Duplication of the male urethra with posterior urethral valves

The only known case of complete duplication of the male urethra associated with posterior urethral valves is reported. The embryological implications of this association are discussed.     

Bilateral high loop ureterostomy in the primary management of posterior urethral valves in a developing country

 Of 200 consecutive cases of posterior urethral valves (PUV) managed in a single institution, 26 underwent bilateral high loop ureterostomy (BU) as their first operative intervention. Indications for BU were persistently raised serum creatinine levels after bladder drainage, severe urosepsis, and urinary ascites, especially in a neonate or infant. After a median period of 1 year of diversion, the serum creatinine fell from a median value of 2.5 to 0.6 mg%. Twelve cases have been fully undiverted without deleterious effects (median serum creatinine level 0.5 mg%). Three patients died. Of the 14 renal units with documented persistent vesicoureteric reflux at the time of undiversion, only 1 has been reimplanted. One child has undergone bladder augmentation. Accepted: 11 January 2000     

Role of vesicostomy in the management of posterior urethral valve in Sub-Saharan Africa

ObjectiveTo review the role of vesicostomy in the management of posterior urethral valve (PUV), in neonates and infants, given the limitations for endoscopic treatment in this setting.MethodsA review of 35 patients who presented with posterior urethral valve over a 10-year period. Demographic and clinical information were prospectively recorded on a structured pro forma, and the data extracted analysed using SPSS 11.0.ResultsThe 35 boys were aged 3 days to 10 years (median 3 weeks). Twenty-three (65.7%) had a vesicostomy (age range 3 days–3 years, median 3 weeks). The mode of presentation was poor urinary stream 15 (65.2%), urinary retention 4 (17.4%), and renal failure 6 (26.1%). Main findings were palpable bladder 23 (100%), hydronephrosis 4 (17.4%). Abdominal ultrasound confirmed hydronephrosis and thickened bladder wall, and voiding/expressive cystourethrogram confirmed dilated posterior urethra and vesicoureteric reflux in all 23 patients. Complications following vesicostomy were stoma stenosis 1 (4.3%), bladder mucosal prolapse 1 (4.3%), perivesicostomy abscess 1 (4.3%); there was no mortality. Following vesicostomy, 10 (43.5%) patients had excision of the valves and vesicostomy closure at age 2–8 years (median 4 years). They are well, with normal renal ultrasonographic findings, bladder capacity range 115–280 ml, and normal urea, serum electrolytes, creatinine, at 3 years of follow up. Thirteen (56.5%) are still awaiting valvotomy but have remained well and with normal ultrasonographic renal findings.ConclusionVesicostomy is a useful temporising mode of urinary diversion in neonates and infants with posterior urethral valve (in the absence of unobstructed upper tracts) when facilities for endoscopic valve ablation are not readily available.     

Evolution of upper urinary tract and renal function in patients with posterior urethral valves

From January 1972 to June 1993, 166 patients with posterior urethral valves (PUV) were treated in our surgical department, 59 with a milder form of PUV (upper urinary tract [UUT] complication rate 29%) and 107 with a severer form (UUT complication rate 96.3%). Only the latter group was studied for long-term (mean 9.3 years) evaluation of the UUT and renal function. A temporary vesicostomy was the primary treatment in 25 patients. Indications for temporary diversion were very young age and/or low birth weight, severe and bilateral UUT complications, and severe renal damage. All the other patients were treated by primary endoscopic valve fulguration. After removal of the lower urinary tract obstruction, vesicorenal reflux (VRR) resolved spontaneously or was ameliorated in 59.2% of the renal units. Spontaneous normalization or evident amelioration were found at long-term follow-up in nearly 70% of dilated, non-refluxing ureters. Ureteral reimplantation was performed on 41 of the 202 dilated or refluxing ureters (surgical rate 20.3%). The surgical failure rate requiring reoperation was 5% (2/41). The prerequisite for successful reimplantation was a large-capacity, stable, and compliant bladder. Ten nephroureterectomies were carried out for unilateral, massive VRR and renal dysplasia; 1 late nephrectomy was performed for arterial hypertension. The evolution of renal function showed statistically significant overall improvement, which was more evident in patients diagnosed and treated in the 1st month of life (P = 0.000) than in those treated between 1 and 12 months (P = 0.004) or after 1 year of age (P = 0.025). Renal function considerably improved in the vesicostomy group (P = 0.000). Thirteen patients (12.4%) are now either dead (2) or have end-stage renal disease (6) or chronic renal insufficiency evolving toward end-stage renal disease (5); 5 of these 13 were treated by vesicostomy in the first days or months of life, and at presentation the glomerular filtration rate (GFR) was less than 25 ml/min . 1.73 m². Determination of basic GFR and, even more, functional renal reserve is relevant in predicting the long-term evolution. In the author's opinion, vesicostomy is the procedure of choice in very ill newborns or infants. Aggressive management with early surgical reconstruction is rarely justified, because frequently UUT complications resolve spontaneously or clearly improve, and their surgical treatment has limited and very precise indications.     

