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Biomedical subjects

R D Jeffs

Publications and source records attributed to R D Jeffs.

At least 73 records · Page 4Linked to original sources
PubMed1991

Failed bladder neck reconstruction: options for management.

During the last 10 years 17 patients have been seen at this institution for persistent urinary incontinence after Young-Dees-Leadbetter bladder neck reconstruction. Of these patients 16 were born with classical bladder exstrophy and 1 with complete epispadias. Six patients underwent 1, 10 underwent 2 and 1 underwent 3 prior bladder neck procedures. As salvage procedures 8 patients underwent another Young-Dees-Leadbetter procedure, 1 repeat bladder neck reconstruction and augmentation cystoplasty, 3 augmentation alone, 4 bladder augmentation with creation of a continent abdominal stoma and 1 augmentation with implantation of an artificial urinary sphincter. Of the 8 patients who underwent a repeat Young-Dees-Leadbetter procedure 7 are dry for 3 hours or more and 1 is dry for greater than 3 hours on intermittent self-catheterization. All of those who are dry for greater than 3 hours are dry at night and 1 wears pads when engaging in strenuous physical activity. Of the 9 patients who underwent augmentation cystoplasty along with other adjunctive procedures 8 are continent for greater than 3 hours on intermittent catheterization, 6 are dry at night if they perform catheterization at bedtime and 1 remains totally incontinent after removal of the artificial urinary sphincter. Thus, with persistence and creativity a child with a previously failed bladder neck reconstruction or even multiple failed repairs can be made socially continent, providing a satisfactory alternative without resorting to urinary diversion.

Adolescent↗
PubMed1991

Endoscopic injection of glutaraldehyde cross-linked bovine dermal collagen for correction of vesicoureteral reflux.

From November 1986 through May 1989, a Food and Drug Administration approved investigational study was done to assess the safety and efficacy of glutaraldehyde cross-linked bovine dermal collagen in the endoscopic treatment of vesicoureteral reflux. Over-all, 57 patients (92 ureters) were treated. The majority of ureters (68.5%) had grade II to III/V vesicoureteral reflux (international classification). One treatment was given in 61.4% of the patients, while 33.3% required 2 and 5.3% required 3 treatments. Nonduplicated/primarily refluxing ureters comprised 68.5% of the total, while 13% were duplex/primarily refluxing and 18.5% were surgical failures. The procedures were performed on an outpatient basis in all but 3 patients. Patients were evaluated by voiding cystourethrogram and renal/bladder sonography before and after treatment at 1 month and 1 year. Cure at 1 month after the last treatment was achieved in 75% of the ureters. Among the ureters cured at 1 month the cure persisted in 79% at 1 year after treatment. Cure at 1 year was achieved in 65% of all ureters evaluated, regardless of the status at 1 month. Procedure-related morbidity was minimal and there were no adverse reactions to the implant substance. Thus, glutaraldehyde cross-linked bovine dermal collagen appears to be safe and effective in the endoscopic treatment of vesicoureteral reflux.

Animals↗
PubMed1991

Use of the Benchekroun hydraulic valve as a catheterizable continence mechanism.

Problems with continence and access for catheterization with previous continent stoma techniques have led us to search for an alternate method. We report our initial experience with the Benchekroun hydraulic valve in 9 patients. Five patients underwent creation of an ileocolic bladder: for myelodysplasia in 1, after cystectomy for bladder cancer in 2 and for exstrophy in 2. Two patients underwent conversion from an uncatheterizable Indiana pouch because of exstrophy (1) and interstitial cystitis (1). Two patients underwent small bowel augmentation of a small, poorly compliant bladder with an abdominal Benchekroun stoma: 1 had exstrophy and 1 had myelodysplasia. Patient age ranged from 7 to 63 years. Two patients had de novo creation of an ileocolic bladder, whereas 7 had bladder or previous bowel conduits incorporated in the continent reservoirs. At up to 20 months of followup all patients had diurnal and nocturnal continence, and none had experienced serious long-term complications. Problems were encountered in 5 patients. One patient had a valve fistula that was surgically revised. One patient experienced transient difficulty with catheterization when the reservoir was overdistended and a false passage developed. Three patients had stomal stenosis and require periodic dilation. We believe the Benchekroun hydraulic valve to be a useful adjunct in the construction of a continent urinary reservoir. It is easy to construct, provides reliable continence and is easy to catheterize. Furthermore, it may be applied to standard ileocolic bladders either de novo or as a salvage procedure for failed efferent limbs.

