Anastomosing haemangioma of adrenal gland: an unusual vascular tumour.

With only 15 reported cases, anastomosing haemangioma of adrenal is a rare entity and usually presents as adrenal incidentaloma. A hypertensive, diabetic, non-smoker man in his late 60s presented with irritative voiding symptoms. On evaluation, he was found to have a urinary bladder mass and left adrenal incidentaloma measuring 8 cm. Metabolic evaluation confirmed it to be non-functional.The patient underwent transurethral resection of bladder tumour with left laparoscopic adrenalectomy. Intraoperatively, the adrenal tumour was highly vascular with multiple feeder vessels. Grossly it was soft, encapsulated with focal grey-brown areas. Microscopically, most of adrenal gland was replaced by anastomosing proliferating capillary vessels within framework of non-endothelial supporting cells reminiscent of splenic sinusoids. The tumour was positive for CD-31, CD-34, Glut-1 and SMA.Anastomosing haemangioma is a benign entity but it must be differentiated from angiosarcoma. Characteristic imaging features are not yet defined and is, therefore, difficult to diagnose preoperatively.

Small Tumor, Major Complication: A Case of Spontaneous Perinephric Hematoma From a Small Renal Angiomyolipoma.

A renal angiomyolipoma is a benign kidney tumor composed of muscle, fat, and blood vessels. It is the most common benign kidney tumor, and it affects women more frequently than men. Angiomyolipomas can be small and asymptomatic, or they can be large, presenting with symptoms such as discomfort, hematuria, and hypertension. Occasionally, the rupture of an angiomyolipoma can cause a perinephric hematoma. This case report discusses a patient who developed a spontaneous large perinephric hematoma alongside a small renal angiomyolipoma. Aneurysm was seen on imaging. Angioembolization was successfully used for treatment. We explore the significance of the presence or absence of an aneurysm in predicting the risk of hemorrhage, particularly in association with small lesions. Angioembolization is an excellent choice for treating angiomyolipomas associated with significant hematomas.

Inner ear barotrauma: A case report of rare post-operative complication following robot-assisted radical prostatectomy.

To highlight a rare case of inner ear barotrauma as a post-operative complication following robot-assisted radical prostatectomy (RARP). A 65-year-old man diagnosed with localised prostate carcinoma underwent elective RARP in a steep Trendelenburg position. Postoperatively, the patient complained of bothersome dizziness and spinning of surroundings associated with nausea. The patient was diagnosed to have vestibular symptoms secondary to inner ear barotrauma. He was started on prochlorperazine 12.5 mg and antibiotics, following which the patient improved. Vestibular rehabilitation exercises were taught to the patient. After 3 days of treatment, the patient did well with no episodes of dizziness. The patient was discharged on tablet cinnarizine 25 mg thrice daily for a week. On follow-up, the patient is asymptomatic. Inner ear barotrauma can be a rare complication of a combination of prolonged steep Trendelenburg during robotic surgery and pneumoperitoneum. Only further reporting of such incidents can help determine predisposing factors and precautions for preventing such incidents.

Recurrent vesico-acetabulo-cutaneous fistula: lessons to be learnt.

A man in early 40s met with an accident with a complex pelvic fracture and extraperitoneal bladder injury and posterior urethral disruption 16 years ago. He additionally had left lumbar spinal segment mixed nerve injury, resulting in a foot drop. He underwent laparotomy and a diverting cystostomy at the time with a primary perineal urethroplasty a year later. He later developed pseudoarthrodesis of the hip joint, and poorly compliant bladder with complete block at bulbar urethra. A redo anastomotic urethroplasty was performed, hyperreflexive neurogenic bladder was managed with intravesical botox injections and underwent a hip replacement. Having defaulted botox injections, he developed a vesico-acetabulo-cutaneous fistula and the hip prosthesis was explanted. Later he underwent a ileal cystoplasty and a revision hip replacement. Ten years later, he presented with a recurrent fistula due to poor compliance with clean intermittent catheterisation. A challenging exploration with fistula excision was done with a primary bladder repair.

Small contracted bladders posing bigger problems: Etiology, presentation, and management and a short review of literature.

