accordance with the National Wilms Tumor Study--NWTS). MATERIAL AN METHOD: One hundred and sixty six operated on patients entered between October 1986 and December 1988, with the data updated until February 1992 were studied. The minimum follow-up period was 24 months for 147 patients (average 36 months). The remaining 19 patients were followed in the outpatient clinic for three to 18 months. RESULTS: After submitting these data to statistical analysis and the obtained results compared to those in the literature we observed that: previous ligature of the renal vessels had no discernible effect on the ultimate outcome (relapses and mortality); intra-operative tumoral rupture with contamination of the peritoneal cavity unfavorably interferes with the mortality rate; even when the adrenal and the perirenal fat are normal from the surgeon's point of view, the histological findings showed tumor contamination in a number of cases; there is a worse prognosis when the tumor weight exceeds 500 g; according to the lympho-node evaluation, surgeon's performance was not in accordance with the protocol recommendations, considering that the node evaluation was neglected in 56.6% of the patients. This led to a surgical staging error and has contributed to some unexpected results like: lower relapses rate in the patients with affected lymph nodes; higher incidence of lung metastases in patients without lymph nodes analysis; stage II patients with better prognosis, compared to stage I patients and patients with stage I and III having similar relapse rates. CONCLUSION: From the above-referred results, we concluded that some omissions have happened jeopardizing accuracy of the surgical staging in a significant way. In this way, in our environment the oncologist in charge of the treatment of Wilms tumor bearers, must establish a close integration with a surgical team, whose members have a precise understanding of the surgeon's importance and his fundamental role, not only of the surgical treatment itself, but who are aware on the minimal details on the surgical staging of this neoplasm, and of its importance in relation to the multidisplinary approach.

Esophageal carcinoma secondary to a chemical injury in a child.

Twin sisters were clinically and endoscopically followed due to chemical injuries to the esophagus after ingestion of muriatic acid at 10 months of age. One of the girls developed esophageal carcinoma 10 years later and died after esophagectomy because of progression of the disease. Her twin sister has a severe stenosis at the distal esophagus and is waiting for surgical treatment. Malignization of a chemical injury to the esophagus in a child has not yet been described in the literature, emphasizing the role of endoscopic follow-up with periodic biopsies.

Gastric-tube esophagoplasty in children.

From 1979 to 1996, 19 patients underwent gastric-tube esophagoplasty. There were 10 boys and 9 girls, aged between 1 year 4 months and 4 years 11 months at the time of surgery. In 1 patient the esophagoplasty was performed due to a long stenosis secondary to reflux; 3 others had caustic stenoses; and the remaining patients had esophageal atresia. In 17 cases an isoperistaltic gastric tube was brought up to the neck through the retrosternal space; in 2 an anisoperistaltic gastric tube was used, cervical mobilization being via the posterior mediastinum without a thoracotomy in 1 case and by the left transpleural route in the other. The cervical anastomosis was carried out in one surgical stage in 12 patients and in two stages in 7. In 1 patient the tube was resected due to necrosis of its proximal third; the child later underwent an esophagocoloplasty. Necrosis of the colic graft, mediastinitis, and septicemia occurred, leading to the only death in the series. There were 12 fistulas of the cervical anastomosis (63.3%) and 8 stenoses (42.1%). All fistulas, with 1 exception, closed spontaneously after 8 days to 2 months, and all stenoses were treated by endoscopic dilatation. Another patient developed a fistula of the gastric tube with chronic evolution to a stenosis of the distal third of the tube and communication with the right lower pulmonary lobe. A lobectomy and closure of the fistula were necessary. All patients were followed for a period of 1 to 16 years. At present, all of them swallow solid food normally. The evolution of the nutritional status was normal (eutrophic) in 14 of the 18 patients (77.7%) who survived the operation; 4 showed variable degrees of malnutrition. In 2 of these 4 cases the malnutrition was due to poor socioeconomic conditions, but was not related to the surgery. Redundancy, a problem associated with esophagocoloplasty, was not observed in any of the gastric tubes, which was attributed to the thickness of the gastric wall. The authors prefer the use of an isoperistaltic gastric tube (with proximal base) for esophageal replacement in children and recommend that the operation should be carried out when the child is able to swallow solid foods and walk. As in any other major surgical procedure, a good nutritional state is essential prior to operation.

