Xanthogranulomatous pyelonephritis in a paediatric cohort (1963-2016): Outcomes from a large single-center series.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic destructive granulomatous inflammation of the kidney. It was first described in 1916, and is thought to affect 6/1000 cases of pyelonephritis. Its manifestations are varied, and with a limited number of cases in the literature, the optimal diagnosis and management of XGP in the paediatric cohort is still unknown. MATERIAL AND METHODS: The medical records of children who were diagnosed and treated for XGP at the current unit during the period 1963-2016, inclusive, were retrospectively reviewed. Information pertaining to each patient was recorded, including: demographic data, past medical history, clinical and biochemical characteristics, diagnostic procedures, treatment methods, histopathologic diagnosis of the removed specimen, and outcome. RESULTS: A total of 66 children with a median age of 4.84 years (range 1.1-14.81), with an M:F ratio 1.35:1 underwent nephrectomy for XGP and had a median follow-up of 7.19 years (range 0.11-17.45). The most common presentations were systemic illness (62.1%), pain (60.6%), urinary tract infections (54.5%) and an abdominal mass (39.4%); pyrexia was present in 53%. Biochemical abnormalities included anaemia (86.3%), thrombocytosis (80.3%) and hypomagnesemia (65.1%). There was an 83.3% concordance between intraoperative cultures and positive mid-stream urines. Index kidneys were significantly larger than the contralateral side (mean 1.32 cm; P = 0.002). Staging of XGP demonstrated extension beyond the kidney in 79% of kidneys. Computed tomography (CT) was performed in 11 cases (Summary figure). Dimercaptosuccinic acid (DMSA) scan showed 0-10% function in 90.47% of cases. Surgical procedures included nephrectomy (n = 63) and partial nephrectomy (n = 3). Perioperative complications included colonic resections (n = 5) and abscess formation in 18%. CONCLUSIONS: This is the largest series to date of XGP in a paediatric cohort. XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscesses, renal masses and/or non-functioning kidneys with/or without associated urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct pre-operative diagnosis and appropriate management.

Xanthogranulomatous pyelonephritis in childhood.

BACKGROUND: Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively. AIM: To examine the relation between clinical history and the results of renal investigations performed in children with xanthogranulomatous pyelonephritis. METHOD: A retrospective review of 31 cases presenting with the histopathological diagnosis of xanthogranulomatous pyelonephritis between 1963 and 1999. RESULTS: The mean follow up was 8.2 years. The male:female ratio was 1:1.1. The left kidney was affected in 26 of the 31 patients. The positive findings on examination and investigation at presentation were: fever, 16 children; pyuria, 26 children; positive urine culture, 16 children. A haemoglobin of < 100 g/l was measured in 27 of 31 patients and 15 of 18 patients tested had a raised erythrocyte sedimentation rate of > 20 mm in the first hour. Twenty six children had renal calculi, with a large reduction in the function of the affected kidney on isotope scintigraphy in 27 of the 29 patients tested. Hypertrophy of the contralateral kidney, shown on imaging, was present in 17 of 31 patients. CONCLUSIONS: Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively.

Urological problems in conjoined twins.

OBJECTIVES: To review the urological abnormalities in conjoined twins, a rare congenital abnormality arising from an incomplete separation of a pair of monozygotic twins, and who can be joined from the head to the pelvis with varying degrees of union. PATIENTS AND METHODS: The records and urological anomalies of conjoined twins presenting from 1985 to 1995 were reviewed retrospectively and the management and surgical reconstruction described. RESULTS: Seven of 11 sets of conjoined twins underwent surgical exploration. Complex urological problems occurred in all twins with omphalo-ischiopagus. The urological anomalies were: Set 1; both twins had one lateral crossed fused renal ectopia with uncrossed ureters draining into a single bladder with one urethra. They had one set of genitalia. Each twin received one crossed fused ectopic kidney, half the bladder and genitalia, with one retaining the urethra and one a vesicostomy. Set 2; these twins had four kidneys (two lateral and two central that were fused), four ureters (two of which crossed) and these drained into two sagittal bladders that emptied by one urethra associated with a single set of external genitalia containing three corpora cavernosa. At separation, the crossed ureters were re-routed, each received a bladder and the genitalia were split. Set 3; there were four kidneys with two crossed ureters draining into two collateral bladders, there was a partial duplication of the urethra and a single hypospadiac penis. After separation, each had kidneys draining into a bladder, one received the urethra and external genitalia, and one a urostomy. CONCLUSION: Complex urological problems occur in conjoined twins, although restricted to those with pelvic fusion (ischiopagus). Assessment and treatment needs to be individualised and overseen by a surgeon with experience in complex urology.