The purpose of this study was to assess various etiologies, diagnosis and management. This rare entity is a neglected condition which should always be under clinical suspicion by broad speciality of practitioners for early treatment. Retrospective data collected from 2018 to 2021 in the All India Institute of Medical Sciences Rishikesh was used. All patients diagnosed with the small contracted bladder in the given period were included. The primary outcome of the study was to find out the common causes, early tests used for diagnosis and management done in the patients of small contracted bladder attending this tertiary care centre. Between 2018 and 2021, a total of 12 patients were diagnosed to have small capacity bladder (SCB). The most common symptom was frequency (75%). On cystoscopy, 33.33% (n = 4) had less than 50 ml and 66.66% (n = 8) had 50-100 ml bladder capacity respectively. 37.5% (n = 3) were diagnosed by urine AFB culture, 62.5% (n = 5) were diagnosed by urine for PCR, 62.5% (n = 5) were diagnosed by radiological investigations. Eight patients (66.66%) underwent surgical treatment in cases diagnosed as tuberculosis like augmentation cystoplasty and supra-trigonal cystectomy. Other rare causes found were eosinophilic cystitis, radiation induced contracture and BCG induced contracture. Small capacity bladder is an unusual condition, with still dilemma on the definition of small capacity and only few literature mentioning the causes, diagnosis and treatment. Even though tuberculosis is a common cause of SCB, still rare causes should always be kept in mind for relieving patient symptoms at the earliest.

Urological Manifestations of Kindler Syndrome: A Case Report.

Kindler syndrome is a rare autosomal recessive skin disorder. It results from mutation of the FERM domain containing kindlin-1 (FERMT1) that leads to loss of function of kindlin-1, which plays a role in keratinocyte adhesion, polarization, proliferation, and migration. It is characterized by skin blistering, photosensitivity, progressive poikiloderma, and skin atrophy. The mucosae genitourinary system is commonly affected. The urological manifestations include meatal stenosis, urethral stricture, phimosis, and scarring of the glans penis. Skin biopsy with genetic analysis is the gold standard for diagnosis. Genetic counseling and a multidisciplinary approach are the mainstays of treatment.

A 17-year-old indwelling ureteral stent with large vesical calculus at one end: The tombstone of a forgotten Double "J" stent.

Objective: To highlight the presentation and management of a 17-year-old forgotten ureteral stent with a large vesical calculus at one end, a very rare yet devastating complication of ureteral stenting. Case Presentation: A 65-year-old gentleman presented with complaints of lower urinary tract symptoms for two years with a history of ureterolithotomy done 17 years ago. Imaging revealed a forgotten ureteral stent embedded in a large vesical calculus. He underwent a percutaneous cystolithotomy with removal of the forgotten stent. Conclusion: DJ stent is commonly used in urologic practice. It is a double-edged sword as it can lead to severe morbidity in patients who have forgotten stent. Knowledge of DJ stent-related complications and need of timely removal will help primary care physician in providing better health care. Proper counselling of patients before and after the placement of stent and maintenance of stent registries play a major role in avoiding this dreadful complication.

Incidental encounter of intraperitoneal tuberculosis during renal surgeries: A surgeon's dilemma.

Tuberculosis is a major healthcare burden in India, which accounts for the maximum number of cases worldwide. Due to its non-specific features, peritoneal tuberculosis has been dubbed as the great mimicker of various other abdominal pathologies. This case series highlights the importance of incidental intra operative detection of peritoneal tuberculosis in cases being operated for renal pathologies. Diagnostic and therapeutic dilemma is bound to occur when surgeon is faced with such an unexpected finding. Incidental peritoneal tuberculosis was defined as peritoneal tubercular lesions (ascites or tubercles) detected intraoperatively in patients being operated for non-tuberculosis related indications and no prior preoperative suspicion of abdominal tuberculosis. We here review 3 cases with different renal pathologies and no prior history or exposure to tuberculosis in which intraperitoneal tuberculosis was encountered incidentally at the time of surgery. Case 1 was a suspected case of right renal cell carcinoma and underwent right robotic nephron sparing surgery. Case 2 underwent robotic assisted lap simple nephrectomy for a right nonfunctioning kidney due to obstructive ureteric calculus. Case 3 was a suspected case of left upper tract urothelial carcinoma who underwent robotic nephroureterectomy with bladder cuff excision. In all 3 cases, on encountering the peritoneal lesions, an intraoperative decision to continue with the proposed surgery was made after frozen section biopsies from the multiple peritoneal and omental deposits revealed no malignant cells. Histopathology of these lesions in all 3 cases revealed caseating granulomas consistent with a diagnosis of disseminated peritoneal tuberculosis. None of the resected specimen had features suggestive of tuberculosis. ATT was started and on follow up the patients are doing well. Peritoneal tuberculosis although uncommon is not a rare presentation of active tuberculosis. Surgeons on encountering such lesions during non-related surgeries should always have a high suspicion of tuberculosis. Despite the existing literature favoring abandoning the procedure in such situations, we successfully completed the proposed surgeries.

