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Undescended Testis / Cryptorchidism
Introduction
The word is from the Greek κρυπτός, kryptos, meaning hidden, and
ὄρχις, orchis, meaning testicle. It is the most common birth defect of the
male genital tract
Cryptorchidism is the absence of at least one testicle from the scrotum. It
is the most common birth defect involving the male genitalia.
About 3% of full-term and 30% of premature male infants are born with
one or both testicles undescended. Approximately 80% of cryptorchid
testes descend by the third month of life. This makes the true incidence
around 1%.
Cryptorchidism may occur on one or both sides, but more commonly
affects the right testicle.
The testicle may be anywhere along the "path of descent," such as:
 Located high in the retroperitoneal abdomen to the inguinal ring
 In the inguinal canal
 Ectopic from the path of descent
 Hypoplastic
 Dysgenetic
 Missing or Absent
 Unilateral (two-thirds)
EMBRYOLOGICAL FEATURES
a. Normally, the testes develop at 7 to 8 weeks gestation and
remain cephalad to the internal inguinal ring until about 28
weeks, when they begin their descent into the scrotum guided
by condensed mesenchyme (the gubernaculum). Onset of
descent is mediated by hormonal (eg, androgens, mullerian-
inhibiting factor), physical (eg, gubernacular regression, intra-
abdominal pressure), and environmental (eg, maternal exposure
to estrogenic or antiandrogenic substances) factors.
b. A true undescended testis remains in the inguinal canal along
the path of descent or is less commonly present in the
abdominal cavity or retroperitoneum. An ectopic testis is one
that descends normally through the external ring but diverts to
an abnormal location and lies outside the normal course of
descent (eg, suprapubically, in the superficial inguinal pouch,
within the perineum, or along the inner aspect of the thigh).
Epidemiology
 The prevalence of cryptorchidism is 30% in premature male
neonates.
 In the United States, cryptorchidism ranges from about 3% at birth
to 1% from 1 year to adulthood.
 Internationally, prevalence ranges from 4% to 5% at birth to about
1% to 1.5% at age three months and 1% to 2.5% at nine months.
 Cryptorchidism occurs in approximately 1.5% to 4% of fathers and
6% of brothers of individuals with cryptorchidism.
 Heritability in first-degree male relatives is estimated to be
approximately 0.5% to 1%.
 There may also be an association between cryptorchidism and
autism.
Risk factors include:
 Premature infants born before the descent of the testicles
 Small for gestational age infants
 Smaller placental weight
 Chemicals endocrine disruptors may interfere with normal fetal
hormone balance
 Maternal obesity
 Maternal diabetes
 Maternal exposure to DES
 Pesticides
 Alcohol consumption during pregnancy (5 or more drinks per
week, 3x increase)
 Cigarette smoking
 Family history
 Cosmetics use
 Exposure to phthalate (DEHP)
 Ibuprofen
 Preeclampsia (The more severe the preeclampsia, the greater
the risk of cryptorchidism)
 Congenital malformation syndromes - Down syndrome, Prader–
Willi syndrome, and Noonan syndrome
 Persistent Mullerian Duct Syndrome
 In vitro fertilization
Pathophysiology
a. A normal hypothalamic-pituitary-gonadal axis is a prerequisite for
normal testicular descent.
b. Birth weight appears to be the main risk factor for undescended
testes, followed by family history.
c. Absence of an appendix testis has been linked to abdominal testes
and cryptorchid testes especially if located proximal to the external
ring. The exact role of the appendix testis in testicular descent is
unclear.
d. In full-term infants, the cause of cryptorchidism often cannot be
determined, making this a common but sporadic, idiopathic birth
defect. It is thought that genetics, combined with maternal and
environmental factors, may disrupt hormones and physical changes
that influence testicular development and descent.
e. One contributing mechanism for the reduced function of
cryptorchid testes is temperature. It is also likely that transient
hormone deficiencies may lead to a lack of testicular descent and
impair the development of spermatogenic tissue.
