Management Outcomes of Large Renal Angiomyolipoma Presenting with Wunderlich Syndrome-Experience from a Tertiary Center.

Renal angiomyolipoma is an uncommon, benign-mixed mesenchymal tumor consisting of thick-walled blood vessels, smooth muscles, and mature adipose tissues. Twenty percent of these tumors are associated with tuberous sclerosis. Wunderlich syndrome (WS), an acute nontraumatic spontaneous perirenal hemorrhage, can be a presentation of large angiomyolipoma. This study evaluated the presentation, management, and complications of renal angiomyolipoma with WS in eight patients who presented to the emergency department between January 2019 and December 2021. The presenting symptoms included flank pain, palpable mass, hematuria, and bleeding in the perinephric space on computerized tomography. Demographic data, symptoms at presentation, comorbidities, hemodynamic parameters, the association with tuberous sclerosis, transfusion requirements, need for angioembolization, surgical management, Clavien-Dindo complication, duration of hospital stay, and 30-day readmission rates were evaluated. The mean age of presentation was 38 years. Of the eight patients, five (62.5%) were females and 3(37.5%) were males. Two (25%) patients had tuberous sclerosis with angiomyolipoma, and three (37.5%) patients presented with hypotension. The mean packed cell transfusion was three units, and the mean tumor size was 7.85 cm (3.5-25 cm). Three of them (37.5%) required emergency angioembolization to prevent exsanguination. Embolization was unsuccessful in one patient (33%) who underwent emergency open partial nephrectomy, and one (33%) patient developed post-embolization syndrome. A total of six patients underwent elective surgery-four underwent partial nephrectomy (laparoscopic - 1, robotic - 1, open - 2) and two underwent open nephrectomy. Three patients encountered Clavien-Dindo complications (Grade 1, n = 2 and IIIA, n = 2). WS is a rare, life-threatening complication in patients with large angiomyolipoma. Judicious optimization, angioembolization, and prompt surgical intervention will help deliver better outcomes.

Outcomes of bladder neck botulinum toxin injection for female primary bladder neck obstruction-does subjective improvement correlate with an objective assessment?

INTRODUCTION AND HYPOTHESIS: Common options for management of primary bladder neck obstruction (PBNO) in women include medications and surgical treatment. Less invasive treatment such as bladder neck botulinum toxin injection can be an alternate therapy in patients with failed conservative management. In this study, we describe the subjective and objective outcomes, patient satisfaction, and willingness for repeat treatment with bladder neck botulinum toxin injection in females with PBNO. METHODS: A retrospective analysis of ten female PBNO patients managed with bladder neck botulinum toxin injection was performed. Subjective parameters were quantified with symptom assessment, International Prostate Symptom Score (IPSS), and Quality of life (QoL) score. Objective parameters were assessed with maximum flow rate (Qmax) in uroflowmetry and postvoid residual (PVR). RESULTS: The mean pre-treatment IPSS, QoL score, Qmax, PVR was 24.2 ± 5.0, 4.8 ± 0.63, 5.73 ± 3.18 ml/s, and 210 ± 66 ml, respectively. Seven of the ten patients subjectively improved (IPSS 12.9 ± 9.6, QoL2.9 ± 1.6, p < 0.05). Three patients improved objectively (mean Qmax 17.3 ± 2.7 ml/s, PVR 42.7 ± 7.5 ml, p < 0.05). Three patients accepted repeat botulinum toxin injection. Three patients who showed no improvement underwent bladder neck incision with resolution of symptoms. CONCLUSION: Botulinum toxin can be an intermediary therapy in female patients with PBNO who want a minimally invasive procedure.

Perioperative and oncological outcomes of renal cell carcinoma with venous tumour thrombus.

