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Persistent mullerian duct syndrome with or without transverse testicular ectopia and testis tumours
1394
INTERNATIONAL
months to 47 years) with a variety of conditions (simple curvature. chordee with and without hypospadias, epispadias, micropenis, epispadias, cripple penis, etc). Erection occurred 2 to 10 minutes after injection and in most cases lasted between 30 minutes and 4 hours. Only seven did not respond. Two patients had prolonged erections, up to 6 hours, but none had priapism. Repair was carried during the erection, which was considered to facilitate correction. The authors concluded that this is a useful alternative to the conventional erection test.-MN. de la Hunt
The Incidence of Cystic Fibrosis Gene Mutations in Patients With Congenital Bilateral Absence of the Vas Deferens in Scotland. R. Donat, A.S. McNeill, D.R. Fitzpatrick, et al. Br J Urol 79:74-77, (January). 1997. Thirry patients with congenital bilateral absence of the vas deferens (CBA\‘t)) were examined clinically and tested for 15 gene mutations using a single-tube polymerase chain reaction. All patients appeared healthy, without clinical evidence of cystic fibrosis, but cystic fibrosis transmembrane-conductance gene mutations were found in 70%. The authors advocate gene testing and genetic counseling for all men with CBAVD.-M.N. de la Hunt
A Short Vas Limiting Successful Laparoscopic scent. S. Perovic. and N. Janic. Br J Urol79:120-121.
Testicular (January),
De1997.
This is a review of 23 boys (mean age, 4.9; range, 1 to 12 years) who had undergone laparoscopic first-stage Fowler-Stephens orchidopexy between 1992 and 1996. During the second-stage procedure. a short vas was found in three boys. In two the testes lay behind the bladder, and one in the internal ring. Two could not be brought into the scrotum, and one was placed in the scrotum under tension. One testis (brought down under tension) underwent complete atrophy, one marked “hypotrophy,” and the other survived. The authors conclude that the staged FowlerStephens approach is not suitable for boys who have a very short vas deferens-M.N. de la Hunt
Spontaneous Ascent of the Testis. J. Gracia, Guirado et al. Br J Uro179: 113-l 15, (January), 1997.
E. Navarro,
F:
The authors reviewed surgically treated “cryptorchid testes,” which were previously verified to be in the scrotum by experienced staff pediatricians or staff pediatric surgeons. From 1,342 children treated for cryptorchidism over a 20-year period, 46 testes were identified (36 boys). Biopsy specimens were obtained from 35 testes. The mean time from the last normal examination to recorded ascent was 4.5 years. At exploration, 18 (70%) had an open processus vaginalis and the mean (SD) tubular fertility index was 40 (37)%. They conclude that spontaneous ascent does occur and that ascending testes have characteristics similar to other cryptorchid testes.-M.N. de la Hunt
Persistent Mullerian Duct Syndrome Associated With Transverse Testicular Ectopia: A Case Report. M. Lima, M. Domini, andM. Libri. Eur J Pediatr Surg 7:60-61. (February), 1997. The authors report on a child, male phenotype, with transverse testicular ectopia (TTE) associated with a rudimentary uterus and two fallopian tubes (persistent mullerian duct syndrome: PMDS). During a former operation for bilateral cryptorchidism, the diagnosis had become apparent. To exclude the presence of PMDS, an abdominal exploration must be performed in all cases with TTE in which the testes are undescended.-Thomas A. Angerpointner
Persistent Mullerian Duct Syndrome verse Testicular Ectopia and Testis Uro179: 122-126. (January), 1997.
