One3 44 Management of abnormalities of external genitalia in boys phimosis, torsion

  1. Normal penile size?
    • The normal penile size in a full-term male neonate is 3.5 ± 0.7 cm in the stretched length
    • Micropenis <1.9 cm in neonates

    [@ ३६ से, १९ से] 
  2. Tanner stage?

    • - Pubic hair 
    • - Penis 
    • - Testis

    [@ PTH - Tetany - Tanners] 
  3. Classification of phimosis?
    Primary - with no sign of scarring

    Secondary (pathological) - due to scarring such as balanitis xerotica obliterans (BXO)
  4. Physiologic phimosis?
    Phimosis has to be distinguished from normal agglutination (adhesion) of the foreskin to the glans, which is a more or less lasting physiological phenomenon with clearly-visible meatus and partial retraction.

    Partial or complete inability to retract the prepuce exists because of natural adhesions between the glans and inner preputial skin and/or a preputial ring.

    • Two factors are involved in the separation of the prepuce from the glans:
    • (1) accumulation of smegma (epithelial debris) under the prepuce during the first 3 to 4 years of age and
    • (2) intermittent penile erections.

    Preputial retractability increases with age, with 90% of uncircumcised boys 3 years of age with completely retractable prepuces and less than 1% by 17 years of age with phimosis. Forceful preputial retraction should be discouraged to avoid cicatrix formation
  5. Management of phimosis?
    • Primary phimosis
    • - Conservative treatment is an option for primary phimosis.
    • - A corticoid ointment or cream (0.05-0.1%) can be administered twice a day over a period of 4-8 weeks with a success rate of > 80% (Strong, EAU 2021). This treatment has no side effects and the mean bloodcortisol levels are not significantly different from an untreated group of patients. Adhesion of the foreskin to the glans does not respond to steroid treatment.
    • - Operative treatment of phimosis in children is dependent on the caregivers’ preferences and can be plastic or radical circumcision after completion of the second year of life.
    • - recurrent balanoposthitis and recurrent urinary tract infections (UTIs) in patients with urinary tract abnormalities are indications for surgical intervention (Strong, EAU 2021).

    • Secondary phimosis
    • - circumcision (Strong, EAU 2021)
    • - Male circumcision significantly reduces the bacterial colonisation of the glans penis with regard to both non-uropathogenic and uropathogenic bacteria.
    • - Simple ballooning of the foreskin during micturition is not a strict indication for circumcision.
    • - Routine neonatal circumcision to prevent penile carcinoma is not indicated.
    • - Contraindications for circumcision are: an acute local infection and congenital anomalies of the penis, particularly hypospadias or buried penis, as the foreskin may be required for a reconstructive procedure.

    • Elective circumcision AAP guideline -
    • AAP - Although health benefits [significant reductions in the risk of UTI, risk of heterosexual acquisition of human immunodeficiency virus, and the transmission of other sexually transmitted infections] are not great enough to recommend routine circumcision for all male newborns, the benefits of circumcision are sufficient to justify access to this procedure for families choosing it and to warrant third-party payment for circumcision of male newborns”
  6. Complications of circumcision?
    Wound infections, meatal stenosis, removal of too much/too little prepuce, cicatrix, and even death are all potential complications of neonatal circumcision.
  7. What is paraphimosis?
    Paraphimosis must be regarded as an emergency situation: retraction of a too narrow prepuce behind the glans penis into the glanular sulcus may constrict the shaft and lead to oedema of the glans and retracted foreskin. It interferes with perfusion distally from the constrictive ring and brings a risk of preputial necrosis.

    • Treatment
    • - manual compression of the oedematous tissue with a subsequent attempt to retract the tightened foreskin over the glans penis
    • - Injection of hyaluronidase beneath the narrow band or 20% mannitol may be helpful to release the foreskin
    • - If this manoeuvre fails, a dorsal incision of the constrictive ring is required.
    • - Depending on the local findings, a circumcision is carried out immediately or can be performed in a second session
  8. Effman urethral duplication classification?
    • Types I and II distinguish between partial and complete urethral duplication
    • Type III - urethral duplication as part of bladder duplication

    • The accessory urethra should not be used as the primary urethra secondary to being hypoplastic with the risk of inadequate urine flow. 
    • Surgical repair includes complete accessory tract excision, electrofulguration or injection of sclerosing agents into the accessory tract, septotomy if the septum between the two urethras is thin, and urethrourethrostomy of the accessory tract into the functional urethra.
  9. Acute intravaginal torsion of testis?
    • Predisposing factors
    • - bell clapper deformity
    • Image Upload 1
    • - Cold temperature activating cremesteric reflex 
    • - rapid testicular growth
    • - Cryptorchid testis are at increased risk of torsion

    • Clinical features 
    • - after 10 years, peak at 12-16 years 
    • - generalized testicular tenderness 
    • - Inspection may identify the high-riding testis from a foreshortened cord and horizontally oriented testis.
    • - absent cremeseric reflex (genitofemoral reflex arc) - normally present after age 2 years, is elicited by scratching the inner thigh with resultant testis elevation

