An unusual presenting symptom of sarcoidosis: neurogenic bladder dysfunction.
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Biomedical subjects
Publications and source records attributed to D A Husmann.
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PURPOSE: In patients with a neurogenic bladder augmentation with the ileal cecal segment has generally been disregarded as an option due to concerns regarding fecal incontinence. We report our results using a hemi-Indiana pouch bladder augmentation for neurogenic bladder. MATERIALS AND METHODS: A review of 63 patients with neurogenic bladder dysfunction treated with a hemi-Indiana pouch bladder augmentation was performed. None of the patients had fecal incontinence before surgery. RESULTS: Median followup interval was 6 years (range 1 to 10). Etiology of the neurogenic bladder was spinal cord injury in 42 patients (66%), myelodysplasia in 18 (29%) and sacral agenesis in 3 (5%). One patient (1.5%) died immediately postoperatively from a pulmonary embolus. Of the remaining 62 patients, urinary continence was achieved in 54 (87%) and 8 were incontinent per the abdominal stoma. All 8 patients were treated with Contigen injections into the stoma and 4 (50%) became continent. The 4 patients with persistent urinary incontinence became continent after open surgical revision. Postoperatively, fecal continence was improved in 14 patients (23%), unchanged in 46 (74%) and worse in 2 (3%). In 2 patients with deterioration in bowel function stool frequency and fecal soilage increased in conjunction with antibiotic usage. No patient had chronic fecal incontinence. CONCLUSIONS: In select patients with neurogenic bladder dysfunction ileal cecal bladder augmentation with a continent tapered ileal limb results in a 100% incidence of urinary continence with minimal alterations in fecal continence.
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PURPOSE: We evaluate the therapeutic effectiveness of treating monosymptomatic primary nocturnal enuresis (PNE) that has persisted into adulthood. MATERIALS AND METHODS: Patients older than 18 years with persistent monosymptomatic primary nocturnal enuresis were treated with 20 to 40 microg. desmopressin (DDAVP) nightly for 6 months. If the patients remained incontinent on maximal pharmacotherapy or if they became incontinent after cessation of DDAVP we initiated treatment with an enuretic alarm for 6 months. Patients not responsive to DDAVP or the enuresis alarm were given a trial of 50 mg. imipramine nightly. All patients were reassessed for continence 18 months after initiation of the treatment protocol. RESULTS: We treated 29 patients of a median age of 20 years (range 18 to 33) who were enuretic more than 4 nights per week. With the initial DDAVP treatment 19 (66%) became continent (enuresis 0 or 1 night a month) but after discontinuation of DDAVP only 2 (7%) remained continent. Of the 27 patients subsequently treated with an enuretic alarm 9 (33%) became continent and 18 had persistent enuresis. Of these 18 patients 11 resumed DDAVP and became dry, while 7 nonresponsive to DDAVP were given imipramine and 2 (29%) are continent. CONCLUSIONS: Overall, 83% of patients (24 of 29) achieved continence, including 38% (11 of 29) who are continent off all treatment modalities and 45% (13 of 29) who are currently continent on pharmacotherapy (11 on DDAVP and 2 on imipramine). The remaining 17% of patients (5 of 29) have persistent primary nocturnal enuresis recalcitrant to all therapeutic attempts.
PURPOSE: We determined whether there is a difference in the incidence of urinary continence in cloacal and classic bladder exstrophy after staged bladder neck reconstruction using the Young-Dees-Leadbetter technique. MATERIALS AND METHODS: We reviewed the records of patients with cloacal and classic bladder exstrophy who underwent staged bladder neck reconstruction from 1971 to 1997. RESULTS: The Young-Dees-Leadbetter bladder neck reconstruction was completed in 23 patients with cloacal exstrophy, of whom 5 (22%) became continent and 18 (78%) have persistent incontinence. A clinically apparent neurological abnormality significantly hindered the achievement of continence. Specifically 1 of the 13 children (7%) with versus 4 of the 10 (40%) without a neurological abnormality became continent (p <0.05). In contrast, staged reconstruction of classic bladder exstrophy resulted in urinary continence in 67 of the 82 patients (82%). Of the 67 continent patients 23 (34%) cannot void and require intermittent catheterization. None of the patients with classic exstrophy had a neurological deficit. CONCLUSIONS: The ability of the Young-Dees-Leadbetter bladder neck reconstruction to result in urinary continence significantly differs in the cloacal and classic bladder exstrophy populations (22 versus 82%, p <0.001). Our findings also suggest that a coexisting neurological abnormality significantly hinders the ability to reconstruct a functional bladder in patients with cloacal exstrophy.
