Pemicu 4

“Air seni tidak memancar lurus”

Adrian Pratama - 405100018
LO 1
 Menjelaskan kelainan kongenital traktus urogenital
secara umum
Anterior urethral anomalies
 Phimosis
 : fibrotic contraction of the preputial opening  not permit
retraction of the foreskin
 Signs:
 severe  impede the urinary flow  bulging of the foreskin during
urination
 Complication: Normal erections and manual retraction  scarring &
infection (posthitis & balantitis)
 Th/ antibiotics and steroid cream + local hygiene  inflammation &
swelling resolve  circumcision

 Paraphimosis
 entrapment of a phimotic prepuce proximal to the coronal margin
 Signs:
 edema and swelling of the glans and foreskin
 Th/ Prompt reduction (+local anesthesia), dorsal slit or circumcision
Anomalies of the Testes and Scrotum
 Testicular torsion
  true emergency
 Epidemiology: 1 in 4000 boys and men younger than age 25;
between 3 and 20 years of age
 Etiology: high insertion of the tunica vaginalis on the
spermatic cord  testicular mobility  twisting
 Signs:
 Acute onset of pain associated with nausea and vomiting;
 Scrotal edema and erythema
 loss of the cremasteric reflex with a high-lying, horizontal testis
 secondary hydrocele
 Th/ infarcted or necrotic  testis removal; Immediate
exploration and detorsion and contralateral testicular fixation
 Hydrocele & hernia
 Inguinal bulges and scrotal masses (usually)
 Etiology: failure of the fusion and obliteration of the processus
vaginalis
 patency of the processus vaginalis:
 Small defects (peritoneal fluid)  hydrocele;
 Larger defects (bowel within the hernia sac )  hernia  ischemic injury
to the testis
 Signs: Communicating hydroceles  fluctuate in size, smaller
when supine and larger when erect
 Hydroceles transilluminate; hernias do not
 Hydroceles that persist after 1 year  surgery
 Inguinal hernias should be repaired when diagnosed
 Varicoceles
 : abnormal dilation and tortuosity of the testicular vein and
pampinoform plexus
 Epidemiology: 15% of adolescent boys, but unusual prior to
puberty
 Occur almost exclusively on the left side
 Asymptomatic
 Pathogenesis
 lack of internal spermatic vein valves  increased hydrostatic
pressure; engorgement of the veins  scrotal temperature > 
interfere with normal testicular development
 Th/ surgery performed at the first sign of decreased testicular
growth
Diagnosis of Abnormalities of Sex Differentiation
LO 2
 Menjelaskan hipospadia
Hypospadia
  a urethral opening that is on the ventral surface of the
penile shaft
 affects 1/250 male newborns
 incomplete development of the prepuce, called a dorsal
hood, in which the foreskin is on the sides and dorsal
aspect of the penile shaft and deficient or absent
ventrally
 Embryologic aspect
 Penile
  failure of the urogenital
folds to fuse on the ventral
surface of the developing
penis and form the spongy
urethra
 Scrotal
  failure of the labioscrotal
folds to fuse and form the
scrotum
Etiology
 Speculations
 multifactorial origin (i.e., genetic and
environmental factors are involved)
 in utero exposure to estrogenic or antiandrogenic endocrine-
disrupting chemicals (e.g., polychlorobiphenyls,
phytoestrogens)
Clinical manifestations
 Boys with hypospadia + undescended testis; inguinal
hernias (10%)
  may interfere with normal urination in the usual male
standing position
 boys with penoscrotal hypospadias  voiding
cystourethrogram  dilated prostatic utricle (remnant of
the müllerian system) 5-10%
 More proximal the opening  chordee
Physical examination

Glanular hypospadias Subcoronal hypospadias Penoscrotal hypospadias

Perineal hypospadias; Megameatal variant of Complete penoscrotal
partial penoscrotal hypospadias transposition with scrotal
transposition hypospadias
Differential diagnosis
 female virilization (congenital adrenal hyperplasia)
 mixed gonadal dysgenesis
 partial androgen insensitivity
 true hermaphroditism
Complication
 deformity of the urinary stream  ventral deflection or
severe splaying
 sexual dysfunction secondary to penile curvature
 infertility if the urethral meatus is proximal
 meatal stenosis (congenital)  uncommon
 cosmetic appearance
Treatment
 Management begins in the newborn period
 Circumcision should be avoided
 ideal age for repair  6-12 mo

