Editing External Genital Anomalies in Boys (section)

== Penile Anomalies ==

=== Prepuce (Foreskin) ===

==== Phimosis ====
* '''See CUA Circumcision Guidelines'''

==== Paraphimosis ====
* In most cases, manual compression of the glans with placement of distal traction on the edematous foreskin allows reduction of the paraphimotic ring
* Insert figure
* Other options include''':'''
*# Application of an iced glove for 5 minutes
*# Application of granulated sugar for 1-2 hours
*# Placement of multiple punctures in the edematous skin

==== Circumcision ====
* '''See CUA Circumcision Guidelines'''
* Potential benefits:
*# Reduced risk of '''Penile cancer'''
*# Reduced risk of '''UTIs'''
*# Reduced risk of '''Sexually transmitted diseases including HIV'''
*# Reduced risk of '''Phimosis'''
*# Reduced risk of '''Balanitis'''
* '''Contraindications (2):'''
*# '''Neonates with other penile conditions (hypospadias, penile curvature, dorsal hood deformity, buried penis, and webbed penis) that require surgical correction'''
*#* '''Other conditions commonly seen in neonates that should be taken into consideration are a large hydrocele or inguinal hernias, which are more likely to develop secondary phimosis, buried penis, and trapped penis.'''
*#* '''There should be complete separation of the prepuce from the glans and complete inspection of the meatus and the corona to confirm the absence of anomalies, including hypospadias.'''
*# '''Coagulopathy'''
* '''Techniques and devices for neonatal circumcision:'''
*# '''Gomco clamp'''
*# '''Mogen clamp'''
*# '''Plastibell device'''
* '''Complications (6):'''
** Risk of complications after circumcision: 0.2-5%
*# '''Bleeding'''
*#* '''Most common complication, occurs in 0.1%''' and is more common in older children
*#* '''Usually localized from the frenulum'''
*# '''Wound infection'''
*#* '''Rare complication'''
*#* '''Antibiotic ointment (e.g., bacitracin) after circumcision usually prevents its development'''
*# '''Penile skin complications'''
*#* '''Insufficient or asymmetrical excision can result in a cosmetic and social dilemma for the parents and child'''
*#* '''Excessive skin excision can result in:'''
*#*# '''Penile chordee''', torsion, and lateral deviation
*#*# '''Trapped penis''' from a cicatricial scar
*# '''Glanular adhesions and skin bridges'''
*#* Glanular adhesions and skin bridges, attachments of the glans and penile shaft, respectively, are common complications
*#* '''Both can occur in the well-circumcised penis and are''' '''usually the result of the physiologic retraction of the penis caused by a suprapubic fat pad and diaper irritation of the penis'''
*#* '''Management'''
*#** '''Persistent adhesions can be lysed in the office with the application of a topical analgesic such as EMLA cream'''
*#** '''Low-dose corticosteroids have been relatively unsuccessful in lysing adhesions'''
*# '''Meatal Stenosis'''
*#* The normal urethral meatus is:
*#** Age<4: 10 Fr
*#** Age 4-10: 12 Fr
*#** Age>10: 14 Fr
*#* '''Symptoms include:'''
*#*# '''Narrow, high-velocity stream'''
*#*# '''Urinary stream deviation, typically in an upward direction''' resulting from a meatal baffle or ventral web located at the inferior aspect of the meatus
*#*# '''Penile pain''' '''at the initiation of micturition'''
*# '''Penile Trauma'''
*#* '''Most serious complication'''
*#* '''Includes urethral injury, excision of the glans and/or penile shaft, and penile necrosis'''
*#** '''Urethral injury requires urethroplasty'''
*#** '''Excision of the glans can be repaired by suturing the excised tissue back to the penis, often without the need for microscopic repair'''
*#*** '''Good results if performed within 8 hours'''

==== Lichen sclerosis (Balanitis Xerotica Obliterans) ====
* '''See Penis and Urethra Surgery Chapter Notes'''
* '''Rare in children age <5 years'''
* '''At puberty, inability to retract the prepuce is the most common presentation'''
* Other presenting symptoms include:
** Local infection
** Irritation
** Discomfort after micturition
** Bleeding
** Occasionally acute urinary retention or urinary incontinence
* '''Circumcision is the preferred treatment along with meatotomy or meatoplasty if there is meatal involvement'''
** Children with meatal involvement should be observed post-operatively because of the risk of recurrent meatal stenosis
** The use of topical corticosteroids has had limited benefit to treat mild BXO of the prepuce with minimal scar formation