Adolescent↗
PubMed1991

Childhood urolithiasis: experiences and advances.

Between June 1979 and June 1989, 54 children with urolithiasis were evaluated and treated at the Johns Hopkins Children's Center. The most common symptoms were flank or abdominal pain (58%) and gross hematuria (28%). In 46 children (86%), stones were secondary to a preexisting condition and in only 8 (14%) no apparent cause of stone formation could be found. Thirty-six patients (66%) had a solitary stone, most commonly found in the kidney. Urinary tract infections were present in 25 (47%) of the patients who had stones. Stones composed either of calcium oxalate or struvite were the most frequently recovered in these patients with infections. Twenty-one patients (39%) spontaneously passed their stones whereas 23 (43%) required either surgery or extracorporeal shock-wave lithotripsy to resolve stones. Ten (20%) showed recurrence of their urolithiasis, with follow-up examination periods ranging from 1 month to 10 years. Recent advances in the management of urolithiasis and their applicability to the pediatric population are discussed.

Adolescent↗
PubMed1990

Continent urinary reservoirs in pediatric urological practice.

During the last 3 years 20 patients (median age 8 years) have undergone construction of a continent urinary reservoir at our pediatric urology service. Of the patients 12 had failed exstrophy reconstruction, 7 had myelodysplasia with a neurogenic bladder and 1 had extensive bladder damage as a result of a previous operation. In 5 patients a continent reservoir was constructed after cystectomy performed in early childhood. Techniques of reconstruction included the Mitrofanoff procedure (4 patients), a modification of the Indiana pouch (12), a modification of the Benchekroun procedure (2), the use of the appendix in situ as a continence mechanism (2) and a modified Kropp procedure using a duplicate vagina for catheterization (1). The morbidity from these procedures was acceptable and consisted primarily of chronic diarrhea (4 patients), stone formation within the pouch (5) and the need to revise the continence mechanism (7). All but 1 patient maintained stable or improved upper tracts after diversion. Over-all, 85% of the patients experienced diurnal continence on an intermittent catheterization regimen, while 75% were dry at night. The plicated/intussuscepted ileal nipples required revision because of difficulty with catheterization (7 patients) and urinary leakage. We currently favor the use of the Benchekroun or Mitrofanoff techniques for the ease of construction and minimal requirements for revision.

Bladder Exstrophy↗
PubMed1990

Nuclear morphometry as a prognostic indicator for genitourinary rhabdomyosarcoma: a preliminary investigation.

Rhabdomyosarcoma of the urogenital tract is a malignant mesenchymal tumor seen primarily in childhood. Multimodal therapy, encompassing surgery, radiotherapy and chemotherapy, has dramatically improved the survival of patients with this disease. However, the quest for markers of tumor aggression is important to decrease the morbidity of treatment given to patients with good prognosis tumors, while at the same time intensifying treatment of tumors with poor prognosis. Using archival tumor specimens from 13 patients with genitourinary rhabdomyosarcoma, a multivariate analysis of multiple nuclear shape descriptors was done with the Hopkins Morphometry System. Three nuclear shape descriptors clearly separated patients with no evidence of disease recurrence or progression from those with recurrent disease, progressive disease or death of disease. These nuclear shape descriptors were standard error of the chain code standard deviation analysis (p = 0.010), range of the feret ellipticity distribution (p = 0.016) and standard error of the chain code range analysis (p = 0.037). With multivariate analysis these shape descriptors taken together separated patients with good and poor prognoses to a level of significance of p = 0.007. Thus, nuclear morphometric analysis may prove to be useful as an individual prognostic indicator in childhood genitourinary rhabdomyosarcoma and warrants further analysis in a much larger, blinded, controlled study.

