| disease | Congenital Megaureter |
This disease is caused by congenital neuromuscular dysplasia of the ureter, characterized by significant enlargement and thickening of the ureter without lower urinary tract obstruction or vesicoureteral reflux. Congenital megaureter, also known as congenital functional obstruction of the distal ureter, is more common in males than females. The exact etiology is unknown, but it may be related to dysfunction of the muscles and ganglia at the distal end of the ureter, where it connects to the bladder, leading to proximal obstruction and dilation. In adults, the disease progresses slowly with relatively mild renal impairment. In children, however, the ureter is often severely dilated, renal function is more significantly impaired, and upper urinary tract infections are frequently present.
bubble_chart Clinical Manifestations
1. There are often no symptoms in the early stages.
2. When complicated by urinary tract stones, hydronephrosis, and infection, symptoms such as lumbago, hematuria, frequent urination, and pyuria may occur. In severe cases, fever may also be present.
3. In cases where it occurs bilaterally, manifestations of renal insufficiency may appear.
﹝Auxiliary Examinations﹞
1. If there are no clinical symptoms or symptoms are mild, examination "A" may suffice for diagnosis and treatment needs.
2. If hydronephrosis is severe and accompanied by infection, stones, etc., examination "A" plus "B" may be performed.
3. If neurological disorders or injury are suspected, urodynamic testing should be conducted to rule out neurogenic bladder. This examination requires well-equipped facilities.
Useful examinations include: (1) Percutaneous renal pelvis pressure measurement; (2) Dynamic radiological examination of the upper urinary tract; (3) Urinary flow rate measurement; (4) Bladder pressure-volume measurement, etc.
4. For pediatric patients, examinations are more challenging. For example, procedures such as cystoscopy with retrograde liver qi flow for pyelography must be performed under anesthesia. {|108|}
1. Early stage is asymptomatic, but complications such as hydronephrosis, stones, and infections may cause lumbago, hematuria, frequent urination, and pyuria.
2. High-dose intravenous urography can reveal massive ureteral dilation, with severe cases showing hydronephrosis.3. Cystoscopy shows no abnormalities, ureteral catheterization reveals no obstruction, and there are no lower urinary tract obstructive lesions.
4. Cystography can demonstrate the absence of ureteral reflux.
5. Urodynamic studies, including voiding time, urine flow rate, bladder and urethral pressure, are all normal, confirming no neurogenic dysfunction.
6. MRI may be performed for patients with renal insufficiency.
bubble_chart Treatment Measures
1. Patients with no obvious symptoms and good renal function can be regularly observed.
2. Ureteral orifice dilation has certain therapeutic effects for mild cases.
3. For patients with obvious symptoms or complications such as stones or infections, if renal function is still good and the ureter exhibits peristalsis, pelvic ureteral tailoring and reconstruction with bladder reimplantation can be performed.
4. Patients with severe unilateral renal impairment may undergo nephroureterectomy.
5. For patients with preserved renal function but absent ureteral peristalsis, ileal bladder replacement can be performed.
6. Pediatric cases should undergo surgical treatment as early as possible to reduce or avoid renal function damage.
7. Antibiotics are essential to ensure surgical success, and reducing intrarenal infection also aims to protect renal function. Therefore, antibiotics with low nephrotoxicity should be used, guided by drug sensitivity tests and renal function indicators for targeted therapy.
8. During surgery, fresh blood transfusions should be prioritized, especially in patients with renal insufficiency. Renal protective therapy is also crucial.
1. Cure: Postoperative hydronephrosis improved, ureteral drainage was unobstructed, infection symptoms disappeared, the wound healed, and renal function improved.
2. Improvement: Hydronephrosis decreased, renal function improved, ureteral drainage was unobstructed, but infection recurred intermittently, or the wound healed poorly.
3. No improvement: Postoperative obstruction in the lower ureter, poor drainage, and no improvement in renal function.