=== Abnormal Penile Number ===

==== Aphallia ====
* '''Penile agenesis results from failure of development of the genital tubercle'''
* '''The karyotype almost always is 46,XY''', and the usual appearance is that of a well-developed scrotum with descended testes and an absent penile shaft.
* Associated malformations are common and include cryptorchidism, vesicoureteral reflux, horseshoe kidney, renal agenesis, imperforate anus, and musculoskeletal and cardiopulmonary abnormalities
* '''Testing should include a karyotype''' '''and other appropriate studies to detect associated malformations of the urinary tract or other organ systems.'''
** MRI may be beneficial in determining the severity of the defect

==== Diphallia ====
* Duplication of the penis is a rare anomaly; incidence of 1 in 5 million live births

=== Abnormal penile size ===
* '''Normal penile size in a full-term male neonate'''
** '''Stretched length: 3.5cm''' (13.3cm in adults)
** Diameter: 1.1cm

==== Inconspicuous Penis ====
* '''Definition of inconspicuous penis: penis that appears to be small but with normal stretched penile length measured from the pubic symphysis to the tip of the glans and normal diameter of the penile shaft'''
** '''Differentiated from micropenis, in which the penis is abnormally small'''
* '''Subtypes (3): buried penis, webbed penis, and trapped penis'''
** '''Buried Penis'''
*** '''Definition of a buried penis: a normally developed penis that is hidden away by a suprapubic fat pad'''
*** '''Classified into 3 categories based on cause of the concealment:'''
***# '''Poor penopubic fixation of the skin at the base of the penis'''
***# '''Obesity'''
***# '''A trapped penis from cicatricial scarring after penile surgery, typically a circumcision'''
***#* '''May occur after neonatal circumcision in an infant with significant scrotal swelling as a result of a hernia or hydrocele or after routine circumcision in an infant with a webbed penis'''
*** Insert figure
*** '''Management'''
**** '''Based on the cause'''
**** '''The trapped penis can be managed with betamethasone, vertical relaxation incision, and formal repair'''
** '''Webbed Penis'''
*** '''Definition of a webbed penis: a congenital or acquired condition resulting from the scrotal skin extending onto the ventrum of the penis'''
*** Also known as penoscrotal fusion

==== Micropenis ====
* '''Definition of micropenis: a normally formed penis that is at least 2.5 standard deviations (SD) below the mean size in stretched length for age'''
** '''Stretched penile length correlates more closely with erectile length than does the relaxed penile length''' and should be compared with standards for penile length
** '''Micropenis in the full-term neonate is stretched penile length <1.9 cm long'''
* '''Causes'''
** See CW11 Box 146-1: Etiology of Micropenis
** '''Results from a hormonal abnormality that occurs after 14 weeks of gestation'''
** '''Hypogonadotropic hypogonadism (most common cause)'''
*** Failure of the hypothalamus to produce adequate quantities of gonadotropin-releasing hormone (GnRH) can occur in '''Prader-Willi syndrome, Kallmann syndrome (genital-olfactory dysplasia), Laurence-Moon-Biedl syndrome, and the CHARGE association'''
** '''Hypergonadotropic hypogonadism (primary testicular failure)'''
** '''Idiopathic'''
** Often associated with major chromosomal defects, including: Klinefelter syndrome (47,XXY) and other X polysomy syndromes, deletions, translocations, and trisomy involving chromosomes 8, 13, and 18
* '''Initial evaluation of a child with micropenis includes a history, physical examination, and a karyotype at birth.'''
* '''The ratio of the length of the penile shaft to its circumference is usually normal, but occasionally the corpora cavernosa are severely hypoplastic. The testes are usually small and frequently cryptorchid, whereas the scrotum is usually fused and often diminutive'''
* '''Although ultimate penile size may be below the normal range, men born with micropenis have male gender identity and most have satisfactory sexual function'''