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PURPOSE: Artificial erections are induced at hypospadias repair to prevent recurrent chordee. We describe the development and etiology of late onset recurrent chordee greater than 10 years after the initial surgery. MATERIALS AND METHODS: We reviewed the medical records of 22 patients with a median age of 4 years at surgery who were referred for evaluation of chordee 10 years after successful hypospadias repair. RESULTS: Of the 22 patients with recurrent chordee 13 had penoscrotal and 9 had proximal penile hypospadias at the initial operation. All originally required corporoplasty for the release of chordee, including Nesbit procedure in 19 and tunica vaginalis graft in 3. Successful artificial erections were induced after corporoplasty in all cases. Urethral reconstruction was performed with full-thickness preputial free grafts in 11 cases, bladder mucosal grafts in 7 and transverse island flap urethroplasty in 4. Although chordee developed during puberty (median age of onset 16 years, range 12 to 18), the median age at presentation for surgical correction was 21 years. Recurrent chordee was due to extensive fibrosis of the reconstructed urethra in 7 cases (32%), corporeal disproportion in 8 (36%) and both conditions in 7 (32%). CONCLUSIONS: A successful artificial erection induced at hypospadias surgery does not prevent the delayed onset of recurrent chordee. Recurrent chordee may be secondary to the redevelopment of corporeal disproportion and/or extensive urethral fibrosis.
PURPOSE: Testicular microlithiasis, a rare ultrasonographic diagnosis in children, has been shown to coexist in benign and malignant conditions. The natural history of incidentally discovered testicular microlithiasis has not been well defined in the pediatric population. The concern that testicular microlithiasis may be a premalignant condition has been raised. Reports indicate as much as a 45% association of germ cell tumors with testicular microlithiasis at the time of tumor diagnosis and there have been 4 cases of interval testicular tumor development associated with preexisting testicular microlithiasis. To address this issue we performed a multi-institutional study to evaluate children with incidentally diagnosed testicular microlithiasis. MATERIALS AND METHODS: Data on 26 patients with a mean age of 12.3 years at presentation with incidentally discovered testicular microlithiasis were collected from 7 institutions. Presenting scrotal conditions were reviewed. Two children with a previous testicular malignancy were excluded from study. RESULTS: Followup ranged from 1 month to 7 years (mean 27.6 months). Testicular biopsy and tumor marker (alpha-fetoprotein and beta-human chorionic gonadotropin) determinations were performed in 9 and 15 patients, respectively. To date no testicular tumor has developed during clinical followup. CONCLUSIONS: Our multi-institutional study has not yet shown a trend toward the malignant degeneration of incidentally diagnosed testicular microlithiasis in children. However, we still advocate close surveillance of patients with testicular microlithiasis, such as yearly testicular ultrasound, physical examination, and judicious tumor marker determinations. We propose that a registry be started to follow prospectively patients with testicular microlithiasis to define its significance better.
PURPOSE: We determine whether testosterone therapy alters penile androgen receptor expression within the hypogonadotropic hypogonadal (HPG) micropenis. MATERIALS AND METHODS: In protocol 1 a strain of mice with micropenis due to congenital HPG and wild type litter mates were divided into testosterone treated and untreated groups. Treatment was initiated on day 15 of life. On day 90 of life (day 75 of treatment) the mice were sacrificed, and the penises were removed and weighed. In protocol 2 microphallic mice treated with testosterone were sacrificed on days 0, 6, 30, 60 and 75 after the initiation of treatment. Untreated HPG and wild type litter mates served as controls. At autopsy the penis was removed and weighed, and androgen receptor content was determined by Western immunoblotting. RESULTS: Testosterone treatment resulted in 6-fold up regulation in HPG penile androgen receptor, approximately 1.4-fold higher than in the normal wild type pubescent penis. Testosterone induced and wild type pubescent penile androgen receptor up regulation was maintained for approximately 75 and 60 days, respectively. Despite improved HPG penile androgen receptor expression penile growth did not become normal. Average total penile weight plus or minus standard deviation was 7.2+/-3.5 mg. in untreated HPG mice. Testosterone significantly improved average HPG penile weight to 23.5+/-1.8 mg. (p <0.001). However, the testosterone treated micropenis failed to reach average normal penile size (38.6+/-2.6 mg., p <0.001). CONCLUSIONS: Testosterone increases the concentration and duration of penile androgen receptor expression within the HPG micropenis. Despite this improvement microphallic HPG penile growth does not become normal.
