History

Urinary disease presents with a relatively small number of defined symptoms as presenting problems. Patients may present with lower urinary tract symptoms (LUTS) that are subcategorised as storage or irritative symptoms (urinary frequency, urgency, nocturia, dysuria), voiding or obstructive symptoms (change in strength of the urinary stream) and incontinence. Ongoing obstructive LUTS may eventually present as retention of urine. Haematuria may be due to benign or malignant disease; renal pain (colic) results from obstruction, most often with ureteric calculi. Occasionally there may be recognition of a renal mass. However, the majority of renal masses are now detected as incidental findings on abdominal imaging performed for investigation of often unrelated symptoms. Prostate cancer is increasingly detected in the context of overall health assessments or as a case finding during assessment of LUTS. A careful assessment of the history often suggests the diagnosis, which is usually supported by an imaging modality.

Common pathologies are congenital anomalies, functional disorders and specific malignancies, including those affecting children. Other malignancies of the urinary tract and prostate increase in frequency with age. Urinary tract trauma and infections are common to all ages. Bladder neck obstruction from prostatic disease is the most common problem in the elderly male.

Patients should be assessed for evidence of renal failure (Ch 10.7). Symptoms of chronic renal failure include nocturnal polyuria and a constellation of nonspecific symptoms: anorexia, nausea and vomiting, headache, visual disturbances, lethargy, sallow skin, oedema and general malaise.

Physical examination

Physical examination is often normal in patients with urinary tract disease. Detection of abnormalities involves identification of renal or bladder masses, together with examination of the lower urinary tract in both sexes. Imaging is often then required.

Renal masses are usually found to be due to simple renal cysts, tumours or obstruction causing hydronephrosis. Abdominal examination may reveal a unilateral renal mass or the bilateral masses of polycystic kidneys.

A renal swelling has the following characteristics:

Figure 9.1 Palpation of right kidney

Digital rectal examination (DRE) may reveal the changes of benign prostatic hypertrophy, prostatitis or carcinoma of the prostate. The external genitalia should also be examined. As with all physical examination, appropriate consent is obtained and privacy should be provided.

Diagnostic tests

Several special investigations are of critical importance. They range from simple examination of the urine (which must never be omitted) to sophisticated functional and imaging tests. Those most commonly employed are:

Figure 9.2a Cystoscopy and ureteric catheterisation

A: tip of cystoscope with elevating bridge; B: trigone of bladder; C: ureteric oriface; D: interureteric bar

Figure 9.2b Normal right ureteric orifice as seen on cystoscopy

Figure 9.2c Right ureteric orifice with guidewire in situ

Causes

Clinical assessment

Diagnostic plan

On presentation to hospital, the diagnosis is usually made after clinical history, examination and then urine dipstick with a commercial kit with positive for red blood cells is demonstrated and infection largely excluded by the absence of nitrites. The imaging modality to confirm the diagnosis will then usually be a non-contrast spiral computed tomography (CT) scan of the abdomen and pelvis. An accompanying plain abdominal X-ray is helpful in planning treatment and elucidating if the stone is radio-opaque or radiolucent. The X-ray may demonstrate an opaque calculus (85% of urinary calculi are radio-opaque — Box 9.1), which needs to be distinguished from phleboliths and other opacities. The CT findings consistent with an obstructing stone include perinephric fat stranding, dilatation of the renal pelvis and/or ureter and identification of the stone itself. The presence of the contralateral kidney should be sought and the size and position of other calculi that appear bright white should be noted. Urine should then be sent for formal microscopy and culture to definitively exclude infection and quantitate the haematuria, and to look for crystals (oxalate). At the time of presentation, blood should be drawn for full examination, creatinine, urea and electrolytes to ascertain renal function and screen for metabolic abnormalities and serum uric acid; calcium and phosphate estimations are also useful screening tests for major metabolic abnormalities. Stone analysis is done if the stone is recovered. The patient is instructed to strain the urine to check for stone passage and obtain the stone for analysis.