Cell Nucleus↗
PubMed1990

Local tissue reaction to the subureteral injection of glutaraldehyde cross-linked bovine collagen in humans.

Although the technique of subureteral injection has been widely accepted as an alternative to reimplantation in the treatment of vesicoureteral reflux, the choice of the material to be used is controversial. We have used glutaraldehyde cross-linked bovine collagen to correct vesicoureteral reflux within the context of a Food and Drug Administration approved investigational study. We report the local tissue reaction to the implanted collagen in 7 patients who underwent reimplantation 3 to 19 months after failed endoscopic therapy. Glutaraldehyde cross-linked bovine collagen engendered a minimal localized inflammatory reaction without causing granuloma formation. Subsequent reimplantation was not hindered by the presence of the implant materials.

Child↗
PubMed1990

Cloacal exstrophy: radiologic findings in 13 patients.

Cloacal exstrophy is a complex, multisystem malformation that has not received much attention in the radiologic literature. The clinical histories and imaging studies of 13 cases of cloacal exstrophy proved surgically or at autopsy were reviewed. Radiologic observations were based on skeletal plain films (13), contrast-enhanced examinations of the genitourinary system (12), abdominopelvic sonograms (nine), gastrointestinal studies (seven), CT scans of the pelvis and CNS (seven), MR images of the pelvis and CNS (six), renal nuclear medicine scans (three), myelography (three), and three-dimensional scans of the pelvis (two). Genitourinary and gastrointestinal abnormalities identified in our 13 patients included bladder malformations (13), hindgut anomalies (13), undescended testes (five), malrotation (five), duplicated müllerian structures (four), and renal ectopia (four). Skeletal abnormalities included symphysis pubis diastasis (13), posterior element dysraphism (12), vertebral segmentation anomalies (12), clubfeet (six), and hip dislocations (five). CNS anomalies included meningocele (nine) and Chiari malformation (one). Immediate surgical closure of the defect with maximal bowel preservation and diverting colostomy offers optimal chances for survival. Appropriate preoperative gastrointestinal and genitourinary evaluation is essential.

Abnormalities, Multiple↗
PubMed1990

Fetal genitourinary tract anomalies: evaluation, operative correction, and follow-up.

The prenatal diagnosis of a genitourinary anomaly was made in 53 fetuses. Sonographic findings, antenatal course, and postnatal treatment and outcomes were examined. No interventional therapy was undertaken in utero, and the natural history could be examined in the 44 of 53 (83%) who did not electively terminate their pregnancies. Twenty-three of the total 53 (43%) had unilateral disease and 30 (57%) had bilateral involvement. Thirty-five of 53 (66%) survived, with 22 undergoing operative therapy postnatally. In all but one of the 35 survivors, the anomaly was isolated to the genitourinary tract, and the majority of surviving fetuses had unilateral disease. Oligohydramnios was present in only three of 35 survivors, and none had severe oligohydramnios. Nine of 53 women underwent termination of pregnancy and nine others experienced a neonatal death. All 18 of their fetuses had bilateral disease, with oligohydramnios present in 14 (78%). All five fetuses with chromosomal abnormalities were found in this group of 18. The majority of fetuses with a genitourinary anomaly will do well postnatally. Pulmonary hypoplasia, extrarenal anomalies, and chromosomal defects are frequent findings in the minority with poor outcomes. A multidisciplinary approach to management of the pregnancy with a fetal genitourinary tract anomaly is essential to optimize outcome.

Abortion, Eugenic↗
PubMed1989

Laparoscopy: its selected use in patients with unilateral nonpalpable testis after human chorionic gonadotropin stimulation.

Laparoscopy has been used to help evaluate patients with a unilateral nonpalpable testis. This procedure can be performed quickly just before exploration with the patient under the same anesthetic. With laparoscopy it has been possible to localize either a testis or the course of the spermatic vessels in 100 per cent of the patients. Preoperative knowledge of testis location is helpful to plan the location of the incision as well as the type of repair. The incidence of vanishing testis in our series is much higher than that reported previously. This difference is attributed to a careful examination with the patient under anesthesia and preoperative treatment with human chorionic gonadotropin, which made many testes palpable. These results indicate that laparoscopy can be performed safely and quickly, and that it is helpful to manage patients with a unilateral nonpalpable testis.

