Bladder agenesis and hypoplasia

　　1, Etiology

　　The cause of this disease has not been definitely determined, because these children have a normal posterior intestine, and it can be imagined that the process of cloaca differentiation into the urogenital sinus and anal rectum is normal. Bladder agenesis may be the result of secondary atrophy of the anterior part of the cloaca, or it may be due to the malcoordination of the mesonephric duct and ureter entering the trigone area, preventing urine from accumulating in the bladder, so there is no urine filling the bladder.

　　2, Pathogenesis

　　The outcome of malformations varies with gender. If the female has normally developed mesonephric ducts, the ureteral orifice can be located on the anterior wall of the uterus, vagina, or vestibule. Patients with ectopic ureteral orifices can often preserve part of the renal function. In males, the ways to obtain urinary excretion are the residual cloaca and ureteral drainage into the rectum or patent urachus.

　　Commonly associated with malformations, including: single kidney, underdeveloped kidney, abnormal kidney development, and the absence of prostate and seminal vesicles, etc. The male valve is often located on the ventral side near the proximal urethra at the junction of the penis and scrotum, presenting as a尖瓣, iris valve, or crescent shape. In addition to causing lower urinary tract obstruction, it can also lead to secondary upper urinary tract dilation and hydrops, symptoms of lower urinary tract obstruction, difficulty urinating, thin and weak urine stream or dripping, and acute urinary retention, etc.

　　Due to the明显 reduction in bladder capacity and reduced urine storage, patients may present with frequent urination. Due to repeated frequent urination, it can induce genital tract infection, and in patients with concurrent infection, symptoms such as perineal redness, ulceration, and fever may occur. Such patients often have serious malformations of the upper urinary tract and other systems, and most are stillborn or die soon after birth due to pyelonephritis, with little clinical significance.

　　The cause of this disease has not been confirmed, because these children have a normal posterior intestine, and it can be imagined that the process of differentiation of the cloaca into the urinogenital sinus and anal rectum is normal. Bladder agenesis may be the result of secondary atrophy of the anterior part of the cloaca, or it may be due to the malcoordination of the mesonephric duct and ureter entering the trigone, blocking the accumulation of urine in the bladder, so there is no urine filling the bladder. Therefore, this disease cannot be prevented, and early detection, early diagnosis, and early treatment are of great significance for the prevention of this disease.

　　Medical imaging examinations can diagnose whether the degree of bladder development is normal. Ultrasound, X-ray plain film plus intravenous pyelography, CT, MRI are all beneficial to the diagnosis of this disease. The key examination methods include urinary system造影 examination, which can understand the shape and size of the bladder, and clarify the degree of malformation. In addition, cystoscopy can also accurately reflect the development of the bladder.

　　Postoperatively, high-protein, high-calorie, and high-vitamin nutritional diets should be provided in principle, such as beef and mutton, lean pork, chicken, fish, shrimp, eggs, ribs, and soy products. Patients can be given more milk, lotus root powder, and fresh fruit juice, as well as more fresh fruits and vegetables.

　　1. Treatment

　　In the reported surviving children, the treatment is mostly urinary diversion, using ureterosigmoidostomy or ureterostomy to solve the problem of urination. At present, artificial bladder technology is still under development. If this technology matures in the future, artificial bladder replacement surgery for this disease is expected to cure the disease.

　　2. Prognosis

　　This disease belongs to developmental malformation. Due to the characteristics of frequent urination and easy infection, it may affect the quality of life of patients. Proper care will not endanger the health of patients.