4 chapter
Abnormalities of the urinary and male reproductive systems
4.1. abnormal development of kidneys abnormalities of development is the congenital deviation from normal development of one or more organs at the same time. Abnormalities of genitourinary system occur in 10–14 % of newborns. They are observed most frequently (up to 35–40 %) among all abnormalities of development. Some of them are not manifested throughout their lives, while others lead to disability from an early age.
classification The most complete classification of kidney abnormalities corresponds to modern requirements proposed by M. O. Lopatkin and O. V. Lyulko in 1987: i. abnormalities of renal vessels 1. Abnormalities of the number and location (dystopia). Abnormalities of renal arteries: a) Accessory renal artery; b) Duplication of renal artery; c) Plural renal arteries. Anomalies of renal arteries location: a) Lumbar dystopia; b) Iliac dystopia; c) Pelvic dystopia. 2. Abnormalities of form and structure of the renal arteries: a) Aneurysms; b) Fibromuscular stenosis. 3. Congenital arteriovenous fistulas. 4. Congenital abnormalities of renal veins: a) Abnormalities of the right renal vein (optional and multiple veins, confluence of the testicular veins into the right renal vein); b) Abnormalities of the left renal vein: сircumaortic ring, retroaortic, extracaval annular; c) Abnormalities of patterns of renal vein (renal vein stenosis). ii. abnormalities of number of kidneys 1. Aplasia. 2. Duplication (full and partial). 3. Accessory (third) kidney.
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iii. abnormalities of kidney size: hypoplasia iv. abnormalities of location and form of the kidneys 1. Dystopia of kidney: a) Unilateral (thoracic, lumbar, iliac, pelvic); b) Cross localization. 2. Fusion of kidneys: a) Unilateral (I-shaped kidney); b) Bilateral (symmetric – horseshoe kidney, cake kidney, asymmetric – L- and S-shaped kidney). v. abnormalities of renal structure 1. Dysplasia: rudimentary, dwarf kidney. 2. Multicystic kidney. 3. Polycystic kidney disease: a) Adult polycystic kidney disease; b) Infantile polycystic kidney disease. 4. Parapelvic cyst, calyceal, pelvic cysts. 5. Caliceal-medullary abnormalities: a) Megacalyx, polymegacalyx; b) Sponge kidney. vi. associated abnormalities of kidneys a) With vesico-ureteral reflux; b) With infravesical obstruction; c) With vesico-ureteral reflux and infravesical obstruction; d) With abnormalities of other organs and systems – reproductive, musculoskeletal, cardiovascular, digestive.
abnormalities of renal vessels Abnormalities of renal vessels are observed most frequently among all upper urinary tract abnormalities (up to 70–80 %).
accessory renal artery Accessory renal artery is an anomaly of development, which is manifested in the presence of one or few arteries with smaller caliber. It may depart from the main renal artery, abdominal aorta, and common iliac, abdominal, diaphragmatic, adrenal arteries and supply the blood to the upper or lower segment of the kidney. The accessory renal artery of lower pole frequently displaces and mechanically clamps ureter, violating urinary flow from kidney causing its hydronephrotic transformation. Circulation of the kidneys is not violated, taking into account much higher pressure in the arteries than in the ureter. The urine stasis in the kidney leads to the appearance of such complications as hydronephrosis, pyelonephritis, and urolithiasis.
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clinical presentation. It depends on the complications occuring due to obstruction of the ureter. It’s asymptomatic in most cases. It is manifested more frequently by signs of hydronephrosis, pyelonephritis, and urolithiasis. The main clinical manifestations: y A dull aching pain (from appropriate side); y Intermittent fever (associated with periodic exacerbations of chronic pyelonephritis); y Hematuria (usually microhematuria); y Presence of tumor formation in the retroperitoneal area or in the corresponding part of abdominal area (in the case of large hydronephrosis). diagnostics 1. Physical examination: a) Palpation (presence of tumor formation in the region of the kidneys); b) Percussion (percussion of dullness of the kidney). 2. Laboratory Methods: a) Urinalysis (proteinuria, leukocyturia in cases of chronic pyelonephritis accession); b) Evaluation of the urea and creatinine concentration in blood plasma (increasing in the case of chronic renal insufficiency). 3. Radiographic methods: a) Intravenous (excretory) urography. A break of contrast in the area of pyelo-ureteric junction is the typical feature of this abnormality, that is often associated with symptoms of hydronephrosis. The contour of renal pelvis in the projection of ureteral discharge has a clear rounded shape (Fig. 4.1); b) CT scan that specifies the anatomical features of the accessory arteries, the stage of hydronephrosis; c) Renal angiography allows to set the number and location of accessory vessels, their areas of blood supply of the kidney. 4. Ultrasound with Doppler effect (see color insert Fig. 4.2). treatment. Tactics of treatment is determined in the case of associated complications. The operation is performed in the case of progression of hydronephrosis, danger of loss of kidney function.
