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Erectile dysfunction (ED) is a syndrome associated with endothelial dysfunction, which may predict cardiovascular events in men presenting with this syndrome. It has been shown to be associated with a higher rate of acute myocardial infarction and cardiovascular mortality, vascular inflammation, and impaired endothelial function. In this review we present the literature findings and describe the mechanistic pathways that are known to be involved in this syndrome and its related clinical consequences.

Objectives To investigate the long-term changes in health-related quality of life (HRQOL), continence, and sexual function after curative therapy (CT) and watchful waiting (WW) for prostate cancer. Methods A self-reported questionnaire of HRQOL and continence was administered to patients with prostate cancer who were candidates for CT and WW. The questionnaire was re-administered during follow-up. The pretreatment and posttreatment HRQOL burden scores were compared and correlated with the treatment, continence, and sexual function. Results Of the 61 patients, 41 chose CT (26 chose prostatectomy and 15 radiotherapy). The average follow-up for the CT patients was 24.3 ± 13.3 months. The pretreatment incontinence score was 0.38 ± 0.9 and the HRQOL burden score was 16.3 ± 8.8 (CT versus WW, P = 0.55). On follow-up, 23 (56%) of 41 CT patients were continent. The HRQOL burden score after treatment was similar between the CT and WW patients (20.4 ± 9.6 versus 18.4 ± 9.0, P = 0.45). The continent patients had a significantly lower HRQOL burden compared with the post-CT incontinent patients (17.41 ± 7.4 versus 24.2 ± 10.8, P = 0.02). The HRQOL burden score after treatment for CT patients who maintained sexual activity and CT patients who lost their sexual activity was similar ( P = 0.28). Conclusions The HRQOL after CT of prostate cancer was related to patients’ self-perception of continence. Incontinence, either urinary or fecal, was associated with an increased self-reported HRQOL burden, although reduced sexual function was not associated with such a change. The WW patients maintain their HRQOL. Separating the continent and incontinent patients during the analysis of the posttreatment HRQOL brought to light the adverse outcomes associated with post-CT incontinence.

OBJECTIVE: To assess the ability of postoperative bladder washing cytology, performed immediately after transurethral resection of mostly stage Ta or T1 papillary urothelial carcinoma, to predict early recurrence. STUDY DESIGN: In a 1-year period, preoperative and postoperative bladder washing cytology specimens were sampled from patients undergoing transurethral re-sections in which all visible tumor was removed. There were 38 resections in 32 patients. RESULTS: Postoperative cytology was satisfactory in 35 of 38 cases and positive in 17 (49%) after a mean of 6.9 months. Follow-up of these 35 transurethral resections disclosed a 15/17 (88%) recurrence rate after positive cytology and a 4/18 (22%) recurrence rate after negative cytology (P

We evaluated the results of a treatment protocol that consisted of neoadjuvant hormonal therapy followed by radical retropubic prostatectomy (RRP) for clinical stage T3 (cT3) prostate cancer. Sixty-six patients with cT3 prostate cancer underwent staging procedures that included metastatic work-up and evaluation under anesthesia. Neoadjuvant hormonal treatment was given for 3 to 6 months, followed by re-evaluation under anesthesia. Patients considered to have a resectable prostate following neoadjuvant treatment underwent operation. Disease-free survival [prostate-specific antigen (PSA) < 0.1 ng/ml] was calculated by the Kaplan-Meier method for cT3 patients and for a group of patients with clinical stage 1 and 2 disease (cT1-2), who underwent RRP without neoadjuvant hormonal therapy. Patients with pT3 disease in both groups received early adjuvant radiation treatment. Patients in the cT3 group who were not clinically downstaged were treated with radiation. Patients with positive lymph nodes continued hormonal therapy. The pretreatment PSA for the cT3 group was 43.6 ± 55.6 ng/ml, and 10.64 ± 7.18 ng/ml for the cT1-2 group (p < 0.05). Of 66 cT3 patients, 53 (80%) were clinically downstaged and operated on and 47 (71%) underwent RRP. At 36 months, there was no significant difference in the PSA relapse rate between these two groups; both had significantly lower rates than did patients in the cT3 without RRP. At 48 months, 79% (21 patients) of the patients in the cT3-RRP group were disease free compared with 50% of those in the cT3 without RRP (4 patients). Neoadjuvant hormonal therapy for stage cT3 prostate cancer selects patients whose cancer can be controlled by RRP for an extended period of time.

