Fused variant of crossed testicular ectopia: A case report and discussion of the literature

Rare case of transverse testicular ectopia – Case report and review of literature

BackgroundTransverse testicular ectopia (TTE) is a rare anomaly in which both testes descend through a single inguinal canal into the same hemiscrotum. Although almost 20–50% of patients with TTE exhibit persistent Müllerian duct syndrome (PMDS) and many genetic analyses have been performed, no reports have described the genes contributing to TTE without PMDS. Here, we report two cases of TTE without PMDS using immunohistochemical staining and genetic analysis.Case presentationTwo Asian patients with TTE without PMDS were subjected to orchiopexy. We performed testicular biopsies during operation and obtained blood samples before the operation. Testicular tissues were stained for c-kit, placental alkaline phosphatase (PLAP), and undifferentiated embryonic cell transcription factor 1 (UTF1) to evaluate the presence of intratubular malignant germ cells. Additionally, we performed polymerase chain reaction-based direct sequencing to identify single nucleotide polymorphisms in genes associated with regression of the Müllerian duct and testicular descent (that is, anti-Müllerian hormone [AMH], AMH receptor 2 [AMHR2], insulin-like 3 [INSL3], and relaxin family peptide receptor 2 [RXFP2]). The three-dimensional structures of proteins were predicted using SWISS-MODEL. In immunohistochemical analysis, c-kit and UTF1 were positive, whereas PLAP was negative in three testicular tissue samples from the two patients. These features were also detected on the unaffected side. In variant analysis, common missense variants in the AMH gene (g.365G>T; c.165G>T; p.Ser49Ile [rs10407022]) were observed. All variants in INSL3 and RXFP2 genes were intronic or silent.ConclusionsBecause UTF1, a specific marker of spermatogonial stem cell activity, was expressed in both the affected and unaffected sides in the testicular tissues of two patients, the risk of malignancy may be high in these patients. Although the etiology of TTE without PMDS remains unclear, our variant analysis results were consistent with previous reports, and variants in the AMH gene (rs10407022) may contribute to the specific phenotype of TTE without PMDS.

Patient: Male, 1.5-year-oldFinal Diagnosis: Abdominal wall testicular ectopiaSymptoms: Abdominal swelling • undescended testisMedication: —Clinical Procedure: Diagnostic laparoscopy and single stage laparoscopic orchidopexySpecialty: Pediatrics and Neonatology • SurgeryObjective:Congenital defects/diseasesBackground:Ectopic testis is an uncommon congenital anomaly that has been reported in different sites in the body. The anterior abdominal wall is an exceptionally rare variant site for ectopic testis. The purpose of this case report is to highlight the importance of being aware of this rare clinical entity, thereby increasing the chance of preserving an ectopic testis. In addition, this case report shows how a laparoscopic approach is advantageous for better localizing and treating such rare cases, as this is the first reported case of such a condition to be managed with a laparoscopic approach.Case Report:This is a rare case of testicular ectopia in an unclassified abdominal wall defect mimicking a Spigelian hernia that occurred in a 1.5-year-old boy. He presented with congenital swelling of the right lower abdominal wall and an empty right hemiscrotum. Diagnostic laparoscopy was performed and the intraoperative findings suggested right testicular ectopia in the anterior abdominal wall defect. The right testis was herniated through an abnormal, unclassified, abdominal wall defect with both a closed inguinal ring and no defect in the semilunar line. Using a laparoscopic approach, the ectopic testis and hernia were managed successfully with primary hernia repair and single-stage orchidopexy. The patient’s postoperative recovery was uneventful. At his 10-month follow-up appointment, he had no evidence of hernia recurrence.Conclusions:As demonstrated in this case, laparoscopic surgery, which has not been used in previously reported cases of ec-topic testis, aids in proper localization and repair of an anterior abdominal wall defect along with orchidopexy.

Introduction and importanceTransverse Testicular Ectopia (TTE) is characterized by the presence of testis in the hemiscrotum, which can be associated with a broad spectrum of complications. It is usually manifested in pediatrics. However, on rare occasions, it can occur in adults. The diagnosis is confirmed by magnetic resonance imaging (MRI). We present two cases of Transverse Testicular Ectopia (TTE), demonstrating the significance of early diagnosis and treatment to reach optimal outcomes. Case presentationWe reported two patients with common features suggestive of Transverse Testicular Ectopia (TTE). Case 1 had open surgery; his left testis was impalpable, whereas his right side was palpable. Case 2 had undergone laparoscopy surgery, and his right and left spermatic cord was discovered on the right side. Clinical discussionTransverse Testicular Ectopia (TTE) is classified according to clinical presentation; Type 1 is associated with inguinal hernia ranging between 40 and 50 %. Type 2 is related to persistent mullerian duct syndrome (PMDS), with a rate of 30 %. Type 3 is associated with genital anomalies and azoospermia, with a rate of 20 %. The pathogenesis is unclear. However, studies suggest that the persistence of the mullerian duct prevents normal descent of the testis. Treatment is purely dependent on early clinical presentation and surgical methods. ConclusionTransverse Testicular Ectopia (TTE) requires delicate care by the pediatric surgeon as it is considered a rare entity in such cases. Heroin, we highlight the significance of early surgical treatment and the possibility of complications if left untreated.