Effect of Botox injection at the bladder neck in boys with bladder dysfunction after valve ablation

ObjectiveAfter posterior urethral valve ablation, some boys are still have a hostile bladder. We conducted a prospective randomized study to determine if Botox injection at the bladder neck will help improve vesical dysfunction in this subgroup of boys or not.Patients and methodsTwenty boys with history of posterior urethral valve ablation and severe bladder dysfunction with a mean age of 16 months were studied. Cases were further randomized into two groups. Group I (study group) had endoscopic injection of a single dose of 100 IU of Botox into the hypertrophied bladder neck at 3, 6, and 9 o’clock. Group II (control group) patients with the same parameters had urethroscopy to exclude residual valves. Both groups had the standard conservative treatment. Cases were followed after 6 months of initiating the management protocol. This includes laboratory studies (urine culture and sensitivity, blood urea nitrogen, serum creatinine), ultrasound of the urinary tract. Voiding cysto-urethrogram and urodynamic study (pressure flow study).ResultsThere was no statistical difference in both groups regarding rate of urinary tract infection, improvement of hydronephrosis, resolution of vesico-ureteral reflux, creatinine level at the start or at the end of the study. Urodynamic parameters revealed an increase in cystometric capacity in both groups at the end of the study but without statistical difference. The mean voiding pressure reduced significantly in both groups but without statistical difference.ConclusionsTemporarily abolishing the effect of bladder neck by Botox injection does not seem to improve the outcome of those boys who had a severe voiding dysfunction after valve ablation.     

Our experience,technique and long-term outcomes in the management of posterior urethral strictures

PurposeTo share our experience, technique and long-term outcomes in posterior urethral stricture management.Materials and methodsThirty-seven boys with post-traumatic posterior urethral stricture underwent resection and end-to-end anastomosis through pre-anal coronal approach or in combination with trans-pubic approach from January 2000 to December 2011. Follow up included symptomatic evaluation by micturating cystourethrogram and retrograde urethrogram in all patients, and urethroscopy in patients with voiding symptoms.ResultsPre-anal coronal approach was used in 29 (78%) cases and in 8 (21%) cases it was combined with trans-pubic approach. In 33 (89.1%) patients it was first attempt, while in 4 (10.9%) it was redo surgery. Two patients required buccal mucosal graft to bridge the deficient urethra. Patient age was 5–17 years (mean 10.8 years). Mean follow up was 48.5 months (range 6–132 months). Thirty-two (86%) patients were symptom free. Failed repairs were successfully managed by urethral dilation in 3 and by redo urethroplasty in the remaining 2. All patients were continent. There was no chordee, penile shortening or urethral diverticula.ConclusionsResection and end-to-end anastomosis of posterior urethral stricture is possible through pre-anal coronal incision; however, if slightest difficulty is envisaged in creating a satisfactory end-to-end anastomosis, extension to trans-pubic approach should be done.     

Infection with Ureaplasma urealyticum and Mycoplasma hominis and the development of chronic lung disease in preterm infants

In a prospective cohort study in a tertiary referral neonatal intensive care unit, the endotracheal secretions of 40 consecutively intubated newborn infants, less than 31 weeks' gestation. were examined weekly for the genital mycoplasmas and all other common bacterial pathogens. Fifteen (37%) infants were positive for Ureaplasma urealyticum and/or Mycoplasma hominis. There were no differences in gestation, birthweight, use of surfactant, or time on ventilator between the culture-positive and negative babies. Thirteen (87%) of the culture-positive group developed chronic lung disease (CLD) compared with 11 (41%) of the negative group ( p = 0.0196). Of those culture-positive, 37% were not identified on the first specimen taken at the time of admission. These data suggest a significant association between infection with the urogenital mycoplasmas and CLD and also stress the need for repeated cultures to identify these organisms.     

The effect of dexamethasone on growth, mineral balance and bone mineralisation in preterm infants with chronic lung disease

The aim of this prospective longitudinal study was to observe the effects of treatment with dexamethasone on somatic growth, mineral balance and bone mineralisation in very low birth weight (VLBW) preterm infants with chronic lung disease (CLD). Dexamethasone was started at a dose of 500 μg/kg body weight per day for 3 days followed by gradually decreasing doses for a total period of 3 weeks' treatment if the infant was still mechanically ventilated in the 3rd postnatal week and had signs of CLD on the chest radiograph. Eleven infants with CLD treated with dexamethasone were studied. Administration of dexamethasone treatment was associated with significantly (P < 0.05) lower weight velocity, head circumference velocity, lower leg length (measured by the neonatal knemometer) velocity compared to pre-treatment changes. The start of dexamethasone treatment was also associated with a fall in calcium absorption (61% to 41.7%, P < 0.05) calcium retention (60.8% to 40.6%, P < 0.05) and phosphate retention (65% to 39.6%, P < 0.05); phosphate absorption was not significantly affected (88.8% to 92%, P > 0.05). Somatic growth and mineral balance improved during the immediate post-treatment period. Acute disturbances of bone mineral content (measured by dual energy radiographic densitometry), plasma calcium and phosphate were also seen but not reaching statistical significance at the P < 0.05 level. Conclusion The start of steroids is associated with a rapid and significant fall in growth velocity, calcium absorption and calcium and phosphate retention in infants with chronic lung disease with recovery occurring after completion of steroid treatment. Received: 15 February 1999 / Accepted: 26 September 1999     

Role of haematological,pulmonary and renal complications in the long-term prognosis of patients with lysinuric protein intolerance

Three patients with lysinuric protein intolerance are reported. The first patient displayed severe haemolytic anaemia, bone marrow erythroblastophagocytosis, renal tubular disease and interstitial lung disease. Despite treatment with citrulline and low-protein diet, this child died at the age of 18 months. The second patient is now 24 years old and has chronic interstitial lung disease and focal renal glomerulosclerosis. The third patient, now 5 years old, has severe chronic interstitial lung disease. A 6-month treatment with prednisone was ineffective in the second and third patients.