(2) Giant ureter can be divided into reflux, obstruction and idiopathic, including various secondary and primary lesions. This section describes the primary obstructive megaureter, which is often accompanied by a non-functional segment of the terminal ureter, leading to functional obstruction. Occasionally there is anatomical obstruction (stenosis). The typical X-ray film shows that the upper ureter expands without distortion, while the lower ureter expands more obviously, which is spindle-shaped or spherical, and becomes a small section without expansion when it enters the bladder, which is about 0.5 ~ 4 cm long. The renal calices and renal pelvis are normal, and the renal function is basically normal. Ureterography showed that there was reverse peristaltic reflux of ureter, and only a small amount of contrast agent entered the bladder. But in severe cases, the whole ureter is extremely dilated, elongated and tortuous. Renal damage is more serious, and calyceal dilatation is generally more obvious in the renal pelvis. It is considered that the abnormal structure of the muscle layer at the end of ureter, the thickening of annular muscle and the lack of longitudinal muscle components are the causes of functional obstruction. Men are mostly on the left side, accounting for 15 ~ 25% on both sides. Often accompanied by other urinary tract malformations.
Urinary tract infection, fever and low back pain, calculus and hematuria, abdominal cystic mass or urography caused by developmental retardation are commonly found. Children's cases often have more obvious symptoms because of serious kidney damage. It should be treated as soon as possible. The diseased segment of the ureter can be removed, and then the ureter and bladder can be replanted and treated by anti-reflux surgery (Politano Leadbetter method). Asymptomatic patients without progressive renal damage do not need treatment. More common in adults.
(3) Ureteral stricture A group of 147 cases of ureteral diseases were reported, of which 55% had ureteral stricture. Histologically, the transitional epithelium is normal, smooth muscle cells decrease, and fibrous tissue does not increase. The etiology of congenital ureteral stricture is unknown. From the ultrastructural observation, simple stenosis may be caused by the developmental disorder of embryo 1 1 ~ 12 weeks. Located at the junction of ureter and bladder, at the junction of pelvis and ureter, the middle part is rare. Its clinical manifestations are mainly symptoms caused by upper urinary tract obstruction, and the treatment depends on the damage and degree of kidney.
(4) Ureteral valve is rare. However, there are transverse mucosal folds containing smooth muscle fibers in the ureter cavity, which can cause obstruction and dilatation of the proximal ureter, and its distal side is normal. Early literature described a ring-shaped or diaphragmatic lesion with a pinhole-like opening. 40 cases of congenital ureteral valve disease were reported. The common site is at the junction of the middle 1/3 segment with the ureteropelvic pelvis. Intravenous and retrograde urography are helpful for diagnosis, and valve resection or ureterectomy and anastomosis can be selected according to the specific situation. 5% newborns have different degrees of transverse mucosal folds, which will gradually disappear after birth and will not cause obstructive symptoms, so it has no clinical significance.
(5) In 12080 autopsy cases of children, only 2 cases found ureteral spiral coil. Due to the abnormal rotation of ureter and kidney, obstruction and hydronephrosis can be caused.
(6) There are three types of ureteral diverticulum: ① It occurs at the blind end of one of the bilateral ureters; (2) Congenital ureteral diverticulum, which contains layers of ureteral tissue; ③ Acquired diverticulum, showing mucosal hernia. Congenital diverticulum is extremely rare. It can occur in the lower ureter near the bladder, the middle ureter and the junction of renal pelvis and ureter, and sometimes it can form a huge cyst. The result is secondary hydronephrosis. The clinical manifestations are abdominal pain or renal colic. Abdominal cystic mass was touched. A diverticulum can be asymptomatic. Usually found in surgical exploration or urography. Can be removed by surgery. (1) Bladder-ureter reflux Primary congenital vesicoureteral reflux is caused by oversize ureter and its lateral opening, which loosely adheres to the underdeveloped triangle of bladder.
(2) The ureteral orifice is ectopic. Under normal circumstances, the ureter opening is at the left and right bottom corners of the triangle of bladder. If the embryo develops abnormally, the ureter can open to the outside of the bladder, the male can open to the posterior urethra, ejaculatory duct, seminal vesicle, vas deferens and rectum, and the female can open to the anterior urethra, vestibular area, vagina, uterus and rectum. Because of the anatomical position, the opening of male ectopic ureter is still controlled by external sphincter, so there is no leakage of urine. However, women often have symptoms of dripping urinary incontinence because their ectopic opening is often not controlled by the external sphincter. So there are more women than men in clinical diagnosis and treatment. According to the literature, women are 2 ~ 12 times as many as men. The bilateral rate was 7.5 ~ 65438 07%. The incidence of autopsy data of children was 1∶ 1900. 80% cases are accompanied by both kidneys and ureters. And most of them come from the upper renal segment of the kidney. But in men, the ectopic opening of single ureter is the most common. The ectopic opening of ureter is often narrow, and the nerve and muscle of ureter are underdeveloped, so the ureter is tortuous, dilated and hydronephrosis. The drained renal segment is underdeveloped, accompanied by hydronephrosis, pyelonephritis and other diseases, and occasionally accompanied by single kidney, horseshoe kidney, ectopic kidney and other malformations.
According to Margueras classification, there are many types of 10:
1. Single focus of kidney, ectopic ureter of upper renal segment.
2. Ectopic ureter orifice of normal kidney.
3. Both kidneys, one side of the upper renal segment with ectopic ureter opening.
4. Both normal kidneys have ectopic ureters.
5. Only focus on the kidney, both ureters have ectopic openings.
6. Both kidneys emphasize that both upper kidneys have ectopic ureter openings.
7. Double kidneys, with incomplete duplication of ureter on one side and ectopic ureter opening on the upper kidney on one side.
