Biomedical subjects
Publications and source records attributed to R C Rink.
Dramatic improvements have occurred in the treatment and prognosis of the child with rhabdomyosarcoma over the past 2 decades. Increased understanding of tumor behavior has improved survival and focused attention on important quality of life issues. Future therapeutic advances will depend largely on an improved molecular understanding of altered cell behavior and the continued efforts of multi-institutional studies.
PURPOSE: Achieving continence and preserving renal function are goals in the care of patients with bladder exstrophy. The Young-Dees-Leadbetter bladder neck reconstruction should ideally provide continence and normal voiding dynamics without the need for intermittent catheterization. We review our experience with bladder neck reconstruction in this population with emphasis on voiding dynamics among those patients doing well. MATERIALS AND METHODS: We retrospectively analyzed all patients with the exstrophy-epispadias complex seen at our institutions since 1985. We reviewed staged reconstruction in 53 patients, including 31 with classic bladder exstrophy, 4 with exstrophy variants and 18 with incontinent epispadias. Patients with additional neurogenic dysfunction were excluded from study. Subjective and objective data regarding voiding function and complications were collected. RESULTS: Complete reconstruction for continence was performed in 38 cases, of which 11 that required bladder augmentation with bladder neck reconstruction or who had a different primary continence procedure were excluded from study. The remaining 27 patients treated with the Young-Dees-Leadbetter bladder neck reconstruction had 2 or more years of followup (mean 5.9). Dry intervals of at least 2 hours were achieved by 18 patients and all were considered by parents to void well. Despite near or total subjective continence and "good" voiding, 13 of these 18 patients (72%) have clinical problems related to emptying, which include recurrent urinary tract infections in 10, epididymitis in 2 and bladder calculi in 4. Objective urodynamic parameters confirm poor voiding in most patients. CONCLUSIONS: Bladder neck reconstruction in patients with exstrophy can achieve continence without intermittent catheterization. In our experience patients who achieve these goals have an alarming frequency of clinical and urodynamic problems related to emptying. One must question the normalcy of the voiding pattern and price to achieve continence among patients with exstrophy.
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PURPOSE: Rather than resecting the distal spongiosum lateral to the open urethra for hypospadias, we mobilized this tissue with the urethral plate away from the corpora cavernosa, subsequently wrapping it around various types of urethroplasty to prevent fistula formation. MATERIALS AND METHODS: The distal spongiosum was preserved and used for coverage in 25 hypospadias repairs in 1 year. We initially applied it in this manner when the distal spongiosum persisted as a pillar of healthy erectile tissue but later when the distal tissue appeared more fibrous in nature. The wrap was used to cover various types of urethroplasty, including advancement in 6 cases, tubularization in 10, flip-flap repair in 6 and an island onlay pedicle graft in 3. RESULTS: All patients have at least 1 year of followup. There has been no fistula formation or residual chordee. In 1 patient minor meatal retraction did not require a secondary procedure. Cosmetic results have been good. CONCLUSIONS: A distal wrap of corpus spongiosum may be used to avoid fistula formation without causing residual or recurrent curvature. It re-creates a nearly normal urethra in some cases.
PURPOSE: We report the results of surgical correction of severe congenital dorsal penile curvature associated with a long slender phallus. MATERIALS AND METHODS: In the last 7 years we treated 16 boys with a mean age of 2 years 10 months who had severe dorsal penile curvature. The series included 5 patients with megameatus variant hypospadias and a full foreskin, 3 who presented with hypospadias and 8 who were referred when dorsal penile curvature was noticed by the family or primary physician. Mean penile length was 6.5 cm. without stretching. In each case length was greater than 2 standard deviations above the mean for patient age. Penile circumference decreased from a mean of 4.2 cm. at the base of the shaft to 4.1 cm. at the mid shaft and 3.7 cm. at the corona. Penile circumference was generally normal for patient age until corrected for length, when it was revealed to be small. Surgical correction of severe curvature was performed in 14 patients, while 2 who presented as newborns had remarkable spontaneous improvement in the first year of life. In each case curvature was due to corporeal disproportion. RESULTS: All patients have erections postoperatively. There has been no residual or recurrent curvature and cosmetic results are good. CONCLUSIONS: Congenital dorsal penile curvature is a potential problem of the long phallus. In severe cases surgical repair improves the appearance of the penis but it must also address the problem of corporeal disproportion.
