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Biomedical subjects

R D Jeffs

Publications and source records attributed to R D Jeffs.

At least 91 records · Page 5Linked to original sources
PubMed1988

Suprapubic bladder neck suspension for the management of urinary incontinence in the myelodysplastic girl.

Four young girls with myelodysplasia who remained incontinent despite intermittent catheterization and pharmacological manipulation underwent suprapubic bladder neck suspension along with bladder augmentation. Of the patients 3 underwent augmentation for decreased detrusor compliance and 1 as a part of a planned urinary undiversion. There was a mean increase in continence length of 1 cm. and a mean increase of 49 cm. water in urethral closure pressure in all patients. All patients currently are dry on intermittent self-catheterization and oxybutinin chloride with a minimum followup of 18 months with no evidence of upper tract deterioration. Therefore, suprapubic bladder neck suspension can be used successfully in the female patient with decreased outlet resistance. The procedure is easy to perform and would be easily reversible should incontinence recur and a further operation be required.

Adolescent↗
PubMed1988

Augmentation cystoplasty in the failed exstrophy reconstruction.

Of the 148 patients with bladder exstrophy seen at this institution during the last 10 years 12 have ultimately required bladder augmentation. In 4 cases augmentation was performed for an inadequate bladder capacity, upper tract decompensation and dry interval of less than 1 hour after bladder neck reconstruction and epispadias repair. Likewise, 3 patients underwent augmentation for an inadequate bladder capacity and dry interval of less than 2 hours after bladder neck reconstruction and epispadias repair. Of these 7 patients 3 had undergone 2 previous bladder neck reconstructions, while 4 had undergone 1 prior repair. Five augmentations were performed for an inadequate bladder capacity before any type of continence procedure had been done. Nine patients underwent adjunctive procedures in addition to bladder augmentation, including a Young-Dees-Leadbetter procedure in 4, an artificial urinary sphincter in 3, transureteroureterostomy and psoas hitch in 1, and a Mitrofanoff procedure and bladder neck closure in 1. Of the 12 patients 11 are continent, although 9 require intermittent catheterization. There were no major complications. However, 1 artificial urinary sphincter was removed for erosion 2 years after placement. Augmentation cystoplasty has provided prolonged stability of the upper tracts and continence in these patients, and it has proved to be a successful alternative to urinary diversion in this select group of exstrophy failures.

Bladder Exstrophy↗
PubMed1987

Anaplastic sarcoma arising in a mature metachronous bilateral Wilms' tumor after irradiation and chemotherapy. Spontaneous versus induced malignant change.

A 1-year-old male infant developed a classic Wilms' tumor of the left kidney. Treatment consisted of a left nephrectomy, chemotherapy, and irradiation to the left flank and associated abdomen. Two years later, a mass in the right kidney was discovered; open renal biopsy demonstrated a mature Wilms' tumor consisting entirely of rhabdomyomatous elements in the biopsy specimen. The patient was given a second course of chemotherapy and 2000 rad to the right flank. Over the next 8 years, the mass continued to grow without evidence of metastatic spread. Renal function deteriorated secondary to compression of the surrounding normal renal parenchyma by the enlarging tumor; creatinine clearance from the solitary kidney decreased from 120 ml/min to 40 ml/min during the 12 months prior to removal of the lesion. Via a nephron-sparing procedure, the 3400 g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms' tumor with a malignant anaplastic sarcoma arising in the central portion. Postoperatively, the patient received a third course of chemotherapy with no irradiation to the tumor bed. Currently, he is disease-free with normal renal function more than 20 years after diagnosis of the metachronous bilateral Wilms' tumor. This is the first reported case of an anaplastic sarcoma arising within a Wilms' tumor; this individual also is the longest surviving patient with metachronous Wilms' tumor. The various possibilities regarding the development of the anaplastic sarcoma within the Wilms' tumor of the right kidney are discussed, including the possible role of chemotherapy and irradiation in the development of a second malignancy.

Antineoplastic Agents↗
PubMed1987

Urinary diversion in hypospadias surgery 1987.

To determine which form of urinary diversion is most effective for each type of hypospadias, we polled all full-time practicing pediatric urologists in the United States and Canada. Each was asked to complete a questionnaire, specifying the method of urinary diversion used, based on the severity of the hypospadias. Ninety-eight per cent (84 of 86) of the urologists contacted replied to the survey. Our findings suggest that: Urinary diversion is usually not necessary for very mild types of hypospadias. Intraurethral drainage is the preferred form of diversion for moderate types of hypospadias. Suprapubic drainage with an intraurethral stent is the favored means of diversion for severe types of hypospadias. Perineal urethrostomy is rarely used today in hypospadias surgery.

