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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 12  |  Issue : 4  |  Page : 379-381  

Agenesis of penis


1 Department of Surgery, MGM Medical College, Kishanganj, Bihar, West Bengal, India
2 Department of Urology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Date of Submission25-Mar-2020
Date of Acceptance16-Aug-2020
Date of Web Publication15-Oct-2020

Correspondence Address:
Prof. Dilip Kumar Pal
Department of Urology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal
India
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DOI: 10.4103/UA.UA_29_20

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   Abstract 


Agenesis of penis is a very rare developmental anomaly, which is usually associated with scrotal hypoplasia or other urological developmental anomalies. Here we psesent such a case who presented to us with stenosis of newly constructed urethra.

Keywords: Agenesis, neo-urethra, penis


How to cite this article:
Pal D, Pal DK. Agenesis of penis. Urol Ann 2020;12:379-81

How to cite this URL:
Pal D, Pal DK. Agenesis of penis. Urol Ann [serial online] 2020 [cited 2020 Oct 29];12:379-81. Available from: https://www.urologyannals.com/text.asp?2020/12/4/379/298183




   Introduction Top


Agenesis of penis is a very rare developmental anomaly with the reported incidence of 1 in 30 million births worldwide.[1] It is believed to result from either the absence of the genital tubercle, or its failure to develop.[2] Absences of corpora cavernosa and corpora spongiosum are prerequisite for this developmental anomaly as claimed by several investigators.[3] Except for the reported XX-XY mosaic, patients have 46 XY karyotypes.[4] More than half of these have associated anomalies, including developmental defects of the caudal axis, genitourinary, and gastrointestinal tract anomalies.[5] The scrotum, testes, and testicular function are usually normal.


   Case Report Top


A 32-year-old male presented with the complaints of the absence of penis since birth and difficulty of micturition through the perineal site since 2 years. His old medical records suggests that he had his urethral opening in the anus at the time of his birth for which, he underwent some sort of surgery at the 1 year of age, when his urethral opening was made at the perineum. He had a complaint of recurrent urinary tract infection in his childhood for which he was evaluated and found to be having right sided gross hydroureteronephrosis and complete duplex system, for which he underwent right nephroureterectomy at his 2 years of age. On histopathological examination, it was found to be having dysgenetic right kidney. In our first visit, on examination, the patient had absent penis with normal looking scrotum and bilateral testes [Figure 1]. Both the testes were normal in size, shape, and consistency. Rugosity was present on the scrotum. Pubic hairs are present with normal male pattern distribution. Anus was present in the normal position, and urethral opening was visible in the perineum [Figure 2]. Rest of the perineal examination was normal. The patient did not have dysmorphic features or clinical evidence of any other associated anomaly. Ultrasonographic (USG) examination of the whole abdomen and pelvis was done which revealed that all the organs are normal (except the absence of right kidney and ureter) and are normally placed in their respective position. Ascending urethrogram was done, which showed to be having meatal stenosis and stenosis of distal urethra [Figure 3]. Meatal dilatation and cystoscopy were done under spinal anesthesia. Cystoscopy suggested as normal urethra, verumontanum, prostate with slightly elevated bladder neck, urinary bladder was normal with only left ureteric opening.
Figure 1: Photograph of pubic area showing the absence of penis

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Figure 2: Urethral opening in the perineum

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Figure 3: Ascending urethrogram showing stricture in the distal urethra

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   Discussion Top


The diagnosis of penile agenesis requires the absence of corpora cavernosa and copora spongiosum with urethra opening at any point on the perineum in midline, over pubis, anterior aspect of the scrotum, or, most frequently just anterior to the anus and anterior wall of the rectum.[6] Embryological basis of this rare anomaly has been described as a result of failure of genital tubercle to develop with incomplete separation of the urogenital sinus from the hindgut by the urorectal septum. This rare entity should be differentiated from concealed penis, rudimentary penis, micropenis, epispadias, hypospadias pseudohermaphroditism, and intrauterine amputation of penis.[7],[8] Anorectal anomalies such as imperforate anus, congenital rectal strictures and rectovesical fistula, cryptorchid testis, hydrocele, hernia, renal dysplasia, horseshoe kidneys, and agenesis of prostate could be associated malformations.[4]

Delay of diagnostic and therapeutic measures resulting in male gender assignment has led to severe psychological and anatomic problems because of difficulties in constructing functioning phallus.[3] In contrast, it is easier to establish normal female appearance. However, no reports concerning long-term physiological and psychological result sexist in immediately gender reassigned patients.

There is a consensus that when the patients with PA are diagnosed in infancy, they should undergo early gender assignment and be raised as girls, despite male karyotype.[9] However, its relevance to the Indian society is still debated. In Indian villages and towns, it would be easier to live as a sexually incompetent male in the society rather than a single unmarried girl.[10]

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kessler WO, McLaughlin AP 3rd. Agenesis of penis. Embryology and management. Urology 1973;1:226-9.  Back to cited text no. 1
    
2.
Gautier T, Salient J, Pena S, Imperto-McGinley J, Peterson RE. Testicular function in 2 cases of penile agenesis. J Urol 1981;126:556-7.  Back to cited text no. 2
    
3.
Ciftci AO, Senocak ME, Büyükpamukçu N. Male gender assignment in penile agenesis: A case report and review of the literature. J Pediatr Surg 1995;30:1358-60.  Back to cited text no. 3
    
4.
Soderdahl DW, Brosman SA, Goodwin WE. Penile agenesis. J Urol 1972;108:496-9.  Back to cited text no. 4
    
5.
Skoog SJ, Belman AB. Aphallia: Its classification and management. J Urol 1989;141:589-92.  Back to cited text no. 5
    
6.
Richart R, Benirschke K. Penile agenesis. Report of case, review of the world literature and discussion of pertinent embryology. Arch Pathol 1960;7:134-42.  Back to cited text no. 6
    
7.
Attie J. Congenital absence of the penis; a report of a case with congenital concealed penile agenesis and congenital absence of the left kidney and ureter. J Urol 1961;86:343-5.  Back to cited text no. 7
    
8.
Oesch IL, Pinter A, Ransley PG. Penile agenesis: A report of six cases. J Pediatr Surg 1987;22:172-4.  Back to cited text no. 8
    
9.
Sarin YK, Sinha A. Aphallia. Indian Pediatr 2003;40:367-8.  Back to cited text no. 9
    
10.
Tiwari S. Should infants with aphelia be raised as females? Indian Pediatr 2003;40:802-3.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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