Introduction. Diphallia is a rare congenital deformity with an incidence of one in every five million births [1]. Glans duplication, its rarest form might only be encountered once in a lifetime. However there might be simpler cases such as in our patient that are underreported due to individual’s embarrassment. So our case is interesting because it might help doctors include this deformity in their differential diagnosis and easier recognise it. Moreover doctors dealing with such patients may benefit from our treatment and outcome experience as this is one of very few cases reported in literature and was treated in a unique way.
Case presentation. A 6-year-old boy presented with a small preputial lesion. Familly GP gave us a differential diagnosis of either a granuloma, or an epidermal inclusion cyst, or an isolated genital wart. The patient was born by vaginal delivery and had no previous medical history. No family history of urological problems was reported. On examination the boy was found to be in good general condition. Physical examination did not reveal any pathologic findings except diphalia.
The penile shaft presented indeed a small lesion (Figure 1). This on closer look was diagnosed as a rare case of partial glans duplication. One, smaller but otherwise normal appearing gland was observed in the sagittal plane on top of the larger one (Figure 1). This sagittal Erection mainly affected the large glans but in a milder degree could also be observed in the smaller. Testes were normal in size and intrascrotally located.
The child was investigated preoperatively. Blood tests were normal. Urethrography revealed a normal urethra ending in a normal urethral meatus on the larger glans. The smaller glans had a urethral meatus that ended blindly. At endoscopy, urethra and bladder were normal. Two ureteral orifices were present.
Since the deformity was accompanied by neither second urethra nor congenital anomalies, the patient was subjected to RFA (RadioFrequency Ablation). RFA is an electrosurgical technique that uses a high frequency alternating current to heat tissues to the point of desiccation. RFA was used because it was available and was considered that it might offer faster and more durable hemostasis for this case, without having to put any sutures, thus reducing anesthetic time, postoperative pain and time to return to full activities. The partial glans was successfully removed. Finally, cosmetic reduction of the excess foreskin was realized and, although the scar was visible at three months, healing was complete (Figure 2). Pathology verified glans histology.
The boy comes yearly for clinical examination. Five years after surgical treatment the patient is well. He voids normally and cosmetic appearance is excellent.
Discussion. Diphallia (penile duplication) has an estimated frequency of one per 5,000,000 neonates [1, 2]. Wecker in 1609 is credited with being the first to publish a case of diphallia. The estimated number of cases reported to date is about 100, but it is possible that minor forms of this anomaly have never been seen by a physician because of the individual’s embarrassment over the malformation [3]. Glans duplication (GD) is the rarest form of diphallia and has unique clinical implications. According to the classification proposed by Jesus et al GD is part of the partial duplication category and is rarely associated with severe malformations. Per definition, the patient presents two glans and a single shaft, with only two palpable corpora. GD occurs almost exclusively following the sagittal plane (1 glans in front of the other) as in our case. Coronal duplication (glans side by side) is uncommon and the reported cases are arguable [2].
There are multiple embryological explanations for diphallia and systemic anomalies, but most researchers agree that it is a defect connecting the genital tubercle. It is hypothesised that diphallia during the gestation period occurs between 23 and 25 days, when the fetal caudal mass of the mesoderm is deeply disturbed by trauma, drugs or infections [4].
The rarity of the malformation limits the experience of each author: only case reports of GD are available and only nine (now ten) cases have been reported since 1980, including our own case, described here [2]. It is interesting to notice that despite the presence of a notable penile abnormality some patients seek medical help late after infancy normally complaining of esthetic and/or sexual problems or an abnormal voiding pattern [5–7]. Others are reported as incidental findings as in our case [8]. Surprisingly, some have been previously submitted to circumcision [8–10] and the accessory hypoplastic glans has been noticed only after the procedure, in one case as “post-circumcision granuloma.” There are two reports of worsening of a “penile nodule” after puberty that corresponded to a duplicated glans, accentuating a mild deformity and causing dyspareunia [7, 11]. GD normally demands only local reconstructive surgery, including penile urethral surgery.
Conflict of interest. There are no conflicts of interest
Funding. No sources of funding
Experimental ethics. Our work conforms to standards currently applied in Greece.
Consent. Patient’s parents gave consent for the material presented about their child to appear in this publication and in related publications. It has been explained to them that the material has educational or scientific value and that publication may help to improve the care that others will receive in the future, however authors/the patient will not receive any financial benefit. Parents had had the opportunity to see and read the material (including the text and any other media – pictures, videos etc.) to be submitted for publication and understand that the final publication may differ in style, grammar, consistency and length. They also understand: 1. Although the publication is primarily aimed at medical professionals and academic researchers, it will potentially be freely available to the general public anywhere in the world without time limit and may be used for commercial purposes. 2. Although parent’s/the patient’s name will not be attached and efforts will be made to protect anonymity, complete anonymity cannot be guaranteed. 3. Parent’s consent and the material itself cannot be withdrawn after publication. 4. The material may be used in full or in part or translated in other publications or products derived from this publication.
Guarantor. Efstratios Christianakis PhD, Chief of Pediatric Surgery Department, General Children’s Hospital of Penteli, Ippokratous 8, Penteli, 15236, Hellas, e-mail: xristianakis@yahoo.gr
