Biomedical subjects
Publications and source records attributed to T M Boemers.
Renal insufficiency developed in a newborn with huge bilateral renal cysts and posterior urethral valves. Definitive therapy consisted of laser coagulation of the valves and transient percutaneous drainage of the cysts.
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BACKGROUND/PURPOSE: The aim of this study was to investigate the effect of rectosigmoidectomy and Duhamel-type pull-through procedure on lower urinary tract function in children with Hirschsprungs disease. METHODS: During a 3-year period the authors assessed 11 consecutive children with Hirschsprung's disease prospectively by standard urodynamic investigations, before and after surgery. Urodynamics included simultaneous measurement of abdominal pressure, bladder pressure, detrusor pressure, and pelvic floor electromyography during filling and voiding. All children were submitted to laparoscopic resection of the aganglionic bowel segment below the cul de sac and a Duhamel-type pull-through procedure. Postoperatively, the children were assessed urodynamically and evaluated every 3 months for urologic problems. RESULTS: Mean age at first urodynamic study was 5 months (range, 2 to 10). Postoperative urodynamics were performed at a mean age of 10 months (range, 5 to 159). The mean interval between operation and postoperative urodynamic study was 6 months (range, 2 to 10). No child had structural urologic anomalies or urologic problems before surgery, and all had normal preoperative urodynamic findings. After surgery, urodynamics were considered normal in 3 children. In 7 children cystometric bladder capacity (CBC) was abnormally large, and 6 of these children had significant residuals. However, all had detrusor contractility and were able to void spontaneously. One child had low bladder compliance postoperatively. Despite the urodynamic changes, no child had clinical urologic problems at further follow-up. Mean follow-up after surgery was 24 months. CONCLUSIONS: This study found that after rectosigmoidectomy below the cul de sac alterations of bladder function can be observed. In 7 of the 11 patients studied, mean cystometric bladder capacity was 87% higher than capacity estimated for age. Moreover, postoperative residuals were 156% higher than the preoperative values. These findings suggest that partial detrusor denervation is likely in these patients. However, because detrusor contractility was present, and none of the children had retention or any urologic problems, the findings must be interpreted carefully. Because children with Hirschsprung's disease generally do not have preexisting urologic problems, routine preoperative urodynamic screening is not necessary. However, children with voiding problems after operation should be investigated urodynamically. For legal reasons parents should be informed of possible urologic problems, especially if subtotal resection of the aganglionic bowel segment is planned.
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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.
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.
A modified Mathieu technique for hypospadias with moderate chordee is presented. The modifications consist of a skin flap with a large pedicle derived from the dartos fascia, correction of the chordee by resection of the fibrous tissue and dorsal plication of the tunica albuginea and possible reconstruction of the prepuce. Defects of up to 2.5 cm long can be corrected with this modified technique. Seventy six children were operated according to this technique, with a follow-up of more than 24 months in 20 cases. The complications observed in these 20 patients were one fistula, two meatal strictures and one total loss of the skin flap due to infection.
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The effect of posterior sagittal anorectoplasty (PSARP) and its variants on lower urinary tract function was investigated urodynamically in 32 patients with anorectal malformations. In 27 patients urodynamic evaluation was performed before and after surgery and in 4 it was done postoperatively only. One child was clinically assessed with no postoperative urodynamic study. Minor postoperative changes in the specific preoperative urodynamic pattern were observed in 4 cases but the changes did not seem to be related to surgery. In 3 boys with rectourethral fistulas detrusor failure consistent with autonomic denervation was noted postoperatively. Standard posterior sagittal anorectoplasty was performed in 1 of the 3 boys and posterior sagittal anorectoplasty combined with additional transabdominal procedures was done in the other 2. In general our findings suggest that posterior sagittal anorectoplasty and its variants do not affect lower urinary tract function unless these surgical techniques are combined with major transabdominal procedures and extensive retrovesical dissection.
PURPOSE: We report a new surgical technique for the correction of buried penis. MATERIALS AND METHODS: The study comprised 10 boys with buried penis. The technique consisted of resection of abnormal dartos attachments, unfurling of the prepuce and correction of the deficient shaft skin by reapproximation of the preputial skin flaps in a defined way to obtain sufficient penile skin coverage. RESULTS: Outcome was excellent in 6 cases, good in 3 and unsatisfactory in 1. CONCLUSIONS: Perfect cosmetic results were obtained and no serious complications developed.
Historically, in female pseudohermaphrodites a staged procedure with early clitoral reduction and delayed vaginoplasty was often the treatment of choice. In recent years several authors have described 1-stage genitoplasty that is performed in the first year of life. The 1-stage procedure for clitoroplasty and vaginal exteriorization is preferable for an optimal functional and cosmetic result. Because of the emotional aspects of the family in intersex cases, neonatal reconstruction offers advantages to the child and parents. Furthermore, the perinatal genital hyperstimulation by maternal and placental estrogens that persists into the first 3 to 4 weeks of life produces vaginal enlargement by mucous secretion and vaginal wall hypertrophy, facilitating identification of the cleavage planes and vaginal pull-through. These arguments have led us to treat these patients by neonatal 1 stage clitorovaginoplasty. Cosmetic and functional results in 5 consecutive cases have been excellent without serious complications.