=== Abnormal Penile Orientation ===

==== Congenital penile curvature ====
* '''Referred to as chordee'''
* '''Results from disproportionate development of the tunica albuginea of the corporal bodies'''
** '''Not associated with urethral malformation'''§
* '''Most commonly in the ventral direction (as opposed to Peyronie’s which is dorsal curvature), with preserved penile length and typically normal erectile function and rigidity'''§
* '''Commonly associated with hypospadias'''
** Isolated chordee may occur with or without a dorsal hood of prepuce and is commonly associated with a deficiency of the ventral skin
* '''Congenital dorsal penile curvature''' may be an isolated condition with or without asymmetrical penile skin or associated with epispadias and a ventral hood of prepuce.
* '''Healthy young men usually present between age 18-30 when the abnormal curvature is brought to their attention from their sexual partner'''
* '''Treatment is surgical''' and is offered to patients whose congenital penile curvatures significantly interferes with satisfactory sexual relations of the patient or partner.
** Plication is used almost exclusively§

==== Penile Torsion ====
* '''Definition of penile torsion: a rotational deformity of the penile shaft, usually in the counterclockwise direction'''
* The cause has not been clearly delineated but may be the result of an anomalous arrangement of penile shaft skin.

=== Penile Masses (5): ===
# '''Penile cysts'''
#* '''Most common penile mass in children'''
#* '''Can be congenital or acquired'''
# '''Parameatal Urethral Cyst'''
#* A rare anomaly and appears as a small blister in proximity to the urethral meatus
# '''Inclusion Cysts'''
#* '''Smegma pearl'''
#** Smegma, produced under the foreskin, is made of 27% fat and 13% protein§
#** '''A smegma pearl is a type of inclusion cyst where smegma becomes entrapped under the unretractable foreskin'''
#*** See Figure
#** '''Most common acquired cystic lesion of the penis'''
#* May form after penile surgery, including circumcision and hypospadias repair, owing to islands of epithelium within the subcutaneous tissue. Excision of the epidermal inclusion is recommended.
# '''Congenital Penile Nevi'''
#* '''Pigmented lesions that can form on the glans and penile shaft tend to be superficial and benign and should be excised'''
# '''Juvenile Xanthogranuloma'''
#* Uncommon benign lesion of the penis
#* '''Lesions appear as solitary or multiple pigmented (yellow, orange, gold, brown, or red) nodules of rapid onset. They measure 2-20 mm in diameter and are well demarcated, firm, and rubbery.'''
#* Predominantly seen in infancy or early childhood.
#* '''The lesion is often self-limited, and a period of 1 year of expectant monitoring is advised to avoid potentially unnecessary ablative genital surgery'''

=== Accessory Urethral Openings ===
* '''Congenital Urethral Fistula'''
* '''Urethral Duplication'''
** '''Duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally'''
*** '''Usually the dorsal urethra is considered the accessory urethra''' with or without a urinary stream, '''whereas the ventral urethra carries the urine stream and the anatomic landmarks such as the external sphincter and verumontanum'''.
** Associated genitourinary, gastrointestinal, and musculoskeletal anomalies may be present
*** Vesicoureteral reflux is the most common associated anomaly
*** Other anomalies include renal agenesis, bilateral cryptorchidism, sacral agenesis, imperforate anus, radial hypoplasia, tracheoesophageal fistula, and other midline defects such as duplicated bladder, duplicated colon, imperforate anus and anorectal agenesis, bifid glans, thoracic hemivertebrae, and partial sacral agenesis
** The most common presentation of urethral duplication is a double meatus and double urinary stream
** Evaluation should include a voiding cystourethrogram, retrograde urethrogram, and direct visualization of the anatomy during cystourethroscopy

=== Genital Lymphedema ===
* A disfiguring disorder characterized by impaired lymphatic drainage that causes progressive penile or scrotal swelling
* Can be congenital or acquired

=== Priapism ===
* '''Stuttering, low-flow, high-flow priapism'''
** '''See Priapism Chapter Notes'''
* '''Spontaneously resolving priapism'''
** '''Form observed in neonates'''
** '''Causes may include idiopathic factors, birth trauma, and polycythemia'''
** '''No intervention is necessary because the priapism usually resolves in 2-6 days without adverse results'''

=== Penoscrotal Transposition (Scrotal Engulfment) ===
* Frequently occurs in conjunction with perineal, scrotal, or penoscrotal hypospadias with chordee
* Also associated with caudal regression, sex chromosome abnormalities, and Aarskog syndrome.
* As many as 75% of patients with complete penoscrotal transposition and a normal scrotum have a significant urinary tract abnormality, including renal agenesis and dysplasia, and other nongenitourinary anomalies