We assessed the role of surgery, particularly exenteration, in the treatment of children with lower urinary tract and pelvic rhabdomyosarcoma.
We treated 23 children with bladder and/or prostate
11
, ...or pelvic retroperitoneal tumors
12
. Initial management was tumor resection in 6 cases, anterior pelvic exenteration in 5 and biopsy only in 12, combined with chemotherapy in 23 and radiotherapy in 20.
The bladder salvage rate for surviving patients with pelvic tumors was 92 percent versus 27 percent for those with prostate/bladder tumors. Estimated 5 and 10-year survival probability for patients with pelvic retroperitoneal tumors was 49 plus/minus 50 percent compared to 81 plus/minus 24 percent for those with bladder and/or prostate tumors (log rank test, p = 0.11).
Exenterative surgery is frequently required to achieve a durable complete response.
The oncogenic E7 proteins of human papilloma virus (HPV 16) and of cottontail rabbit papilloma virus (CRPV) have been purified
from an expression system in Escherichia coli. The proteins as purified ...from E. coli contain one tightly bound Zn(II) ion
per molecule. The metal site shows facile exchange with either Cd(II) or Cu(I). The HPV 16 E7 maximally bound one Cd(II) or
two Cu(I) ions, while the CRPV E7 bound two Cd(II) or three Cu(I) ions. The Cd(II) and Cu(I) E7 molecules exhibited optical
transitions in the ultraviolet suggestive of metal:thiolate coordination. E7 proteins from HPV 16 and CRPV contain 7 and 8
cysteines/molecule, respectively. Reaction of the E7 proteins with the sulfhydryl reagent, dithiodipyridine, revealed that
all the cysteinyl sulfurs are present in the reduced thiol state. Cu(I)-E7 molecules are luminescent with maximal emission
at 570 nm. The observed emission at room temperature is indicative of metal coordination within a compact protein environment
shielded from solvent interactions. The emission maxima occurs at the same wavelength (570 nm) as Cu(I)-cysteinyl sulfur clusters
in Cu(I)-metallothioneins. The single Zn(II) atom in each protein can be removed from E7 in the presence of EDTA. The resulting
apoE7 molecules remain soluble and can be partially reconstituted with Cd(II) to regain the ultraviolet charge transfer transitions.
We reviewed 52 children with ureteroceles in an effort to evaluate the various facets of this disorder that influenced our surgical management. There were 12 single system ureteroceles and 40 duplex ...system ureteroceles. Total reconstruction was performed in 16 duplex system and 8 single system ureteroceles, of which 88 per cent required no further surgery. Upper pole heminephrectomy or ureteropyelostomy with partial ureterectomy was performed in 22 patients with duplex system ureteroceles with the goal of obviating lower tract surgery, of whom 12 (55 per cent) required subsequent surgery. In 6 patients, 4 with single system and 2 with duplex system ureteroceles transurethral incision of the ureterocele was the initial procedure with the expectation that improvement in function and hydronephrosis would facilitate subsequent lower tract surgery. Two of these patients required subsequent reconstruction. Recommendations regarding management are based on initial pathological condition, patient age and the presence of a single or duplex system ureterocele.
In most recent series of artificial sphincter implantations there has been a reduction in mechanical and surgical complications, with continence rates approaching 90 per cent. Despite initially ...excellent results in 47 children, with increasing durations of followup we noted a transient hydronephrosis related to incomplete bladder emptying in 5 and a persistent physiological alteration of detrusor dynamics consisting of a rigid, noncompliant bladder in 7. Preoperatively, all patients had had indepth radiological studies and most had a urodynamic evaluation, and were considered to be excellent candidates. The etiology of these alterations is not understood at this time. Bladder and even upper tract deterioration can occur without the appearance of urinary leakage. Therefore, children with an artificial sphincter must be monitored indefinitely with semiannual assessment of the upper tracts and periodic urodynamic evaluation.
