Jose Cortez MD George Seremetis MD Danielle Sweeney MD

Jose Cortez MD
George Seremetis MD
Danielle Sweeney MD
Amanda Hodge
CPNP
Sarah Bushner CPNP
Doctor’s Line 512-474-6642
Page Operator 512-406-3112
Common Problems In
Pediatric Practice
Penile Problems and Hypospadias
Scrotal Pain,Swellings and Undescended Testes
Urine Infections, Voiding Dysfunction and
Incontinence
Hydronephrosis, Obstructions and Ureteral Reflux
Complex Problems
not covered today
Ano-Rectal Malformations
Cystic Kidneys
Spina Bifida and Spinal Cord disorders
Disorders of Sexual differentiation
Exstrophy
Urogenital Sinus/Vaginal and Uterine Anomalies
RhabdoMyoSarcoma/Wilms Tumor/Gonadal Tumors
Genital Problems in Boys
Outline
Foreskin problems
Circumcision problems
Penile Angulation/Chordee
Concealed Penis
Hypospadias
Foreskin Normal Development
Normal and Supple
No Cicatrix
Normal
Physiologic Adhesions
Ease of Retractibility Increases with Age
90% can be retracted by Age 3 years
Boys > 4-5 years and/or those with Balanitis and/or Cicatrix
Treat with .05% Betamethasone Cream t.i.d. x 4 wks
Consider Circ or Preputioplasty if Recurrent Problems
Early Forceful Retraction is
NOT recommended
True
Congenital Phimosis
Foreskin
Problems
cicatrix
Para-Phimosis
Secondary Phimosis
Rx: Manual
Reduction
Rx: Betamethasone
Balanitis
Bacterial Colonization/UTI
BXO-Balanitis Xerotica Obliterans
Consider Circumcision or Preputioplasty for
recurrent problems
Rx: Antifungal
Circumcision
Problems
Newborn Circ
Post-Circ Adhesions
Recurrent Phimosis
Buried/Trapped Penis
Penile Inclusion Cysts
Skin Chordee/Tethering
Meatal Stenosis
Early Post-Circumcision Retraction is
Recommended!
Circ Problems
Circ Revision
Coronal adhesions
Dense Adhesions
Buried Penis
Secondary Phimosis
Inclusion
Cyst
Meatal Stenosis
Urethromeatoplasty
Meatal Stenosis
Meatotomy
Meatoplasty
Slit-like Meatus
Penile Angulation/Chordee
With or Without
Hypospadias
Skin Tethering/Torsion
Corporal Disproportion
Urethral Hypoplasia
Hypospadias Associated Fibrous Chordee
Correction of Penile Angulation
Dorso-lateral Curve
>30 degrees
Plication
Saline Erection
after De-gloving
Correction Confirmed
Corporal Dissection
Functional Result
Concealed Penis
Post-Circumcision Scarring
Mega-Prepuce/Obstructive Phimosis
Peno-Scrotal Webbing
Prominent Pre-Pubic
Fat
Normal !
Retracts with normal Circ Margin
Normal !
Concealed Penis Repair
MegaPrepuce
Floating Junction/Uncircumcised
Hypospadias
Exam Points
Identify by Meatal Position
Dorsal Hood, Penile Angulation,
Scrotal development
Absence of Palpable Gonads
Should Prompt Urgent Evaluation
for Possible Disorder of Sexual
Differentiation (DSD)
Imaging not usually needed for
most
Referral in the first few months
Timing of Surgery - Ideally
between 6 - 12 months
Mega-Meatus Intact Prepuce Hypospadias
**Retract Foreskin Completely Before Circ !**
Basic Goals of Hypospadias Repair
Correct Curvature
Create a Urethral Conduit
Create Meatus
Form Glans
Skin Re-Positioning over Shaft
•
Circumcision or Reconstruct Foreskin
•
Interpose Flap
between Urethra and
Skin
Most One-Stage Repairs
•
> 95% success
Urethral Tube Created
from Foreskin
Dorsal Hooded Penile Skin
Urethral Meatus at Scrotum
after Excision of HypoPlastic Urethra
Island Tube
