Current trends in the management of Wilms' tumour.
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Humans↗
Biomedical subjects
Patrick G Duffy
Publications and source records attributed to Patrick G Duffy.
Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy-epispadias complex: long-term results.
OBJECTIVE: To present our experience with the use of injectable polydimethylsiloxane (Macroplastique, Uroplasty, Minneapolis, MI, USA) for treating incontinence in children with the exstrophy-epispadias complex (EEC), as incontinence continues to be a challenging problem in such children, and although the primary management of EEC has developed over the last few decades, with early closure and reconstruction of the penis, achieving satisfactory continence status remains elusive. PATIENTS AND METHODS: We retrospectively reviewed the hospital records of 52 patients (41 boys and 11 girls, mean age at first injection 6.6 years, range 3.6-16.7) with EEC who had injections with Macroplastique between January 1991 and February 2004; 34 had bladder exstrophy and 18 primary epispadias. For this study we defined success as complete dryness with no use of pads or nappies. Improvement was defined as being occasionally wet but with dry intervals lasting >or= 4 h. RESULTS: The mean (range) follow-up was 4.6 (0.5-9) years. Twenty patients had one injection, 10 had two, 13 had three, six had four, two had six and one had seven injections. In most patients a maximum of three injections predicted the outcome. The injection of Macroplastique was successful in nine patients (17%; with an annual follow-up, two at 1-2 years, three at 2-5 years and four at >5 years), whilst 17 (33%) improved significantly (one at <1 year, two at 1-2 years, eight at 2-5 years and six at >5 years). Those patients comprised five of 18 (27%) with epispadias and four of 34 (12%) with exstrophy. A history of previous surgery and gender had no significant effect on the outcome. Overall half the patients benefited from the procedure. CONCLUSIONS: This series confirms that injection with Macroplastique is minimally invasive, durable in significantly many patients and has a reasonable success rate. A history of previous surgery and gender had no significant effect on the outcome. Patients with epispadias are more likely to benefit from an injection with Macroplastique than those with bladder exstrophy. A maximum of three injections is predictive with reasonable certainty of any benefit from the procedure.
Adolescent↗
Laparoscopy for impalpable testes.
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Cryptorchidism↗
Renal function and outcome following salvage surgery for bilateral Wilms' tumor.
PURPOSE: Maintaining adequate renal function without compromising cure in children with bilateral Wilms' tumor is surgically demanding and challenging. The aim of this study was to assess renal function and outcome in children treated in one institution for bilateral Wilms' disease by renal salvage procedures. METHODS: This study is a retrospective review of all patients with histologically proven bilateral nephroblastoma who underwent a renal salvage procedure between November 1973 and June 2002. The median follow-up time was 52 months (range, 8 to 326 months). RESULTS: Twenty-three patients were included in the study. The median age at diagnosis was 19 months (range, 5 to 65 months). Patients who presented before 1982 (n = 5) were treated surgically first followed by chemotherapy. The remainder (n = 18) received initial chemotherapy before the operation. Of the 46 kidneys, 18 had a nephrectomy. The remaining 28 underwent a renal salvage procedure. At follow-up, 19 patients had good renal function, 2 had satisfactory function, and 2 had renal failure. Seventeen of the 23 children are alive and tumor free at follow-up (74%). Four children died of distant metastases and 2 of renal failure. Local recurrence in the salvaged kidney was detected in 1 patient 12 years after surgery after the commencement of immunosuppression for renal transplantation. CONCLUSIONS: These results confirm that renal salvage procedures, in combination with chemotherapy, are a safe and effective way of treating children with bilateral Wilms' disease. In addition, renal salvage surgery can maintain satisfactory renal function in the majority of these patients without an increased risk of local recurrence. When transplantation is required, the remaining native kidney should be removed to prevent tumor recurrence.
Child, Preschool↗
A novel, inexpensive, double lumen suprapubic catheter for urodynamics.
PURPOSE: We describe a novel, double lumen, intravesical, suprapubic catheter designed to meet the requirements of pediatric urodynamics that is easy to use and has minimal complications. MATERIALS AND METHODS: A commercially available 10Fr pediatric suprapubic pigtail catheter forms the outer lumen for instilling filling media. A 16 gauge epidural catheter is inserted through the outer catheter providing an inner lumen for measuring intravesical pressures. The resultant double-lumen catheter is inserted suprapubically using a peel away needle supplied with the 10Fr catheter, with the patient under general anesthetic. RESULTS: The catheter has been used for 15 years in more than 700 patients with good reliability and few complications. The concentric construction of the double lumens and the rigidity of the inner intravesical pressure channel ensure there is no transmission of pressure from the filling channel to the inner lumen. The catheter has a circular cross section and a pigtail distal end which help to retain it within the bladder. There is low resistance to filling that allows adequate filling rates to be achieved by gravity rather than necessitating a pump. The catheter is easily made from readily available components and is less expensive than other double-lumen catheters suitable for suprapubic use. CONCLUSIONS: A reliable, double lumen catheter that fulfills criteria not found in commercially available alternatives can be inexpensively made for urodynamics.
Catheterization↗
Tethering of the spinal cord in mouse fetuses and neonates with spina bifida.
OBJECT: Tethering of the spinal cord is a well-known complication in humans with spina bifida aperta or occulta. Its pathogenesis consists of a pathological fixation of the spinal cord resulting in traction on the neural tissue which, in turn, leads to ischemia and progressive neurological deterioration. Although well established in humans, this phenomenon has not been described in animal models of spina bifida. METHODS: A fetal mouse model with naturally occurring, genetically determined spina bifida was produced by generating double mutants between the curly tail and loop-tail mutant strains. Microdissection, labeling with 1,1'-dioctadecyl-3,3,3,',3'-tetramethylindocarbocyanine perchlorate, immunohistochemistry for neurofilaments, H & E staining of histological sections, and whole-mount skeletal preparations were performed and comparisons made among mutant and normal fetuses. Normal fetuses exhibited the onset of progressive physiological ascent of the spinal cord from embryonic Day 15.5. Spinal cord ascent resulted, by embryonic Day 18.5, in spinal nerve roots that pass caudolaterally from the spinal cord toward the periphery. In contrast, fetuses with spina bifida exhibited spinal cord tethering that resulted, at embryonic Day 18.5, in nerve roots that run in a craniolateral direction from the spinal cord. The region of closed spinal cord immediately cranial to the spina bifida lesion exhibited marked narrowing, late in gestation, suggesting that a potentially damaging stretch force is applied to the spinal cord by the tethered spina bifida lesion. CONCLUSIONS: This mouse model provides an opportunity to study the onset and early sequelae of spinal cord tethering in spina bifida.
Animals↗