[Present and future of pediatric urology].
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Biomedical subjects
Publications and source records attributed to P D Mouriquand.
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BACKGROUND/PURPOSE: The management of children who have congenital intestinal dysfunction and continuous feces soiling has been improved significantly by the Malone continent caecostomy. When the appendix is not available, the Monti tube represents a good alternative to create a catheterizable conduit. The authors report here 7 cases of left continent colonic access (left Monti-Malone). METHODS: From July 1999 to January 2001 7 patients have been operated on in our unit: 6 spina bifida, 1 cloacal exstrophy. Technically, a ring of descending colon of 1.5 to 2 cm width is isolated with its meso, the Monti's tube fashioned, and implanted into the left colon according to the Malone's technique. Antegrade bowel washouts start 3 weeks after surgery with standard saline (200 to 500 mL) +/- phosphate enemas. A period of 1 to 3 months is needed to adjust to the right enema regimen to get a satisfactory result. RESULTS: The follow-up range is 18 months to 1.5 months. One patient is excluded. The duration of the enemas varies between 10 and 30 minutes, and the number of enemas varies from 1 to every 2 or 3 days. Five children are clean and perform their antegrade enemas after dinner to avoid the embarrassment of post enema leakages. CONCLUSION: Although it is a short series with a short follow-up, the first results collected show a significant shortening of the duration of the enema with excellent outcomes in terms of continence.
Upper tract dilatation presenting as renal pelvis dilatation with or without ureteric dilatation is one of the more common abnormalities detected on prenatal ultrasound scanning. The majority of cases are due to pelvi-ureteric and vesico-ureteric junction anomalies, and vesico-ureteric reflux. Prenatal diagnosis with ultrasound may be aided by biochemical analysis of fetal blood and urine. The natural history of prenatally detected abnormalities remains under review. Precise diagnosis, and therefore prognosis, may not be made until after birth.
The development of antenatal ultrasonography and the detection of fetal uropathies has created a new field for paediatric urologists. The embryology and physiology of the fetal renal tract is still poorly understood. It is, however, recognised that delays in the maturation of the renal system can lead to transient dilatation of the urinary tract. Structural and functional anomalies can also result in dilatation, but these are permanent. The ability to distinguish between the transient and permanent impairments to urine flow represents a diagnostic challenge. This review article discusses the pathophysiology of urine flow impairment, antenatal detection and the postnatal management of the common congenital uropathies.
OBJECTIVE: To assess the outcome of the Pippi Salle (Kropp onlay) urethral lengthening procedure in the treatment of neuropathic urinary incontinence. PATIENTS AND METHODS: Twenty-eight patients (12 males and 16 females, mean age at surgery 12.6 years, range 7-32) were identified who underwent the procedure between 1993 and 1997 in the United Kingdom. Outcomes were assessed by a review of the case notes. RESULTS: Of the 28 patients, 18 (64%) were rendered continent by day, and 17 (61%) by day and night. Twelve of the 16 females were completely dry, and a further girl was dry by day, giving overall daytime continence in 13 patients; five males were rendered continent (P=0.03, chi-square test). Four patients required revision surgery; in five patients (two females and three males) the method was abandoned and they underwent an alternative procedure, and a further four are being reassessed. CONCLUSION: The Pippi Salle procedure should be considered as a first-line treatment option for neuropathic incontinence in females. Its place in the management of incontinent males is less convincing.
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Renal malformations account for most children with chronic renal failure and are often associated with urinary tract anatomical obstruction. We examined cellular and molecular events after experimental urinary flow impairment in fetal sheep. Ovine gestation lasts 144 to 150 days with the metanephros appearing at 27 to 30 days. We generated complete unilateral ureteric anatomical obstruction at 90 days when a few layers of glomeruli had formed. After 10 days, we recorded ureteric and pelvic dilatation with renal parenchymal weight greater than contralateral organs or those from unoperated fetuses. The nephrogenic cortex was replaced by disorganized cells separated by edema and prominent vascular spaces. Cortical histology was dominated by cysts associated with malformed glomerular tufts. Cystic epithelia expressed PAX2, a growth-stimulating transcription factor down-regulated during normal maturation, and proliferating cell nuclear antigen, a surrogate marker of cycling cells. Detection of apoptosis using propidium iodide and in situ end labeling showed a significant increase of the point prevalence of death in the obstructed cortex. Hence, PAX2 and proliferating cell nuclear antigen expression as well as death were deregulated, as we previously reported in human kidney malformations. Medullary collecting ducts and loops of Henle were also disrupted, correlating with impaired urinary dilution and sodium reabsorption. Therefore, complex aberrations of morphogenesis, gene expression, cell turnover, and urine composition occur relatively early after experimental impairment of fetal urinary flow.
