Chordee without Hypospadias with a Communicating Symptomatic Epidermoid Cyst: An Unusual Presentation


We present an unusual case of chordee without hypospadias with a communicating, symptomatic epidermoid cyst. A 12-year-old boy presented with painful erections, especially early in the morning, over the last year and a watery discharge from swelling located near the meatus for 2 months. On examination, ventral chordee was present, the meatus was orthotopic, the prepuce was normal, and a ~0.5X0.5 cm swelling was present near the meatus on the ventral aspect with watery discharge. During operation, it was found that this swelling had communication with the urethra, and it was an epidermoid cyst on histopathologic examination.

Avinash Dutt Sharma, Malay Kumar Bera, Anup Kumar Kundu

Submitted January 30, 2012 - Accepted for Publication February 21, 2013

KEYWORDS: Chordee, hypospadias, congenital, preputial

CORRESPONDENCE: Avinash Dutt Sharma, M.S., Institute of Postgraduate Medical Education & Research, Kolkata, West Bengal, India ()

CITATION: UroToday Int J. 2013 April;6(2):art 24.



Ventral penile curvature, or chordee, is common in hypospadias but is much less frequent when the meatus is orthotopic [1]. It rarely presents in isolated form, and its association with a symptomatic epidermoid cyst is very rare and has not been reported. The etiology and management of this condition continues to be a subject of debate in the literature.


A 12-year-old boy presented with chief complaints of painful erections, especially early in the morning, over the last year and a watery discharge from swelling located near the meatus for 2 months. There was no history of fever, nausea, vomiting, or voiding difficulty. He attained all developmental milestones normally. There was no other comorbid illness. His general and systemic examination revealed no abnormality. On local examination, ventral chordee was present, the meatus was orthotopic and normal, the prepuce was normal, and a ~0.5X0.5 cm swelling was present near the meatus on the ventral aspect and had a watery discharge. Both testes had normally descended. A testicular and renal ultrasound was done to rule out any other associated malformation, and imaging results were normal.

After a proper pre-anesthetic check-up, he was taken for definitive surgery. A circumcising incision 5 mm behind the corona was made dorsally, and then the swelling communicating with the urethra was excised ventrally, and chordee correction was done. A transverse preputial island flap was made to cover the short urethra. Closure was done after a dorsal incision on the penile skin. The postoperative period was satisfactory. The histopathologic diagnosis of the swelling was an epidermoid cyst.


Isolated chordee without hypospadias is rare, and its association with a symptomatic, communicating epidermoid cyst is very rare. The etiology and management of this condition continues to be a topic of debate in literature. Penile epidermoid cysts are uncommon. They are lined by well-developed, stratified epithelium without skin appendages. Although the etiology is unknown, some reports have suggested that it may originate from a median raphe cyst, possibly arising from abnormal embryological closure of the median raphe or, rarely, may be acquired after mechanical implantation, such as that involving injection of epidermal fragments. They usually do not communicate with the urethra. This type of presentation of a communicating, symptomatic epidermoid cyst has not been reported in published literature to the best of our knowledge [2]. Developmental arrest is a presumed cause of hypospadias and often results in curvature with a relative shortening of ventral structures.

In 1973, Devine and Horton classified chordee without hypospadias into groups based on the different etiologies involved. Type I is considered the most 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. 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. In 1937, Young proposed that chordee without hypospadias was due to congenital, short urethra and suggested transection and reconstruction of the curved ventral urethra. 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 [3].

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 the ventral corporal organs in severe penile curvature [5,6,7]. Successful repair of chordee without hypospadias should produce a straight phallus with a urethral meatus that permits normal urination.


Congenital, isolated chordee is a rare entity, especially when it presents in association with a symptomatic, communicating, epidermoid cyst. 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. Preschool age is the ideal age for correcting this pathology.


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  1. Donnahoo, K. K., M. P. Cain, et al. (1998). “Etiology, management and surgical complications of congenital chordee without hypospadias.” J Urol 160(3 Pt 2): 1120-1122.
  2. Asarch, R. G., L. E. Golitz, et al. (1979). “Median raphe cysts of the penis.” Arch Dermatol 115(9): 1084-1086.
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  3. Devine, C. J., Jr. and C. E. Horton (1973). “Chordee without hypospadias.” J Urol 110(2): 264-271.
  4. Kramer, S. A., G. Aydin, et al. (1982). “Chordee without hypospadias in children.” J Urol 128(3): 559-561.
  5. Snodgrass, W. (2007). “A farewell to chordee.” J Urol 178(3 Pt 1): 753-754.
    PubMed | CrossRef
  6. Duckett, J. W. (1998). “Hypospadias.” In: P. C. Walsh, A. B. Retic, E. D. Vaughan, A. J. Wein, eds. Campbell’s Urology, 7th ed. W. B. Saunders; Philadelphia, PA: 2112.
  7. Duckett, J. W. (2002). “The island flap technique for hypospadias repair. 1981.” J Urol 167(5): 2148-2152; discussion 2157-2148.