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Case Scenario
Presented by
Abdulaziz Bagasi – medical intern
Supervised by Dr.Badr BinHimd
December 2016
Case
• A 6-month-old healthy girl presents with a bulge at
her umbilicus since birth.
• No accompanying symptoms and has been growing
and developing normally.
• Physical examination of the abdomen reveals a soft,
non-tender bulge at the umbilicus that is easily
reduced into the peritoneal cavity with gentle
pressure.
• Reduction allows palpation of the abdominal fascia,
revealing an 8 mm fascial defect.
What do you think ?
Pediatric Umbilical Hernia
What questions we should always
ask about ?
 Swelling Hx. :
- Where is it ?
- When did you notice it ?
- How did you notice it ?
 Swelling Hx. :
- Does it disappears ?
- Is there any change in it’s size,
shape and color ?
- Did she had had a previous similar
case?
- Does she has any other lumps?
What complications we are afraid of ?
COMPLICATIONS
- Incarceration.
- Strangulation.
- Perforation.
Strangulated hernias are differentiated from
incarcerated hernias by the following:
1. Pain out of proportion to examination
findings.
2. Fever or toxic appearance.
Physical Examination
• Identify The hernia.
• Look for a mass in the area of the fascial defect.
• Define the borders of the fascial defect.
• The size of the fascial defect
• Generally asymptomatic , it may cause mild
discomfort in some children.
• If presents with tenderness, incarceration and
strangulation should be suspected.
Pediatric Umbilical Hernia
• Definition
• Anatomy
• Risk factors.
• Clinical features
• Complications
• Management
Definition
Umbilical hernia :
Part of the intestine or fatty tissue bulges
through the muscle near umbilicus.
Anatomy
-The fascial opening (umbilical ring) exists to allow passage of the umbilical
vessels from the mother into the fetus.
-After birth, this fascial opening closes spontaneously with continued growth
of the rectus abdominis muscles toward one another.
-Closure of the umbilical ring is complete in almost all children by five years of
age.
Risk factors
• Incomplete closure of umbilical opening.
• Premature babies.
• Black infants.
• In patients with Ehlers-Danlos ,Beckwith-Wiedemann
syndrome ,Down syndrome, mucopolysaccharidoses ,
hypothyroidism ,or trisomy 18.
• Increased intraabdominal pressure from ascites or crying .
Clinical features
-Asymptomatic.
- Interfere with feeding if contain bowel wall .
Management
(Infant)
• Observation and reassurance as these defects
typically close by age 4 or 5 years.
• Rarely incarcerated.
What are the indications for referral?
indications for referral
1-More than 1.5 cm defect.
2- Bowel incarceration or strangulation.
3- if persistant more than 5 years of age.
4-Children with large, proboscoid (trunk-like).
5-Signs of infection.
Proboscoid hernia warranting early
repair
Case
• A 6-month-old male is brought in for a routine
checkup.
• Only one testicle is palpable.
• The genital examination is otherwise within
normal limits.
Which one of the following would be most
appropriate at this time?
-Introduction
-Normal Testicular Descent
-Terminology
-Risk factors
-Associated conditions
-Complications
-Management
Undescended testes (cryptorchidism)
-The most common genital disorder identified at birth.
-It is important to treat b/c increased risks of impairment of
fertility potential, testicular malignancy, torsion and/or
associated inguinal hernia.
-Between 2 and 5 percent of full-term and approximately 30
percent of premature male infants are born with an
undescended testis.
Normal Testicular Descent
Terminology
1)Cryptorchidism
a)True Undescended testes
b)Absent testis
2)Retractile testes
3)Ascending testes
4)Ectopic testes
1)Cryptorchidism : a testis that is
not within the scrotum and does
not descend spontaneously into the
scrotum by four months of age
b)Absent testis – An
absent testis may be due
to agenesis or atrophy
a)True Undescended
testes have stopped short
along their normal path of
descent into the scrotum
2)Retractile testes –are
normal testes that have been
pulled into a suprascrotal
position by the cremasteric
reflex.
