Diphallia: Difference between revisions

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'''Diphallia''', or penile duplication, is a [[medical]] condition in which a male infant is born with two [[penis]]es. It is estimated that one out of 5 million live births in the United States results in a diphallic [[birth defect]].

{{short description|Genital medical condition}}

{{Infobox medical condition (new)

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| alt = The external male genitalia of a prepubescent male with a form of bifid diphallia with an anomaly around the mons pubis

| caption = The external male genitalia of a prepubescent male with a form of bifid diphallia

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'''Diphallia''', '''penile duplication''' ('''PD'''), '''diphallic terata''', or '''diphallasparatus''' is an extremely rare [[developmental abnormality]] in which a male is born with two [[Human penis|penises]].<ref name=":0">{{Cite journal|last=Tirtayasa|first=Pande|date=2013|title=Diphallia with Associated Anomalies: A Case Report and Literature Review|journal=Case Reports in Urology|volume=2013|page=192960|pmc=3870645|pmid=24383036|doi=10.1155/2013/192960|doi-access=free}}</ref><ref name=":1">{{Cite journal|last=Aparicio-Rodríguez|first=J|date=2010|title=Disorders of sexual development in genetic pediatrics: Three different ambiguous genitalia cases report from hospital para el Nino Poblano, Mexico|url=https://www.researchgate.net/publication/259764332|journal=International Journal of Genetics and Molecular Biology|volume=2 |issue=10|pages=207–216}}</ref> The first reported case was by [[Johannes Jacob Wecker]] in 1609.<ref name=j1>{{cite journal|url=http://www.jiaps.com/article.asp?issn=0971-9261;year=2000;volume=5;issue=1;spage=18;epage=21;aulast=Sharma;type=0|title=Concealed diphallus :a Case report and review of the literature|journal=Journal of Indian Association of Pediatric Surgeons |year = 2000 |volume = 5 | issue = 1 | pages =18–21|vauthors=Sharma KK, Jain R, Jain SK, Purohit A }}</ref><ref name=j2>{{cite journal|pmid=23056729|year=2010|last1=Mirshemirani|first1=AR|last2=Sadeghyian|first2=N|last3=Mohajerzadeh|first3=L|last4=Molayee|first4=H|last5=Ghaffari|first5=P|title=Diphallus: Report on six cases and review of the literature|volume=20|issue=3|pages=353–7|pmc=3446048|journal=Iranian Journal of Pediatrics}}</ref> Its occurrence is 1 in 5.5 million boys in the United States.<ref name=r1>{{cite web|url=http://today.reuters.com/News/CrisesArticle.aspx?storyId=DEL270522|title=Indian man wants op to remove extra organ|publisher=Reuters|access-date=18 August 2006|archive-url=https://web.archive.org/web/20070122012925/http://today.reuters.com/News/CrisesArticle.aspx?storyId=DEL270522|archive-date=22 January 2007|url-status=dead|date=19 August 2006}}</ref>

When diphallia is present, it is usually accompanied by other [[congenitalkidney|renal]] anomalies such as, [[renalvertebra]]l, [[Vertebra|vertebralhindgut]], [[hindgutanorectal]] or other [[anorectalcongenital]] duplicationanomalies. There is also a higher risk of [[spina bifida]].<ref name=j2 /> Infants born with PDdiphallia and its related conditions have a higher death rate from various infections associated with their more complex renal or colorectal systems.{{Citation needed|date=January 2023}}

It is thoughtgenerally believed diphallia occurs in the [[fetus]] between the 23rd and 25th days of [[gestation]] when an injury, chemical stress, or malfunctioning [[homeobox]] genes hamper proper function of the [[caudal cell mass]] of the fetal [[mesoderm]] as the [[urogenital sinus]] separates from the [[genital tubercle]] and [[rectum]] to form the penis.{{Citation needed|date=January 2023}}

