Bilateral renal haematuria and obstructive renal failure from acquired haemophilia A: a medical cause for a surgical problem.

A 55-year-old male presented to our emergency department with haematuria and abdominal pain. Investigations including a computed tomography (CT) scan revealed an intraluminal filling defect within the left collecting system, consistent in appearance with blood clot. With an initial working diagnosis of upper tract urothelial cell carcinoma, he was discharged with plans for an urgent cystoscopy and ureteroscopy. He subsequently represented with ongoing frank haematuria, anasarca, dropping haemoglobin and new right collecting system blood clot. Subsequent investigations showed that the patient had acquired haemophilia A resulting in the episodes of haematuria, highlighted after an elevated activated partial thromboplastic time prompted a thrombophilia screen. The patient was subsequently treated with factor eight inhibitor bypass activity, corticosteroids and cyclophosphamide.

Imaging of Small Renal Masses before and after Thermal Ablation.

Thermal ablation of small renal masses is increasingly accepted as an alternative to partial nephrectomy, particularly in patients with multiple comorbidities. Many professional societies support this alternate treatment with updated guidelines. Before performing thermal ablation, it is important to stratify risk and assess technical feasibility by evaluating tumor imaging features such as size, location, and centrality. Routine postablation imaging with CT or MRI is necessary for assessment of residual or recurrent tumor, evidence of complications, or new renal masses outside the ablation zone. The normal spectrum and evolution of findings at CT and MRI include a halo appearance of the ablation zone, ablation zone contraction, and ablation zone calcifications. Tumor recurrence frequently manifests at CT or MRI as new nodular enhancement at the periphery of an expanding ablation zone, although it is normal for the ablation zone to enlarge within the first few months. Recognizing early tumor recurrence is important, as small renal masses are often easily treated with repeat ablations. Potential complications of thermal ablation include vascular injury, urine leak, ureteral stricture, nerve injury, and bowel perforation. The risk of these complications may be related to tumor size and location.©RSNA, 2019.

Bilateral duplex collecting system with bilateral vesicoureteral reflux: a case report.

BACKGROUND: A bilateral duplex collecting system is an unusual renal tract abnormality. Vesicoureteral reflux may be associated. We describe a rare case of bilateral duplex collecting system with bilateral vesicoureteral reflux in which the refluxing ureter on the left side drains the upper pole moiety contrary to what is often found. CASE PRESENTATION: A 24-year-old married Arab woman presented with ascending left-sided flank pain during micturition. She complained of recurrent urinary tract infections. A physical examination and laboratory tests were normal. Voiding cystourethrography and computed tomography scan detected bilateral duplex collecting system, grade IV vesicoureteral reflux on the left side, and grade I vesicoureteral reflux on the right. She underwent left heminephrectomy and dextranomer/hyaluronic acid injections on the right side. After a year of follow-up, a clinical examination and imaging findings were unremarkable. CONCLUSIONS: A bilateral duplex collecting system with refluxing upper pole moiety ureter is a very rare entity. The diagnosis should be suspected when exploring any flank pain with recurrent urinary tract infections to avoid subsequent renal impairment. Furthermore, this case shows how some common symptoms may lead to finding an unexpected urinary tract abnormality.

Renal Contrast on CT Myelography: Diagnostic Value in Patients with Spontaneous Intracranial Hypotension.

BACKGROUND AND PURPOSE: The significance of renal contrast on CT myelography is uncertain. This project examined different patient populations undergoing CT myelography for the presence of renal contrast to determine whether this finding is of diagnostic value in spontaneous intracranial hypotension. MATERIALS AND METHODS: Four groups of patients were analyzed for renal contrast on CT myelography. The control group underwent CT myelography for reasons other than spontaneous intracranial hypotension (n = 47). Patients in study group 1 had spontaneous intracranial hypotension but CT myelography negative for dural CSF leak and CSF venous fistula (n = 83). Patients in study group 2 had spontaneous intracranial hypotension and CT myelography positive for dural CSF leak (n = 44). Patients in study group 3 had spontaneous intracranial hypotension and CT myelography suggestive of CSF venous fistula due to a hyperdense paraspinal vein (n = 17, eleven surgically confirmed). RESULTS: Renal contrast was present on the initial CT myelography in 0/47 patients in the control group, 10/83 patients in group one, 1/44 patients in group 2, and 7/17 patients in group 3. Renal contrast on initial CT myelography in patients with suspected or surgically confirmed CSF venous fistula was significantly more likely than in patients with a dural CSF leak (P = .0003). CONCLUSIONS: Renal contrast on initial CT myelography was seen only in patients with spontaneous intracranial hypotension. This was more common in confirmed/suspected CSF venous fistulas compared with dural leaks. Early renal contrast in patients with spontaneous intracranial hypotension should prompt scrutiny for a hyperdense paraspinal vein, and, if none is found, potentially advanced diagnostic studies.

