Case Report: Penile Agenesis

By Dr. Laishram Ranbir & others *

Abstract

A case of penile agenesis in a full term male baby without any other associated anomaly is reported because of its rarity.

Introduction

Penile agenesis is a very rare congenital anomaly resulting from failure of development of genital tubercle, the anomaly is generally associated withother genitourinary or systemic defects, some of which are incompatiable with life. We report one such entity with a review of literature. Besides reporting a rarity, the purpose of this report is to disucss briefly the clinical signs, embryologic basis and the management of the entity.

Case Report

A full term male baby weighing 3.5 kg born to a 30 year old second gravida after an uneventful pregnancy and labour showed complete absence of penile shaft (Fig.l). The external urethral opening was noticed on the anal verge at 6 O' clock position hidden by a small skin tag. The scrotal sac was well developed with normally descended testes.

No other associated congenital

anomaly could be detected. The baby passed meconium and urine through the normally located anus. There was no history of consanguinity, exposure to teratogenic agaents or radiation in the antenatal period.

The haematological and biochemical parameters were normal. Ultrasonography (Fig.2) and intravenous urography

demonstrated normal kidneys and urinary bladder but with the urethra opening into the lowermost part of the anal canal.

Discussion

Penile agenesis is a very rare developmental anomaly with a reported incidence of 1 in 10 to 30 million birth(l). Only about 70 cases have been reported so far (2). It results from failure of development of the genital tubercle.

The urethra generally opens on the perineum near or into the anal canal. The scrotum is usually well developed with normal descent of testes.

The associated genito-urinary and systemic anomalies reported include renal agenesis, horse -shoe kidney, vesico-urethral reflux, recto-vesical fistula, cryptorchidism, musculoskeletal and cardio-pulmonary abnormalities.

Total penile reconstruction being unsatisfactory, such babies are brought up as females with early orchidectomy and gender assignment. The scrotum is preserved for a feminizing genito-plasty at a later date during which a neo-vagina is fashioned and the external urethral opening transposed anteriorly away from the anal verge.

References

1. 1. Kessler WO, McLaughlin AP Agenesis of penis - embryology and management Urology 1973; 1: 33-39
2. Jack S, Elder MD. Congenital Anomalies of the Genitalia. In Patrick C Walsh, Alan B Retik, E Daracott Vaughan, Jr, Alan J Wein, eds. Compbell's Urology; Voll, 7th Ed. Philadelphia; W B Saunders, 1990: 2123-2177.
3. Bansal RK, Singh J. Congenital absence of penis.Indian Pediatrics 1990; 27(8): 882-883.
4. Sodenhahl DW, Brosman SA, Goodwin WE. Penile agenesis. J Urol 1972; 108;496-499.
5. Klauber GT, Sant Gr. Disorders of the male external genitalia. In: Kelalis PP, King LR, Belman, eds. Clinical Pediatric Urology: Vol 2, 2ndEd. Philadelphia, Saunders Company, 1985: 833- 834

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* Dr. L. Ranbir Singh (MD, FIAP, WHO Fellow) is a Professor and Head, Department of Pediatrics, at the Regional Institute of Medical Sciences (RIMS), Imphal Manipur. He contributes to e-pao.net regularly.
Lalmalsawni Hmar, MBBS, PG Student, Paediatrics, L Braja Mohan Singh, MD, Professor, Paediatrics, S Sekharjit, MCh, Associate Professor, Urology, RIMS also are part of this paper presentation at "Journal Of National Neonatology Forum" - Recent Concept in Neonatal Intensive Care Volume 16. No.1 January - March 2001 .
This paper was webcasted at e-pao.net on 08th February 2010.

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