Diphallia

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Diphallia
Diphallia 01.jpg
The external male genitalia of a prepubescent male with a form of bifid diphallia

Diphallia, penile duplication (PD), diphallic terata, or diphallasparatus is an extremely rare developmental abnormality in which a male is born with two penises. [1] [2] The first reported case was by Johannes Jacob Wecker in 1609. [3] [4] Its occurrence is 1 in 5.5 million boys in the United States. [5]

Contents

When diphallia is present, it is usually accompanied by renal, vertebral, hindgut, anorectal or other congenital anomalies. There is also a higher risk of spina bifida. [4] Infants born with diphallia and its related conditions have a higher death rate from various infections associated with their more complex renal or colorectal systems.[ citation needed ]

It is generally 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 ]

The first case was reported by Wecker in Bologna, Italy, in 1609, and since then, about one hundred cases have been reported. [1] [6] This condition has existed in humans since ancient times. [7] The two external genitalia may vary in size and shape, [8] either lying beside each other in a sagittal plane or one above the other in a frontal plane. [9]

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. [10] According to Vilanora and Raventos, in 1954, a fourth group called pseudodiphallia was added. [11]

The current widely accepted classification, introduced by Aleem in 1972, classifies double penis into two groups: true diphallia and bifid phallus. [8] True diphallia is caused by cleavage of pubic tubercle; bifid phallus is caused by separation of pubic tubercle. [8] [12] Each of these two groups is further subdivided into partial or complete. [8] True diphallia is where each phallus has two corpora cavernosa and a single corpus spongiosum containing a urethra. [8] [12] 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. [8] In bifid phallus, each phallus has only one corpus cavernosum and one corpus spongiosum containing a urethra. [8] [12] Separation of penises down to the base of the penile shaft is complete bifid, whereas to glans is partial bifid. [8] 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. [13] In partial bifid phallus, the duplication of urethra, 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. [14]

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. [1] [8] Penile duplication also varies from a single penis with double glans to complete double penises. [6] The meatus may be normal at tip of glans, hypospadiac, or epispadiac; the scrotum may be normal or bifid. [14] True diphallia is more likely accompanied with associated anomalies and malformations compared with bifid phallus. [1] Infants born with diphallia have higher death rate due to infections associated with anomalies. [13]

Complete true diphallia with associated anomalies

A two-day-old male newborn, [6] associated with complex genitourinary and ano-rectal malformation. He had imperforated anus, hypospadias, bifid scrotum, meatuses on both glandes, two bladders and two colons, and had normal testes, kidneys and ureters.

Partial true diphallia or pseudodiphallia, without associated anomalies

Partial true diphallia corresponds to pseudodiphallia. [1] Pseudodiphallia is formed from erectile tissue only, undifferentiated and non-functional. [13] It is independent of the normal penis [10] and can be removed surgically without problem. [13] This rare case was reported, [13] 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, [1] associated with bifid scrotum, epispadia and 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, [14] 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 shaft, and only urinated through the larger glans. He had a patent anus, one bladder, one normal urethra, normal gastrointestinal and genitourinary systems.

Cause

Cause of diphallia is unknown, [13] and also because its associated anomalies vary largely, it is impossible to give a simple, single explanation of its cause. [12] But it is thought to have started from duplication of cloacal membrane in early embryonic development stages in the fetus, between third and sixth week of gestation, [13] [7] because normal development of cloacal folds in the fetus is complete between this time. [10] [15]

Normal development of penis occurs with the fusion of cloacal tubercles at anterior end of urogenital sinus. [1] [13] 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. [13] [1] [12] 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. [1] [12] The concept of caudal duplication syndrome is used to explain the symptoms of diphallia with associated complex anomalies in lower abdomen and urinary tract. [15] [12] 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 [1] and double penises, as well as producing associated anomalies such as double bladders, double urethra, double colons and imperforated anus. [13] [12] Failure in proper fusion of urethral folds results in hypospadias. [16] Failure in mesoderm cell migration results in epispadia. [17]

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. [11] [2]

