Anogenital distance in a cohort of 169 infant boys with uni- or bilateral cryptorchidism including 18 boys with vanishing testes.

STUDY QUESTION: Do different boys with different types of cryptorchidism exhibit different anogenital distances (AGDs)? SUMMARY ANSWER: Length of AGD seemed to differ in different groups of patients with cryptorchidism. WHAT IS KNOWN ALREADY: AGD, which is used as an indicator of prenatal androgen action, tends to be shorter in boys with cryptorchidism compared to unaffected boys. Shorter AGDs have also been reported in boys with hypospadias, in men with poor semen quality, and in men with testicular cancer. STUDY DESIGN, SIZE, DURATION: A prospective descriptive cohort study was performed using data from consecutively selected boys with cryptorchidism (n = 169) operated in a single center over a period of 3 years (September 2019 to October 2022). PARTICIPANTS/MATERIALS, SETTING, METHODS: AGD was measured in 169 infant boys, at 3 to 26 months of age, during anesthesia with a vernier caliper measuring the distance from the anus to the base of the scrotum (AGDAS) and from the anus to the anterior base of the penis (AGDAP) in two body positions according to the methods by 'The Infant Development and the Environment Study' (TIDES) and 'Cambridge Baby Growth Study', resulting in four mean values per patient (TIDES AGDAS/AP and Cambridge AGDAS/AP). Normal values for AGD by age were set by our hospital Department of Growth and Reproduction based on a large cohort of healthy infant boys (n = 1940). Testicular biopsies were performed at orchidopexy as a clinical routine. The germ cell number (G/T) and type Ad spermatogonia number (AdS/T) per cross-sectional tubule of at least 100 and 250 tubules, respectively were measured and related to normal samples. Blood samples were obtained by venipuncture for measuring serum LH, FSH, and inhibin B. They were analyzed in our hospital Department of Growth and Reproduction where the normal reference was also established. Correlations between the four mean AGD measurements for each boy were evaluated by Spearman rank correlation analyses. The AGD measurement of every boy was transferred to the multiple of the median (MoM) of the normal AGD for age and named MoM AGD. MAIN RESULTS AND THE ROLE OF CHANCE: There were 104 boysoperated for unilateral, and 47 boys operated for bilateral, undescended testes, whereas 18 boys had vanished testis including one boy with bilateral vanished testes. Only 6% of cases with vanished testes had a MoM AGD higher than the normal median compared to 32% with undescended testes (P < 0.05). MoM AGD increased with the age at surgery for boys with vanished testis (Spearman r = 0.44), but not for boys with undescended testes (Spearman r = 0.14). Boys with bilateral cryptorchidism had longer AGDs and more often had hypogonadotropic hypogonadism than boys with unilateral cryptorchidism (P < 0.005) and (P < 0.000001). LIMITATIONS, REASONS FOR CAUTION: Although being the largest published material of AGD measurements of infant boys with cryptorchidism, one limitation of this study covers the quite small number of patients in the different groups, which may decrease the statistical power. Another limitation involves the sparse normal reference material on G/T and AdS/T. Finally, there are currently no longitudinal studies evaluating AGD from birth to adulthood and evaluating childhood AGD in relation to fertility outcome. Our study is hypothesis generating and therefore the interpretation of the results should be regarded as exploratory rather than reaching definite conclusions. WIDER IMPLICATIONS OF THE FINDINGS: The study findings are in agreement with literature as the total included group of boys with cryptorchidism exhibited shorter than normal AGDs. However, new insights were demonstrated. Boys with vanished testis had shorter AGDs compared to unaffected boys and to boys with undescended testes. This finding challenges the current concept of AGD being determined in 'the masculinization programming window' in Week 8 to 14 of gestation. Furthermore, boys with bilateral cryptorchidism had longer AGDs and more often had hypogonadotropic hypogonadism than boys with unilateral cryptorchidism, suggesting that the lack of fetal androgen in hypogonadotropic hypogonadism is not that significant. STUDY FUNDING/COMPETING INTEREST(S): No external funding was used and no competing interests are declared. TRIAL REGISTRATION NUMBER: The trial was not registered in an ICMJE-recognized trial registry.