3)Ascending testes –are
noted to be in a scrotal
position in early childhood and
then to "ascend" and become
undescended
4)Ectopic testes – Ectopic
testes descend normally
through the external ring
but then are diverted to an
aberrant position
-The testicle moved by hand from
the groin into the scrotum and
won't immediately retreat to the
groin.
-Spontaneously appear in the
scrotum and remain there for a
time , then disappear again for a
time .
Retractile testicle is different from
undescended testicle is that
undescended never entered the
scrotum.
Undescended testes
1- abdomen
2- inguinal canal
3-suprascrotal(prepubic) .
Ectopic testes
4-suprapubic (penile)
5-femoral region
6-perineal region
7- contralateral
hemiscrotum
Laparoscopic view of intraabdominal
testis
Bilateral suprascrotal undescended
testes
Right ectopic testis palpable in the
perineum.
Bilateral cryptorchidism
Risk factors
• Prematurity
• Small for gestational age at birth.
• Prenatal exposure to endocrine disruptors (eg,
diethylstilbestrol, pesticides).
Associated conditions
●Abdominal wall defects
●Neural tube defects
●Cerebral palsy
●Disorders of sexual development (eg, mixed gonadal
dysgenesis, ovotesticular disorders of sex
development, persistent Müllerian duct syndrome
●Genetic disorders causing diminished testosterone
secretion (eg, Kallmann syndrome, Klinefelter
syndrome, Prader-Willi syndrome)
What complications we are afraid of ?
Complications :
• Inguinal hernia : 90 percent of congenital undescended testes
have an associated patent processus vaginalis
• Testicular torsion:10 times more common in undescended testis.
• Testicular trauma
• Subfertility: increased incidence of lower sperm counts and lower
fertility rates
• Testicular cancer : incidence is approximately 5.4 per 100,000
Imp. Historical Points
• Whether or not the testes were in a scrotal location since
birth so Undescended (not retractile).
• Evidence of endocrine abnormalities during pregnancy (eg,
maternal androgen exposure, which may result in significant
virilization of a female fetus)
• Family history of unexplained neonatal deaths or genital
anomalies, or infertility (may indicate an associated genetic or
endocrinologic abnormality)
In physical examination
• The testicular examination in the infant and young
child requires two hands.
• One hand is placed near the anterior superior iliac
spine and the other on the scrotum
• The first hand is swept from the anterior iliac spine
along the inguinal canal to gently express any
retained testicular tissue into the scrotum
If patient come to you with undescended tesis
Palpable
DD.
True
undescend
ed testis
-In inguinal
canal .
- Since birth
Retractile
testis
spontaneously
appear in the
scrotum and remain
there for a time ,
then disappear again
.
Follow up
should be
examined
annually until
the outcome
of descent or
nondescent
becomes clear
Ectopic
testis
away from
undescended testes
(eg, suprapubic,
perineal)
Non-Palpable
DD.
True
undescended
testis
Absent testis
-agenesis
-intrauterine vascular
compromise.
Ectopic testis
4 Case Scenarios
1) Bilateral Palpable
2) Bilateral non- Palpable
3)Unilateral non-Palpable
4)Unilateral Palpable
1) Bilateral
Palpable
newborn
Refer at 6 month
for surgary
Older child (after
infancy)
Immediate
surgary
2) Bilateral
non-
Palpable
newborn
Referral for
evaluation of
sexual
disorders
Older child
(after
infancy)
Hormonal
evaluation for
testicular
absence
surgical
exploration
3)Unilateral
non-Palpable
newborn
With
hypospadia
Referral for
evaluation of
sexual
disorders
Normal
genitalia
Refer at 6 month
for surgary
Older child
(after
infancy)
Surgary
4)Unilateral
Palpable
newborn
Refer at 6 month
for surgary
Older child (after
infancy)
Immediate
surgary
What are the indications for referral?
INDICATIONS FOR REFERRAL
• Congenital palpable or nonpalpable undescended
testes (unilateral or bilateral); referral between 6 and
12 months of age is recommended
• Ascending testis in boys beyond infancy
• Palpable tissue in the scrotum that is thought to be
an atrophic testis
• Difficulty differentiating between undescended,
retractile, or ectopic testis (at any age)
Case
A 6-month-old male is brought in for a routine
checkup. Only one testicle is palpable. The genital
examination is otherwise within normal limits.