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. [1]

See also

Related Research Articles

<span class="mw-page-title-main">Glans penis</span> End of the penis

In male human anatomy, the glans penis, commonly referred to as the glans, is the bulbous structure at the distal end of the human penis that is the human male's most sensitive erogenous zone and primary anatomical source of sexual pleasure. The glans penis is present in the male reproductive organs of humans and most other mammals where it may appear smooth, spiny, elongated or divided. It is externally lined with mucosal tissue, which creates a smooth texture and glossy appearance. In humans, the glans is located over the distal ends of the corpora cavernosa and is a continuation of the corpus spongiosum of the penis. At the summit appears the urinary meatus and at the base forms the corona glandis. An elastic band of tissue, known as the frenulum, runs on its ventral surface. In men who are not circumcised, it is completely or partially covered by a fold of skin called the foreskin. In adults, the foreskin can generally be retracted over and past the glans manually or sometimes automatically during an erection.

The glans is a vascular structure located at the tip of the penis in male mammals or a homologous genital structure of the clitoris in female mammals.

<span class="mw-page-title-main">Hypospadias</span> Medical condition

Hypospadias is a common variation in fetal development of the penis in which the urethra does not open from its usual location on the head of the penis. It is the second-most common birth defect of the male reproductive system, affecting about one of every 250 males at birth. Roughly 90% of cases are the less serious distal hypospadias, in which the urethral opening is on or near the head of the penis (glans). The remainder have proximal hypospadias, in which the meatus is all the way back on the shaft of the penis, near or within the scrotum. Shiny tissue that typically forms the urethra instead extends from the meatus to the tip of the glans; this tissue is called the urethral plate.

<span class="mw-page-title-main">Epispadias</span> Medical condition

An epispadias is a rare type of malformation in which the urethra ends, in males, in an opening on the upper aspect of the penis, and in females when the urethra develops too far anteriorly. It occurs in around 1 in 120,000 male and 1 in 500,000 female births.

Genital reconstructive surgery may refer to:

<span class="mw-page-title-main">Intersex medical interventions</span> Performed to modify atypical or ambiguous genitalia

Intersex medical interventions (IMI), also known as intersex genital mutilations (IGM), are surgical, hormonal and other medical interventions performed to modify atypical or ambiguous genitalia and other sex characteristics, primarily for the purposes of making a person's appearance more typical and to reduce the likelihood of future problems. The history of intersex surgery has been characterized by controversy due to reports that surgery can compromise sexual function and sensation, and create lifelong health issues. The medical interventions can be for a variety of reasons, due to the enormous variety of the disorders of sex development. Some disorders, such as salt-wasting disorder, can be life-threatening if left untreated.

<span class="mw-page-title-main">Genital tubercle</span> Body of tissue present in the development of the reproductive system

A genital tubercle, phallic tubercle, or clitorophallic structure is a body of tissue present in the development of the reproductive system. It forms in the ventral, caudal region of mammalian embryos of both sexes, and eventually develops into a primordial phallus. In the human fetus, the genital tubercle develops around week 4 of gestation, and by week 9 becomes recognizably either a clitoris or penis. This should not be confused with the sinus tubercle which is a proliferation of endoderm induced by paramesonephric ducts. Even after the phallus is developed, the term genital tubercle remains, but only as the terminal end of it, which develops into either the glans penis or the glans clitoridis.

<span class="mw-page-title-main">Bladder exstrophy</span> Medical condition

Bladder exstrophy is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex, and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall. Its presentation is variable, often including abnormalities of the bony pelvis, pelvic floor, and genitalia. The underlying embryologic mechanism leading to bladder exstrophy is unknown, though it is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm. Exstrophy means the inversion of a hollow organ.

<span class="mw-page-title-main">Male reproductive system</span> Reproductive system of the human male

The male reproductive system consists of a number of sex organs that play a role in the process of human reproduction. These organs are located on the outside of the body, and within the pelvis.