Signs and symptoms
Infertility
Psychological Consequences
Boys with undescended testicles do not tend to be effeminate, gender-
disordered, or pre-homosexual. A disturbed self-image may occur when
the family dynamics are destructive toward male self-esteem.
Cancer
Overall, the risk of testicular cancer if orchiopexy is done before puberty
is around 2 to 3 times that of the general population. The most common
type of testicular cancer in untreated undescended testes is seminoma.
Complications
Orchiopexy is associated with two major testicular complications:
atrophy and testicular ascent
DIAGNOSIS
1-History collection
2-physical examination- From the AUA Guidelines: “In the hands
of an experienced provider, more than 70% of cryptorchid testes
are palpable by physical examination and need no imaging. In the
remaining 30% of cases with a nonpalpable testis, the challenge is
to confirm absence or presence of the testis and to identify the
location of the viable nonpalpable testis."
3-Ultrasound is non-contributory in routine use, with sensitivity and
specificity to localize nonpalpable testes at 45% and 78%,
respectively.
4- CT scanning
5-MRI with or without angiography has been more widely used with
greater sensitivity and specificity but is discouraged due to its cost,
low availability, and the need for anesthesia.
6-A karyotype can confirm or exclude dysgenetic primary
hypogonadism. Hormone levels such as gonadotropins and Anti-
Mullerian Hormone (AMH) may confirm hormonally functional
testicles worth salvation, as can stimulation with human chorionic
gonadotropin to elicit a rise in the testosterone level.
Treatment / Management
Medical Treatment
I. Hormonal Therapy
The American Pediatric Association Guidelines do recommend the use
of hormones for cases of undescended testis associated with Prader-Willi
Syndrome.
The most commonly used hormone is human chorionic gonadotropin
(HCG). The success rate is reported as 5% to 50%.
 Hormone treatment also will confirm Leydig cell responsiveness
and induce additional growth of a small penis due to a rise in
testosterone levels.
The cost of hormone treatment is less than surgery, and the chance of
complications are minimal.
II. Surgery
Orchidopexy
Surgery is recommended for congenital undescended testes between the
ages of 6 and 18 months (AUA Guidelines). For premature babies,
corrected age is used to determine surgery timing. Fertility is improved
if the orchidopexy is performed early. Patients with bilateral
undescended testes who receive orchidopexies as adults are almost
always infertile and azoospermic
Technique of Orchiopexy
For palpable undescended testes, an inguinal or scrotal orchiopexy is
recommended.
1. An incision is made in the high scrotum, median scrotal raphe,
high edge of the scrotum, or groin. Many different type of
retractors can be used depending of the size of the incision.
Inguinal incisions can be as small as 1 cm. Scrotal incisions can be
larger as they tend to heal concealed specially when in the median
raphe.
2. The testis can be approached first or the cord first; for scrotal
cases, the testis is found first. For an inguinal approach, the testis
can be approached first or the external oblique fascia opened
proximal to the external ring and the cord approached first.
3. When approaching the testis first, all the cremasteric muscles are
divided as well as everything not going into the external ring.
4. The more difficult part of the case is separating the hernia sac from
the vas and testicular vessels. This can be approached anteriorly or
posteriorly. The posterior approach is much easier to teach and
learn.
5. How the testis is positioned and secured in the scrotum varies.
Most would agree that a sub-dartos pouch is desirable. Some
surgeons do not suture the testis in place, others use absorbable
sutures, others non-absorbable, and others just close the passage
into the groin.
For nonpalpable testes under anesthesia, exploratory laparoscopy is
recommended. If a testis is found during exploratory laparoscopy, the
options are:
1. Laparoscopic orchiopexy preserving the vessels: the testis is
dissected off a triangular pedicle containing the gonadal vessels
and the vas.
2. Laparoscopic one stage Fowler Stevens (FS) orchiopexy: gonadal
vessels are divided and the testis is dissected off a pedicle of the
vas and brought down in one stage.