Box 9.1

Types of urinary calculi

70% are calcium oxalate and phosphate in acid urine (radio-opaque)

15% are urate calculi in acid urine (radiolucent)

10% are magnesium ammonium phosphate in alkaline, usually infected urine (radio-opaque)

1% are cystine (partly radio-opaque)

Prior to the popularity of CT for diagnosis, which has the advantages of high sensitivity, speed, lack of contrast administration and ability to detect other intra-abdominal pathologies, intravenous urography (IVP) was used to confirm the diagnosis of urinary obstruction, with demonstration of the causative calculus, either as a radio-opaque shadow in line with the ureter or as a radiolucent filling defect (Figs 9.4a–c), or showing a dilated upper urinary tract as the aftermath of a stone that has passed. IVP is now rarely performed in most emergency departments but is a useful adjunct if the diagnosis is equivocal. Ultrasound can be helpful in excluding other intra-abdominal and pelvic lesions or to demonstrate and serially monitor upper urinary tract dilatation due to obstruction. Ultrasound is thus of particular value in children, in whom repeated X-rays should be avoided. Renal colic with symptoms and signs of pyelonephritis (fever, systemic toxicity) always requires urgent imaging. An obstructed and infected kidney requires urgent relief, whereas obstruction in the absence of infection can be observed over the course of a week or more without likelihood of renal parenchymal damage.

Figure 9.4a Ureteric stone

Right-sided obstruction from stone causing hydronephrosis.

Figure 9.4b CT stone coronal view of VUJ stone

Reproduced with permission from John Kourambas

Figure 9.4c IVP demonstrating proximal ureteric stone

Reproduced with permission from John Kourambas

Treatment plan

Management of urinary calculi

Most urinary calculi pass spontaneously, especially if they are small or less than 5 mm. Treatment is therefore initially expectant. The patient is treated with euvolemia, as pushing fluids will exacerbate pain, and observed at regular intervals by repeat imaging; a plain film if the stone was initially visualised this way will suffice. Additional CT, ultrasound or IVP may be necessary to confirm if the stone has passed. If the stone enters the bladder its spontaneous passage is usually assured; occasionally a stone subsequently impacts in the urethra causing acute stoppage of the urinary stream. Conservative management may be supported by agents that aid stone passage by ureteric relaxation such as an alpha-blocker (e.g. tamsulosin) or calcium channel antagonists (verapamil) alone or in combination.

Indications for stone removal (Box 9.2). Removal is indicated only when parenchymal damage is a concern, for example, with unresolved urinary infection or the stone seems very unlikely to pass spontaneously, as with large calculi (>1 cm diameter) or persisting pain without progress. It is also mandatory in the case of a solitary kidney, where anuria may ensue.

Box 9.2

Indications for removal/disruption of symptomatic renal or ureteric stone

Solitary kidney

Persistent symptomatic obstruction with urinary infection and danger of renal parenchymal damage

Large calculus (>1 cm) that is unlikely to pass spontaneously

Persistent symptomatic obstruction without improvement or onward passage over one or two weeks

Methods of stone removal. Stone removal is largely an endoscopic procedure via the upper or lower urinary tract depending on the site of the stone, with or without the use of an energy source to shatter the stone prior to removal (Fig 9.4d). The other key method of removal is extracorporeal shock wave lithotripsy (ESWL, Fig 9.4e). Rarely is open stone removal required (open ureterolithotomy, pyelolithotomy or anatrophic nephrolithotomy). Laparoscopic surgery may now be used for difficult, large, impacted ureteric stones that cannot be manipulated up or down. The following methods are most frequently used in the operative management of urinary tract calculi.

