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Chordee

Chordee is a medical condition that describes an abnormal curvature of the penis. It is most commonly associated with hypospadias and may require surgical correction if it is particularly severe.


Presentation

Physicians usually suspect chordee when they detect hypospadias or abnormalities with the shape of the foreskin. This usually occurs directly after birth and in very early infancy. Parents can also notice a curvature of the penis with or without the presence of hypospadias. There are several forms of chordee that include penile torsion, skin tethering, a webbed penis and dorsal preputial hood.

Penile torsion is caused by an abnormal midline raphe, that envelops the whole of the penis. The midline raphe is a ridge that is present on the bottom side of the penis in normal individuals. Surgical correction is recommended when the pulling of the midline raphe is around 90 degrees and is usually done in conjunction with circumcision.

Skin tethering leads to a very thin urethra at the tip of the penis. It is corrected by initially creating a hypospadias, permitting a reconstruction of the urethra, and then later by fixing the hypospadias. The penis may also be straightened with lifelong sutures.

A webbed penis refers to the fusion of the penis with the scrotum. In this case, the physician may perform a surgery to partially separate the penis from the scrotum. The foreskin can be used for the reconstruction of the bottom side of the penis.

A dorsal preputial hood describes cases in which the foreskin only partially covers the head of the penis, particularly the upper side. It is associated with both hypospadias and chordee. Patients with dorsal preputial hood need to be assessed for hypospadias and chordee before undergoing circumcision.

Surgical Procedure
  • This response pattern simplifies the required surgical procedure and appears to improve surgical results.[ncbi.nlm.nih.gov]
  • Chordee-related structural defect was considered the only criterion for classification, and urethral dysgenesis influenced the choice of surgical procedure.[ncbi.nlm.nih.gov]
  • Depending on the severity of chordee, the urologist may recommend a surgical procedure to correct the curvature of the penis. If surgical correction is recommended, it is typically an outpatient procedure done under general anesthesia.[luriechildrens.org]
  • A variety of surgical procedures have been used, but all essentially work on the principle of shortening the convex side to equal the concave aspect.[sandiegosexualmedicine.com]
Wound Infection
  • Complications in the group of 20 patients consisted of 1 fistula, 2 meatal stenoses and 1 glanular dehiscence due to severe wound infection. There were no late complications.[ncbi.nlm.nih.gov]
  • In immediate postoperative period, wound infection rate was 23.0% (6/26), which was managed conservatively.[urologyannals.com]
  • These may include: wound infections scarring the need for further surgery allergic reaction to anesthesia It is important for parents to discuss all possible risks with a doctor before surgery.[medicalnewstoday.com]
Enuresis
  • الصفحة 365 - The genetics of enuresis. In: Kolvin I, MacKeith RC, Meadow SR (eds): Bladder control and enuresis. ‏[books.google.com]
  • Pagina 365 - The genetics of enuresis. In: Kolvin I, MacKeith RC, Meadow SR (eds): Bladder control and enuresis. ‎[books.google.ro]
Suggestibility
  • The results suggested that chordee without hypospadias may also be categorized into hypospadias from the aspect of its pituitary and gonadal functions in addition to the embryogenesis of chordee.[ncbi.nlm.nih.gov]
  • CONCLUSION: To avoid the risks of persistent chordee, the authors suggest more radical and accurate operations for the treatment of type I and II pseudohypospadias.[ncbi.nlm.nih.gov]
  • PURPOSE: Prior reports have suggested that tunica vaginalis free grafts for the treatment of chordee are associated with a high failure rate and residual curvature.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Our outcomes suggest that small intestine submucosa is safe and effective for corporal body grafting in the correction of severe chordee, especially in the setting of a multistage hypospadias and chordee repair.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Our long-term followup suggests that ventral grafting of the corporal wall in patients with severe chordee as a staged procedure is superior to the dorsal plication or Nesbit procedure.[ncbi.nlm.nih.gov]
Chordee
  • No recurrence of chordee or surgery related morbidity was observed after recurrent chordee repair by midline dorsal plication. CONCLUSIONS: Chordee may recur during puberty following successful chordee repair.[ncbi.nlm.nih.gov]
  • With 50 degrees chordee, urethral plate manipulation was used 34% of the time. Sixty percent of the respondents believed the urethral plate did not often contribute to chordee.[ncbi.nlm.nih.gov]
  • Ventral chordee ranged from 45 to 100 , with a median of 50 . A ratio of 1:9.6 was found for chordee without hypospadias compared to the total hypospadias cases, with an incidence of 9.5%.[ncbi.nlm.nih.gov]
  • It is associated with both hypospadias and chordee. Patients with dorsal preputial hood need to be assessed for hypospadias and chordee before undergoing circumcision. A thorough physical examination can establish the diagnosis of chordee.[symptoma.com]
  • Tunica vaginalis grafts had been placed at repair in all patients with residual chordee but there was no recurrent chordee after dermal graft placement. Chordee recurred in 60% of the patients who received a tunica vaginalis graft.[ncbi.nlm.nih.gov]
Painful Erection
  • […] chor·dee \ ˈkȯr-ˌdē, -ˌdā, ˌkȯr-ˈ \ Medical Definition of chordee : painful erection of the penis often with a downward curvature that may be present in a congenital condition (as hypospadias) or accompany gonorrhea Comments on chordee What made you want[merriam-webster.com]
  • Health News from Medical News Today Known Forms of Gonorrhoea_), Vienna and Leipzig, 1907, that the painful erections ( chordee ) which so commonly accompany gonorrhoea in adults, are very rare indeed in the case of gonorrhoea in children, and even in[wordnik.com]
  • Painful erection of the penis in association with gonorrhea or Peyronie disease, with curvature resulting from lack of distensibility of the corpora cavernosa of the urethra. 2.[medical-dictionary.thefreedictionary.com]
  • Penis disorders include Erectile dysfunction - inability to get or keep an erection Priapism - a painful erection that does not go away Peyronie's disease - bending of the penis during an erection due to a hard lump called a plaque Balanitis - inflammation[icdlist.com]
Penile Pain
  • Peyronie's disease: 16 patients presented with penile pain and difficulty during sexual intercourse, all cases had a palpable plaque in the tunica albuginea.[indianjurol.com]
  • This generally means that there should have been no change in the degree of curvature, penile pain, size of the plaque or deterioration in erectile dysfunction for at least 6 months.[metrovanurology.com]

