Patient Presentation
A 2-week-old male came to clinic for his well-child care. He had been born at another hospital and this was his first visit. His parents were concerned that his “penis looked funny,” after his circumcision. They reported multiple wet diapers without any blood or odor, and the infant did not seem to be bothered when urinating or at other times. The past medical history revealed a full-term infant without prenatal or natal problems. The family history had no genitourinary or genetic problems.
The pertinent physical exam showed a vigorous infant with a weight of 3645 grams up past his birth weight of 3490 grams (50%), head circumference of 35 cm (50%) and length 51 cm (75%). His examination was normal except for a circumcised phallus that was well-healed. The meatal opening was elongated ventrally from the tip of the glans to almost the corona. The rest of the phallus did not show any abnormalities including no chordee and both testicles were located within the scrotum. The anus was patent and in a normal position.
The diagnosis of a distal hypospadias was made. The diagnosis was discussed with the parents and a referral to urology was made. “At this time, there is nothing special for you to do, but if there is a need for surgical correction, then the pediatric urologists are the ones who will help you. He is able to urinate normally now and doesn’t seem to be bothered. But I understand the concerns about this and sexual function in the future, so we’ll have you talk with the urologist,” said the pediatrician.
Discussion
Hypospadias is one of the most common congenital malformations and is the most common penile malformation. It is defined as the urethral opening occurring on the ventral side of the penis. Other ventral tissues can be affected thus other problems such as penile curvature and/or scrotal problems can also occur. Embryologically the penis is formed in the following way:
“At the beginning of the ambisexual stage, the urethral groove [is] bounded by the urethral folds [and] extends about halfway distally along the ventral aspect of the elongating genital tubercle. At the distal aspect of the urethral groove is the solid urethral plate…that extends to the glans of the developing penis. The urethral groove [then] extends distally to the glans by canalization of the urethral plate…. The urethral folds grow to the midline where they fuse to extend the penile urethra distally to the glans….” Examples of this process can be found here in Yamada et.al.’s article in Figures 4 and 5.
Hypospadias prevalence world-wide and over time is “difficult to estimate,” as published studies show immense heterogenity. Some world-wide geographical areas show increased prevalence but others do not and methodologies are not consistent. A systematic review found the 5 largest studies estimating a prevalence of 1.4-50 per 10,000 live births from various time periods including 1964-2010. Hypospadias is considered a high heterogeneous problem influenced by genetics and environmental factors including ethnic and geographic factors.
Hypospadias treatment has been referenced back to Ancient Greece. Distal hypospadias is much more common than proximal and complications generally are fewer with distal hypospadias. Treatment success with distal hypospadias ranges from 85-95%, while proximal is 75-95% but with many caveats that proximal rates are reported by some groups as much higher. Most complications occur in the first year after repair, but puberty also can also see additional problems including cordee. There are multiple types of procedures and some need to be staged over time as well.
Repair complications can include diverticulum, dehiscence, fistula, strictures, chordee, and residual hypospadias. One study reports that “[p]reserving penile length and correcting curvature was perceived as more important than a distal positioning of the meatus,” by patients.
Learning Point
The desired goals are the same for any hypospadias repair but per Long and Canning, “[o]bjectives for the reconstruction of proximal hypospadias have now been established: to enable voiding with normal velocity and laminar flow, to obtain satisfactory sexual function with a straight penis, and from a cosmetic standpoint, achievement of a slit-like meatus with a well approximated glans. Although nobody would consider sitting to void due to a sprayed urinary stream, or painful or awkward sexual function due to penile curvature to be life threatening, everyone would agree that quality of life is compromsied for many of these boys and men that suffer from said complications.”
Urethral strictures are one of the most common problems. Laminar fluid flow, or a straight, unimpeded fluid flow, is affected by fluid volume, resistance and oriface opening shape. Examples of the fluid flow changes caused by different sized lumens and different shaped openings can be easily understood by looking at piping of icing (a fluid) on confectionaries, and can be found here and here.
Questions for Further Discussion
1. How are circumcisions performed?
2. How is vesicoureteral reflux identified and treated?
3. What are other congenital abnormalities of the male genitourinary system?
Related Cases
- Disease: Hypospadias | Birth Defects | Penis Disorders
- Symptom/Presentation: Minor Congenital Anomaly | Health Maintenance and Disease Prevention
- Specialty: Nephrology / Urology
- Age: Newborn
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Birth Defect and Penis Disorders.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Yamada G, Satoh Y, Baskin L, Cunha G. Cellular and molecular mechanisms of development of the external genitalia. Differ Res Biol Divers. 2003;71:445-460. doi:10.1046/j.1432-0436.2003.7108001.x
Wheeler APS, Morad S, Buchholz N, Knight MM. The Shape of the Urine Stream – From Biophysics to Diagnostics. PLOS ONE. 2012;7(10):e47133. doi:10.1371/journal.pone.0047133
Long CJ, Canning DA. Hypospadias: Are we as good as we think when we correct proximal hypospadias? J Pediatr Urol. 2016;12(4):196.e1-196.e5. doi:10.1016/j.jpurol.2016.05.002
Springer A, van den Heijkant M, Baumann S. Worldwide prevalence of hypospadias. J Pediatr Urol. 2016;12(3):152.e1-152.e7. doi:10.1016/j.jpurol.2015.12.002
Gulseth E, Urdal A, Andersen MA, et al. High satisfaction on genital self-perception and sexual function in healthy Norwegian male adolescents. J Pediatr Urol. 2021;17(4). doi:10.1016/j.jpurol.2021.02.015
Sinatti C, Schechter MY, Spinoit AF, Hoebeke P. Long-term outcome of urethral and genital reconstruction in hypospadias and exstrophy-epispadias complex. Curr Opin Urol. 2021;31(5):480-485. doi:10.1097/MOU.0000000000000920
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa
Pediatric Education 