PURPOSE: Inadequate bladder emptying is a common urinary dysfunction in children. The role of alpha-blockers for managing bladder outlet obstruction remains relatively unexplored in children. Because of the well established impact of alpha-blocker therapy in men, we investigated its use for treating inadequate bladder emptying in the pediatric population. MATERIALS AND METHODS: We treated 17 children 3 to 15 years old with documented poor bladder emptying of various etiologies, including dysfunctional voiding, the Hinman syndrome, the lazy bladder syndrome, posterior urethral valves, myelomeningocele and the prune-belly syndrome, using the alpha-1 adrenergic receptor antagonist, doxazosin. The initial dose of 0.5 to 1.0 mg. nightly was increased according to patient response and as tolerated. Patients were followed weekly to monthly by symptomatic history, and urine flow and/or post-void residual urine volume measurement. Two patients with neurogenic bladder were also followed with cystometrography and leak point pressure determination. RESULTS: Bladder symptomatology and/or emptying improved in 14 patients (82%). Ten patients had decreased post-void residual urine during treatment and in 3 uroflowmetry showed increased maximum flow. Two patients with neuropathic bladder secondary to myelomeningocele had decreased leak point pressure on alpha-blocker therapy and in 2 with a history of posterior urethral valves new onset bilateral hydronephrosis completely resolved. Only 1 patient had mild postural hypotension, which resolved with dose reduction. CONCLUSIONS: Selective alpha-blocker therapy seems to be well tolerated in children and appears effective for improving bladder emptying in various pediatric voiding disorders at short-term followup. Long-term followup and further investigation are warranted to validate the potential role of alpha-blocker therapy in pediatric urinary dysfunction.
PURPOSE: We investigated the long-term metabolic effects of gastrointestinal composite urinary reservoirs in patients with myelomeningocele or exstrophy. MATERIALS AND METHODS: Seven patients with myelomeningocele or exstrophy who required complex urinary reconstruction in the setting of metabolic acidosis or the short bowel syndrome underwent construction of a gastrointestinal composite reservoir, including a staged and a single procedure in 3 and 4, respectively. Preoperatively and postoperatively serum electrolytes were measured, and urinalysis and urine cultures were performed in all patients. In 5 patients serum pH was compared preoperatively and postoperatively, and in all serum gastrin was measured postoperatively. RESULTS: At an average followup of 62 months (range 52 to 87) serum chloride and bicarbonate significantly normalized (p <0.05) in all 7 patients with bladder exstrophy or myelomeningocele. Serum pH also significantly normalized (p <0.05) in 5 patients at long-term followup. Serum gastrin and creatinine were normal and urinary pH fluctuated insignificantly throughout followup. None of the patients had urolithiasis or symptoms of the hematuria-dysuria syndrome. Periodic symptomatic urinary tract infections developed but none required chronic antibiotic therapy. CONCLUSIONS: Gastrointestinal composite urinary reservoirs appear to be beneficial for patients with myelomeningocele or exstrophy who have preexisting metabolic acidosis or the short bowel syndrome. Serum electrolyte neutrality is achieved during long-term followup. No patient had the hematuria-dysuria syndrome or urolithiasis.