PURPOSE: The recommendation to treat micropenis with androgen early in infancy and childhood is based on the fact that the normal penile androgen receptor decreases in concentration 2 to 3-fold in early adulthood. Hypothetically the early administration of androgen takes advantage of elevated penile androgen receptor concentration, resulting in optimal penile growth. We verify that there are similar patterns of androgen receptor expression in micropenis and the normal penis. MATERIALS AND METHODS: We used a strain of mice with micropenis due to congenital hypogonadotropic hypogonadism (HPG). Affected mice and normal controls were sacrificed before puberty, and during puberty and early and late adulthood (15, 30, 60 and 90 days, respectively). At autopsy the penis was removed, weighed, pooled and processed for protein extraction. Androgen receptor concentration was determined by Western blot analysis. RESULTS: In controls penile androgen receptor expression increased 2-fold from before puberty (0.49 fmol./microg. protein) to puberty (0.92 fmol./microg.). At adulthood a 3-fold decrease in penile androgen receptor occurred with the receptor decreasing to 0.33 fmol./microg. in early adulthood and 0.27 fmol./microg. in late adulthood. In HPG animals the penile androgen receptor concentration increased throughout development from 0.20 fmol./microg. before puberty 0.33 fmol./microg. in adulthood. CONCLUSIONS: Androgen receptor expression in the microphallic HPG penis does not mimic normal developmental penile androgen receptor expression. It remains to be elucidated whether hormonal therapy reverses these deficiencies.
OBJECTIVES: Intestinal metaplasia often coexists with adenocarcinoma of the urinary bladder, suggesting to some investigators that it is premalignant. However, the natural history and long-term outcome of intestinal metaplasia in isolation are unknown. We report 53 cases of intestinal metaplasia of the urinary bladder followed for more than 10 years. METHODS: We reviewed the Mayo Clinic surgical pathology files between 1926 and 1996 and all patients with exstrophic bladder recorded in the files of the Hospital for Sick Children (Toronto, Ontario, Canada) and Dallas Children's Hospital (Dallas, Texas) between 1953 and 1987, and identified all patients with intestinal metaplasia of the bladder. RESULTS: A total of 53 cases were identified from both series, and none of the patients developed adenocarcinoma of the bladder. The Mayo Clinic series consisted of 24 patients. Nineteen of the 24 (79.1%) were alive without evidence of cancer (median follow-up 14 years, range 0.9 to 53), and 5 patients died of intercurrent disease (at 0.9, 4, 8, 11, and 53 years after diagnosis) without evidence of bladder cancer. The Dallas Children's Hospital and the Hospital for Sick Children series consisted of 29 patients. Twenty-seven of the 29 (93.1%) were alive without evidence of cancer (median follow-up 13 years, range 3 to 23.9). Two patients died of trauma (at 10.9 and 12 years after diagnosis) and at autopsy had no evidence of bladder cancer. CONCLUSIONS: Intestinal metaplasia of the urinary bladder is not a strong risk factor for adenocarcinoma or urothelial cancer.
PURPOSE: In infants with posterior urethral valves in whom renal function fails to normalize following decompression of the lower urinary tract supravesical urinary diversion is customarily recommended for presumed concomitant ureterovesical junction obstruction. We determined the true incidence of fixed or permanent ureterovesical junction obstruction and the renal prognosis for infants treated with proximal urinary diversion. MATERIALS AND METHODS: We evaluated 26 patients with posterior urethral valves treated with supravesical urinary diversion. Mean gestational age at birth was 35 weeks (range 27 to 40). After initial decompression via an indwelling catheter for a median of 7 days (range 4 to 18) persistently high serum creatinine was present (median 2.5 mg./dl., range 1.9 to 3.5). One month after proximal urinary diversion median creatinine was 1.3 mg./dl. (range 0.5 to 2.8). At 1 year, median nadir creatinine was 1.0 mg./dl. (range 0.3 to 2.5). At reconstruction a Whitaker test in all 26 patients (52 renal units) demonstrated fixed ureterovesical junction obstruction in 2 units (4%). RESULTS: Renal biopsy in 44 of the 52 renal units (85%) revealed renal dysplasia. At a median followup of 9 years (range 1 to 14) end stage renal disease developed in 11 patients (42%). CONCLUSIONS: In neonates with posterior urethral valves who undergo proximal urinary diversion fixed ureterovesical junction obstruction is rare, renal biopsy invariably demonstrates areas of renal dysplasia and end stage renal disease frequently develops despite proximal diversion. These findings lead us to question the necessity of supravesical urinary diversion.