<span class="mw-page-title-main">Spongy urethra</span> Part of the male urethra

The spongy urethra is the longest part of the male urethra, and is contained in the corpus spongiosum of the penis.

<span class="mw-page-title-main">Urinary meatus</span> Orifice of the urethra

The urinary meatus, also known as the external urethral orifice, is the opening of the urethra. It is the point where urine exits the urethra in both sexes and where semen exits the urethra in males. The meatus has varying degrees of sensitivity to touch.

<span class="mw-page-title-main">Human penis</span> Human male external reproductive organ

In human anatomy, the penis is an external male sex organ that additionally serves as the urinary duct. The main parts are the root, body, the epithelium of the penis including the shaft skin, and the foreskin covering the glans. The body of the penis is made up of three columns of tissue: two corpora cavernosa on the dorsal side and corpus spongiosum between them on the ventral side. The human male urethra passes through the prostate gland, where it is joined by the ejaculatory duct, and then through the penis. The urethra traverses the corpus spongiosum, and its opening, the meatus, lies on the tip of the glans. It is a passage both for urination and ejaculation of semen.

Aphallia is a congenital malformation in which the phallus is absent. It is also known as penile agenesis in the case of males. The word is derived from the Greek a- for "not", and phallos for "penis". It is classified as an intersex variation.

The development of the reproductive system is the part of embryonic growth that results in the sex organs and contributes to sexual differentiation. Due to its large overlap with development of the urinary system, the two systems are typically described together as the genitourinary system.

<span class="mw-page-title-main">Foreskin</span> Retractable fold of skin which covers and protects the glans of the penis

In male human anatomy, the foreskin, also known as the prepuce, is the double-layered fold of skin, mucosal and muscular tissue at the distal end of the human penis that covers the glans and the urinary meatus. The foreskin is attached to the glans by an elastic band of tissue, known as the frenulum. The outer skin of the foreskin meets with the inner preputial mucosa at the area of the mucocutaneous junction. The foreskin is mobile, fairly stretchable and sustains the glans in a moist environment. Except for humans, a similar structure known as a penile sheath appears in the male sexual organs of all primates and the vast majority of mammals.

Male genital examination is a physical examination of the genital in males to detect ailments and to assess sexual development, and is normally a component of an annual physical examination. The examination includes checking the penis, scrotum, and urethral meatus. A comprehensive assessment of the male genitals assesses the pubic hair based on Sexual Maturity Rating and the size of the testicles and penis. The exam can also be conducted to verify a person's age and biological sex. The genitourinary system can also be assessed as part of the male genital examination. During a genital examination, the doctor can detect any of the following: structural abnormalities, urethral opening abnormalities, problems related to not being circumcised, lumps, tumors, redness, excoriation, edema, lesions, swelling, cancer, hair-related issues, and many others. In some instances where a physical examination of the male genitals is not sufficient to diagnose an individual, then an internal genital examination using imaging or ultrasounds will be needed for further evaluation.

<span class="mw-page-title-main">Septum glandis</span> Part of the human glans penis

The septum glandis, also septum of the glans, refers to the fibrous partition of the ventral aspect of the glans penis that separates the two glans wings in the ventral midline. The septum extends from the urethral meatus through the glanular urethra and ends in the tunica albuginea of the human penis. Externally it is attached to the frenulum which extends lower on the neck of the penis.

A penile injury is a medical emergency that afflicts the penis. Common injuries include fracture, avulsion injury, strangulation, entrapment, and amputation.

<span class="mw-page-title-main">Penile raphe</span> Line of tissue on the human penis

The penile raphe is a visible line or ridge of tissue that runs on the ventral (urethral) side of the human penis beginning from the base of the shaft and ending in the prepuce. The line is typically darker than the rest of the shaft skin, even though its shape and pigmentation may vary greatly among males. The penile raphe is part of a broader line in the male reproductive organs, that runs from the anus through the perineum and continues to the scrotum and penis, collectively referred to as median raphe. The penile raphe is homologous to the female labia minora.

