Bladder dysfunction in Duchenne muscular dystrophy.

Although bladder function is thought to be unaffected in Duchenne muscular dystrophy, 46/88 boys interviewed had urinary problems. Nine underwent video urodynamics, showing in eight a small capacity, hyperreflexic bladder, and in the ninth (post spinal surgery) hyperreflexia and detrusor sphincter dyssynergia. Urinary dysfunction is a treatable feature of DMD.     

Neuropathic bladder and intermittent catheterization: social and psychological impact on families

Clean intermittent catheterization (CIC) is the mainstay of management in neuropathic vesicourethral dysfunction, both to improve continence and, more importantly, to preserve renal function. We looked at the effects of this procedure on children, adolescents, and their families. In particular, we wished to see if there were any differences between those who successfully catheterized and those who did not. Forty families were enrolled into the study. Ages of children and adolescents (23 females, 17 males) ranged from 1 to 20 years. Most participants (n=31) had spina bifida. Other causes of bladder dysfunction included transverse myelitis, spinal cord injury, and spinal neuroblastoma. Parents were assessed using the Effects of Handicap on Parents semi-structured interview, the Socioemotional Functioning Interview, and a semi-structured interview, specifically designed for the study, which looked at family characteristics and experience related to diagnosis and catheterization. In addition, the Rutter Parental 'A' Scale Questionnaire was used to screen for emotional and behavioural disorders in the child. Results showed that CIC by carer or self-catheterization itself did not cause major emotional and behavioural problems but the bladder problem may act as a focus that puts considerable strain family relationships. Although most parents disliked CIC they complied with the suggested management. It is important that all those involved understand the aims of management and success can be achieved by combined input from medical, psychological, and specialist nursing staff. The problem is lifelong and continued support from a multidisciplinary team is essential.     

The congenital varicella syndrome

Two children, aged 18 months and 3 years, with the congenital varicella syndrome are described. Neuropathic bladder and lax abdominal wall musculature were present in one case. In the other, paralysis and marked hypoplasia of one arm, and an ipsilateral Horner's syndrome, were the main abnormalities. Extensive skin scarring corresponding to a dermatome distribution was present in both children. This constellation of abnormalities, which may best be explained on the basis of a developmental radiculopathy, emphasises the neuropathic effects on the fetus of varicella zoster (VZ) infection in early pregnancy.     

Spinning top urethra: not a normal variant

Spinning top urethra (STU) is a term used to describe a widened posterior urethra seen mainly in girls. It is commonly regarded as a normal variant. The authors studied 30 girls with STU using videourodynamics. Twenty-eight showed bladder instability; 21, a congenital wide bladder neck anomaly; and 20, both instability and a wide bladder neck. One patient had a sensitive bladder. All patients had a urodynamic abnormality. The authors believe that the STU is nearly always an indication of bladder instability or wide bladder neck anomaly. The most common mechanism for the dilatation of the posterior urethra is that unstable contractions are resisted by a voluntary increase in distal sphincter tension so as to prevent leakage of urine. The resulting pressure rise produces distention of the posterior urethra, which will be maximal in subjects with a weak bladder neck mechanism as in the congenital wide bladder neck anomaly. The authors believe that STU is seldom if ever a normal variant.     

Predictive role of arterial carboxyhemoglobin concentrations in ovine burn and smoke inhalation-induced lung injury

Inhalation injury frequently occurs in burn patients and contributes to the morbidity and mortality of these injuries. Arterial carboxyhemoglobin has been proposed as an indicator of the severity of inhalation injury; however, the interrelation between arterial carboxyhemoglobin and histological alterations has not yet been investigated. Chronically instrumented sheep were subjected to a third degree burn of 40% of the total body surface area and inhalation of 48 breaths of cotton smoke. Carboxyhemoglobin was measured immediately after injury and correlated to clinical parameters of pulmonary function as well as histopathology scores from lung tissue harvested 24 hours after the injury. The injury was associated with a significant decline in pulmonary oxygenation and increases in pulmonary shunting, lung lymph flow, wet/dry weight ratio, congestion score, edema score, inflammation score, and airway obstruction scores. Carboxyhemoglobin was negatively correlated to pulmonary oxygenation and positively correlated to pulmonary shunting, lung lymph flow, and lung wet/dry weight ratio. No significant correlations could be detected between carboxyhemoglobin and histopathology scores and airway obstruction scores. Arterial carboxyhemoglobin in sheep with combined burn and inhalation injury are correlated with the degree of pulmonary failure and edema formation, but not with certain histological alterations including airway obstruction scores.     

Bladder dysfunction and neurological disability at presentation in closed spina bifida

Congenital closed spinal anomalies are associated with distortion of the spinal cord, the spinal nerve roots or both, and can result in neurological abnormalities of the lower limbs and neuropathic bladder dysfunction. This study reports clinical and videourodynamic findings in a group of 51 patients with closed spina bifida. The mean age at presentation to a specialist neurourological clinic was 3.3 years. Twenty five patients presented with urinary tract disturbance and 12 presented with neurological problems. Thirty three had normal neurological examination or only minor objective signs, 21 had normal renal tract ultrasonography but only two patients had normal videourodynamics, with 31 having two or more abnormalities during this assessment. Neither clinical neurological assessment nor the history of voiding behaviour are reliable indicators of bladder dysfunction and subsequent risk of renal damage. Therefore, all patients with a known or suspected diagnosis of closed spina bifida should have videourodynamic assessment.

     

Neuropathic bladder and spinal dysraphism.

The association between spinal dysraphism and a neuropathic bladder is well known, but the diagnosis of the spinal lesion and the associated renal problems is often delayed. Four children referred with orthopaedic problems and in whom the bladder abnormally proved to be the major disability are described. Despite widely differing vertebral involvement, all had lower motor neurone neurological deficits confined to lumbar and upper sacral segments. All had unstable, variably thickened, small bladders, and it is proposed that the bladder abnormality is the result of a partial lesion of lumbosacral innervation, and not of an upper motor neurone lesion.