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Posterior urethral valves (PUV) is the most common congenital urine flow impairment in boys. Long-term prognosis involves: renal function impaired in 30 to 50% of PUV and leading to hyperdiuresis, low GFR and acidosis; bladder urodynamics impaired in 75% of PUV with abnormal urine storage, abnormal micturition and vesicoureteric reflux. Incontinence and recurrent urinary tract infections commonly reflect bladder and renal failures; abnormal bladder outlet leads to incontinence and abnormal ejaculation. The roles of antenatal treatments (vesico-amniotic shunts), neonatal treatments (resuscitation and endoscopic treatment of PUV) and long-term treatments (urinary diversions, bladder augmentation, alpha blockers, anticholinergic, dialysis and renal transplant) in the long-term outcomes of PUV are reviewed.
OBJECTIVES: To assess the use of a multipurpose stent (the 'Blue stent', Angiomed Urosoft Pyeloplasty Stent, Bard, UK) in children undergoing pyeloplasty and ureteric reimplantation. PATIENTS AND METHODS: Between August 1994 and August 1996, the Blue stent was used in 50 renal units in 46 children aged 2 months to 12 years and 6 months. Twenty-five children underwent pyeloplasty, 11 had ureteric reimplantation for vesico-ureteric reflux (VUR), eight had ureteric reimplantation with remodelling for obstructed megaureters and in two patients it was used during the removal of stones. The mean follow-up was 18 months (range 6-30 months). RESULTS: After pyeloplasty, 22 patients (88%) had improved renal function and drainage with a decrease in hydronephrosis; two patients had a decrease in hydronephrosis only, one had an anastomotic leak and needed a repeat pyeloplasty and four developed a urinary tract infection (UTI). After ureteric reimplantation, VUR was not detected in any patient. Two patients had no change in drainage after remodelling and reimplantation of a megaureter, one was later diagnosed as having a neuropathic bladder and one child developed a UTI after ureteric reimplantation. The hospital stay was 3 days after pyeloplasty and 5 days after reimplantation. CONCLUSION: The design of the multipurpose Blue stent provides versatility; it can be used as a stent, and both an internal and external drain. Its use does not prolong the hospital stay. Insertion causes minimal trauma to the renal parenchyma, and removal is easy, pain-free and requires no anaesthesia. The complication rates in the present series compare favourably with other reported series.
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.
A 12-year-old girl with bladder hemangioma presenting with massive hematuria is described. The patient was previously operated for removal of cystic hygroma from the chest wall. The association of those two pathological entities suggests possible common etiology and pathogenesis and the various therapeutic options are reviewed.
PURPOSE: Since 1980 numerous variations of the Mitrofanoff principle have been described. We report on 22 children in whom a ureteral Mitrofanoff channel was created. MATERIALS AND METHODS: Between 1986 and 1995 a ureteral Mitrofanoff channel was constructed as a catheterizable conduit in 22 children 2 to 15 years old (average age 6.5) with various abnormalities of the lower urinary tract, mainly exstrophy and neurogenic bladder. Indications included unavailability or unsuitability of the appendix, preference for appendix as a catheterizable colonic stoma for antegrade washouts or concomitant removal of a nonfunctioning kidney, leaving the ureter available for use. Surgical technique was based on the principles of appendicovesicostomy and in 9 cases the ureteral Mitrofanoff channel was reimplanted. RESULTS: Followup ranged from 1 to 72 months (average 30.5). Complications included stenosis of the conduit that caused difficult catheterization in 3 patients, necessitating dilation or minor revision in 2 and complete replacement by appendix in 1. Urinary leakage from the Mitrofanoff channel in 5 patients was treated with polydimethylsiloxane injection or oxybutinin. In 1 patient the channel was reimplanted, since the catheter struck the bladder neck during catheterization and caused severe pain. CONCLUSIONS: Results of the ureteral Mitrofanoff channel seem somewhat less satisfactory than those of appendicovesicostomy but they remain acceptable and even comparable, strongly supporting its use in certain circumstances.
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.
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