The first case was reported by Wecker in Bologna, Italy, in 1609, and since then, about one hundred cases have been reported.<ref name=":0" /><ref name=":2">{{Cite journal|last=Mirshemirani|first=Ali-Reza|date=2010|title=Diphallus: Report on Six Cases and Review of the Literature|journal=Iranian Journal of Pediatrics|volume=20 |issue=3|pages=353–357|pmid=23056729|pmc=3446048}}</ref> This condition has existed in humans since ancient times.<ref name=":3">{{Cite journal|last=Karabagli|first=Murat|date=2017|title=Bifid phallus with complete duplication and a separate scrotum in a German shepherd dog: a case report|journal=Veterinarni Medicina|volume=62 |issue=4|pages=226–230|doi=10.17221/16/2017-VETMED|doi-access=free}}</ref> The two external genitalia may vary in size and shape,<ref name=":4">{{Cite journal|last=Gyftopoulos|first=Kostis|date=2002|title=Clinical and embryologic aspects of penile duplication and associated anomalies|journal=Urology|volume=60 |issue=4|pages=675–679|doi=10.1016/S0090-4295(02)01874-5|pmid=12385932}}</ref> either lying beside each other in a [[Sagittal plane|sagittal]] plane or one above the other in a [[Coronal plane|frontal]] plane.<ref>{{Cite journal|last=Wojewski|first=A|date=1964|title=Total diphallia: A case of plastic repair|journal=Plastic and Reconstructive Surgery|volume=34 |issue=1|pages=84–6|doi=10.1097/00006534-196407000-00032|pmid=14104422}}</ref>

This rare condition has been documented in [[pig]]s and other [[mammal]]s. It is commonly mistaken that all [[shark]]s have this condition, but in reality they have a pair of "[[clasper]]s" which serve a reproductive function.

According to Schneider classification in 1928, double penis is classified into three groups: (a) glans diphallia, (b) bifid diphallia and (c) complete diphallia or double penis.<ref name=":5">{{Cite journal|last=Aboodi|first=M.d M|date=2005|title=Accessory Pseudophallus With Accessory Pseudoscrotum Detected During Antenatal Sonographic Scanning|journal=Journal of Ultrasound in Medicine|volume=24 |issue=8|pages=1163–1166|doi=10.7863/jum.2005.24.8.1163|pmid=16040834|s2cid=42171596}}</ref> According to Vilanora and Raventos, in 1954, a fourth group called pseudodiphallia was added.<ref name=":6">{{Cite journal|last=Kundal|first=Vijay|date=2013|title=A rare case of isolated complete diphallia and review of the literature|journal=BMJ Case Reports|volume=2013|pages=bcr2012008117|pmc=3603707|pmid=23413289|doi=10.1136/bcr-2012-008117}}</ref>

[[Category:Andrology]]

The current widely accepted classification, introduced by Aleem in 1972, classifies double penis into two groups: true diphallia and bifid phallus.<ref name=":4" /> True diphallia is caused by cleavage of pubic tubercle; bifid phallus is caused by separation of [[pubic tubercle]].<ref name=":4" /><ref name=":7">{{Cite web|url=https://www.siu-urology.org/themes/web/assets/files/ICUD/pdf/congenital_anomalies.pdf|title=Congenital anomalies in children|last=deVries|first=Catherine|date=2013|access-date=18 April 2018}}</ref> Each of these two groups is further subdivided into partial or complete.<ref name=":4" /> True diphallia is where each phallus has two [[Corpus cavernosum penis|corpora cavernosa]] and a single [[Corpus spongiosum penis|corpus spongiosum]] containing a urethra.<ref name=":4" /><ref name=":7" /> True diphallia can be either complete with both penises similar in size, or partial when one of the phallia is smaller in size or immature, though structurally same as the larger phallus.<ref name=":4" /> In bifid phallus, each phallus has only one corpus cavernosum and one corpus spongiosum containing a urethra.<ref name=":4" /><ref name=":7" /> Separation of penises down to the base of the penile shaft is complete bifid, whereas to glans is partial bifid.<ref name=":4" /> For complete bifid phallus associated with anomalies, the anterior urethra is absent from each penis and the [[prostatic urethra]] is situated in the skin between the two penises.<ref name=":8">{{Cite journal|last=Rossete-Cervantes|first=H|date=2016|title=Diphallia: a case report|url=http://revistamedica.imss.gob.mx/editorial/index.php/revista_medica/article/view/349/1149|journal=Revista Médica del Instituto Mexicano del Seguro Social|volume=54 |issue=3|pages=401–3|pmid=27100989|via=PubMed}}</ref> In partial bifid phallus, the duplication of urethra, [[Corpus cavernosum penis|corpora cavernosa]] and corpus spongiosum in one penis is incomplete, and there is only a corpus cavernosum and a spongiosum surrounding the functioning urethra in the other penis.<ref name=":9">{{Cite journal|last=Karagöz|first=Yeşim|s2cid=35108939|date=2014|title=Isolated penile duplication: case report and literature review|journal=Causapedia|volume=3|pages=762}}</ref>

