Abstract
Introduction
Polyorchidism is a rare congenital genitourinary anomaly defined by more than two histologically confirmed testes. Triorchidism predominates, whereas tetraorchidism — four testes — is exceedingly rare, with fewer than 10 reported cases. We present a case of tetraorchidism, emphasizing imaging findings.
Case Info
An 11-year-old male with ADHD, atopic dermatitis, and resolved hydronephrosis presented with two months of intermittent right groin pain. The episodes of pain occurred multiple times per week with occasional radiation to the right lower quadrant. Symptoms improved with ibuprofen but often required school absences. Physical exam revealed bilateral scrotal testes, tenderness of the right groin and scrotum, and a right inguinal hernia with an inguinal bulge. Ultrasound revealed the right testis in the inguinal canal with a moderate hydrocele and a mobile left testis in the inguinal canal and scrotum. Additional bilateral scrotal masses with the same vascularity and echogenicity as testes were identified, consistent with supernumerary testes. The supernumerary right testicle was noted to be atrophic.
Urology consultation recommended right inguinal hernia repair, orchiopexy, and hydrocelectomy. Prior to scheduled surgery, the patient experienced an episode of severe pain and increased right scrotal swelling, concerning for an incarcerated hernia and requiring urgent surgical intervention. Exploration confirmed four distinct testes located as follows: right testis in the inguinal canal with inguinal hernia, right type A3 supernumerary testis in the scrotum, left testis in the scrotum, and left type A3 supernumerary testis in the scrotum.
Intervention included right inguinal orchiopexy, removal of the atrophic right supernumerary testis, and left scrotal orchiopexy of both testes. Histopathology of the removed right supernumerary testis confirmed testicular tissue with seminiferous tubules, germ cells, and Sertoli cells. At six-month follow-up, the patient had recovered well without complication. He will continue monitoring for pain or concerns following self-examination or puberty.
Disc Clinical
Fewer than ten histologically confirmed cases of tetraorchidism have been reported. It is thought to result from anomalous transverse division of the genital ridge. Epididymal and vas deferens anatomy vary in supernumerary testes. Testes without connection to an epididymis and vas deferens lack reproductive potential. Scrotal ultrasound is the first-line imaging modality. MRI can further characterize supernumerary or extrascrotal testes. Given misdiagnosis rates of approximately 22% based on ultrasound alone, accurate imaging is essential.
Management is individualized using the Bergholz classification. Asymptomatic, scrotal testes with favorable anatomy may be observed, while extrascrotal location, cryptorchidism, or torsion may warrant surgery. Neoplasm occurs in approximately 6.4% of cases, with malignancies occurring predominantly in cryptorchid supernumerary testes. Thus, cryptorchidism is an important factor when considering surveillance, orchiopexy, or orchiectomy. Given the rarity of tetraorchidism, management should be individualized to the patient’s history and clinical symptoms, finding balance between preservation of reproductive function and malignancy risk.
CONCLUSION Tetraorchidism is an exceedingly rare anomaly. Management depends on the location and anatomy of each supernumerary testis. The Bergholz functional classification guides individualized care, favoring surveillance for scrotal, asymptomatic, imaging-confirmed testes and surgery for extrascrotal location, cryptorchidism, or torsion. Because cryptorchidism is the principal malignancy risk factor, accurate characterization of each testis is essential.