Congenital urinary system malformations

　　Renal malformations in congenital urinary system malformations include cystic renal lesions, horseshoe kidney, solitary kidney, etc.; congenital ureteral malformations include complete absence of ureter, as well as duplicated ureteral malformation, ureteral diverticulum, ectopic ureteral orifice, retrocaval ureter, abnormal connection between ureter and renal pelvis, and other malformations.

　　Duplicated kidney, duplicated ureter, and ectopic ureteral orifice are not uncommon congenital malformations in children, but due to the great variation in clinical manifestations, they bring certain difficulties to clinical diagnosis and treatment. During the 4th to 7th weeks of embryogenesis, the ureter differentiates from the mesonephric duct. The lower end of the mesonephric duct emits the ureteric bud, which develops upwards, forming the ureter, entering the renal tissue, and gradually forming the renal pelvis, renal calyces, and collecting system. If two ureteric buds occur during this process, repeated ureteral malformation will occur.

　　The symptoms and hazards of congenital urinary system malformations actually come from complications. Therefore, for patients without symptoms and good renal function, treatment may not be required, and follow-up observation is sufficient. For patients with symptoms that affect renal function, treatment is necessary, and surgery is the main treatment method. The determination of the surgical plan should be based on the actual situation. For cases with acceptable renal function and hydronephrosis of the ureter, the duplicated kidney and ureter can be retained, and only the ureteral reimplantation into the bladder is required to solve the problems of hydronephrosis and reflux; if there is a ureteral cyst, a small cyst (diameter

　　The common symptoms of congenital urinary system malformations are hematuria, abnormal urination, abdominal pain, and abdominal mass. The specific clinical manifestations are described as follows.

　　1. Urinary incontinence, the main manifestation, is caused by ectopic ureteral orifice.

　　2. Difficulty in urination, the main manifestation, is caused by ureteral cysts.

　　3. The main manifestation of urinary system infection is caused by urine reflux and urinary tract hydronephrosis.

　　4. Those with abdominal mass as the main manifestation are caused by repeated kidneys and repeated ureteral hydronephrosis.

　　Congenital urinary system malformations are mainly congenital diseases, so there is no direct prevention for this disease. Early detection, early diagnosis, and early treatment are of great significance for preventing the disease. Regular checks should be done during pregnancy, and if there is a tendency of developmental abnormalities in the child, chromosome screening should be done in a timely manner, and if confirmed, artificial abortion should be performed in a timely manner to avoid the birth of children with the disease.

　　The examination of congenital urinary system malformations includes rectal examination, blood and urine routine tests, liver and kidney function tests, etc., and the specific examination methods are described as follows.

　　1. Rectal examination: It can provide important clues for determining the location and degree of urethral stricture and whether there is concurrent rectal injury, which is an important examination.

　　2. Diagnostic cystoscopy: can cause partial lacerations to develop into complete ruptures, thereby exacerbating bleeding, causing hematoma, and secondary infection.

　　3. Urethral metal probe or guidance probe examination: can determine the location and degree of urethral stricture.

　　4. Routine blood tests, urine tests, liver and kidney function tests, electrolytes, electrocardiogram, X-ray flat film, urological ultrasound, cystourethrography, intravenous urography, cystoscopy, ureteroscopy.

　　In addition to routine treatment, attention should also be paid to some aspects of diet for patients with congenital urinary system malformations: patients should eat foods rich in vitamins, high-quality protein, and diuretics; spicy and greasy foods should be avoided.

　　The treatment of congenital urinary system malformations includes surgical and non-surgical treatment, and the methods are described as follows.

　　Firstly, treatment principles

　　1. Actively treat the infection around the urethra.

　　2. The purpose is to restore the urinary function of the urethra, and to restore the anatomical continuity and integrity of the urethra.

　　3. Avoid the occurrence of new complications during the treatment process.

　　4. Perform cystostomy in cases with chronic renal insufficiency.

　　5. Perform colonic fistula first in cases with urethral rectal fistula.

　　Secondly, non-surgical treatment

　　Non-surgical treatment mainly relies on urethral dilation, and even in cases after surgical treatment, regular dilation should be performed to prevent recurrence of stricture. Urethral dilation should not be performed when there is acute inflammation in the urethra, and it should be carried out under good anesthesia and strict aseptic conditions. Avoid using force during dilation. If necessary, use one finger in the rectum to guide to prevent penetration into a false passage or the rectum. Dilation must be gradually increased from a small probe rod to a larger probe rod in sequence, and impatience should be avoided. Rapid dilation can easily cause lacerations of the urethral wall, followed by scar formation and exacerbation of stricture. Generally, it is advisable for men to dilate to F24. After each urethral dilation, there is urethral congestion and edema, which takes about 2 to 3 days to subside, so it is not advisable to dilate continuously within 4 days. The interval between two dilations usually starts from about one week and gradually extends.

　　Intravesical instillation of urinary tract irrigation fluid can prevent the recurrence of urethral stricture and achieve a soft dilation effect. Physical therapy methods such as audio and iodine ion permeation can accelerate scar softening and consolidate the effect of dilation.

　　3. Surgical Treatment

　　1. Treatment of posterior urethral stricture:The treatment of urethral stricture after urethral injury is most suitable for 3 to 6 months. According to the degree of injury, the following surgical methods can be selected:

　　(1) Urethral incision (optional):Use urethral surgery knife (cold knife) or laser to incise the scar at the stricture, expand the lumen of the urethra after incision, and leave a catheter in place. Applicable to short stricture segments

　　(2) Urethral anastomosis (recommended):Incise the perineal incision, resect the stricture segment and scar, anastomose the two ends of the urethra, and it is applicable to the stricture segment

　　(3) Urethral advancement (optional):Applicable to patients who cannot undergo urethral anastomosis. After resecting the narrowed end of the urethra, the distal urethra is freed, allowing it to pass through the proximal stricture segment appropriately, and is fixed to or passed through a traction line to fix it to the abdominal wall. The disadvantage is that it may cause penile shortening and downward curvature during erection.

　　(4) Urethral Substitution成形surgery (optional):Applicable to longer segment urethral stricture or atresia. Use pedicled flaps and free grafts to repair urethral defects.

　　① Pedicled flap:Commonly used penile, perineal skin. The flap needs to have good blood supply; complications include hair, calculus, and diverticulum formation. The incidence of urethral stricture recurrence in the long term is still relatively high.

　　② Free graft:Various autologous mucosal, skin, tissue engineering materials (decellularized matrix) are suitable for long-segment urethral成形reconstruction.

　　2. Treatment of anterior urethral stricture:The appropriate time for the treatment of urethral stricture after urethral injury is 3 months after the injury.

　　Urethral stricture involving the superficial urethra海绵body

　　For urethral stricture less than 2 cm in the bulb, scar resection and anastomosis is a relatively suitable treatment method (recommended), with a success rate of up to 95%. For penile urethral stricture and longer bulb urethral stricture (>2cm), it is not recommended to use simple end-to-end anastomosis, as this may cause patients to have downward curvature and pain during erection. For such patients, it is recommended to use alternative urethral成形surgery with pedicled flaps or free grafts (recommended). It is not recommended to use urethral stents for patients with traumatic urethral stricture.

　　4. Postoperative Management

　　1 to 2 weeks after simple internal incision, an indwelling catheter is left in place. For other types of surgery, an indwelling catheter is generally left in place for 3 to 4 weeks after surgery. Encourage patients to drink plenty of water and appropriately apply antibiotics.