chordee in the absence

3
CLINICAL CASE  Rev Mex Urol 2010;70(1):48-50 48 1Urology Resident at the  Licenciado Adolfo López Mateos Regional Hospital of the ISSSTE , Mexico City. 2Urology Surgeon, Urology Ser-  vice Staff Physician at the Centro de Cirugía Ambulatoria del ISSSTE , Mexico City. 3Doctor in Psychology and Medical Anthropology,  FES  Iztacala UNAM . 4Urology Surgeon, Urology Service Staff Physician at Chordee in the absence of hypospadias  Valdepeña-E strada RE, 2 Castellanos-Hernánde z H, 1 Córdoba-Basulto DI, 3 Bernal-García R, 1 De la Cruz-Gutiérrez SM, 1 Figueroa-Zarza M, 1 Velázquez-Macías RF. 4  ABSTRACT Ventral penile curvature or chordee is common in hypospadias but is much less frequent when the meatus is orthotopic. It rarely presents in isolated form and as such represents approximately 4-10% of chordee cases. Etiology and management of this condition continues to be a subject of debate in the literature. Key words: chordee, hypospadias, congenital, preputial. RESUMEN  La cuerda congénita (chordee) o curvatura peniana ven- tral es común en el hipospadias, pero lo es mucho menos cuando el meato es ortotópico, y es rara cuando se pre-  senta de forma aislada, en que. representa alrededor de 4% a 10% de los casos de cuerda congénita. La etiología y el manejo de esta afección continúan sujetos a debate en  la literatura.  Palabras clave : cuerda, hipospadias, congénita, pre-  pucial. INTRODUCTION Congenital chordee or ventral penile curvature is common in hypospadias but it is a rare abnormality when the meatus is orthotopic. 1,2,3 Its precise etiology is not yet known, even though now it is well-accepted that various anatomical changes are related to chordee with or without hypospadias and they require various surgical procedures. It has been suggested that chordee is due to a congenitally short urethra and that the urethra should be sectioned. The theory that chordee without hypospadias is the result of dysgenesis of the fascia surrounding the urethra has also been proposed. Chordee has been classied into 3 groups depending on the affected layers. In Group I the corpus spongiosum, dartos and Buck’s fascia are defective, in Group II the the  Licenciado Adolfo López Mateos Regional Hospital of the ISSSTE, Mexico City. Corresponding author: Dr. Hibert Castellanos Hernández Av. Universi- dad No. 1321 Col. Florida, Delegación Álvaro Obregón, Distrito Federal  Telephone: 044 55 29019733 Email: [email protected]

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Page 1: Chordee in the Absence

8/3/2019 Chordee in the Absence

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CLINICAL CASE

 Rev Mex Urol 2010;70(1):48-5048

1Urology Resident at the Licenciado Adolfo López Mateos RegionalHospital of the ISSSTE , Mexico City. 2Urology Surgeon, Urology Ser-

 vice Staff Physician at the Centro de Cirugía Ambulatoria del ISSSTE ,Mexico City. 3Doctor in Psychology and Medical Anthropology, FES Iztacala UNAM . 4Urology Surgeon, Urology Service Staff Physician at

Chordee in the absence of hypospadias Valdepeña-Estrada RE, 2 Castellanos-Hernández H,1 Córdoba-Basulto DI,3 Bernal-García R,1 De la Cruz-Gutiérrez

SM,1 Figueroa-Zarza M,1 Velázquez-Macías RF.4

• ABSTRACT

Ventral penile curvature or chordee is common in

hypospadias but is much less frequent when the meatusis orthotopic. It rarely presents in isolated form and as

such represents approximately 4-10% of chordee cases.

Etiology and management of this condition continues tobe a subject of debate in the literature.

Key words:chordee, hypospadias, congenital, preputial.

•RESUMEN

  La cuerda congénita (chordee) o curvatura peniana ven-tral es común en el hipospadias, pero lo es mucho menoscuando el meato es ortotópico, y es rara cuando se pre-  senta de forma aislada, en que. representa alrededor de4% a 10% de los casos de cuerda congénita. La etiología y el manejo de esta afección continúan sujetos a debate en la literatura.

  Palabras clave: cuerda, hipospadias, congénita, pre- pucial.

•INTRODUCTION

Congenital chordee or ventral penile curvatureis common in hypospadias but it is a rare abnormality

when the meatus is orthotopic.1,2,3

Its precise etiology is not yet known, even though

now it is well-accepted that various anatomical changesare related to chordee with or without hypospadias and

they require various surgical procedures. It has been

suggested that chordee is due to a congenitally shorturethra and that the urethra should be sectioned. The

theory that chordee without hypospadias is the result

of dysgenesis of the fascia surrounding the urethra has

also been proposed.

Chordee has been classied into 3 groups dependingon the affected layers. In Group I the corpus spongiosum,

dartos and Buck’s fascia are defective, in Group II the

the Licenciado Adolfo López Mateos Regional Hospital of the ISSSTE,Mexico City.Corresponding author: Dr. Hibert Castellanos Hernández Av. Universi-dad No. 1321 Col. Florida, Delegación Álvaro Obregón, Distrito Federal

 Telephone: 044 55 29019733 Email: [email protected]

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 Rev Mex Urol 2010;70(1):48-50

 Valdepeña-Estrada RE, et al . Chordee in the absence of hypospadias

49

dartos is decient and in Group III only the dartos fascia

is affected. Corporal disproportion is another cause of 

chordee which is classied as Group IV corresponding

to chordee without hypospadias.1

•CLINICAL CASE

The case of a 10-year-old boy is presented. He had no

important medical history related to his disease, was

the product of a normal second birth from a 22-year-old

mother and has a 6-year-old brother who is apparently

healthy. He sought medical attention due to strain upon

urination as well as a weakening of the urinary stream.