Management strategies and outcome of ureterovaginal fistulae: A systematic review and meta-analysis.

BACKGROUND: Genitourinary fistula is a distressful condition involving mental, social, marital, and financial repercussions. OBJECTIVE: The objective of this study is to systematically evaluate etiology, clinical presentation, diagnosis, the timing of repair, and perform a meta-analysis evaluating the success rate of various treatment modalities with respect to time taken to seek treatment. SEARCH STRATEGY: We performed a critical review of PubMed/Medline, Embase, and the Cochrane Library in April 2020 according to the PRISMA statement. Seventeen studies were included in the final analysis and all were retrospective in design. SELECTION CRITERIA: Each article was rated by the evidence-based medicine levels of evidence scale and the Methodological Index for Nonrandomized Studies scale for assessment of bias among nonrandomized studies. MAIN RESULTS: Of the 799 fistulae reported in 17 studies, endoscopic management was done in 35.6% (12 studies), whereas surgical management was preferred in 85.6% fistulae (15 studies). The pooled success of endoscopic stenting was 32% (95% confidence interval [CI]: 7-64) and 100% (95% CI: 98-100) in operated patients. Patients who underwent stenting within 2 weeks (20%), 2-6 weeks (21%), and >6 weeks (40%) had pooled success rates of 95% (95% CI: 87-100), 46% (95% CI: 0-100), and 20% (95% CI: 1-49), respectively. Patients who underwent surgical management <6 weeks (15.9%) and >6 weeks (22%) of diagnosis had pooled success rates of 100% (95% CI: 99-100) and 100% (95% CI: 99-100), respectively. CONCLUSIONS: Stent placement as early as <6 weeks (preferably < 2 weeks) had better outcomes as compared to >6 weeks. Proceeding to surgery regardless of timing in cases of stent failure seems to be a feasible option.

A Clinicopathological Correlation of Bladder Cancer in Young and Old Patients: Our Experience and Review of Literature.

To compare the clinical, cystoscopic, and pathological characteristics of bladder cancer in patients younger than 40 years of age and those of patients older than 40 years of age. We conducted a prospective observational study at our department from September 2019 to February 2021 to compare the clinico-pathological characteristics of young and old patients with biopsy proven bladder cancer after a transurethral resection of bladder tumour. The patients were managed according to standard guidelines. Two hundred sixty-eight patients of bladder cancer were included in the analysis. Out of these, 58 patients were < 40 years of age and 210 were > 40 years. The mean age of two groups were 31.43 ± 6.30 vs 59.08 ± 9.87 years. With respect to tumour grade, 36.2% (versus 17.6%) of young patients had low grade tumour, and 58.6% had high-grade tumour (versus 82.4%). In the young population, 63.8% patients had NMIBC (versus 61.9%) and 36.2% had MIBC (versus 38.1%). Even in the NMIBC group, a large majority of young patients had high-risk disease (51.4%). The incidence of bladder cancer is on the rise in the northern belt of India, especially in the younger age group. High exposure to smoke and heavy metals in drinking water/occupation are the major risk factors. Majority of young patients aged < 40 years had a high-grade disease on presentation and a large subset had muscle invasive bladder cancer, contrary to previously reported studies.

Efficacy and Safety of Percutaneous Nephrolithotomy in Patients with Chronic Kidney Disease: Outcomes from a Tertiary Care Center.

