Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights.

BACKGROUND: About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. PATIENTS AND METHODS: A retrospective study of pediatric Wilms tumors treated at three sites (January 1999-June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. RESULTS: Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd-Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5-20 years), overall survival was 89% and event-free survival was 78%. CONCLUSIONS: Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

Persistent omphalomesenteric duct causing small bowel obstruction in children.

BACKGROUND: Small bowel obstruction is a common surgical emergency and a frequently encountered problem in abdominal surgery, but persistent omphalomesenteric duct as a cause of this condition is an exceptional finding. AIM: To report through this observation an omphalomesenteric duct causing small bowel obstruction in children. REPORT: A 10-years-old male patient without medical history, and specially without umbilical secretion or umbilicoileal fistula, presented with colicky abdominal pain, vomiting, absence of passage of gas and feces, and abdominal distension of 24 hours duration. Physical examination and blood tests were normal. Abdominal X-ray showed small bowel obstruction. In exploratory laparotomy, persistent omphalomesenteric duct (10cm) causing volvulus of small bowel was identified and resected. The patient had an uneventful recovery and was discharged on the 5th postoperative day. CONCLUSION: Although persistent omphalomesenteric duct is an extremely infrequent cause of small bowel obstruction in children patients. The practitioner has to think of this etiology in front of every patient without surgical histories presenting an occlusive syndrome to avoid the complications: occlusion and hemorrhage.

Management of perineal ectopic testes.

BACKGROUND: Perineal ectopic testis (PET) is a rare congenital anomaly in which the testis is abnormally situated between the penoscrotal raphe and the genitofemoral fold. AIM: we report six patients treated for PET. The epidemiological, clinical, radiological and therapeutic aspects of this rare entity are discussed in light of data of the literature. RESULTS: Between 2000 and 2009, six patients (0, 2%) treated for PET were diagnosed among 2156 patients operated upon for undescended testes in unity of paediatric surgery in Tunis children's hospital. The mean age was 21+/- 25 months. The abnormality was associated with an inguinal hernia in two cases. The diagnosis was based on the presence of an empty scrotum or perineal swelling. In all, orchidopexy in a dartos pouch was easily performed through an inguinal skin crease incision.The length of the testicular vessels and vas deferens was adequate with a favourable course in every case. Although the complications of undescended testes are the same as for PET, the timing of surgery should be different. CONCLUSION: It is generally accepted that children must not be below 6 months of age for surgical correction of undescended testes, but there is no need to delay surgery in PET, which can easily be diagnosed by physical examination in the neonatal period. Surgery is indicated even if there is no hernia present. The functional prognosis, always difficult to define, appears to be identical to that of other sites.

Indications for nephrectomy in children: what has changed?

BACKGROUND: The last decade has witnessed significant refinements in preoperative diagnostic evaluation and an improvement in surgical techniques and postoperative management for paediatric patients. There has been an improvement in our understanding of the natural history of some congenital renal anomalies which has caused some changes in management approach. AIM: To review the indications for nephrectomy in children between 1996 and 2008, at the departement of paediatric surgery, children's hospital in Tunis. METHODS: There were 80 nephrectomies. A retrospective review of the patients' notes was performed. The 13-year period was divided into two halves (1996-2000 and 2001-2008) which were then compared. RESULTS: The total number of nephrectomies per year significantly increased over the period of the study (4, 6 and 8 nephrectomies per year for 1996-2000 and 2001-2008, respectively; P < 0.05), as did the number of nephrectomies for Multicystic dysplastic kidney (MCDK) (zero and 5 for 1996-2000 and 2001-2008, respectively) and wilms'tumour (8.3% and 29,16% for 1996 - 2000 and 2001 - 2008, respectively). Wilms' tumour, vesico-ureteric reflux (VUR) and pelvi-ureteric junction (PUJ) obstruction accounted for more than half of the nephrectomies (80% and 58% for 1996-2000 and 2001-2008, respectively). The proportion of nephrectomies performed for VUR did not change (15% and 12% for 1996-2000 and 2001-2008, respectively) but fewer nephrectomies were performed for pelvi-ureteric junction (PUJ) obstruction in the second half of the study period (44% and 4,16% for 1996-2000 and 2001-2008, respectively ; P < 0.05). CONCLUSION: The total number of nephrectomies, including partial nephrectomies, has increased significantly. The decrease in nephrectomies for PUJ obstruction could be accounted for by a more aggressive approach in the management and follow up of prenatally diagnosed hydronephrosis. Of note is that there was no significant change in the proportion of nephrectomies performed for VUR. On the contrary, the proportion of nephrectomies increased for neoplastic lesions and MCDK.