Does diagnostic delay lead invariably to damage in episodic pelvi-ureteric junction obstruction?

OBJECTIVE: To determine the relationship between the length of history and renal function in patients with symptomatic pelvi-ureteric junction (PUJ) obstruction in childhood. PATIENTS AND METHODS: The study included 41 children with symptomatic PUJ obstruction. The duration and details of their symptoms were recorded and the differential renal function estimated by isotopic renography. RESULTS: The median (range) duration of symptoms was 24 (0.25-84) months; there was no statistically significant relationship between the duration of symptoms and differential function at presentation (P=0.148). CONCLUSION: There is no correlation between the duration of symptoms and renal function in children with PUJ obstruction. A delay in diagnosis cannot be directly linked to permanent renal compromise.

The use of renal scintigraphy in assessing the potential for recovery in the obstructed renal tract in children.

OBJECTIVE: To assess the value of renal scintigraphy with 99mTc-dimercaptosuccinic acid (DMSA) in predicting functional recovery after the surgical relief of obstructed kidneys in children. Patients and methods Forty-three children underwent surgery to relieve upper urinary tract obstruction; 37 had pelvi-ureteric junction obstruction and six had vesico-ureteric junction obstruction. The indication for surgery was a combination of an obstructed renogram with symptoms of either pain or pyelonephritis. Most children (41) had < 40% function on the affected side before surgery, with just two having hyperfunction (> 55%). In all patients intravenous urography before surgery showed hydronephrosis, and a micturating cystogram was used to exclude coexisting reflux in the presence of an associated megaureter. Diuretic renography (using 99mTc-mercaptoacetyl triglycine or 123I-hippuran) and DMSA scintigraphy were both carried out before surgery and the renography repeated 6 months afterward. RESULTS: The renographic drainage curves improved from obstructed to unobstructed or 'dilated unobstructed' on all postoperative studies. Regression analysis showed that preoperative DMSA scan was an excellent predictor of outcome (P < 0.001) whilst the preoperative renogram was a relatively poor predictor of the functional result. In four patients where the initial renographic function was < 10%, DMSA scintigraphy predicted correctly the capacity for recovery in three and the inability to improve in the fourth. Conclusion Before surgery, DMSA scintigraphy in children with upper urinary tract obstruction is a more useful estimate of probable long-term renal function than value from diuresis renography. If there is doubt about the desirability of reconstructive surgery, a DMSA scan may eliminate the need for more invasive methods of estimating recovery, e.g. a period of nephrostomy drainage.

Enterocystoplasty.

Despite the problems of augmentation cystoplasty, on balance it has been a much better form of management of the lower urinary tract in patients with bladder neuropathy or high pressure detrusor contractions than the alternatives of rectal diversion, indwelling catheter or external urinary diversion. The metabolic consequences do not seem to interfere with general health in the medium term. The risk of perforation appears to be present with other forms of augmentation cystoplasty or bladder replacement. However, the results are far from perfect and the ideal technique will be one that: removes the need for intraperitoneal surgery and prevents the risk of intestinal adhesions; stops the development of intestinal mucus and stone formation; prevents the metabolic complications and potential bony complications during adolescence; at the same time improves the patient's resistance to UTI; maintains the same degree of long-term, good, low-pressure urine storage and the consequent improvement and stability of the upper urinary tract.

Paediatric anatrophic nephrolithotomy; stone clearance - at what price?

OBJECTIVE: To evaluate the functional outcome of anatrophic nephrolithotomy in children. PATIENTS AND METHODS: All children undergoing anatrophic nephrolithotomy for complex branching and multiple renal calculi over an 11-year period were studied prospectively. Demographic data, treatment details and outcome, as assessed by X-ray, ultrasonography and isotope studies, were recorded. Anatrophic nephrolithotomy was carried out with surface cooling of the kidney followed by nephrostomy drainage for 5-7 days. RESULTS: Nine children (median age 4 years, range 7 months to 9 years) underwent anatrophic nephrolithotomy. Predisposing factors included urinary tract infection (by Proteus mirabilis) in all and hyper-calciuria in two children. The median (range) total ischaemic time at operation was 25 (15-40) min and the operative duration 150 (120-200) min. Three children required a blood transfusion. Stone clearance was incomplete in one child. There was no recurrent stone formation after a long-term follow-up (median 32 months, range 14-107) in the other patients. Isotope studies showed impaired split renal function (<40%) in six children before surgery; there was a significant decline (>5%) in divided function in five children (range 6-16%) after surgery. CONCLUSION: Anatrophic nephrolithotomy is an effective means of rendering children with branching calculi stone-free, but this study suggests that it leads to some further parenchymal damage.

