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Biomedical subjects

R D Jeffs

Publications and source records attributed to R D Jeffs.

At least 37 records · Page 2Linked to original sources

Penile length in adulthood after exstrophy reconstruction.

PURPOSE: We attempted to determine whether the penis in adulthood after exstrophy reconstruction is short because of a congenital defect in the size of the corpora cavernosa. MATERIALS AND METHODS: Pelvic magnetic resonance imaging was performed on 10 men who underwent exstrophy reconstruction in childhood, and 10 age and race matched controls. Measurements of penile and pelvic anatomy were compared. RESULTS: The corpora cavernosa in men after exstrophy reconstruction were shorter than normal. Dividing total corporeal length into an anterior and posterior segment revealed that the anterior segment was short but the posterior segment attached to the pubic ramus was normal. However, the diameter of the posterior corporeal segment was greater than in controls. Although diastasis of the symphysis pubis increased the intersymphyseal and intercorporeal distances, the angle between the corpora cavernosa was unchanged, presumably because the corporeal bodies were separated in a parallel fashion. CONCLUSIONS: After exstrophy reconstruction the penis is short in adulthood, at least partially due to a congenital deficiency of corporeal tissue. Since diastasis of the pubic symphysis and chordee decrease penile visibility, approximation of the pubic symphysis and procedures to straighten the penis may improve cosmesis. However, because the corpora cavernosa are short, after exstrophy reconstruction the penis will always be shorter than normal in adulthood.

Anthropometry↗

Evolution of outcomes with the ileal hydraulic valve continent diversion: reevaluation of the Benchekroun catheterizable stoma.

Initial reports with limited follow-up suggested that the ileal hydraulic valve was a satisfactory approach to continent urinary diversion that was associated with acceptable early complication rates and uniform efficacy in achieving continence. We sought to evaluate the later outcomes of patients with this form of continent urinary diversion. An average extended follow-up of 5 years was available on 11 patients who had undergone ileal hydraulic valve diversion. Although only 13% of patients had complications requiring surgical revision at 18 months of follow-up, at the 5-year follow-up, 91% had developed complications requiring surgical revision. Of these, 64% entailed major revision of the urinary diversion. The most common late complication was stomal stenosis (73%); calculus formation (36%) and devagination (36%) were also common. Although in some cases, stomal stenosis was successfully managed by superficial revision with skin flaps and grafts, most cases required complete replacement of the efferent limb. Use of the devaginated or stenosed ileal hydraulic valve to construct a flap-valve efferent limb based on the Mitrofanoff principal succeeded in salvaging the continent urinary diversions in these patients. Novel forms of urinary diversion require extended follow-up for accurate assessment of their feasibility and efficacy. Attentive surveillance with surgical revision as necessary in such cases is mandatory for the preservation of continence and renal function.

Adolescent↗

Ureteral bladder augmentation using the lower pole ureter of a duplicated system.

Megaureters of nonfunctioning renal segments in a duplex system are commonly available for use in reconstructive procedures secondary to high-grade reflux, ectopic ureter, ectopic ureterocele, or obstructive megaureter. The use of megaureters subtending a nonfunctioning lower pole renal segment for bladder augmentation in 2 patients is reported.

Adolescent↗

The multiple reoperative bladder exstrophy closure: what affects the potential of the bladder?

OBJECTIVES: To define the possible cause of failure and the eventual potential of the bladder in 23 exstrophy patients, who underwent more than two failed prior attempts at closure. METHODS: Twenty-three patients were selected from the exstrophy data base who had two or more prior closures. Eighteen patients had undergone 2 previous closures and 5 patients 3 previous closures for either complete dehiscence or significant prolapse. At the time of initial closure, 19 patients did not have an osteotomy. At secondary closure, 10 underwent osteotomy while at third closure 5 had an osteotomy. At the time of reclosure at our institution all underwent an osteotomy. RESULTS: Reoperative repair at our institution was successful in all patients. Six patients achieved a bladder size suitable for bladder neck reconstruction and of them 3 are dry. The bladder size was inadequate in 9 patients and 8 are being monitored for possible bladder growth. CONCLUSIONS: Tension-free closure with osteotomy and immobilization are important factors both in an initial or any subsequent closure. The chance of obtaining an adequate bladder capacity for bladder neck plasty and eventual continence, following multiple reclosures, is markedly diminished.

