Clinical results with anterior diagonal iliac osteotomy in bladder exstrophy.

PURPOSE: We report our clinical experience with anterior diagonal iliac osteotomy in 10 patients who underwent surgery for bladder exstrophy. Technique and long-term results are discussed. MATERIALS AND METHODS: A total of 10 boys 1 month to 9 years old with bladder exstrophy underwent this procedure during a 2-year period. RESULTS: None of the patients had bladder closure dehiscence or prolapse after the operation. There were neither infectious complications nor injury to the vessels or nerves in any case. Blood loss was minimal for anterior diagonal iliac osteotomy. The only significant complication in our series was the polypropylene erosion of the urethra, necessitating endoscopic removal in 1 patient 1 month postoperatively. All patients had wide diastasis of the pubis preoperatively (average pubic distance 53.3 cm., average pubic ratio 0.9). At surgery suturing the symphysis after bilateral osteotomy resulted in a satisfactory symphyseal approximation and tension-free closure of the abdominal wall was easily achieved in all cases. Radiological studies at a mean followup of 34.6 months (range 14.8 to 49.5) revealed significant recurrent diastasis of the pubic bones in all but 1 patient in whom bone grafts were applied between the iliac fragments. Mean interpubic distance was 42 cm. and mean pubic ratio was 0.6 at long-term followup. CONCLUSIONS: Diagonal osteotomy may correct the principal bony deformity in exstrophy and enables initial symphyseal approximation. Pubic diastasis may recur, probably due to opening forces generated by soft tissue elements of the pelvis.

Safer circumcision in patients with haemophilia: the use of fibrin glue for local haemostasis.

OBJECTIVE: To evaluate the efficacy and the reduced costs of factor concentrates in circumcision by using fibrin glue in patients with haemophilia. PATIENTS AND METHODS: Eleven patients with haemophilia (age range 6-14 years, 10 with haemophilia A, one with haemophilia B) were circumcised using fibrin glue for local haemostasis and to reduce the duration of clotting factor replacement after surgery. Circumcision was carried out under general anaesthesia; the prepuce was incised circumferentially and excised using the Gomco clamp technique. Haemophiliac patients were divided into two groups: in group 1 (four patients, three with haemophilia A and one with haemophilia B) the factor levels were assessed every 8 h and bolus injections of factor repeated during the first 4 days after surgery; in group 2, the seven remaining haemophilia A patients received a postoperative bolus injection and approximately 4 U/kg per hour of factor substitution for the first 2 days after surgery by continuous infusion. Eleven other patients with haemophilia A underwent circumcision using same surgical procedure but were given only factor substitution without fibrin glue, and served as a control group (group 3). RESULTS: None of the patients had significant bleeding or complications. The total costs were significantly reduced, to $8898 per patient in group 1 and $4866 per patient in group 2, when compared with $12875 per patient in group 3 (both P<0.05). CONCLUSION: Fibrin glue is a useful treatment for circumcision in patients with haemophilia; it lessens the need for factor substitution after circumcision and thus reduces the high cost of treatment.

Is resolution of vesicoureteric reflux by conservative management predictable in patients with myelodysplasia?

OBJECTIVE: To evaluate the probable factors that might predict the outcome of conservative management of vesicoureteric reflux (VUR) in myelodysplastic patients. PATIENTS AND METHODS: A retrospective review of 24 children with VUR secondary to neurogenic bladder (15 girls and 9 boys, age range 1-18 years) out of 75 myelodysplasia patients between 1994 and 1996 was made. Patients were grouped according to their response to conservative management: Group I: patients with their VUR resolved or downgraded (n = 15), and group II: patients with their VUR unchanged or increased (n = 9). The following parameters were compared between the two groups: age, sex, VUR grade and laterality, urodynamic parameters (bladder capacity, compliance, leak point pressure), type of bladder neuropathy, accompanying neuropathology (walking problem, anal incontinence). RESULTS: Most of the parameters studied failed to predict the outcome of conservative management of VUR in patients with neurogenic bladder dysfunction. Higher grades of VUR if bilateral seem to benefit more from conservative management than lower grades do. Conservative management appears to be more effective in hyperreflexic bladders than areflexic bladders in terms of VUR resolution. CONCLUSION: Although prediction of patients resistant to conservative management of VUR could save myelodysplastic children from prolonged risk of renal damage, current methods of evaluation are of very little help in this aspect.