The Mitrofanoff procedure: does it last?

OBJECTIVE: To examine the long-term effectiveness of the Mitrofanoff principle and establish if the catheterizing channel is sufficiently robust for long-term use. PATIENTS AND METHODS: Ten patients who had undergone Mitrofanoff reconstruction between 1989 and 1991 (minimum follow-up 10 years) were offered reinterview by one of the authors (J.F.), which involved a structured questionnaire assessing catheterization, continence and complications. RESULTS: One patient had died; nine patients were alive and eight agreed to the structured interview. All the patients had their original stoma and all were completely continent. Four of the patients had experienced stenosis, four had had stones and four had been ill with urinary tract infection(s). CONCLUSION: Despite the complications of infection, stones and some episodic stenosis, the Mitrofanoff channel remains functional for long periods without sustaining structural damage.

Adrenal cortical tumors in childhood.

In a 41-year period, 18 children with a diagnosis of an adrenal cortical tumor were identified (14 carcinoma: 4 adenoma). The majority of patients had clinical signs of endocrine dysfunction at presentation, with virilization (11 patients) and a cushingoid appearance (8 patients) the commonest findings. Abnormal biochemical activity was identified in 16 tumors (94%). The primary treatment in 17 patients was surgical. In addition, 12 children, all with carcinomas, had radiotherapy. Of those children with a carcinoma, 12 are dead, with a median survival of 52 months (range 1-317 months). The three second primary tumors all developed at sites within the field of previous radiotherapy; and proved fatal at 127, 176, and 317 months (median 207 months). This series confirms the poor prognosis in adrenocortical carcinoma in childhood, but a complete resection is compatible with cure of the primary disease. The frequency of second, fatal, primary tumors is of particular concern and long-term follow-up is mandatory in survivors, especially if radiotherapy was part of the treatment protocol.

The Malone antegrade colonic enema procedure: outcome and lessons of 6 years' experience.

The successful management of faecal incontinence can dramatically improve the quality of life of affected children. The introduction of the non-refluxing, catheterisable appendico-caecostomy provides the opportunity to treat previously resistant patients. Over a 6-year period, 29 children had a Malone antegrade continent stoma for enema administration (MACE). Incontinence was related to spina bifida in 12 children, ano-rectal anomaly in 12, Hirschprung's disease in 2, followed excision of a pelvic tumour in 2, and was secondary to intractable chronic constipation in 1. The conduit was fashioned from the appendix (20), a caecal tube (8), or a gastric tube (1). Surgical complications were stomal stenosis (11), wound infection (1), anastomotic leak (1), MACE stoma prolapse (1), and a pressure sore (1). Colonic irrigation was achieved with washouts of saline (24), saline plus phosphate (4), and saline plus Picolax (1). Twenty-three patients have complete control of bowel function, but 4 still soil. Two remain incontinent, 1 of whom is still being instructed. One child subsequently had a colostomy, but still uses the MACE stoma. Successful bowel management requires motivation, dedication, commitment, and the input of a clinical nurse specialist. The MACE is a relatively straightforward operative procedure that provides an effective washout technique that is acceptable to both parents and children.

Bladder washout and stone formation in paediatric enterocystoplasty.

OBJECTIVE: To review the incidence of stone formation in our patients with enterocystoplasty to determine the effect of regular bladder washout. METHODS: From 1988 to 1995, a prospective cohort of 30 children underwent enteroplasty with continent diversion. Over the same period, a consecutive group of 30 children had an augmentation alone. All were instructed to wash out their bladder on a weekly basis with sterile water. The frequency of the washouts increased if there were problems with increasing mucus production. Their incidence of stone formation has been compared to a similar group of 30 children performing clean intermittent self catheterisation (CISC) on their native bladders. RESULTS: Five (17%) children with continent diversions formed bladder stones (mean time to formation 35 months, range 13-59 months) were compared with 2 (7%) of children with augmentation. No child performing CISC alone formed stones. CONCLUSIONS: A regime of regular bladder washout in children with enterocystoplasty did not significantly reduce the incidence of stone formation when compared to previously published data.

Histology of the upper pole in complete urinary duplication--does it affect surgical management?

