(1)
Pediatric Surgery, Al Azher University, Cairo, Egypt
Abstract
In primary penile epispadias, the urethra opens on the dorsal aspect of the penis, or the glans. The extent of the defect can vary from a mild glanular defect to complete defect as the one observed in bladder exstrophy, with extension to the bladder neck. Simple epispadias occurs less commonly than the more severe form associated with exstrophy of the bladder. Both corpora cavernosa don’t come close to each other, as in normal condition, giving the look of wide broad and short penis, with a variable degrees of dorsal curvature, usually the anus is appropriately sited and the scrotum also appears normal.
Keywords
Isolated epispadiasBladder exstrophy epispadias complexEpispadias with an intact prepuceEpispadias anomaly which will be discussed herein is the one with normal bladder wall “Isolated epispadias” as the bladder exstrophy epispadias complex is not a penile anomaly and should be discussed with bladder anomalies elsewhere [1].
Nomenclature
Isolated Epispadias, Primary epispadias and Simple epispadias.
Definition
In primary penile epispadias, the urethra opens on the dorsal aspect of the penis, or the glans. The extent of the defect can vary from a mild glanular defect to complete defect as the one observed in bladder exstrophy, with extension to the bladder neck. Simple epispadias occurs less commonly than the more severe form associated with exstrophy of the bladder. Both corpora cavernosa don’t come close to each other, as in normal condition, giving the look of wide broad and short penis, with a variable degrees of dorsal curvature, usually the anus is appropriately sited and the scrotum also appears normal. A variable diastasis of pubic bones exist, that tends to be less severe than in bladder exstrophy. The pelvic ring is often complete, with an apparently normal abdominal wall, but sometimes a defect over the symphysis pubis is aberrant.
25.1 Incidence
Isolated epispadias without exstrophy is a rare anomaly, the incidence being 1 in 120,000 of males; it may be detected in female with different varieties. The male-to-female ratio is 2.3:12. Epispadias is classically associated with bladder exstrophy in over 90 % of cases of primary epispadias [2].
25.2 Historical Background
The first recorded case of epispadias is attributed to the Byzantine emperor Heraclius for his unknown disease. Isolated epispadias remained unknown and untreated until it was described by Morgagni in 1761. The initial attempts to treat this anomaly were restricted to controlling the incontinence. In 1869, Karl Thiersch described the etiology and anatomy of epispadias and reported a case of epispadias reconstruction with a long-term follow-up of 11 years [3].
25.3 Classification
Depending on the severity of the clefted urethra, epispadias is classified to:
- 1.
Glanular epispadias: the urethra opens on the dorsal aspect of the glans, which is broad and flattened (Fig. 25.1).
Fig. 25.1
Glanular type of epispadias
- 2.
Penile type: the urethral meatus, which is often broad and gaping, is located between the pubic symphysis and the coronal sulcus. A distal groove usually extends from the meatus through the splayed glans (Fig. 25.2).
Fig. 25.2
Penile epispadias
- 3.
Penopubic type: which has the urethral opening at the penopubic junction, and the entire penis has a distal dorsal groove extending through the glans (Fig. 25.3).
Fig. 25.3
Penopubic epispadias, opening of bladder neck can be seen at the vicinity of the proximal end of the defect
The penopubic type is the most frequent, with an underlying deficiencies of the bladder neck and proximal urethra and striated sphincter complex, which determine the degree of associated incontinence. In these cases, the posterior urethra merges with the bladder neck, and the verumontanum may lie at the level of the bladder neck or even within the bladder itself. The ureteral orifices often lie close together and are normal or narrowed in caliber, contrasting with the wide refluxing orifices seen in exstrophy. Patients with glanular epispadias seldom have urinary incontinence, however, with penopubic and penile epispadias, incontinence is present in 95 % and 75 % of cases respectively [4].
25.4 Variant of Primary Epispadias
In some rare cases, epispadias could be just presented as a small dimple on the tip of the penis above the normal urethral opening (Fig. 25.4).
Fig. 25.4
Cleft of the dorsum of the glans, with a normally situated urethra and meatus, a variant of glanular epispadias
Typically, epispadias is associated with defective prepuce on the dorsal aspect of the penis and redundant prepuce on the ventral aspect, but rare cases may have a completely intact prepuce.
Epispadias with an intact prepuce as in case in Figs. 25.5, 25.6, and 25.7 is extremely rare, its incidence is not known, however there are few case reports, either as a single case or case series [5]. In such cases the epispadic urethra may be in the form of glanular epispadias (the common variant), really intact prepuce may hide a complete penile or even a penopubic type, in our series of 7 cases we also encountered a case of epispadic megameatus with an intact prepuce (Fig. 25.7), this entity was discussed with the megameatus anomaly (Chap. 20).