Testicular ectopia: Why does it happen and what do we do?

BackgroundTransverse testicular ectopia (TTE) is a rare congenital anomaly in which both the testis are in the same hemiscrotum or one testis in the inguinal canal of the same side. It is usually associated with other anomalies such as inguinal hernia, persistent Mullerian duct syndrome (PMDS), true hermaphroditism, and pseudo-hermaphroditism. In this case report, we present a rare case of TTE in an adult patient with fused vas deferens, aplastic right seminal vesicle, and right side inguinal hernia.Case presentationA 33-year-old male came with complaint of severe pain in the scrotum for 2 days with a long-standing history of right inguinoscrotal swelling. Clinical examination revealed a right inguinoscrotal swelling in which right testis was not palpable separately and left testis was palpable at periphery of the left hemiscrotum. Ultrasound imaging and MRI of the scrotum revealed TTE with both testes in the left hemiscrotum, fused vas deferens, right aplastic and left hypoplastic seminal vesicle, right side patent process vaginalis with a non-obstructive, and non-strangulated inguinal hernia. Surgical intervention with transeptal orchidodpexy was advised but not performed due to the patient’s unwillingness. Hence, we recommended an annual follow-up for the same.ConclusionThe present case report emphasizes that though TTE is a rare congenital anomaly, it should be considered as a differential diagnosis in patients with an absent testis and/or infertility, and a detailed imaging and biochemical investigation should be employed considering the wide spectrum of associated conditions.

İntroductionTransverse testicular ectopia (TTE) is a rare anomaly characterized by the presence of both testicles in the same hemiscrotum or inguinal region. The most common clinical findings of TTE are unilateral nonpalpable testis in the scrotum and inguinal hernia on the side of palpable testis in the scrotum. It should be kept in mind that TTE may coexist with Persistent Mullerian Duct Syndrome (PMDS). Therefore, appropriate treatment should be performed considering PMDS. Presentation of caseType 2 transverse testicular ectopia was diagnosed in one patient who was operated with bilateral undescended testis. He was treated with transseptal orchiopexy and excision of mullerian structures. DiscussionIn case of TTE with PMDS, optimal surgical approach with orchiopexy and excision of Müllerian duct is necessary. Intraoperative aggressive dissection of vas deferens and testicular vessels should be avoided in TTE patients. They should be closely followed because of the increased risk of malignant transformation in the postoperative period. ConclusionAn investigation of transvers testicular ectopia should be performed in all nonpalpable undescended testis anomalies.

Transverse testicular ectopia is an uncommon disorder of testicular ectopia. Nearly thirty percent of the cases is associated with Persistent mullerian duct syndrome which is characterized by karyotypically normal males with retained mullerian derivatives. Understanding the natural process of the condition and the association with malignant potential will allow for a better understanding of the optimal surgical approach. This is a case report of young male presented a left sided inguinal hernia in which the sac contained both testes and uterus. The literature review of the syndrome will be discussed.

BackgroundCrossed testicular ectopia or transverse testicular ectopia is an extremely rare urogenital anomaly. In this condition, on average at 4 years of age the testes migrate through the inguinal canal and one or both testes may turn up in the abdomen, inguinal region, or in the hemiscrotum, with an empty contralateral hemiscrotum. Our case report documents transverse testicular ectopia in a 5-year-old boy who presented with right inguinal hernia and nonpalpable left testis. He underwent previous right herniorrhaphy at the age of 1 year.Case presentationA 5-year-old Iranian boy was diagnosed with a right inguinal hernia. He underwent right inguinal herniorrhaphy at the age of 1 year. For this case report, the hernia symptoms had returned. Both testicles were palpated in the right scrotum, an ultrasound examination also revealed both testicles to be present in the right scrotum, and a hernia sac located in the right inguinal region with an internal ring. The patient was recommended to undergo a surgical reconstruction. Surgical reconstruction was performed by crossing the left testis in the transseptal orchiopexy technique.ConclusionIn patients with cryptorchidism on one side and an inguinal hernia on the other side, the surgeon must consider a rare condition known as transverse testicular ectopia. Sonography can be helpful for diagnosing cases where transverse testicular ectopia is suspected, evaluating other anomalies, and selecting the most appropriate treatment.