OBJECTIVE: To review the histological change in the upper pole of excised duplex kidneys and assess whether ante-natal diagnosis might predispose to more conservative surgical management of this abnormality. PATIENTS AND METHODS: Fifty consecutive patients undergoing upper pole hemi-nephroureterectomy for ectopic ureter or ectopic ureterocele between 1980 and 1992 had their histology reviewed and assessed for dysplastic, inflammatory and obstructive change. RESULTS: Segmental scarring and chronic and acute inflammatory change occurred consistently and the degree of inflammation seemed unaffected by antenatal diagnosis. Dysplasia was seen in 70% of patients with ureterocele and in only 30% of those with ectopic ureter. One patient had normal histology. CONCLUSION: There was no evidence of reversible histological change in patients with ectopic ureter and ectopic ureterocele who were diagnosed ante-natally. Preservation of the upper pole of the kidney does not seem justified in the light of the histological evidence.

Buccal mucosal graft for secondary hypospadias repair and urethral replacement.

OBJECTIVE: To assess the effectiveness of buccal mucosa in the treatment of complex hypospadias. PATIENTS AND METHODS: Seventeen patients had an onlay patch of buccal mucosa to reconstruct the urethra after failed previous surgery; five had a tubed buccal mucosal graft as an urethral replacement in the 'primary' repair of severe hypospadias. RESULTS: There were no complications in 19 patients; two developed fistulae and one a stricture due to graft narrowing. There were no meatal complications. CONCLUSION: Buccal mucosa taken from the inner aspect of the lip is very suitable for reconstructing a failed hypospadias repair, especially when used as an onlay graft.

Unilateral multicystic dysplastic kidney: the case for nephrectomy.

Management of unilateral multicystic dysplastic kidneys (MCDK) presents physicians and surgeons with a significant dilemma. Recent studies have indicated that the incidence of short term complications of MCDK is low and many authors have recommended conservative non-operative treatment. Surgery has been proposed by some because of the potential complications of hypertension, infection, and malignant change. Three children with hypertension secondary to MCDK seen at this institution in the past four years, one of whom had been discharged from follow up as a result of 'disappearance' of the cystic kidney on ultrasound examination, are reported. We believe that the risks of hypertension secondary to MCDK have been understated, and that based on the conclusions of these studies, many children may be receiving suboptimal follow up. We currently favour elective nephrectomy as the treatment of choice for this lesion.

Multicystic renal dysplasia diagnosed in the antenatal period: a note of caution.

OBJECTIVE: To document the urological problems associated with multicystic renal dysplasia (MCRD), to assess the efficacy of radiological methods for following the natural history of MCRD and to review current experience in the light of historical data regarding this condition. PATIENTS AND METHODS: From January 1982 to December 1992 data were obtained prospectively on 62 patients (41 boys and 21 girls, age range 1-11 years) who had a diagnosis of MCRD suspected on antenatal ultrasonography. All patients had the diagnosis confirmed with radioisotope scans and micturating cystograms. Serum creatinine was measured in each patient and repeated clinically as indicated. Follow-up was from 1 to 11 years, with a clinical review and an annual ultrasonogram. The natural history of these patients was compared with those in other published studies. RESULTS: Of the 62 patients, 12 had associated vesicoureteric reflux. Four patients developed impaired renal function during the course of observation and 10 patients required urological surgery for other pathologies. In the patients managed conservatively, 38% underwent no resolution on sequential ultrasonograms during the 2-year follow-up, and in four children, where complete resolution of the MCRD had occurred on ultrasonography, significant cellular renal masses were excised at subsequent surgery. CONCLUSION: Patients with MCRD have significant associated urological malformations and the natural history in such patients is unpredictable. We do not regard MCRD as an entirely benign condition and feel that all patients require appropriate investigation of their urinary tract and long-term follow-up where conservative treatment is instituted. From the natural history of these patients, reviewed in conjunction with the literature, we recommend that decisions for management can only be taken with the full informed consent of the parents and that surgical excision is offered as part of that process.

Ileocystoplasty in children: assessing safety and success.

From 1987 to 1992, bladder augmentation using clam ileocystoplasty was performed in 39 children 1.5-17.5 years old (mean age 9.7 years). Indications for augmentation included neuropathic bladder, epispadias-exstrophy complex and posterior urethral valves. A satisfactory bladder capacity at safe storage pressures of < 20 cm saline was achieved in all patients. Hydroureteronephrosis resolved or improved in 33 of the 36 moieties (91.7%). No patient showed biochemical or radiological deterioration of the upper tracts. The incidence of symptomatic urinary tract infection fell significantly postoperatively. Seven patients (17.9%) developed a total of 10 complications, with rupture of the augmented bladder occurring in 4 patients (10.3%).