High frequency and wide range of human kidney papillary crystalline plugs.

Most of kidney stones are supposed to originate from Randall's plaque at the tip of the papilla or from papillary tubular plugs. Nevertheless, the frequency and the composition of crystalline plugs remain only partly described. The objective was to assess the frequency, the composition and the topography of papillary plugs in human kidneys. A total of 76 papillae from 25 kidneys removed for cancer and without stones were analysed by immunohistochemistry combined with Yasue staining, field emission-scanning electron microscopy and Fourier transformed infrared micro-spectroscopy. Papillary tubular plugs have been observed by Yasue staining in 23/25 patients (92%) and 52/76 papillae (68%). Most of these plugs were made of calcium phosphate, mainly carbonated apatite and amorphous calcium phosphate, and rarely octacalcium phosphate pentahydrate. Calcium and magnesium phosphate (whitlockite) have also been observed. Based upon immunostaining coupled to Yasue coloration, most of calcium phosphate plugs were located in the deepest part of the loop of Henle. Calcium oxalate monohydrate and dihydrate tubular plugs were less frequent and stood in collecting ducts. At last, we observed calcium phosphate plugs deforming and sometimes breaking adjacent collecting ducts. Papillary tubular plugging, which may be considered as a potential first step toward kidney stone formation, is a very frequent setting, even in kidneys of non-stone formers. The variety in their composition and the distal precipitation of calcium oxalate suggest that plugs may occur in various conditions of urine supersaturation. Plugs were sometimes associated with collecting duct deformation.

[A Case Report : Metastatic Carcinoma of the Collecting Ducts of Bellini in a Hemodialysis Patient Treated with Temsirolimus].

Carcinoma of the collecting ductsof Bellini isa rare histological subtype of renal cell carcinoma and mostly has unfavorable prognosis. Radical nephrectomy is generally chosen for the 1st line treatment but therapeutic approaches for the metastasis/recurrence have not been established. We report a case of carcinoma of collecting ducts of Bellini in a patient receiving hemodialysis treated with temsirolimus. A 62- year-old man receiving hemodialysis was admitted to our hospital with drug-resistant anemia and high-grade cyclic fever. Computed tomography revealed the right renal tumor and multiple metastatic lung tumors. Open radical nephrectomy wasperformed. Pathological findingswere compatible with carcinoma of the collecting ducts of Bellini. He was given weekly temsirolimus treatment. The disease progressed modestly but kept the stable disease (SD) status for six months. He died of the cancer 11 months after the initial diagnosis.

Soft 3D-Printed Phantom of the Human Kidney with Collecting System.

Organ models are used for planning and simulation of operations, developing new surgical instruments, and training purposes. There is a substantial demand for in vitro organ phantoms, especially in urological surgery. Animal models and existing simulator systems poorly mimic the detailed morphology and the physical properties of human organs. In this paper, we report a novel fabrication process to make a human kidney phantom with realistic anatomical structures and physical properties. The detailed anatomical structure was directly acquired from high resolution CT data sets of human cadaveric kidneys. The soft phantoms were constructed using a novel technique that combines 3D wax printing and polymer molding. Anatomical details and material properties of the phantoms were validated in detail by CT scan, ultrasound, and endoscopy. CT reconstruction, ultrasound examination, and endoscopy showed that the designed phantom mimics a real kidney's detailed anatomy and correctly corresponds to the targeted human cadaver's upper urinary tract. Soft materials with a tensile modulus of 0.8-1.5 MPa as well as biocompatible hydrogels were used to mimic human kidney tissues. We developed a method of constructing 3D organ models from medical imaging data using a 3D wax printing and molding process. This method is cost-effective means for obtaining a reproducible and robust model suitable for surgical simulation and training purposes.

Right lump kidney with varied vasculature and urinary system revealed by multidetector computed tomographic (MDCT) angiography.