== Notable clinical cases ==

Diphallia is usually accompanied by systemic anomalies; their extent varies, ranging from no associated anomaly to multiple anomalies including urogenital, gastrointestinal and musculoskeletal systems.<ref name=":0" /><ref name=":4" /> Penile duplication also varies from a single penis with double glans to complete double penises.<ref name=":2" /> The meatus may be normal at tip of glans, hypospadiac, or epispadiac; the scrotum may be normal or bifid.<ref name=":9" /> True diphallia is more likely accompanied with associated anomalies and malformations compared with bifid phallus.<ref name=":0" /> Infants born with diphallia have higher death rate due to infections associated with anomalies.<ref name=":8" />

=== Complete true diphallia with associated anomalies ===

A two-day-old male newborn,<ref name=":2" /> associated with complex genitourinary and ano-rectal malformation. He had imperforated anus, [[hypospadias]], bifid scrotum, [[meatus]]es on both glandes, two [[Urinary bladder|bladders]] and two [[Large intestine|colons]], and had normal [[Testicle|testes]], [[kidney]]s and [[ureter]]s.

=== Partial true diphallia or pseudodiphallia, without associated anomalies ===

Partial true diphallia corresponds to pseudodiphallia.<ref name=":0" /> Pseudodiphallia is formed from erectile tissue only, undifferentiated and non-functional.<ref name=":8" /> It is independent of the normal penis<ref name=":5" /> and can be removed surgically without problem.<ref name=":8" /> This rare case was reported,<ref name=":8" /> based on the age of the 83-year-old man when diphallia was only detected then when he was hospitalised, and also because of the absence of other anatomical malformations. The small, immature and nonfunctional penis protruded on one side of the large, normal penis. The secondary penis had glans but without urinary meatus.

=== Complete bifid diphallia with associated anomalies ===

A 12-year-old boy,<ref name=":0" /> associated with bifid [[scrotum]], [[Epispadias|epispadia]] and [[Diastasis symphysis pubis|pubic symphysis diastasis]]. He had two separate penises, similar in size and shape, each penis had an epispadia urethral meatus. He had bifid scrotum, one testicle on each side of the scrotum. A bowel loop-like structure was over the pubis region. This structure had no communication with any other structures. He had a single normal bladder and ureter.

=== Partial bifid diphallia without associated anomalies ===

A 15-year-old boy,<ref name=":9" /> apart from having two penises, had normal external genitalia. His two testicles were within normal scrotum and normally positioned. He had two unequal sized glans at tip of a thick penile [[Body of penis|shaft]], and only urinated through the larger glans. He had a patent anus, one bladder, one normal urethra, normal [[Gastrointestinal tract|gastrointestinal]] and [[Genitourinary system|genitourinary]] systems.

'''Complete diphallia with associated anomalies'''

A 14-year-old boy, presented with continuous urinary incontinence since birth, was found to have complete penile duplication: a primary phallus of normal size with dorsal urethral meatus (epispadias) and a hypoplastic secondary phallus with urethral atresia. Both testes were descended in a normal scrotum. Associated anomalies included congenital fusion of L3–L4 vertebrae, mild lumbar scoliosis, high-pressure neurogenic bladder with low compliance, left grade II vesicoureteral reflux, severe cortical loss in the right kidney, and partial cortical loss in the left kidney. Initial management with clean intermittent catheterization and oral oxybutynin failed to normalize bladder pressures; intradetrusor onabotulinumtoxinA (300 U) injection reduced leakage episodes. Renal function preservation was prioritized before any reconstructive surgery<ref>{{Cite journal |last=Saberi |first=Narjes |last2=Rajaei Rizi |first2=Farid |last3=Nozari |first3=Mohammad |date=2025-08-19 |title=Penile Duplication (Diphallia) with Epispadias, Neurogenic Bladder, and Lumbar Vertebral Fusion: An Exceptionally Rare Multisystem Congenital Association |url=https://www.sciencedirect.com/science/article/pii/S2214442025002402 |journal=Urology Case Reports |pages=103169 |doi=10.1016/j.eucr.2025.103169 |issn=2214-4420|doi-access=free }}</ref>.