Figure 9.4d URS/ureteroscopic view of stone

Reproduced with permission from John Kourambas

Figure 9.4e Extracorporeal shock wave lithotripsy (ESWL)

Photo courtesy of Dornier MedTech GmbH

Cystoscopy and ureteroscopy with stone basket extraction is suitable for small calculi in the lower or intramural ureter that fail to pass despite persisting pain. Energy sources commonly used to fragment the calculi may include pneumatic lithotripsy or laser (holmium). The patient may require temporary stenting with a flexible, double J pigtail stent to guard against recurrence of colic from the oedema left by the stone itself or the procedure to remove it, which can be removed under local anaesthetic at a later date. Stent symptoms include frequency, urgency and loin pain, especially with voiding (stents cause reflux) and haematuria. Patients should be assessed for infection, bearing in mind a degree of red and white cell loss in the urine is consistent with the stent itself.

Extracorporeal shock wave lithotripsy (ESWL). A semi-ellipsoid reflector is used and shock waves are generated from the near focal point and directed to the distant focal point to converge on the calculus in the kidney or upper ureter to fragment it. Many shock waves are required (2500 to 3000) and patients are maintained under anaesthesia or with an epidural block to maintain constant position. Stones can usually be adequately fragmented at one sitting. Most fragmented stones will then be passed spontaneously and subsequently most patients remain stone-free. About 10% of patients require additional percutaneous, ureteroscopic or open surgery to remove residual renal or ureteric fragments. Stones in the middle or lower ureter are shielded by the bony pelvis and need to be manipulated into the pelvis or upper ureter if ESWL is to be used.

Percutaneous nephrolithotomy. A percutaneous nephrostomy tract is first established and a nephroscope introduced along the track. Small stones are removed with grasping forceps; larger stones are initially fragmented with an ultrasonic lithotriptor.

Percutaneous stone surgery and, more recently, ESWL have revolutionised management of renal stones. Most upper tract stones can now be removed by ESWL or percutaneous and endoscopic means. If all modalities are available, ESWL is successful for most stones and percutaneous techniques can deal with almost all the remainder.

Causes

Painless haematuria has a number of common and less common causes (Fig 9.5).

Figure 9.5 Common causes of painless haematuria

A: renal tumour; B: tuberculosis; C: transitional cell tumour; D: engorged prostatic veins

Clinical assessment and urine microscopy

In localising the most likely source of bleeding, help may be obtained from the history and by urine microscopy. Macroscopic haematuria arising from kidney or ureter is usually dark or smoky and evenly mixed with the urine. Blood entering from the bladder, prostate or urethra is usually brighter red. Bleeding from the bladder may be evenly mixed or terminal. From the prostate, bleeding may be heaviest initially or terminally; urethral bleeding is usually heaviest initially. Bleeding from the urethra or prostate may appear at the external urethral meatus apart from micturition and cause bloodstaining of underpants or pyjamas. It should be noted if clots are present; threadlike clots may signify upper tract bleeding. These characteristics are, however, only rough guides to the source of bleeding. The history taken must also check for other evidence of a bleeding tendency, drug intake including analgesics, recent streptococcal sore throat, oedema of face or limbs, family history and evidence of renal injury. Examination will concentrate on identifying renal masses or evidence of diseases associated with haematuria and should include taking the patient’s blood pressure, which may be elevated with glomerulonephritis. Commonly, a physical examination is unrewarding in finding a cause for haematuria.

Normal urine shows fewer than four erythrocytes per high power field in microscopy of fresh centrifuged specimens. Microscopy of a fresh specimen can distinguish between glomerular and urothelial erythrocytes. The former are irregular in outline and haemoglobin content (dysmorphic). The latter are usually undamaged circular cells with normal haemoglobin content or ghost cells of normal shape lacking haemoglobin. Dysmorphic cells are most easily recognised under phase-contrast microscopy. The presence of red cell casts or heavy proteinuria is also indicative of glomerular disease.

Diagnostic and treatment plan

Cases of haematuria require full investigation and the following are suggested.

Macroscopic haematuria must be considered as being caused by a urinary tract malignancy until proven otherwise. If macroscopic haematuria remains unexplained after full investigation, the urine should be examined frequently for malignant cytology and investigations repeated in a month.

Treatment depends on the cause. Haematuria rarely causes shock requiring transfusion or iron deficiency anaemia, except after trauma.