OBJECTIVE: To determine the effect of neurosurgical untethering on the lower urinary tract and male sexual function, in patients with tethered spinal cord. PATIENTS AND METHODS: Thirty-six children with tethered spinal cord due to neurospinal dysraphism were assessed clinically and urodynamically before and after surgical untethering. Sexual function was evaluated in 14 boys before and after neurosurgery. In young boys, the assessment was by parental observation and was considered normal if erections had been observed regularly, or if the child had mentioned having an erection at any time. Older patients were asked if they had achieved voluntary erections and whether or not they were able to ejaculate. RESULTS: The mean follow-up was 91 months and the mean follow-up after neurosurgery was 34 months. A lasting improvement of urinary tract function occurred in only one patient, while another became worse. Seven children had temporary changes of bladder/sphincter function; two developed detrusor overactivity with sphincter dyssynergia and five had signs of bladder denervation. Long-term follow-up showed no permanent changes of lower urinary tract function in 94% of 34 patients. Sexual function was considered normal in 13 boys. One boy apparently gradually lost erectile ability before untethering and regained it after surgery, while another boy had erectile dysfunction post-operatively for almost 6 months. CONCLUSION: Changes in bladder-sphincter function after untethering are usually transient and often the result of partial denervation. Although a small group of children seem to benefit from untethering, others can become worse and the individual outcome cannot be predicted. Erectile dysfunction can occur and parents and patients have to be informed of this possible complication. As pre-operative progression of neurological symptoms and post-operative denervation were observed only in patients with myelomeningocele and lipomyelomeningocele, this group may be distinct from patients with occult forms of spinal dysraphism. The natural history in the latter group of patients is unclear and a beneficial effect of prophylactic untethering remains to be proven by controlled prospective studies.
OBJECTIVE: To treat enuresis risoria (giggle micturition) by a self-administered electric and imaginary shock and to evaluate the outcome after behavioural therapy. PATIENTS AND METHODS: Six boys and three girls with enuresis risoria were evaluated and treated. The mean age at referral was 10.4 years (range 5.7-14.2). All children had episodes of involuntary complete bladder emptying triggered by hearty laughter or giggling. The frequency ranged from four times per day to twice a week. No other voiding problems were noted. Five patients (four boys and one girl, mean age 10.2 years, range 5.7-14.2) received conditioning training which consisted largely of the self-administration of a harmless, painless electric shock to the back of one hand, at the moment when micturition was induced by laughter, leading to inhibition of the voiding reflex. Later, the electric shock was replaced by an imaginary shock. Training was undertaken on an outpatient basis. A mean of eight sessions, each of 45 min duration, was necessary to train the children. The mean follow-up was 26 months (range 12-51). RESULTS: The frequency of wetting was reduced by a mean of 89% in all children 1 year after the successful completion of the training. In three children, followed for 2 years, this result stabilized at that proportion. One of these children, followed for > 3 years, gradually reverted to the original pattern of daily wetting and another patient, with a follow-up of > 4 years, maintained an 86% reduction of wetting incidents. CONCLUSION: Until now, there was no specific treatment for enuresis risoria and not all patients outgrow the problem; this experimental conditioning programme shows promise in diminishing wetting incidents. However, the programme needs further testing in a prospective study.
The value of radiography, ultrasound and magnetic resonance imaging (MRI) in detecting skeletal and intraspinal pathology was assessed in infants with anorectal malformations, and the need for spinal MRI examination in this group of patients was evaluated. Twenty-one infants were examined with radiography, ultrasound and MRI of the lower spine. The detection of skeletal and intraspinal abnormalities was compared for the three imaging modalities. Fifteen patients were normal in all three examinations. Radiography showed bony skeletal abnormalities in six children, although sometimes very subtle. By ultrasound both bony and cartilaginous malformations were detected in all six patients and by MRI in five patients. Five of the six children with skeletal abnormalities had intraspinal pathology, detected in all cases by ultrasound and MRI. However, the abnormalities were more clearly demonstrated by MRI than by ultrasound. Spinal radiographs must be examined carefully for abnormalities, because they can indicate the presence or absence of intraspinal pathology. Normal radiographic and sonographic appearance of spinal anatomy in children with anorectal malformation makes MRI superfluous, but if radiographs or ultrasound are abnormal, MRI should be used to accurately depict possible intraspinal pathology.
We evaluated 8 girls and 3 boys with benign sacrococcygeal teratoma for lower urinary tract dysfunction. All children underwent ultrasonography of the urinary tract, voiding cystourethrography and at least 2 standard urodynamic evaluations. Mean patient age at first urodynamic study was 59 months (range 1 to 136). Nine of the 11 children had abnormal studies, including detrusor instability and associated pelvic floor overactivity during micturition in 2, an anatomical infravesical obstruction in 2 and neurogenic bladder-sphincter dysfunction in 5 (2 detrusor hyperreflexia with sphincter dyssynergia, 2 hyporeflexic bladder-sphincter function and 1 detrusor hyporeflexia with normal sphincter function). Although the major cause of neurogenic lower urinary tract dysfunction seems to be related to surgical trauma of the pelvic plexus and sacral nerves, we observed tethered spinal cord as a primary cause in 2 patients. The incidence of voiding dysfunction in children with sacrococcygeal teratoma is high and is usually neurogenic in origin.