Systemata genitalia
The genital system develops from primordia initially common to both sexes, and this shared origin explains why its variants are numerous and often silent until reproductive age. Malformations of Müllerian derivation — septate, bicornuate, unicornuate or didelphys uterus — are described in a substantial share of the female population, with higher proportions among women assessed for recurrent pregnancy loss or infertility, and their correct classification guides the choice between surveillance and surgical correction. In the male, the asymmetric drainage of the testicular veins — the left into the renal vein, the right directly into the cava — accounts for the marked left-sided predominance of varicocele, while an unexpected course of the ductus deferens exposes it to injury during inguinal surgery. Because genital development proceeds in parallel with urinary development, a variant of one calls for a search for the other: an absent or ectopic kidney is frequently associated with unilateral uterine anomalies. Documenting these configurations with shared terminology is the premise for comparing case series that still adopt different classifications.
14 published articles
"Sterility in the Male.--Pajot has examined the spermatic fluid of over 200 men, aet. from twenty-five to sixty, and he emphasizes the fact, of which too little notice is generally taken, that in sterile marriages, oftener than one is disposed to imagine, the fault lies at the door of the husband. The spermatic fluid may be rich, ordinary, poor or insufficient, or absolutely worthless."--From Edinburgh Medical Journal, 1825.
Absence of the seminal vesicles is very rare. They are often bilaterally unequal in size, shape, and form. Cysts of the seminal vesicles are uncommon (+/- 100 total cases reported) but when present they are associated with unilateral renal agenesis in 68% of cases. Berengario da Carpi (1470-1530) was the first anatomist to describe the seminal vesicles (1523). Variations in Seminal Vesicles
Apart from variations in size, the prostate is subject to few variations. Persistence of the original independence of the lateral lobes, absence of the middle lobe, and presence of a fourth lobe have been reported. Variations in the relations and mode of ending of the ejaculatory ducts (fusion into a single canal, or termination in the prostatic utricle or by a special canal below the crest) or in the prostatic utricle (absence, enlarged size, or unusual opening) are related to deviations in the development of the generative tract.
Bifid conditions and complete duplication (diphallus) may occur. Diphallia has a report frequency of 1/5.5 million births (US Public Health Service) (Cernach) Congenital absence of the penis has a frequency of 1/30,000,000 (documentation unavailable). An os penis has been reported. The first reported case of doubled penis was found on a cadaver, in Bologna, in 1609 by Wecker. It was reported as follows: Observatio. Pene gemino visus quidam. Vidimvs Bononia in publicis dissectionibus cadauer viri qui duplicem habuit penem. Cl. v.
Congenital variations are more common in men than in women (1.5-2.0:1). Variations and anomalies of the reproductive tract, however. are less common in men. Genital variations with renal agenesis are reported found in 12% of men and 40% of women.
According to Gutierrez, 40% of pathologic conditions of the urinary system are due to variations. Urologic variations are found in 10% of vertebral variations, in 15% of meningocele or myleomeningocele, in 25% of double anus, 42% of duplication of colon, 90% of duplication of genitalia (Cernak, 1989). The kidneys sometimes vary from their normal form, being either longer and narrower or shorter and more rounded. The characteristic fetal lobulation may persist in the adult (7%). Occasionally, one kidney is very small, while the other is proportionally enlarged.
The most important variations are related to defective development and imperfect fusion of the component müllerian ducts, which are often associated with anomalies of the uterus. When these ducts fail to reach the urogenital sinus, the vagina (and often the uterus) may be absent. Vaginal agenesis was first described by Realdus Columbus in 1572. Duplication, more or less complete, follows persistence of separate or imperfectly fused müllerian ducts.
The chief anomalous conditions of the uterus are due to defective development or imperfect fusion of the müllerian ducts, which unite to form the usual organ. Arrested development of the lower part of these fetal canals accounts for the complete absence of the uterus and vagina. Depending on the extent to which failure of fusion occurs, all degrees of doubling are produced.
Apart from various locations that accompany malposition of the uterus and ovary, variations of the uterine tube usually depend on developmental faults traceable to imperfect or aberrant formation of the müllerian ducts. Retention of the fetal tortuosity, stunted development, or complete absence may involve one or both tubes. Complete doubling of the uterine tubes may occur in association with supernumerary ovaries.
Abnormalities in the sex organs of the female are, for the most part, due to developmental deviations. Anomalies of the reproductive tract are more frequent in women than men (1.5-2.0/1). Incomplete or modification of its descent from the abdomen affect the final position of the ovary. It may retain its original suprapelvic position and lie above or upon the psoas major muscle. If the female gubernaculum (ovarian ligament) fails to adhere to the uterus the ovary may follow the round ligament and pass partly or entirely through the inguinal canal into the labium majus.
The mammae frequently develop asymmetrically, the right often being larger and lower than the left. While absence of one or both mammae is very rare, with or without associated absence of the nipple, an increase in their number is relatively common occurrence. The supernumerary mammae vary in the extent to which they are developed, being represented sometimes by well-formed accessory glands (polymastia) that may become functioning organs, but more often, particularly in the male subject. Only by rudimentary nipples (polythelia) or even pigmented areas suggesting areolae.
Cases of individuals with a functional ovary and uterus (with monthly bleeding) and with a penis., testicle (with spermiogenesis), epididymis, seminal vesicle, and prostate have been reported.
Congenital bilateral absence of the vas deferens without associated kidney and urinary tract anomalies has been reported. Reference Godard, E. (1859) Note sur l'absence congénitale du canal excréteur et du réservois de la semenence, le testicule existant. Soc. Biol. Comptes Rendus des Séances et Mémoires. 11:329-342.
In addition to abnormalities in size, the two glands may be fused into a single mass, or one or both may be absent. Sometimes their absence is questionable since the organs may be represented by rudimentary glands embedded entirely within the substance of the corpus cavernosum urethrae.