We determined the preferred treatment of staghorn calculi.Between January 1992 and December 1994 we performed a prospective, randomized, single center study involving 50 kidneys with complete staghorn calculi: 27 renal units were treated with extracorporeal shock wave lithotripsy (ESWL) monotherapy (group 1) and 23 were treated with combined (initial) percutaneous nephrostolithotomy with ESWL (group 2). The 2 treatment groups were compared regarding stone size, grade of collecting system dilatation and urine culture at presentation. The number of treatment sessions, narcotic doses, renal colic episodes, septic complications, unplanned ancillary procedures, length of hospitalization, total treatment duration and stone-free rate at 6 months were recorded and compared.At the conclusion of therapy the stone-free rate was significantly greater in group 2 than in group 1 (74 versus 22%, respectively, p = 0.0005). The complication rate was significantly greater in group 1, with 15 septic complications (fever greater than 38.5C for longer than 3 days) in 10 patients compared to only 2 episodes in group 2 (p = 0.007). The unplanned ancillary procedure rate was significantly greater in group 1 (8 procedures in 7 patients versus 1 procedure in group 2, p = 0.03). The overall treatment length was significantly shorter in group 2 (1 versus 6 months, p = 0.0006). There was no significant difference in the number of procedures performed with anesthesia or in the number of hospitalization days between the 2 treatment groups.Combined percutaneous nephrostolithotomy and ESWL should be recommended as the first line treatment choice for most patients with staghorn stones.

Cystinuria is an autosomal recessive disorder of amino acid transport. It is a common hereditary cause of kidney stones worldwide, and is associated with significant morbidity. In 17 affected families, we found linkage between cystinuria and three chromosome 2p markers. Maximal two-point lod scores between cystinuria and D2S119, D2S391 and D2S288 were 8.23 (theta = 0.07), 3.73 (theta = 0.15) and 3.03 (theta = 0.12), respectively. Analysis of recombinants and multipoint linkage data indicated that the most likely order is cen-D2S391-D2S119-cystinuria-D2S177-tel. We also observed high rates of homozygosity for markers in this chromosomal region among 11 affected offspring of consanguineous marriages. Based on its map position and function, the recently cloned SLC3A1 amino acid transporter gene is a primary candidate gene for this disease.

We used a whole canine ureter, under constant inflow hydrostatic pressure, in a tissue bath, to investigate the effect of lidocaine on flow and contractility in the ureter, simultaneously. Lidocaine caused increased contractility and reduction of flow in 9 ureters tested. In 5/9 ureters exposed to lidocaine in concentration of 50 to 500 μg/mL in the bath, the contraction became continuous and the flow ceased. This could be reversed by irrigating the bath with fresh solution. We conclude that lidocaine causes increased contractility and reduction of flow in canine ureters in vitro.

Purpose: We determined whether the thin ureter of the young child transports stone fragments after extracorporeal shockwave lithotripsy (ESWL) as efficiently as the adult ureter does. This determination was done by comparing the outcome after lithotripsy of renal stones greater than 10 mm. between young children and adults.Materials and Methods: Our study group consisted of 38 children 6 months to 6 years old (median 3 years) with renal stones greater than 10 mm. in diameter. This group was further divided into 3 subgroups according to the longest stone diameter on plain abdominal film. There were 21 children with a renal stone diameter of 10 to 15 mm. (subgroup 1), 8, 16 to 20 mm. (subgroup 2) and 9 greater than 20 mm. (subgroup 3). The control group consisted of 38 adults older than 20 years randomly selected from the local ESWL registry. Each adult was matched with a child regarding stone diameter and localization. The control group was similarly divided into subgroups 1a, 2a and 3a. ESWL was performed...

The sophistication of percutaneous nephrolithotomy and ureteroscopy challenges the efficacy of ESWL for urolithiasis in prepubertal patients. We evaluated our long-term experience with ESWL in these patients and determined its efficiency.We retrospectively reviewed the charts of all prepubertal patients who underwent ESWL. We evaluated the need for tubing, the 3-month stone-free rate, the need for additional ESWL, and the effect of stone size and location, and cystinuria on the 3-month stone-free rate.Between 1986 and 2008, 119 males and 97 females with a mean age of 6.6 years who had urolithiasis underwent ESWL using the Dornier HM3 lithotriptor. We treated 157 children with renal calculi with an average +/- SD diameter of 14.9 +/- 8.9 mm, of whom 66 (42%) required a tube in the urinary system. The 3-month stone-free rate was 80% and 31 patients (19.7%) needed an additional procedure. Stone location did not affect the stone-free rate but stone size did. We treated 59 patients for ureteral stones with an average stone length of 9.5 +/- 4.8 mm, of whom 41 (69%) required tube insertion. The 3-month stone-free rate was 78% and 13 patients (22%) needed an additional procedure. The 3-month stone-free rate did not depend on stone location or size. The rate was 37.5% in patients with cystinuria and 82.5% in all others (p0.0001). Six patients (2.8%) had complications.The 3-month stone-free rate after ESWL in prepubertal patients is 80% and 20% of patients require additional procedures. ESWL is most effective for kidney stones less than 11 mm. ESWL has inferior results for cystine stones compared to other calculi. Complications are rare.

