Biomedical subjects
Publications and source records attributed to T P de Jong.
A 5-year-old girl presented with repeated urinary tract infections. A voiding cystourethrogram showed bilateral reflux in the lower poles of a bilateral duplicated collecting system.
Pediatric urodynamics taught us that detrusor-sphincter dyssynergia creates a bladder outlet obstruction in about 50% of any population of children with myelomeningocele. This functional obstruction causes renal damage due to obstructive uropathy, exactly the same way as a congenital anatomical urethral obstruction does. Pediatric urodynamics also taught us that in children with myelomeningocele pelvic floor activity and detrusor activity can be abnormal (hyperactive or inactive) completely independent from each other. These insights have changed the management of myelomeningocele. Children with overactivity of the pelvic floor can be singled out at infant age, and started on clean intermittent catherization, to prevent obstructive uropathy and preserve renal function. Children with detrusor overactivity can be singled out too at very early age, and treated with anticholinergics, to prevent irreversible structural damage to the detrusor and preserve normal bladder capacity and compliance.
We describe four cases of eosinophilic cystitis in whom no specific cause could be found, and review the literature. Complaints at presentation included urgency, frequency, abdominal pain, and haematuria. In three patients the symptoms and ultrasound pictures suggested a bladder tumour. One patient was treated with anticholinergics and corticosteroids without relief of symptoms; a localised eosinophilic tumour was excised in one patient who remained symptom free; and two patients were managed conservatively with spontaneous resolution of bladder pathology and symptoms. One case was identified by random bladder biopsy in 150 consecutive patients with unexplained irritable micturition complaints. Eosinophilic cystitis is rare in children. After biopsy, we consider a wait and see policy is justified as symptoms tend to disappear spontaneously. Routine bladder biopsies in children with unexplained bladder symptoms is not justifiable.
PURPOSE: There is growing interest in preputial reconstruction combined with hypospadias repair. We retrospectively analyzed its results for future developments and patient information. MATERIALS AND METHODS: We evaluated 77 boys who underwent distal hypospadias repair combined with preputial reconstruction to determine complications, risks and failures. RESULTS: At a mean followup of 2.5 years 52 patients had an anatomically normal penis with a normal retractable foreskin, while 25 (33%) presented with a complication. The most common complications were partial dehiscence, and fistula of the prepuce and urethra. There was a complication of the reconstructed foreskin only in 16 cases (21%), a combined problem with the reconstructed foreskin and reconstructed urethra in 7 (9%), and a problem with the reconstructed urethra in 2 (3%). Of the 25 patients with complications 19 underwent reoperation with closure of the fistula or dehiscence and 5 were circumcised, while in 1 the parents accepted the minor cosmetic problem and refused reoperation. CONCLUSIONS: Preputial repair combined with hypospadias repair may lead to anatomically correct reconstruction of the penis at the cost of a 33% complication rate. Parents are informed about this risk and to date in 15% of all boys with distal hypospadias the parents have elected preputial reconstruction.
A 6-year-old boy developed macroscopic haematuria on the 4th day after appendectomy for acute appendicitis, at which the appendix was found to be perforated. During the next few days the urine secretion decreased and malaise, pain in the lower abdomen, nausea and vomiting occurred. On a management of ample fluid administration, the urine secretion recovered and the symptoms subsided in a few days. In the early postoperative stage after appendectomy in children the possibility should be kept in mind of the development of acute renal insufficiency due to bilateral ureteral obstruction as a result of oedema of the posterior bladder wall, even if by means of ultrasonography only mild to moderate abnormalities are noted. Awaiting decompression by means of the introduction of bilateral ureteric stents, in order to prevent irreversible renal damage, supportive therapy with fluid administration depending on the diuresis seems indicated.
PURPOSE: Female epispadias is a rare anomaly. According to the literature it is usually treated with staged procedures, including bladder neck reconstruction, to achieve continence. We developed a 1-stage surgical technique that offers the possibility of achieving continence and a cosmetically normal appearance of the vulva. MATERIALS AND METHODS: We treated 4 patients 4 months to 8 years old. The main point of the technique is to free completely the urethral plate and bladder neck from surrounding tissue. After tubularizing the urethral plate into a urethra modified needle suspension brings the bladder neck and proximal urethra into the intra-abdominal position. The pelvic floor is then reconstructed between the anterior vaginal wall and urethra. Thus, continence may be attained by intra-abdominal positioning of the bladder neck and proximal urethra as well as by pelvic floor reconstruction. RESULTS: Of our 4 consecutive cases of primary untreated epispadias the technique proved successful in 3, while followup is too short in 1. One patient is completely dry and voids without a further procedure. Postoperatively 2 patients with 5 years or more of followup required injection of a bulking agent at the bladder neck level to achieve continence, including 1 who is damp during the day without the need to change clothes and 1 on clean intermittent catheterization twice daily because post-void residual urine volume causes recurrent urinary tract infection. CONCLUSIONS: The described technique is promising for treating this disabling anomaly.
