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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Table of Contents   
CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 6  |  Page : 537-540
 

Congenital pseudodiphallia –A rare case presentation


Department of General Surgery, School of Medical Sciences and Research, Sharda Hospital, Greater Noida, Uttar Pradesh, India

Date of Submission10-Feb-2023
Date of Decision07-Aug-2023
Date of Acceptance08-Aug-2023
Date of Web Publication02-Nov-2023

Correspondence Address:
Amit Kumar Mishra
Department of General Surgery, School of Medical Sciences and Research, G. B. Nagar, Greater Noida, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_30_23

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   Abstract 


Diphallia (penile duplication) is a rare congenital malformation with an incidence of about 1 per 5–6 million newborns. The severity of diphallia varies from a small accessory penile-like tissue to complete true penile duplication with other deformities, usually involving the urogenital, gastrointestinal, and musculoskeletal systems. Pseudodiphallia, as a rare kind of diphallia, is characterized by a small accessory penile-like tissue without a normal penile anatomy structure. A 5.5-year-old male child was brought to the pediatric surgery outpatient department by the parents with complaint of difficulty in retracting the prepucial foreskin and the presence of some growth near the glans. There were no other complaints in specific. Clinical examination revealed foreskin retractable with difficulty and small conical lump smaller than the original glans approximately size ~1 cm diameter at the base attached horizontally at the left side of the original glans at the coronal sulcus and visible incomplete clefting in between the 2 glans visible from the aerial view. After approval from anesthetist, the patient was operated under general anesthesia by excision of pseudodiphallia. Urethral catheterization and circumcision of the penis after taking informed parental consent. Postoperatively, the period was uneventful. The patient responded well to the symptomatic treatment and was orally allowed on the same day evening. Urinary catheter was removed on 5th postoperative day. The patient was discharged on oral symptomatic medication and was advice for follow-up.


Keywords: Diphallia, penis, phallus, pseudo


How to cite this article:
Mishra AK, Anand V, Kale R. Congenital pseudodiphallia –A rare case presentation. J Indian Assoc Pediatr Surg 2023;28:537-40

How to cite this URL:
Mishra AK, Anand V, Kale R. Congenital pseudodiphallia –A rare case presentation. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Nov 28];28:537-40. Available from: https://www.jiaps.com/text.asp?2023/28/6/537/389326





   Introduction Top


Diphallia or penile duplication is rare congenital anomaly, the incidence being 1/5 to 6 million new births.[1],[2] Despite being a rarity, the severity of confirmed cases has myriad presentations ranging from just a small accessory appendage such as penis on one end of the spectrum to complete true penile duplication on other extreme ends, which may or may not be associated with concomitant anomalies of urogenital, musculoskeletal, and gastrointestinal systems.[3] In contrast to true diphallia, pseudodiphallia is furthermore rarity, usually presenting as a small accessory penile-like structure without a normal complete internal penile anatomy. Pseudodiphallia patients usually do not have associated ureteral, renal, or other congenital malformations.[4],[5] We present such a case of pseudodiphallia. Despite being known since the 17th century (Wecker in 1609), there have been about 100 cases reported so far.


   Case Report Top


A 5.5-year-old male child was brought to the pediatric surgery outdoor patient department by the parents with complaints of difficulty in retracting the prepucial foreskin and the presence of some growth near the glans. There were no other complaints in specific. Perinatal, personal, developmental, and family histories were unremarkable.

Clinical examination revealed foreskin retractable with difficulty and small conical lump smaller than the original glans approximately size ~1 cm diameter at base attached horizontally at the left side of the original glans at the coronal sulcus and conspicuous incomplete clefting in between the 2 glans visible from the aerial view [Figure 1]a. The additional lump was visible only on full retraction of the prepucial skin. The lateral lump has had no opening or dimple or fossa at its apex [Figure 1]b. The child used to pass urine from the urethral meatus situated at the navicular fossa of the central large glans. Once the prepucial skin was rolled back, the covered phallus was externally almost normal and unremarkable in size and appearance. There were no complaints of any deviation of the penis or any pain or discharge. Bilateral testes were normally palpable in the scrotum, and there was no inguinoscrotal swelling or lump present. The patient was admitted, investigated, and treated symptomatically. Ultrasound whole abdomen and inguinoscrotal region were done to aid to the diagnosis of pseudodiaphallia.
Figure 1: (a) Preoperative photographs showing small smooth conical lump slightly adjacent to original glans separated by incomplete clefting. (b) Original meatal opening at the navicular fossa of the main penis was otherwise unremarkable. (c) Intraoperative images showing catheterization of urethra with dissection of the prepucial skin to open up the coronal sulcus to demarcate the accessory phallus boundaries followed by excision and subsequent circumcision

