Robert D. Jeffs

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.

Ureteral Structure and Ultrastructure. Part II. Congenital Ureteropelvic Junction Obstruction and Primary Obstructive Megaureter

The structure and ultrastructure were studied of 48 specimens from cases of congenital ureteroplevic junction obstructions and primary obstructive megaureters. Under light microscopy a spectrum of findings occurs, extending from the nearly normal to the clearly abnormal obstructive segments. However, under electron microscopy there were consistent abnormalities: 1) excessive collagen fibers between and around the muscle cells and 2) a group of compromised muscle cells proximal to the collagenous segment. These findings are responsible for functional discontinuity via the nexus and indistensibility of the pathologic areas. The high resolution of the electron microscope allows clearer definition of these obstructions and the impact of these findings on surgical remodeling is discussed.

The Factors in Successful Exstrophy Closure

Abstract A review of the successes, failures and complications of more than 100 cases of bladder exstrophy provides the basis for treatment now being used on new patients. The technical aspects of this treatment that provide the best chance for continence without renal damage and satisfactory external genitalia are discussed.

The inheritance of the exstrophy-epispadias complex.

The inheritance pattern of the exstrophy-epispadias complex was investigated by a combined literature review and a survey of the personal experience of pediatric urologists in North and South America, and Europe. Bladder exstrophy recurred in only 9 of approximately 2,500 families (1 in 275) with bladder exstrophy or complete epispadias. The recurrence of the exstrophy-epispadias complex in offspring of parents with bladder exstrophy or complete epispadias has never been described previously. We identified 215 offspring produced by parents with bladder exstrophy or epispadias, and bladder exstrophy was inherited in 3 of the offspring (1 in 70 live births). The recurrence of bladder exstrophy in offspring of parents with the exstrophy-epispadias complex is greater than previously assumed.

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.

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.

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.

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.

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.

Anterior Innominate Osteotomies For Failure or Late Closure of Bladder Exstrophy

The bony pelvis was analyzed in 12 patients undergoing a further operation after initial bladder closure. Of the patients 5 had undergone a prior posterior osteotomy. All patients had wide diastasis of the pubis (average 5.5 cm.). In 9 of these patients late closure or reclosure of a failed initial bladder repair was done and 3 underwent a repeat bladder neck reconstruction. In all patients a new procedure, anterior iliac osteotomy with internal or external fixation, was performed. This procedure provides increased mobility of the pubis and increased correction. It avoids turning of the patient while under anesthesia for repeat preparation and in most cases postoperative traction is not needed. There were no instances of dehiscence, nonunion or infection. Three cases of transient femoral palsy were noted. All patients had a normal gait 4 months postoperatively. The mobility obtained after anterior osteotomy allows for excellent approximation. In view of evidence that approximation of the pubis improves closure and eventual continence results, we believe that osteotomies, even when repeated, are useful in revision surgery if there is bony diastasis.