RESUMO
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.
RESUMO
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.