Middle aortic syndrome: clinical and radiological findings.

Eight patients with the middle aortic syndrome are described. They were aged 2 months to 14 years at diagnosis; follow up was one to 11 years. Clinical presentations included asymptomatic hypertension (n = 5), severe headache, nose bleed, and chest pain (n = 1), and cardiac failure (n = 1). All had severe hypertension requiring multiple drug treatment. Diminished peripheral pulses were not helpful in the diagnosis, which is made on aortography. Associated clinical findings were Williams' syndrome (n = 3) and appreciable eosinophilia (n = 3). The differential diagnosis includes Takayasu's arteritis, fibromuscular dysplasia, and neurofibromatosis. Blood pressure was adequately controlled by medical treatment in six patients. Surgical angioplasty was performed in two. One patient remained normotensive without drug treatment 21 months after operation; the other died of sepsis and uncontrollable haemorrhage in the postoperative period. Medical treatment is satisfactory in most cases: surgery should be reserved for those in whom blood pressure cannot be controlled without unacceptable side effects of drug treatment. Although rare, the middle aortic syndrome should be considered in the differential diagnosis of hypertension when commoner causes have been excluded. Aortography is necessary for diagnosis.

Nonobstructive posterior urethral widening (spinning top urethra) in boys with bladder instability.

Seven boys between the ages of 5 and 10 years with symptoms of urinary frequency and urgency and daytime wetting were studied with urodynamics and were shown to have bladder instability and a dilated posterior urethra. In two the dilatation occurred predominantly during bladder filling. Unstable contractions caused filling of the posterior urethra, and leakage was prevented by voluntary contraction of the distal urethral sphincter; with voiding, the urethra showed a more normal appearance. In the remaining five, there were similar changes during filling, but dilatation persisted during voiding. In six the measured urine flow rate was normal, and none showed any evidence of anatomic obstruction. The mechanism of urethral distention appears to be similar to that previously shown in girls with spinning top urethra: Unstable contractions resisted by voluntary sphincter contraction cause posterior urethral dilatation. Boys with dilated posterior urethras who have urinary frequency and urgency and daytime wetting and normal urine flow rates should be assumed to have bladder instability.

Renal artery stenosis in children.

In a large paediatric renal unit over the last 14 years, 19 children (10 male and 9 female, aged 1 week to 16 years, mean 7 years) with renal artery stenosis (RAS) were evaluated. Transplant RAS cases were not included. All 19 children were hypertensive. In 10 this was an incidental finding. Based on clinical findings and arteriography, the causes of RAS included a middle aortic syndrome (MAS) (n = 5), neurofibromatosis (n = 3), William's syndrome (n = 3), fibromuscular hyperplasia (FMH) (n = 4), idiopathic RAS (n = 2) and isolated branch artery stenosis (n = 2). Previous studies have suggested FMH is the commonest cause of RAS in the paediatric population. In our study the largest subgroup are MAS/William's syndrome children, in whom the angiographic appearances were indistinguishable. Where possible, management, both surgical and radiological, and eventual outcomes have been described.

Urodynamics in the investigation of women with frequency, urgency, and incontinence, and voiding difficulties.

Women with voiding problems first require a full clinical history and examination to exclude nonvesical causes for their symptoms. Those with simple stress leakage do not normally require further study, but women with frequency, urgency, or nocturia with or without incontinence require proper investigation either by cystometrogram or video urodynamics. So too do women with hesitancy or difficulty in voiding. The technique of video urodynamics is outlined and the findings in different conditions leading to voiding problems is discussed, in particular the findings in the unstable bladder, the sensitive bladder, stress incontinence, poor bladder compliance, bladder outlet obstruction, and neuropathy.

Percutaneous stone and stent removal from renal transplants.

Techniques developed for removal of stones from normally sited kidneys can be safely employed in the transplanted kidney. We describe our experience in removing stones, stent material and organised blood clot from renal transplant collecting systems, using modified percutaneous techniques.

Normal and near normal caliceal patterns in reflux nephropathy.