The place of Mitrofanoff neourethra in the repair of exstrophy-epispadias complex.

The authors present their experience with seven patients having an exstrophy-epispadias complex who had undergone Young-Dees-Leadbetter bladder neck reconstruction with an adjuvant Mitrofanoff neourethra. All the patients, but one, were male with a mean age of 7.4 +/- 3.9 years. Six of them had had previous primary closure of their exstrophied bladders accompanied by anterior diagonal iliac osteostomies, and one having pure epispadias had an incompetent bladder neck and a very low bladder capacity. Four of these patients had breakdown of their reconstructed bladder necks due to clean intermittent catheterization (CIC) via urethra. Ileocystoplasties were performed in all of the patients for their unacceptably low bladder capacities (mean 20.9 +/- 12.9 ml) with an adjuvant Mitrofanoff neourethra. The patients were put on CIC every 3-4 hours via Mitrofanoff channel. Six were totally continent, and one had to use a pad during daytime. There were no complications related to the Mitrofanoff stoma except two patients complained about temporary difficulty on catheterization. The authors conclude that incorporating a Mitrofanoff stoma to bladder neck reconstruction procedures in exstrophy-epispadias complex, prevents injury to the bladder neck during CIC, and thus lowers the risk and incidence of failure.

The effect of suturing technique and material on complication rate following hypospadias repair.

Ninety-seven children with distal hypospadias were treated surgically using perimeatal-based flap urethroplasty (Mathieu procedure) in a two and a half years period. A review of the medical records revealed two distinct groups of patients according to the suturing type and suture material. In the first group of 36 patients (group I), neourethra was constructed using 6/0 polyglactine (Vicryl) in a single layer, full-thickness, uninterrupted fashion. Skin flaps were approximated using interrupted simple 5/0 polyglactine (Vicryl) sutures. In the second group of 61 patients (group II), 7/0 polydioxanone (PDS) was used in the urethral anastomosis performed in a subcuticular, uninterrupted fashion. The skin flaps were closed using interrupted simple 5/0 rapidly absorbable polyglactine (Rapid Vicryl) sutures. Patients were followed-up from 6 to 12 months. Urethral or meatal stenosis was not observed in any patient. There was no infectious complication. Urethrocutaneous fistula rate was significantly higher in group I (16.6%) compared to group II (4.9%) (p < 0.01). Complication rate following hypospadias repair can be reduced by the use of a subcutaneous suture technique utilizing polydioxanone suture material in urethroplasties.

Safer and much cheaper circumcision using fibrin glue in severe haemophilia.

Circumcision is a traditional ceremony for Muslims and Jewish people. In order to reduce the high cost of factor concentrates, we administered locally manufactured heat-treated fibrin glue during operation. Circumcision can be performed safely and is much cheaper using fibrin glue plus a small amount of factor concentrates.

Evaluation of the lower urinary tract function in caudal duplication (dipygus) anomaly.

Caudal duplication is a rare anomaly with less than 30 reported cases. For those patients who also have double bladders, there are not enough data regarding the function of the lower urinary tract. A boy with caudal duplication anomaly was evaluated fluoroscopically and urodynamically. The results of the evaluation showed that the bladders were filling and emptying synchronously with normal and almost identical detrusor pressures. The child did not require either or both bladders to be resected. Because the level of the duplication in dipygus cases varies, thorough evaluation of the lower urinary tract, including urodynamics, should be considered for every case.

Posterior urethral injuries in children.

OBJECTIVE: To review the results of the operative treatment of posterior urethral injuries in children. PATIENTS AND METHODS: A total of 29 children (25 boys and two girls, age range 3-14 years) with injuries to the posterior urethra were admitted to this department over 14 years. Twenty-three patients presented immediately after trauma and six were referred after unsuccessful attempt(s) at surgical repair. RESULTS: Fourteen patients underwent suprapubic diversion and primary realignment over a catheter. Urethral continuity with normal urinary continence was achieved in seven of these patients. Four patients underwent a re-operation; urethral reconstruction was successful in these patients, but one patient remained incontinent. Primary realignment with anastomosis was performed in nine patients; the results were satisfactory in six. Urethral stricture developed in all of four patients who were managed with a suprapubic cystostomy alone; a staged repair using the transpubic approach was carried out in two of them and one improved. Partial urethral tears in two patients healed with urethral catheterization alone. CONCLUSION: Primary realignment of the urethra with anastomosis and suprapubic diversion resulted in the highest rate of success for normal urethral continuity. Urethral strictures or urinary incontinence were not major problems in this group. Therefore, we recommend this approach for the initial management of urethral injuries in childhood. Transpubic urethroplasties may be reserved for secondary repair.