AIM: The aim of the present study is to report the perioperative and long-term surgical outcomes of renal cell carcinoma (RCC) with venous tumour thrombus (VTT). MATERIALS AND METHODS: Data of 34 patients (males = 23, females = 11) from 2009 to 2020 who underwent radical nephrectomy with thrombectomy for RCC and VTT was retrospectively analysed. The parameters recorded include tumour laterality, size, level of thrombus, surgical approach, blood loss, transfusion rates, operative time, hospital stay, Clavien complications, tumour histology, follow-up duration, local recurrence, distant metastasis, overall and cancer-specific survival rates. RESULTS: The extent of thrombus was level I in thirteen, level II in twelve, level III (a-1, b-2, c-1, d-2) in six and level IV in three patients respectively. Mean operative time was 320 (±145) min, mean blood loss was 1371.15 (±1020.8) ml and mean hospital stay was 9.6 (±7.4) days. Mean transfusion rate was 6.4 (±3.2) units. Three patients died within the first 30 days of surgery. Cardiopulmonary bypass (CPB) was utilized in three patients. Median follow-up period was 58 (Range: 4-101) months. A statistically significant correlation was found between operative time (p = 0.014) and median survival (p = 0.003) respectively and tumour thrombosis level. Nine patients died due to metastasis, and ten due to unrelated causes. The estimated actuarial survival rates at a median of 58 months were 35.3%. CONCLUSION: An accurate preoperative assessment of the thrombus extent with the involvement of a multidisciplinary team approach is crucial in achieving optimal surgical outcomes in patients of RCC with VTT, particularly with level III and IV thrombus.

Does successful urethral calibration rule out significant female urethral stenosis? confronting the confounder- an outcome analysis of successfully treated female urethral strictures

ABSTRACT Objective: The diagnosis and treatment of female urethral stricture disease (FUSD) are practiced variably due to the scarcity of data on evaluation, variable definitions, and lack of long-term surgical outcomes. FUSD is difficult to rule out solely on the basis of a successful calibration with 14F catheter. In this study, we have tried to characterize the variable clinical presentation of FUSD, the diagnostic utility of calibration, videourodynamic study(VUDS), and urethroscopy in planning surgical management. Materials and Methods: A retrospective review of records of 16 patients who underwent surgical management of FUSD was analyzed. The clinical history, examination findings, and the results of all the investigations (including uroflowmetry, VUDS findings, urethroscopy) they underwent, the procedures they had undergone, and the follow-up data were studied. Results: A total of 16 patients underwent surgical management of FUSD. 13 out of 16 patients had successful calibration with 14F catheter on the initial presentation. These 13 patients on VUDS demonstrated significant BOO and had variable stigmata of stricture on urethroscopy. The mean IPSS, flow rate, and PVR at presentation and after urethroplasty were 23.88±4.95, 7.72±4.25mL/s, 117.06±74.46mL and 3.50±3.44, 22.34±4.80mL/s, and 12.50±8.50mL, respectively. (p <0.05). The mean flow rate after endo dilation(17F) (n=12) was 11.4±2.5mL/s while after urethroplasty improved to 20.30±4.19mL/s and was statistically significant(p <0.05). Conclusions: An adept correlation between clinical assessment, urethroscopy findings, and VUDS is key in objectively identifying FUSD and planning surgical management. A good caliber of the urethra is not sufficient enough to rule out a significant obstruction due to FUSD. Early urethroplasty provides significantly better outcomes in patients who have failed dilation as a treatment.

Does successful urethral calibration rule out significant female urethral stenosis? confronting the confounder- an outcome analysis of successfully treated female urethral strictures.

OBJECTIVE: The diagnosis and treatment of female urethral stricture disease (FUSD) are practiced variably due to the scarcity of data on evaluation, variable definitions, and lack of long-term surgical outcomes. FUSD is difficult to rule out solely on the basis of a successful calibration with 14F catheter. In this study, we have tried to characterize the variable clinical presentation of FUSD, the diagnostic utility of calibration, videourodynamic study(VUDS), and urethroscopy in planning surgical management. MATERIALS AND METHODS: A retrospective review of records of 16 patients who underwent surgical management of FUSD was analyzed. The clinical history, examination findings, and the results of all the investigations (including uroflowmetry, VUDS findings, urethroscopy) they underwent, the procedures they had undergone ,and the follow-up data were studied. RESULTS: A total of 16 patients underwent surgical management of FUSD. 13 out of 16 patients had successful calibration with 14F catheter on the initial presentation. These 13 patients on VUDS demonstrated significant BOO and had variable stigmata of stricture on urethroscopy. The mean IPSS, flow rate, and PVR at presentation and after urethroplasty were 23.88±4.95, 7.72±4.25mL/s, 117.06±74.46mL and 3.50±3.44, 22.34±4.80mL/s, and 12.50±8.50mL, respectively. (p < 0.05). The mean flow rate after endo dilation(17F) (n=12) was 11.4±2.5mL/s while after urethroplasty improved to 20.30±4.19mL/s and was statistically significant(p < 0.05). CONCLUSIONS: An adept correlation between clinical assessment, urethroscopy findings, and VUDS is key in objectively identifying FUSD and planning surgical management. A good caliber of the urethra is not sufficient enough to rule out a significant obstruction due to FUSD. Early urethroplasty provides significantly better outcomes in patients who have failed dilation as a treatment.