Canada↗
PubMed1987

Metachronous bilateral Wilms tumor. Report of longest-known survivor and guidelines for conservative management.

In a one-year-old black male patient a classic Wilms tumor of the left kidney was treated with left nephrectomy, chemotherapy, and irradiation to the entire abdomen. Two years later a mature Wilms tumor, consisting predominantly of rhabdomyomatous elements, was discovered in the right kidney. Although a second course of chemotherapy was given and 2,000 rad were delivered to the right flank, the mass continued to enlarge with progressive deterioration of renal function. A nephron-sparing procedure was performed, in which a 3,400-g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms tumor with a malignant anaplastic sarcoma arising in the central portion. Currently, he is disease-free with normal renal function more than twenty years after diagnosis of the metachronous bilateral Wilms tumor. We believe he is the longest known surviving metachronous Wilms tumor patient and emphasizes the importance of conservative, meticulous surgery in the management of both unilateral and bilateral Wilms tumors. The role of multimodal therapy (chemotherapy, irradiation, and surgery) as well as the current guidelines for the management of bilateral Wilms tumors as proposed by the National Wilms Tumor Study are reviewed.

Combined Modality Therapy↗
PubMed1987

The use of parenteral testosterone therapy in genital reconstructive surgery.

Previously, testosterone cream has been used for penile enlargement before genital surgery. The results not only were inconsistent but the absorption was variable. Therefore, we elected to study the use of parenteral testosterone as an adjunct before reconstructive surgery. In 44 patients with hypospadias (36), epispadias (5) or urethral fistulas (3) 2 mg. per kg. testosterone enanthate were given intramuscularly 5 and 2 weeks before reconstructive surgery. Testosterone caused a mean increase in penile length of 2.7 cm. and in circumference of 2.3 cm. as well as local vascularity in all patients. In addition to surgical results the potential side effects of testosterone treatment were monitored 3 months to 1 year postoperatively. Basal testosterone levels were obtained in patients before and during therapy, and postoperatively. In addition, side effects, such as increased bone age and excessive pubic hair, were not a problem. The use of preoperative testosterone significantly contributed to the successful reconstruction of these patients, particularly those with a paucity of penile skin and those who had undergone previous repairs. Temporary penile stimulation by testosterone enanthate allows for an earlier penile operation as well as provides negligible side effects.

Child, Preschool↗
PubMed1987

A unified approach to early reconstructive surgery of the child with ambiguous genitalia.

Gender assignment in the newborn with ambiguous genitalia poses a dilemma for the parents and physician, even if the infant has a normal 46XX or 46XY karyotype. In general, female pseudohermaphrodites are reared as female subjects, since they can undergo reconstruction to appear as phenotypic female subjects and they may even be able to bear children. Male pseudohermaphrodites, patients with mixed gonadal dysgenesis and male patients with a micropenis may not be so straightforward, and proper gender assignment in these infants may be difficult. A unified surgical approach to early reconstruction of the external genitalia in these patients, who are to be reared as female subjects, has been developed. Essential to this perineal reconstruction are phallic reduction and recession in which erectile tissue is removed with preservation of the neurovascular bundles and glans, creation of labia minora from flaps of phallic skin and foreskin, a labioscrotal Y-V plasty to produce normal-appearing labia majora and construction of a neovagina with a perineal flap. If the patient is a male pseudohermaphrodite or an infant with a micropenis creation of the vagina is deferred until late adolescence or young adulthood. In other patients in whom the vagina enters the urethra or urogenital sinus proximal to the external sphincter a pullthrough vaginoplasty is performed when the child is 2 to 3 years old. If the vaginal entry is distal to the external urinary sphincter a flap vaginoplasty is performed at the same time as the phallic reduction, and creation of the labia minora and labia majora during infancy. Ten patients (4 with the adrenogenital syndrome, 2 male pseudohermaphrodites, 2 genetic male patients with a micropenis, 1 with mixed gonadal dysgenesis and 1 true hermaphrodite) have undergone reconstruction with this composite procedure. All 10 patients have had a successful cosmetic and early functional result.

Adrenal Hyperplasia, Congenital↗
PubMed1987

The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985.

