Congenital giant ureter, also known as primary giant ureter, is caused by functional obstruction at the end of the ureter, resulting in dilatation of the ureter and renal pelvis, while no organic obstruction has been found at the site of the lesion (end of the ureter), and the disease should be distinguished from giant ureter due to lower urinary tract obstruction, which has a normal anatomy at the ureter-bladder junction.
[Etiology] There is no consensus, and it is mostly believed that the disease is caused by abnormal development of the muscular layer at the end of the ureter, resulting in functional obstruction here.
Diagnosis
(A) Clinical manifestations
1, pus urine, hematuria Due to the dilatation of the ureter, the upper urinary tract is poorly drained and prone to urinary tract infection. Infants and children show fever, vomiting and pus urine, and children mostly have hematuria.
2, abdominal pain, abdominal distension in extremely dilated people with an enlarged abdomen, and abdominal pain episodes in older children.
3, abnormal urination if accompanied by other deformities may have dribbling urine, difficult urination, urinary incontinence and other symptoms.
(B) examination
1, abdominal examination of small infants with enlarged abdomen, cystic masses can be found, positive transillumination test. Children rarely have positive signs.
2, urine examination appears a large number of red and white blood cells, culture with bacterial growth, blood culture may also be positive.
3.Renal function measurement may be impaired with azotemia and elevated values of muscle drunkenness and urea nitrogen.
4.Intravenous urography shows spherical dilatation or distortion of the ureter throughout the whole process, and mild hydronephrosis of the renal pelvis and calyces; or the affected kidney and ureter are not visualized.
5, B-mode ultrasound detects dilated ureter and hydronephrosis in the pelvis and calyces, or a large cystic mass in the abdomen with a small, dysplastic kidney.
6, voiding cystourethrography primary reflux giant ureter showing reflux dilated ureter; secondary showing urethral valves, diverticula, atelectasis or cucullar type of neurogenic bladder.
7, Cystoscopy and retrograde angiography to understand the presence of tumors and cysts in the bladder and the location of the ureteral opening; retrograde intubation angiography to understand the site of ureteral obstruction.
8, diuretic nephrogram Nephrogram scan shows dilated ureter, obstructive giant ureter shows slow excretion of nucleus. Congenital giant ureter may not be shown in the affected kidney, and the renal function may be reduced.
9. Percutaneous pyelogram clearly shows dilated and distorted ureter, and obstructive can clearly show the distal stenosis of dilated segment
【Treatment
1. For mild to moderately dilated giant ureter without infection, a small amount of prophylactic antibiotics can be applied. Follow-up observation, regular B-type ultrasonography and urinalysis, measurement of length and weight, and attention to development.
2 Surgical treatment is appropriate for severe dilatation and serious infection.
(1) Ureteral bladder reanastomosis: primary reflux and primary non-reflux non-obstructive using Cohen or
A Leadbetter ureteral reimplantation, and obstructive reimplantation should be performed after removal of the stenotic segment.
(2) Giant ureteral trimming: If the ureter is overly dilated and redundant, the distal ureter should be trimmed or folded before reimplantation.
(3) Renal and ureteral resection: congenital giant ureter is often accompanied by renal dysplasia, and renal and ureteral resection or repeat nephrectomy with corresponding ureter should be performed.
(4) Ileal substitution cystectomy or intestinal sheath encapsulated ureter: it is suitable for those who failed ureteral reimplantation or ureter without peristaltic function.