Bladder Exstrophy↗

Reappraisal of the role of human chorionic gonadotropin in the diagnosis and treatment of the nonpalpable testis: a 10-year experience.

PURPOSE: We retrospectively evaluated the ability of human chorionic gonadotropin (HCG) to make the nonpalpable cryptorchid testis become palpable and promote testicular descent. MATERIALS AND METHODS: Through surgical bookings we identified 94 patients younger than 11 years who received HCG between 1984 and 1994 for the diagnosis or treatment of a nonpalpable undescended testis. The dose of HCG was 1,500 IU/m.2 intramuscularly 2 times weekly for 4 weeks. Testis location was determined by physical examination before and after hormone administration, and confirmed at surgical exploration. RESULTS: Of the 99 nonpalpable testes identified in 94 patients 39 (39%) became palpable following HCG administration and only 2 (2%) completely descended. A total of 60 testes remained nonpalpable with the most common reason being an absent or severely atrophic testis (40, 67%). Of the testes remaining nonpalpable after hormonal stimulation 73% were surgically located at or distal to the internal ring. CONCLUSIONS: HCG is preoperatively efficacious in causing the nonpalpable undescended testis to become palpable. For patients failing to respond to hormonal stimulation we recommend preliminary inguinal exploration, since most testes or testicular remnants are located within the inguinal canal or immediately below the internal ring.

Adolescent↗

Bladder exstrophy from childhood into adult life.

Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging from 1.5-5 to 1(1-4). It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders.

Adolescent↗

A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy.

PURPOSE: We describe a new combined horizontal and vertical pelvic osteotomy procedure for bladder exstrophy. MATERIALS AND METHODS: A total of 36 patients with the bladder exstrophy complex underwent this procedure during a 3-year period (8 primary and 18 secondary bladder closures, and 6 at bladder neck reconstruction). RESULTS: There were no instances of dehiscence and only a minor bladder prolapse in 1 patient with cloacal exstrophy. Two patients had a transient femoral nerve palsy and there was 1 superficial pin infection. Urological complications included symptomatic urinary tract infections in 5 patients, acute epididymitis in 1 and bladder calculi in 2. CONCLUSIONS: This new osteotomy procedure is of great benefit in initial or repeat closure of bladder exstrophy and may help in eventually achieving continence.

Bladder Exstrophy↗

The outcome of patients with classic bladder exstrophy in adult life.

PURPOSE: We determined the outcome of 20 older adult bladder exstrophy patients regarding urinary continence, sexual function, fertility and psychosocial integration. MATERIALS AND METHODS: A total of 16 men and 4 women completed an anonymous questionnaire and the charts were reviewed for medical history. RESULTS: Of 9 patients who void spontaneously 6 are dry for 4 hours and 3 for 2 hours. Among the remaining 11 patients 5 are dry on clean intermittent catheterization, 3 who had undergone uterosigmoidostomy are dry for more than 4 hours and 3 had an incontinent stoma. Four men and 2 women are married, including 2 men and 1 women who had a total of 7 children. Ten of 16 men (63%) reported that they ejaculate a few cubic centimeters in volume, 3 ejaculate only a few drops and 3 have no ejaculation (2 of whom underwent cystectomy). Semen analyzed in 4 patients (3 with azoospermia and 1 with oligospermia) and average volume of ejaculate was 0.4 cc (range 0.2 to 1). All women reported regular and normal menstrual periods. A total of 15 patients experienced normal erections that were described as satisfactory by 8 and not satisfactory by 6 due to a small penis, with dorsal chordee in 1. Of the 16 men 12 (75%) experienced satisfactory orgasms while 10 had participated in sexual intercourse with complete partner satisfaction in 9. Half of the men and all women describe intimate relationships as serious and longterm. Of the 20 patients 15 (75%) achieved a high level of education. CONCLUSIONS: Bladder exstrophy patients generally achieve good results but the fertility of most men is in doubt.