Workup

A thorough physical examination can establish the diagnosis of chordee. Urologists can also detect the condition by performing special procedures that enable artificial erections and the subsequent observation of the curvature of the penis. Chordee may also be found incidentally when performing other surgeries on the penis.

Some cases are only detected late in childhood.

Treatment

The cornerstone of treatment of chordee is surgical correction in early infancy, between the ages of 6 and 18 months [12]. The procedure is usually performed by a pediatric urologist. Z-Plasty is the treatment modality of choice for cases that result from circumcision [7].

After diagnosing the condition and deciding to pursue surgical treatment, chordee needs to be properly assessed by triggering an artificial erection in the sleeping child. The latter is performed through the infusion of sterile saline within the erectile tissue of the penis. When the curvature exceeds 30 degrees relative to a straight erection, it can be surgically corrected along with the performance of a circumcision. Hypospadias can also be corrected at the same time.

In cases that involve adults or older children, a number of surgeries may be eventually required to establish full correction.

Prognosis

Prognosis of chordee is variable. It can be associated sometimes with sexual dysfunction, discomfort and pain for the patient and the partner if it is not detected or treated at infancy. Surgical treatment is usually performed before the patient is two years of age. It is a simple procedure done in the outpatient department and the patient can expect to leave the clinic in the same day.

Etiology

The etiological mechanisms responsible for Chordee take place during development in the womb. They are due to problems in the urethra or in the skin of the penis. A urethra that is either short or covered by thickened tissue may trigger the process by which Chordee develops. In addition, short skin on the underside of the penis can also contribute.

Chordee may also result from complications of circumcision or sex disorder problems [7]. Because corporal bodies are still normal in complications of circumcision, some clinicians do not believe that the condition in question is a true chordee. Nonetheless, damages to the corpus cavernosum do occur when circumcision is performed and some cases of congenital chordee are not necessarily associated with abnormal penile corpora [8].