Splenogonadal fusion: a case report.

BACKGROUND: Accessory splenic tissue is a commonly encountered phenomenon in medical literature. Typically, these accessory spleens are found in close proximity to the main spleen, either in the hilum or within the surrounding ligaments. Nevertheless, it is noteworthy that they can also be located in unusual sites such as the jejunum wall, mesentery, pelvis, and, exceptionally rarely, the scrotum. The first documented case of accessory splenic tissue in the scrotum was reported by Sneath in 1913 and is associated with a rare congenital anomaly called splenogonadal fusion. This report describes an infant who presented with a scrotal mass noted by his mother and after examination, investigations, and surgical exploration, it was revealed to be splenogonadal fusion. CASE DESCRIPTION: An 8-month-old Caucasian male patient presented with a mass in the left testicle and bluish discoloration of the scrotum, which had been incidentally noticed in the previous 2 months. The general physical examination was unremarkable. Other than a palpable scrotal mass that was related to the upper pole of the testis, the rest of examination was unremarkable. Imaging revealed that this mass originated from the tail of the epididymis without infiltrating the testis and tumor markers were normal. On inguinal exploration, a reddish brown 2 × 2 cm mass was found attached to the upper pole and was completely excised without causing any harm to the testis, vessels, or epididymis. Histopathological evaluation confirmed the presence of intratesticular ectopic splenic tissue. CONCLUSION: Although uncommon, splenogonadal fusion can be included in the differential diagnosis of a testicular swelling. Accurate diagnosis allows for appropriate treatment planning which helps to avoid unnecessary radical orchiectomy, which can have a significant impact on the patient's reproductive and psychological wellbeing.

Where is the testis? The role of ultrasound and diagnostic laparoscopy for Crossed Testicular Ectopia (CTE): Case report and review of literature.

Crossed Testicular Ectopia (CTE) or transverse testicular ectopia is an anecdotic urogenital anomaly in which both testes are located on the same side, generally associated with a patent processus vaginalis (PPV). The condition can be detected by ultrasound. Nevertheless, the diagnosis is often missed preoperatively and CTE is recognized intraoperatively. Controversy exists regarding management and the role of diagnostic laparoscopy. The surgical technique depends on the anatomy of vas, vessels and testis found on surgical exploration. Diagnostic laparoscopy can be useful to rule out a vanishing testis and detect Müllerian remnants. We present the case of 8-months infant with no palpable testis on the right side and no signs of inguinal hernia, reporting the management and reviewing the scarce existing literature in this regarding. KEY WORDS: Crossed Testicular Ectopia, Laparoscopy, Ectopia, Testis, Transverse Testicular Ectopia, Urogenital Abnormalities.

Transverse testicular ectopia with persistent Mullerian duct syndrome: Report and review of two cases.

Transverse testicular ectopia is a rare anomaly characterized by both testes descending through a single inguinal canal. The objective of this study was to investigate the pathogenesis, diagnosis, and treatment of transverse testicular ectopia (TTE) with persistent Mullerian duct syndrome (PMDS), and to deepen the understanding of the disease in clinical. A retrospective analysis of the clinical manifestation, diagnosis, and treatment of two children suffering from TTE with PMDS was conducted. Previous studies on the characteristics, diagnosis, and treatment of this disease were reviewed. The two patients were treated with laparoscopy-assisted transseptal orchidopexy-inguinal evaluation. After the surgery, the two patients recovered well. The follow-up visits were done 3 months after the operation. An ultrasound examination confirmed that the two patients had testes in the orthotopic position and normal size. TTE with PMDS is an exceedingly rare disease. The patients manifested cryptorchidism on one side; contralateral inguinal hernia was suspected. Detailed physical and ultrasound examinations before the operation are the key to the early diagnosis of TTE. Laparoscopic evaluation is helpful for the diagnosis and finding of other abnormalities. Surgical treatment is the only method to cure the disease; long-term follow-up is needed after TTE operation.