We treated 12 patients with sixteen renal and one ureteral cystine stones primarily with extracorporeal shock-wave lithotripsy (ESWL). Among the stones thirteen were greater than 25 mm. In four stones less than 25 mm, three stones completely disappeared and 1 was reduced to small fragments, following ESWL. In thirteen stones greater than 25 mm, twelve were treated by ESWL initially, and one by surgery. Of the 12 cases treated initially by ESWL, 4 became stone-free, 4 remained with small fragments, and 1 remained with large fragments. Two patients were operated on because of poor response to ESWL and 1 patient lost kidney function because of prolonged obstruction. Disintegration of cystine stones greater than 25 mm required an average of 8,522 shock-waves in 4.33 sessions. The problems associated with application of ESWL monotherapy to cystine stones are presented.

Four patients underwent extracorporeal shock wave lithotripsy (ESWL) for large stones in the kidney resulting in disappearance of the radiopaque stones from the plain film. However, contrast study revealed that the collecting system was occupied by radiolucent filling defects, which were found to be composed of stone matrix material. All four patients had a history of urinary tract infection and deranged function of the kidney prior to the treatment. We suggest that the possibility of persistence of matrix material in the kidney after ESWL be taken into account when selecting the best way to treat similar patients.

To assess the ability of postoperative bladder washing cytology, performed immediately after transurethral resection of mostly stage Ta or T1 papillary urothelial carcinoma, to predict early recurrence.In a 1-year period, preoperative and postoperative bladder washing cytology specimens were sampled from patients undergoing transurethral resections in which all visible tumor was removed. There were 38 resections in 32 patients.Postoperative cytology was satisfactory in 35 of 38 cases and positive in 17 (49%) after a mean of 6.9 months. Follow-up of these 35 transurethral resections disclosed a 15/17 (88%) recurrence rate after positive cytology and a 4/18 (22%) recurrence rate after negative cytology (P.001). Postoperative cytology demonstrated a sensitivity for recurrence of 79%, specificity of 88%, positive predictive value of 88% and negative predictive value of 77%. In contrast, tumor in the transurethral resection specimen had a positive predictive value of 54% for recurrence, and its grade and stage were inferior to cytology in predicting recurrence.Postoperative bladder washing cytology is a useful adjunct to the management of papillary urothelial carcinoma. A positive result, signifying residual tumor, should encourage prompt follow-up and possibly repeat transurethral resection.

Urethrocutaneous fistula is the most common (2% to 10%) complication of hypospadias surgery. The correction of such fistula is associated with a 10% to 40% failure rate. The key measure to ensure a successful repair is separation of the suture lines in the urethra and skin, using well vascularized elastic tissue. If the dartos fascia is unavailable and local penile skin is fibrotic as a result of previous operations, a tunica vaginalis flap may be considered. We report our experience with tunica vaginalis flap as an adjunct to fistula repair.We used tunica vaginalis flap for the repair of recurrent urethrocutaneous fistulas in 14 children with a mean age of 7.6 years (range 3 to 15). All patients had undergone previous hypospadias repairs and previous attempts to close the fistula had failed. The mean number of fistulas per patient was 1.6 (range 1 to 4), and the locations were perineal (1), penoscrotal (3), midshaft (10), and subcoronal (8). The mean number of failed previous closures with local penile skin flaps was 2.4 (range 1 to 5). Surgery was initiated by injecting povidone solution via the urethral meatus to identify all fistulas. Calibration or cystoscopy excluded distal urethral strictures. Surgery was performed using a microscope and fistulas were closed primarily in 12 patients and with an onlay island flap in 2. The urethral suture line was covered with a flap of tunica vaginalis, which was harvested through a small scrotal incision and mobilized via a subcutaneous tunnel into the penis. The testis was then fixed to the scrotum. A urethral stent with or without suprapubic catheter drainage provided urinary diversion for 2 to 7 days.The repair was successful in all patients. During a mean followup of 44 months (range 8 to 60) there was no evidence of recurrent fistulas or urethral strictures. Penile cosmesis was excellent, and all parents reported a straight penis when erected. No postoperative complications were encountered in the testicles.Repair of recurrent urethrocutaneous fistulas with a tunica vaginalis flap is highly effective regardless of fistula location. This flap is easy to mobilize and provides excellent coverage of the urethral suture line. It is a simple procedure with no complications to the testicles.