Which one of the following would be most
appropriate at this time?
A) Observation only, until 18 months of age
B) Abdominal ultrasonography
C) Urologic referral for surgical exploration
D) HCG treatment for 3 months
Guideline statement
• Providers should refer infants with a history of cryptorchidism
(detected at birth) who do not have spontaneous testicular
descent by six months to an appropriate surgical specialist for
timely evaluation. (Grade B)
• Providers must immediately consult an appropriate specialist
for all phenotypic male newborns with bilateral, nonpalpable
testes for evaluation of a possible disorder of sex
development (DSD). (Grade A)
Guideline statement
• Providers should not perform ultrasound (US) or other
imaging modalities in the evaluation of boys with
cryptorchidism prior to referral as these studies rarely assist in
decision making. (Grade B)
• In boys with retractile testes, providers should assess the
position of the testes at least annually to monitor for
secondary ascent. (Grade B)
Guideline statement
• Surgical treatment as soon as possible after six months of age
for congenitally undescended testes and completed before
the child is two years old (ideally before one year) of age.
• In children with testicular ascent later in childhood, surgery
generally should be performed within six months of
identification.
• Treatment of undescended testes before puberty also may
reduce the risk of testicular cancer, and prevent testicular
torsion.
Umbilical hernia ,undescended testis
Umbilical hernia ,undescended testis

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Umbilical hernia ,undescended testis

  • 1. Case Scenario Presented by Abdulaziz Bagasi – medical intern Supervised by Dr.Badr BinHimd December 2016
  • 2. Case • A 6-month-old healthy girl presents with a bulge at her umbilicus since birth. • No accompanying symptoms and has been growing and developing normally. • Physical examination of the abdomen reveals a soft, non-tender bulge at the umbilicus that is easily reduced into the peritoneal cavity with gentle pressure. • Reduction allows palpation of the abdominal fascia, revealing an 8 mm fascial defect.
  • 3. What do you think ?
  • 5. What questions we should always ask about ?
  • 6.  Swelling Hx. : - Where is it ? - When did you notice it ? - How did you notice it ?
  • 7.  Swelling Hx. : - Does it disappears ? - Is there any change in it’s size, shape and color ? - Did she had had a previous similar case? - Does she has any other lumps?
  • 8. What complications we are afraid of ?
  • 10. Strangulated hernias are differentiated from incarcerated hernias by the following: 1. Pain out of proportion to examination findings. 2. Fever or toxic appearance.
  • 11. Physical Examination • Identify The hernia. • Look for a mass in the area of the fascial defect. • Define the borders of the fascial defect. • The size of the fascial defect • Generally asymptomatic , it may cause mild discomfort in some children. • If presents with tenderness, incarceration and strangulation should be suspected.
  • 12. Pediatric Umbilical Hernia • Definition • Anatomy • Risk factors. • Clinical features • Complications • Management
  • 13. Definition Umbilical hernia : Part of the intestine or fatty tissue bulges through the muscle near umbilicus.
  • 14. Anatomy -The fascial opening (umbilical ring) exists to allow passage of the umbilical vessels from the mother into the fetus. -After birth, this fascial opening closes spontaneously with continued growth of the rectus abdominis muscles toward one another. -Closure of the umbilical ring is complete in almost all children by five years of age.
  • 15.
  • 16. Risk factors • Incomplete closure of umbilical opening. • Premature babies. • Black infants. • In patients with Ehlers-Danlos ,Beckwith-Wiedemann syndrome ,Down syndrome, mucopolysaccharidoses , hypothyroidism ,or trisomy 18. • Increased intraabdominal pressure from ascites or crying .
  • 17. Clinical features -Asymptomatic. - Interfere with feeding if contain bowel wall .
  • 18. Management (Infant) • Observation and reassurance as these defects typically close by age 4 or 5 years. • Rarely incarcerated.
  • 19. What are the indications for referral?
  • 20. indications for referral 1-More than 1.5 cm defect. 2- Bowel incarceration or strangulation. 3- if persistant more than 5 years of age. 4-Children with large, proboscoid (trunk-like). 5-Signs of infection.
  • 22.