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature.

The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon histopathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing's sarcoma (ES)/PNET of the renal pelvis. The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.

Association of spontaneous expulsion with C-reactive protein and other clinico-demographic factors in patients with lower ureteric stone.

The purpose of our study is to analyze the definitive relation of C-reactive protein (CRP) and other factors with the spontaneous stone passage in patients with distal ureteric calculus of 5-10 mm and to calculate the risk of failure of expectant management in patients. 185 patients of ureteric colic, who were subjected to medical expulsive therapy (MET), were included prospectively from August 2016 to May 2018 and followed up for 4 weeks. Patients were divided into two groups. Group A included successful spontaneous passage patients and group B included failure in the same. The parameters analyzed were age, gender, longitudinal and transverse diameter of stone, CRP, total leucocyte count, ureteric diameter and hydroureteronephrosis (HUN). We performed univariate and multivariate analysis. Receiver operating characteristics curve was used to determine the cutoff value for significantly associated variables. 122 (65.90%) and 63 (34.10%) patients were included in group A and B, respectively. In univariate analysis, CRP, longitudinal and transverse diameter of stone, HUN, proximal and distal ureteric diameters were statistically significant. However, in multivariate analysis, only negative CRP (p = 0.002), smaller longitudinal diameter of stone (p < 0.001) and absence of HUN (p = 0.005) were significantly associated with successful expulsion. Cutoff for CRP was 0.41 mg/dl and longitudinal diameter was 6.7 mm. The success rate in the group of patients with no risk factor was 96.7% and with all three risk factors was 16.7%. Patients with a longitudinal diameter of stone > 6.7 mm, HUN, and CRP > 0.41 mg/dl should be considered for early intervention. The success rate of MET can be increased to 86% after exclusion of patients with all three risk factors.

Emphysematous pyelonephritis: Does a standard management algorithm and a prognostic scoring model optimize patient outcomes?

OBJECTIVE: The objective of the study is to analyze the risk factors determining the outcomes of patients with emphysematous pyelonephritis (EPN) by the adoption of a standardized management algorithm as well as to develop a prognostic scoring model to risk stratify these patients. MATERIALS AND METHODS: The hospital records of 72 consecutive patients with EPN from February 2012 to January 2018 were retrospectively reviewed. Demographic, clinicoradiographic, and laboratory characteristics were recorded. Patients were managed with a standard management protocol and based on outcomes divided into three groups. Group I survived with conservative management, Group II survived after emergency nephrectomy, and Group III expired. The risk factors for nephrectomy and mortality were analyzed. RESULTS: The mean age was 53 years. Male to female ratio was 4:5. There were 61 (84.7%), 4 (5.6%), and 7 (10%) patients in Groups I, II, and III, respectively. Diabetes mellitus was the most common comorbidity detected in 62 (86%) of patients. Type II EPN was the most common radiological presentation observed in 32 (44%) patients. Overall survival rate was 90%, and kidney salvage rate was 80%. Escherichia coli was the most common organism isolated. Thirty-two (45%) patients exhibited resistance to third-generation cephalosporin antibiotics. Thrombocytopenia, low body mass index (BMI), presence of >2 comorbidities, high total leukocyte count (TLC), and hypoalbuminemia were significantly associated with mortality. On adoption of the prognostic scoring system, mortality rates according to the risk subgroups were as follows: favorable - 0%, intermediate - 19%, and poor - 100%. CONCLUSION: Conservative management adopting appropriate algorithm reduces mortality and avoids unnecessary emergency nephrectomies. Thrombocytopenia, low BMI, presence of >2 comorbidities, high TLC, and hypoalbuminemia were significantly associated with mortality.