Malignant mesothelioma of the tunica vaginalis testis is a rare tumor that usually presents as a benign hydrocele but may give the clinical impression of testicular tumor.1 Cases in the world literature total 80.1, 2 The only established risk factor is asbestos exposure, which was present in 34% of the patients whose history was reported.1 Of 62 patients with followup 33 (53%) had recurrence and 24 (39%) died. We report the twenty-fifth fatal case of this tumor and the third to metastasize to the liver.3

We report on an 18-year-old woman with a combined urogynecological malformation who presented with anuria. At the age of 8 years she presented elsewhere with urometrocolpos secondary to a left hypoplastic kidney and an ectopic ureter that inserted into the vagina. A retroiliac right ureter also was identified. She underwent surgical treatment and was reported in the urological literature. At menarche she began to have severe dysmenorrhea. After multiple hospitalizations elsewhere without diagnosis she presented to our emergency room with anuria. The anuria was the direct result of additional, previously unidentified malformations, consisting of uterus duplex bicollis and vaginal hypoplasia. We emphasize the importance of identifying all components of the genital and urological systems whenever anomalies are present in either system.

Objectives. To present our experience with the clinical use of a modified American Urological Association Symptom Score (AUASS) and a quality-of-life (QOL) questionnaire in obtaining clinically available information and to investigate whether a relatively short questionnaire, administered in the clinic, can help in identifying symptoms and QOL issues associated with various types of definitive treatments (DTs) for prostate cancer (PCa) and its impact on patients’ QOL. Methods. The AUASS was modified to contain questions on continence and was combined with a short QOL questionnaire. It was administered to control groups and all patients with PCa during their clinic visit. The responses were analyzed and correlated with the DT of PCa. Results. The questionnaire was administered 846 times to 375 patients with PCa and to 170 controls during clinic visits. We found that patients on watchful waiting had symptom scores and QOL responses similar to controls and that DT was associated with worse scores and a reduced QOL. DT with continence was associated with a better QOL than DT with incontinence. Incontinence of either urine or stool, among DT patients, had similar adverse impact on QOL, compared with continent DT patients. Conclusions. The questionnaire, administered in a clinical setting, provides insight into the QOL and symptoms associated with interventions aimed at curing PCa. The use of such a questionnaire enhances the objective documentation of treatment outcomes of PCa and benefits the patients by increasing their opportunities to express their problems.

Purpose The recent advancements in ureteroscopy, with reported success rate between 77% and 100%, have challenged the efficacy of ESWL monotherapy for ureterolithiasis in prepubertal children (PC). We, therefore, evaluated our long-term experience with ESWL in these patients, and determined its efficiency. Material and Methods We conducted a retrospective chart evaluation, and retrieved all PC who underwent ESWL for ureteral stones. The need for tubing, 3 month stone free rate (3SFR), the need for additional ESWL sessions, and the effect of stone size and location were evaluated. Results Fifty nine patients (32 males, 27 females; mean age 7.3 years, range 10 months to 12 years) underwent in-situ ESWL by the unmodified Dornier HM3 lithotriptor for ureteral calculi between 1986 and 2008. All patients required general anesthesia for their treatment. Average stone length was 9.5 mm (S.D. 4.8 mm). In 41 children (69%) a tube was inserted prior or during the procedure; A nephrostomy, a double pigtail ureteral stent, or an externalized ureteral catheter were inserted in 6 (14%), 9 (22%), and 23 (63%) patients respectively. All ureteral catheters were removed at the end of the procedure. 3SFR was 78%, and 13 patients (22%) required an additional procedure with subsequent total 96.5% and 98.3% SFR after a 2nd and a 3rd ESWL. SFR was not dependent on patients’ age, stone location (proximal, mid or distal), or stone size. Conclusions The 3SFR after ESWL monotherapy for ureteral stones in PC is only 78% regardless of the necessity for tubing in 69% of them. Therefore, ESWL does not have an advantage over ureteroscopy in this population. Recent reported results of ureteroscopy in PC place this modality equal, and even superior to ESWL.