PURPOSE: The treatment of ectopic ureterocele is controversial. In addition to debate on optimal therapy, discussion exists on whether there is further risk of deteriorating bladder function after extensive bladder surgery during the first year of life, which is a reason to postpone surgery. In a prospective nonrandomized trial we treated 40 patients regardless of age who had ectopic ureterocele with complete surgical reconstruction of the lower urinary tract and upper pole resection of poorly functioning upper pole moieties at referral. Excluded from study were 3 patients with only 1 affected renal moiety initially. MATERIALS AND METHODS: We treated 31 female and 9 male patients 0 to 8.8 years old (mean age 2.17) at surgery for ectopic ureterocele extending into the bladder neck and urethra, including 19 younger than 1 year. Primary ureterocele excision was performed in 37 cases with reconstruction of the urethra, bladder neck and trigone, and ureteral reimplantation. Because of small ureterocele size, the ureterocele was left in situ in 3 patients, leading to secondary ureterocele removal due to obstructive voiding and urinary incontinence in 1 each. A staged procedure in 5 neonates involved primary lower urinary tract reconstruction with upper pole cutaneous ureterostomies followed by upper pole resection or ureteral reimplantation a few months later. After bladder neck reconstruction in 16 cases colposuspension was also done to create a normal vesicourethral angle. All patients underwent clinical and urodynamic evaluation at least 1.25 years after surgery (mean followup 5.59). Patients who were too young for the clinical assessment of continence by January 1999 were excluded from study. RESULTS: All patients are continent. A secondary endoscopic procedure was required in 13 cases, including cystoscopy only in 2, scar incision near the ureteral orifice in 3, endoscopic reflux treatment in 4, ureterocele remnant resection in 2 and bladder neck incision for obstructive voiding in 2. Secondary open bladder reconstruction was performed in another case due to a diverticulum. Postoperatively only 1 or 2 uncomplicated episodes of urinary tract infection developed in 11 patients, while there were recurrent urinary tract infections in 4. In a patient with a preexisting loss of renal function a severe infection led to renal scarring. The voiding pattern was normal in 29 patients but 11 had dysfunctional voiding, including 5 with recurrent urinary tract infection. Urodynamic followup confirmed these clinical findings. Bladder capacity in these patients was relatively high at an average of 124% of expected capacity for age. We noted no statistically significant difference in followup parameters in patients who underwent surgery before and after age 1 year. Additional colposuspension in 16 patients did not result in any significant change in outcome compared with that in patients without this procedure. CONCLUSIONS: When compared with results in the literature, complete primary lower urinary tract reconstruction in patients with ectopic ureterocele appears to have better results than a staged approach with initial endoscopic treatment. Moreover, our study provides no proof that extensive reconstructive bladder surgery in neonates and infants leads to bladder function deterioration at a later age.
Lasting cure rates in monosymptomatic nocturnal enuresis (MNE), using the alarm, imipramine or desmopressin, have been quoted as 43%, 17% and 22%, respectively. The low cure rates in addition to the number of different treatments indicate insufficient knowledge of MNE. Only research on arginine vasopressin (AVP) levels and nocturnal enuresis is unique in attempting to find a group within the MNE population that could benefit from substitution therapy with desmopressin. AVP levels are restored or amplified during desmopressin treatment. However, low nocturnal AVP production with high nocturnal urine output may be indicative of a disturbance in circadian rhythm. Pre-clinical data suggest a role for melatonin in the regulation of endogenous AVP and in the regulation of the sleep/wake cycle.