PURPOSE: Duplicated upper pole systems associated with a ureterocele frequently have areas of segmental renal dysplasia. Since dysplasia has been related to the development of renin mediated hypertension, we hypothesized that preservation of functional upper pole moieties may result in an increased incidence of high blood pressure. MATERIALS AND METHODS: We evaluated 115 patients with a history of renal duplication and an ipsilateral upper pole ureterocele. Patients were separated into group 1-nonfunctional upper pole managed by partial nephrectomy, group 2-functional upper pole moiety managed by partial nephrectomy and group 3-functional upper pole moiety managed by a nephron sparing procedure. All charts were reviewed for hypertension, febrile urinary tract infection, vesicoureteral reflux and renal scarring. RESULTS: At a median followup age of 15 years (range 1 to 33) hypertension developed in 13 of the 115 patients (11%), including 8 of the 87 (9%) with nonfunctional upper pole systems managed by partial nephrectomy, 1 of the 12 (8%) with a functional upper pole moiety managed by partial nephrectomy and 4 of the 16 (25%) with a functional upper pole system managed by an upper pole salvage procedure. Statistical evaluations failed to reveal any relationship between hypertension and preservation of the upper pole system or between hypertension and vesicoureteral reflux. Rather, elevation in blood pressure was found to be related to development of a renal scar after a febrile urinary tract infection. CONCLUSIONS: Hypertension in patients with a history of renal duplication associated with a ureterocele is directly related to renal scarring.
Symptomatic bladder dysfunction occurs at some time in most patients with multiple sclerosis. The relapsing-remitting course and progressive loss of mobility associated with multiple sclerosis make management of urinary urgency and incontinence difficult. Urodynamic evaluation serves as a guideline for appropriate treatment. After accurate diagnosis of bladder dysfunction, a management program is developed with use of fluid schedules, voiding techniques, neuropharmacologic manipulation, intermittent catheterization, surgical treatment, and other adjunctive measures as indicated. The goals of treatment are to protect and preserve renal function, relieve symptomatic voiding dysfunction, and avoid subsequent urinary complications. A management program should be individualized, dynamic, and monitored with periodic, systematic urologic review to maintain these goals.
PURPOSE: Infected enlarged prostatic utricles and infected remnant fistula tracts of high imperforate anus are usually managed by a suprapubic, transtrigonal or posterior sagittal approach. We describe a minimally invasive endoscopic approach to these entities. MATERIALS AND METHODS: We treated 12 patients with infected enlarged prostatic utricles and 4 with infected remnant fistula tracts using endoscopic techniques. Specifically a resectoscope with a bulb electrode or a cystoscope with a Bugby electrode was used to fulgurate circumferentially the dilated utricle or remnant fistula. After fulguration a Councill catheter was placed in the lesion for 3 to 5 days and urine was diverted via a suprapubic tube for 2 to 3 weeks. Obliteration of the abnormality was verified by a voiding cystourethrogram. RESULTS: Using this technique median postoperative hospital stay was 2 days (range 0 to 7). The enlarged prostatic utricle or remnant fistula tract was completely obliterated in 87% of the cases (62% after 1 and 25% after 2 treatments). Of our patients 13% had a significant (greater than 50%) decrease in utricular cyst size although a urethral abnormality persisted. Postoperative morbidity was minimal. One patient (6%) had a fever for 3 days postoperatively and none has had a urethral stricture during a median followup of 2 years (range 3 months to 4 years). CONCLUSIONS: Although it is not a panacea, electrofulguration of an enlarged prostatic utricle and/or remnant fistula of imperforate anus is a simple procedure that has a high rate of success, does not require prolonged hospitalization and is associated with minimal morbidity.
BACKGROUND AND OBJECTIVE: Urethral hemangiomas are rare. They vary in size from pinpoint masses to extensive honeycomb-shape deformities leading to significant hematuria. For extensive lesions, therapeutic options have included extensive surgical resection and reconstruction or multistaged neodymium:yttrium-aluminum-garnet (Nd:YAG) laser photocoagulation. We report our experience with the use of potassium titanyl phosphate (KTP/532) laser for treatment of the extensive form. STUDY DESIGN/MATERIALS AND METHODS: A 7-year-old boy presented with a 2-week history of urethral bleeding. He had extensive hemangiomas of the genital and perineal regions. Cystourethroscopy disclosed diffusely scattered honeycomb-shape hemangiomatous malformation of the anterior urethra. KTP/532 laser energy was delivered transurethrally to the hemangiomatous areas until they blanched. RESULTS: The Foley catheter was removed 24 hours postoperatively, and the patient voided clear urine without difficulty. He has remained trouble-free for more than 2 years. CONCLUSION: Judicious endoscopic single-stage therapy with KTP/532 laser may obviate open surgical intervention in most cases of extensive and symptomatic urethral hemangiomas. In view of our observation and the literature, KTP/532 laser therapy should be considered the first line of treatment.