30. Congenital anomalies of the testis Flashcards
what is the main function of the testes
to produce sperm
list the Main male reproductive organs (in ascending order); •
Testes -> adjacent epidydymis -> Vas deferens (part of spermatic cord) contents are nerves and vessels of spermatic cord). VD is approximately 30-50cm long. ->2Ejaculatory ducts- that are result of confluence of distal or terminal part of vas deferens and the 2 excretory duct of seminal vesicles at the level of the ->seminal colliculus the EjD open into the prostatic urethra-> membranous urethra->penile/spongy urethra-> penis-> External urethral orifice
Prostatic urethra + membranous = prostatic urethra.
what are the two parts of male genitalia
External genitalia: penis, scrotum, testis
Internal genitalia: prostate, seminal vesicle, ejaculatory duct,
what are the four embryological staages of the testes
- mesonephros
- dimophism
- wolfian duct developement
- mullerian duct regression
Embryology of the testis
- 1)Testes come from mesonephros
- Collecting duct of mesonephros is called wolffian duct = generates male reproductive organs and the kidney, is formed
- 2)Dimorphism= Sexual development occurring betw/ 6-8 weeks of gestation until 20th week of gestation. When male or female phenotype is recognised.
- Dimorphism means presence of testes or ovaries,
- Physiological position of testicles is scrotum at birth
- When male phenotype is recognised,3) wolffian duct give rise to ureters, part of the urinary bladder, VasD, Epidydymis and seminal vesicles.
- 4)Müllerian duct regresses d/2 inhibitory hormone by sertolli cells. along w/ testosterone and other hormones
- in a female phenotype, Müllerian duct gives rise to female reproductive organs - uterus and Fallopian tubes
what are the 2 stages of testicular descent
-
Intrabdominal phase
- kidney=> deep inguinal ring=>equal to peritoneum
-
Inguinal scrotal stage
- deep inguinal ring=>scrotum at 7-9 mo gestation
when does the testis descend into the scrotum
scrotum at 7-9 mo gestation
should be in the scrotum at birth
60% of undescended testes fully descend within the first year
whaat are the 3 layers/ tunics of the testes
Tunica Vaginalis
Tunica Albuginea
Tunica Vasculosa
what are the types of congeital anomalies of the testes
- congenital anomalies according to number
- congenital anomalies according to size/structure
- congenital anomalies according to position
congenital anomalies of testis according to number
monorchidism = absence of one testes
- not a problem for fertility but for cosmetics
- solved by placement of testicular prosthesis
anorchidism = Absence of both testes. Very difficult situation.
- definite male infertility
- biological castration of Fetus by Rubella infection during pregnancy.
polyorchidism = Presence of 2+ histological proven testes
Very rare congenital anomaly
congenital anaomalies of the testis according to size/structure
what are the 2 parts,
in terms of structural anomalies what’s the dx betw/ dysgenesis and agenesis
which are the genetic syndromes that affect testicular structure
size =small testicles •
structure= atypical d/2 defective early embryogenesis
dysgenesis presence of defective anatomical organ
agenesis there is no anatomical organ.
•Genetic syndromes
- Klinefelter syndrome - 47XXY.
- Kallmann syndrome -
- Persistent Müllerian duct syndrome PMDS
- Del Castillo syndrome or sertolli cell only syndrome or germ cell aplasia
describe klinefelter syndrome
Klinefelter syndrome - 47XXY.
Clinical features are gynecomastia, small testicles, low intelligence, infertility
describe Kallman syndrome
- sx: hypogonadotropic hypogonadism + anosmia.
- impaired hormone production from hypothalamus GnRH. Mild infertility as a result.
what is Persistent mullerian Duct syndrome
- great example of Disorder of Sexual Development
- persistent Mullerian duct in males causes pseudohermapgroditism
- Pseudohermaphroditism = Normal external and internal male genitalia but with presence of female reproductive organs.
- Diagnosis is difficult d/2 normal exterior
what is Del Castillo syndrome
aka or _sertolli cell only syndrom_e or germ cell aplasia
- complete lack of germ epithelium with only of sertolli cells in seminiferous tubules.
- sx:
- definite male infertility
- No erectile dysfunction, normal erectile function due to normal production of testosterone by leydig cells.
- no hypogonadism
- Diagnosis
- made by open excisional testicular biopsy.
- The main indication for testicular biopsy is infertility and azoospermia.
- Final histological report shows absence of germ epithelium.
- pts w/ castillo can still achieve fatherhood by: assisted reproductive technologies with donor insemination
what is the indication for dg for del castillo
Diagnosis made by open excisional testicular biopsy.
indicated by infertility and azoospermia.
definitive dg on histology is absence of germ epithelium on excisional testicular biopsy
pts w/ castillo can still achieve fatherhood by: assisted reproductive technologies with donor insemination
what are the congenital anomalies of the testes according to position
- Cryptochoridsm-mc testicular anomaly
- Ectopy
- Retractile testes
- Non palpable testes
what is the most common testicular cong anomlalie
Cyrptochordism aka undescended/ maldescended testes:
def: arrested testes on the normal path of testicular descent between renal and scrotal areas
- two major unfavourable effects of cryptorchidism is 1)infertility and 2)significant risk for testicular cancer
- NB to prevent this unfavourable effect of cryptorchidism, early operation is strongly recommended. Name of operation is orchidopexy.
- This operation performed; in USA up to first year, in Europe between 1-3 years
when is orchidopexy done to prevent the complications (infertility, testicular cancer) of crpytochordism
- in USA up to first year,
- in Europe between 1-3 years.
descrribe ectopy
give examples
what is the treatment for ectopy of the testes
def: testes has strayed from the normal path of descent.
- Means that testicle is in an unusual position e.g,
- suprapubic, penile ectopy, perineal ectopy, femoral
- transversal / crossed ectopy very rarely when both testicles are in one part of the scrotum these terms are also eligible for renal ectopy.
Operation is orchidectomy
what is rectrale testes
what are the four criteria for a testes to be considered retractile
what is the treatment for rectractile testes if any
Retractile testes = the testes intermittently migrate to a higher position along the normal descent.
criteria for retractile testicle NB
- Testes can be manipulated to the bottom of the scrotum during physical examination
- Tested re_mains in the scrotum during_ physical examination
- Testes is normal in size e.g. aplastic
- Testes have been seen in the scrotum by the parents
surgical operation is not recommended, it’s important to identify when the testicle is retractile.
what is the definition of a non palpable testes
what does it mean if it is bilateral
what is DSD
def= testicle can’t be palpated during physical exam even under anaesthesia
4 causes of non palp testes on phys exam
- Anorchidism: congeital abscence of one or both testes
- testicular atrophy
- intraabdominal location of testes
- incorrect physical examination
Bilateral non palpable testicles is a very dangerous situation.
- can be caused by DSD means disorder of sex development, in strict sense this means any congenital condition with atypical development.
