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Congenital sclerosis (contracture) of the bladder neck (Marion's disease)
Last reviewed: 07.07.2025
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Congenital sclerosis of the bladder neck (Marion's disease) is rare. In boys, it is characterized by the development of ring-shaped fibrous tissue in the submucosal and muscular layers of this zone. The degree of its expression varies. Histological examination of older children often reveals signs of chronic inflammation along with fibrosis.
Symptoms Congenital sclerosis (contracture) of the bladder neck (Marion's disease)
Symptoms of the disease depend on the severity of the neck obstruction and the duration of the disease. With minor development of fibrous tissue, slight difficulty in urination is noted. There is no residual urine, kidney function is not impaired. With severe obstruction, difficulty in urination is noted and residual urine is detected. Sclerosis of the neck of the bladder can progress to urinary retention such as paradoxical ischuria, the development of vesicoureteral reflux, ureterohydronephrosis and renal failure.
[ Diagnostics Congenital sclerosis (contracture) of the bladder neck (Marion's disease)
Sclerosis of the bladder neck is established based on the data of ascending urethrocystography and urethrocystoscopy. UFM in combination with cystomanometry confirms the presence of obstruction in the neck area and allows to assess the functional state of the detrusor.
Urethrocystography shows a contrasting elevation of the fundus and trabecular walls, insufficient contrasting of the urethra, cystoscopy shows trabecular walls of the bladder, sometimes a protrusion of the posterior wall of the neck is determined, and in some patients hypertrophy of the interureteral fold. A characteristic sign on the cystogram is an elevation of the fundus.
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Treatment Congenital sclerosis (contracture) of the bladder neck (Marion's disease)
Sclerosis of the neck of the urinary bladder is treated using transvesical surgical interventions - longitudinal dissection, U-shaped plastic surgery or TUR, which has now become the method of choice.