Giant omphalocele: A novel approach for primary repair in the neonatal period using botulinum toxin.

INTRODUCTION: Giant omphalocele (GO) is a complex condition for which many surgical treatments have been developed; however, no consensus on its treatment has been reached. The benefits and efficacy of botulinum toxin A (BTA) in the repair of large abdominal wall defects in adults has been proven, and its reported use in children has recently grown. The goal of this study is to describe a novel technique for primary repair of GO using BTA during the neonatal period and report our initial experience. METHODS: patients were followed from August 2020 to July 2022. BTA was applied to the lateral abdominal wall in the first days of life followed by surgical repair of the abdominal defect. RESULTS: while awaiting surgery, patients had minimal manipulation, without requiring mechanical ventilation, were on full enteral feeding, and in contact with their parents. The midline was approximated without tension and without the need for additional techniques or the use of a prosthesis. Patients were discharged with repaired defects. CONCLUSION: this approach represents a middle ground between staged and the nonoperative delayed repairs. It does not require aggressive interventions early in life, allowing maintenance of mother-child bonding and discharge of the patient with a repaired defect without the need for additional techniques or the use of a prosthesis. We believe that this technique should be considered as a new possible asset when managing this complex condition.

A comparison between ultrasonography and single-phase computed tomography for preoperative assessment of solid abdominal tumors in children

Abstract Objective: This study aimed to estimate the performance of single-phase-enhanced computed tomography and ultrasonography examinations in the preoperative evaluation of solid abdominal tumors and their relationship with relevant adjacent structures in children. Methods: This retrospective study included 50 pediatric patients with malignant solid abdominal tumors treated with surgical resection between 2009-2017. Preoperative computed tomography and ultrasonography were compared to operative findings (gold standard) in the diagnosis of invasion or encasement of adjacent structures. Accuracy, sensitivity, specificity, and positive and negative predictive values were evaluated. Results: Renal (20.4%) and neuroblastic (19.4%) tumors were the most common. Complete surgical resection with negative margins was achieved in 44 (88%) patients. The comparison between single-phase-enhanced computed tomography and ultrasonography findings showed the following results: sensitivity = 90.3% vs 86.6%, specificity = 86.8% vs 94.6%, negative predictive value = 95.3% vs 94.4%, positive predictive value = 75.3% vs 86.9%, and accuracy = 87.9% vs 92.2%. The correlation (kappa index) between computed tomography and ultrasonography examinations was 0.72 (p < 0.001). In 14% (7/50) of the patients, the invasion of adjacent structures was diagnosed by ultrasonography but not by computed tomography (1 patient had 2 invaded structures).

A comparison between ultrasonography and single-phase computed tomography for preoperative assessment of solid abdominal tumors in children.

OBJECTIVE: This study aimed to estimate the performance of single-phase-enhanced computed tomography and ultrasonography examinations in the preoperative evaluation of solid abdominal tumors and their relationship with relevant adjacent structures in children. METHODS: This retrospective study included 50 pediatric patients with malignant solid abdominal tumors treated with surgical resection between 2009-2017. Preoperative computed tomography and ultrasonography were compared to operative findings (gold standard) in the diagnosis of invasion or encasement of adjacent structures. Accuracy, sensitivity, specificity, and positive and negative predictive values were evaluated. RESULTS: Renal (20.4%) and neuroblastic (19.4%) tumors were the most common. Complete surgical resection with negative margins was achieved in 44 (88%) patients. The comparison between single-phase-enhanced computed tomography and ultrasonography findings showed the following results: sensitivity = 90.3% vs 86.6%, specificity = 86.8% vs 94.6%, negative predictive value = 95.3% vs 94.4%, positive predictive value = 75.3% vs 86.9%, and accuracy = 87.9% vs 92.2%. The correlation (kappa index) between computed tomography and ultrasonography examinations was 0.72 (p < 0.001). In 14% (7/50) of the patients, the invasion of adjacent structures was diagnosed by ultrasonography but not by computed tomography (1 patient had 2 invaded structures). CONCLUSION: Ultrasonography can be considered a complementary method to single-phase-enhanced computed tomography in the preoperative evaluation of children with an abdominal tumor. The present study showed that ultrasonography and single-phase-enhanced computed tomography each possess a high accuracy in the preoperative planning of resection of solid abdominal tumors in children. Thus, it seems that the combination of both imaging methods would be enough for the evaluation of most abdominal tumors in the pediatric population.

