Crouching carcinoma, hidden ureter: A blind ending burnt out cancerous moiety

Background: A 76-year-old male presenting with macroscopic haematuria was found to have a lobulated mass infiltrating along the urothelium at the site of insertion of the upper moiety of a complete duplex right kidney. Suspected of being upper tract urothelial carcinoma, cystoscopy, bilateral retrograde pyelograms and transurethral resection of bladder tumour were attempted. Intra-operative findings revealed no tumour burden in the bladder or left ureter. The insertion of the upper pole moiety of the right ureter was not identified intra-operatively. Pelvic MRI demonstrated a markedly dilated upper pole moiety of the right ureter with a soft tissue mass in its distal aspect. Interestingly, the distal portion of the ectopic upper pole moiety was found to insert into the bladder neck. Objective and Methods: We report on an unusual case of upper tract urothelial carcinoma arising from the upper moiety of a complete duplex kidney. Our aim was to demonstrate the importance of thorough investigation of suspected urothelial carcinomas occurring in association with variant upper tract anatomy. Results and Conclusion: This case demonstrates the importance of thorough radiological and endourological investigation of suspected upper tract urothelial carcinoma and the various congenital abnormalities that may complicate the surgical management of this common malignancy. Level of evidence: 4 (case report)

Congenital ureterovaginal fistula: a rare case of single-system ectopic ureter with ipsilateral ectopic kidney managed by vaginal approach: a case report

Background Diagnosing urinary incontinence from organic causes such as ectopic ureter is particularly important because of the potential for cure by surgical correction. The prevalence of ectopic ureter is uncertain because many are asymptomatic and the diagnosis is usually overlooked. Eighty percent of ectopic ureters in females are often associated with duplex kidney. However, an ectopic ureter draining a single-system ectopic dysplastic/atrophic but functioning kidney is rare, especially in females. The overall long-term continence rate after successful correction of ectopic ureter is satisfactory. Case presentation This case is reported to highlight a rare situation, where a 22-year-old nulligravid Ethiopian women presented with a complaint of continuous wetting of her underwear since childhood, but she had normal voiding pattern. Localized right pelvic kidney ultrasound and computed tomography scan with contrast revealed right ectopic ureter and atrophied ipsilateral pelvic kidney with good function. Surgical reimplantation through vaginal approach was performed, and the outcome was good. The patient’s subsequent follow-ups were uneventful. Conclusion An extramural vaginal ectopic ureter is better accessed through transvaginal approach than abdominal, especially when it is associated with pelvic ectopic kidney. This modified approach is less invasive and has lower morbidity and better success rate than a transabdominal approach.

Duplex kidney complicated with preoperative inferior nephroblastoma rupture in children: a case report and literature review

Background We admitted a child with a duplex kidney combined with preoperative rupture of nephroblastoma and used this case to discuss the clinical features and treatment of this disease. Case presentation We retrospectively analyzed the clinical data of a 5-year-old girl with preoperative duplex kidney rupture combined with inferior nephroblastoma who was admitted to the Fourth Hospital of Baotou. In addition, we reviewed the relevant literature. The patient’s details were as follows: weight, 17 kg; height, 108 cm; and body surface area, 0.7 m2. Abdominal ultrasound for abdominal pain revealed the presence of a left-sided renal mass; enhanced abdominal computed tomography further confirmed it to be a left-sided duplex kidney measuring approximately 6 × 5 × 5 cm, with a rupture originating from the lower kidney. The PubMed database was searched from 2010 to 2020 for the terms “Wilms’ tumor” and “Duplex” and “Wilms’ tumor” and “Rupture.” The treatment plan was preoperative chemotherapy (vincristine/dactinomycin, VA regimen) + left kidney tumor radical surgery + postoperative chemotherapy (actinomycin-D/VCR/doxorubicin, AVD regimen). Postoperative pathology revealed an International Society of Pediatric Oncology intermediate-risk stage-3 nephroblastoma (mixed type) in the left kidney. Literature review was performed with 71 cases meeting the set criteria with an aim to analyze and summarize the clinical characteristics and treatment of patients with ruptured nephroblastoma and duplex kidney combined with nephroblastoma. Conclusions To our knowledge, no previous studies have reported preoperative duplex kidney combined with nephroblastoma rupture. In patients with this condition, preoperative chemotherapy is recommended when the vital signs are stable and tumor resection can be performed after the tumor has shrunk to prevent secondary spread. If the patient’s vital signs are unstable, emergency exploratory surgery is needed. If the nephroblastoma rupture is old and limited, surgery can be performed when the tumor size is small.