Island Flap
Staged Repair for Complex Hypospadias
Buccal Graft
from Inner Lip
3 wks later
7 months later
Distal Repairs
One-Stage / 1-2 Hours / Outpatient
General Anesthesia/Caudal Block
Meatal Advancement
Tubularization
Flaps
Functional and Cosmetic
Proximal repairs
Usually Severe Ventral Curvature
Deficient Preputial and Penile Skin
Small penis/glans
Associated Scrotal Anomalies
One versus Two Stage Repair
3 - 6 hour surgery
More Complex/More Potential for Post-op Problems
Single Stage Proximal
Repairs
Flaps with blood supply attached
Island Onlay-Single and Double
Tube Flaps
TPIF
Koyanagi
Two-Stage Repairs
Usually for Scrotal/Perineal Scrotal Hypo’s with Small
Penis and little skin to work with
Curvature Correction-Dermal or Tunica Vaginalis
Grafting
Repositoning of Preputial Skin to Ventrum to be
tubularized as a second Stage
Augment with Free Graft ( Buccal )
Wait 6 months then proceed with Tubularization to
create Urethral Conduit
Complications of Hypospadias
Repair
Bleeding and or Hematoma
Wound Infection/Separation/impaired healing
Fistula
Stenosis
Diverticulum
Recurrent Chordee
Intra-Urethral Hair Growth
Acute Scrotum
Acute Scrotal Pain, Tenderness,
Redness or Swelling at any Age
Requires Prompt Evaluation &
Differential Diagnosis
Consider Spermatic Cord Torsion
Testis torsion is a Surgical
Emergency and requires Immediate
Surgical Exploration
Irreversible Ischemic Injury may occur
as soon as 4 hours after onset
Other Diagnoses
Can be indistinguishable from Spermatic Cord Torsion
Torsion of Appendix Testis
Epididymitis/Orchitis/UTI
Acute Hydrocele or Inguinal Hernia
Varicocele/Spermatocele
Vasculitis of Cord/Testis-Hoenoch-Schonlein Purpura
Scrotal Wall Edema/Trauma/ Insect Bites
Tumors of the Testis / Paratesticular
Referred Pain
Exam Points
Size, Shape,Consistency
Position
Cord
Check Cremasteric
Reflex
Check Asymptomatic
Side First
Spermatic Cord Torsion likely
if:
Peri-Pubertal and Pubertal Boys (infants
less frequent)
Pain- Usually Abrupt Onset, Awaken
from Sleep, or associated Trauma,
Athletics
Nausea, Vomiting, Ipsi-lateral Abdominal
Pain
Previous Episodes of Abrupt Onset
Severe Self-Limited Pain/Swelling
Dysuria and/or voiding symptoms
usually absent
Exam Findings that Suggest Spermatic Cord Torsion
Torsion in an Adolescent
*Absence of Cremasteric Reflex*
Transverse Lie
Bell-Clapper
Deformity
Bell-Clapper
Abnormal Testicular Position-Tranverse Lie
Torsion in an Infant
Firm, Hard, Tender Testis
Anatomic Landmarks- indistinguishable
Torsion of Testicular/Epididymal
Appendages
Gradual Onset Pain, less severe,
over several days
Appendages
Blue Dot Sign
Pain mostly with pressure or
movement (walking)
Typically sitting up, alert, in no
distress, ‘walking funny’
Fever/Nausea/Vomiting/Dysuria
unusual
Scrotal redness, ‘Blue Dot sign’ variable
Tenderness usually over the
superior pole
Can be indistinguishable from
Spermatic Cord Torsion
Red,Swollen,
Tender, Firm
Infarcted
Appendix
Epididymitis/Orchitis
Usually Reactive and not infection in pre-pubertal
non-sexually active
Swelling, Redness and Pain
Can be indistinguishable from Spermatic Cord
Torsion
Consider Infection if Fever, dysuria or Risk Factors
Check Urine!!!
Urethral Stricture?
Vasal or Ureteral Ectopia?