PURPOSE: Concealed penis is an uncommon condition due to poor skin fixation at the base of the penis, cicatricial scarring after penile surgery and excessive obesity. The condition varies in severity and several surgical options are available, such as excision of previous scarring, degloving the penile shaft, reconstructing the penile shaft skin with flaps, fixing the penile skin at the penopubic and penoscrotal angles, and removing excess suprapubic fat. MATERIALS AND METHODS: We reviewed the records of 43 patients treated for concealed penis from 1993 to 1998. We categorized the cases as type 1-congenital concealed penis, type 2-concealed penis due to scarring from previous surgery and type 3-complex cases involving excessive obesity. Cases were reviewed in regard to surgical techniques and outcomes. We identified 18 type 1, 18 type 2 and 7 type 3 cases. Mean age of type 1 patients at surgery was 12.4 months with 1 patient presented at age 7 years. None had previously undergone penile surgery. All patients underwent complete penile degloving. To reconstruct the penile shaft flaps or Z-plasties with penile skin were used in 12 patients and scrotal skin flaps were used in 2. In 12 patients the penile skin was fixed at the penoscrotal and penopubic angles to maintain penile length and in 2 excess fat was excised. Mean age of type 2 patients at surgery was 19.8 months. All had previously undergone surgery, including hypospadias in 1 and circumcision in 17. All patients underwent complete penile degloving and the cicatricial scar that trapped the penis was excised. Penile skin flaps and Z-plasties were used in 12 cases, scrotal skin flaps were used for reconstruction in 2 and skin grafting was done in 1. In 10 patients the penile skin was fixed with sutures to maintain penile length. Mean age of type 3 patients at surgery was 15.8 years. Of the 7 boys 6 had previously undergone penile surgery. All required extensive scar excision and complex reconstruction involving penile skin flaps in 3, scrotal flaps in 5 and penile skin fixation in 6. Excessive suprapubic fat was removed in 5 patients, of whom 3 underwent liposuction. RESULTS: Surgical results were uniformly good in type 1 patients except in 1 who was believed to have excessive suprapubic fat. Results were good in 14 of the 18 type 2 patients, although 2 retained excessive suprapubic fat and 2 had some unsightly scarring. No type 1 or 2 patient required additional surgery. Of the 7 type 3 patients 6 had a good result and required no additional surgery. One patient has recurrent concealed penis after 2 procedures and awaits additional surgery. CONCLUSIONS: Concealed penis has a varied etiology and requires a flexible surgical approach. The common surgical options in all cases include complete penile degloving, excising the scarring due to previous surgery, removing excess suprapubic fat, reconstructing the penile skin with local flaps, and fixing the penile skin at the penopubic and penoscrotal angles.
PURPOSE: We present our experience using the various Mitrofanoff techniques to create a continent catheterizable stoma as an adjunct to continent urinary tract reconstruction in children and young adults. MATERIALS AND METHODS: Between 1990 and 1998 a Mitrofanoff procedure was performed at our institution in 55 male and 45 female patients with a mean age of 10.5 years. The etiology of incontinence was diverse but more than 90% of the patients had neurogenic bladder, the epispadias-exstrophy complex or a cloacal anomaly. Surgery included appendicovesicostomy in 57 cases, a Yang-Monti ileovesicostomy in 21, continent vesicostomy in 21 and formation of a tapered ileal segment as a catheterizable channel in 1. Simultaneously bladder augmentation was performed in 52 patients, bladder neck reconstruction was done in 48 and a Malone antegrade colonic enema stoma was constructed for fecal incontinence in 17. RESULTS: The abdominal stoma is continent in 98 of our 100 patients. Mean followup is 2 years (range 2 months to 8 years) with the longer followup in the appendicovesicostomy group. One patient with stomal incontinence who underwent revision is now dry. Postoperative complications requiring an additional procedure developed in 20 patients, including stomal stenosis in 12. Continent vesicostomy was most prone to stomal problems (6 of 21 patients, 29%). CONCLUSIONS: The Mitrofanoff procedure is a reliable technique for creating a continent catheterizable urinary stoma. Appendicovesicostomy continues to be our first option for this procedure, although we have also had good results with the Yang-Monti ileovesicostomy and continent vesicostomy. These newer options have allowed preservation of the appendix for the Malone antegrade colonic enema stoma procedure in patients with urinary and fecal incontinence.