Microscopic haematuria is investigated along similar lines, but often percutaneous renal biopsy will be necessary to identify glomerular lesions.

Bleeding due to prostatic hypertrophy, acute glomerulonephritis, bleeding disorders and less common causes, such as renal papillary necrosis, hydronephrosis, congenital renal anomalies and renal tuberculosis, will usually be associated with other diagnostic features on clinical assessment and investigations.

Urothelial tumours. Urothelial tumours are usually transitional cell tumours. The rare, squamous cell carcinoma follows chronic infection and urolithiasis.

Transitional cell tumours result from diffuse and multicentric epithelial dysplasia, often secondary to urinary carcinogens. Cigarette smoking and heavy analgesic consumption are the common risk factors. Exposure to organic solvents in the dye and paint industries is a further risk, as is chronic bilharzial (schistosomiasis) infection in endemic areas. Tumours occur after middle age and are more common in men.

The bladder is the most frequent site but all urothelium is at risk. Tumour malignancy and prognosis exhibit a spectrum from fronded papillary tumours of low grade or medium malignancy that do not invade the lamina propria, to those that are sessile, ulcerated and invasive from the onset. These latter tumours spread by direct invasion, via lymphatics and at a later stage by the bloodstream. Simple staging into superficial disease and muscle-invasive disease is helpful in determining treatment and prognosis (Figs 9.6a and b).

Figure 9.6a Staging of bladder tumour

T1: confined to lamina propria; T2: invading the muscle wall of the bladder; T3: extending through the bladder wall; T4: involving other organs or nodal spread

Figure 9.6b Endoscopic photo of TCC

More precise staging is from Ta/T1 to T4, namely:

Endoscopic and surgical ablations are the preferred treatments for superficial disease (i.e. Ta and some T1 and Tis). These patients require permanent, repeated urinary cytology and endoscopic review to monitor progress.

Deeper tumours extending into the detrusor muscle need surgical resection with radical cystectomy and diversion of ureters to an ileal conduit or in some circumstances to a neobladder created from a bowel segment. Radiotherapy and endoscopic removal may be used as primary therapy in muscle-invasive disease with or without chemotherapy but is more often reserved for those unfit for radical surgery.

Radiotherapy is particularly helpful for palliation of advanced disease. Intravesical immunotherapy (BCG) or chemotherapy can control superficial and multiple bladder tumours and is usually indicated for Tis, recurrent Ta and some cases of T1 disease.

The BCG is given as an instillation after the insertion of a catheter with an aseptic, and in particular an atraumatic, technique. A six-week course of once weekly instillation is usually undertaken; in some cases a maintenance schedule is given beyond this. BCG is usually well tolerated but has some significant side effects including sepsis (usually in association with concurrent bacterial UTI and/or traumatic catherisation), common local side effects such as dysuria, frequency, macroscopic haematuria or less commonly granulomatous prostatitis, epididymo-orchitis and rarely in the long term, a small contracted bladder. Uncommon systemic side effects, such as pneumonitis, arthritis and hepatitis, are fortunately rare. For the prevention of potentially life-threatening sepsis all patients undergo urinalysis to check for infection prior to treatment each week and treatment is delayed if infection is detected.

Renal transitional cell carcinoma is most common in association with analgesic abuse and presents as haematuria or colic. Treatment is by nephroureterectomy with excision of a cuff of adjacent bladder. This is now more frequently laparoscopically assisted.

Renal cell carcinoma (RCC). RCC is an adenocarcinoma that is more common in men and in smokers, occurs usually after middle age, and spreads by direct extension into perinephric fat, the renal vein and vena cava and via lymphatic spread to locoregional nodes. Metastases to lung, adrenal or bone are the most common sites of advanced disease and may be solitary in nature.

