Pediatric Surgery, Al Azher University, Cairo, Egypt
I’m suggesting herein that the pathological anomaly lies mainly in the configuration of the urinary meatus, which instead to be a slit like, located in the undersurface of the glans penis, slightly at the tip, it will be in this rare anomaly so widely opened “mega meatus” with a different abnormal positions, either orthotopic, hypospadiac or epispadic and to be either associated with a normally intact or a deficient prepuce, so we will end with a 4 different subtypes of megameatus; the intact prepuce orthotopic megameatus subtype was not previously described.
KeywordsIntact prepuce megameatusIntact prepuce with epispadiasIntact prepuce with orthotopic megameatus
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We opted to title this chapter as “Megameatus”, and not an Intact prepuce megameatus (IPM), like other text books and different literature, as I’m suggesting herein that the pathological anomaly lies mainly in the configuration of the urinary meatus, which instead to be a slit like, located in the undersurface of the glans penis, slightly at the tip (Figs. 20.1 and 20.2), it will be in this rare anomaly so widely opened “mega meatus” with a different abnormal positions, either orthotopic, hypospadiac or epispadic and to be either associated with a normally intact or a deficient prepuce, so we will end with a four different subtypes of megameatus; the intact prepuce orthotopic megameatus subtype was not previously described.
Normally situated urinary meatus in a flaccid penis
Normally slightly widened meatus during erection
Megameatus is a congenitally abnormally wide urinary meatus closely associated with an abnormal location; hypospadiac, orthotopic or episodic, with an abnormal or normal prepuce.
20.1 Spectrum of the Megameatus Anomaly
Intact prepuce Megameatus (IPM), with three subtypes:
Intact prepuce with hypospadiac megameatus (Fig. 20.3a, b).
(a) Intact prepuce, (b) when prepuce retracted, it revealed a hypospadiac megameatus
Intact prepuce with epispadias (Fig. 20.4a, b).
(a) Intact prepuce, (b) an epispadiac megameatus seen when prepuce retracted
Intact prepuce with orthotopic megameatus (Fig. 20.5a, b).
(a) Intact prepuce (b) with an orthotopic megameatus
Megameatus with hypospadias, usually associated with a deficient hooded prepuce (Fig. 20.6 ).
(a) Megameatus with hypospadias and a hooded prepuce. (b) Wide megameatus with hypospadias. (c) Abnormally wide megameatus with deficient glanular tissue
Each type will be described separately with illustrations
20.2 Intact Prepuce with Hypospadiac Megameatus
Commonly known as an Intact prepuce Megameatus (IPM), it is not a rare condition, with a different varieties, several presentations and of a paramount importance as regard the recognition and management.
Intact Prepuce Megameatus, Pseudo Iatrogenic Hypospadias, Hypospadias Variant.
The overall incidence of the MIP is approximately 1 in 10,000 live births and represent a 3–6 % of cases of anterior hypospadias, but this condition may be more often with underestimation and reporting .
MIP is not known till recently, when it was described for the first time at 1989, by Duckett and Keatting .
Obviously, MIP by definition is an association of a widely opened splayed glans, deeply grooved, patulous, large urinary meatus in the ventral aspect, at or even distal to the coronal sulcus, with a completely formed prepuce in contrast to the ventrally deficient foreskin in other cases of hypospadias. Furthermore, the meatus is abundantly large in the MIP variant, whereas many boys with distal hypospadias have a rather small- appearing meatus. Ventral curvature is much less likely to occur in a patient with MIP than in those with other varieties of distal hypospadias (Fig. 20.3a, b).
Because there is no external clue to the presence of this variant, the megameatus intact prepuce sometimes comes to light for the first time in a boy who is about to undergo circumcision. Recognition of this anomaly is important, not only for the sake of the child, but also from the medico-legal point of view. In some countries where circumcision performed early for infants in the first weeks of life, the families may attribute MIP as a complication of circumcision. The clues in such cases are the absence of any evidence of glanular scarring, no history of bleeding at the time of circumcision and the smooth edges of the widely splitted meatus with a healthy mucosal covering (Fig. 20.7).
A circumcised child had a typical megameatus, with a wide deep urethral plate and wide meatus extends along the whole glans without any scaring
Sometimes MIP may be presented with paraphimosis, if the intact prepuce is forcible retracted during routine circumcision in a young infant (Fig. 20.8).