Adolescent↗
PubMed1989

Nutritional consequences of bowel segments in the lower urinary tract.

To assess for altered fat absorption in a group of 26 patients who underwent bladder replacement or augmentation between 1975 and 1988 serum samples were assayed for levels of B12 and carotene. Reconstruction was done with ileum and/or cecum in 22 patients, and 4 who had undergone sigmoid cystoplasty and were not expected to be at risk for fat malabsorption were included as controls. Followup ranged from 4 months to more than 8 years. No patient demonstrated low values of B12 or carotene regardless of postoperative duration, bowel segment location or length of segment. Use of ileal segments less than 45 cm. long even with associated incorporation of the ileocecal valve and adjacent cecum does not appear to compromise fat absorption or the enterohepatic circulation.

Carotenoids↗
PubMed1989

Bladder exstrophy: increase in capacity following epispadias repair.

The achievement of satisfactory continence in the management of classical bladder exstrophy remains a surgical challenge. This may be particularly difficult in the patient with a small bladder capacity after initial closure. The 12-year experience at our institution with bladder exstrophy is reviewed and attention is focused on the approach to those patients with a small bladder capacity. A total of 155 children with bladder exstrophy were treated. Of these 155 patients 28 boys were believed to have a bladder capacity inadequate for satisfactory bladder neck reconstruction (less than 60 cc). All patients have undergone staged reconstruction with a urethroplasty for epispadias and they are available for evaluation. After a median interval of 22 months bladder capacity increased by a median 54.5 cc. No child had hydronephrosis after the epispadias repair or bladder neck reconstruction. Of the 28 patients 25 have undergone bladder neck reconstruction with a median followup of 4.5 years and 88 per cent (22 of 25) demonstrate continence with a dry interval greater than 3 hours. Another 2 patients have been rendered continent following bladder augmentation. These results demonstrate that satisfactory continence is an achievable goal in the exstrophy patient with a small bladder capacity when bladder neck reconstruction is preceded by epispadias repair.

Bladder Exstrophy↗
PubMed1989

A new technique of using the in situ appendix as a catheterizable stoma in continent urinary reservoirs.

A new technique of using the in situ appendix to construct a continent catheterizable stoma is described in a patient who underwent continent urinary diversion, and the technical aspects of this procedure are illustrated in detail. The appendix is remodeled by invaginating its base into the cecum for 1 cm., cecoplicating the middle portion for 2 cm. and bringing the distal end to the skin as a cutaneous stoma. This new technique demonstrates that the in situ appendix can be constructed successfully to provide continence without the need for isolating it from the cecum and implanting the distal end into the urinary reservoir as described in the Mitrofanoff technique.

Adult↗
PubMed1988

Delayed bladder rupture after augmentation enterocystoplasty.

Delayed bladder perforation with peritonitis following augmentation enterocystoplasty in children with spina bifida is a serious and potentially life-threatening complication. Our experience with 4 such cases is presented. All patients had spina bifida with a neuropathic bladder and they had undergone augmentation enterocystoplasty with a tubular colonic segment of large bowel as part of an undiversion procedure. All patients were being managed with intermittent self-catheterization. The interval from augmentation enterocystoplasty until presentation ranged from 6 months to 3 years. Diagnosis was delayed in all cases, including 3 in which cystogram studies were normal despite findings of extravasation of urine at exploration. In 1 patient generalized sepsis developed with the respiratory distress syndrome and, subsequently, she died.

Adolescent↗
PubMed1988

Abdominal wall defects and cryptorchidism: an animal model.

Intra-abdominal pressure has been implicated in the mechanism of testicular descent. Boys born with abdominal wall defects reportedly have an increased incidence of cryptorchidism. To examine this relationship further a rat model was developed. The rat testis normally descends postnatally on or about day 21. In this study newborn male rats underwent surgical excision of all anterior abdominal musculature from the umbilicus to the xiphoid, while a control group had a sham procedure. In both groups steel wire was inserted into both testes as radiological markers to monitor descent. Animals were explored on day 28 to confirm the position of the testes. A total of 36 animals survived all sections of the protocol. Eight animals had adhesions and were dropped from the study. The results of 17 rats in the experimental group and 11 in the control group revealed that the surgically created abdominal wall defect did not result in cryptorchidism. This finding suggests that an intact anterior abdominal wall is not a prerequisite for testicular descent in the rat.