He had no history of infection and was referred to the

out-patient surgery center.

Physical examination revealed normal retractile

testes, a sunken penis surrounded by pubic fat,

bifurcated prepuce in the dorsal region and orthotopic

urinary meatus, absence of prepuce in the ventral region

of the penis and preputial chordee. No hypospadias was

identied at any level (Image 1).

Testicular and renal ultrasound was done to rule out

any other associated malformation and imaging results

were normal.

Preputial plasty and chordee correction were

decided upon and were carried out with the technique

described by Jednak et al. A “tennis racket” incision was

made 5 mm behind the dorsal corona (Image 2) and

Byar’s aps were formed (Image 3). Fibrous chordee

tissue at the ventral side of the penis was resected and

involvement of Buck’s fascia only was identied. Thedartos and corpus spongiosum were not involved.

The ventral region of the penis was then covered with

the aps (Image 4).

The patient was managed as an out-patient and

surgical progression was satisfactory. There was

improvement in the mechanical aspects of urination

as well as in genital aesthetics.

•DISCUSSION

Isolated chordee without hypospadias is rare and

represents 4-10% of cases of congenital chordee.

Etiology and management of this condition continue

to be topics of debate in the literature. 3 Developmental

arrest is a presumed cause of hypospadias and often

results in curvature with a relative shortening of ventral

structures. Sometimes ventral shortening of the skin

and dartos produces curvature.2

In 1973 Devine and Horton classied chordee

without hypospadias into groups based on the different

etiologies involved. Type I is considered to be the most

Image 1. Penis with bifurcated prepuce in dorsal region as well as ab-sence of prepuce in ventral region.

Image 2. “Tennis racket” incision 5 mm behind the dorsal corona of thepenis

Image 3. Byar’s aps to be placed in the ventral region of the penis

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50  Rev Mex Urol 2010;70(1):48-50

 Valdepeña-Estrada RE, et al . Chordee in the absence of hypospadias

serious defect. This comes about when the corpusspongiosum, dartos and Buck’s fascia are decient in

the portion of the urethra that is involved. Therefore the

urethra is located directly below the skin and the brous

tissue under the urethra causes the chordee. In Type II

the corpus spongiosum is normal while the dartos and

Buck’s fascia are dysgenetic. In Type III only the dartos

is decient causing penile curvature.5

Kramer subsequently recognized that corporal

disproportion is an additional cause of penile curvature

and classied this type as Type IV chordee without

hypospadias. Congenital short urethra is also recognized

as a rare cause of congenital chordee.4

The present case is a Type II chordee withouthypospadias.

In 1937 Young proposed that chordee without

hypospadias was due to congenital short urethra and

suggested transection and reconstruction of the curved

ventral urethra.1

In 1973 Devine and Horton proposed that chordee

without hypospadias was due to abnormal development

of the fascial layers surrounding the urethra. In their

experience the majority of patients were successfully

treated by brous tissue resection while urethral

transection was rarely required to straighten the penis.5

Image 4. Ventral region of the penis covered with Byar’s aps

In 1982 Kramer recognized that corporal

disproportion was an important cause of isolated

chordee and recommended carrying out dorsal

plication following the Nesbit principle to correct this

type of chordee without hypospadias.4 However, others

suggest that elongating ventral corporal organs withgraft material is superior to plication of ventral corporal

organs in severe penile curvature.3

Successful repair of chordee without hypospadias

should produce a straight phallus with a urethral meatus

that permits normal urination.6

In the present case the technique described by

 Jednak et al.6 was used. This technique originally was

employed in Type I cases with urethral involvement. In

the case described here there was no urethral

involvement but treating the dorsal coronal skin and

taking the ap to the ventral region was considered to

be the most adequate way to manage this particularcase.

•CONCLUSIONS

Congenital chordee is a rare entity when it presents

in isolated form. It can negatively affect the urination

mechanism and cause alterations in genital aesthetics.

There are multiple techniques for correcting this

pathology but each case should be individualized. Itis important to rst dene which structures are involved

to avoid carrying out extensive unnecessary procedures.

Pre-school age is the suggested age for correcting this

pathology.

BIBLIOGRAPHY

1. Yun Man T, Shao Ji C, Lu Gang H. Chordee without hypospadias: re-port of 79 chinese prepuberal patients. J Androl 2007;28(4):630-633.

2. Snodgrass W. A farewell to chordee. J Urol 2007;178 (3 Pt 1):753-4.3. Donnahoo KK, Cain MP, Pope JC. Etiology, management and surgical

complications of congenital chordee without hypospadias. J Urol1998;160(3 Pt 2):1120-2.

4. Kramer SA, Aydin G, Kelalis PP. Chordee without hypospadias in chil-dren. J Urol 1982;128(3)559-61.

5. Devine CJ, Horton CE. Chordee without hypospadias. J Urol1973;110(2):264-71.

6. Jednak R, Hernandez N, Spencer J. Correcting chordee without hy-pospadias and with decient ventral skin: a new technique. BJU Int2001;87(6):528–30.