A total of 33 patients underwent hypospadias or epispadias repair by patch or tubular grafts of skin or bladder epithelium from July 1980 to January 1985. Indications included previous circumcision, ...proximal primary hypospadias, severe chordee alone, inadequate local skin during extended urethroplasty at 2-stage hypospadias repair, multiple previous reconstructions and failed epispadias repair. Genital skin grafts were used in 25 patients, extragenital skin grafts in 4 and bladder epithelial grafts in 4. Complications occurred in 13 patients (39.4 per cent): 8 required reoperation for a fistula, stricture or diverticulum, and 5 underwent internal urethrotomy or urethral dilation. Of the 8 patients 3 needed more than 1 revision. These revisions were short and generally uncomplicated. Final results uniformly were good functionally and cosmetically. Based on our experience and that of others we believe that despite a high incidence of minor complications a free graft serves as an excellent substitute urethra for treatment of a variety of urethral problems in children.
A 10-year-old boy had intermittent ureteropelvic junction obstruction that proved to be owing to benign fibroepithelial polyps. The clinical features and management of this rare lesion are discussed.
Proline uptake by rat renal brush border membrane vesicles from animals 7 days of age and older has been examined to delineate developmental changes in membrane function that may underlie the ...physiological hyperprolinuria of young animals. Although the two proline transport systems normally present in adult membranes were found in membranes from young animals, the proline ``overshoot'' resulting from a sodium ion gradient is minimal and increases with age of the animal from which the membranes were isolated. This is associated with a severalfold faster entry of 22Na into vesicles of the 7-day-old animal compared to entry into membranes prepared from adult kidneys. The very rapid dissipation of the sodium gradient thus diminishing the driving force for transmembrane proline movement may explain the changes in proline overshoot observed in membranes from young animals. The altered sodium permeability is consistent with the fact that young animals have a generalized inability to reabsorb other amino acids whose transport is known to be sodium gradient stimulated.
We report a case of a male newborn with complete epispadias and an umbilical lesion. Histological examination of the surgically removed lesion revealed a colonic remnant with no communication to the ...underlying bowel. This entity is one of the exstrophy/epispadias complex variants known as covered exstrophy with sequestered bowel, and is case 4 in the world literature. Simple excision of the lesion was accomplished with staged correction of the epispadias planned.
Surgical correction of major hypospadias defects is a challenging problem. Bladder mucosa has been reported as an excellent tissue for construction of a neourethra. We used bladder mucosa in 13 boys ...to create 15 neourethras when there was inadequate preputial skin to perform a vascularized pedicle flap or a free preputial graft. In 3 cases a bladder mucosa graft was used for initial repair of the hypospadias. All other boys had undergone prior hypospadias surgery. In 4 boys the initial result was satisfactory. Seven minor complications occurred in 5 boys, while 4 had more major complications. In 2 boys severe problems with stricture necessitated graft replacement and a satisfactory outcome has not been achieved. Our problems with stricture and stenosis seem to be related to the use of a subcutaneous tunnel for the graft bed and coring of the glans to create the meatus. Over-all, in 11 of the 13 boys the bladder mucosal graft provided an excellent resolution of the complex problem with easily managed postoperative complications.
Urinary continence is one of the more difficult goals to achieve in patients with exstrophy or epispadias. The artificial genitourinary sphincter provides an alternative to reconstruction of the ...bladder neck for management of this problem. Although excellent continence can be obtained with bladder neck reconstruction, results of a second procedure of this type seldom are reported. We used the AS800 device in 16 patients with exstrophy and epispadias, 13 of whom had undergone a prior bladder neck operation. We defined our results as total success and continence success, and have achieved over-all rates of 69 per cent (excludes those awaiting revision) and 90 per cent (those with an active device), respectively. Although the frequency of revision and erosion was significant, the ultimate outcome in this difficult patient group was satisfactory.
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