Adolescent↗

Evaluation of smooth muscle and collagen subtypes in normal newborns and those with bladder exstrophy.

PURPOSE: Many patients who undergo bladder exstrophy closure as newborns, subsequent epispadias repair and later bladder neck reconstruction become completely continent yet complications can occur. After successful initial exstrophy closure and later epispadias repair some patients may fail to gain sufficient capacity for bladder neck reconstruction or satisfactory capacity and continence after bladder neck reconstruction. In an attempt to understand the pathogenesis of these failures we compared bladder biopsies from normal neonates and those with exstrophy. MATERIALS AND METHODS: Bladder biopsies obtained from the midline of the bladder wall just above the base of the trigone from 12 newborns with exstrophy were compared to bladder sections from 9 neonatal cadavers. All bladder specimens were stained with monoclonal antibodies against type I, III or IV collagen and a subset was further stained with Masson's trichrome to define the extracellular matrix. All specimens were then analyzed using a color digital image analysis system. RESULTS: At initial examination of the extracellular matrix there was an increase in the collagen-to-smooth muscle ratio from 0.38 in controls to 1.2 in newborns with exstrophy, comprising an increase in collagen and decrease in smooth muscle. The collagen component of the extracellular matrix was then further defined to quantitate the amount of each collagen type (I, III and IV) deposited. We then evaluated the ratio of collagen type-to-total collagen sampled. Compared to control bladders there was no statistical difference in the amount of type I or IV in the bladders of newborns with exstrophy at initial closure. However, there was a 3-fold increase in type III collagen (0.14 +/- 0.05 to 0.46 +/- 0.2%, p < 0.001) in the bladders of neonatal controls versus newborns with exstrophy. CONCLUSIONS: This alteration in collagen makeup may represent an earlier developmental stage of the exstrophy bladder at birth, which then remodels and changes after successful initial closure. Further studies are underway to examine the collagen composition of bladders at bladder neck reconstruction, failed closures and augmentation.

Biopsy↗

Criteria for the prenatal diagnosis of classic bladder exstrophy.

OBJECTIVE: To define ultrasonographic criteria for the prenatal diagnosis of classic bladder exstrophy. METHODS: Forty-three prenatal ultrasound scans were studied from 25 pregnancies in which live delivery of an infant with classic bladder exstrophy occurred. The diagnosis of bladder exstrophy could be made retrospectively in 29 prenatal studies from 17 pregnancies. The time of the fetal ultrasound varied from 14-36 weeks' gestation (mean 23). The diagnosis of bladder exstrophy was made before delivery in only three cases. RESULTS: Five factors associated with bladder exstrophy were identified: 1) The bladder was not visualized on ultrasound in 12 of 17 cases (71%); 2) a lower abdominal bulge representing the exstrophied bladder was seen in eight of 17 cases (47%); 3) a small penis with anteriorly displaced scrotum was identified in eight of 14 males (57%); 4) the umbilical insertion was low set in five of 17 cases (29%); and 5) abnormal widening of the iliac crests was seen in three of 17 cases (18%). CONCLUSION: The prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visualized or any of the aforementioned factors are noted.

Bladder Exstrophy↗

Ureteral graft in urological reconstruction: clinical experience and review of the literature.

We report 7 cases in which grafts of ureter were used as tube and patch segments in urethral reconstruction and formation of a continent catheterizable stoma. The ureteral grafts survived without long-term stricture in all cases. Meticulous preparation of the graft and recipient bed is required for success, as previously demonstrated experimentally. When available, ureter is an excellent source of tissue for urological reconstruction.