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After preanesthetic approval from anesthetist, the patient was operated under general anesthesia by excision of pseudodiphallia. The conical lump situated at the left side coronal sulcus was attached with small sessile base that was carefully excised in toto from the base and hemostasis was secured by bipolar cautery. Excised specimen was checked for any uretheral opening and the absence of the same was documented. Urethral catheterization and circumcision of the penis after taking informed parental consent [Figure 1]c. Excised lump was sent for histopathological examination. Small aseptic dressing with an infant feeding tube of small size as an indwelling urinary catheter was continued. Postoperative period was uneventful. The patient responded well to the symptomatic treatment and was orally allowed on the same day evening. Urinary catheter was removed on 5th postoperative day [Figure 2]a. The patient was discharged on oral symptomatic medication and was advice for follow-up. Excised tissue sent for histopathological examination revealed pictures tissue partly lined by keratinized stratified squamous epithelium. Subepithelium shows fibro connective tissue with focal areas of inflammatory infiltrate comprising lymphocytes, plasma cells, and neutrophils [Figure 2]c. The patient remained complaint free at home and was able to attend nature's call on its own unaided. In the follow-up, the surgical wound has healed fully and the patient was having almost a normal-looking penis with normal functioning as in ab intio preoperative level [Figure 2]b.
Figure 2: (a and b) Immediate postoperative period. (c) Microscopic pictures tissue partly lined by keratinized stratified squamous epithelium and subepithelium shows fibro connective tissue with focal areas of inflammatory infiltrate comprising lymphocytes, plasma cells, and neutrophils

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   Discussion Top


During normal penile, organogenesis bilateral cloacal tubercles start coalescing at the anterior end of the pars phallica of the urogenital sinus. Mesoderm columns growing rapidly around the lateral margins of the cloacal plate form the genital tubercle. These mesodermal bands arise from more than one area and failure of these bands to fuse is expected to result in bladder exstrophy and split penis, but not true diphallia, leading to assumptions that additional steps are needed for complete diphallia. Probably, a longitudinal duplication of cloacal membrane would permit 3 or 4 columns of primitive streak mesoderm to migrate ventrally around the two cloacal membranes to eventually form two genital tubercles culminating in collateral urethral duplication. Such duplication of cloacal membrane could also explain the frequent concomitant bladder, colon, anal, and spinal anomalies.[2],[3],[4],[5] One school of thought presumes 3–5 weeks of gestation as optimal time for diphallia to set in during embryogenesis. The other thinkers presume that anomalies take place in the process of migration ventrally and fusion of the paired mesodermal anlage by the 15th week of gestation.[4] Although the exact mechanism during embryogenesis leading to penile duplication is uncertain (as genital tubercle is believed to arise as a single structure), it can be understood by defined time frame alterations leading to following (i) pubic tubercle separation during embryogenesis resulting in gain of one corpora and one urethra by each phallus or (ii) cleavage of pubic tubercle leading to each phallus gaining 2 corporal bodies and urethra.[2] Consensus is that the anomaly is due to a defective fusion of the genital tubercle.[2],[3] Moreover, the penile duplication is a result of lack of fusion of the paired mesodermal anlagen of the genital tubercle by the 15th week of gestation.[6]