Renal ectopia also known as ectopic kidney is an embryological renal anomaly characterised by abnormal anatomical location of one or both of the kidneys. This can occur in various forms such as (1) crossed fused renal ectopia, (2) ectopic thoracic kidney and (3) pelvic kidneys. The lump kidney is one of the six variations of crossed fused ectopic kidney. Throughout life, the patient may remain asymptomatic, however, symptomatic patients may present with minor traumatic injuries due to the abnormal location or normal kidney pathologies. During normal embryological development, there is cephalic migration during which the kidneys ascend to their normal retroperitoneal location; therefore, an ectopic location is as a result of arrested migration. During this embryological development the kidney has multiple aorto-illiac branches, which degenerate when the kidney reaches its normal location. Here they develop new renal branches from the aorta and during an arrested ascent the ectopic kidney tends to retain some of the older aorto-iliac vessels. Hence, the arterial supply and the veineux drainage are grossly abnormal, reflecting the metanephric malascent and the primitive vascular arrangement. The collecting systems also present with important anatomical variations. We present an extremely rare case of right lump kidney with six renal arteries, two renal veins and two duplicated pelvicalyceal systems.

Robot assisted heminephrectomy for duplicated renal collecting system: technique and outcomes.

BACKGROUND: The aim of this study was to describe experience with robotic assisted hemi-nephrectomy (RAHN) for the management of duplicated renal collecting system. METHODS: The authors' institutional database was queried to identify patients who underwent RAHN from 2007 to 2013 for the management of a duplicated system. Patient demographics, indications and surgical outcomes were reviewed and analyzed. RESULTS: Five patients were identified with a duplicated system of which all had a history of pain and recurrent infection. Three patients underwent hemi-nephrectomy for a poorly functioning lower pole and two for an abnormal upper pole moiety. All operations were completed successfully using a robotic approach. There were only two minor (Clavien grade 1-2) complications. CONCLUSIONS: RAHN is a viable option for the surgical treatment of an atrophic, chronically infected duplicated system. The unique features of robotic technology offer enhanced dexterity and vision facilitating the management of complex vascular and ureteral anatomy.

CT urography for the diagnosis of medullary sponge kidney.

BACKGROUND: Medullary sponge kidney (MSK) is characterized by malformation of the terminal collecting ducts and is associated with an increased risk of nephrolithiasis, nephrocalcinosis, urinary tract infections, renal acidification defects, and reduced bone density. It has been historically diagnosed with intravenous pyelography (IVP), which is falling out of favor as an imaging modality. CT urography (CTU) performed with multidetector CT (MDCT) has been shown to create images of the renal collecting system with similar detail as IVP; however, its utility in diagnosing MSK has not been defined. CASE REPORT: We present the first 15 patients with recurrent symptomatic nephrolithiasis who were evaluated in our renal stone clinic with CTU. Four patients were diagnosed with MSK after visualization of the characteristic radiologic findings. DISCUSSION: CTU effectively demonstrates the characteristic radiologic findings of MSK including collecting tubule dilatation, medullary nephrocalcinosis, nephrolithiasis, and medullary cysts. Dose reduction protocols can reduce radiation exposure below that associated with conventional IVP. We propose CTU be considered for the diagnosis of MSK.

Should blunt segmental vascular renal injuries be considered an American Association for the Surgery of Trauma Grade 4 renal injury?

BACKGROUND: Renal segmental vascular injury (SVI) following blunt abdominal trauma is not part of the original American Association for the Surgery of Trauma (AAST) renal injury grading system. Recent recommendations support classifying SVI as an AAST Grade 4 (G4) injury. Our primary aim was to compare outcomes following blunt renal SVI and blunt renal collecting system lacerations (CSLs). We hypothesize that renal SVI fare well with conservative management alone and should be relegated a less severe renal AAST grade. METHODS: We retrospectively identified patients with SVI and G4 CSL admitted to a Level 1 trauma center between 2003 and 2010. Penetrating trauma was excluded. Need for surgical intervention, length of stay, kidney salvage (>25% renal preservation on renography 6-12 weeks after injury), and delayed complication rates were compared between the SVI and CSL injuries. Statistical analysis used χ, Fisher's exact, and t tests. RESULTS: A total of 56 patients with SVI and 88 patients with G4 CSL sustained blunt trauma. Age, Injury Severity Score (ISS), and length of stay were similar for the two groups. Five patients in each group died of concomitant, nonrenal injuries. In the G4 CSL group, 15 patients underwent major interventions, and 32 patients underwent minor interventions. Only one patient in the SVI group underwent a major intervention. The renal salvage rate was 85.7% following SVI versus 62.5% following CSL (p = 0.107). CONCLUSION: Overall, surgical interventions are significantly lower among the SVI cohort than the G4 CSL cohort. Further analysis using a larger cohort of patients is recommended before revising the current renal grading system. Adding SVI as a G4 injury could potentially increase the heterogeneity of G4 injuries and decrease the ability of the AAST renal injury grading system to predict outcomes, such as nephrectomy rate. LEVEL OF EVIDENCE: Epidemiologic study, level IV.