Scrotal orchidopexy for perineal ectopic testis.

Perineal ectopic testis (PET) is a rare form of testicular migration abnormality. We report a case of neonatal PET diagnosis and early management. In reported cases, diagnosis is often late, well after the age of 1 year, and surgery is conventionally performed by an inguinal and scrotal approach. Here, surgery consisted of scrotal orchidopexy, with placement in the dartos, at the age of 6 months. Follow-up was uncomplicated. This approach has not been reported for this form of ectopy, but seems perfectly suitable. There is no advantage to delaying the treatment of this type of ectopy.

Pattern of Male Hypogonadism and Outcome of Treatment in South Rajasthan.

Male reproductive functions are governed by hypothalamic pituitary testicular axis. If any component of this axis malfunctions, then hypogonadism will develop which is characterized by ill-defined secondary sexual features and low serum testosterone. The common patterns seen are primary and secondary testicular failure in the young; and late onset hypogonadism in the elderly. All such cases can be virilized and managed by androgen supplementation. Spermatogenesis can be induced by use of appropriate gonadotropins in selected cases. The aim of the study was to find out the pattern, management and outcome of male hypogonadism. MATERIAL: It was a prospective questionnaire based observational study, carried out on infertile hypogonadic males, attending medicine outdoor at medical college hospital from January 2015 to December 2020. There were 50 males with hypogonadic features, cases of late onset of hypogonadism were excluded. Sexual maturity rating, semen analysis, serum testosterone and FSH level were estimated in all patients at baseline and at 6 months duration of treatment. Testicular biopsy, Karyotyping and MRI brain were performed in selected cases. Azoospermic patients having hypogonadotropic hypogonadism were treated with long-acting testosterones and gonadotropins. OBSERVATION: The study subjects were infertile azoospermic males. On the basis of serum FSH and testosterone levels, they were classified into two groups. Group A (n=42) cases were hypergonadotropic hypogonadism or primary testicular failure; of these 32 were Klinefelter syndrome (XXY), 7 chronic orchitis and 3 empty scrotal syndrome including one case of anorchia. These patients also developed other systemic illnesses in addition to hypogonadism. Group B (n=8) hypogonadotropic hypogonadism or secondary testicular failure; of these 2 were Kallman syndrome and the rest were idiopathic. After testosterone replacement all patients were virilized and there was marked improvement in libido and androgenization. CONCLUSION: The study brings out that primary testicular failure is more common than secondary testicular failure. Both pattern of hypogonadism should receive lifelong androgen replacement therapy; otherwise, they will be a basket of multiple systemic disorders. Presently testosterone undecanoate once in every three months is the agent of choice.

Androgens and spermatogenesis.

Male infertility contributes to 50% of all cases of infertility. The main cause is low quality and quantity of sperm. In humans, spermatogenesis starts at the beginning of puberty and lasts lifelong. It is under the control of FSH and testicular androgens, and mainly testosterone (T), and therefore requires a normal gonadotroph axis, intratesticular T production by Leydig cells and functional androgen receptors (ARs) within testicular Sertoli cells. Various clinical cases illustrate the roles of T in human spermatogenesis. Men with complete congenital hypogonadotropic hypogonadism (HH) are usually azoospermic. Treatment by exogenous testosterone injection and FSH is not able to produce sperm. However, combined treatment with FSH and hCG is effective. This example shows that intratesticular T plays a major role in spermatogenesis. Furthermore, testicular histology of men with LH receptor mutations shows Leydig cell hypoplasia/agenesis/dysplasia with conserved Sertoli cell count. The sperm count is reduced, as in males with partial inactivating mutation of the androgen receptor. Some protocols of hormonal male contraception or exogenous androgen abuse induce negative feedback in the hypothalamic pituitary axis, decreasing FSH, LH and T levels and inducing sperm defects and testicular atrophy. The time to recovery after cessation of drug abuse is around 14 months for sperm output and 38 months for sperm motility. In summary, abnormal androgen production and/or AR signaling impairs spermatogenesis in humans. The minimal level of intratesticular T for normal sperm production is a matter of debate. Interestingly, some animal models showed that completely T-independent spermatogenesis is possible, potentially through strong FSH activation. Finally, recent data suggest important roles of prenatal life and minipuberty in adult spermatogenesis.

