Spontaneous perirenal hemorrhage (Wunderlich syndrome): An analysis of 28 cases.

INTRODUCTION: This study aimed to analyze the characteristics, etiology, and treatment of a series of patients with spontaneous perirenal hemorrhage (Wunderlich syndrome [WS]). METHODS: We retrospectively reviewed the records of 26 patients hospitalized for WS in a tertiary urological center between 2011 and 2018. All patients were evaluated for perirenal hemorrhage observed on computed tomography (CT) in the emergency department. Clinical variables (age, underlying diseases, symptoms, shock, and hospitalization period), laboratory test results, and radiological and pathological results were reviewed. RESULTS: The series included 28 events from 26 patients with a mean follow-up period of 20.2 ±â€¯18.0 months. Flank pain was most common symptoms (92%). Twelve patients (46%) had visible renal lesions and associated hematoma and 14 only showed perirenal hematoma. In six patients with shock (systolic blood pressure < 90 mm Hg), 2 underwent emergency angioembolization. Twelve patients (46%) underwent exploration and total nephrectomy. In the final diagnosis, 4 cases of renal cell carcinoma, 3 of angiomyolipoma, 4 of simple renal cyst, 2 of acquired cystic kidney disease, 4 of sarcoma or other malignancy, 4 of chronic pyelonephritis, and 5 of idiopathic WS were observed. Patient age was associated with prediction of renal cell carcinoma in the patients with WS. CONCLUSION: Renal masses are the main cause of WS, and CT is the diagnostic procedure of choice. Old age is a possible risk factor for renal cell carcinoma in etiology of WS. Surgical treatment is preferred in patients diagnosed with renal malignancy and in cases of hemodynamic instability.

Inherited renal diseases.

Genetic disorders of the kidney include cystic diseases, metabolic diseases and immune glomerulonephritis. Cystic diseases include autosomal dominant and recessive polycystic kidney disease (ADPKD, ARPKD, respectively). Neonates with enlarged, cystic kidneys should be evaluated for PKD. Patients with ADPKD have cysts and renal enlargement. Most patients present with hypertension, hematuria or flank pain; the most common extrarenal manifestation is polycystic liver disease. Oligohydramnios, bilaterally enlarged kidneys and decreased urine are featured in utero in ARPKD. Medullary sponge kidney is uncommon and features nephrocalcinosis, recurrent calcium stones and a history of polyuria/nocturia and/or urinary tract infections. Alport syndrome (AS) is an inherited disease of the glomerular basement membrane that is usually inherited as an X-linked dominant trait. Most patients with AS present in the first two decades of life with persistent microscopic or gross hematuria. Later, proteinuria is seen and its presence portends disease progression. Other findings may include sensorineural hearing loss and ocular abnormalities. There are various inherited tubulopathies, including Bartter syndrome, a group of renal tubular disorders that consist of two phenotypes with four genotypes. Patients usually present early in life with salt wasting, hypokalemia and metabolic alkalosis. Other features, depending on genotype, may include polyhydramnios and premature birth. Gitelman syndrome is also a salt-losing tubulopathy characterized by hypokalemic alkalosis. The majority of patients with Gitelman syndrome present during adolescence or early adulthood.

Active surveillance for renal angiomyolipoma: outcomes and factors predictive of delayed intervention.

OBJECTIVE: To present the outcomes of active surveillance (AS) for renal angiomyolipomas (AMLs) and to assess the clinical features predicting delayed intervention of this treatment option. PATIENTS AND METHODS: We retrospectively reviewed the outcomes of patients diagnosed with AMLs on computed tomography (CT) who were managed with AS at our institution. The AS protocol consisted of 6- and 12-month, then annual follow-up visits, each one including a physical examination and CT imaging. Discontinuation of AS was defined as the need or decision for an active procedure during the follow-up period. Causes of delayed intervention, as well as the type of active treatment (AT), were recorded. Clinical features at presentation of patients failing AS were compared with those who remained under AS at the time of the last follow-up. Predictive factors of delayed intervention were analysed using univariate and multivariate Cox regression models. RESULTS: Overall, 130 patients were included in the analysis, of whom 102 (78.5%) were incidentally diagnosed, while 15 (11.5%) and 13 patients (10%) presented with flank pain and haematuria, respectively. After a mean (sd) follow-up of 49 (40) months, 17 patients (13%) discontinued AS and underwent AT. Patients who underwent delayed intervention were more likely to present with a higher body mass index, larger tumours and symptomatic disease. Angioembolization represented the first-line AT after AS (64.7%), whereas partial nephrectomy was adopted in 29.4% of patients. On the univariate analysis, risk factors for delayed intervention included tumour size ≥4 cm, symptoms at diagnosis, and history of concomitant or contralateral kidney disease. On the multivariate analysis, only tumour size and symptoms remained independently associated with discontinuation of AS. CONCLUSIONS: Tumour size and symptoms at initial presentation were highly predictive of discontinuation of AS in the management of AMLs. Selective angioembolization was the first-line option used for AT after AS was discontinued.

Atypical presentation of xanthogranulomatous pyelonephritis: a case report.

Xanthogranulomatous pyelonephritis (XPN) is a rare form of chronic pyelonephritis, which is usually caused by calculous obstructive uropathy. We present a previously healthy 45-year-old housewife, who was admitted to The Dammam Central Hospital, Dammam, Saudi Arabia with left loin pain and increased frequency of micturition of four days duration. She also had icterus and features of disseminated intravascular coagulation. Abdominal ultrasound and computed tomography of the abdomen was suggestive of XPN. She responded well to treatment with antibiotics and nephrectomy. Histology of the resected kidney confirmed a diagnosis of XPN. Our case suggests that the diagnosis of XPN should be kept in mind when a middle-aged female patient presents with unilateral non-functioning hydronephrotic kidney, which is totally distorted, and has enhancing as well as non-enhancing regions on computed tomography.