Evaluation of augmentation cystoplasty in childhood with reference to vesico-ureteric reflux and urinary infection.

OBJECTIVES: To assess the non-operative treatment of vesico-ureteric reflux in patients with abnormal bladder compliance, to determine whether reduction in storage pressure by augmentation leads to cessation of reflux and whether symptomatic urinary tract infection worsens or renal function deteriorates if reflux continues after augmentation and clean intermittent catheterization (CIC). PATIENTS AND METHODS: Thirty-nine children (17 boys, 22 girls) who underwent clam ileocystoplasty over a 5-year-period were evaluated. The mean age at operation was 9.7 years (range 1.5-17.5). Pre-operative assessment included renal imaging and slow fill video-cystometry. Vesico-ureteric reflux, if present, was graded I-V. The frequency and severity of symptomatic urinary tract infections were recorded. Cystometry was performed 6 months after reconstruction. Storage pressures below 20 cm saline were regarded as safe. The mean follow-up was 3.3 years (range 10 months-6.1 years). RESULTS: Of the 17 renal units assessed for the outcome of reflux, 11 stopped reflux and one showed a lower grade. No patient showed worsening of upper tract dilatation or progressive renal scarring. Thirty-seven patients (95%) had symptomatic urinary tract infection pre-operatively. Following bladder augmentation, clean intermittent catheterization and trimethoprim prophylaxis, seven continued to have symptomatic infection. CONCLUSIONS: Augmentation cystoplasty is effective in resolving reflux in a majority of the patients. This is achieved by reducing storage pressures to below 20 cm saline and emptying the bladder regularly by CIC. There is also a reduction in the incidence and severity of symptomatic urinary tract infections. Even when reflux persists there is no evidence of deterioration of the upper tracts or progressive renal scarring.

Chronic hydronephrosis: renographic drainage patterns and renal morphology in an animal model.

OBJECTIVE: To develop an animal model of chronic progressive hydronephrosis; to establish patterns of diuretic renography during development of the condition; and to relate renographic abnormality to morphological change in the renal parenchyma. MATERIALS AND METHODS: The cellophane sclerosis technique was used to produce a hydronephrosis in the left kidney of six rabbits. Post-operatively MAG3 diuretic renography was performed at weekly intervals. When a steady renographic trend was noted the rabbits were killed and the kidneys examined histologically. RESULTS: Four rabbits had persistent type II renographic curves on the left for 12 weeks. The measured split renal function fell steadily to < 20% in this time. Morphologically there was hydronephrosis with marked structural changes in the parenchyma seen on histological examination. Two rabbits had stable type IIIa renographic drainage patterns on the left with preserved renal function. Morphologically there was hydronephrosis but histologically the kidneys were normal. There were no renographic or structural changes in a control rabbit after a sham laparotomy. CONCLUSIONS: The cellophane sclerosis technique can be used to create a reproducible chronic progressive hydronephrosis. In this animal model hydronephrosis and a persistent type II curve are associated with renographic evidence of deteriorating renal function and disruption of renal architecture histologically. Hydronephrosis and a type IIIa curve are associated with preservation of normal renal function and histological structure.

The radionuclide assessment of pre-natally diagnosed hydronephrosis.

OBJECTIVE: To determine if a pre-operative non-invasive radionuclide study can predict the results of surgery in infants with pre-natally diagnosed hydronephrosis. PATIENTS AND METHODS: Eight male infants with prenatally diagnosed hydronephrosis were treated by pyeloplasty within the first 4 months of life. All infants underwent a pre-operative diuresis renogram and a dimercapto-succinic acid (DMSA) scan, followed by a further renogram 6 months after surgery to assess renal function and drainage. RESULTS: There was a significant difference between the results of the relative renal function estimates obtained by the pre-operative renogram and the pre-operative DMSA scan (P = 0.001). In all patients the recovery in renal function following surgery was accurately predicted by the pre-operative DMSA scan. CONCLUSION: The results of this preliminary study suggest that a pre-operative DMSA scan could replace the use of invasive nephrostomy drainage to assess the potential for recovery in the poorly functioning neonatal kidney and give an indication of those kidneys most likely to benefit from reconstructive surgery.