Extracorporeal shock-wave lithotripsy (ESWL) has been accepted as the method of choice for most upper urinary tract calculi. However, in cases of stones in the lower ureter, ureteroscopic procedures have generally been preferred. Using the Dornier HM3 lithotriptor with modifications in the patient's position, we were able to successfully treat 155 unselected cases of lower ureteral calculi. The average stone size was 9.6 mm (range 5-23 mm). One hundred forty-three patients had stones located below the lower margin of the sacroiliac joint. These patients were placed in a supine position. The stones were visualized radiologically without use of a ureteral catheter in 78 percent of the patients; in 22 percent a ureteral catheter was inserted prior to ESWL to aid in stone localization. In 145 patients (94%) treatment was completed in one session; 10 patients (6%) required two sessions. Of the patients, 38 percent were free of stones one day after ESWL; 97 percent became stone free within three months, and only 3 patients required endoscopic manipulation, after ESWL. Twelve patients had stones in the midureter overlying the sacroileum. They were placed in the prone position, and the calculi were visualized with the aid of a ureteral catheter. All these patients became free of stones one month after treatment. There were no significant treatment-related complications except for bacteremia in 1 case. In view of the remarkable efficacy, negligible complication rate, and shorter hospital stay as compared to ureteroscopic stone manipulations, we recommend high energy ESWL as the primary monotherapy of mid and lower ureteral stones.

ContextAt least 50% of patients with a history of bladder cancer have recurrences, so rigorous surveillance is necessary. Cystoscopy is standard but can fail to detect some bladder cancers, so a urine test is frequently part of the evaluation.ObjectiveTo investigate whether a point-of-care proteomic test that measures the nuclear matrix protein NMP22 in voided urine could improve detection of recurrence during monitoring of patients with a history of bladder cancer.Design, Setting, and PatientsFrom September 2001 to February 2002, 23 academic, private practice, and hospital facilities in 9 US states prospectively enrolled 668 consecutive patients with a history of bladder cancer in this cross-sectional study. Patients provided a voided urine sample for analysis of NMP22 protein and cytology prior to cystoscopy.Main Outcome MeasuresDiagnosis of bladder cancer recurrence, based on cystoscopy with biopsy, was accepted as the reference standard. The performance of the NMP22 test was compared with voided urine cytology as an aid to detection. Testing for the NMP22 tumor marker was conducted in a blinded manner.ResultsBladder cancer was diagnosed in 103 patients. Cystoscopy alone identified 91.3% of the cancers (94/103; 95% confidence interval [CI], 84.1%-95.9%). The combination of cystoscopy with the NMP22 assay detected 99.0% of the malignancies (102/103; 95% CI, 94.7%-100%; P = .005). The NMP22 assay detected 8 of 9 cancers that were not visualized during initial cystoscopy, including 7 that were high-grade. The sensitivity and specificity of the NMP22 test alone were 49.5% (51/103; 95% CI, 39.5%-59.5%) and 87.3% (493/565; 95% CI, 84.2%-89.9%), respectively. Voided cytology detected only 3 of the malignancies missed during initial cystoscopy and did not significantly increase the sensitivity of cystoscopy (94.2%; 95% CI, 87.7%-97.8%; P = .08).ConclusionThe noninvasive point-of-care assay for elevated urinary NMP22 protein can increase the ability to detect recurrent bladder cancer, with test results available during the patient visit.