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Fig. 4.1. Accessory inferior polar vessel: a – retrograde ureteropyelogram, b – excretory urogram
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The main surgery option is antevasal ureteroanastomosis – incision of the ureter at the site of clamping (which, if necessary, supplemented by resection of the narrowed areas), and its subsequent linking to the vessel. This movement of the ureter removes the pathological pressure on it and restore normal flow of urine in most cases. With the considerable expansion of pelvis, simultaneous resection of part of pelvis wall is performed, that prevents stasis of urine. It should be noted that, that it is easier to cut the vessel and release pressure from the ureter without dissection, but this intervention is rarely done, as there is a risk of circulatory disorders of the lower pole of the kidney. There are also several ways to move up the artery to the pelvis level.
accessory superior polar artery
Fig. 4.3. Accessory superior polar vessel of the right kidney. Symptom Fraley
The intrarenal artery usually passes in the area of the upper neck of the calyx. The clinical significance is in possibility of urine stasis in the upper calyx with the associated complications as chronic pyelonephritis, and nephrogenic hypertension. There are no clinical manifestations of accessory upper pole artery, and it is very similar to the manifestations of accessory lower pole artery. The main method of diagnosis is excretory urography. Fraley syndrome is manifested with it as a break of contrast at the level of upper calyx neck, with its extension (Fig. 4.3). treatment. The main method of treatment is active observation. The surgical treatment is indicated in case of complications. The operation is quite complicated. The resection of upper pole is usually performed and more rarely – the plasty of vessels.
abnormalities of renal artery location (dystopia) The location of the renal artery divergence is the determining factor. Lumbar dystopia is formed in low divergence of the renal artery from the aorta in the lumbar region. The iliac dystopia is formed in place of divergence from the common iliac artery. The pelvic dystopia is formed in place of divergence from the internal iliac artery. Treatment is necessary only in cases of crossroads with ureter and as a result – the appearance of renal obstructive symptoms (plasty of ureter).
aneurysm of renal artery Aneurysm is the anomaly of the renal artery form and structure. The aneurysm is located in the extrarenal part of renal artery in the most cases. It is associated with the absence or
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underdeveloped muscular layer of arterial wall. The anomaly can cause nephrogenic hypertension. The treatment is surgical and lies in excision of the aneurysm.
Fibromuscular stenosis of renal artery The fibromuscular stenosis is manifested in the form of one or more narrowings along the renal artery. They are associated with excessive fibrosing of its walls and elevated tone of muscular layer, due overdevelopment. This is one of causes of nephrogenic arterial hypertension. The operative treatment lies in excision of the stenossed sections of artery or prosthetic renal artery.
congenital arteriovenous fistula It is observed more often between segmental artery and vein there are single and multiple congenital arteriovenous fistulas. It is manifested clinically by hypertension, varicocele, and intermittent hematuria as result of the formation of fistulas and venous hypertension.
abnormalities of renal veins The abnormalities of the right renal vein (accessory and multiple veins) usually don’t have clinical significance, except of falling of testicular veins in the right renal vein, that is manifested as right-sided enlargement of spermatic cord veins (varicocele). The abnormalities of the left renal vein (annular, retroaortal, extracaval confluence) are manifested by violations of venous drainage and venous hypertension due to the compression of the aorta. Furthermore, abnormal renal vein may overlap with the ureter causing the certain stage of its obstruction. It disrupts the flow of urine from the kidneys and causes the hydronephrotic transformation. The treatment strategy in these cases depends on the degree of obstruction and clinical symptoms. Stenosis of the renal vein is the cause of venous stasis in the kidney, renal blood circulation and appearance of hematuria, proteinuria, and hypertension.