Apendicitis aguda en los tres primeros años de vida / Acute appendicitis in infants

Entre enero de 1983 y diciembre de 1993, se operaron veinticuatro niños menores de 36 meses, entre 402 casos de apendicitis aguda. Fueron estudiados en cuanto a sexo, color, procedencia, tiempo de evolución, diagnóstico preoperatorio, exámen físico al ingreso, técnica y hallazgos intraoperatorios, exámen anatomopatológico y evolución postoperatoria.Hubo predominio del sexo femenino y color blanco.La edad de mayor incidencia fue de 2 años (12/24).La media de intervalo de tiempo entre el inicio de los síntomas y el diagnóstico fue de 4 días (M=4).El diagnóstico pre-operatorio de apendicitis aguda fue hecho en 9 niños , en 12 de abdomen agudo inflamatorio y en 3 de abdomen agudo inflamatorio y en 3 de abdomen agudo obstructivo.En el examen físico, 6 pacientes (25 por ciento)presentaron un plastrón o masa abdominal palpable en la fosa ilíaca derecha.El apéndice se encontraba perforado en más del 50 por ciento de los casos (13/24).La técnica quirúrgica más utilizada fue la de sutura en bolsa de Ochsner (14/24) en relación a la de Parker-Kerr (10/24).En la evolución postoperatoria hubo complicaciones asociadas en 50 por ciento de los casos,distribuidas en :7 abcesos y 2 dehiscencia de pared abdominal, 1 abceso pélvico y otro subfrénico, 1 caso de bronconeumonía bilateral y uno con oclusión por bridas y síndrome de Mallory-Weiss.Falleció un niño de 9 meses en el 4§ día del postoperatorio a causa de peritonitis aguda purulenta, abseco subfrénico izquierdo y neumonía instersticial asociada a hidrotórax bilateral comprobado por autopsia.Se recomienda un diagnóstico precoz y tratamiento quirúrgico inmediato. La precisión depende de la habilidad y experiencia del examinador más que los exámenes complementarios

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

The occurrence of a wandering spleen is rarely referred to in the pediatric surgical literature. In the last three years we treated four patients with this anomaly. The first case had an acute abdominal problem associated to a wandering mass with spleen characteristics. The ultrasonography has confirmed the absence of spleen from its proper place. In the second case it was detected a mass with the same features of the first patient associated with choledochal cyst. In this case the ultrasound has demonstrated that spleen was the referred mass. In the third case a patient was submitted to a surgery with the preoperative diagnosis of ovarian torsion. The patient had an associated osteogenesis imperfecta. During the operation it was found that spleen was the referred mass. In the fourth patient the preoperative diagnosis was "abdominal mass". It is given an emphasis to the preservation of the spleen in wandering spleen, mainly in childhood, due to its immunological importance.

Acute thrombosis of Sorin tilting disc mitral prostheses.

We observed four cases of prosthetic thrombosis in 77 patients who had had a tilting disc valve (Sorin model) inserted in the mitral position. In all cases, the clinical findings on admission consisted of congestive heart failure, absence of prosthetic closing click and mitral regurgitation murmur. M-mode and cross-sectional echocardiography were invariably diagnostic. Surgery was performed without delay and consisted of thrombectomy in one case and valve replacement with porcine xenograft in three. Examination of the explanted prostheses disclosed fibrous tissue overgrowth surrounding the valvular ring, as well as thrombotic vegetations on the ventricular and atrial sides. The disc appeared blocked in a semiopening position at an angle of 45 degrees. Our experience suggests that the thrombosis is secondary to disc malfunction itself produced by periannular tissue growth. It is related to the porosity of the fabric which surrounds the metal ring, the valve design and its orientation. Correct anticoagulation therapy, performed in all cases, had no significant effect on tissue overgrowth. The high postoperative mortality rate depended on the severity of the preoperative clinical condition of the patients.

Caroli's disease in childhood: report of two new cases.

Caroli's disease is a rare congenital disorder characterized by segmentary saccular dilatation of the intrahepatic bile ducts, which manifests itself clinically by cholangitis and bile stones. We report two cases of Caroli's disease in schoolgirls with clinical signs of recurrent abdominal pain associated with intermittent crises of cholestatic jaundice. Diagnosis was made by retrograde cholangiography in one patient and by intravenous cholangiography in the other.

Doenca de Caroli. Causa de dor abdominal recorrente na infancia. / Caroli's disease: cause of recurrent abdominal pain in children

Doenca de Caroli e uma enfermidade congenita rara caracterizada por dilatacao sacular segmentar dos ductos biliares intra-hepaticos e que se manifesta clinicamente com colangite e litiase biliar.Descrevemos dois casos de doenca de Caroli em criancas de idade escolar com quadro clinico de dor abdominal recurrente associada a crisis intermitentes de ictericia colestatica. O diagnostico foi caracterizado por colangiografia retrograda em uma e na outra por colangiograma intravenosa

Invaginacao colo-colica cronica. Apresentacao de um caso. / Chronic colonic intussusception. Report of a case

Os autores apresentam um caso de invaginacao colo-colica-cronica (30 dias), devido a um polipo juvenil do colo descendente. Tecem comentarios sobre a incidencia, quadro clinico, radiologico e tratamento desta patologia