Higher-order suppression in diffraction-grating monochromators using thin films.

Although a continuously tuneable source of photons is a very desirable feature of synchrotron radiation it has one main drawback: the contamination of the photon beam by higher-order diffracted light. Several elements have absorption edges which lie between 10 and 200 eV, a range prone to high second- and third-order content in XUV monochromators. They can, therefore, be used as transmission filters to reduce this higher-order content. This paper describes the use of thin filters to reduce the higher-order content in diffraction-grating monochromators. Their suppression efficiency, transmission and ageing have been characterized using photoelectron spectroscopy and compared with calculated values. The effect of oxide contamination on their performance has been assessed. Filters are now installed on eight XUV beamlines and have been in routine use for several years.

Development of high-throughput flexible multichannel electron detectors at Daresbury Laboratory.

Continuing demands from Synchrotron Radiation Source (SRS) end-users for higher throughput and improved reliability in photoelectron spectroscopy experiments have driven an intensive development programme for new multichannel electron detectors. The development philosophy focuses on high throughput to match present and future source intensity, flexible structures to allow increased mobility of designs and modular design for easy maintenance and repair. Developments include parallel readout electronics and innovative detector heads for the hemispherical deflection analysers currently in use on the SRS. Novel anode arrays have been implemented in the detector heads and extensive microchannel plate (MCP) characterization has been undertaken to source the MCPs most suited to this application. The present multichannel detection systems provide a significant enhancement to single-channel detection systems. They have also surpassed previous multichannel detection systems due to their high throughput, flexible structure and modular design. Information on these developments and experimental results obtained at Daresbury Laboratory are presented.

Combined Mitrofanoff and antegrade continence enema procedures for urinary and fecal incontinence.

PURPOSE: Fecal soiling or intractable constipation frequently occurs in association with urinary incontinence in children undergoing major reconstructive urological operations. To treat double incontinence or the combination of wetting and severe constipation, we constructed a Mitrofanoff conduit and a channel for antegrade continence enemas in 18 patients between 1989 and 1995. We review the underlying pathological conditions, various surgical techniques and outcomes of these operations. MATERIALS AND METHODS: Underlying abnormalities mainly included spinal lesions, bladder exstrophy, imperforate anus and various cloacal anomalies. Patient age ranged from 2 to 18 years (average 8.4). In 13 patients both procedures were done simultaneously. The appendix was used to construct the antegrade continence enema channel in 8 cases and the Mitrofanoff channel in 5. It was long enough to be divided and used for both procedures in 2 cases but it was missing or unsuitable in 3. Alternative antegrade continence enema conduits were cecal flap in 7 patients and ileum in 1, while the ureter, ileum and detrusor tube were used to establish Mitrofanoff channels in 5, 5 and 1, respectively. Stomas were constructed according to the V-flap or V. Z. Q. technique and situated in close proximity in the right lower abdominal quadrant in 13 cases. RESULTS: Convalescence was uneventful except for 1 abscess near an antegrade continence enema stoma. Ten patients needed dilation or minor revisions due to difficulty in catheterizing the antegrade continence enema (5), Mitrofanoff (3) or both conduits (2). Subsequently 3 patients underwent repeat operations for reconstruction of 2 antegrade continence enema channels (cecal flap and ileum) and 1 detrusor tube Mitrofanoff channel. Currently 15 patients are dry on regular clean intermittent catheterization using 10 to 12F catheters. Outcomes of the antegrade continence enema channels are satisfactory in 15 patients who are clean or rarely soil. Failure occurred in 1 patients with severe constipation necessitating colostomy and 2 (1 noncompliant who stopped catheterizing regularly) in whom the channels subsequently closed. CONCLUSIONS: Synchronous construction of antegrade continence enema and Mitrofanoff channels is successful in the majority of doubly incontinent patients. Selection of patients with high motivation is important to obtain satisfactory results.

Redefining the genital abnormality in the Robinow syndrome. -004.

