A case report of hypospadias combined with polyorchidism

Abstract

Hypospadias is a common congenital malformation of the reproductive system; however, to date, no cases of hypospadias complicated by polyorchidism have been reported in the literature. This article reports a case of a 1-year-old boy presenting with hypospadias, who was also diagnosed with polyorchidism combined with cryptorchidism. Preoperative Doppler ultrasound revealed two testes on the right side, and an atrophic testis on the left. After surgical treatment, the patient recovered well and was discharged. Hypospadias combined with polyorchidism is highly prone to misdiagnosis or missed diagnoses. This case provides insight into the recognition, diagnosis, and treatment of this condition.

1 Introduction

Hypospadias is the second most common congenital malformation of the reproductive system after cryptorchidism. Currently, standardized diagnostic and treatment protocols for hypospadias exist, with the most common associated conditions being inguinal hernia, hydrocele, and incomplete testicular descent, each occurring in approximately 9% of cases. However, there have been no reported cases of hypospadias combined with polyorchidism, and standardized diagnostic and treatment protocols for this condition have not yet been established. Moreover, the treatment of polyorchidism remains controversial and is often determined by various factors, such as testicular location, reproductive potential, testicular size, and patient age. In this case, the occurrence of left-sided testicular atrophy is extremely rare. We will now discuss this case in conjunction with clinical data and relevant literature.

2 Case description

The patient is a male child aged 1 year and 5 months. He was admitted to the hospital on April 10, 2024, due to an abnormal urethral opening observed since infancy. Physical examination revealed an underdeveloped penis with the urethral opening located at the base of the ventral side near the scrotum, a markedly chordee and a split scrotal raphe ( Fig. 1 A). No palpable testis was found in the left scrotum or inguinal region, and two peanut-sized testicular-like masses that could slide into the scrotum were detected in the right inguinal area. The patient was not treated in other hospital. The patient had no other family history. Auxiliary examinations: Anti-Müllerian hormone (AMH) level was 22.96 ng/ml, estradiol 5.00 pg/ml, luteinizing hormone (LH) 0.275 mIU/ml, progesterone 0.167 ng/ml, and testosterone <0.03 ng/ml. Chromosome analysis indicated a 46XY karyotype. Ultrasound examination showed two testicular-like echo areas in the right spermatic cord measuring 7.4 × 4.3 mm and 11.6 × 5.0 mm, and a solid hypoechoic nodule of approximately 5.6 × 4.3 mm in the left inguinal canal ( Fig. 1 B and C). Color Doppler Flow Imaging (CDFI) revealed blood flow signals within the right testis. The child also presented with a mild left-sided spermatic sheath effusion, and based on the test results, we suspected that the child had Müllerian duct persistence syndrome. The preoperative pelvic floor ultrasound did not reveal any Müllerian duct remnants, and the patient’s family declined the suggestion for an MRI for further evaluation. Therefore, we performed laparoscopic exploratory surgery to investigate Müllerian duct remnants and simultaneously manage the spermatic sheath effusion. On April 29, 2024, hypospadias repair (Duckett) + pedicled axial flap grafting + change to repair of chordee + bilateral orchiopexy + laparoscopic ligation of the bilateral tunica vaginalis was performed under general anesthesia. Intraoperative laparoscopy showed no significant remnants of the Müllerian duct in the abdominal cavity, and bilateral tunica vaginalis were found to be patent. At laparoscopy, the left tunica vaginalis revealed remnants of the vas deferens, and the spermatic vessels were markedly atrophied; the right vas deferens and spermatic vessels appeared normal. Squeezed the inguinal region bilaterally to avoid inadvertent ligation to testicular tissue. After ligation of the bilateral tunica vaginalis, the right scrotum was incised and exploration of the inguinal region failed to reveal any other abnormal tissue, revealing two soy-sized testes in the right tunica vaginalis cavity connected in tandem by the tail of the epididymis ( Fig. 2 A). The left scrotal tunica vaginalis was incised, and hydrocele fluid was drained. The same examination of the inguinal region did not reveal any other abnormal tissue. A rice-sized atrophic testicular-epididymal-like tissue observed in the left scrotum was excised ( Fig. 2 B). The right spermatic cord was freed, and one testis was placed into the left scrotal cavity and secured to the dartos, while the other was fixed to the right scrotal dartos. Subsequently, a midline incision was made along the urethral plate for release, and a dorsal plication suture was placed to correct the chordee. A pedicled transverse flap from the dorsal penile skin was harvested and sutured to the defect in the midline of the proximal urethra, and the flap was inverted and sutured to form the urethra ( Fig. 2 C–F). Finally, the corpora cavernosa were wrapped around the new urethra and sutured in place, each layer closed sequentially, and the penis was compressed and bandaged. Postoperative pathological examination of the atrophic testicular-like tissue on the left side suggested fibrous connective tissue with benign glandular structures. The discharge diagnosis: (I) Hypospadias, (Ⅱ) Polyorchidism, (Ⅲ) Left testicular atrophy, (Ⅳ) Bilateral patent sheath, (Ⅴ) cryptorchidism. Before discharge, the urethral surgical area healed well, and the patient was able to urinate normally after the catheter was removed ( Fig. 3 ). Four months after surgery, follow-up showed good recovery of the surgical area ( Fig. 4 A). Ultrasound re-examination indicated that both testes were in ideal positions, with visible blood flow signals within both testes ( Fig. 4 B and C). All study methods were performed in accordance with relevant guidelines and regulations. This study was approved by the XXX Ethics Committee. Informed consent was obtained by the parents for all procedures and for the publication of this case.