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Neurogenic LUT Dysfunction
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== Miscellaneous conditions definitely, probably, or possible related to neuromuscular dysfunction == === Detrusor sphincter dyssenergia === * '''Detrusor sphincter dyssynergia, unless specified otherwise, refers to dyssynergia of the striated sphincter and bladder''' * '''True DSD should exist only in patients who have an abnormality in pathways between the sacral spinal cord and the brainstem PMC.''' ** '''The diagnosis of DSD should be suspected in any patient with a neurologic lesion in this area.''' * '''Common causes include:''' *# '''Traumatic SCI''' *# '''MS''' *# '''Transverse myelitis''' ** '''Conversely, in patients without such a lesion, this diagnosis should always be viewed with skepticism''' * '''It is important to remember that sphincter electromyographic activity that increases simultaneously with intravesical or detrusor pressure does not always indicate true DSD. These instances are referred to as pseudodyssynergia; common causes of pseudodyssynergia include:''' *# '''Abdominal straining to either initiate or augment a bladder contraction or in response to discomfort''' *# '''Attempted inhibition of a bladder contraction either because of its involuntary nature or because of discomfort''' * '''Without proper treatment, > 50% of men with DSD will develop significant complications, such as''' *# '''VUR''' *# '''Upper tract deterioration''' *# '''Urolithiasis''' *# '''Urosepsis''' *# '''Ureterovesical obstruction''' * '''Therapy for DSD is designed to either eliminate or significantly minimize the abnormal sphincter activity or to bypass the sphincter itself.''' ** '''Oral medical therapy directed toward the striated sphincter has not enjoyed wide success.''' ** '''The most common treatment approaches currently are:''' **# '''CIC (usually combined with therapy to control detrusor overactivity)''' **# '''Sphincterotomy''' **# '''Stent placement across the sphincter''' **# '''Injection of onabotulinumtoxinA into the sphincter''' **# '''Continuous indwelling catheterization''' **# '''Urinary diversion''' === Dysfunctional voiding === * '''Urodynamically appears to be involuntary obstruction at the striated sphincter level existing in the absence of demonstrable neurologic disease''' * '''Very difficult to prove urodynamically;''' '''unequivocal demonstration of this''' '''entity requires pressure-flow electromyographic evidence of bladder emptying occurring simultaneously with involuntary striated sphincter contraction in the absence of any element of abdominal straining, either in an attempt to augment bladder contraction or as a response to discomfort during urination''' === Bladder neck dysfunction === * '''Defined as incomplete opening of the bladder neck during voluntary or involuntary voiding; has also been referred to as smooth sphincter dyssynergia''' * '''The dysfunction is found almost exclusively in young and middle-aged men, who characteristically report long-standing voiding and storage symptoms''' * These patients have often been seen by many urologists and have been diagnosed as having psychogenic voiding dysfunction because of a normal prostate on rectal examination, a negligible residual urine volume, and a normal endoscopic bladder appearance. * The differential diagnosis also includes: ** Anatomic bladder neck contracture ** Benign prostatic enlargement (BPE) or BPO ** Dysfunctional voiding ** Prostatitis ** Neurogenic micturition dysfunction ** Low pressure and low flow (anxious) bladder * '''Objective evidence of outlet obstruction in these patients is easily obtainable by urodynamic study'''. '''Once obstruction has been diagnosed, it can be localized to the level of the bladder neck by video-urodynamic study,''' cystourethrography during a bladder contraction, or micturitional urethral profilometry. The diagnosis may also be made indirectly by the urodynamic findings of outlet obstruction in the absence of urethral stricture, prostatic enlargement, and DSD. * The exact cause of this problem is unknown. Some have proposed that there is an abnormal arrangement of musculature in the bladder neck region, such that coordinated detrusor contractions cause bladder neck narrowing instead of the normal funneling. * '''The occurrence of this problem in young, anxious, and “high-strung” individuals, and its partial relief by α-adrenergic blocking agents, have prompted some to speculate that it may in some way be related to sympathetic hyperactivity.''' * '''Management''' ** '''Although α-adrenergic blocking agents provide improvement in some patients with bladder neck dysfunction, definitive relief in men is best achieved by a bladder neck incision''' === Bladder outlet obstruction in women === * Uncommon * Defined as radiographic evidence of obstruction between the bladder neck and the distal urethra in the presence of a sustained detrusor contraction of any magnitude which is usually associated with reduced or delayed urinary flow rate. ** Obstruction at the level of the bladder neck is diagnosed when the bladder neck is closed or narrowed during voiding. ** Obstruction of the urethra was diagnosed as a discrete area of narrowing associated with proximal dilatation. * '''Causes:''' ** '''Dysfunctional