1. Etiology
By the sixth week of embryo development, the mesonephric duct and mesonephric ducts form, which tubes will differentiate into male and female reproductive tracts. When the embryonic reproductive glands differentiate into testes and produce testosterone, under the action of androgens, the mesonephric duct gradually differentiates into male reproductive ducts. The head part of the mesonephric duct becomes the epididymal appendage, and the epididymal duct derived from the mesonephric tubules, together with the epididymal duct formed by the elongated and tortuous winding of the mesonephric duct below, constitutes the head of the epididymis. The remaining epididymal ducts form the body and tail of the epididymis.
The etiology of congenital epididymal malformation is not yet clear. Since patients with cryptorchidism often have epididymal malformation, its occurrence may be related to endocrine dysfunction during embryonic development. Due to low testosterone levels, the mesonephric tubules and mesonephric duct do not develop or are underdeveloped, leading to various types of epididymal malformations. If the mesonephric duct does not develop at all, it can lead to congenital absence of the epididymis and vas deferens. If development stops at a certain location, it will form an atresia at that location. When there is a blockage in the coiling of the epididymal duct, it can result in the significant elongation of the epididymis and the formation of long-loop epididymal malformation.
Second, Pathogenesis
The epididymis is the excretory duct connecting the testis, with a thin, elongated, flat shape, located on the posterior and lateral side of the testis. 10-15 testicular efferent ducts curve into a conical shape, and their ends merge into a long epididymal duct that is 4-6 cm long and highly coiled. According to Turck's examination of 94 non-undescended testicles, such as hernia, hydrocele, varicocele, etc., the epididymal head and tail were found to be attached to the testis in 83.9% of cases, while there was a certain distance between the epididymal body and the testis, which could generally accommodate a finger tip. Only 12.5% of the epididymis and testis were in complete contact.
The malformation of the epididymis mainly manifests as developmental disorders of the epididymis and abnormal attachment to the testis. The former includes the absence of the epididymis, cystic transformation of the head, underdevelopment of the middle and tail, fibrous cord-like atresia, and the epididymis significantly elongated into a long-loop shape, etc. The absence of the epididymis can be further divided into:
1. The mesonephric duct does not develop at all, and the vas deferens, seminal vesicle, and ejaculatory duct are all absent.
2. The mesonephric duct is underdeveloped, and the body and tail of the epididymis are absent, accompanied by the absence of the vas deferens.
3. The mesonephric duct does not develop into the epididymal duct but directly differentiate into the vas deferens, seminal vesicle, and ejaculatory duct, and the efferent duct of the testis is connected with the vas deferens.
4. Without epididymis, the vas deferens does not connect with the testis, and its proximal end is blind. Abnormal attachment of the epididymis includes complete separation and partial separation of the epididymis from the testis, the latter referring to the head of the epididymis not connected with the testis, and the epididymis not attached to the inferior pole of the testis, etc.
Since Scorer and Farrington first classified epididymal malformation in 1971, many different classification methods have been reported. In 1990, Koff and Scaletscky made some modifications based on the Scorer classification method. The malformation of the epididymis is divided into 5 categories:
Type I: Long-loop epididymis: The epididymis is in the shape of a long loop, and it is significantly longer than the testis in size, and it is divided into 4 types: ①Twice as long as the testis; ②2-3 times; ③3-4 times; ④More than 4 times.
Type II: Separation of epididymis and testis: According to the site and degree of separation, this type can be divided into the following 3 situations: ①Only the tail is separated; ②Both the head and tail are separated from the testis, but are relatively close; ③Both the head and tail are separated from the testis, but are relatively far apart.
Type III: Angle between epididymis and testis. ①Simple angle; ②Accompanied by epididymal stenosis.
Type IV: Atresia of the epididymis or any part of the continuity between the epididymis and the sperm duct.
Type V: Longer testicular mesentery.
The above different types of epididymal anomalies can coexist in several types in the same patient. Koff's data shows that long-loop epididymal anomalies are the most common in cryptorchidism and ectopic testicles, accounting for about 79.3%, the separation of epididymis and testicle is 45.1%, the angular separation of epididymis and testicle is 8.5%, the atresia of epididymis or sperm duct is 3.7%, and the longer testicular mesentery is 1.2%. Koff's classification method cannot include all types of epididymal anomalies. Based on relevant data, various types of epididymal anomalies are summarized.