References

  1. 1 2 3 4 5 6 7 8 9 10 11 Tirtayasa, Pande (2013). "Diphallia with Associated Anomalies: A Case Report and Literature Review". Case Reports in Urology. 2013: 192960. doi: 10.1155/2013/192960 . PMC   3870645 . PMID   24383036.
  2. 1 2 Aparicio-Rodríguez, J (2010). "Disorders of sexual development in genetic pediatrics: Three different ambiguous genitalia cases report from hospital para el Nino Poblano, Mexico". International Journal of Genetics and Molecular Biology. 2 (10): 207–216.
  3. ↑ Sharma KK, Jain R, Jain SK, Purohit A (2000). "Concealed diphallus :a Case report and review of the literature". Journal of Indian Association of Pediatric Surgeons. 5 (1): 18–21.
  4. 1 2 Mirshemirani, AR; Sadeghyian, N; Mohajerzadeh, L; Molayee, H; Ghaffari, P (2010). "Diphallus: Report on six cases and review of the literature". Iranian Journal of Pediatrics. 20 (3): 353–7. PMC   3446048 . PMID   23056729.
  5. ↑ "Indian man wants op to remove extra organ". Reuters. 19 August 2006. Archived from the original on 22 January 2007. Retrieved 18 August 2006.
  6. 1 2 3 Mirshemirani, Ali-Reza (2010). "Diphallus: Report on Six Cases and Review of the Literature". Iranian Journal of Pediatrics. 20 (3): 353–357. PMC   3446048 . PMID   23056729.
  7. 1 2 Karabagli, Murat (2017). "Bifid phallus with complete duplication and a separate scrotum in a German shepherd dog: a case report". Veterinarni Medicina. 62 (4): 226–230. doi: 10.17221/16/2017-VETMED .
  8. 1 2 3 4 5 6 7 8 9 Gyftopoulos, Kostis (2002). "Clinical and embryologic aspects of penile duplication and associated anomalies". Urology. 60 (4): 675–679. doi:10.1016/S0090-4295(02)01874-5. PMID   12385932.
  9. ↑ Wojewski, A (1964). "Total diphallia: A case of plastic repair". Plastic and Reconstructive Surgery. 34 (1): 84–6. doi:10.1097/00006534-196407000-00032. PMID   14104422.
  10. 1 2 3 Aboodi, M.d M (2005). "Accessory Pseudophallus With Accessory Pseudoscrotum Detected During Antenatal Sonographic Scanning". Journal of Ultrasound in Medicine. 24 (8): 1163–1166. doi:10.7863/jum.2005.24.8.1163. PMID   16040834. S2CID   42171596.
  11. 1 2 Kundal, Vijay (2013). "A rare case of isolated complete diphallia and review of the literature". BMJ Case Reports. 2013: bcr2012008117. doi:10.1136/bcr-2012-008117. PMC   3603707 . PMID   23413289.
  12. 1 2 3 4 5 6 7 8 deVries, Catherine (2013). "Congenital anomalies in children" (PDF). Retrieved 18 April 2018.
  13. 1 2 3 4 5 6 7 8 9 10 Rossete-Cervantes, H (2016). "Diphallia: a case report". Revista Médica del Instituto Mexicano del Seguro Social. 54 (3): 401–3. PMID   27100989 – via PubMed.
  14. 1 2 3 Karagöz, Yeşim (2014). "Isolated penile duplication: case report and literature review". Causapedia. 3: 762. S2CID   35108939.
  15. 1 2 Elumalai, Ganesh (2017). ""PENILE DUPLICATION" Embryological basis and its clinical importance". Elixir Embryology. Retrieved 14 April 2018.
  16. ↑ Elumalai, Ganesh (2017). ""HYPOSPADIAS" ITS EMBRYOLOGICAL BASIS AND CLINICAL IMPORTANCE". Elixir Embryology: 44481–44487.
  17. ↑ Levin, Terry (2007). "Congenital anomalies of the male urethra". Pediatric Radiology. 37 (9): 851–862. doi:10.1007/s00247-007-0495-0. PMC   1950215 . PMID   17572890.

Notes

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