3. Laparoscopic two stage Fowler Stevens orchiopexy: vessels are
divided with clips but dissection of the testis is postponed for 6
months to allow for optimal development of collaterals.

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Undescended testtis

  • 1. Undescended Testis / Cryptorchidism Introduction The word is from the Greek κρυπτός, kryptos, meaning hidden, and ὄρχις, orchis, meaning testicle. It is the most common birth defect of the male genital tract Cryptorchidism is the absence of at least one testicle from the scrotum. It is the most common birth defect involving the male genitalia. About 3% of full-term and 30% of premature male infants are born with one or both testicles undescended. Approximately 80% of cryptorchid testes descend by the third month of life. This makes the true incidence around 1%. Cryptorchidism may occur on one or both sides, but more commonly affects the right testicle. The testicle may be anywhere along the "path of descent," such as:  Located high in the retroperitoneal abdomen to the inguinal ring  In the inguinal canal  Ectopic from the path of descent  Hypoplastic  Dysgenetic  Missing or Absent  Unilateral (two-thirds) EMBRYOLOGICAL FEATURES a. Normally, the testes develop at 7 to 8 weeks gestation and remain cephalad to the internal inguinal ring until about 28 weeks, when they begin their descent into the scrotum guided by condensed mesenchyme (the gubernaculum). Onset of
  • 2. descent is mediated by hormonal (eg, androgens, mullerian- inhibiting factor), physical (eg, gubernacular regression, intra- abdominal pressure), and environmental (eg, maternal exposure to estrogenic or antiandrogenic substances) factors. b. A true undescended testis remains in the inguinal canal along the path of descent or is less commonly present in the abdominal cavity or retroperitoneum. An ectopic testis is one that descends normally through the external ring but diverts to an abnormal location and lies outside the normal course of descent (eg, suprapubically, in the superficial inguinal pouch, within the perineum, or along the inner aspect of the thigh). Epidemiology  The prevalence of cryptorchidism is 30% in premature male neonates.  In the United States, cryptorchidism ranges from about 3% at birth to 1% from 1 year to adulthood.  Internationally, prevalence ranges from 4% to 5% at birth to about 1% to 1.5% at age three months and 1% to 2.5% at nine months.  Cryptorchidism occurs in approximately 1.5% to 4% of fathers and 6% of brothers of individuals with cryptorchidism.  Heritability in first-degree male relatives is estimated to be approximately 0.5% to 1%.  There may also be an association between cryptorchidism and autism. Risk factors include:  Premature infants born before the descent of the testicles
  • 3.  Small for gestational age infants  Smaller placental weight  Chemicals endocrine disruptors may interfere with normal fetal hormone balance  Maternal obesity  Maternal diabetes  Maternal exposure to DES  Pesticides  Alcohol consumption during pregnancy (5 or more drinks per week, 3x increase)  Cigarette smoking  Family history  Cosmetics use  Exposure to phthalate (DEHP)  Ibuprofen  Preeclampsia (The more severe the preeclampsia, the greater the risk of cryptorchidism)  Congenital malformation syndromes - Down syndrome, Prader– Willi syndrome, and Noonan syndrome  Persistent Mullerian Duct Syndrome  In vitro fertilization Pathophysiology
  • 4. a. A normal hypothalamic-pituitary-gonadal axis is a prerequisite for normal testicular descent. b. Birth weight appears to be the main risk factor for undescended testes, followed by family history. c. Absence of an appendix testis has been linked to abdominal testes and cryptorchid testes especially if located proximal to the external ring. The exact role of the appendix testis in testicular descent is unclear. d. In full-term infants, the cause of cryptorchidism often cannot be determined, making this a common but sporadic, idiopathic birth defect. It is thought that genetics, combined with maternal and environmental factors, may disrupt hormones and physical changes that influence testicular development and descent. e. One contributing mechanism for the reduced function of cryptorchid testes is temperature. It is also likely that transient hormone deficiencies may lead to a lack of testicular descent and impair the development of spermatogenic tissue. Signs and symptoms Infertility Psychological Consequences