8. The ureter moves to the opposite side and the opening is ectopic.
9. horseshoe kidney, double kidney, and one of the ureteral openings is ectopic.
10. ectopic ureteral orifice in solitary kidney.
The main clinical manifestations are drip urinary incontinence and urinary tract infection. In addition to normal micturition, female patients have drops of urine phenomenon during micturition, and often wet their pants, which is more obvious in sitting position than lying position. The vulva is often wet and flushed, and eczema and erosion appear in the surrounding skin, and symptoms such as urinary tract infection, pyuria and abdominal mass can also appear. If the vestibular area is ectopic, urine beads can be seen dripping intermittently. If the opening is in the vagina, it will overflow from the vaginal opening. If ectopic opening can be found, retrograde ureterography can be inserted to make a definite diagnosis. There is no drops of urine phenomenon in men. Sacral pain and recurrent epididymitis may occur if you open your mouth in the seminal vesicle. Intravenous urography combined with cystoscopy and retrograde pyelography is a necessary means to diagnose and make a surgical plan. In addition, micturition cystourethrography also has certain diagnostic value for urethral opening.
Surgical treatment should be based on the specific conditions of kidney and ureter. If the water in the ectopic opening is obvious, the drained kidney will be seriously infected and the renal function will be basically lost. If the contralateral kidney is good, the diseased kidney should be removed. If the kidney is heavy, nephrectomy should be performed to remove most of the ureter to avoid stump syndrome.
(3) Some cases have good renal function and no serious infection, so ectopic ureter can be transplanted into bladder. Ureteral cyst is cystic dilatation at the end of ureter. Its outer layer is bladder mucosa, and its inner layer is ureter mucosa, with fine muscle fibers and connective tissue between them. The reason is ureteral stricture or functional contracture. During embryonic development, the diaphragm between ureter and urogenital sinus is not absorbed and sinks, resulting in different degrees of ureteral stricture. It can also be caused by factors such as weak fiber structure at the end of ureter, too long behavior and bending between walls. After being impacted by urine flow, it forms cystic expansion and protrudes into the bladder. According to Ericsson's clinical classification, it can be divided into in-situ (simple) ureteral cyst and ectopic ureteral cyst. The former is more common in adults, and the opening is normal or slightly biased. Cysts are often very small, located in the bladder, which only produces slight ureteral obstruction and does not block the bladder neck, so the damage to the kidney is very light or unaffected. The latter is more common in children, and the cyst is generally large, combined with double kidney and double ureter malformation. It is common that the upper renal ureter opens at the bladder neck or posterior urethra, causing urinary tract obstruction. Occasionally, it can occur in the ureter of the lower renal segment. According to Stephen's pathological anatomy, it can be divided into three types: stenosis, inclusion body and stenosis. The incidence rate of women is 3 ~ 4 times higher than that of men, with the most on the left side and 10 ~ 15% on both sides.
Early cases, clinically asymptomatic, are often found when severe renal malformation is diagnosed. Symptoms are mainly urinary tract obstruction, causing repeated urinary tract infections. Because the opening of the cyst is small, the continuous obstruction of the ureteral orifice will lead to hydronephrosis, loss of renal function, dysuria or interruption of urine flow caused by the cyst blocking the bladder neck, as well as recurrent urinary tract infection. Sometimes a girl's cyst can protrude from the urethral orifice through the bladder neck and urethra, and generally it can be reset by itself. But it can also appear as a purple tumor.
Intravenous urography, if the renal function is good, shows that there is a shadow with reduced density in the bladder, which is vacuolar, round and biased to one side. When the upper kidney with double ureters is undeveloped due to back pressure, hydrops, infection and dysfunction, it can be seen that the developed lower kidney is displaced outward and downward under pressure, and there is a round and smooth filling defect in the bladder neck. Cystography can sometimes supplement intravenous urography.
The principle of treatment is to relieve obstruction, prevent reflux and deal with complications. If the upper part of the affected side has poor renal function, heminephrectomy is feasible. About 20 ~ 25% cases still have symptoms after operation, and then cyst treatment. If the renal function is good, we can do ureterocele resection and replantation of ureter and bladder to prevent reflux. (1) During the normal development of the posterior ureter of the inferior vena cava, the right posterior main vein gradually degenerated and atrophied, and the inferior vena cava mainly evolved from the inferior main vein and the superior main vein. Therefore, the ureter is located outside the inferior vena cava. If the embryo develops abnormally, the right posterior main vein does not degenerate, and continues to exist and evolve into the main component of the inferior vena cava, the ureter will be located behind the inferior vena cava, wound forward, and turn downward into the bladder through its lateral side, thus forming the posterior ureter of the inferior vena cava. So they all happen on the right side, which is relatively rare and mostly male.
The main clinical manifestations are upper urinary tract obstruction caused by compression of ureter by inferior vena cava, dilatation of renal pelvis and ureter, hydronephrosis, urinary tract infection, calculus and hematuria. The diagnosis was mainly based on intravenous urography and retrograde ureterography, which showed that the right ureter shifted to the center and crossed the third and fourth lumbar vertebrae, forming an S-shaped abnormality. If IVC can be done at the same time, the relationship between the right ureter and the inferior vena cava can be displayed. In the treatment, ureterectomy, reduction and anastomosis were performed according to the degree of renal damage.
(2) Ureteral hernia is rare. Hernia can reach the groin, scrotum (male) or femoral canal (female), and most of them are paraperitoneal ureteral hernia. Ureteral loop is located in peritoneal sac, and there is generally no hernia sac. Those who hernia into scrotum will swell, which may cause upper urinary tract obstruction. The clinical manifestations are low back pain or urinary tract infection. Intravenous urography can show partial ureteral displacement in groin or scrotum. Ureterectomy and anastomosis can be used.