Epididymal disjunction anomalies in undescended testis - a factor associated with spermatic obstruction

ABSTRACT Background: To analyze the incidence of epididymal anomalies (EAs) associated to spermatic obstruction in patients with undescended testis (UT) according to testicular position and age. Materials and Methods: We studied 87 patients (110 testis) with cryptorchidism and analyzed the presence of EAs correlated with the testicular position, age and patency of the processus vaginalis (PV). To analyze the relations between the testis and epididymis we considered three situations: (a) Normal pattern: the epididymis was attached to the testis at the head and tail and epididymis totally attached to the testis; (b) EAs: when the epididymis was attached to the testis only at the head (Figure-1A) and (c) EAs associated to spermatic obstruction: epididymis was attached to the testis only at the tail (Figure-1B) and when there are no visible connection between testis and epididymis (Figure-1C). We used the Wilcoxon-Mann-Whitney test and the Chi-square test for contingency analysis (p <0.05). Results: The mean age of the patients was 5.18 years (SD=2.867). Of 110 testes analyzed, 14 were abdominal (12.72%); 83 inguinal (75.45%) and 13 suprascrotal (11.81%). Normal relationships between testis and epididymis were observed in 54 patients (62.1%) with no significant differences in relation to the patient's age (p=0.666). Epididymal tail disjunction was observed in 23 patients (26.44%), with no significant differences in relation to age (p=0.59). EAs associated to spermatic obstruction were observed in 16 patients (18.4%), also with no significant differences in relation to age (p=0.684). We did not observe significant correlation between the testis position and the incidence of EAs (p=0.119). We did not observe significant correlations between patency of the PV (64.7%) and incidence of EAs (p=0.742). Conclusions: Epididymal anomalies associated with spermatic obstruction are present in almost 20% of undescended testes, without significant correlation with age, testicular position and patency of the PV. This information needs to be correlated to the infertility risk of this congenital anomaly.

Multiple vas deferens with polyorchidism and many congenital malformations in a symptomatic 11-year-old male patient: a rare case report.

BACKGROUND: Ductus deferens may manifest in a variety of anomalies such as its absence, duplication, ectopy, or diverticulum. Ectopic seminal tract opening has two main types, ectopic ejaculatory duct opening, and ectopic vas deferens opening. Generally, ductus deferens anomalies affect approximately 0.05% of the population. Patients may be asymptomatic or complaining of urinary tract infections and/or epididymitis. Most of these cases are associated with renal dysplasia. To confirm the diagnosis Cystourethroscopy catheterization and retrograde urethrogram should be performed, but the definitive diagnosis is done by vasography. The definitive treatment is complete surgical resection of the pathological urogenital connection. This case is commonly discovered while exploring other findings such as testicular torsion and inguinal hernia. CASE PRESENTATION: We report a rare case of an 11-year-old male who presented with gross hematuria and numerous congenital malformations including a left polydactyly clubfoot, polyorchidism, with several surgical procedures, and left kidney dysgenesis. Surgery was performed for a left inguinal hernia, during which a third undescended testicle was discovered incidentally and was eradicated. A retrograde urethrogram was performed to establish the diagnosis. A fistula- that is connected with the left ureter- was resected. The histopathologic findings confirmed the diagnosis of true duplication of the Vas deferens, with communication between the ureter and the vas deferens. By follow-up, the kidney function tests were within normal limits. CONCLUSIONS: This case report aims to highlight the early diagnosis and management of the duplicated vas deferens and the associated congenital malformations to improve the prognosis and kidney function and to avoid long-term complications.

Splenogonadal fusion: aiding detection and avoiding radical orchidectomy.