ContextA combination of methods is used for diagnosis of bladder cancer because no single procedure detects all malignancies. Urine tests are frequently part of an evaluation, but have either been nonspecific for cancer or required specialized analysis at a laboratory.ObjectiveTo investigate whether a point-of-care proteomic test that measures the nuclear matrix protein NMP22 in voided urine could enhance detection of malignancy in patients with risk factors or symptoms of bladder cancer.Design, Setting, and PatientsTwenty-three academic, private practice, and veterans’ facilities in 10 states prospectively enrolled consecutive patients from September 2001 to May 2002. Participants included 1331 patients at elevated risk for bladder cancer due to factors such as history of smoking or symptoms including hematuria and dysuria. Patients at risk for malignancy of the urinary tract provided a voided urine sample for analysis of NMP22 protein and cytology prior to cystoscopy.Main Outcome MeasuresThe diagnosis of bladder cancer, based on cystoscopy with biopsy, was accepted as the reference standard. The performance of the NMP22 test was compared with voided urine cytology as an aid to cancer detection. Testing for the NMP22 tumor marker was conducted in a blinded manner.ResultsBladder cancer was diagnosed in 79 patients. The NMP22 assay was positive in 44 of 79 patients with cancer (sensitivity, 55.7%; 95% confidence interval [CI], 44.1%-66.7%), whereas cytology test results were positive in 12 of 76 patients (sensitivity, 15.8%; 95% CI, 7.6%-24.0%). The specificity of the NMP22 assay was 85.7% (95% CI, 83.8%-87.6%) compared with 99.2% (95% CI, 98.7%-99.7%) for cytology. The proteomic marker detected 4 cancers that were not visualized during initial endoscopy, including 3 that were muscle invasive and 1 carcinoma in situ.ConclusionThe noninvasive point-of-care assay for elevated urinary NMP22 protein can increase the accuracy of cystoscopy, with test results available during the patient visit.

Mitomycin is used extensively for the prevention of recurrence of superficial bladder cancer. Most treatment regimens of mitomycin are long term, since this seems more effective in preventing recurrence. During treatment some patients develop cystitis of variable severity, which may lead to mucosal ulcerations and cessation of treatment. We report a case in which long-term treatment with mitomycin, following a single episode of transitional cell bladder cancer, has led to bladder fibrosis and loss of its function, without evidence of tumor recurrence.

Urethral strictures are difficult to cure. In 1988, a new treatment was reported, consisting of dilation or incision of the stricture followed by implantation of a specially designed stent meant to allow the urethral epithelium to heal and cover the stent while preventing shrinkage of the scar and reappearance of the stricture. Two cases are reported here in which implantation of the stent resulted in a foreign-body reaction and obstruction of the lumen of the urethra. The area obstructed was reopened by transurethral removal of the reactive tissue. The long-term effect of this reaction on the patency of the urethra is still unknown.

Extracorporeal shock wave lithotripsy monotherapy was used to treat 41 patients with complete staghorn calculi. In 20 patients polyethylene angiographic pigtail catheters were inserted prophylactically as ureteral stents and the other 21 patients did not receive stents. Prophylactic stenting reduced the incidence of complications and the need for percutaneous nephrostomy tubes to relieve subsequent ureteral obstruction. Internal stenting maintained the sterility of the urinary tract and reduced the average hospital stay by a third. Residual stone fragments representing less than 5 per cent of the original stone mass remained in 56 per cent of the patients, particularly in those with hydronephrotic kidneys. We suggest that extracorporeal shock wave lithotripsy monotherapy with prophylactic stenting is the preferred treatment for noninfected complete staghorn calculi.

Thirty-six patients have been operated on for coarctation of the aorta. The age at operation ranged from 4 to 45 years with a mean of 14 years. Preoperative systolic blood pressure (BP) was above the normal mean + 2 SD in 34 (94%) of the patients. The surgical techniques used for correction of coarctation were: resection and end-to-end anastomosis (50%), resection and graft interposition (25%), patch graft angioplasty (22%) and subclavian artery to descending aorta bypass graft (3%). There was no hospital mortality. Systolic BP measured on hospital discharge available in 30 patients showed an average decrease of 26 mm Hg. The mean follow-up period in 16 of 30 patients (53%) was 7.2 years. All of these patients had a further decrease in systolic BP, and in 14 of 16 (87%) BP was within the mean + 2 SD. Twelve patients were examined in the vascular diagnostic laboratory to determine adequacy of the repair by hemodynamic measures. After average of 7.8 years following operation, all of them had ankle systolic BP higher than brachial systolic pressure; ankle BP did not drop after treadmill walking exercise and the directional Doppler velocity curves from the legs were normal. This study suggested that BP continued to decrease after hospital discharge. We were unable to demonstrate correlation between age at operation and decrease in BP. The adequacy of surgical repair can be assessed by mild effort stress test combined with ankle/arm ratio measurement and directional Doppler velocity curves.

An infant with congenital varicella syndrome due to maternal varicella zoster is presented. Unlike other reports in which such infection caused multiple congenital defects, our patient suffers from atonic bladder and anal dysfunction as the sole manifestation of congenital varicella syndrome. Attention is called for maternal varicella as a possible cause for limited neurologic damage in the fetus.