Lenz Majewski hyperostotic dwarfism


A rare genetic disorder characterized by dense, thick bones and symphalangism


* Failure to thrive * Short stature * Severe emaciation * Mental deficiency * Large cranium


* Hypospadias –Most common penile anomaly –Incidence of 1/500 –Urethral meatus is typically located on the ventral surface of the glans penis –The meatus may also be located on the ventral surface of the penile shaft, the scrotum, or the perineum –Frequently associated with a ventral curvature of the penis (chordee) and/or a hooded prepuce –Less commonly associated with undescended testes or inguinal hernia o Epispadias –Less common than hypospadias –Urethral meatus on dorsal surface of the penis o Chordee –Ventral curvature of the penis –Most often associated with hypospadias –May occur without hypospadias when the ventral tissue is hypoplastic or fibrotic * Dorsal hood –Incomplete formation of the ventral foreskin –May occur with hypospadias * Micropenis (microphallus) –Defined as stretched penis length shorter than 2 standard deviations below the mean for gestational age –Associated with Prader-Willi, Kallmann Laurence-Moon-Biedl syndrome, and growth hormone deficiency


* Routine circumcision should be avoided as the foreskin may ultimately be used in the repair * Surgical revision is usually performed for cosmetic and functional reasons o Hypospadias and epispadias –Canalization of the penis improves urinary flow, erectile function, and fertility –Optimally, a skin flap is created using the foreskin * Chordee –Ventral release and urethroplasty at age 6–12 months o Dorsal hood –May not require treatment, or a modified routine circumcision may restore expected appearance * Micropenis –Androgen stimulation before puberty has been used but is controversial because it may impair penile growth during puberty