Nephrostomy in early posttransplantation period in patients with nonfunctional graft and nondilated collecting system.

BACKGROUND: The purpose of our study was to determine whether renal function can be restored by early nephrostomy in patients who fail to develop renal function immediately after transplantation. METHODS: Between 2001 and 2010, we have performed external/internal nephrostomy in 13 patients during the early posttransplant period. The reason for the procedure was graft nonfunction with oliguria/anuria in the presence of normal renal perfusion, absence of signs of rejection, and/or renal collecting system dilation. The nephrostomy was created under ultrasound/fluoroscopic guidance. RESULTS: Nephrostomy was technically successful in all cases. On days 1-3 following nephrostomy creation, 10 of 13 patients showed an increase in diuresis and subsequent graft function development. Once the obstruction had been removed, the graft remained functional for months up to years posttransplantation. Three patients failed to respond to nephrostomy. There were no nephrostomy-related complications. CONCLUSIONS: Our study documents that, in patients who failed to develop graft function posttransplant for unknown reasons, nephrostomy may result in graft function development.

Segmentation of renal parenchymal area from ultrasound images using level set evolution.

This paper presents a framework for segmentation of renal parenchymal area from ultrasound images based on a 2-step level set method. We used distance regularized level set evolution method to partition the kidney boundary, followed by region-scalable fitting energy minimization method to segment the kidney collecting system, and determined renal parenchymal area by subtracting the area of the collecting system from the gross kidney area. The proposed method demonstrated excellent validity and low inter-observer variability.

Current computed tomography techniques can detect duct of Bellini plugging but not Randall's plaques.

OBJECTIVE: To assess the ability of noninvasive computed tomography (CT) scans to detect interstitial calcium phosphate deposits (Randall's plaques) and duct of Bellini plugs, which are possible stone precursor lesions. METHODS: At time of percutaneous nephrolithotomy (PCNL) for stone removal, all accessible individual papillae of 105 patients were endoscopically visualized and video recorded. Image-processing software was used to estimate the percentage of papillary surface occupied by plaque or plug in each pole (upper, middle, lower). The location of stones was also recorded. A radiologist blinded to the mapping results scored presurgical (n = 98) and postsurgical (n = 105) abdominal CT scans for the presence or absence of calcification by pole. RESULTS: The cohort was a mean age of 56 years (range, 23-84 years). Maximum papillary surface area of each area of the kidney occupied by plug correlated with CT calcifications on pre- and postprocedure images by rank sum test. However, maximum plaque surface area did not correlate with radiographic findings (P = .10-.90 for each pole by rank sum test). Sensitivity was 81% and specificity was 69% of CT to detect plugs of at least 1% of the papillary surface area. CONCLUSION: Calcifications seen on current generation clinical CT scans correspond to ductal plugging involving at least 1% of the papillary surface area. Current clinical CT scan technology appears inadequate for detecting Randall's plaques.

Robot-assisted upper pole nephrectomy in adult patients with duplicated renal collecting systems.

Duplicated renal collecting systems are a common congenital anomaly, with patients usually presenting in childhood, rarely in adult life. To our knowledge, only one case of robot-assisted heminephrectomy in adults has been described. We reviewed the medical records of four adult patients with symptomatic unilateral duplicated collecting systems and nonfunctioning upper pole renal units (NFUPRU) who underwent robot-assisted laparoscopic heminephrectomy at our institutions. Heminephrectomy for NFUPRU should be approached differently than partial nephrectomy, because of complex vascular anatomy. Patients who are undergoing this procedure have brief hospital stays, minimal morbidity, preservation of renal function, and resolution of symptoms. Robot-assisted laparoscopy is well suited for this procedure because it allows improved visualization during dissection of the unique vascular anatomy as well as scaling of surgeon movements and improved ergonomics.