ABSTRACTS
With or Without TransTumours. E Berkmen. Br J
Three patients with persistent mullerian duct syndrome (PMDS) and a testicular tumor were reported. All were evaluated with physical examination. imaging, surgical exploration, and chromosome analysis. The position of the uterus and fallopian tubes was different in all three: one in the scrotum, one in the abdomen. and one in the inguinal canal. All had a normal karyotype. Two were cousins, and their pedigree suggested a probable sex-limited group. Both also had testicular ectopia. The authors recommend radiological confirmation of the diagnosis, and detection of mullerian inhibiting factor and prophylactic orchidectomy. They do not recommend hysterectomy because there is no risk of malignancy in the retained mullerian duct structures. Biopsy of the scrotal testis was not helpful because impalpable tumors could be detected by ultrasonography.-M.N. de la Hunt The Varicocele in Pediatric Age: 207 Cases Treated With MicroSurgical Technique. M. Lima, M. Domini, and M. Libri. Eur J Pediatr Surg 7:30-33, (February), 1997. The authors report on 207 boys who underwent surgery for varicocele. All were treated employing a venous microvascular terminoterminal anastomosis between spermatic and inferior epigastric veins. The recurrence rate with this technique (3%) was much lower than with other techniques (15%). The clinical results were optimal, with disappearance of varices in the majority of cases and improvement of testicular trophism in the remaining cases. This technique enables the establishment of an immediate and physiological testicular venous outflow for testicular growth and development.-Thomas A. Angerpointner Lower Urinary Tract Changes After Early Valve Ablation in Neonates and in Infants: Is Early Diversion Warranted? C.E. Close, M.C. Carr; M. W Burns et al. J Urol 157:984-988, (March), 1997. The authors retrospectively reviewed the records of infants treated for posterior urethral valves before age 1 year during the last 8 years. Treatment consisted of primary valve ablation in 23 patients and urinary diversion in eight. Preoperative and serial postoperative voiding cystourethrograms were scored for degree of trabeculation, bladder neck hypertrophy, and prostatic urethral dilatation in all patients undergoing primary valve ablation. All patients treated with primary valve ablation had marked improvement or resolution of bladder abnormalities or voiding cystourethrography by 1 year postoperatively. Bladder compliance and volume were significantly better than in patients treated with primary diversion. Upper tract diversion failed to halt progressive renal failure in five of the six patients who underwent diversion. Similarly, primary valve ablation did not stop progressive renal failure in a matched group of patients. Early ablation of posterior urethral valves results in the recovery of normal bladder appearance and function when performed in the first months of life. Severe renal insufficiency tends to progress even with upper tract diversion. Furthermore, this treatment prevents normal bladder cycling, which may inhibit bladder recovery in the patient with posterior urethral valves.-George W Holcomb, Jr Posterior Urethral Valves: Long-Term Renal Function Consequences After Transplantation. L. Salomon, E. Fontaine, M.E Gagnadoux, et al. J Urol 157:992-995, (March), 1997. The authors retrospectively compared the outcomes of renal transplantation in 66 children with posterior urethral valves and 116 with malformation uropathies (controls). Graft survival rates for those with posterior urethral valves and the control group were 69% and 72% at 5 years and 54% and 50% at 10 years. respectively (not statistically
INTERNATIONAL
months to 47 years) with a variety of conditions (simple curvature. chordee with and without hypospadias, epispadias, micropenis, epispadias, cripple penis, etc). Erection occurred 2 to 10 minutes after injection and in most cases lasted between 30 minutes and 4 hours. Only seven did not respond. Two patients had prolonged erections, up to 6 hours, but none had priapism. Repair was carried during the erection, which was considered to facilitate correction. The authors concluded that this is a useful alternative to the conventional erection test.-MN. de la Hunt
The Incidence of Cystic Fibrosis Gene Mutations in Patients With Congenital Bilateral Absence of the Vas Deferens in Scotland. R. Donat, A.S. McNeill, D.R. Fitzpatrick, et al. Br J Urol 79:74-77, (January). 1997. Thirry patients with congenital bilateral absence of the vas deferens (CBA\‘t)) were examined clinically and tested for 15 gene mutations using a single-tube polymerase chain reaction. All patients appeared healthy, without clinical evidence of cystic fibrosis, but cystic fibrosis transmembrane-conductance gene mutations were found in 70%. The authors advocate gene testing and genetic counseling for all men with CBAVD.-M.N. de la Hunt
A Short Vas Limiting Successful Laparoscopic scent. S. Perovic. and N. Janic. Br J Urol79:120-121.
Testicular (January),
De1997.
This is a review of 23 boys (mean age, 4.9; range, 1 to 12 years) who had undergone laparoscopic first-stage Fowler-Stephens orchidopexy between 1992 and 1996. During the second-stage procedure. a short vas was found in three boys. In two the testes lay behind the bladder, and one in the internal ring. Two could not be brought into the scrotum, and one was placed in the scrotum under tension. One testis (brought down under tension) underwent complete atrophy, one marked “hypotrophy,” and the other survived. The authors conclude that the staged FowlerStephens approach is not suitable for boys who have a very short vas deferens-M.N. de la Hunt
Spontaneous Ascent of the Testis. J. Gracia, Guirado et al. Br J Uro179: 113-l 15, (January), 1997.