  • 23. Case • A 6-month-old male is brought in for a routine checkup. • Only one testicle is palpable. • The genital examination is otherwise within normal limits. Which one of the following would be most appropriate at this time?
  • 24. -Introduction -Normal Testicular Descent -Terminology -Risk factors -Associated conditions -Complications -Management
  • 25. Undescended testes (cryptorchidism) -The most common genital disorder identified at birth. -It is important to treat b/c increased risks of impairment of fertility potential, testicular malignancy, torsion and/or associated inguinal hernia. -Between 2 and 5 percent of full-term and approximately 30 percent of premature male infants are born with an undescended testis.
  • 27. Terminology 1)Cryptorchidism a)True Undescended testes b)Absent testis 2)Retractile testes 3)Ascending testes 4)Ectopic testes
  • 28. 1)Cryptorchidism : a testis that is not within the scrotum and does not descend spontaneously into the scrotum by four months of age b)Absent testis – An absent testis may be due to agenesis or atrophy a)True Undescended testes have stopped short along their normal path of descent into the scrotum
  • 29. 2)Retractile testes –are normal testes that have been pulled into a suprascrotal position by the cremasteric reflex. 3)Ascending testes –are noted to be in a scrotal position in early childhood and then to "ascend" and become undescended 4)Ectopic testes – Ectopic testes descend normally through the external ring but then are diverted to an aberrant position -The testicle moved by hand from the groin into the scrotum and won't immediately retreat to the groin. -Spontaneously appear in the scrotum and remain there for a time , then disappear again for a time . Retractile testicle is different from undescended testicle is that undescended never entered the scrotum.
  • 30. Undescended testes 1- abdomen 2- inguinal canal 3-suprascrotal(prepubic) . Ectopic testes 4-suprapubic (penile) 5-femoral region 6-perineal region 7- contralateral hemiscrotum
  • 31. Laparoscopic view of intraabdominal testis
  • 33. Right ectopic testis palpable in the perineum.
  • 35. Risk factors • Prematurity • Small for gestational age at birth. • Prenatal exposure to endocrine disruptors (eg, diethylstilbestrol, pesticides).
  • 36. Associated conditions ●Abdominal wall defects ●Neural tube defects ●Cerebral palsy ●Disorders of sexual development (eg, mixed gonadal dysgenesis, ovotesticular disorders of sex development, persistent Müllerian duct syndrome ●Genetic disorders causing diminished testosterone secretion (eg, Kallmann syndrome, Klinefelter syndrome, Prader-Willi syndrome)
  • 37. What complications we are afraid of ?
  • 38. Complications : • Inguinal hernia : 90 percent of congenital undescended testes have an associated patent processus vaginalis • Testicular torsion:10 times more common in undescended testis. • Testicular trauma • Subfertility: increased incidence of lower sperm counts and lower fertility rates • Testicular cancer : incidence is approximately 5.4 per 100,000
  • 39. Imp. Historical Points • Whether or not the testes were in a scrotal location since birth so Undescended (not retractile). • Evidence of endocrine abnormalities during pregnancy (eg, maternal androgen exposure, which may result in significant virilization of a female fetus) • Family history of unexplained neonatal deaths or genital anomalies, or infertility (may indicate an associated genetic or endocrinologic abnormality)
  • 40. In physical examination • The testicular examination in the infant and young child requires two hands. • One hand is placed near the anterior superior iliac spine and the other on the scrotum • The first hand is swept from the anterior iliac spine along the inguinal canal to gently express any retained testicular tissue into the scrotum
  • 41.
  • 42. If patient come to you with undescended tesis
  • 43. Palpable DD. True undescend ed testis -In inguinal canal . - Since birth Retractile testis spontaneously appear in the scrotum and remain there for a time , then disappear again . Follow up should be examined annually until the outcome of descent or nondescent becomes clear Ectopic testis away from undescended testes (eg, suprapubic, perineal)
  • 45. 4 Case Scenarios 1) Bilateral Palpable 2) Bilateral non- Palpable 3)Unilateral non-Palpable 4)Unilateral Palpable
  • 46. 1) Bilateral Palpable newborn Refer at 6 month for surgary Older child (after infancy) Immediate surgary
  • 47. 2) Bilateral non- Palpable newborn Referral for evaluation of sexual disorders Older child (after infancy) Hormonal evaluation for testicular absence surgical exploration
  • 49. 4)Unilateral Palpable newborn Refer at 6 month for surgary Older child (after infancy) Immediate surgary
  • 50. What are the indications for referral?
