Fabio Ferro

Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases

BackgroundUterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised.ObjectiveTo highlight the imaging diagnostic clues in this rare condition.Materials and methodsWe report on 11 adolescents with this condition.ResultsSonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents.ConclusionEarly and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.

Prenatal diagnosis and clinical outcome of ovarian cysts

Technical refinements of ultrasound (US) have greatly affected the antenatal diagnosis and treatment of ovarian cysts. From 1985 to 1990 25 consecutive fetuses with ovarian cysts were followed-up by US both during pregnancy and postnatally. All cases were diagnosed between the 28th and 39th weeks of gestation. Deliveries were all at term; cesarean section was required only for obstetric complications. Eight fetuses (32%) showed US patterns of cyst torsion, a finding confirmed at surgery in all. In five patients US patterns suggested complications postnatally that were also confirmed at operation. In six cases cysts increased or remained unchanged in size after 15 days of life: in 50% of these surgery showed ovarian torsion. In the remaining six cases spontaneous resolution occurred within 1 to 4 months. One patient required intrauterine needle aspiration. There were two cases of intestinal obstruction. To date, more than 60% of newborns with ovarian cysts require oophorectomy; however, different treatments (cystectomy, needle aspiration, uncapping) combined with a close US follow-up are likely to reduce this percentage.

Maternal exposures to endocrine disrupting chemicals and hypospadias in offspring

BACKGROUND Prenatal exposures to endocrine-disrupting chemicals (EDCs) are suspected risk factors in the etiology of hypospadias. The aim of this case-control study was to test the hypothesis of an association between maternal environmental exposures to EDCs and hypospadias in the offspring. METHODS Detailed questionnaire data on occupational and dietary exposures to EDCs in the perinatal period were collected from 80 mothers with hypospadiac infants and from 80 mothers with healthy controls within 24 months of childbirth. Maternal exposure to selected EDCs was also ascertained by measuring the concentration of dichlorodiphenyldichloroethylene, hexachlorobenzene, and several polychlorinated biphenyl congeners in the serum of primiparous mothers of 37 cases and 21 controls. RESULTS The risk to bear an hypospadiac infant was associated with perinatal maternal occupational exposures to EDCs evaluated by a job-exposure matrix: jobs with exposure to one class of EDCs (odds ratios [OR](crude), 2.83; 95% confidence intervals [CI], 1.32-6.07; OR(adjusted), 2.44; 95% CI, 1.06-5.61) and jobs with exposure to more than one group of EDCs (OR(crude), 4.27; 95% CI, 1.43-12.78; OR(adjusted), 4.11; 95%CI, 1.34-12.59). Increase in risk was also found among mothers consuming a diet rich in fish or shellfish (OR(crude), 3.41; 95% CI, 1.42-8.23; OR(adjusted), 2.73; 95%CI, 1.09-6.82). Serum hexachlorobenzene concentration above the median of all subjects was significantly associated with the risk of hypospadias (OR(adjusted), 5.50; 95% CI, 1.24-24.31). CONCLUSIONS This study, although based on a limited number of cases, for the first time provides evidence of an association between maternal exposure to EDCs, in particular elevated plasma hexachlorobenzene concentration, and the development of hypospadias in the offspring.

ADOLESCENT VARICOCELE: TAUBER ANTEGRADE SCLEROTHERAPY VERSUS PALOMO REPAIR

PURPOSE There is general agreement on treatment for varicocele in pediatric patients. Randomized prospective studies have shown that anatomical and functional lesions may be corrected. Due to the impossibility of seminal examination patients with moderate to large varicocele or ipsilateral testicular hypertrophy, characterized by a change in testicular consistency or symptoms, should undergo surgical correction. The best therapeutic approach is still under discussion. MATERIALS AND METHODS At 2 centers 2 therapeutic approaches to varicocele treatment in pediatric patients were compared, namely the Palomo repair and antegrade sclerotherapy according to Tauber. The 89 patients from the same geographical area elected 1 procedure after an explanation. From the medical records we retrospectively evaluated operative time, postoperative analgesics, postoperative fever onset, complications, convalescence, recurrence and postoperative hydrocele. RESULTS After Palomo repair in 45 patients there were 2 recurrences (4.4%) and 2 postoperative hydroceles (4.4%). Of 44 antegrade sclerotherapy cases 1 was converted to Palomo repair, there was no hydrocele formation and recurrence developed in 2 (4.5%). Testicular atrophy was not observed in any patient regardless of the method used. The cost of the procedure was lower in the sclerotherapy group. CONCLUSIONS These data suggest that the failure rate was similar in both groups. The principal advantages of sclerotherapy are simplicity, decreased cost and lack of hydrocele formation.