PURPOSE: We defined the anatomical structure of the male genital abnormality in the Robinow syndrome. Features of this syndrome include mesomelic brachymelia of the arms, bifid terminal phalanges of the hands and feet, characteristic facies, skeletal anomalies and hypoplastic external genitalia. MATERIALS AND METHODS: Penile anatomy of 3 patients with the Robinow syndrome was assessed using computerized tomography and magnetic resonance imaging. Results were compared to those of 4 controls who underwent imaging for pelvic malignancies. RESULTS: Cross-sectional imaging showed that normal penile crura were inserted onto the anteromedial aspect of the pubic bone. In contrast, in the Robinow syndrome they were inserted inferiorly and posteriorly onto the medial aspect of the ischial tuberosity. In addition, the crura in the Robinow syndrome extended posterior to a line intersecting both femoral shafts. Compared to controls, there was a significant gap between the symphysis pubis and dorsal aspect of the penis. CONCLUSIONS: The penile anomaly in the Robinow syndrome is due to abnormal insertion of the penile crura, resulting in a penis that appears shorter and more inferiorly placed between the legs.

Radical single stage reconstruction in failed exstrophy.

Staged repair of exstrophy-epispadias complex cases with initial bladder closure followed by epispadias and bladder neck repair at a later date is well documented. However, the role of single stage repair as a primary and salvage procedure after failed closure is not well established. A total of 12 male patients with bladder exstrophy has undergone single stage repair at our facility (10 secondary and 2 primary procedures). Mean age of the 10 patients referred for secondary repair was 5.5 years (8 months to 13 years). In this group there had been 16 previously unsuccessful attempts at bladder closure and 15 operations for epispadias repair. The 2 primary repair patients were 15 months and 13 years old. Continence was achieved by bladder neck reconstruction combined with epispadias repair and all cases were augmented by enterocystoplasty in conjunction with a Mitrofanoff channel. Pelvic osteotomy was performed in 3 cases. After bladder neck revision in 2 children all are dry day and night on clean intermittent catheterization and have a satisfactory cosmetic appearance. Single stage reconstruction of the exstrophy complex is a satisfactory method with a low morbidity for treating children in whom previous repair has failed. Its more extended role in the primary case requires further evaluation.

Augmentation cystoplasty in boys with posterior urethral valves.

The role of augmentation cystoplasty in the neuropathic and exstrophy bladder has been well documented. However, its place and the timing of such surgery in the "valve bladder" are not well established. We report our experience with augmentation cystoplasty in 20 boys with previously treated posterior urethral valves. Urodynamic studies confirmed poorly compliant, unstable bladders with low functional capacities, which had failed to respond to anticholinergic treatment in all patients. The bladder was augmented with ileum in 9 cases, stomach in 7, colon in 2 and ureter in 2. A Mitrofanoff channel was fashioned in 6 cases. Upper tract dilatation improved in 17 patients and remained stable in 3. Of the patients 17 are dry day and night. Eleven patients void spontaneously without significant residual urine, 7 are on clean intermittent catheterization for residual urine of greater than 50 ml. and 2 are completely dependent on catheterization. Augmentation cystoplasty is a safe and effective method to achieve continence with a low capacity, poorly compliant bladder in children with posterior urethral valves who do not respond to medical management. In contrast to the neuropathic and exstrophy bladder, the augmented valve bladder allows spontaneous voiding without significant residual urine in the majority of cases. Early intervention in these patients may prevent deterioration in renal function.

The influence of trisomy 21 on outcome in children with Hirschsprung's disease.

The association of Hirschsprung's disease (HD) and trisomy 21 has been well recognised. Seventeen (13%) of 135 patients presenting with HD between 1975 and 1992 had trisomy 21. Nine (53%) presented in the neonatal period, with intestinal obstruction (5), enterocolitis (2), or perforation of the colon (2). Eight patients presented after the neonatal period, with constipation. Pathological involvement included rectosigmoid (12), long segment (4), and total colonic aganglionosis (1). Definitive surgery was performed in 14 patients. At the mean follow-up of 8 years (4 to 15 years), only one of the 13 patients has normal bowel function. Eight have persistent soiling, and two have reverted to permanent stomata. There were two deaths in the series; one resulted from enterocolitis complicating HD, and the other from congenital cardiac disease. These data suggest that long-term bowel function in children with HD and trisomy 21 is poor and should be taken into consideration when planning the management.

Are the selection criteria for the conservative management in spina bifida still applicable?