Serum creatine kinase enzyme levels in the early diagnosis of spermatic cord torsion.

Delay in the diagnosis of spermatic cord torsion (SCT) is still a significant cause of testicular loss in children. The aim of this experimental study was to assess the diagnostic value of serum creatine kinase (CK) in the early period following SCT. Forty male rats were assigned randomly into five similar groups: group A, control; group B, sham, right testis exposed, manipulated, and blood sampling at 6th h; group C, right SCT, blood sampling at 2nd h; group D, right SCT, blood sampling at 4th h; and group E, right SCT, blood sampling at 6th h. Ck and its isoenzymes were measured in the sera of all animals. All testes were removed and examined histopathologically. Significant increases in serum CK levels compared to control and sham groups were observed at 4 and 6 h following SCT. The major increase in CK was observed in the CK-MM isoenzyme fraction. Histologic pictures showed varying degrees of edema, vascular congestion, and hemorrhage in the testicular tissue, but no necrosis in any of the study groups. These results showed that serum CK levels in rats in the early period following SCT increase significantly before necrosis of testicular tissue. This may be of value as a diagnostic test, to corroborate findings from clinical studies.

Microcolon-intestinal hypoperistalsis without megacystis: uncommon form of neonatal intestinal pseudoobstruction.

Two cases of microcolon-intestinal hypoperistalsis without megacystis are reported. They had dilated proximal small bowel and narrowed distal small bowel and malrotated microcolon. No organic obstructive intestinal lesion was found and double-barrel ileostomy was performed. The biopsy specimens showed ganglion cells to be normal in number and appearance in the entire intestinal wall. The ileostomy did not function postoperatively and drugs stimulating bowel movement failed to induce peristalsis. We have suggested that microcolon-intestinal hypoperistalsis without megacystis may be the cause of functional intestinal obstruction in neonates and it is a variant of megacystis-microcolon-hypoperistalsis syndrome.

Correction of vesico-ureteric reflux by subureteric fibrin injection in dogs.

OBJECTIVE: To study the histological and radiological potential of the biomaterial fibrin in the treatment of vesico-ureteric reflux by a subureteric injection. MATERIALS AND METHODS: For histological studies the bladders of 22 non-refluxing adult female dogs were exposed and in 20 of these two-component fibrin glue was injected into the right subureteric intramuscular portion of the bladder and teflon was injected into the lamina propria of the left ureter; two of them acted as controls. Two dogs were killed on alternate days over the course of 21 post-operative days and the bladders and distal portions of the ureters were examined histologically. For radiological studies micturating cystograms were obtained from 15 female puppies. In only eight puppies was vesico-ureteric reflux naturally present. In six of these two-component fibrin was injected into the right and teflon into the left subureteric areas, and in two puppies fibrin was injected into the subureteric areas, while the left sides acted as controls. Micturating cystograms and intravenous urograms were obtained from all puppies 3 weeks post-operatively. RESULTS: Histopathological studies showed persistence of increased amounts of endogenous collagen at the fibrin-injected sites; at the Teflon-injected sites a mass surrounded by dense granulation tissue was seen. Radiological studies revealed complete disappearance of reflux in the study group of six fibrin and Teflon-injected puppies; in two control puppies fibrin was injected into the right subureteric area only; the left sides were left untouched to show that maturation did not play a role in abolishing the spontaneously occurring reflux. Follow-up intravenous urography showed no evidence of vesico-ureteric obstruction in the treated ureters. CONCLUSION: Fibrin has the properties of a substance inducing a controlled increase in the number of endogenous collagen fibres.

Polyorchidism: a case report and review of the literature.

We report on a case of polyorchidism associated with left-sided inguinal hernia and hydrocele. Biopsies of both testes revealed normal histological pattern. After the hernia repair and hydrocelectomy both testes were left at their original places in the scrotum and fixed. Polyorchidism and its management are discussed briefly.

Superior vesical fistula: a case report.

Variants of bladder exstrophy are rare developmental abnormalities. Only a few cases have been reported in the literature to date. A case of superior vesical fistula with split symphysis and dorsal chordee is presented. If associated anomalies are absent, diagnosis and management of these lesions are easy and have a good prognosis. The patient is usually continent after surgical correction.