CLINICAL CASE
Chordee in the absence of hypospadias Valdepeña-Estrada Valdepeña-E strada 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
• ABSTRA ABSTRACT CT
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 curvat curvatura ura peniana ventral es común en el hipospadias, pero lo es mucho menos cuando el meato es ortotópico, y es rara cuando se pre senta de forma aisla aislada, da, en que. represen representa ta alrededo alrededorr de 4% a 10% de los casos de cuerda congénita. La etiología y el manejo de esta afección continúan sujetos a debate en la literatur literatura. a. 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
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 UNAM . 4Urology Surgeon, Urology Service Staff Physician at
48
Rev Mex Urol 2010;70(1):48-50
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 classified 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. Universidad No. 1321 Col. Florida, Delegación Álvaro Obregón, Distrito Federal Telephone: T elephone: 044 044 55 29019733 29019733 Email:
[email protected]
Valdepeña-Estrada ValdepeñaEstrada RE, et al . Chordee
in the absence of hypospadias
dartos is deficient and in Group III only the dartos fascia is affected. Corporal disproportion is another cause of chordee which is classified 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.
Image 1. Penis with bifurcated prepuce in dorsal region as well as absence of prepuce in ventral region.
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 identified 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 flaps were formed ( Image 3). Fibrous chordee tissue at the ventral side of the penis was resected and involvement of Buck’s fascia only was identified. The dartos and corpus spongiosum were not involved. The ventral region of the penis was then covered with the flaps (Image 4).
Image 2. “Tennis racket” incision 5 mm behind the dorsal corona of the penis
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 classified chordee without hypospadias into Igroups based on different etiologies involved. Type is considered to the be the most
Image 3. Byar’s flaps to be placed in the ventral region of the penis
Rev Mex Urol 2010;70(1):48-50
49
Valdepeña-Estrada ValdepeñaEstrada RE, et al . Chordee
in the absence of hypospadias
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 with graft 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
Image 4. Ventral region of the penis covered with Byar’s flaps
serious defect. This comes about when the corpus spongiosum, dartos and Buck’s fascia are deficient in the portion of the urethra that is involved. Therefore the urethra is located directly below the skin and the fibrous 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 deficient causing penile curvature.5 Kramer subsequently recognized that corporal disproportion is an additional cause of penile curvature and classified 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 without hypospadias. 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 fibrous tissue resection while urethral transection was rarely required to straighten the penis.5
50
Rev Mex Urol 2010;70(1):48-50
In the present case the technique described by Jednak et al. al .6 was used. This technique originally was employed in Type I cases with urethral involvement. invol vement. In the case described here there was no urethral involvement but treating the dorsal coronal skin and taking the flap to the ventral region was considered to be the most adequate way to manage this particular case. •
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. It is important to first define which structures are involved to avoid carrying out extensive unnecessary procedures. Pre-school age is the suggested age for correcting this pathology. BIBLIOGRAPHY
1. 2. 3. 4. 5.
Yun Man T, T, Shao Ji C, Lu Gang H. Chordee without hypospadias: report of 79 chinese prepuberal patients. J Androl 2007;28(4):630-633. Snodgrass W. A farewell to chordee. chordee. J Urol 2007;178 (3 Pt 1):753-4. Donnahoo KK, Cain MP MP,, Pope JC. Etiology, management and surgical complications of congenital chordee without hypospadias. J Urol 1998;160(3 Pt 2):1120-2. Kramer SA, Aydin Aydin G, Kelalis Kelalis PP. Chordee without hypospadias hypospadias in children. J Urol 1982;128(3)559-61. Devine CJ, CJ, Horton CE. Chordee without hypospadias. J Urol 1973;110(2):264-71 Jednak R, Hernandez N, N, Spencer J. Correcting chordee without hypospadias and with deficient ventral skin: a new technique. BJU Int 2001;87(6):528–30. .
6.