Adolescent↗

Complete male epispadias: genital reconstruction and achieving continence.

We report on 15 patients who were primarily treated for complete male epispadias at our institution since 1975. Repair of epispadias was performed using a modified Young urethroplasty in 13 patients and a Cantwell-Ransley urethroplasty in 2. In addition, 2 patients underwent a Cantwell-Ransley urethroplasty with chordee repair after a previous Young urethroplasty failed. Bladder capacity increased from a mean of 50 cc before repair to 92 cc after urethroplasty. A urethrocutaneous fistula developed in 6 cases, including 5 Young repairs and 1 Cantwell-Ransley. Three fistulas resolved spontaneously and there were no urethral strictures. Bladder neck reconstruction was performed in 11 patients. Time to initial continence ranged from 21 days to 6 months (mean 3 months) postoperatively. All patients attained daytime continence in a mean of 9 months (range 21 days to 24 months) after bladder neck reconstruction, including 9 of 11 (82%) who achieved total day and night continence. Mean followup was 7 years (range 1 to 10). Modern treatment of complete male epispadias allows for an excellent genital appearance and achievement of urinary continence.

Child, Preschool↗

Epispadias with phimosis: an unusual variant of the concealed penis.

An unusual variant of concealed penis is described. Two neonates with epispadias and complete phimosis were diagnosed at birth and underwent delayed repair. These 2 cases are the 5th and 6th such reports in the literature. This anomaly's possible embryogenesis is reviewed.

Abnormalities, Multiple↗

The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned.

OBJECTIVES: We evaluated our experience with the Cantwell-Ransley epispadias repair to determine the lessons that have been learned with the increased experience and follow-up. METHODS: A total of 75 boys (60 with bladder exstrophy and 15 with complete epispadias) underwent a Cantwell-Ransley epispadias repair at our institute in the last 6 years. Primary repair was performed in 58 boys (45 with exstrophy and 13 with epispadias), and secondary repair was performed after prior failed closure in 17 boys (12 at the secondary exstrophy closure, 3 with exstrophy, and 2 with complete epispadias). RESULTS: At a mean follow-up of 28 months, all patients had a horizontal or downward angled penis while standing. The incidence of urethrocutaneous fistulas in the immediate postoperative state was 21% and at 3 months was 15%. The incidence of urethrocutaneous fistulas was no more in those patients in whom paraexstrophy skin flaps were used at anterior closure than in those in whom the urethral plate was left intact. Two patients developed a urethral stricture at the proximal anastomotic area, and 4 patients had minor skin separation of the dorsal penile skin closure. Catheterization or cystoscopy, or both, has been performed in 60 patients and revealed an easily negotiable urethral channel in all. CONCLUSIONS: The Cantwell-Ransley epispadias repair offers a straighter urethra, better correction of chordee and cosmesis, and a lower fistula rate in the exstrophy or epispadias patient.

Adolescent↗

Submucosal bladder neck injections of glutaraldehyde cross-linked bovine collagen for the treatment of urinary incontinence in patients with the exstrophy/epispadias complex.

During the last 7 years 19 patients underwent 33 transurethral injections of glutaraldehyde cross-linked bovine collagen into the bladder neck for stress incontinence. Of the 15 patients 14 have classic bladder exstrophy, 3 have complete male epispadias and 2 have cloacal exstrophy. The procedure was performed after a Young-Dees-Leadbetter bladder neck reconstruction in 15 patients and before it in 4. Injections were repeated in 10 patients after a mean of 12 months. After a mean followup of 26 months (range 9 to 84) improvement of continence was noted in 10 of 19 patients (53%) of whom 4 have significant improvement. Of the 8 patients whose condition failed to improve after collagen injections 6 underwent additional successful surgery to achieve urinary continence. Of the 10 patients who underwent repeated collagen injections 9 (90%) had additional improvement. Although there were no complications related to the injected collagen itself, postoperative complications developed in 2 patients. Submucosal injection of collagen to the bladder neck is simple and safe, and has a reasonable success rate. Thus, it may be used to improve continence in patients with the exstrophy/epispadias complex who lack full control after appropriate reconstructive surgery.