Epididymal Abscess
Proteus UTI
Hydroceles
Simple Hydrocele -present at birth
and constant but
decreasing........follow for 1-2 years
until resolution
Communicating Hydrocele
Inguinal Hernia
Inguinal Hernia Repair
1.Supra-inguinal Incision
2.Cord Dissection
3.Omentum
in Hernia Sac
4.Trans-inguinal
Laparoscopy
Consider Spermatic Cord
Torsion When Evaluating Scrotal
Redness,Pain and/or Swelling
Children’s Urology Doctor’s Line 512-474-6642
Mon- Fri between 8 am and 5 pm
Varicocel
e
Ectatic/Tortuous veins of the spermatic cord
15% male adolescents
Most are Asymptomatic
Examine in Supine then Standing Position
Adverse Effects: Testis Growth failure,
Leydig Cell Dysfunction, Histologic
changes, Semen Abnormalities
Indications for Surgery : Testis Volume
Loss > 2 cc
Varicocele Ablation
Options
Sub-Inguinal / Inguinal / High
Retroperitoneal
Selective Venography / Percutaneous
Embolization
Laparoscopic
Persistence / Recurrence Rates about
same - 10-15%
Hydrocele most common post-op
Problem
Testis Tumors
1%-2% of all pediatric solid tumors
Incidence: 1 per 100,000 < 15 y
Bi-modal distribution: peak at 2
years/then at puberty
Blacks and Asiatic Children : very
rare
74% are Benign if pre-pubertal Teratoma/Epidermoid/Gonadal Stromal
Intersex / DSD are significant risk
factors
Pre-Pubertal Testis Tumors
Germ Cell:
Teratoma, Yolk Sac, Seminoma
Gonadal Stromal: Leydig , Sertoli , Juvenile
Granulosa
GonadoBlastoma
Lymphomas/Leukemias
RhabdoMyosarcoma
Epidermoid Cysts
Pre-Pubertal Testis Tumors
Firm Hard Painless Non-Transilluminating Scrotal
Mass is the most common finding
Color Doppler Ultrasound
Alpha Feto-Protein (AFP), HCG level
Testis Sparing Surgery
Radical Orchiectomy
Undescended Testes
Exam Points
Infant Referred for Bilateral UDT
No
No
No
Proper Exam is Key
Yes
*
Palpate Along Path of
Descent
Check Ectopic Sites
Check Penis,Scrotum
and Meatus
Imaging less Reliable
Yes
Yes
Undescended Testis
(UDT)
3% of Term Newborns
30% of Preemies (SGA, LBW < 2500 g, twins)
Usually Unilateral/Epididymal Anomalies common
20% Intra-Abdominal / nonpalpable
80% Inguinal or Ectopic / palpable
70-77% descend by 3 months
Testis Embryology and
Descent
Week 7- SRY Gene on Short Arm of the Y-Chromosome initiates
Testis Differentiation
Sertoli, Leydig and Germ Cells Develop
Gubernaculum Appears
Week 8- Testosterone, Mullerian Inhibitory Substance initiates and
sustains normal male development
In the Fetus, Testicular Descent occurs in 10% at 24 weeks, 50% at
27 weeks, 75% at 28 weeks and 80% after 34 weeks
Gonadal Descent
Testicular Descent normally
complete by 32 weeks and
requires Normal
Hypothalamic-PituitaryGonadal Axis, Testosterone
and DHT
Gubernaculum has a role/
but does not pull the testis
into the scrotum
Intra-Abdominal Pressure?
probably important for
Inguinal Phase
Important Long Term Considerations
Decreased Fertility
Newborn intra-abdominal UDT’s have normal Germ Cell Numbers
By Age 1 - 2 years UDT’s have Decreased Leydig Cells, Abnormal Sertoli
Cells and Reduced Germ Cell Counts
These findings may be present in the Normal Contralateral Testis
Paternity is Significantly Lower with Bilateral but Not Unilateral UDT
Neoplasia
Increased Risk for Testis Malignancy After Puberty
Most Common Tumor in a UDT is a Seminoma
Early Orchiopexy < 1 year May Protect
Even with Successful Early Orchiopexy must monitor with Testis Self
Exam
Management Of UDT
Orchiopexy between 6 - 12 months age
Orchiectomy considered for Post-Pubertal UDT when
normal contralateral testis is descended
Hormonal Therapy- exogenous HCG or GnRH
Standard Inguinal Exploration for Palpable UDT
Laparoscopic for Intra-Abdominal UDT
Fowler-Stephens and Staged Procedures
Orchiopexy
Laparoscopic Orchiopexy
Bilateral Non-Palpable
UDT
Consider DSD (Intersex) if Hypospadias or any
degree of Genital Ambiguity is present
(CAH work-up : 17-OH Progesterone , ASD , DHEA-S , Cortisol)
Most are XY Boys with otherwise Normal Genital
Development
Sonogram to evaluate for Mullerian Structures and
Gonads
Karyotype, FSH , Testosterone , MIS , Inhibin B
HCG Stimulation Test
Learning Objectives
The learner should be able to:
identify Common Penile Problems and Hypospadias
and illustrate their management.
assess Scrotal Pain, Redness and Swellings and
recognize problems that require urgent evaluation by
a Pediatric Urologist.
diagnose an Undescended Testis and describe
management and Long term morbidity.
list our Contact numbers for Urgent or Emergent
Consultation
Jose Carlos Cortez MD
Pediatric Urology Subspecialty Certification, American Board of Urology
Diplomate, American Board of Urology
Subspecialty Fellow, American Academy of Pediatrics
Doctor’s Line 512-474-6642
Page Operator 512-406-3112
Caring for the Children of the Austin Community and Central Texas since 1992
Thank You