Jennifer A. Hagerty, DO
BASICS
DESCRIPTION
• Chordee is ventral penile curvature that occurs with or without hypospadias:
– Epispadias can occur with dorsal curvature
– Lateral curvature also can occur with or without hypospadias
EPIDEMIOLOGY
Incidence
The incidence of chordee is unknown
Prevalence
• 44% of fetuses through the 2nd trimester suggesting chordee is a normal part of development (1)[A]
• Chordee occurs without hypospadias in 4–10% of cases of congenital chordee (2)[C]
• Hypospadias occurs in 1 of 250 live births (3)[A]
– Chordee is identified in 1/3 of these patients (3)[A]
RISK FACTORS
• Congenital
• Prior penile surgery
• Trauma
Genetics
• Found in syndromes associated with hypospadias
• Chromosomal abnormalities found in 22% of individuals with severe hypospadias associated with undescended testicles
• 14% of hypospadias in siblings
• 8% incidence in offspring
PATHOPHYSIOLOGY
• Chordee could be considered an arrest of normal embryologic development
• Different proposed etiologies for chordee without hypospadias (2,4):
– Class I: Results when corpus spongiosum, dartos, and Buck fasciae are deficient over the involved portion of the urethra; urethra is just below the skin, and the dense fibrous tissue beneath the urethra is responsible for the chordee.
– Class II: Spongiosum is normal while the dartos and Buck fasciae are dysgenetic.
– Class III: Only the dartos fascia is deficient.
– Class IV: Corporeal disproportion.
ASSOCIATED CONDITIONS
• Hypospadias
• Epispadias
• Penile torsion
• Cryptorchidism
• Disorders of sexual development
GENERAL PREVENTION
None known
DIAGNOSIS
HISTORY
• Visualized curvature of the penis with an erection
• Presence of hypospadias
PHYSICAL EXAM
• Observe the individual’s erection if possible
• Possible coexisting findings:
– Hypospadias or epispadias
– Incomplete foreskin ventrally
– Penoscrotal webbing
– Penile torsion
– Hypoplasia of the ventral shaft skin
– Cryptorchidism
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Routine lab testing not typically indicated
• Chromosomal testing and/or biochemical testing in the individual with a suspected syndrome or disorder of sexual differentiation
Imaging
• Renal and bladder ultrasound routinely recommended only in individuals with:
– Severe hypospadias
– Hypospadias associated with other organ system anomalies
Diagnostic Procedures/Surgery
• Intraoperative artificial erection test at the time of repair
– Infusion of injectable saline into the corpora with a tourniquet at the base of the penis
Pathologic Findings
N/A
DIFFERENTIAL DIAGNOSIS
• Disorder of sex development
• Epispadias
• Hypospadias
• Normal penile variant
• Penile torsion
TREATMENT
GENERAL MEASURES
Chordee repair is the standard approach
MEDICATION
First Line
None usually indicated specifically for chordee
Second Line
N/A
SURGERY/OTHER PROCEDURES
• Specific surgery dependant on the associated conditions and the severity of the curvature
• Performed typically after 6 mo of age
• General points:
– Following penile skin release, induce artificial erection. This should be repeated to confirm correction.
– Chordee without hypospadias often can be corrected by penile degloving with excision of the fibrous tissue superficial to Buck fascia.
– More moderate chordee requires simple plication and/or excision of ellipses from the site of maximum curvature.
– In the most severe cases, often associated with hypospadias the urethra may be foreshortened and need to be transected.
– Chordee secondary to corporeal disproportion involves incising the tunica albuginea on the ventral surface of the penis, transversely over the point of maximal curvature; than covering the defect with either a free dermal, tunica vaginalis or single ply small intestinal submucosal (SIS) graft.
– It is critical to identify and preserve the neurovascular bundles during dissection and plication.
– Skin flaps may be required for penile skin coverage after correction of the chordee.
ADDITIONAL TREATMENT
Radiation Therapy
N/A
Additional Therapies
N/A
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
• Excellent prognosis postoperatively with a low complication rate
• There may be progression of chordee after puberty (5)[C]
COMPLICATIONS
Recurrence of chordee
FOLLOW-UP
Patient Monitoring
• Postoperative checkup within several weeks after surgery
• Consider follow-up after puberty
Patient Resources
• http://men.webmd.com/guide/chordee-repair-treatment
• http://www.mayoclinic.com/health/hypospadias/DS00884
REFERENCES
1. Kaplan GW, Lamm DL. Embyrogenesis of Chordee. J Urol. 1975;114:769–772.
2. Kramer S, Aydin G, Kelalis P. Chordee without hypospadias in children. J Urol. 1982;128:559–561.
3. Paulozzi LJ, Erickson JD, Jackson RJ. Hypospadias trends in two US surveillance systems. Pediatrics. 1997;100:831–834.
4. Devine CJ Jr., Horton CE. Chordee without hypospadias. J Urol. 1973;110:264–271.
5. Vandersteen DR, Husmann DA. Late onset recurrent penile chordee after successful correction at hypospadias repair. J Urol. 1998;160:1131–1133.
ADDITIONAL READING
Bologna RA, Noah TA, Nasrallah PF, et al. Chordee: Varied opinions and treatments as documented in a survey of the American Academy of Pediatrics, Section of Urology. Urology. 1999;53:608–612.
See Also (Topic, Algorithm, Media)
• Chordee Image 
• Disorders of Sexual Development (DSD)
• Epispadias
• Hypospadias
CODES
ICD9
• 607.89 Other specified disorders of penis
• 752.61 Hypospadias
• 752.63 Congenital chordee
ICD10
• N48.89 Other specified disorders of penis
• Q54.4 Congenital chordee
• Q54.9 Hypospadias, unspecified
CLINICAL/SURGICAL PEARLS
• Chordee most commonly occurs with hypospadias.
• Repair recommended after 6 mo of age.
• Consider ongoing monitoring after puberty.