Diphallia may occur as sporadic or isolated cases or as a part of constellation of multiple congenital malformations not clearly fitting into known congenital anomaly syndromes. Variable presentation ranging from just a small accessory penis-like appendage on one end of the spectrum to complete true penile duplication on other extreme end which may or may not be associated with concomitant anomalies of urogenital such as renal anomalies, ectopic kidney, horseshoe kidney, bladder exstrophy or bladder duplication hypospadias, musculoskeletal such as pubic diastasis, lumbosacral anomalies, and gastrointestinal systems such as imperforate anus.[3] Penile duplication may be orthotropic or ectopic. The penile division may be sagittal or frontal and symmetric or asymmetric, in shape and size.[1],[3] Schneider had divided diphallia into three groups:[2],[3],[4] isolated diphallia of the glans alone, bifid diphallia, and complete diphallia. Villanova and Raventos added a fourth category, pseudodiphallia. The urethra shows a range of variations, from functioning double urethras to the complete absence of the urethra in each penis.[2] The majority have a single corpus cavernosum in each organ. The meatus may be normal, hypospadias, or epispadia, and the scrotum may be normal or bifid. The testes are normal, athropic, or undescended.[2],[4] The most commonly accepted classification for urethral duplication anomalies is described by Effmann et al.[6] Almost all karyotype analyses of patients diagnosed with diphallia are found to be normal.

Gyftopoulos et al.[3] has proposed a classification dividing cases into two broad categories: true diphallia and bifid phallus. Both groups can be further subclassified into partial or complete duplication. True complete diphallia will have two well-developed penises (with 2 corpora cavernosa and 1 corpora spongiosum).[4] True partial diphallia will have a smaller or rudimentary duplicate penis (with complete structures that is 2 corpora cavernosa and 1 corpora spongiosum). If the duplicate penis does not have all the structures, for example, one corpora cavernosum they are classified as bifid phallus. Depending on the degree of separation, bifid phallus is further subclassified into complete and partial. Complete bifid phallus has separation at the base whereas partial bifid phallus has separation at the glans. When the degree of separation is complete to the base of the shaft or to just the glans, the anomaly is considered complete or partial bifid phallus, respectively. The term “pseudodiphallia,” as originally described by Villanova and Raventos, corresponds to true, partial diphallia.[3],[5]

Treatment requires correct diagnosis by imaging techniques starting from ultrasound of the abdomen and genitalia, with other modalities such as contrast-enhanced computerized tomographic scan, magnetic resonance imaging, micturating cystourethrogram, and retrograde urethrogram as required. Treatment planning needs to address ethical, medical, and cosmetic/esthetic issues. Once the corporal development and urethral anatomy are delineated by history, clinical, and radiological assessment, classifying the degree of penile duplication to decide the treatment of excision or reconstruction of duplicated penis. Cases associated with complete duplication with or without associated other systems anomalies present as a surgical challenge, mandating multiple surgeries, or staged procedures.


   Conclusion Top


Such presentations being extremely rare mandates to have a high index of suspicion for early diagnosis in ambiguous cases with early surgical treatment. Patients have variable presentations and so each patients require a separate individualized tailor-made approach taking into account both the penile as well as associated anomalies to get a satisfactory outcome. In pseudodiphallia, the rudimentary phallus is rarely functional, making excision decision easy to finalize a surgical remedy which aims at preserving urinary continence, erectile function, optimum urinary stream, and perfect cosmesis after removal of less or nonfunctioning, noncommunicating, or accessory phallus.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Acknowledgment

The authors express their thanks to the mentioned patient for allowing to share her case in a surgical journal.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Karagözlü Akgül A, Uçar M, Çelik F, Kırıştıoğlu İ, Kılıç N. Complete penile duplication with structurally normal penises: A case report. Balkan Med J 2018;35:340-3.  Back to cited text no. 1
    
2.
Bhat HS, Sukumar S, Nair TB. Successful surgical correction of true diphallia, scrotal duplication, and associated hypospadias. J Pediatr Surg. 2006; 41:13-14.  Back to cited text no. 2
    
3.
Gyftopoulos K, Wolffenbuttel KP, Nıjman RJ. Clinical and embryologic aspects of penile duplication and associated anomalies. Urology. 2002; 60:675-79.  Back to cited text no. 3
    
4.
Gupta M, Virdi VJ. Rare case of isolated true complete diphallus – Case report and review of literature. SAS J Surg 2016;2:23-25.  Back to cited text no. 4
    
5.
Torres ME, Sanchez PJ, Aragon TA, Camacho TV, Colorado GA. “Diphalia.” Rev Mex Urol 2009;69:32-5.  Back to cited text no. 5
    
6.
Effmann EL, Lebowitz RL, Colodny AH. Duplication of the urethra. Radiology. 1976;119:179-85.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

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    Abstract
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