  • 5. Boys with undescended testicles do not tend to be effeminate, gender- disordered, or pre-homosexual. A disturbed self-image may occur when the family dynamics are destructive toward male self-esteem. Cancer Overall, the risk of testicular cancer if orchiopexy is done before puberty is around 2 to 3 times that of the general population. The most common type of testicular cancer in untreated undescended testes is seminoma. Complications Orchiopexy is associated with two major testicular complications: atrophy and testicular ascent DIAGNOSIS 1-History collection 2-physical examination- From the AUA Guidelines: “In the hands of an experienced provider, more than 70% of cryptorchid testes are palpable by physical examination and need no imaging. In the remaining 30% of cases with a nonpalpable testis, the challenge is to confirm absence or presence of the testis and to identify the location of the viable nonpalpable testis." 3-Ultrasound is non-contributory in routine use, with sensitivity and specificity to localize nonpalpable testes at 45% and 78%, respectively. 4- CT scanning 5-MRI with or without angiography has been more widely used with greater sensitivity and specificity but is discouraged due to its cost, low availability, and the need for anesthesia. 6-A karyotype can confirm or exclude dysgenetic primary hypogonadism. Hormone levels such as gonadotropins and Anti- Mullerian Hormone (AMH) may confirm hormonally functional
  • 6. testicles worth salvation, as can stimulation with human chorionic gonadotropin to elicit a rise in the testosterone level. Treatment / Management Medical Treatment I. Hormonal Therapy The American Pediatric Association Guidelines do recommend the use of hormones for cases of undescended testis associated with Prader-Willi Syndrome. The most commonly used hormone is human chorionic gonadotropin (HCG). The success rate is reported as 5% to 50%.  Hormone treatment also will confirm Leydig cell responsiveness and induce additional growth of a small penis due to a rise in testosterone levels. The cost of hormone treatment is less than surgery, and the chance of complications are minimal. II. Surgery Orchidopexy Surgery is recommended for congenital undescended testes between the ages of 6 and 18 months (AUA Guidelines). For premature babies, corrected age is used to determine surgery timing. Fertility is improved if the orchidopexy is performed early. Patients with bilateral undescended testes who receive orchidopexies as adults are almost always infertile and azoospermic Technique of Orchiopexy For palpable undescended testes, an inguinal or scrotal orchiopexy is recommended. 1. An incision is made in the high scrotum, median scrotal raphe, high edge of the scrotum, or groin. Many different type of retractors can be used depending of the size of the incision.
  • 7. Inguinal incisions can be as small as 1 cm. Scrotal incisions can be larger as they tend to heal concealed specially when in the median raphe. 2. The testis can be approached first or the cord first; for scrotal cases, the testis is found first. For an inguinal approach, the testis can be approached first or the external oblique fascia opened proximal to the external ring and the cord approached first. 3. When approaching the testis first, all the cremasteric muscles are divided as well as everything not going into the external ring. 4. The more difficult part of the case is separating the hernia sac from the vas and testicular vessels. This can be approached anteriorly or posteriorly. The posterior approach is much easier to teach and learn. 5. How the testis is positioned and secured in the scrotum varies. Most would agree that a sub-dartos pouch is desirable. Some surgeons do not suture the testis in place, others use absorbable sutures, others non-absorbable, and others just close the passage into the groin. For nonpalpable testes under anesthesia, exploratory laparoscopy is recommended. If a testis is found during exploratory laparoscopy, the options are: 1. Laparoscopic orchiopexy preserving the vessels: the testis is dissected off a triangular pedicle containing the gonadal vessels and the vas. 2. Laparoscopic one stage Fowler Stevens (FS) orchiopexy: gonadal vessels are divided and the testis is dissected off a pedicle of the vas and brought down in one stage. 3. Laparoscopic two stage Fowler Stevens orchiopexy: vessels are divided with clips but dissection of the testis is postponed for 6 months to allow for optimal development of collaterals.