UNLABELLED: Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary lymphoedema of the penis and scrotum are described. The first case presented with lymphoedema of the prepuce and the phallic skin that persisted after the age of 1 year. Circumcision and excision of the subcutaneous tissues of the phallic skin were successful in producing the appearance of a normal circumcised penis. The second case presented with oedema of both phallic and scrotal skin. At age 1 year only a pastous thickening of the prepuce remained, scrotal and phallic skin normalised spontaneously. Routine circumcision was successful with a 5-year follow up. The third case presented with persistent oedema of both the scrotum and the phallus. He suffered leakage of lymph requiring incontinence pads, with complicating skin infections recurring every 4-6 weeks at age 8 years. After complete peeling of the scrotal and phallic skin from subcutaneous tissue some leakage persisted, infections subsided. The cause of this disorder remains unknown, although hypoplasia of the lymphatic vessels is reported in most cases. In the literature, several congenital malformations have been associated with primary lymphoedema. These were not noted in our patients. Rarely, the lymphoedema regresses spontaneously. The treatment of persistent lymphoedema is surgical and consists of meticulous excision of all subcutaneous layers of the affected skin, combined with reconstruction of the penis and/or scrotum. CONCLUSION: Primary lymphoedema of the male external genitalia is an extremely rare malformation of the lymphatic vessels of unknown origin. If persistent, surgical treatment is necessary.
OBJECTIVE: To evaluate whether colonic enema irrigation influences the urodynamic characteristics of patients with spina bifida, an overactive bladder and detrusor sphincter dyssynergia (DSD). PATIENTS AND METHODS: Since 1991, 83 patients with spina bifida at our institution have treated their bowel dysfunction by colonic irrigation every 24-48 h. In 12 patients (seven boys and five girls, mean age 7.7 years, range 0.7-13.8) with an overactive bladder and DSD, urodynamic studies of the bladder before and after enema treatment were available with no intercurrent changes in urological therapy. RESULTS: There were no significant changes overall in bladder capacity, leak-point pressure, bladder compliance and bladder instability in the selected group of children. CONCLUSION: Although enema therapy for bowel treatment in patients with spina bifida gave good results for faecal incontinence, with good patient compliance, no favourable effect on bladder function should be expected in most patients with a high-risk urinary tract dysfunction. Further study is needed to determine factors in patients who will benefit urologically from enema treatment.
OBJECTIVE: To assess the effectiveness of a 10-day inpatient treatment programme for persistent urge incontinence based on behavior modification via biofeedback of micturition behavior. METHODS: 95 patients aged 6-17 (86 girls, 9 boys) with documented and persistent urge incontinence, with or without dysfunctional voiding, mostly based on recurrent urinary tract infections, and at least a 1 year lasting failure of standard regimen and pharmacological therapy, were 'cognitively' treated. After 6 months the patients were evaluated for flow pattern, number of wet incidents, micturition frequency and urge compliants. RESULTS: 65 patients (68.4%) obtained good results, 12 (12.6%) showed average improvement, 18 patients (19%) did not improve. CONCLUSIONS: This cognitive noninvasive treatment programme seems promising in its effectiveness and compares favorably with existing biofeedback methods based on urodynamic procedures, although expensive by its inpatient status. Further study towards an outpatient implementation is needed.
Controversy exists about the timing of surgery in neonates and infants with congenital anomalies such as refluxing and/or obstructing megaureters and ectopic ureteroceles. Discussion acuminates to the fact whether or not early reconstruction causes irreversible damage to the urodynamic properties of the bladder. Between 1986 and 1992, 49 neonates and infants with obstructing or refluxing megaureters and 23 neonates and infants with ectopic ureteroceles have been operated in our hospital with a mean follow-up of 7.3 years. Reimplant surgery consisted of a modified Politano Leadbetter procedure, ureterocele surgery consisted of complete excision of the ureterocele, including the urethral part, with reconstruction of the urethra, bladder neck and bladder base combined with ureteral reimplants. Urodynamically no unexpected changes or deteriorisation have been seen in any of the patients. Bladder capacity for age, especially in the reflux group, averages 200%. Two of the ureterocele patients needed clean intermittent catheterisation for several years. Results of reflux cure in megaureter surgery were disappointing in ureters with a flat diameter between 6 and 9 mm's that were not recalibrated leading to the conclusion that in young children recalibration of the distal ureter should be done from 6 mm's upwards. No post-operative ureteral obstruction was observed in any of the cases. The conclusion is that early major reconstructions of the lower urinary tract causes no specific harm to the urodynamic properties of the bladder and pelvic floor, provided that the surgery is performed by specialised pediatric urological surgeons. The reported urodynamic problems in this patient group are probably related to lack of experience to deal with dysfunctional voiding habits that are quite common in these children, also after successful surgery. These micturation problems are not related to the surgical procedures, they are the result of pre-existing urodynamic changes of bladder function in these children.