Abdominal Muscles↗
PubMed1988

Epispadias and incontinence: the challenge of the small bladder.

The 12-year experience at our hospital with epispadias and incontinence is reviewed and attention is focused on the approach to patients with a small bladder capacity. Of 29 children 20 had incontinence associated with the epispadias (13 boys and 7 girls). A total of 15 patients underwent definitive management of the incontinence with a Young-Dees bladder neck reconstruction with or without ureteral reimplantation and they are evaluable. Nine patients (6 boys and 3 girls) had a bladder capacity that was believed to be inadequate for satisfactory bladder neck reconstruction (less than 60 ml.). To date, 7 of these children have undergone staged reconstruction with urethroplasty for epispadias as the initial operation. Bladder capacity increased 95 ml. on the average (range 60 to 140 ml.) after the urethroplasty. No child had hydronephrosis before or after bladder neck reconstruction. Review of the results of bladder neck reconstruction in all patients with a mean followup of 2.9 years (range 7 months to 13 years) demonstrates an 87 per cent satisfactory continence rate (9 of 9 boys and 4 of 6 girls). In the patients who underwent staged reconstruction the results were similar, with a 100 per cent continence rate in evaluable patients (5 of 5 boys and 2 of 2 girls). In conclusion, urethroplasty before bladder neck reconstruction in the epispadiac child with a small bladder allows an increase in capacity, thus, obviating for the most part the need for procedures such as bladder augmentation in the majority of these patients.

Child, Preschool↗
PubMed1988

Androgen receptor levels and 5 alpha-reductase activities in preputial skin and chordee tissue of boys with isolated hypospadias.

The cause of hypospadias in the majority of patients is unknown. We examined the hypothesis that hypospadias might be explained by androgen receptor abnormalities in the atretic spongiosal tissue commonly known as chordee. We studied 10 patients with relatively severe hypospadias but with a predominantly male phenotype and no readily ascertained explanation for the defect, including no evidence of an abnormality in testosterone biosynthesis. Eight subjects had midshaft hypospadias and 2 had a penoscrotal meatus. All 10 patients had severe chordee. Serum concentrations of testosterone, and luteinizing and follicle-stimulating hormones were measured before human chorionic gonadotropin stimulation and a serum testosterone level was determined 24 hours after the last dose of a 5-day human chorionic gonadotropin stimulation (3,000 units per M.2 per day). Androgen receptor content and binding affinity were assayed in fibroblasts cultured from preputial skin and chordee tissue of patients and foreskin from normal male neonates. With the endogenous ligand dihydrotestosterone the mean number (maximum binding capacity) of androgen receptors was 1,013 fmol. per mg. deoxyribonucleic acid in preputial skin and 833 fmol. per mg. deoxyribonucleic acid in chordee tissue of patients, and 627 fmol. per mg. deoxyribonucleic acid in the foreskin of controls. With the nonmetabolizable, synthetic androgen methyltrienolone (R1881) the mean maximum binding capacity was 1,004, 722 and 758 fmol. per mg. deoxyribonucleic acid, respectively. Dihydrotestosterone receptor affinity (dissociation constant) was similar in preputial skin (0.20 nM.) and chordee tissue (0.21 nM.) from patients, and foreskin (0.29 nM.) from controls. Androgen receptor binding affinity of R1881 also was similar (0.30, 0.24 and 0.21 nM., respectively). Furthermore, the 5 alpha-reductase activity of preputial skin and chordee tissue of patients with hypospadias was similar to that of foreskin from normal neonates. In conclusion, isolated hypospadias in these subjects was not associated with androgen insensitivity of the spongiosal tissues on the basis of either decreased androgen receptor binding affinity, receptor number or conversion of testosterone to dihydrotestosterone.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