 Most common procedure 

tubularized incised plate (TIP) repair
LO 3
 Menjelaskan undesensus testis
Undescended testis (Cryptorchidism)
 Failure to find the testis in the scrotum indicates that the
testis is undescended, absent, or retractile
 testicular descent occurs at 7-8 mo of gestation

 Epidemiology
 30% of premature male infants have an undescended
testis; incidence is 3.4% at term
 testes descend spontaneously during the first 3 mo of life
 6 mo the incidence decreases to 0.8%
 If it hasn’t descended until 4 mo  remain undescended
 testis has not descended or is not retractable (capable of
being drawn down)  cryptorchidism

 The undescended testis usually lies somewhere along the

normal path of its prenatal descent, commonly in the
inguinal canal
Classification
 abdominal (nonpalpable)
 peeping (abdominal but can be pushed into the upper
part of the inguinal canal)
 Inguinal
 gliding (can be pushed into the scrotum but retracts
immediately to the pubic tubercle)
 Ectopic (superficial inguinal pouch or, rarely, perineal)
Pathogenesis & embryologic factors
 Descent of testis  interaction between hormonal &
mechanical factors
 testosterone, dihydrotestosterone, mullerian inhibiting factor,
gubernaculum, intra-abdominal pressure, genitofemoral
nerve
 Development of testis (7-8 wk)  leydig cells produce
testosterone (10-11 wk)  differentiation of the wolffian
(mesonephric) duct into the epididymis, vas deferens,
seminal vesicle, and ejaculatory duct
 Testis at internal inguinal ring begin its process of decent
(32-36 wk)  gubernaculum distends the inguinal canal
 guides the testis into the scrotum
Clinical manifestation
 palpable just distal to the inguinal canal over the pubic
tubercle (most)
 a newborn phenotypic male with bilateral nonpalpable
testes, as the child could be a virilized girl with congenital
adrenal hyperplasia  disorder of sexual development
(suspected)

 Can be normal at birth  pathologic changes (6-12 mo)

 Delayed germ cell maturation,
 reduction in germ cell number,
 hyalinization of the seminiferous tubules,
 reduced Leydig cell number are typical
 “Acquired” or ascending undescended testes
 boy has a descended testis at birth, during childhood (4-10 yr)
 testis doesn’t remain in scrotum
 History of retractile testis
 Retractile testes (physiologic chryptorchidism)
 misdiagnosed as undescended testes.
 e/ cremasteric reflex (>1 yr boys)
 Exam: legs in a relaxed frog-leg position  testis manipulated
into the scrotum  (+)  monitored every 6-12 mo; may
develop into acquired undescended testis (risk: < 7 yr)
Examination
 child should be entirely undressed, to help him relax
 If the testis is nonpalpable use “soap test”
 Pulling on the scrotum might make an inguinal testis
palpable

 CT & MRI
Complication
 Infertility
 Testicular malignancy
 peak age: 15-45 yr  most common: seminoma (65%)
 associated hernia
 inguinal pain and/or swelling
 torsion of the cryptorchid testis
 inguinal pain and/or swelling
 psychologic effects of an empty scrotum
Treatment
 treated surgically no later than 9-15 mo of age
 Most testes can be brought down to the scrotum with an
orchiopexy (success rate 98%)
 inguinal incision, mobilization of the testis and spermatic
cord, and correction of the indirect inguinal hernia
 Hormonal treatment (infrequently)
 HCG, which stimulates Leydig cell production of testosterone
 luteinizing hormone–releasing hormone (LHRH)
 Saline testicular implant (Solid silicone “carving block”
implants)  for anorchia
References
 Dalley, Arthur F. Keith L Moore. Clinically Oriented
Anatomy. 5th edition. Lippincott Williams & Wilcins; 2006
 Nelson Textbook of Pediatric, 19th edition
 Rudolph’s pediatric, 21st edition