    • Diagnosis 
    • -  CDUS - reduced or absent Doppler color or waveforms and parenchymal heterogeneity compared with the contralateral testis.  Testes with heterogeneous echogenicity are necrotic,
    • whereas homogeneous echogenicity predicted a lower risk of orchiectomy

    • The two most important determinants of early salvage rate of the testis are
    • - time between onset of symptoms and detorsion
    • - degree of cord twisting

    Severe testicular atrophy occurred after torsion for as little as four hours when the turn was > 360°. In cases of incomplete torsion (180-360°), with symptom duration up to twelve hours, no atrophy was observed. However, a necrotic or severely atrophied testis was found in all cases of torsion > 360° and symptom duration > 24 hours.
  10. Management of torsion of testis?
    • - Urgent surgical exploration is mandatory in all cases of testicular torsion within 24 hours of symptom onset. In patients with testicular torsion > 24 hours, exploration may be performed as a semi-elective exploration procedure, unless there is a clear history of torsion-detorsion in which urgent exploration should still be considered. In case of prolonged torsion (> 24 hours) it is still subject to debate whether the surgically detorsed testis should be preserved. An alternative to detorsion and fixation may be to perform orchiectomy.
    • - Intermittent intravaginal spermatic cord torsion - requires elective bilateral orchidopexy. Three-point fixation in medial, lateral and in inferiorly. Use non-absorbable suture. (C12-896) Fixation in tunica albuginea and not in vaginalis. Orchiectomy is performed by dividing the cord into segments, each of which is ligated with nonabsorbable suture.
    • - - Manual detorsion - medial to lateral (like opening the book) 
    • [@ Book open - vascularity opens, Book closes - vascularity closes] 
    • - Manage torsion of the appendix testis conservatively. Perform surgical exploration in equivocal cases and in patients with persistent pain (Strong, EAU 2021)

    • Complications 
    • - testicular compartment syndrome 
    • - poor fertility
  11. Testicular compartment syndrome?
    Intratesticular pressure elevated after prolonged torsion with tense congestion. 

    Treated with tunica vaginalis vascularized patch - used to fill the defect in tunical albuginea
  12. Torsion in neonates - extravaginal spermatic cord torsion ?
    • Torsion in the newborn period is extravaginal, which involves twisting of the tunica vaginalis and the spermatic cord contained within.
    • Torsion of the entire cord occurs before fixation of the tunica vaginalis and dartos within the scrotum (extravaginal). 
    • Extravaginal torsion may be categorized into two separate phenomena, prenatal torsion (in utero) and postnatal torsion (acute) (during the first 30 days of life). This distinction is important because the salvage rate of a prenatally torsed testis is nearly nonexistent, whereas a postnatal extravaginal torsion carries a salvage rate of up to 44%.

    • Prenatal torsion 
    • - enlarged discolored scrotum accompanied by a nontender, solid testis that is fixed to the scrotal wall.
    • - testis will likely appear hyperechoic and avascular.  
    • - contralateral orchidopexy may be performed on an urgent basis to avoid the devastating consequence of asynchronous contralateral torsion.
    • - The risk for extravaginal torsion is thought to be present for only the first few weeks of life, and intervention should be pursued  before this window closes.

    • Postnatal torsion 
    • - postnatal change in scrotal examination (erythema and/or swelling), and the testis will be tender.
    • - management should be similar to what is recommended for acute testicular torsion that appears later in life, recognizing
    • that the timing of presentation varies.
    • - The decision to proceed with immediate surgical exploration should be measured in which the parents are aware of the
    • low salvage rate for the affected testis
    • - The main rationale behind intervention for postnatal torsion is the prevention of asynchronous contralateral torsion.
  13. Whirlpool sign?
    Defined as the presence of a spiral-like pattern when the spermatic cord is assessed during ultrasonography (US), using standard, high-resolution ultrasonography (HRUS) and/or color Doppler sonography (CDS), in the presence of testicular torsion

    Image Upload 2
  14. Most common cause of acute scrotum in prepubertal children?
    Appendage torsion (Appendix of testis or epididymis) 

    Blue dot sign (discoloration of upper pole of testis) seen through stretched scrotal skin 

    It is self limiting - no treatment required
  15. Signs of torsion testis?
    • Prehn sign - relief of pain with elevation of testicle, positive in epididymorchitis 
    • Affected testicle lies high up (Deming’s sign; 26-80%)
    • The horizontal lie of the contralateral testicle (Angell’s sign; 25-90%)
    • Anterior position of epididymis rather than posterior
    • Absent cremasteric reflex (40-100%), cremasteric reflex should be present in epididymitis.
  16. Cremesteric reflex?
    • Nerve supply 
    • Image Upload 3

    The presence of a cremasteric reflex correlates with intact testicular blood flow but does not unequivocally indicate normal testicular perfusion, especially if the clinical presentation is otherwise suggestive of torsion. 

    The absence of a cremasteric reflex (light touch to inner thigh resulting in cremasteric muscle contraction and ascension of the ipsilateral testicle) is a very specific sign for testicular torsion in pediatric patients.
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One3 44 Management of abnormalities of external genitalia in boys phimosis, torsion