Distorted, clubbed calices associated with focal renal scars or areas of more diffuse parenchymal loss make up the accepted urographic diagnostic criteria of chronic reflux nephropathy. It is widely believed that this diagnosis should not be suggested in the absence of the above findings and that scarring with normal calices indicates a vascular aetiology. This study describes five children with marked renal parenchymal loss all of whom have normal or near normal calices underlying the areas of parenchymal thinning and all of whom show severe vesico-ureteric reflux on micturating cystography. We suggest that these patients represent examples of true reflux nephropathy and that normal or near normal caliceal patterns may be seen underlying areas of renal parenchymal damage in this condition. No localised scars were seen in association with normal calices and we suggest that the marked tissue distortion caused by localised indented scars plays a significant part in producing the caliceal appearances more usually seen in reflux nephropathy. Other possible mechanisms responsible for the maintenance of relatively normal caliceal architecture are also discussed.

More medullary sponge variants.

Sponge kidney has characteristic plain film and urographic findings which are generally regarded as being limited to the medullary pyramids. We describe two groups of patients with renal changes of medullary sponge kidney who show additional renal abnormalities. In the first group there were multiple cortical cysts; in the second there were cavities deep in the medulla, many of which communicated with the calices. Neither of these findings has been described before and we suggest that the spectrum of changes associated with this condition is wider than has previously been appreciated.

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.

Radionuclide cystography in vesicoureteric reflux.

We investigated 47 children in whom vesicoureteric reflux was clinically suspected by both conventional radiological and indirect radionuclide cystography. Comparison of the two methods showed no significant difference in their accuracy in detecting vesicoureteric reflux. The advantages and disadvantages of, and indications for, indirect radionuclide cystography are discussed.

Arteriography of renal transplantation.

The arterial anatomy of renal transplants is often complex, with overlapping, tortuous vessels which prevent easy visualisation of the origins of the transplant artery. We have adopted a technique using smallbore catheters with non-selective 'flush' injections of contrast medium, high resolution magnification techniques and multiple oblique projections. A retrospective study over six years (1980 to 1985 inclusive) revealed 34 significant (greater than 50%) transplant artery stenoses (13 adults, 21 children) from 200 transplant arteriograms performed. During this period, 452 transplants were performed, 38% in children. The incidence of transplant artery stenosis was 7.5%. Percutaneous transluminal angioplasty was attempted in 11 patients (four adults, seven children), with technical success in only five (45.5%). The predominance of children in this group contributes to the poor success rate of percutaneous transluminal angioplasty in our hands, which we attribute to the small arteries involved, the acute vessel angulations seen and the tough undilatable stenoses which are encountered in transplants in paediatric patients. Fourteen patients with post biopsy arterio-venous fistulae are described and the role of embolisation in this condition is discussed. A simplified approach for the arteriography of live related donors is also described.

Sphincter behaviour in myelomeningocele.

Four hundred and two videourodynamic studies were performed on 207 children with neuropathic vesicourethral dysfunction due to myelomeningocele. The children were divided into three groups (contractile, intermediate and acontractile) according to the urodynamic behaviour of their bladders, and the behaviour of the bladder neck and distal sphincter mechanism was assessed in each group. The bladder neck may be competent or incompetent in children with contractile bladders but is never obstructive, and is always incompetent in children with either intermediate or acontractile bladders at their usual bladder volumes. The distal sphincter mechanism is nearly always dynamically obstructive (detrusor-sphincter dyssynergia) in children with contractile bladders but is rarely if ever incompetent, whereas in children with intermediate or acontractile bladders, both (static) distal sphincter obstruction and sphincter weakness incontinence exist to some degree, although the predominance of one of these may mask the existence of the other unless it is specifically looked for or some therapeutic manoeuvre unmasks it. The main conclusion from this study is that, with the possible exception of those few children with more minor partial cord lesions, dysfunction of one or both sphincter mechanisms is the rule in congenital cord lesions.

Videourodynamic evaluation of neuropathic vesicourethral dysfunction in children.

The findings of the videourodynamic (VUD) assessment of the vesicourethral dysfunction of 103 children with neurological disease are described. Detrusor-sphincter dyssynergia was the main determinant of voiding efficiency and the most important cause of impaired renal function. Storage efficiency was mainly determined by the behaviour of the bladder neck during the filling phase. The response to treatment of the child's incontinence was largely determined by the detrusor compliance. Detrusor contractility was found to be of 2 types distinguishable by several physical and functional characteristics rather than by amplitude alone. This may be of more than just academic and semantic importance. VUD studies are essential if conservative treatment modalities are to be rationally and selectively employed.