Giant omphalocele: A novel approach for primary repair in the neonatal period using botulinum toxin / Onfalocele gigante: Uma nova abordagem para reparo primário no período neonatal utilizando toxina botulínica

ABSTRACT Introduction: Giant omphalocele (GO) is a complex condition for which many surgical treatments have been developed; however, no consensus on its treatment has been reached. The benefits and efficacy of botulinum toxin A (BTA) in the repair of large abdominal wall defects in adults has been proven, and its reported use in children has recently grown. The goal of this study is to describe a novel technique for primary repair of GO using BTA during the neonatal period and report our initial experience. Methods: patients were followed from August 2020 to July 2022. BTA was applied to the lateral abdominal wall in the first days of life followed by surgical repair of the abdominal defect. Results: while awaiting surgery, patients had minimal manipulation, without requiring mechanical ventilation, were on full enteral feeding, and in contact with their parents. The midline was approximated without tension and without the need for additional techniques or the use of a prosthesis. Patients were discharged with repaired defects. Conclusion: this approach represents a middle ground between staged and the nonoperative delayed repairs. It does not require aggressive interventions early in life, allowing maintenance of mother-child bonding and discharge of the patient with a repaired defect without the need for additional techniques or the use of a prosthesis. We believe that this technique should be considered as a new possible asset when managing this complex condition.

RESUMO Introdução: onfalocele gigante (OG) é uma condição complexa para a qual muitas alternativas terapêuticas foram desenvolvidas; no entanto, não há consenso sobre qual o melhor tratamento. Os benefícios e eficácia da toxina botulínica A (TBA) no reparo de grandes defeitos da parede abdominal em adultos foram comprovados, e o relato de uso em crianças cresceu recentemente. O objetivo deste estudo é descrever uma nova técnica para reparo primário de OG utilizando TBA durante o período neonatal e relatar nossa experiência inicial. Métodos: os pacientes foram acompanhados de agosto de 2020 a julho de 2022. A TBA foi aplicada na parede abdominal lateral nos primeiros dias de vida, seguida de correção cirúrgica do defeito abdominal. Resultados: enquanto aguardavam a cirurgia, os pacientes tiveram mínima manipulação, sem ser exigida ventilação mecânica, permaneceram em alimentação enteral plena e mantiveram contato com os pais. A linha média foi aproximada sem tensão e sem necessidade de técnicas adicionais ou uso de prótese. Os pacientes receberam alta com o defeito reparado. Conclusão: essa abordagem representa um meio-termo entre o reparo estagiado e o tratamento não-operatório. Não requer intervenções agressivas no início da vida, permitindo a manutenção do vínculo materno-infantil e a alta do paciente com defeito reparado sem a necessidade de uso de técnicas adicionais ou prótese. Acreditamos que esta técnica deva ser considerada como um novo possível recurso no manejo desta complexa condição.

Malignant triton tumor of the kidney in a child: A case report.

