Conspicuous or abnormal external genitals - Pediatric Surgery

Conspicuous or abnormal external genitals - Pediatric Surgery Conspicuous or Abnormal External Genitals . Hypospadias.click the images for more details   Incidence. A deficiency of the urethra at the ventral surface of the penis is a frequent abnormality in boys with 1 in 300 newborns. In some of the patients a familial occurrence may be observed.  Clinical significance. 1. Hypospadias impair the esthetic appearance (conspicuous external genitals). 2. Voiding disorders are possible in hypospadias. 3. Sexual intercourse and fertility may be hindered. 4. Confusion with a girl or an intersex may be possible in some types of hypospadias.  Etiology.  Unknown.  Pathology, anatomical types. Illustrations The classification differentiates between the more frequent anterior and less frequent middle and posterior types of hypospadias: The orifice of the urethra is situated below the glans and the sulcus (= anterior hypospadias), on the shaft of the penis (= middle hypospadias), or at the base of the scrotum (penoscrotal), between the halves of the scrotum (scrotal) or behind the scrotum (perineal) ( = posterior hypospadias). Mainly in posterior hypospadias there are a curvature of the penis and other anomalies such as bifid scrotum, penoscrotal transposition, and a male utricle. In slight types (anterior) hypospadias the glans often tilts in the ventral direction and the orifice may be stenotic. Except for undescended testis and inguinal hernia which are frequent anomalies, other associated urological malformations are rare.show details Pathophysiology .   The functional disturbances are due to the site of the urethral orifice and a possible stenosis: In posterior hypospadias the discharge of urine happens perpendicularly to the shaft, and voiding occurs in standing position between the legs, or, in anterior hypospadias with meatal stenosis, with increased effort and a thin, forced stream. Or, due to additional malformations as later hindrance of sexual intercourse by a penile curvature and a possible micropenis, as voiding disorder by an large utricle, or as infertility by severe types of undescended testicles.   Clinical presentation (history, findings, clinical skills).Illustrations Findings: The leading sign is a defect of the foreskin below the glans and its hood-like deformity, and prominence dorsally with a widening of the tip of the glans. The localization of the orifice and the presence or absence of additional anomalies allow a classification of all hypospadias in one of three main groups. Clinical skills: By pulling the foreskin, the ventral surface of the penis can be seen and closely examined from below and from the side. During spontaneous micturation, the course of the urine stream in relation to the shaft and its calibre can be observed, and during a spontaneous erection a possible curvature of the penis. In case of a possible meatal stenosis, the width of the orifice should be probed. In case of a scrotal or perineal hypospadias with barely visible penis and a bifid and empty scrotum the differentiation from female or intersexual external genitals may be difficult (vulvar hypospadias), and the first move is to carefully look for testicles because undescended testis is a frequent associated finding in severe types of hypospadias, and the presence of testicles excludes female or intersexual external genitals. show details Natural history.  There is no spontaneous resolution of the findings with growth; however, there is no urinary incontinence, and usually, normal sexual development occurs. On the other hand, the conditions for fertility may change for the worse without treatment.   Differential diagnosis.Illustrations The differential diagnosis includes all lesions with conspicuous external genitals, with voiding disorders, and with anomalies leading to infertility. The lesions with conspicuous external genitals belong to the abnormal external genitals in boys and in girls, and to the external genitals in intersex. show details Work-up examinations.Illustration The additional examinations include photographs and a precise description of the local findings, and measurements of the size of the testicles and the length of the penis. In posterior types of hypospadias a VCUG is necessary to exclude a male utricle, and, in ambiguous forms, blood examinations (chromosomes) and x-ray (ultrasound) for the determination of the sex and internal genitals. In case of one or several combined non-urogenital malformations, x-ray exams for exclusion of other urological anomalies are recommended. show details Therapy. Illustrations Anterior hypospadias: Indication for surgery is mainly for esthetic reasons except for meatal stenosis. Middle and posterior hypospadias: Indication for surgery additionally for functional reasons. Age at time of surgery: either in the second half of infancy, or in toddlers. Anterior types: Reconstruction of the absent urethra with advancement of the orifice to the tip of the glans using local tissue and excision, or reconstruction of the foreskin (MAGPI=meatal advancement and glanuloplasty procedure, Mathieus's procedure, etc.). Middle and posterior types: Release of the penile curvature and reconstruction of the absent urethra with some type of preputial island flap. In addition, a possible bifid scrotum, a torsion of the penis and/or an utricle need correction.show details Prognosis.  The rate of expected urethrocutaneous fistulas is 10 to 15 %, needing follow-up surgery. The prognosis concerning esthetics, normal micturation and intercourse is favourable; disturbances of fertility in severe types of hypospadias and cryptorchidism are possible.