1491 Hemi-Nephroureterectomy For Duplex Kidney in Children - The Resulting Effect on Renal Function

Introduction Urinary duplication systems occur in approximately 1% of the population, and may present with recurrent UTIs, incontinence, or be incidentally detected on imaging. DMSA (dimercaptosuccinic acid) imaging is used in these patients to assess split renal function. If found significantly reduced in a single moiety, children may be offered hemi-nephroureterectomy (HNU). We analysed the rate of remnant moiety loss following HNU comparing age and affected moiety. Method All HNUs performed at our paediatric tertiary centre 2005-2019 were analysed. Children <16yrs, with pre– and post-operative DMSA imaging were included. Renal loss was categorised as: significant (≥50% of pre-existing function), non-significant (≥25% pre-existing function), no renal loss (<25%), and complete loss (post-operative remnant moiety function ≤5%). Subgroup analysis was performed using χ² statistic. Results 73 patients were included, mean age 2.1yrs. Median pre-operative function of the affected kidney was 42%. 12 patients (16.4%) had significant renal loss, 13 (17.8%) non-significant loss and 6 (8.2%) had complete renal loss. Children <2yrs had significant and complete renal loss more frequently than those aged ≥2yrs (9/35 and 5/35 vs 3/38 and 1/38 respectively, p = 0.069). Patients with upper moiety HNU (UMHNU) had higher rates of significant and complete renal loss than lower moiety HNU patients (12/53=significant, 6/53=complete vs 0/20 significant/complete, p = <0.05). Conclusions HNU for duplex kidney is associated with high rates of remnant moiety damage, with ¼ of patients experiencing significant or complete renal loss. Subgroup analysis suggests this risk is higher in children <2yrs or UMHNU. HNU should therefore only be reserved for symptomatic patients failing conservative management.

Clinical challenges and management of primary renal epithelioid angiomyolipoma of duplex kidney with paraneoplastic syndrome

Giant renal epithelioid angiomyolipoma of a duplex kidney has rarely been reported, especially in patients with paraneoplastic syndrome. The present report describes a 33-year-old man of Miao nationality who presented with a 6-month history of intermittent dull pain in the left upper abdomen that occurred after eating. Ultrasonography, intravenous pyelography, and computed tomography revealed a mass lesion localized in the left kidney and connected to the left renal artery. Radical nephrectomy was successfully performed, and the postoperative histopathological examination verified the lesion as epithelioid angiomyolipoma. Inpatient treatment for paraneoplastic syndrome was also performed. The present case is discussed in the context of the patient’s clinical presentation and imaging findings, drawing attention to the challenges and management of this condition to assist clinicians in practice.

Technical standardization of ICG near-infrared fluorescence (NIRF) laparoscopic partial nephrectomy for duplex kidney in pediatric patients

Purpose This study aimed to standardize the operative technique of indocyanine green (ICG) near-infrared fluorescence (NIRF) laparoscopic partial nephrectomy (LPN) and compare it with the standard technique. Methods In the last 4 years, we performed 22 LPN (14 right-sided, 8 left-sided) in children with non-functioning moiety of duplex kidney. Patients included 12 girls and 10 boys with a median age of 3.9 years (range 1–10). Patients were grouped according to the use of ICG-NIRF: G1 included 12 patients operated using ICG-NIRF and G2 included 10 patients receiving the standard technique. We standardized the technique of injection of ICG in three different steps. Results The median operative time was significantly lower in G1 [87 min (range 68–110)] compared with G2 [140 min (range 70–220)] (p = 0.001). One intra-operative complication occurred in G2. At post-operative ultrasound (US), the residual moiety was normal in all patients. An asymptomatic renal cyst related to the site of surgery was visualized at US in 8/22 (36%), with a significantly higher incidence in G2 (6/10, 60%) compared with G1 (2/12, 16.6%) (p = 0.001). Renogram demonstrated no loss of function of residual moiety. No allergic reactions to ICG occurred. Conclusion ICG-NIRF LPN is technically easier, quicker, and safer compared with the standard technique. The main advantages of using ICG-NIRF during LPN are the clear identification of normal ureter, vasculature of non-functioning pole, and demarcation line between the avascular and the perfused pole. The main limitation of ICG technology remains the need for specific laparoscopic equipment that is not always available.