The staged functional closure of classical bladder exstrophy has produced improved results for many urologists involved in the surgical management of this congenital anomaly. To determine which factors are most important for achieving a successful outcome (urinary continence with preservation of normal renal function) the 144 patients treated at The Johns Hopkins Hospital between 1975 and 1985 were reviewed. Of these patients 51 were managed entirely at our institution, while 93 had had the initial surgical treatment elsewhere. All patients who had undergone primary bladder closure and bladder neck reconstruction were divided into 2 groups: group 1--patients who had a successful initial bladder closure (an exstrophied bladder that is converted into a complete epispadias without wound infection, dehiscence or any degree of bladder prolapse on the first attempt) and group 2--children whose initial bladder closure was not successful. Both groups were analyzed with respect to bladder capacity at the time of bladder neck reconstruction, time required for the bladder to become sufficiently large for bladder neck reconstruction, urinary continence rate, and interval between bladder neck reconstruction and achievement of urinary continence. Patients in group 1 had the largest bladders at the time of bladder neck reconstruction (mean capacity 79 cc, p equals 0.03), shortest intervals between primary closure and bladder neck reconstruction (mean 3.5 years, p equals 0.006), highest urinary continence rate (92 per cent, p equals 0.002), and the shortest interval between bladder neck reconstruction and achievement of urinary continence (mean 1.5 years, p equals 0.18). These findings suggest that a successful initial bladder closure is an important factor for obtaining a larger bladder more quickly and for achieving a high urinary continence rate in patients with classical bladder exstrophy undergoing the staged functional bladder closure.

Bladder Exstrophy↗
PubMed1987

Exstrophy, epispadias, and cloacal and urogenital sinus abnormalities.

The congenital defects in the pelvis and perineal area, which are derived from defective development of the cloacal membrane, the urorectal septum, the mesonephric and paramesonephric (müllerian) ducts and the urogenital sinus, have been described. These may be life threatening, are often complex, and are certainly devastating to the parents. With knowledge of their embryologic origin, of their anatomy in detail, and the effect on function and drainage, treatment can be planned so that a good quality of life usually can be expected. Improved reconstructive programs and techniques can usually achieve near-normal appearance and function, with the expectation of good social adjustment.

Abdominal Muscles↗
PubMed1986

Incidental ganglioneuroblastoma.

A case is described in which an incidental ganglioneuroblastoma was discovered at the time of surgical correction of upper pole hydronephrosis secondary to an obstructing ectopic ureterocele in a duplicated system. We believe this is the first case of incidental ganglioneuroblastoma reported in the urologic literature. The management of this patient's disease is discussed as is the entity of incidental neuroblastoma.

Biopsy↗
PubMed1986

Pediatric applications of augmentation cystoplasty: the Johns Hopkins experience.

Since 1976, 23 children with bladder or cloacal exstrophy, meningomyelocele, sacral agenesis, the prune belly syndrome and noncompliant bladders associated with urethral valves or prior diversion underwent augmentation cystoplasty. Of these procedures 7 were combined with some type of urinary undiversion. Bowel segments used for augmentation included ileum alone in 10 patients, ileocecal segments in 4, a sigmoid patch in 8 and a hindgut patch in 1. An artificial urinary sphincter was placed at the time of bladder augmentation in 3 patients. There were no urinary fistulas or cases of urinary rediversion. Two patients required oral alkalizing agents as a result of persistent systemic acidosis. One patient required reoperation twice for ureteral obstruction, 1 had removal of the sphincter device secondary to erosion, 1 required reinforcement of the ileocecal valve owing to persistent reflux and 1 required reoperation for small bowel obstruction. Other complications included a superficial wound infection and 5 urinary tract infections, all of which were managed easily. Three patients were voiding and continent, 18 were dry with intermittent self-catheterization, 1 had giggle incontinence and 1 remained incontinent after sphincter removal. Augmentation cystoplasty appears to offer a reliable alternative to urinary diversion in the reconstructive management of children with small capacity bladders.

Adolescent↗
PubMed1986

Intraoperative urethral pressure profilometry as an adjunct to bladder neck reconstruction.

Between 1975 and 1984 we performed 17 bladder neck reconstructions with bladder neck suspension using intraoperative urethral pressure profilometry. Studies were done intraoperatively after bladder neck reconstruction and then they were repeated after bladder neck suspension. A continence length of 25 to 35 mm. and a urethral closure pressure of greater than 60 cm. water were obtained in all patients. Bladder neck suspension was noted to increase the continence length in all patients and allowed for at least a 2-fold increase in the closure pressures that were obtained. Urinary continence was assessed postoperatively from parental interviews. It seems important to achieve an initial closure pressure of at least 60 cm. water, while at the same time attempting to ensure anterior fixation. Intraoperative urethral pressure profilometry can serve as a valuable adjunct to bladder neck reconstruction by confirming the adequacy of bladder neck resistance after bladder neck reconstruction and again after bladder neck suspension.

Child↗
PubMed1986

Urethral prolapse in children: insights into etiology and management.

