Bladder exstrophy

　　1. Etiology

　　Regarding the etiology of bladder exstrophy, it is currently believed that errors occur during embryonic development rather than a stoppage at a certain stage, as human embryonic development typically does not pass through the corresponding stage of bladder exstrophy.

　　2. Pathogenesis

　　The embryological basis of bladder exstrophy malformation is the abnormal development of the cloacal membrane, which obstructs the migration of mesenchymal tissue to the middle part, causing developmental disorders of the lower abdominal wall. The rupture and defect of the cloacal membrane lead to various forms of bladder exstrophy and urethral underdevelopment.

　　Bladder exstrophy is often a complex malformation, with common associated malformations including urethral cleft and cloaca exstrophy. Since the bladder and urethra are homologous in embryonic development, the most common complex malformation is bladder exstrophy with urethral cleft.

　　In addition, some patients only have developmental defects in the abdominal wall skeletal muscles, while the development of the urethra is complete. This condition is called pseudoexstrophy. The main characteristics include an overly long umbilical cord, low umbilical position, and rectal muscles scatteredly attached to the pubic bone.

　　Children may develop inguinal hernia due to abnormal development of the abdominal wall muscles; due to symphysis pubis separation, abnormal development of the pelvis may cause external rotation of the femur or hip joint dislocation. The child's gait is a swing gait when walking. Children with exstrophy of the bladder may also have spina bifida, horseshoe kidney, cleft lip, cleft palate, anterior displacement of the anus, atresia, prolapse, and other deformities.

　　Male children have poor development of the corpora cavernosa, varying degrees of urethral cleft, flat glans, and prepuce stacked on the ventral side. Due to the separation of the corpora cavernosa and the upward tilting of the penis, the penis is short. It can be accompanied by cryptorchidism. In females, the clitoris is separated, the labia majora is separated on the midline, and the vaginal orifice is anteverted. They can reproduce, but are prone to uterine prolapse after vaginal delivery. The long-term complications of exstrophy of the bladder mainly include vesicoureteral reflux, recurrent infection of the urinary system, and calculus.

　　The anterior wall and anterior abdominal wall of the everted bladder are defective, the mucosal margin of the posterior bladder wall is connected with the abdominal wall skin, and the entire posterior wall bulges in the area of abdominal wall defect. The exposed bladder mucosa is bright red, easily scratched and bleeding, with severe pain. Due to chronic inflammation and long-term mechanical stimulation, the mucosal epithelium can degenerate, even become malignant. On the posterior wall, there are slightly elevated ureteral orifices with intermittent urine ejection. Urine often wets the surrounding skin, causing dermatitis or eczema. Most children die in early childhood due to ascending urinary tract infection. Exstrophy of the bladder is almost always accompanied by urethral cleft and symphysis pubis separation, or associated with hip joint dislocation.

　　Exstrophy of the bladder is relatively rare. Most children die in early childhood due to ascending urinary tract infection. The lower abdominal wall has a partial defect, and the everted bladder mucosa is exposed, bright red, abnormally sensitive, easily scratched and bleeding. Bilateral slightly prominent ureteral orifices exhibit periodic urination, clothes are wet, accompanied by urinary odor. The skin on the lower abdominal wall, perineum, and inner side of the thigh may appear dermatitis or eczema. The size of the everted bladder varies greatly. The everted bladder mucosa is smooth in the early stage, but it can become bristly, polypous, or show squamous epithelial metaplasia due to long-term exposure and mechanical friction. The bladder wall thickens and hardens due to detrusor fibrosis.

　　1. If not treated, 2/3 of the cases will die from hydronephrosis and urinary tract infection before the age of 20. Short-term complications after surgery include urethral fistula, urethral stricture, and skin splitting.

　　2. The purpose of all surgical repairs and plastic surgery for this disease is to restore anatomical shape, protect renal function, and control urination. However, the surgical steps are numerous, and they must be carried out step by step according to their age and physical condition. Therefore, in addition to early surgery, the complexity and the possibility of failure of surgery cannot be ignored. Parents should be mentally prepared, closely cooperate with the doctor's care, and strengthen observation and care. Even if the surgery is successful, it is necessary to follow up and review, pay attention to whether there is any upper urinary tract dilation, reflux, or concurrent infection. In addition, 10% of the children with incontinence that cannot be controlled or surgical failure need to undergo urinary diversion surgery.

　　1. Plain Film

　　Abnormal development of the pelvis, complete separation of the pubic symphysis, the width of the separation is approximately equal to the width of the sacrum, causing the pelvis to open up in a horseshoe shape, and the two femurs are externally rotated.

　　2. Urography

　　The position of the bladder has dropped.

　　What foods are good for bladder exstrophy:

　　According to different symptoms, there are different dietary requirements, and specific questions should be asked to the doctor, and different dietary standards should be formulated for specific diseases.

　　1. Treatment Objectives

　　Protect kidney function, control urination, repair bladder, abdominal wall, and external genitalia. Female surgery repair is easier than male.

　　2. Surgical Suture

　　Due to edema and chronic inflammation of the bladder wall due to fibrosis of the bladder wall and long-term exposure, a simple bladder inversion suture should be performed within 72 hours after birth. If pelvic osteotomy is not performed, the fascia and fibrocartilaginous tissue of the pubic symphysis can be sutured along the midline after the bladder is returned to the pelvis. If the distance between the pubic symphysis is too wide and it is estimated that it cannot be sutured, the operation is postponed to 7-10 days after birth for pelvic osteotomy and bladder inversion suture. The initial successful suture is very important for the future bladder capacity and control of urination. A bladder stent should be left in place for 3-4 weeks during bladder inversion suture, and there is no need to place a urethral stent. Bryant traction should be used 3-4 weeks after the operation to prevent wound dehiscence. If the child recovers well, the bladder capacity can be measured under anesthesia at the age of 1.5-2.5 years, and if the bladder capacity is above 60ml, the bladder neck and urethral cleft can be repaired simultaneously. If the capacity is below 40ml, only the urethral cleft is repaired to increase the capacity, and the bladder neck is repaired again at the age of 3-5 years. Five weeks before the repair of the urethral cleft, intramuscular injection of testosterone propionate (testosterone propionate) 2mg/kg can increase the size of the penis, which disappears 4 weeks after the operation. After the repair of the bladder neck using the Young-Dees-Leadbetter technique, intermittent catheterization is not necessary.