  • 51. INDICATIONS FOR REFERRAL • Congenital palpable or nonpalpable undescended testes (unilateral or bilateral); referral between 6 and 12 months of age is recommended • Ascending testis in boys beyond infancy • Palpable tissue in the scrotum that is thought to be an atrophic testis • Difficulty differentiating between undescended, retractile, or ectopic testis (at any age)
  • 52. Case A 6-month-old male is brought in for a routine checkup. Only one testicle is palpable. The genital examination is otherwise within normal limits. Which one of the following would be most appropriate at this time? A) Observation only, until 18 months of age B) Abdominal ultrasonography C) Urologic referral for surgical exploration D) HCG treatment for 3 months
  • 53. Guideline statement • Providers should refer infants with a history of cryptorchidism (detected at birth) who do not have spontaneous testicular descent by six months to an appropriate surgical specialist for timely evaluation. (Grade B) • Providers must immediately consult an appropriate specialist for all phenotypic male newborns with bilateral, nonpalpable testes for evaluation of a possible disorder of sex development (DSD). (Grade A)
  • 54. Guideline statement • Providers should not perform ultrasound (US) or other imaging modalities in the evaluation of boys with cryptorchidism prior to referral as these studies rarely assist in decision making. (Grade B) • In boys with retractile testes, providers should assess the position of the testes at least annually to monitor for secondary ascent. (Grade B)
  • 55. Guideline statement • Surgical treatment as soon as possible after six months of age for congenitally undescended testes and completed before the child is two years old (ideally before one year) of age. • In children with testicular ascent later in childhood, surgery generally should be performed within six months of identification. • Treatment of undescended testes before puberty also may reduce the risk of testicular cancer, and prevent testicular torsion.

Hinweis der Redaktion

  1. In children, umbilical hernias rarely become incarcerated (inability to be reduced by manipulation) or strangulated (vascular compromise of the contents of an incarcerated hernia) or even more rarely rupture.
  2. The fascial defect, not the degree of protrusion, is most indicative of whether spontaneous closure will occur. It is important to differentiate umbilical hernias from the less common abdominal wall (also called ventral or supraumbilical) hernias that do not close spontaneously
  3. Because the natural course of the umbilical ring is eventual closure, most umbilical hernias will spontaneously resolve. In general, asymptomatic children with an umbilical ring that continues to decrease can be observed. Surgical intervention is required only in a minority of patients.
  4. Because the natural course of the umbilical ring is eventual closure, most umbilical hernias will spontaneously resolve. In general, asymptomatic children with an umbilical ring that continues to decrease can be observed. Surgical intervention is required only in a minority of patients.
  5. (A) The testes appear on the urogenital ridge (second month). (B) The coelomic cavity evaginates into the scrotal swelling where it forms the processus vaginalis (middle of the third month). (C) Testes begin descent into the scrotum guided by the gubernaculum (seventh month). (D) The processus vaginalis obliterates spontaneously (shortly after birth).
  6. . They may be palpable in the superficial inguinal pouch (most common), suprapubic region, femoral canal, perineum , or contralateral scrotal compartment (least common)
  7. Congenital adrenal hyperplasia (CAH) ●An ovotesticular disorder of sex development (ie, presence of ovarian and testicular tissue in the same individual) ●Bilateral undescended testes ●Anorchia The evaluation includes measurement of serum testosterone, gonadotropins (LH, FSH), and MIS, and a human chorionic gonadotropin (hCG) stimulation test (to evaluate the presence and function of testicular tissue and elicit testosterone production in response to intramuscular administration of hCG) [32,49,68-70]. Elevated gonadotropins, absence of MIS, and undetectable or very low baseline testosterone level with little or no increase after hCG stimulation confirm the diagnosis of testicular absence. However, normal gonadotropin levels or detectable MIS warrant surgical exploration, even if the hCG stimulation test is negative,