Urinary incontinence in pediatric and adolescent patients has been successfully managed with the artificial urinary sphincter for several decades. Placement of the sphincter can be difficult in the preadolescent girl due to poorly developed vaginal tissue that can result in difficulty establishing the surgical plane between the bladder and vagina. We report 2 patients in whom the sphincter was placed around the urethra and vagina, a complication that has been reported in only 1 patient previously. All 3 patients presented with hematometrocolpos and bloody vaginal discharge. All were successfully managed with replacement of the sphincter cuff around the urethra and delayed vaginoplasty.
OBJECTIVES: To review our results of patients who underwent repeat hypospadias surgery using local skin flaps with preservation of the urethral plate. METHODS: We retrospectively reviewed the medical records of all patients who underwent a reoperative hypospadias repair using the urethral plate between 1988 and 1996. A total of 53 patients were identified who developed either a large fistula (47 patients) or severe stricture (6 patients) after the initial repair. Seventeen patients underwent a repeat Mathieu flip-flap and 36 an onlay flap. The mean age at the time of surgery was 5.2 years (range 1 to 27). The mean duration of follow-up was 17 months (range 6 to 108). RESULTS: The initial failed hypospadias repair was corrected with a single procedure in 44 (83%) of the 53 patients. The 9 patients who had further complications required 17 additional procedures. A meatal-based flap had been used in 4 of these patients and an onlay flap in 5. Three of the 9 patients who had further complications presented with urethral strictures after the previous repair. CONCLUSIONS: Fistula and stricture are common complications of hypospadias surgery. The use of the urethral plate in primary repairs has helped decrease the complication rate. We report the effectiveness of using local skin flaps and preserving the urethral plate in complex reoperative hypospadias surgery. These techniques were successful in 83% of these challenging patients. Patients with stricture after hypospadias surgery are at increased risk of further complications.
Remarkable progress has been made in lower urinary tract reconstruction in children. Nowhere is this more valuable than in augmentation cystoplasty, where there are several available options. This article discusses the current techniques and the benefits and limitations of these procedures. A section on tissue regeneration techniques and the author's current recommendations are included.
PURPOSE: We review our experience with the Young-Dees-Leadbetter bladder neck repair among patients with neurogenic incontinence. MATERIALS AND METHODS: Between 1978 and 1997, 25 girls and 13 boys with a mean age of 10.5 years (range 5 to 25) underwent a standard Young-Dees-Leadbetter bladder neck repair for neurogenic incontinence. Of the 38 patients 26 had undergone prior urological surgery, including bladder neck surgery in 6 and bladder augmentation in 4. A primary Young-Dees-Leadbetter bladder neck repair was performed in 24 patients, a secondary Young-Dees-Leadbetter procedure in 6 and a primary Young-Dees-Leadbetter procedure with periurethral silicone sheath placement in 8. RESULTS: Of the 38 patients 30 (79%) are dry, 7 (18%) are partially dry and 1 remains incontinent. Total or partial dryness was achieved after the initial repair in 26 cases (68%), while 8 required an additional procedure and 3 required more than 2 procedures to achieve continence. All patients who underwent silicone sheath placement were initially dry but incontinence developed subsequently in 5 due to sheath erosion. Of the 38 patients 35 (92%) ultimately required bladder augmentation. CONCLUSIONS: The management of neurogenic incontinence remains difficult. Success with the Young-Dees-Leadbetter procedure in our experience nearly always requires augmentation cystoplasty. The majority of patients will achieve continence after the initial procedure, and persistent incontinence can frequently be cured with further bladder neck surgery.