True testicular ectopic represent 1% of testicular migration abnormalities. Transverse or crossed form is a very rare form of testicular ectopia. It is defined as an abnormal localization of both testicles in the same hemi-scrotum. Association of a persistence of vaginal process or a syndrome of persistence of the Müllerian canal is more commonand deserves to be underlined. The diagnosis is often intraoperative but can be facilitated by an ultrasound performed preoperatively. Before any case of testicular ectopy, notably an empty scrotum associated with an inguinal hernia, transverse testicular ectopy should be suspected. Orchiopexy in-dartos on both sides after a trans septal lowering of the testicle concerned is the rule. Currently with the development of laparoscopy, management can be performed by open surgery but also depending on the availability of the technical platform and the experience of the surgeon, laparoscopic treatment is possible. Transverse testicular ectopy is a rare form of testicular ectopy. the principle of the treatment consists in the lowering of the testicle in intra scrotum and the orchidopexy. Long-term follow-up of testicules should be systematic because risks of infertility and malignant degeneration exist and are multiplied respectively by six and eight compared to the rest of the population. We report a case, diagnosed in a secondary health center, and review the literature.

Crossed testicular ectopia (CTE)/transverse testicular ectopia (TTE) is a rare but well known congenital anomaly, in which both gonads migrate toward the same hemiscrotum. It is usually associated with other abnormalities such as persistent Mullerian duct syndrome, true hermaphroditism, inguinal hernia, hypospadias, pseudohermaphroditism, and scrotal anomalies. About 100 cases of transverse testicular ectopia have been reported in published studies. We report a case of transverse testicular ectopia in an 8-month-old boy who presented with right inguinal hernia and nonpalpable left testis. On exploration, both testes were present in the right inguinal region. Bilateral orchiopexy was performed by crossing the left testis in the extra-peritoneal space and ipsilateral scrotal orchiopexy. The diagnosis could not be made preoperatively in most of reported cases.

Crossed testicular ectopia also known as transverse testicular ectopia is a rare anomaly of the testis which is ectopically located in contralateral hemiscrotum with absent testis in the ipsilateral hemiscrotum. Most case are incidentally discovered intraopeartively during operation of inguinal hernia and few case reports are available which have reported preoperative diagnosis of crossed testicular ectopia. We report a case of crossed testicular ectopia in 12 year old boy who presented with right undescended testis diagnosed preoperative on Ultrasound and Magnetic Resonance Imaging as crossed testicular ectopia.

BackgroundCrossed testicular ectopia is a rare form of urogenital anomalies in which both testes are migrated and descend through a single inguinal canal, one or both testes may be ectopic in the abdomen, the inguinal region or descent to the hemi-scrotum with empty contralateral hemi-scrotum. Case presentationA 1.5-year-old male patient had an empty left scrotum with no palpable testis in the inguinal region with a palpable testis in the right scrotum with palpable other testis in the right inguinal region. Ultrasound examination showed the right testis in the right scrotum and another testis measuring in the right inguinal region giving suggestion of both testes in the right side. During operation two testes was found in the right inguinal region with two small indirect inguinal sacs. Herniotomy was performed for both sacs. The right testis was placed and fixed in the right hemi-scrotum and the left testis was fixed in the left hemi-scrotum through trans-septal window. ConclusionDuring surgery, the surgeon should adopt a conservative approach to preserve the fertility. Trans-septal orchiopexy or extra-peritoneal transposition orchiopexy is advised. In case of persistent Müllerian duct syndrome, preservation of the Müllerian remnants is recommended to preserve blood supply to the vas deferens and testis. Karyotyping may be required in some cases specially if associated with other congenital anomalies of the genitalia. Patients usually need long term follow up because such patients may have future fertility problems and there is an increased risk of the development of testicular cancer.

IntroductionWolf-Hirschhorn syndrome is a rare condition including a characteristic facial appearance, delayed growth and development, hypotonia, intellectual disability and seizures. Its frequency is estimated at 1/20000 to 1/50000 births with...

BackgroundTransverse testicular ectopia (TTE) is a rare congenital anomaly where both testes descend through the same inguinal canal and are located on the same side. Cases with fused spermatic cords are particularly rare and present unique surgical challenges.Case DescriptionWe report the case of an 8-month-old male infant with bilateral cryptorchidism and an empty scrotum since birth. Preoperative ultrasonography identified two testicular-like structures near the right internal inguinal ring. Laparoscopic exploration revealed bilateral patent processus vaginalis, with both testes located near the right internal inguinal ring. The left spermatic cord crossed the pelvis and fused with the right spermatic cord. Both testes appeared normal in size, with separate epididymides and vas deferens. Surgical management involved careful dissection and separation of the fused spermatic cords, followed by bilateral orchiopexy via the respective inguinal canals and repair of the processus vaginalis. At an 8-year postoperative follow-up, ultrasound confirmed that both testes were well-positioned with normal size and blood supply. Testosterone levels were monitored annually and remained within the normal range. This case highlights its significant role in the management of complex anomalies like TTE.ConclusionsThe postoperative course was uneventful, and an 8-year follow-up demonstrated that both testes remained well-positioned, with normal size, blood supply, and testosterone levels.