Abdominoscrotal hydrocele: a reliable surgical technique

Abdominoscrotal hydrocele (ASH) consists of two large sacs, both abdominal and scrotal, connecting with the inguinal channel. The diagnosis is made only by ultrasound scan. Surgical treatment is mandatory since no spontaneous resolution has been reported. A new surgical procedure used successfully to treat 11 patients with ASH is described.

Benefits and Afterthoughts of Laparoscopy for the Nonpalpable Testis

PURPOSE Recent reports in the literature indicate that laparoscopy tends to be seen as the most appropriate approach to the nonpalpable testis for diagnosis and therapy. The aim of our study was to evaluate the real benefits of laparoscopy in terms of diagnostic accuracy, safety, costs and validity of the chosen treatment. MATERIALS AND METHODS We compared anatomical findings and results of the treatment of impalpable testes in 2 pediatric surgical groups, including 47 children treated laparoscopically during a 2 1/2-year period and 296 treated with open surgery (classic orchiopexy) in a 6 1/2-year period. RESULTS There was no significant difference in the diagnosis of abdominal testes (51 versus 50%), whereas a difference was noted in inguinal (4 versus 15%) and absent testes (45 versus 35%). Differences in treatment were more striking. In the laparoscopic group standard orchiopexy was performed in 62.5% of cases versus 83% in the open group. Conversely the rate of Fowler-Stephens repairs increased from 5.5% of open surgery cases to 37.5% of laparoscopic cases. There have been no serious complications in the laparoscopic procedures. To date 6 of the 9 patients who underwent a staged Fowler-Stephens procedure have undergone complete repair (open second stage). A review of the literature revealed a similar but lower tendency to over perform the Fowler-Stephens operation in laparoscopic cases (34%) versus open surgery (8%). Also, in previous series there was a 29% orchiectomy rate during laparoscopy compared to only 5% in classic open surgery. In Italy under current public health programs overall costs of the laparoscopic approach to the nonpalpable testis become noncompetitive when the procedure is extended from only diagnostic to interventional use due to the need for additional trocars and other special instruments. In contrast, private health insurers provide an additional 30% for laparoscopic cases over the cost of open orchiopexy. CONCLUSIONS Laparoscopy is definitively accurate in establishing the differential diagnosis of impalpable testis. The number of Fowler-Stephens repairs in the laparoscopic group seems inordinately high, probably due to a lack of definite standards in the proper assessment of the length of the internal spermatic vessel pedicle and the potential scrotal displacement of the testis. This reason may explain the higher number of orchiectomies reported in the literature.

IS PREOPERATIVE LAPAROSCOPY USEFUL FOR IMPALPABLE TESTIS

PURPOSE Laparoscopic assessment of pelvic anatomy has gained wide popularity over the years. Today surgical treatment of impalpable testis is nearly always preceded by diagnostic laparoscopy. The actual role of such a procedure remains undefined. We performed a prospective randomized clinical trial in patients with impalpable testis to evaluate the clinical usefulness of laparoscopy before surgical exploration. MATERIALS AND METHODS We studied pediatric, age matched patients with impalpable testis who were randomized to group 1-30 who underwent open surgery only and group 2-31 who underwent laparoscopy and open surgery. Anatomical findings, operative procedures, operative time and cost, number of recurrences and testicular volume at followup were then compared in the 2 groups. RESULTS There were no statistically significant differences in the 2 groups for any of the considered parameters except operative cost and time, which were significantly higher in the laparoscopy group. CONCLUSIONS Preoperative laparoscopy does not provide any significant advantage over open surgery for treating impalpable testis.

