A case of von Hippel-Lindau disease with multi-organ involvement: a rare case report

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant syndrome manifested by a spectrum of tumours in the central nervous system (CNS) and other visceral organs. We herein report a case of 35 years aged newly diagnosed diabetic female patient presented with headache, gait instability, loss of vision in both eyes, left sided hearing impairment and subsequently diagnosed to have VHL disease. The pathophysiology involves the inactivation of the VHL tumour suppressor gene. Early recognition and treatment remains the mainstay of management. Even many years after the complete tumour excision, newer neoplasms may develop. Increasing knowledge about the molecular enabled us to investigate the role of anti-angiogenic drugs. Continuous surveillance at regular interval must be conducted in patients with VHL disease.

Post traumatic ureteral defect brigded with ileal segment (Yang-Monti) for inferior ureterocalycostomy: a rare case report with review of literature

Ureteric substitution using the Yang-Monti principle was reported as a modification of simple ileal ureter replacement. A patient underwent ileal ureteral substitution using a reconfigured ileal segment of Yang-Monti principle in our clinical centre. Authors report a case of a 41year old male involved in a homicidal stab injury with isolated renal pelvis injury underwent Thompson’s renal capsular flap repair. After 6 weeks, double J (DJ) stent was removed and following that patient developed urinoma. Percutaneous drain was placed to drain urinoma on emergency basis and again 6 Fr DJ stent was inserted but patient had recurrent fever and chills. On CECT evaluation authors noticed that DJ stent was outside the upper urinary tract. And then patient was re-explored where authors found 6 cm stricture of the proximal left ureter. A reconfigured small bowel tube was interposed between inferior calyx and proximal ureteral stumps. This technique offers certain distinct advantages. A short ileal segment is included with the consequent absence of metabolic complications. Yang-Monti Principle is a safer and efficient technique for clinical partial and complete ureteral defects with sustained, good, long-term results. Yang-Monti reconfigured tube seems to be promoted an equally efficient urine transport mechanism that persists unaltered for long periods if patients and potential risks could be well prepared.

Dorsal onlay versus dorsal inlay buccal mucosal graft urethroplasty for anterior urethral stricture: a single centre retrospective study

Background: Urethral stricture is a relatively common disease. The choice of surgery is based on the stricture location, length of the stricture and etiology. Buccal mucosal graft (BMG) urethroplasty with Asopa and Kulkarni techniques, revolutionized the approach to anterior urethral stricture repair. Objective of the study was to compare both the dorsal onlay BMG urethroplasty technique of Kulkarni and the dorsal inlay BMG urethroplasty technique of Asopa for the management of long anterior urethral stricture.
Methods: From January 2015 to October 2019, a total of 90 patients with long anterior urethral strictures were randomized into two groups. Group A (42 patients) managed by Kulkarni technique. Group B (48 patients) managed by Asopa technique. BMG urethroplasty was considered successful if no further procedure required postoperatively with maximum flow rate >15 ml/s during the follow-up period.Results: The success rate in group A and B were 80.9% and 87.5%, respectively. The mean operative time was significantly longer in group A (175±22.6 min) than in group B (102±18.14 min, p-value <0.001). The average blood loss was significantly higher in group A (154±15.65 ml) than in group B (112.76±12.62 ml, p-value <0.012).Conclusions: The dorsal onlay technique of Kulkarni and the dorsal inlay technique of Asopa buccal mucosal graft urethroplasty are reliable and satisfactory procedures with good success rates and minimum complications.

Prognostic significance of inflammatory markers in patients with rare kidney cancers

Background: Although clear cell renal cell carcinoma (ccRCC) is the most common histological variety of malignant renal tumor, histological variants are often encountered in clinical practice which behaves differently. Paucity of such tumors makes them a subject of interest worldwide. As per European Association of Urology, since ccRCC is a non-designation, they included all non-clear cell RCC under one nomenclature as rare kidney cancer (RKC). The objective of our study is to determine influence of inflammatory markers on the prognosis of RKC.Methods: Data from cancer registry was retrieved and all rare kidney cancer patient’s data were analysed particularly the markers of inflammation like neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), systemic inflammatory immune index (SIII) and C reactive protein (CRP) to albumin ratio and their probable influence on cancer free survival (CFS), progression free survival (PFS) and overall survival (OS).Results: Data of 33 cases of rare kidney cancers were included in this study. The follow up duration ranges from 6.8 months to 38.6 months. In the univariate analysis, NLR had a significant influence on CFS, PFS and OS (cutoff value-3.2, 95% confidence interval [CI], CFS: p<0.05; PFS: p=0.05; OS: p<0.05), PLR in respect to CFS (cutoff value-67.5, 95% CI, p<0.05) and SIII had a significant impact on CFS and OS (cutoff value-8.67, 95% CI, 11.10-19.57, CFS: p<0.05; OS: p<0.05).Conclusions: Inflammation markers such as NLR, PLR, SII Index and CRP or albumin ratio could be independent predictors of clinical outcome and prognostics factors in rare kidney cancers. However, this needs to be validated by multicentre randomised studies.