E. Navarro,
F:
The authors reviewed surgically treated “cryptorchid testes,” which were previously verified to be in the scrotum by experienced staff pediatricians or staff pediatric surgeons. From 1,342 children treated for cryptorchidism over a 20-year period, 46 testes were identified (36 boys). Biopsy specimens were obtained from 35 testes. The mean time from the last normal examination to recorded ascent was 4.5 years. At exploration, 18 (70%) had an open processus vaginalis and the mean (SD) tubular fertility index was 40 (37)%. They conclude that spontaneous ascent does occur and that ascending testes have characteristics similar to other cryptorchid testes.-M.N. de la Hunt
Persistent Mullerian Duct Syndrome Associated With Transverse Testicular Ectopia: A Case Report. M. Lima, M. Domini, andM. Libri. Eur J Pediatr Surg 7:60-61. (February), 1997. The authors report on a child, male phenotype, with transverse testicular ectopia (TTE) associated with a rudimentary uterus and two fallopian tubes (persistent mullerian duct syndrome: PMDS). During a former operation for bilateral cryptorchidism, the diagnosis had become apparent. To exclude the presence of PMDS, an abdominal exploration must be performed in all cases with TTE in which the testes are undescended.-Thomas A. Angerpointner
Persistent Mullerian Duct Syndrome verse Testicular Ectopia and Testis Uro179: 122-126. (January), 1997.
ABSTRACTS
With or Without TransTumours. E Berkmen. Br J
Three patients with persistent mullerian duct syndrome (PMDS) and a testicular tumor were reported. All were evaluated with physical examination. imaging, surgical exploration, and chromosome analysis. The position of the uterus and fallopian tubes was different in all three: one in the scrotum, one in the abdomen. and one in the inguinal canal. All had a normal karyotype. Two were cousins, and their pedigree suggested a probable sex-limited group. Both also had testicular ectopia. The authors recommend radiological confirmation of the diagnosis, and detection of mullerian inhibiting factor and prophylactic orchidectomy. They do not recommend hysterectomy because there is no risk of malignancy in the retained mullerian duct structures. Biopsy of the scrotal testis was not helpful because impalpable tumors could be detected by ultrasonography.-M.N. de la Hunt The Varicocele in Pediatric Age: 207 Cases Treated With MicroSurgical Technique. M. Lima, M. Domini, and M. Libri. Eur J Pediatr Surg 7:30-33, (February), 1997. The authors report on 207 boys who underwent surgery for varicocele. All were treated employing a venous microvascular terminoterminal anastomosis between spermatic and inferior epigastric veins. The recurrence rate with this technique (3%) was much lower than with other techniques (15%). The clinical results were optimal, with disappearance of varices in the majority of cases and improvement of testicular trophism in the remaining cases. This technique enables the establishment of an immediate and physiological testicular venous outflow for testicular growth and development.-Thomas A. Angerpointner Lower Urinary Tract Changes After Early Valve Ablation in Neonates and in Infants: Is Early Diversion Warranted? C.E. Close, M.C. Carr; M. W Burns et al. J Urol 157:984-988, (March), 1997. The authors retrospectively reviewed the records of infants treated for posterior urethral valves before age 1 year during the last 8 years. Treatment consisted of primary valve ablation in 23 patients and urinary diversion in eight. Preoperative and serial postoperative voiding cystourethrograms were scored for degree of trabeculation, bladder neck hypertrophy, and prostatic urethral dilatation in all patients undergoing primary valve ablation. All patients treated with primary valve ablation had marked improvement or resolution of bladder abnormalities or voiding cystourethrography by 1 year postoperatively. Bladder compliance and volume were significantly better than in patients treated with primary diversion. Upper tract diversion failed to halt progressive renal failure in five of the six patients who underwent diversion. Similarly, primary valve ablation did not stop progressive renal failure in a matched group of patients. Early ablation of posterior urethral valves results in the recovery of normal bladder appearance and function when performed in the first months of life. Severe renal insufficiency tends to progress even with upper tract diversion. Furthermore, this treatment prevents normal bladder cycling, which may inhibit bladder recovery in the patient with posterior urethral valves.-George W Holcomb, Jr Posterior Urethral Valves: Long-Term Renal Function Consequences After Transplantation. L. Salomon, E. Fontaine, M.E Gagnadoux, et al. J Urol 157:992-995, (March), 1997. The authors retrospectively compared the outcomes of renal transplantation in 66 children with posterior urethral valves and 116 with malformation uropathies (controls). Graft survival rates for those with posterior urethral valves and the control group were 69% and 72% at 5 years and 54% and 50% at 10 years. respectively (not statistically