Epidemiology

Chordee and penile torsion tend to be more common among patients with hypospadias or with a family history of hypospadias. Hypospadias refers to a condition in which the urethral meatus is on the underside of the penis. Nonetheless, a meatus in the orthotopic location can also be associated with chordee. In fact, around 1 of every 200 male births may exhibit chordee in the absence of hypospadias and 4 to 10% are born with penile curvatures of different degrees. Torsion, on the other hand, can be present at birth or later in life in cases where circumcision occurred [9].

Congenital short urethra is an extremely rare condition [10]. It is characterized by an absence of the corpus spongiosum, dartos and buck's fascia [11]. Only two cases were reported by Devine.

Sex distribution
Age distribution

Pathophysiology

The characteristic ventral bowing of the penis in chordee is due to shortened subcutaneous tissue as well as tethering of the skin. It can also manifest with upward curving, where the head of the penis meets the shaft. The abnormal curvature usually manifests upon erection but can sometimes be apparent when the penis is flaccid, especially when resistance is applied.

Prevention

There are no known preventive measures for chordee.

Summary

Chordee refers to an abnormal anatomical curvature of the penis that can occur in a rotational, ventral or lateral fashion [1]. It usually develops in the womb and can be caused by either a short urethra, presence of excessive skin around the urethra or tightened skin on the underside of the penis. A difference in size between the corpora cavernosa can also contribute.
The characteristic curvature in chordee is usually detected in the erect state although it can sometimes be noticed when the penis is flaccid and resistance is applied. It tends to be associated with hypospadias, a condition that describes a ventrally located urethral meatus [2] [3]. It is important to distinguish chordee from Peyronie's disease, a disfigurement of the shape of the penis that occurs subsequent to trauma in adult life. Chordee can also occur following a circumcision procedure or complications of surgical repairs [4] [5].

Siever was the first to describe chordee in 1962. Devine et al. would later devise a classification for the condition with five subtypes [6]. Type I and V correspond to a subcategory with congenital short urethra. Type II is related to the fibrous form of chordee. Type III is cutaneous chordee and type IV is designated when problems with the corpora cavernosa underlie the condition. This classification remains controversial and many clinicians nowadays recommend a simpler division based on the steps involved in surgery rather than etiology.

Patients usually present right after birth or very early on during infancy. Some cases, however, are only detected in late childhood or adulthood. A presence of hypospadias or shortened foreskin should prompt the physician to investigate the possibility of having the condition. Severe chordee may necessitate surgery. The procedure is simple. It is performed in the outpatient department usually along with circumcision and the patient is sent home the same day. In some cases, however, correction of the hypospadias is done at the same time. Before deciding on surgery, the patient is referred to an urologist, who then proceeds with an accurate assessment of the severity of the deformity. In young boys, a sterile saline solution is infused within the tissues of the penis to induce an erection. If the bending of the penis is greater than 30 degrees relative to the upright erection, the surgeon can proceed with the corrective treatment.

Chordee has a very good prognosis if it is caught early and a corrective surgery is usually done before the age of two. In severe cases where treatment is delayed, the patient and the partner may suffer in the future from pain and sexual dysfunction.

Patient Information

Chordee is a medical condition characterized by a curvature of the penis, particularly in the region where the head and the shaft of the penis meet. It is commonly observed in erection but it can also be sometimes apparent in the flaccid state when resistance is applied to the penis. Chordee is usually associated with hypospadias, a condition characterized by an opening of the urethra (the tube that runs along the course of the penis and drains urine from the tip of the penis) on the underside of the penis. There are many possible causes for chordee. It is thought to develop when the boy is still in the womb and may be caused by a short urethra, a urethra covered by thick skin or tight skin on the bottom side of the penis. Sometimes chordee can develop as a complication from circumcision or because of a disorder in sexual development. The condition is relatively common and, in fact, up to 10% of boys may show curvatures of the penis of varying degrees. The presence of hypospadias is not a necessary condition for the development of chordee. 1 in every 200 male live births may have the condition in the presence of a normally located urethral meatus.