voiding''' ** '''Cystocele''' ** '''Prior incontinence surgery''' ** '''Urethral stricture''' ** '''Uterine prolapse''' ** '''Urethral diverticulum''' ** '''Rectocele''' * '''Management''' ** '''Other than obvious pathology related to obstruction (cystocele, prior incontinence surgery, etc.), surgical treatment for bladder outlet obstruction in women should be approached with caution because sphincteric incontinence is a significant risk. Rather, pelvic floor therapy (biofeedback), behavioral modification, and the addition of pharmacotherapy should be used''' === Low-pressure and low-flow voiding in younger men – anxious bladder === * When this occurs in a young man, it is usually characterized by frequency, hesitancy, and a poor stream. The entity is readily demonstrated on urodynamic assessment and with no coexisting endoscopic abnormality. * The patient usually notes marked hesitancy when attempting to initiate micturition in the presence of others, and some have therefore described this condition as an “anxious bladder” or a “bashful bladder.” * Psychologically, these men tend to be obsessional rather than anxious * Behavioral therapy should be attempted === Urinary retention: Fowler syndrome in women === * '''Fowler syndrome refers specifically to urinary retention in young women in the absence of overt neurologic disease.''' * Potential causes or urinary retention are classically cited as neurologic, pharmacologic, anatomic, myopathic, functional, and psychogenic. * '''The typical history is that of a woman age < 30 who has found herself unable to void for a day or more with no urinary urgency but increasing lower abdominal discomfort.''' * '''A bladder capacity of over 1 L with no sensation of urgency is necessary for the diagnosis.''' * '''Concentric needle electrode examination of the striated muscle of the urethral sphincter may demonstrate impaired sphincter relaxation.''' * Urodynamic finding is detrusor acontractility. * Management: neuromodulation === Postoperative urinary retention === * Incidence 4-25% * '''Contributing factors:''' *# '''Diminished awareness of bladder sensation''' *# '''Bladder overdistention''' *# '''Decreased bladder contractility''' *# '''Decreased micturition reflex activity''' *# '''Traumatic instrumentation''' *# '''Increased outlet resistance''' *# '''Nociceptive inhibitory reflex''' *# '''Pre-existent outlet pathology (e.g., BPH)''' ** '''Anesthesia and analgesia can contribute to factors 1-4''' === Radiation === * '''Early radiation reaction most prominent at 4 to 6 weeks''', with an incidence as high as 70%. * Storage symptoms are most common * '''Urodynamic studies have demonstrated:''' *# '''Reduced volume at first desire to void''' *# '''Reduced cystometric capacity''' *# '''Reduced compliance''' ** '''These parameters tend to return to pretreatment values by 6 months.''' * '''Symptoms associated with later radiation effects are less common but may be progressive and intractable. Storage symptoms again predominate''' === Defunctionalized bladder === * '''The previously normal defunctionalized bladder will often show decreased capacity and involuntary bladder contractions and/or decreased compliance.''' * '''Rehabilitation of a defunctionalized bladder is certainly possible and should definitely be attempted by cycling with progressively increasing volumes.''' === Aging === * '''Aging-related changes to micturition (4)''' #'''Decreased afferent activity (bladder sensation''') # '''Decreased efferent activity''' # '''Decreased detrusor contractility''' # '''Decreased urethral pressure''' === Other conditions === *Hyperthyroidism ** Can have incomplete emptying, frequency, straining * Schizophrenia ** Can have involuntary bladder contractions * Gastroparesis * Isaacs syndrome * Wernicke encephalopathy ** Caused by a deficiency in thiamine (vitamin B1) ** The two major clinical manifestations of thiamine deficiency involve the cardiovascular and neurologic systems, with the latter manifesting in general as a peripheral neuropathy ** Can have urgency incontinence ** Management: thiamine replacement * '''Myasthenia gravis''' ** Autoimmune disease caused by autoantibodies to acetylcholine nicotinic receptors. This leads to neuromuscular blockade and subsequent weakness in a variety of striated muscle groups. ** Can have urinary incontinence from poor tone of sphincter or urinary retention from detrusor areflexia *** '''Increased risk of urinary incontinence even after a well-performed transurethral or open prostatectomy.''' * Systemic sclerosis (scleroderma) ** Disease of the connective tissue characterized by thickening and fibrosis of the skin, abnormalities of the small arteries, and involvement of the gastrointestinal tract, heart, lung, and kidneys. ** Can have storage and voiding symptoms * Ehlers-Danlos syndrome ** Inherited abnormalities of connective tissue ** Main clinical manifestations are skin fragility, skin hyperextensibility, and joint mobility ** No characteristic pattern of dysfunction. * Corticobasal degeneration * Sacral coccygeal teratoma * Subacute combined degeneration * Willams-Beuren syndrome * Amyloidosis * Machado-Joseph disease * Congenital adrenal hyperplasia * Benign joint hypermobility syndrome * Attention-deficit/hyperactivity disorder.
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