abnormalities of number of kidneys Abnormalities of number of kidneys are the most common (10–15 % of all renal abnormalities). renal agenesis (aplasia) Renal agenesis reaches 1 % of all abnormalities. It is fixed 2 times more often in women than in men. Renal agenesis is observed in 1 case for 1100 autopsy according to the autopsy data. Renal aplasia is a complete lack of kidney and renal vessels. It is always observed as compensatory hypertrophy of the opposite kidney. There is no clinical significance. There are no complications if the opposite kidney functions normally. duplication of kidneys Duplication of kidneys takes about 10 % of all kidney abnormalities. The duplex kidney occurs 2 times more often in females than in males. It is observed in 1 case per 150 autopsies according
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to the autopsy. The duplex kidney is unilateral in 90 %, and bilateral in 10 %. There are differences between the duplex kidney and accessory kidney. In the first case, the duplex kidney is slightly bigger than normal. The two halves of it are often separated by moderate sulcus. They have individual blood supply, venous and lymphatic flow, but anatomically it is one organ. Accessory kidney is totally independent unit that can be located at different levels and is not associated with the other two kidneys. There are distinguished: complete and incomplete duplication of kidney. There are two cavitary systems in incomplete duplication (upper underdeveloped system) with two ureters (Fig. 4.4). The ureters fall apart to the bladder (duplication of the ureters), or are connected over it (split ureter). The incomplete duplication of kidney has the main pyelocaliceal system and additional pyFig. 4.4. Excretory urogram in 15 min. elocaliceal system that is located above it (Fig. 4.5). Complete type of the left duplex The additional pyelocaliceal system is mostly underkidney, with the splitting of the left developed. It has not clearly separated large calyces ureter. Concrement of the upper third and is connected with the main pyelocaliceal system. section of right ureter. The right-sided hydronephrosis It is quite often difficult to distinguish branching, ramified upper calyx and incomplete duplication of the kidneys. clinical presentation. This anomaly is not evident clinically in most cases. There is stasis of urine in the additional pyelocaliceal system of kidney in some patients. This is related with stenosis in the location of the confluence of ureter, and the occurrence of ureterorenal and intrarenal reflux. Such situation leads to chronic inflammation, lithogenesis, and hydronephrosis of additional pyelocaliceal system of the kidney. diagnosis is based on the clinical manifestations of the disease and data of supporting instrumental methods. Basic methods of diagnostics: y excretory urography; y ultrasound examination; a b y CT scan; y cystoscopy. Fig. 4.5. Incomplete duplication of kidney: Additional diagnostic methods: a – infusional urogram in 10 min; y MRI; b – excretory urography in 7 min
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y retrograde ureteropyelography (in case of the absence of kidney function or uninforming of excretory urography). treatment. The conservative treatment is associated with the treatment of possible complications (hydronephrotic transformation of kidneys, acute and chronic pyelonephritis, and urolithiasis). The operative treatment is performed only in the absence of effect in conservative treatment of complications. It includes the hemiresection of additional pyelocaliceal system of kidney, and rarely the plasty of ureter (hydronephrosis in kidneys with frequent exacerbations of chronic pyelonephritis).
accessory kidney It is always unilateral malformation. The accessory kidney has independent blood supply, venous, lymphatic outflow and ureter, that either flows into the bladder by separate orifice lower and medially from basic orifice of ureter or fuses with the ureter of main kidney in the type of split ureter. It is not clinically evident and does not require treatment.
abnormality of kidney size renal hypoplasia is the reduction of kidneys in the size with maintaining of morphological structure and functional capacity. It is observed in 1 % of all renal abnormalities. The reduction of kidneys is accompanied by a corresponding decrease of functioning nephrons up to 50 % than in normal kidney. It is accompanied by compensatory hypertrophy of the opposite kidney. It is not clinically evident and does not require treatment.