Background: The management of renal stone disease in the presence of chronic kidney disease (CKD) is a challenging scenario, both in terms of surgical safety and perioperative outcomes. The aim of the present study is to study the efficacy, safety, and outcomes of percutaneous nephrolithotomy (PCNL) in patients with CKD. Materials and Methods: A prospective study was conducted including adult patients with renal stone disease and a creatinine clearance of <90 mL/min (Stage 2 CKD or more) who underwent PCNL. Pre- and postoperative serum creatinine and glomerular filtration rates (GFRs) were compared. Patients were divided into CKD Stages 1 to 5 having creatinine clearance >90 mL/min, 60 to 90 mL/min, 30 to 60 mL/min, 15 to 30 mL/min, and <15 mL/min, respectively. Based on up migration or down migration of CKD stages, patients were classified as improved, deteriorated, or stable. Perioperative complications and outcomes were also compared. Results: A total of 185 patients with CKD Stage ≤2 underwent PCNL. The mean age of the patients was 43.24 ± 14.32 years. The mean preoperative estimated glomerular filtration rate (eGFR) was 62.88 ± 23.42 mL/min/1.73 m2. Preoperative CKD stage distribution was as follows: Stage 2 to 121 (65.4%), Stage 3 to 34 (18.4%), Stage 4 to 24 (13%), and Stage 5 to 6 (3.2%). On last follow-up of patients, the mean creatinine was 1.07 ± 0.65 mg/dL and the mean eGFR was 82.75 ± 31.22 mL/min/1.73 m2. The median change in creatinine and mean change in GFR were 0.23 (0.16-0.27) g/dL and 19.87 ± 19.77 mL/min/1.73 m2, respectively. Improvement in kidney function with a stage down migration was seen in 115 cases (62.2%), slight improvement with no change in stage in 69 cases (37.3%), and deterioration of CKD stage was present in one case (0.5%). Conclusion: PCNL is associated with favorable functional outcomes in CKD patients including severe CKD (Stages 4 and 5). Improvement or stabilization of CKD stage was seen in 99.5% of patients post-PCNL.

Successful management of a high-output urinary fistula following radical cystectomy and ileal conduit: a conservative approach.

Urinary leak following ileal conduit after a radical cystectomy is a rare yet serious complication which presents early in the postoperative period. We herein present a case of a 38-year-old man diagnosed with recurrent high-grade non-muscle invasive bladder carcinoma. He underwent robot-assisted radical cystectomy and bilateral pelvic lymph node dissection. Postoperatively, the patient developed a high output urinary fistula (800-1000 mL/day) which was confirmed by fluid creatinine levels and a contrast study. He was managed successfully with a conservative approach. The leak subsided in 6 weeks and on follow-up patient is doing well. Most of the literature favours a surgical approach in such cases, however with optimal nutritional support (enteral/parenteral), adequate diversion of urine, percutaneous drainage of undrained collections, adequate intravenous antibiotics and good nursing care, resurgery with its associated morbidity can be avoided resulting in successful outcomes.

Robot-Assisted Laparoscopic Approach in a Patient of Zinner Syndrome with Hematuria: A Rare Presentation.

Congenital malformations of the seminal vesicles (SVs) are rare and are often associated with abnormalities of the ipsilateral upper tracts as embryologically both the ureteral buds and SVs arise from the mesonephric ducts. The triad of SV cysts, ipsilateral renal agenesis, and ejaculatory duct obstruction is known as the Zinner syndrome. We herein present the case of Zinner syndrome with hematuria as the mode of presentation. A 52-year-old gentleman presented with complaints of gross and painless hematuria for 3 months. An initial ultrasound revealed absent right kidney with a cystic structure abutting the urinary bladder. Cystoscopic examination revealed a high bladder neck. Magnetic resonance imaging of the abdomen revealed an absent right kidney and a large tubular structure in the region of the right ureter extending up to right SV with blood content and stones within. It was distally seen communicating with one of the cysts of the right SV. The cystic structure was removed with a robot-assisted laparoscopic approach. The console time was 110 min with minimal blood loss. Postoperative course was uneventful. Histopathology of the cyst wall revealed chronic inflammation. The patient is doing well on 6 months follow-up. This case was unique in terms of it presenting with a large intra-abdominal cyst with sharp stones within, probably first of its kind to be ever reported. Surgery is mandated for such symptomatic cysts and the daVinci robot with its minimally invasive approach offers the perfect platform for treating such challenging cases.

Catastrophic complications of urolithiasis in pregnancy.