The pathophysiology of urethral prolapse has never been clearly elucidated. Following en bloc removal of the urethra, bladder, vagina, uterus and pelvic floor tissues from a 4-year-old burn victim with urethral prolapse at the time of death and from a female newborn cadaver, careful step-sectioning of the specimens at 5 mm. intervals for histological examination was performed. The anatomical defects of urethral prolapse noted were marked eversion of the urethral mucosa, vascular congestion of the "corpus spongiosum urethrae muliebris", and a cleavage plane between the inner longitudinal and outer circular-oblique smooth muscle layers of the urethra. We propose that urethral prolapse results from poor attachments between the smooth muscle layers of the urethra in association with episodic increases in intra-abdominal pressure. A clinical review of 12 cases revealed that at least 8 (67 per cent) had antecedent episodes of marked increases in intra-abdominal pressure. Obliteration of the cleavage plane between the smooth muscle layers is important for successful treatment of this lesion.

Abdomen↗
PubMed1986

Ectopic ureter and ureterocele: their varied sonographic manifestations.

The sonographic examinations of four patients with simple ectopic ureters and 11 with ectopic ureteroceles were reviewed to determine distinguishing characteristics. Ectopic ureters, in cases of extreme dilatation and tortuosity, sometimes mimic multiseptated, cystic abdominal masses. However, the proximal portions of some severely dilated ureters are surprisingly small. Ectopic ureters sometimes indent the lower vesical wall, simulating a ureterocele. Ectopic ureteroceles are dynamic structures, changing in shape and size according to intravesical pressure. The lower pole of a duplex kidney may be difficult to detect because of displacement by the dilated upper renal pelvis and ureter. The renal parenchyma associated with an ectopic ureter may be equally difficult or impossible to find because of diminutive dysplasia or, less commonly, acquired atrophy. Dysplasia is characterized sonographically by highly echogenic parenchyma, lack of corticomedullary differentiation, and occasionally massive enlargement by cysts. Ectopic ureters and ureteroceles can be identified by fetal sonography.

Adolescent↗
PubMed1985

The use of topical vasodilators as an adjunct in infant renal surgery.

In infant renal surgery there is a well recognized problem with vasospasm and resultant renal ischemia. Recently, we applied vasodilators to the perivascular tissues during renal surgery to prevent vasospasm. With this mode of treatment no renal units were lost owing to ischemia secondary to the operative procedure. The treatment has been especially useful in infants with duplicated systems undergoing partial nephroureterectomy. Owing to the diminutive size of infant renal vessels, vasospasm can be triggered easily even with the most careful hilar dissection. Thus, to assure maximal preservation of tissue, vasospasm must be prevented before renal mobilization and hilar dissection. Therefore, to prevent the development of renal ischemia in infants undergoing renal surgery we recommend initial exposure of the hilar vessels before dissection, application of papaverine and lidocaine to the perivascular tissues, and delaying further dissection until maximal vasodilation has occurred.

Administration, Topical↗
PubMed1985

Muscarinic cholinergic receptors in bladder exstrophy: insights into surgical management.

The surgical management of classical bladder exstrophy (functional bladder closure or urinary diversion) should be influenced by the inherent detrusor function of the exstrophied bladder. Cystometrograms performed previously on individuals with successful exstrophy closures demonstrate normal bladder function. The biochemical and neurophysiological properties of the exstrophied bladder have otherwise not been investigated. In this study radioligand receptor binding techniques were used to compare the density and equilibrium dissociation constant of muscarinic cholinergic receptors in control and exstrophy bladders. The density of muscarinic cholinergic receptors in the control and exstrophy groups was 1.97 plus or minus 0.29 and 1.44 plus or minus 0.21 fmol. per microgram deoxyribonucleic acid (mean plus or minus standard error of mean), respectively. The dissociation constant of the control and exstrophy groups was 0.15 plus or minus 0.02 and 0.14 plus or minus 0.02 nM. (mean plus or minus standard error of mean), respectively. These data show that the muscarinic receptor density and binding affinity in control and exstrophy bladders are similar. Therefore, the neurophysiological composition of the exstrophied bladder is not grossly altered during the anomalous development.

Bladder Exstrophy↗
PubMed1985

Neonatal gonadal stromal tumor of the testis: limitations of tumor markers.

We report case 10 of a gonadal stromal tumor of the testis in a neonate. This tumor may occur in the testis or ovary, and is defined as a tumor of any of the specialized supporting structures of the male or female gonad. All neonatal tumors reported to date have been benign. The serum alpha-fetoprotein level, although elevated for adults, was within the normal range for an infant.

Humans↗