abnormalities of kidney location renal dystopia is observed in 3 % of all renal abnormalities. It is caused by violation of renal embryogenesis at the stage of migration from pelvic (where they are formed) and lumbar (where they are in adults) area. There are two major changes in the kidneys during this process: 1. Change of the kidney location. 2. Rotation of kidney (rotation of kidney from sagittal plane to front plane). The violation of one of these processes or both may occur in dystopia. The dystopia according to the location level can be pelvic, iliac, lumbar, thoracic and crossed. All kinds of dystopia (except lumbar) may be accompanied or not by incomplete rotation. Lumbar dystopia has normal location of kidney, but its rotation is necessarily incomplete (Fig. 4.6). classification of dystopia composed according to the principle of divergence level of renal arteries from the aorta: y Pelvic dystopia occurs when renal artery departs from the internal iliac artery. The kidney is located in the pelvis between the bladder and the rectum (Fig. 4.7). y Iliac dystopia occurs when renal artery departs from the common iliac artery. The kidney is located in the iliac fossa.
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a
b
Fig. 4.6: a – excretory urogram in 10 min. Lumbar dystopia of both kidneys (rotation of right kidney to 90°, the left kidney – to 180°); b – retrograde ureteropyelogram (rotation of left kidney – 90°)
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Fig. 4.7. Pelvic dystopia of kidney: a – retrograde ureteropyeloghram; b – magnetic resonance scan of 3D-modeling
y Lumbar dystopia occurs when normal discharge of the renal arteries is accompanied by incomplete rotation of the kidney. y Thoracic dystopia occurs when renal arteries are divergenced at the 12th thoracic vertebra. A variation of this is a subdiaphragmatic dystopia in which the kidney is located highly under the diaphragm, but not in the thoracic cavity.
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y Crossed renal dystopia occurs when the kidney is located on the opposite side near or at a distance from contrlateral kidney. There is unilateral and bilateral crossed renal dystopia (Fig. 4.8). This kidney has normal functional ability, and there are different pathological processes in it over the years. The violation of the urine outflow is a main basis for this. It is occured due to crossing of ureter by abnormal located vessels. The complications of it are pyelonephritis, hydronephrosis, urolithiasis and more. Most of kidneys with dystopia have abnormal blood flow. The pelvic dystopia has the most clinical significance. It is associated with the location of kidney, compression of adjacent organs and structures, and high risk of injury. clinical presentation. It is usually associated with complications as hydronephrosis, pyelonephritis, urolithiasis. There are no clinical manifestations of dystopia in most cases. Fig. 4.8. Cross renal dystopia of treatment. It does not require treatment if clinical right kidney. Bilateral retrograde symptoms are absent. Conservative treatment is indicated ureteropyeloghram in case of complications that described above. Surgical treatment is indicated only in severe complications and usually involves the removal of concrement or plasty of vesico-ureteral segment. Such operations are more difficult technically from similar interventions on normal kidney due to violation of anatomical landmarks. renal fusion anomaly is observed in 3–4 % of all renal abnormalities. It is caused by close anatomical contact between two existing kidneys. They are almost impossible to separate one from other. Renal fusion: a) unilateral – L-shaped kidney; b) bilateral (symmetrical – horseshoe kidney, lump or cake kidney, and asymmetrical – Land S-shaped kidney) (Fig. 4.9). There are 90 % of all renal fusion include horseshoe kidney (Fig. 4.10). 60 % of patients have abnormalities of other organs, in addition. The horseshoe renal fusion is in the presence of isthmus between its lower poles. Such kidney is located most often below the usual position. Isthmus is located at the level of L4–L5. The parenchyma of such kidney has normal structure as in previous abnormalities, but there are certain complications in 85–90 %. They are not associated only with violation of the outflow of urine from the kidney but also with the pressure of isthmus on the surrounding structures as retroperitoneal vessels, nerve plexus, intestine. This anomaly may cause the abnormalities of the other systems and organs, except urological complications. clinical presentation. Quite often there is dull abdominal pain, dysfunction of the gastrointestinal tract, sometimes venous and lymphatic stasis of the lower extremities is observed. The psychological disorders occure in result of the long-term pain syndrome with repeated
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Fig. 4.9. L-shaped kidney
Fig. 4.10. Horseshoe kidney: 3D-computer simulation
treatment (by gastroenterologist) and without effect. An approach to diagnosing and treatment of this anomaly is the same as in other renal abnormalities. diagnostics a) The basic instrumental methods of diagnostics: y excretory urography (Fig. 4.11);
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y ultrasound examination; y CT scan; y isotope renography. b) Additional diagnostic methods: y MRI; y renal angiography; y dynamic renal scintigraphy. differential diagnosis: a) with renal dystopia (a clear separation of kidneys is fixed on excretory urography or CT); b) with the tumor of kidney (typical macrohematuria, pain, significant asymmetric increasing in size of kidney, pathological angiomatosis in the area of the tumor in angiographic study). treatment. The main treatment is conservative. It is usually associated with such complications as hydronephrosis, pyelonephritis, urolithiasis, and hypertension. Surgical treatment may include removing of stones, plasty of ureteropelvical segment, resection of the isthmus.