We report on a large family with the ectrodactyly, ectodermal dysplasia, clefting (EEC) syndrome. The clinical manifestations in this family show great variability. Specific genitourinary anomalies were found. The propositus with micturition problems is discussed in detail. A dysplastic bladder epithelium might be the cause of these problems. A remarkable improvement of the complaints was achieved upon treatment with synthetic sulfonated glycosaminoglycans.
A consecutive series of 90 children with anorectal malformations was reviewed for urologic problems related to lower urinary tract dysfunction. Neurogenic bladder-sphincter dysfunction was seen in 22 patients (24%), all but one of whom had sacral agenesis. Vesicoureteral reflux was present in 60% of the patients with dysfunction, and 32% had reflux nephropathy. Urinary incontinence was present in 91% of the patients who had bladder-sphincter dysfunction. However, the management of bladder-sphincter dysfunction and urinary incontinence was not as straightforward as for patients with myelodysplasia because the parents were more reluctant to accept the therapeutic measures. It was particularly difficult to introduce clean intermittent catheterization (CIC), especially in older patients, because genital and urethral sensation often was undisturbed. Only if CIC had been started in the neonatal period or early infancy were there no problems with acceptance and parental compliance. The authors emphasize the importance of urodynamic testing of neonates and infants who have an anorectal malformation and associated sacral agenesis in identifying those who have neurogenic bladder-sphincter dysfunction. Consequently, patients with lower urinary tract dysfunction should receive prompt treatment, including CIC if necessary, to prevent or reduce secondary urologic morbidity, especially loss of renal function.
A series of 90 children with anorectal malformations received a total of 163 urodynamic studies. The mean number of studies per patient was 1.8 (range, 1 to 7), and the mean age at the time of initial evaluation was 17 months (range, 1 to 128). Sacral anomalies were found in 38 children (42%); 30 of them had partial sacral agenesis and eight had sacral dysplasia without bony defect. Normal lower urinary tract function was found in 98% of the children who had a normal sacrum, in all children who had sacral dysplasia, and in a small number of those who had partial sacral agenesis that affected smaller parts of the S-4 and S-5 pedicles. Severe lower urinary tract dysfunction was observed in 22 patients (24%), all but one of whom had partial sacral agenesis. There was no correlation between the type of sacral agenesis and a specific urodynamic pattern. It is concluded that virtually all children with an anorectal malformation and associated sacral agenesis have neurological bladder-sphincter dysfunction and therefore should receive early urodynamic assessment and appropriate urological treatment.
Based on several intervention programmes, a strategy for the treatment of nocturnal enuresis has recently been developed by an expert committee in the Netherlands. It consists of three parts. First, two structured interviews are given: one to differentiate between enuresis and incontinence and one to detect associated problems such as diurnal enuresis, constipation or behavioural problems. Secondly, a medical examination is made, confined to the inspection of the external genitalia and lower back, palpation of the abdomen and urine examination. Thirdly, the following guidelines for treatment at different age levels are applied: up to the age of 6 years no intervention is needed; between the ages of 6 and 8 years, lifting out of bed and/or the calendar method; between the ages of 8 and 12 years, enuresis alarm (if not successful, medication with desmopressin is prescribed for a restricted period of time), and ambulatory dry-bed training in a group setting may follow; over 13 years of age, clinical dry-bed training according to the Messer/Azrin method is advised. According to the expert committee, these guidelines offer sufficient possibilities to deal with the problem of nocturnal enuresis.
PURPOSE: We assessed urological problems and complications after scoliosis surgery in children with myelomeningocele. MATERIALS AND METHODS: A total of 16 children with myelodysplasia underwent surgery for scoliosis using different surgical techniques and instrumentation systems. Mean patient age at operation as 11 years and mean postoperative followup was 3 years. All patients had urological assessment before and after surgery, including urodynamics. RESULTS: Urological problems after surgical correction of scoliosis developed in 6 patients (38%). Three girls had difficulty performing clean intermittent self-catheterization postoperatively because of altered body posture, a genital pressure sore and a plaster body cast, respectively. In 4 patients, including 1 girl with problems performing clean intermittent self-catheterization, lower urinary tract function was altered, leading to upper urinary tract deterioration in 1 and worsening of urinary incontinence in 3. CONCLUSIONS: Children with myelomeningocele have a high incidence of urological complications after surgical treatment of scoliosis. Anticipation of the problems and a thorough postoperative urological evaluation, including urodynamics, can reduce morbidity and facilitate appropriate treatment.
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