INTRODUCTION: Malignant triton tumor (MTT) is an extremely rare variant of the malignant peripheral nerve sheath tumors (MPNSTs) with rhabdomyosarcomatous differentiation, which was first described in 1932 by Mason. MTT affects, in most cases, patients under 35 years of age, and it is usually manifested as a mass that may or not be painful. However, the incidence in pediatric patients is atypical. This tumor presents an aggressive course and limited survival rate, and the prognosis is different between individuals with or without a concomitant diagnosis of neurofibromatosis type 1 (NF1). Currently, the recommended treatment is surgical resection, and adjuvant chemotherapy and radiotherapy, but its efficacy is not yet clear. PRESENTATION OF THE CASE: A 13-year-old female patient was referred to the pediatric oncology service due to the presence of an abdominal mass and weight loss, initially diagnosed with Wilms' tumor. After extensive investigation, surgical resection, and immunohistopathological evaluation, the diagnosis of malignant triton tumor was confirmed. The patient also underwent cycles of chemotherapy after resection, and is currently awaiting immunotherapy. DISCUSSION AND CONCLUSION: Malignant triton tumor is extremely rare and difficult to diagnose, especially in children or young people, age groups in which the incidence of the disease is even lower. This may be the reason it is rarely suspected, and it was a great challenge for the clinical care team. It is essential to consider and investigate this possibility of differential diagnosis, as patients diagnosed with this malignant tumor have a low survival rate and poor prognosis.

Vaginal ectopic ureter diagnosed by magnetic resonance imaging in a child with complete transverse vaginal septum and hydrocolpos

Ectopic ureter draining into the vagina is a rare cause of urinary incontinence. Some cases have been reported in association with malformations of the genitourinary tract, but very few published cases are associated with vaginal septum. Our article describes the case of a girl who presented with hydrocolpos shortly after birth and was found to have a complete transverse vaginal septum. Despite successful correction of this genital anomaly, the patient developed persistent dribbling of clear fluid from the genital area. Extensive testing revealed unilateral kidney dysplasia and probable genital ectopia of the corresponding ureter. Magnetic resonance imaging (MRI) showed insertion of the ureter into the upper third of the vagina. The patient underwent nephroureterectomy of the dysplastic kidney with subsequent improvement in urinary incontinence. Ureteral ectopia is difficult to diagnose with conventional imaging methods (plain radiography, ultrasound, and CT). MRI has proved to be an excellent method for assessment of genitourinary tract conditions, particularly when other diagnostic modalities have failed or are limited, due to the high resolution of MRI scans and the possibility of native image acquisition on all three orthogonal planes. MRI is the best diagnostic modality for visualization of the course and insertion of ectopic ureters, and enables precise surgical correction.

Tumor de Wilms: características clínicas e cirúrgicas / Wilms'tumor: clinical and surgical features

O tumor de Wilms (TW) é o tumor renal maligno mais comum na infância. O conhecimento de certas características clínicas e a realização de procedimentos cirúrgicos adequados podem ter impacto no prognóstico desta doença. Revisados prontuários de pacientes com diagnóstico de TW entre 1989 e 2005. Coletados dados demogrãficos, caracteríisticas clínicas e avaliação de procedimentos cirúrgicos. Durante o ato operatório, 38 pacientes foram submetidos à avaliação do rim contralateral através da palpação e em 13 relatos de cirurgia não foram encontradas descrições. Ruptura tumoral em 1 paciente; em dez prontuários havia registro de ausência de ruptura tumoral e em 41 prontuários não havia qualquer menção quanto à presença ou ausência dessa complicação. A histopatologia confirmou 45 casos de histologia favorável e os demais de histologia desfavorável. Os resultados apresentados permitem concluir que os pacientes estudados apresentam características demográficas gerais semelhantes aos da literatura.Considerando-se que em um número expressivo de pacientes observou-se falta de aderência a certas etapas do procedimento cirúrgico, incluindo ausência de biópsia de linfonodos e atrasos na realização da ressecção tumoral, os autores recomendam que o cirurgião pediátrico tenha uma participação mais efetiva na equipe multidisciplinar e na elaboração das rotinas do protocolo cirúrgico para pacientes com TW