Management of spina bifida is still controversial. At one extreme is the policy of total care in all cases while some form of selection is practised in many centres. We reviewed 104 patients treated over the last five years. Forty-nine patients, who had one or more adverse criteria, were managed conservatively. Twenty-four of these 49 patients required VP shunt insertions subsequently. Four patients had delayed closure of back lesions, three of them were followed by shunts. There was an overall increased (57%) incidence of surgical intervention in conservatively managed patients over the last five years as compared with 34% from our previous report. We found no difference in the timing of insertion of shunts in actively and conservatively managed patients in this study. The mortality in the actively and conservatively managed patients was 7% and 47% respectively. Eighty-five per cent of the patients who had no surgery died at an average age of 23 days. Patients who died without surgical intervention did not survive long enough to warrant surgery, while the mortality was 20% and had among those who survived surgical intervention. Paraplegia alone was not a predictor of high mortality but the presence of two or more adverse criteria were more accurate indicators of poor prognosis. As 78% of all the deaths in conservatively managed patients occurred within three months, reappraisal of management policy is necessary in the infants surviving beyond three months of age as death is less likely after this time.

Evaluation of the scrotal testis before and after orchidopexy in experimental unilateral cryptorchidism.

Unilateral cryptorchidism was induced in newborn Wistar rats. With standard histological techniques, no abnormality was found in the scrotal testis at any age. Using DNA flow cytometry to measure the proportion of cells in the haploid phase in the testis, secondary degenerative changes were noted at 40 days of age. Orchidopexy carried out at 30 days of age prevented these changes appearing; at 50 days reversed the abnormality already present; and at 90 days failed to reverse the degeneration of the scrotal testis. These data would suggest that although early orchidopexy can prevent secondary changes in the scrotal testis, operation up to puberty will allow these changes to be reversed.

Reversal of degenerative changes in the scrotal testis after orchidopexy in experimental unilateral cryptorchidism.

Unilateral cryptorchidism was induced in newborn Wistar rat pups. Using DNA flow cytometry to compare scrotal testes of control and cryptorchid animals, there was a significant difference decrease in the haploid cell population in cryptorchid animals from 40 days of age. Orchidopexy was performed at 30 days (at the time of normal descent), 50 days (pubertal), and 90 days (fully mature adult). When with sham-operated control and cryptorchid animals of the same age were compared, there was no decrease in the haploid population at any age in the scrotal testis if orchidopexy was performed at 30 days; the degenerative changes could be reversed to normal if orchidopexy was performed at 50 days; however, if operation was delayed to 90 days, the decreased numbers of haploid cells in the scrotal testis were neither prevented nor reversed and remained significantly lower than normal controls (P less than .005). This study shows that orchidopexy performed before adulthood reverses secondary degenerative changes in the scrotal testis in an animal model of unilateral cryptorchidism.

Secondary changes in the scrotal testis in experimental unilateral cryptorchidism.

Unilateral cryptorchidism was induced in under 2-day-old Wistar rat pups. A control group of rats underwent sham operation at the same age. The animals were killed at intervals from 5 to 120 days, both testes were excised, weighted, and processed for histological examination, morphometric measurement of the seminiferous tubules, and DNA flow cytometry. There was no difference in weight, Johnsen score, and tubular size between the scrotal testis of cryptorchid animals and control testes at any age. Significant decreases in all of these parameters occurred in the undescended testis from 30 days when compared with the scrotal testis in cryptorchid animals and controls. Using flow cytometry to measure changes in the DNA ploidy of the cells of the seminiferous epithelium during spermatogenesis, a significant decrease in the haploid population of cells occurred in the scrotal testis of cryptorchid animals at 40 days. This difference continued into adult life (P less than .005). Flow cytometry demonstrates a secondary decrease in spermatogenesis in the scrotal testis in experimental unilateral cryptorchidism.

Endoscopic management of vesico-ureteric reflux in children with neuropathic bladder secondary to myelomeningocoele.

Vesicoureteric reflux (VUR) is a major cause of morbidity and mortality in children with neuropathic bladders secondary to myelomeningocoele. The majority deteriorate with age and early treatment is necessary to avoid this. Endoscopic subureteric Teflon injection ("STING") allows correction of reflux in small children who would otherwise be difficult to manage. The results of treatment of VUR in 41 ureters in 24 children with neuropathic bladders using "STING" are reported. All ureters had grade III-V VUR. Thirty ureters (73%) showed disappearance of reflux after one injection. Of the remaining 11, VUR disappeared in 7 after a second injection. Apart from failure, the only other major complication was septicaemia in one patient. The mean hospital stay was 1.4 days (1-8 days). At a mean follow-up time of 22 months there was radiological improvement in the upper renal tracts in 27 of the 37 (73%) in which VUR disappeared, none has deteriorated and there has been only one relapse. These results suggest that "STING" should be considered as the method of choice in the management VUR in children with neuropathic bladder in view of the problems associated with open operation in these children.