== Cause ==

Cause of diphallia is unknown.<ref name=":8" /> Its associated anomalies vary largely, ergo it is impossible to give a simple, single explanation of its cause.<ref name=":7" /> It is thought to occur due to duplication of cloacal membrane in early embryonic development stages in the fetus, between the third and sixth week of gestation,<ref name=":8" /><ref name=":3" /> as normal development of cloacal folds in the fetus is typically complete between this time.<ref name=":5" /><ref name=":15">{{Cite journal|url=https://www.academia.edu/31567090|title="PENILE DUPLICATION" Embryological basis and its clinical importance|last=Elumalai|first=Ganesh|date=2017|website=Elixir Embryology|access-date=14 April 2018}}</ref>

Normal development of penis occurs with the fusion of cloacal tubercles at anterior end of urogenital sinus.<ref name=":0" /><ref name=":8" /> Mesenchyme migrate around cloacal membrane, proliferate and expand around cloacal plate, forming a pair of cloacal folds that fuse to form genital tubercle which develops into penis.<ref name=":8" /><ref name=":0" /><ref name=":7" /> If cloacal membrane is doubled, mesenchyme will migrate and surround both cloacal membranes, leading to the formation of two pairs of cloacal folds around two cloacal membranes, resulting in formation of two genital tubercles and thus two penises.<ref name=":0" /><ref name=":7" /> The concept of caudal duplication syndrome is used to explain the symptoms of diphallia with associated complex anomalies in lower abdomen and urinary tract.<ref name=":15" /><ref name=":7" /> Further, as mesenchyme migrate from more than one area, failure in migration and in the fusion of mesoderm results in formation of two genital tubercles <ref name=":0" /> and double penises, as well as producing associated anomalies such as double bladders, double urethra, double colons and imperforated anus.<ref name=":8" /><ref name=":7" /> Failure in proper fusion of urethral folds results in hypospadias.<ref>{{Cite journal|last=Elumalai|first=Ganesh|date=2017|title="HYPOSPADIAS" ITS EMBRYOLOGICAL BASIS AND CLINICAL IMPORTANCE|url=https://www.researchgate.net/publication/313468678|journal=Elixir Embryology|pages=44481–44487}}</ref> Failure in mesoderm cell migration results in epispadia.<ref name=":16">{{Cite journal|last=Levin|first=Terry|date=2007|title=Congenital anomalies of the male urethra|journal=Pediatric Radiology|volume=37|issue=9|pages=851–862|doi=10.1007/s00247-007-0495-0|pmid=17572890|pmc=1950215}}</ref>

Diphallia is a rare abnormal external genitalia. The cause is uncertain, but most scientists agree that diphallia is a defect of genital tubercle, and occurs at about week three of gestation, when caudal cell mass of mesoderm is affected by various external environmental factors including drugs, infections and malfunctioning [[Homeobox Genes|homeobox genes]].<ref name=":6" /><ref name=":1" />

== Treatment ==

Treatment is a case-by-case analysis taking into account considerations for medical and ethical reasons, and involves surgical excision of the non-functioning penis.<ref name=":0" />

== See also ==

* [[Bifid penis]]

* [[Penile agenesis]]

* [[Polyorchidism]]

* [[Supernumerary body part]]

* [[Uterus didelphys]]

* [[Vaginal septum]]

== References ==

{{reflist}}

== Notes ==

* A scientific paper of ''triphallia'' (three penises) in a marine snail was reported.({{cite journal|doi=10.4067/S0717-95022012000300003 |title=One Case of Triphallia in the Marine Snail ''Echinolittorina peruviana'' (Caenogastropoda: Littorinidae)|year=2012|last1=Castillo|first1=Viviana M|last2=Brown|first2=Donald I|journal=International Journal of Morphology|volume=30|issue=3|pages=791–796|doi-access=free}})

* In 2021, the first known case of a human with triphallia was recorded in Iraq.({{cite web|url=https://www.sciencetimes.com/articles/30463/20210403/baby-iraq-first-known-case-humans-born-three-male-genitalia.htm|title=Triphallia: First Human Case of Baby Born With Three Male Genitalia Reported|date=3 April 2021}})

== Further reading ==

* Chadha R, Bagga D, Gupta S, Mahajan JK. (July 2001). [https://pubmed.ncbi.nlm.nih.gov/11431797/ "Complete diphallia associated with features of covered exstrophy."] 36 (7): E12, [[W.B. Saunders Company]]. [[National Center for Biotechnology Information]], [[US National Library of Medicine]], [[National Institutes of Health]]

== External links ==

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{{Male congenital malformations of genital organs, indeterminate sex and pseudohermaphroditism}}

{{Authority control}}

[[Category:Congenital disorders of male genital organs]]

[[Category:Penis]]

[[Category:Supernumerary body parts]]

[[Category:Rare diseases]]

[[Category:Intersex variations]]