RCC used to present with a classic triad of haematuria, pain and a renal mass. All are features of relatively advanced disease. However, the most common presentation now is occult disease found on imaging initiated for investigation of an unrelated problem such as upper abdominal discomfort, back pain or voiding symptoms. Rarely will a patient present with one of the paraneoplastic syndromes associated with RCC such as pyrexia of unknown origin (PUO), anaemia, hypercalcaemia or symptoms due to synthesis of hormones by the tumour (e.g. erythropoietin, renin, parathyroid hormone). As a result of the plethora of paraneoplastic syndromes associated with RCC, it is sometimes termed ‘the physician’s tumour’.

Investigation needs accurate imaging by triple-phase CT of the abdomen and pelvis to delineate the lesion and any locoregional invasion of nodes or veins or to identify adrenal or liver metastases and assess the contralateral renal unit for presence, size, stones, synchronous cancer or other pathology. Occasionally an ultrasound is also required if there is difficulty differentiating between a solid tumour and a solitary renal cyst (the most common mass lesion in the kidney). Rarely, percutaneous needle biopsy or cyst aspiration is required. Plain chest X-ray or chest CT is used to detect metastases.

The usual treatment is radical nephrectomy if the tumour is locally confined. Small RCCs are often now managed with laparoscopic radical nephrectomy. Where there is a requirement for nephron-sparing surgery, for example, in a single kidney, a partial nephrectomy can be performed. Cancer-specific survival figures for the management of small RCCs of less than or equal to 4 cm with partial nephrectomy are now equivalent to those for radical nephrectomy, making partial removal an alternative. Occasionally, radical nephrectomy can be combined with removal of a locally resectable single-lung metastasis.

Prognosis is good for small tumours, especially those less than 4 cm and even those up to 7 cm. If, however, there is extensive, histologically poorly differentiated (especially sarcomatoid) or metastatic disease, the prognosis is poor. About one-third of patients have metastases on initial diagnosis. Other poor prognostic parameters are lymph node spread and spread beyond the renal capsule. Renal vein extension at the time of nephrectomy has less prognostic significance and removal of intravenous extension of tumour is worthwhile. The tumour is resistant to radiotherapy and traditionally has been highly chemoresistant, though newer immunotherapy and tyrosine kinase inhibitors (sorafenib and sunitinib) based therapies are showing some promise with regard to small improvements in survival time for those with metastatic disease.

Wilms’ tumour (nephroblastoma). This is the second most common abdominal neoplasm of childhood and occurs in children under six years of age (mean age at presentation 3.5 years). Presentation is most frequently because of increased abdominal girth, abdominal mass or serendipitous finding on ultrasound performed for other indications. Children may have microscopic haematuria (25%) but macroscopic haematuria is uncommon.

Imaging by ultrasound usually enables the differentiation to be made from a hydronephrotic or cystic kidney and gives information on renal vein or caval involvement. CT or MRI scan of the abdomen and chest will confirm the diagnosis and provide staging information about retroperitoneal and lung involvement. Wilms’ tumours may be hereditary or sporadic and may be bilateral.

These tumours may be classified as having either favourable or unfavourable histology. They are sensitive to radiotherapy and chemotherapy and usually managed with a combination of surgery and chemotherapy. (In the US, most will have surgery followed by chemotherapy. In Europe and Australia, most will have induction chemotherapy to shrink the tumour, followed by surgery. Radiotherapy is reserved for chemoresistant tumours.) Prognosis has improved with multimodal therapy and high cure rates are possible.

Neuroblastoma is a common tumour in infants and children. It is a malignant tumour of the autonomic nervous system, particularly the adrenal gland. It usually presents younger than Wilms’ (mean age at presentation 1.5 years) and often occurs in neonates. It can be confused with Wilms’ tumour initially, with a rather similar clinical picture. However, these children are usually more unwell, with anaemia, weight loss, irritability, fever and pain. They have high levels of urinary vanillylmandelic acid (VMA) and the mass can extend across the midline and around major vessels. A neuroblastoma displaces the normal kidney and is often calcified. Surgical biopsy and complex chemotherapy is required; however, delayed definitive surgical resection in advanced disease rarely improves the poor outcome for this tumour.