Long-segment thoracoabdominal aortic coarctation in a child with Down syndrome.

Midaortic syndrome is a rare vascular anomaly characterized by coarctation of the descending thoracic and abdominal aorta. Down syndrome is associated with multiple congenital cardiac malformations but is rarely associated with developmental vascular anomalies. Midaortic syndrome may result in severe renovascular hypertension that requires early intervention to prevent life-threatening complications. We report a child with Down syndrome who presented with occlusion of the aorta and was treated with aortic bypass. More than 4 years after the procedure, the patient's renal function remains normal, and there is no evidence of recurrent hypertension. Long-term follow-up is important to assess the benefits of surgical repair.

Vascular anastomosis for paediatric renal transplantation and new strategy in low-weight children.

The technical aspects of RT in low-weight children should be specific, particularly with regard to VA. This retrospective study assesses the main VA options in paediatric RTs and proposes a new strategy for renal artery trajectory when using the Ao and the right iVC. The sample included 81 patients and was categorized into a group of children weighing <16 kg and the other group of children weighing 16 kg or more. The smaller children received the graft predominantly on the Ao and iVC (63%); however, the VA options varied in children weighing more than 16 kg, with anastomoses predominantly to the common iliac vessels (46%). In the first group, when the Ao was the selected vessel for anastomosis on the right side, the trajectory adopted for the transplanted kidney artery was posterior to the iVC. This strategy may reduce the risk of compression of the iVC by the renal artery of the donor kidney and may reconstitute the normal anatomy of the renal artery. Moreover, it did not represent a risk factor for graft loss in this sample.

Urethral duplication--a wide spectrum of anomalies.

OBJECTIVE: Urethral duplications are rare lower urinary tract anomalies, with multiple anatomical variants described. This paper aims to separate this complex anomaly into different diseases, each with distinct clinical forms according to the disturbance during embryogenesis, yet noting a few similarities that may be helpful in their management. The classification system of urethral duplication is also discussed. MATERIAL AND METHODS: Twelve urethral duplication cases over a 14-year period were reviewed. Clinical presentation, the imaging studies used to ascertain anatomical details, type of urethral duplication and surgical correction used in the treatment of patients are presented. RESULTS: Nine patients had urethral duplication in the sagittal plane and three patients in the coronal plane. Of the patients with sagittal urethral duplication, 3 had pre pubic sinus, 3 had epispadiac urethral duplication, 1 had a dorsal urethral duplication deviated from the midline and 2 had hypospadiac urethral duplication. All the patients with coronal urethral duplication had associated bladder duplication. The surgical correction of the patients with sagittal urethral duplication included excision of the pre pubic sinus, excision of the duplicated urethra, and urethroplasty. Excision of the hemibladders' septum and closure of one bladder neck was the treatment for patients with coronal urethral duplication and bladder duplication. CONCLUSION: Urethral duplication is a complex anomaly and the different manifestations probably have different embryological origins. Each group, sagittal or coronal, has a few similarities that may be helpful in their management, although every diagnosed case presents a unique anatomy and surgical treatment must be individualized.

Pelvic fracture urethral distraction defects in children managed by anterior sagittal trans anorectal approach: a facilitating and safe access.

PURPOSE: Trauma injuries of the posterior urethra resulting from pelvic fracture in children tend to be complete ruptures, with upper dislocation of the prostate. This paper aims to show our experience in using an anterior sagittal transanorectal approach (ASTRA) in the treatment of such injuries. MATERIALS AND METHODS: The medical records of 11 patients with pelvic fracture urethral distraction defects who had undergone anastomotic urethroplasty through ASTRA between 1997 and 2009 were reviewed. Ages ranged from 1 year and 6 months to 23 years (mean age 11 years). Of the 11 patients, 8 had previously undergone failed urethroplasties. RESULTS: In 10 patients it was possible to perform tension free urethroplasty. One patient required inferior pubectomy and separation of the corpora cavernosa. Patients' follow-up time varied from 10 months to 10 years and 9 months (mean 41 months). One patient had a urethral fistula and evolved with a urethral diverticulum successfully managed by diverticulectomy. One patient presented a urethral stenosis managed by urethral dilatation. Of the 11 patients, 9 presented functional urethral flow and are continent. Two patients had no urethral flow. One is undergoing bladder catheterization through the Mitrofanoff principle and the other one through the urethra. No patient presented fecal incontinence or rectourethral fistula. CONCLUSION: This access, which is increasingly being used to approach posterior urethral diseases, has proved to be safe and effective in the treatment of pelvic fracture urethral distraction defects.

Tratamento endoscópico do refluxo vesicoureteral após a cura da instabilidade vesical / Endoscopic treatment of vesico-ureteral reflux following cure of vesical instability

Objetivo: Avaliar a eficácia do uso de "dextranomer hyaluronic acid copopymer (Deflux®)" no tratamento de pacientes com disfunção miccional e refluxo vesicoureteral (RVU) persistente após a cura da disfunção miccional. Pacientes e Métodos: Dos pacientes portadores de disfunção miccional e RVU, 30 foram submetidos ao tratamento do refluxo persistente com Deflux®, após a cura da disfunção miccional. Dos pacientes, 22 eram do sexo feminino e oito do sexo masculino, com idade variando de um ano e dez meses a 16 anos. De um total de 46 unidades ureterovesicais refluxivas, nenhuma apresentava RVU grau I, quatro RVU grau II, 38 apresentavam RVU grau III e quatro apresentavam RVU grau IV. O período de acompanhamento variou de seis meses a cinco anos. Quando o RVU persistiu, apesar do tratamento, uma segunda e, eventualmente, uma terceira injeção foi programada, utilizando a mesma técnica descrita. Resultados: Em todas as quatro unidades ureterais apresentando RVU grau II o refluxo desapareceu após uma simples injeção de Deflux®. Das 38 unidades ureterais apresentando RVU grau III, em 30 o refluxo desapareceu com apenas uma aplicação, em duas unidades após duas aplicações e em uma unidade desapareceu somente após a terceira aplicação. Das quatro unidades que apresentavam refluxo grau IV, somente em uma unidade o RVU desapareceu. Conclusão: O tratamento com Deflux® foi efetivo em mais de 80% dos casos em que existia um RVU grau II ou III, mas foi capaz de tratar o RVU em somente em uma das quatro unidades com RVU grau IV. Uma segunda injeção endoscópica pode eventualmente ser eficaz nos casos de falha inicial do tratamento.