Unilateral cryptorchidism corrected in prepubertal age: Evaluation of sperm parameters, hormones, and antisperm antibodies in adult age

OBJECTIVE To evaluate whether prepubertal orchidopexy for unilateral cryptorchidism can reduce adult dysspermia. DESIGN Patient follow-up comparison with control groups. SETTING Surgical and endocrinologic sections of a childrens hospital and a university infertility-care center. PATIENT(S) Seventy-one patients with unilateral cryptorchidism who underwent orchidopexy in prepubertal age (6.4 +/- 2.8 years) were followed up as adults (20.0 +/- 2.8 years). MAIN OUTCOME MEASURE(S) Patients underwent testicular examination and hormonal evaluation, 49 of these had semen analysis and antisperm antibody tests. Semen results were compared with those of two age-matched control groups: a group of 20 healthy, randomly selected subjects and a group of 20 patients operated on in postpubertal age for cryptorchidism. RESULT(S) Unilateral reduced testis size was found in 30.1% of patients, eight patients had a low LH level, eight had a low T level, and none had abnormal FSH values. Antisperm antibodies were found in 1 of 49 cases. Cluster analysis of sperm parameters showed that the mean values of patients were worse than those of the healthy controls but better than those of the subjects operated on in postpubertal age. CONCLUSION(S) This study indicates that prepubertal orchidopexy can given better results than postpubertal correction.

The gliding testis: minor degree of true undescended testis?

The gliding testis is located below the external ring; it can be manipulated to the upper scrotum but tends to ascend to its original position. Histologic changes can be detected in these gonads by 7 years of age. We evaluated 427 consecutive prepubertal boys referred for cryptorchidism. One hundred and twenty-three had classical undescended testes: 71 ectopic, 55 retractile, and 178 (mean age 6 yrs. 2 mos.) gliding testes. The gliding testes were smaller than controlaterals in 24% of boys. All gliding testes were unilateral, whereas bilaterality was 85% in the retractile group (P<0.0001), 17.5% in the undescended (P<0.001), and 10% in the ectopic group (P<0.01). There was a history of one or more of the following conditions: orchidopexy (3), hormonal treatment (5) late testicular descent (9), spermatic cord torsion (5), testicular pain (10), actual retractile testes (20) or actual gliding testis (58), was present in 93 (52.2%) of the fathers of the gliding group. Forty-seven (81%) paternal gliding testes were hypotrophic. Seventy-five boys with gliding testis underwent initial hormonal therapy with transient benefit, and 57 were operated on. Two anatomical findings are typical of the gliding testis: (i) the absence of the gubernaculum, and (ii), a processus vaginalis partially patent from the upper scrotum to the mid groin area. This latter feature explains the mobility of the gliding testis from the external ring to the upper scrotum. The absence of the gubernaculum may be responsible for a higher incidence of spermatic cord torsion in this population. The gliding testis is a distinct entity, representing the mildest degree of a true undescended testis. As hormonal treatment gives only transient results, orchidopexy should be considered before testicular damage occurs.

Impact of primary surgical approach in the management of the impalpable testis.

There is no univoque opinion about the place of preoperative studies in non-palpable testes. During a 6.5-year period, we operated on 296 impalpable testes in prepubertal boys. A combined inguinal-abdominal approach was used in all cases verifying the eventual abdominal testis and its exact vascular anatomy before any manipulation of the cord was undertaken. Forty-five testes (15.2%) were canalicular, 142 (48%) were abdominal, 5 (1.7%) dysgenetic and 104 (34.1%) absent (agenesis or vanishing testis). Of the abdominal testes, 122 underwent a standard orchidopexy in dartos pouch, 11 a staged repair, 8 a Fowler-Stephens operation and 1 orchiectomy. All means of investigation for impalpable testes are either unreliable, too expensive or too invasive for routine use, and in most cases, a surgical exploration has to be performed anyway. The primary surgical approach has the most favorable cost/benefit ratio, being diagnostic and therapeutic at one time. Provided the exploration is performed correctly, all the advantages of previous laparoscopy can be achieved with surgery alone.