Splenogonadal fusion is a rare benign congenital anomaly in which there is an abnormal connection between the gonad and the spleen. It was first described over 100 years ago with limited reports in the literature since then. Its similarity in presentation to testicular neoplasia poses a significant challenge in diagnosis and management, often resulting in radical orchidectomy. We present the case of a 31-year-old man who presented with a rapidly growing left-sided testicular mass and suspicious ultrasound findings; histology from the subsequent radical inguinal orchidectomy showed findings consistent with splenogonadal fusion. We describe points for consideration in the clinical history, examination and imaging that could suggest splenogonadal fusion, including preoperative technetium-99m-sulfur colloid imaging and intraoperative frozen section evaluation, which may confirm the diagnosis and prevent unnecessary orchidectomy.

Crossed Testicular Ectopia: Report of Two Cases in Children of Consanguineous Parents.

Crossed testicular ectopia (CTE) is an extremely rare anomaly of urogenital development. The etiopathogenic mechanism is unknown. Medical records of two biological siblings with a confirmed diagnosis of CTE being managed at our center were collected. The first patient was born with a non-palpable left testis. An exploratory laparoscopy was performed and a CTE was found. The second patient had an incarcerated left inguinal hernia and a mesopenile hypospadias. During surgery a CTE was observed. The parents were consanguineous. This is the first reported case of CTE in siblings.

Splenogonadal fusion: aiding detection and avoiding radical orchidectomy.

Splenogonadal fusion is a rare benign congenital anomaly in which there is an abnormal connection between the gonad and the spleen. It was first described over 100 years ago with limited reports in the literature since then. Its similarity in presentation to testicular neoplasia poses a significant challenge in diagnosis and management, often resulting in radical orchidectomy. We present the case of a 31-year-old man who presented with a rapidly growing left-sided testicular mass and suspicious ultrasound findings; histology from the subsequent radical inguinal orchidectomy showed findings consistent with splenogonadal fusion. We describe points for consideration in the clinical history, examination and imaging that could suggest splenogonadal fusion, including preoperative technetium-99m-sulfur colloid imaging and intraoperative frozen section evaluation, which may confirm the diagnosis and prevent unnecessary orchidectomy.

Atypical scrotal masses: Surgical surprise.

We present three unusual cases of atypical scrotal lesions in children. The first was a firm left scrotal mass with the testis indistinguishable. The second with apparently a large hydrocoele, which was a cyst, and the third with multiple nodular lesions, pushing the left testis into the right hemiscrotum. These turned out to be a pigmented neuroectodermal tumour, a lymphatic malformation and neurofibromas respectively. Paediatric surgeons should be aware of such surgical surprises.

Effects of melatonin on both testicular regeneration and recovery of spermatogenesis in busulfan-treated rats

Abstract Testicular damage is one of the most hazardous effects of chemotherapy as it is frequently associated with oligozoospermia and azoospermia. This study aimed at evaluating the protective effect of melatonin in a rat model of busulfan-induced testicular injury. Rats were divided into four groups: control, melatonin, busulfan, busulfan plus melatonin. After 15 days, the semen was collected from the epididymis and testes were assessed. Sperm removed from cauda epididymis and analyzed for sperm count and viability. Testis tissues were also removed, fixed in formalin and were embedded in paraffin. Sections of testis tissue were stained with hematoxylin-eosin for histological examination and prepared for TUNEL (Terminal deoxynucleotide transferase dUTP Nick End Labeling) assay to detect apoptosis and PCNA (proliferating cell nuclear antigenassay) to detect proliferation cells. Serum and testes supernatants were separated to detect testosteron level and oxidative stress parameters. In histological examination, degenerative changes in seminiferous tubules were observed in the experimental groups. In biochemical examination, the total oxidant status (TOS) levels in Busulfan group were significantly higher than in the control group while the total antioxidant status (TAS) levels of all the groups were similar. In conclusion, the beneficial properties of melatonin treatment by its potent anti-oxidants may reduce adverse effects of chemotherapy in the reproductive system in a rodent system.

