COMPLETE PENOSCROTAL TRANSPOSITION - A RARE CONGENITAL ANOMALY
Shashidhar A, Bijal Rughani, S R Keshavamurthy
Department of Pediatrics, Kempegowda Institute of Medical sciences, Bangalore, India
Address for Correspondence
Dr Shashidhar, #114 KIMS Mens Hostel, BSK, 2nd Stage, Bangalore 70, India.
Email
shashiishere@gmail.com
The infant weighing 3 kg, was born at term, by vaginal route, to a 26yr old non-consanguineously married gestational diabetic who required insulin for glycemic control. She had a previous normal childbirth and had no significant antenatal problems. The antenatal ultrasound had missed the anomaly and the child was noted to have complete inversion of external genitalia at birth. The scrotum was bifid. Both the testes were palpable in the well formed scrotal sac. The rudimentary penis was placed just anterior to the anal opening which itself was displaced anteriorly. (Figure 1) There was no hypospadias or chordee. A diagnosis of complete form of penoscrotal transposition with ectopic anus was made and the child was evaluated for other associated anomalies. The child also had a ventricular septal defect and atrial septal defect with a normal male XY karyotype while other investigations like cranial and abdominal ultrasonogram, thyroid profile, serum testosterone levels were normal. The baby underwent staged surgery at our hospital for the correction of the anomaly and is on follow-up.

Figure 1: Complete penoscrotal transposition


Complete penoscrotal transposition (CPST) is a rare and unusual malformation in which the scrotum is located cephalad to the penis, frequently associated with major and often life-threatening malformations involving the genitourinary, cardiovascular, or skeletal systems. (1) Parida et al (2) have noted a 90% incidence of major renal anomalies including complete agenesis of the urinary system, unilateral or bilateral renal agenesis, horse-shoe kidney, ectopic pelvic kidney, polycystic or dysplastic kidneys, obstructive uropathy with reflux, megaureter, and hydronephrosis. The most common non-urogenital abnormalities associated with PST were: mental retardation (60%), anorectal malformations (33%), central nervous system anomalies (29%), vertebral defects (29%), preaxial limb defects including radial dysgenesis (24%), and congenital heart disease (19%). It therefore appears likely that CPST may represent only one of the results of a major localized or generalized embryological insult to the fetus during the 4th-6th weeks of gestation, when the major organ systems are passing through a crucial phase of development and differentiation. Abnormal positioning of the genital tubercle in relation to the scrotal swellings during the critical period at 4-5 weeks' gestation may affect the inferomedial migration and fusion of the scrotal swellings. If the phallic tubercle is also intrinsically abnormal, development of the corporal bodies and the urethral groove and folds may be affected; this explains the frequent occurrence of the other genital abnormalities. In presence of normal penis this anomaly does not cause any sexual dysfunction. However, its surgical repair is for psychological reasons. (1)
Funding
None
Conflict of Interest
None
References :
  1. Chadha R, Mann V, Sharma A, Bagga D. Complete penoscrotal transposition and associated malformations. Pediatr Surg Int 1999; 15: 505-507
  2. Parida SK, Hall BD, Barton L, Fujimoto A. Penoscrotal transposition and associated anomalies: report of five new cases and review of the literature. Am J Med Genet. 1995; 59: 68-75
Last Updated : Tuesday, February 01, 2011 Vol 8 Issue 2 Art #13
How to Cite URL :
A S, Rughani B, Keshavamurthy R S. COMPLETE PENOSCROTAL TRANSPOSITION - A RARE CONGENITAL ANOMALY. Pediatric Oncall [serial online] 2011[cited 2011 February 1];8. Art #13. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=381&type=J&tid=&imgid=&reportid=227&tbltype=
Disclaimer: The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.