PURPOSE: We identified the incidence and types of genital and urinary anomalies, and established a plan for evaluating the urinary system in the CHARGE association. MATERIALS AND METHODS: We retrospectively reviewed the charts of 32 patients in whom the CHARGE association was diagnosed. RESULTS: Of the 32 patients identified 22 (69%) had genitourinary abnormalities. Genital anomalies, including micropenis, penile agenesis, hypospadias, chordee, cryptorchidism, a bifid scrotum, atresia of the uterus, cervix and vagina, and hypoplastic labia majora, labia minora and clitoris, were present in 18 patients (56%). Of the 24 patients who underwent renal ultrasound 10 (42%) were diagnosed with urinary tract anomalies including a solitary kidney, hydronephrosis, renal hypoplasia and duplex kidneys. Further evaluation revealed vesicoureteral reflux, neurogenic bladder secondary to spinal dysraphism, nephrolithiasis, ureteropelvic junction obstruction and a nonfunctioning upper pole in both duplex kidneys. CONCLUSIONS: There is a high incidence of genitourinary anomalies in the CHARGE association. Because of this high incidence of anomalies, patients with this condition should undergo a careful genitourinary evaluation, including renal and bladder ultrasound, and voiding cystourethrography screening.
We present a case of extrinsic compression of a ureter by spinal fixation hardware and adjacent fibrosis that resulted in progressive obstructive hydronephrosis, loss of renal function, and, ultimately, nephrectomy.
Surgical management of intersex states continues to evolve such that today's efforts achieve near normal cosmetic and functional results. In this article we discuss the reconstruction of the female genitalia in those that have had significant androgen stimulation as well as those with near normal external genitalia but significant urogenital sinus anomalies. The initial evaluation as well as postoperative follow-up management is addressed.
PURPOSE: The conventional technique for ureterocystoplasty includes complete mobilization and incision of the ureter. We describe a modified procedure in which the distal 3 cm. of ureter are left in place and intact. MATERIALS AND METHODS: This modification has been used in our last 13 cases of ureterocystoplasty. The first 7 patients with followup of more than a year (mean 28 months) are included in this series, and 6 have undergone video urodynamic evaluation before and after reconstruction. RESULTS: Clinical results have been good. Four patients who have been toilet trained are continent. There have been no problems from stagnant urine in the intact ureter with only 1 case of pyelonephritis and no bladder calculi. Mean bladder capacity on cystometrogram has increased from 103 to 236 ml. after reconstruction and reached 137% of expected capacity for age and size (range 110 to 155%). No uninhibited contractions or problems with compliance have been noted. CONCLUSIONS: The distal ureter may be left intact for ureterocystoplasty to protect ureteral blood supply. This modified technique is sound from a physiological standpoint, technically easier and associated with good results.
PURPOSE: Bowel used for bladder reconstruction regardless of detubularization occasionally retains its contractile properties. Of 323 patients who underwent primary enterocystoplasty we identified 19 who continue to have high pressure bladder contractions and required augmentation of the previously augmented bladder. MATERIALS AND METHODS: Reason for repeat augmentation, upper tract changes, original and secondary bowel segments used, and urodynamic findings were evaluated in all patients. Current status and followup also were noted. RESULTS: After initial augmentation 8 patients had persistent incontinence, 5 bladder perforation, 3 isolated upper tract changes, 2 incontinence and bladder perforation, and 1 incontinence plus intractable pain. Preoperative urodynamics revealed detrusor pressures from 30 to 100 cm. water. All patients had adequate bladder outlet resistance. The original bowel segments used were sigmoid in 12 cases, stomach in 4, ileum in 2 and cecum in 1. Bowel segments for re-augmentation were ileum in 16 cases and sigmoid in 3. Of the 11 patients with incontinence 10 are now dry. All cases of upper tract changes resolved. Mean followup since re-augmentation is 52 months. CONCLUSIONS: If the outcome of bladder augmentation is less than optimal, it is important to reevaluate the bladder dynamics. In rare instances these patients may continue to have high pressure contractions with a functionally small bladder capacity. In such situations reaugmentation with an additional bowel segment is an excellent alternative to a difficult clinical problem and provides good results in the vast majority of cases. This treatment may not totally alleviate the contractions but it does decrease them and increase the volumes at which the contractions occur, making them no longer clinically or functionally significant.