Association of antimullerian hormone with the size of the appendix testis, the androgen and estrogen receptors and their expression in the appendix testis, in congenital cryptorchidism.

OBJECTIVES: Antimullerian hormone (AMH) causes regression of the mullerian ducts in the male fetus. The appendix testis (AT) is a vestigial remnant of mullerian duct origin, containing both androgen (AR) and estrogen (ER) receptors. The role of both AMH and AT in testicular descent is yet to be studied. We investigated the possible association of AMH with AT size, the AR and ER, and their expression in the AT, in congenital cryptorchidism. METHODS: A total of 26 patients with congenital unilateral cryptorchidism and 26 controls with orthotopic testes were investigated, and 21 ATs were identified in each group. AMH and insulin-like three hormone (INSL3) concentrations were measured with spectrophotometry. AR and ER receptor expression was assessed with immunohistochemistry using monoclonal antibodies R441 for AR and MAB463 for ER. For the estimation of receptor expression, the Allred Score method was used. RESULTS: AMH concentrations did not present significant differences between patients with congenital cryptorchidism and the controls. Also, no correlation was found between AMH, INSL3, and AT length. Allred scores did not present significant differences. However, expression percentiles and intensity for both receptors presented significant differences. Three children with cryptorchidism and the highest AMH levels also had the highest estrogen receptor scores in the AT. CONCLUSIONS: No association was found between AMH and the studied major parameters. However, higher AMH concentrations, in combination with higher estrogen receptor scores in the AT, may play a role in cryptorchidism in some children. Larger population samples are needed to verify this observation.

[Prevalence of male infertility in a university hospital in Morocco]. / Prévalence de l´infertilité masculine dans un hôpital universitaire au Maroc.

INTRODUCTION: infertility in couples has become a public health problem in recent years. It can be related to a problem in the male, female or both. Men infertility accounts for 40% of cases. In Morocco, most studies have focused on the causes and risk factors for male infertility. The purpose of our study was to assess the prevalence of male infertility and semen parameters in infertile men or in men at high risk of developing infertility in a tertiary hospital in Rabat. METHODS: we conducted an analysis of 482 patients referred for evaluation of infertility in the couple or as part of preoperative assessment of varicocele or testicular ectopia. Demographic data, risk factors for infertility, primary or secondary infertility were recorded for each patient. Semen parameters were assessed and interpreted according to WHO standards updated in 2010 based on studies of factors associated with their disturbance. RESULTS: the average age of patients was 35.35±8.81 years. Primary infertility was found in 61.8% of cases. The most common risk factors for infertility were tobacco followed by varicocele and infection. Spermogram was altered in 53.1% of cases. The most common abnormality was sperm vitality issues (36.9%) followed by spermatic concentration (29.7%) and morphology (29.3%). Age was the unique factor which had a significant impact on spermogram (p=0.002). Abnormalities in mobility were detected in patients ≥31 years, sperm vitality issues in patients aged 34 years, abnormalities in morphology in patients aged 35 years and concentration in patients aged 37 years. Azoospermia was found in 16.4% of cases and was mainly associated with primary infertility. Oligo-astheno-teratozoospermia was the most common association (26.2%). CONCLUSION: male infertility is common in our context. The major risk factor is age. Mobility is the earliest parameter achieved.

Successful Fowler-Stephens Orchiopexy for an Abdominal Testis Associated With Continuous Type of Splenogondal Fusion: A Case Report.

Splenogonadal fusion (SGF) is a rare congenital anomaly in which there is abnormal adhesion between splenic tissue and gonads. Several patients with cryptorchidism associated with this anomaly have undergone orchiectomy because of the suspicion of tumors. Preoperative computed tomography (CT) is useful in differentiating between SGF and tumors, and in evaluating the vasculature of the SGF. We report a boy with an abdominal testis associated with SGF. Based on the CT findings, we successfully performed Fowler-Stephens orchiopexy. To the best of our knowledge, no SGF cases in which Fowler-Stephens orchiopexy could salvage the testis without testicular atrophy have been previously reported.