Chordee is initially suspected when doctors notice hypospadias or abnormalities in the foreskin. It is usually found right after birth or in very early infancy. Some cases, however, may only be found much later in childhood or even in adulthood as it can be hard to assess in the non-erect penis. It can also present in other forms such as what is known as webbed penis, penile torsion or a dorsal preputial hood. A webbed penis occurs when the penis is fused with the scrotum, the sac that contains the testicles. It is surgically corrected by partially separating the two structures from each other and later reconstructing the bottom side of the penis with its foreskin. Penile torsion results from an abnormal ridge on the bottom side of the penis. Normally, that ridge is restricted to the underside. In penile torsion, it surrounds the whole penis and acts by pulling it downward. Penile torsion also requires surgical correction. Finally, a dorsal preputial hood is a benign condition that refers to cases in which the foreskin only partially covers the head of the penis, particularly its upper side. It is important to detect it because it can be associated with both hypospadias and chordee.

Chordee is diagnosed by performing a thorough physical examination. It can also be incidentally discovered when physicians or surgeons are in the process of fixing another possible problem with the penis. For confirmation and potential surgical treatment, the patient may be referred to a physician who is specialized in the male urinary and genital system (urologist), who would then perform a procedure to artificially induce an erection in a sleeping child. This is done by injecting a sterile salt and water solution into the tissues of the penis. If the bending is greater than 30 degrees relative to a straight erection, the patient can be referred for surgery. A correction of the hypospadias as well as circumcision can be performed at the same time. The corrective procedure is usually performed in an outpatient clinic, allowing the patient to go home on the same day.

Chordee has a good prognosis when treated early, usually before the age of 2. If it is relatively severe and treatment is delayed, it may lead to sexual dysfunction and pain in adulthood for both the patient and the partner.

References

Article

  1. Gerald HJ, Schlossberg SM. Surgery of penis and urethra. In: Walsh PC, Vaughan ED, Retik AB, Wein AJ, editors. Campbell's Urology. 8th ed. Philadelphia: WB Saunders; 2002. pp. 3886–952.
  2. Donnahoo KK, Cain MP, Pope JC, et al. Etiology, management and surgical complications of congenital chordee without hypospadias. J Urol. 1998;160(3 Pt 2):1120–2.
  3. Tang YM, Chen SJ, Huang LG, Wanh MH. Chordee without hypospadias: Report of 79 Chinese prepubertal patients. J Androl. 2007;28:630–3.
  4. Perovic SV, Djordjevic ML. A new approach in hypospadias repair. World J Urol. 1998;16:195–9.
  5. Upadhyay J, Khoury A. Single-stage procedure for severe hypospadias: Onlay-Tube-Onlay modification of the transverse island preputial flap. In: Hadidi AH, Azmy OF, editors. Hypospadias Surgery an Illustrated Guide. 1st edition. Berlin, Heidelberg: Springer-Verlag; 2004. pp. 173–85.
  6. Azmy AF. Chordee (penile curvature) In: Hadidi AH, Azmy OF, editors. Hypospadias Surgery an Illustrated Guide. 1st edition. Berlin, Heidelberg: Springer-Verlag; 2004. pp. 115–8.
  7. Kaplan GW. Complications of circumcision. Urol Clin North Am. 1983 Aug;10(3):543-9.
  8. Palmer JM, Link D. Impotence following anesthesia for elective circumcision. JAMA. 1979 Jun 15;241(24):2635-6.
  9. Montag S, Palmer LS. Abnormalities of penile curvature: chordee and penile torsion. ScientificWorldJournal. 2011 Jul 28;11:1470-8.
  10. Devine CJ, Jr, Horton CE. Chordee without hypospadias. J Urol. 1973;110:264–71.
  11. Kramer SA, Aydin G, Kelalis PP. Chordee without hypospadias in children. J Urol. 1982;128:559–61.
  12. Braga LH, Pippi Salle JL, Dave S, Bagli DJ, Lorenzo AJ, Khoury AE. Outcome analysis of severe chordee correction using tunica vaginalis as a flap in boys with proximal hypospadias. J Urol. 2007 Oct;178(4 Pt 2):1693-7.

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Last updated: 2019-07-11 22:49