Congenital sclerosis (contracture) of the neck of the bladder (Marion's disease)
Last reviewed: 23.04.2024
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Congenital sclerosis of the neck of the bladder (Marion's disease) is rare. Boys are characterized by the development of ring-shaped fibrous tissue in the submucosal and muscle layers of this zone. The degree of its expression is different. When histologically examined in older children, along with fibrosis often show signs of chronic inflammation.
Symptoms of the congenital sclerosis (contracture) of the neck of the bladder (Marion's disease)
Symptoms of the disease depend on the degree of severity of cervical obstruction and the duration of the disease. With a slight development of fibrous tissue there is a slight difficulty in urinating. There is no residual urine, the kidney function is not broken. With severe obstruction, difficulty urinating is noted and residual urine is detected. Sclerosis of the neck of the bladder can progress up to the delay of urination by the type of paradoxical ishuria, the development of vesicoureteral reflux, ureterohydronephrosis and renal insufficiency.
Diagnostics of the congenital sclerosis (contracture) of the neck of the bladder (Marion's disease)
Sclerosis of the neck of the bladder is established based on the data of ascending urethrocystography and urethrocystoscopy. UFM in combination with cystomanometry confirms the presence of obstruction in the neck and allows to assess the functional state of the detrusor.
With urethrocystography, the elevation of the bottom and the trabecularity of the walls contrast, the inadequate contrast of the urethra, cystoscopy indicates the trabecularity of the walls of the bladder, sometimes the protrusion of the posterior wall of the cervix is determined, and in some patients the hypertrophy of the interfluoric fold. On the cystogram, a characteristic feature is the elevation of the bottom.
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Treatment of the congenital sclerosis (contracture) of the neck of the bladder (Marion's disease)
The sclerosis of the neck of the bladder is treated with the use of transvesical surgery - longitudinal dissection, Y-shaped plastic or TUR, which has now become the method of choice.