Urolithiasis is the most common non-obstetric complication in pregnancy and has the potential to cause grave consequences resulting in pregnancy loss. We present two such cases. First, a 24-year-old woman, 5 weeks pregnant with a history of urolithiasis presented with right flank pain and fever. She was found to have a right perinephric collection and during the course of her treatment suffered an abortion. The second case was a 25-year-old woman who presented in septic shock. She underwent emergency lower segment caesarean section elsewhere 10 days ago for intrauterine death at 38 weeks of gestation. On evaluation, she was found to have bilateral stone disease with a left subcapsular haematoma. Both the cases were managed conservatively and are planned for definitive management. Thus, women of childbearing age with diagnosed urolithiasis should get themselves evaluated and be free of stone disease before planning a family to prevent increased obstetric complications during pregnancy.

Ganglioneuroma presenting as an adrenal incidentaloma: Feasibility of adrenal-sparing surgery.

Adrenal ganglioneuromas (GNs) are very rare tumours that originate from neural crest cells. Most of the time, they are diagnosed incidentally as they are usually non-functional and remain asymptomatic. Nowadays, they are being detected more often due to better availability of imaging facilities such as computed tomography (CT)/magnetic resonance imaging (MRI). Minimally invasive adrenalectomy (laparoscopic or robotic) remains the standard of care for such lesions. Hereby, we report a case of a 15-year-old young girl with right adrenal incidentaloma which was diagnosed on CT with the features suggestive of GN. She underwent robot-assisted excision of the mass with adrenal-sparing surgery. Histopathology revealed benign GN and no adjuvant treatment was required. As GN is not known for recurrence or metastasis, minimal invasive adrenal-sparing surgery should be a preferred modality of choice.

Neoadjuvant Therapy for Cisplatin Ineligible Muscle Invasive Bladder Cancer Patients: A Review of Available Evidence.

Neoadjuvant chemotherapy followed by radical cystectomy is the standard of care for muscle invasive non-metastatic bladder cancer patients. While cisplatin based neoadjuvant chemotherapy has been recommended, systemic therapy in a neoadjuvant setting for cisplatin ineligible patients still needs to be addressed. Various strategies like split dosing cisplatin chemotherapy, carboplatin based chemotherapy and taxanes based chemotherapy have been tried as neoadjuvant therapy for cisplatin ineligible patients. Immunotherapy is a promising tool in this regard with a need for the development of predictive and prognostic biomarkers which can bring out the true potential of these immunotherapeutic agents.

Female counterpart of the Zinner syndrome in men: decoding the 'OHVIRA' syndrome.

The obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, a rare Mullerian duct anomaly, is a triad of uterine anomalies with ipsilateral renal agenesis and obstructed hemivagina. The aetiopathogenesis of this developmental anomaly is debatable, with several theories being postulated to explain its occurrence. We report two cases of this rare syndrome which were detected on imaging done for unrelated reasons. Case 1 is a 24-year-old woman who presented with primary infertility. After an incidental detection of the syndrome and ruling out a male factor, she has been planned for excision of the vaginal septum. Case 2 is a 47-year-old woman with OHVIRA detected during the evaluation of urolithiasis and is asymptomatic for the syndrome. The OHVIRA syndrome can be asymptomatic or may present as haematocolpos, pelvic pain and infertility which requires surgical intervention, after which successful pregnancies have been reported.

A wide spectrum of rare clinical variants of Zinner syndrome.

Congenital malformations of the seminal vesicles (SVs) are rare and are associated with abnormalities of the ipsilateral urinary tracts as embryologically both the ureteral buds and SVs arise from the mesonephric ducts. The triad of SV cysts, ipsilateral renal agenesis and ejaculatory duct obstruction is known as the Zinner syndrome. We, herein, present three very rare presentations of Zinner syndrome. Case 1 presented with haematuria, and was found to have a large SV cyst with stones and underwent a robotic cyst excision. Case 2 presented with primary infertility, and was found to have a variant of Zinner syndrome. Case 3 was a known case of chronic kidney disease on maintenance haemodialysis who presented with fever and oliguria. He was found to have Zinner syndrome and underwent aspiration of SV abscess. To the best of our knowledge, such varying presentations of Zinner syndrome have been rarely reported thus far.