Adolescent↗

Applications of osteotomy in the cloacal exstrophy patient.

During the last 18 years we treated 22 patients with cloacal exstrophy of whom 13 were referred for further treatment after initial treatment elsewhere. One patient underwent cystectomy with ileal conduit urinary diversion soon after birth and 9 of the remaining 21 underwent initial closure without osteotomy. Of these 9 patients significant complications developed in 8 (89%) after bladder closure, including dehiscence in 6 (1 underwent 2 unsuccessful closures), a vesicocutaneous fistula and postoperative ventral hernia in 1, and bladder prolapse in 1. In contrast, complications developed in only 2 of the 12 patients (17%) who underwent osteotomy at the time of initial closure, including bladder dehiscence in 1 and significant prolapse in 1. Patients who underwent osteotomy and those who did not were similar in terms of the size of omphalocele, presence of myelomeningocele and time of primary closure. We also found that osteotomy or failed closure has no effect on the eventual continence of cloacal exstrophy patients. While osteotomy is not the only variable involved in successful cloacal exstrophy closure, our results indicate the need for osteotomy in these patients to increase the success rate at the time of initial bladder closure.

Child↗

The application of continent urinary stomas to bladder augmentation or replacement in the failed exstrophy reconstruction.

OBJECTIVE: To determine the application and success of continent urinary stomas in the failed bladder exstrophy reconstruction. PATIENTS AND METHODS: A total of 35 patients underwent construction of a continent urinary stoma during bladder replacement or bladder augmentation between 1987 and 1993. The continent stoma was constructed with appendix (19 patients), tapered ileum (2), Benchekroun (12), Indiana (1) and Mainz type stoma (1). RESULTS: Three patients required revision of their continent stoma, one for incontinence and two for difficulty in catheterization. Overall, 95% experienced daytime continence on an intermittent catheterization regimen, while 90% were dry at night. The upper tract remained normal in 34 of 35 patients. CONCLUSIONS: While the Benchekroun technique is reliable in producing continence, difficulty with catheterization has been a chronic problem due to stomal stenosis. Our positive experience with using the appendix as a catheterizable conduit into either the bladder template or bowel segment has led us to prefer this method of continent stoma construction in the patient with a failed exstrophy. In the absence of an appendix, our preference is a tapered ileal segment. The failed exstrophy reconstruction can be salvaged with a continent stoma and substitution or augmentation cystoplasty in most patients, thus avoiding urinary diversion.

Adolescent↗

The anatomy of the pelvis in the exstrophy complex.

We compared computerized tomography scans of the pelvis of twenty-four patients who had exstrophy of the bladder with scans of age-matched controls in order to analyze the pelvic deformity that accompanies the variably severe manifestations of this condition. The patients who had classic exstrophy of the bladder were found to have a mean of 12 degrees of external rotation of the posterior aspect of the pelvis on each side, retroversion of the acetabula, a mean additional 18 degrees of external rotation and 30 per cent shortening of the pubic rami, and progressive diastasis of the symphysis pubis. The foot-progression angle demonstrated 20 to 30 degrees of external rotation beyond the normal limits seen in early childhood, but this improved with age. The patients who had exstrophy of the cloaca and the bladder not only had all of these pelvic deformities to a greater degree but also had asymmetry of measured parameters between the right and left sides of the pelvis, malformation of the sacro-iliac joints, and occasional dislocation of the hip. An understanding of the pelvic anatomy that accompanies exstrophy is essential when corrective approaches are planned. Such an understanding will improve the rate of success of both closure of the bladder and control of urinary continence postoperatively.

Adolescent↗