Fig. 4.11. Excretory urogram in 15Â min. Horseshoe kidney. Stone of the upper third of the right half of the horseshoe kidney. Right-sided hydronephrosis
anomalies of renal structure Polycystic kidney disease Polycystic kidney disease relates to anomaly of kidneys structure and makes 10–17 % of all renal abnormalities. This is a serious hereditary anomaly, that is characterized by a large number of different-sized cysts in both kidneys. Violation the process of parenchymal formation, when there is no connection between the straight and convoluted tubules of nephrons is the cause of their formation. The urine formed in glomeruli has no outflow tract and is accumulated in the form of a large number of cysts. At the same time, some nephrons are formed normally and due to this process partial renal function is stored. There are two types of polycystic kidney disease: y infantile polycystic kidney disease; y adult polycystic kidney disease. For polycystic kidney disease there is typical significant damage of the parenchyma in both kidneys that combine with the creation of a large number of cysts in the liver, lungs, in pancreas and thymus. This anomaly is transmitted by an autosomal-recessive type. These children are born with signs of dysfunction not only in kidney and also in the liver, lungs. They usually die in the first year of life. The renal involvement is smaller with polycystosis of adults (Fig. 4.12) and cysts in other organs are also rare. This anomaly is transmitted by an
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Fig. 4.12. Polycystic kidney disease. Macropreparations
autosomal-dominant type. The clinical manifestations are rare in childhood. The disease is diagnosed between the ages of 20–40 years, when progressive deterioration of renal function leads to signs of chronic kidney disease. clinical presentation occurs in result of associated complications, that can be divided into the following groups: 1. Urologic: y chronic renal insufficiency; y pyelonephritis; y urolithiasis; y nephrogenic hypertension; y bleeding and suppuration of cyst; y secondary anemia. 2. Lesion of cardiovascular system as a result hypertension, diseases of the vascular wall: y thrombosis, thromboembolism; y dyselectrolytemia. 3. Neurological complications as a result of hypertension and vascular disease: y stroke; y polyneuropathy. Symptoms: y dull, aching pain in the lumbar region; y general weakness. Clinical disease is determined by the severity of certain groups of complications, but primarily the signs of chronic kidney disease and chronic pyelonephritis appear. Renal function becomes significantly worse at phase of acute pyelonephritis, and then chronic kidney disease
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occurs. In the later stages neurological and cardiac complications are joined, and level of calcium decreases. diagnostics i. urine changes 1. Hyposthenuria (density of urine 1.002–1.008); 2. Proteinuria (up to 1 g/l); 3. Leukocyturia (exceeding 10 leukocytes in view of the general analysis, or more than 4 thousand in Nechiporenko urinalysis). ii. Blood changes 1. Increasing of urea concentration in plasma (more than 8.3 mmol/l); 2. Increasing of creatinine concentration in plasma (more than 110 mmol/l); 3. Hypercalcemia, hyperkalemia, hyponatremia. iii. X-ray methods 1. Basical: y excretory urography; y ultrasonography (Fig. 4.13a); y CT scan (Fig. 4.13b). 2. Additional: y MRI; y renal angiography; y isotope renography; y dynamic nephroscintigraphy.
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Fig. 4.13. Polycystic kidney disease: a – ultrasonogram, b – CT scannogram
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