Wilms´tumor (WT) is the most common malignant renal tumor in childhood. The knowledge of the clinical characteristics and the accomplishment of standard surgical procedures may have an impact in the prognosis of this disease. Medical records of newly diagnosed WT patients treated from 1989 to 2005 were reviewed. We collected data on demographics, clinical characteristics and whether certain recommended surgical standard procedures were carried out.The surgeon in 38 patients performed palpation of the contralateral kidney and in 13 medical records there was no report whether this procedure was carried out. Tumor spillage was reported 1 patient, reported as absent in 10 patients; we were unable to find any mention about tumor rupture on the surgeon’s report for 41 patients. There were 45 cases of favorable histology and 7 of unfavorable histology. The OS was of 69%, 71%, 79%, 50% and 40% for the stages I, II, III, IV and V, respectively. Five years OS was 73% and 65,2% for patients submitted to surgery before and after the 6th week after diagnosis, respectively. The results of the present study indicate that patients in this study show demographic characters similar to the literature. Considering that surgeons did not performed standard recommended surgical procedure such as lymph nodes biopsy and carried out late surgical resection of the primary tumor in many patients, there is a need for a more effective participation of the surgeon in the multidisciplinary team and possiblyin the designing protocols for the surgical management of patients with TW

[Anular pancreas--pancreatic resection or duodenal by-pass]. / Pâncreas anular--ressecção pancreática ou derivação duodenal.

PURPOSE: To present the authors experience managing anular pancreas and to compare the results found by the authors with the ones found in the literature. METHODS: A retrospective review of four patients treated at the Departamento de Cirurgia da Faculdade de Medicina da Universidade Federal do Rio Grande do Sul with anular pancreas. The disease was present in three different ages: one in the neonatal age, one in childhood and two in the adulthood. Gastroduodenal obstructive symptoms were the most common. Abdominal pain was present in adult patients. The diagnostic investigation began with radiological studies such as upper gastrointestinal barium series, upper endoscopy and abdominal computed tomographic scan, although all the diagnoses required surgery for confirmation. The duodenoduodenostomy was the treatment of choice in the pediatric patients, and division of the anulus was carried out in the adults. RESULTS: All patients had symptomatic relief and postoperative recovery. The hospital stay ranged from 9 to 12 days (median 10.5 days). There were no postoperative complications. All patients remain asymptomatic up to now. CONCLUSION: The rare condition of anular pancreas does not allow a more detailed and comparative study. The results of the authors showed that both gastrointestinal by-pass and division of the pancreas are effective and safe treatments.

Pâncreas anular: ressecção pancreática ou derivação duodenal / Anular pancreas: pancreatic resection or duodenal by-pass

OBJETIVOS: Apresentar a experiência dos autores no tratamento do pâncreas anular, comparando os dados com os da literatura. MÉTODOS: É relatada a experiência do Departamento de Cirurgia Geral do Hospital de Clínicas de Porto Alegre em pâncreas anular com a descrição de quatro pacientes. A doença manifestou-se em três faixas etárias distintas: uma no período neonatal, outra na infância e duas na fase adulta. O quadro clínico de obstrução intestinal alta esteve presente em três dos quatro pacientes. Dor abdominal foi queixa constante entre os adultos. A investigação diagnóstica foi realizada através do estudo contrastado do estômago e duodeno, da endoscopia digestiva e da tomografia abdominal. A confirmação diagnóstica somente foi possível durante a laparotomia. A derivação duodenal foi a técnica cirúrgica empregada em dois casos e a ressecção do tecido pancreático nos pacientes adultos. RESULTADOS: Os quatros pacientes evoluíram favoravelmente no período pós-operatório. O tempo de internação hospitalar variou de 9 a 12 dias, com média de 10,5 dias. Não houve complicação cirúrgica e todos os pacientes permanecem assintomáticos em acompanhamento ambulatorial. CONCLUSAO: A raridade desta anomalia não permite um estudo mais detalhado e comparativo. Os resultados aqui apresentados como os encontrados na literatura demonstram que a derivação duodenal e a ressecção pancreática são alternativas eficazes e seguras quando bem indicadas.