Circumcision is the most common surgical procedure in the United States. It is usually performed because of religious preference or for social reasons (i.e., older brothers and father are circumcised). In 1999, the American Academy of Pediatrics (AAP) observed that routine newborn circumcision may not be necessary (1). Families should be instructed in care of normal foreskin. The American Academy of Pediatrics brochure is a good instructional guide (2). Forcible retraction of the prepuce is painful, harmful, and unnecessary. Tearing the prepuce places the child at risk for cicatrix formation and phimosis, and is therefore inadvisable. Medical benefits of circumcision include prevention of penile carcinoma, phimosis, balanoposthitis, and neonatal urinary tract infection (UTI) (1,3,4). An uncircumcised male neonate is 20 times more likely to have a UTI than a circumcised infant, because of preputial colonization of the prepuce by urinary pathogens (2,3). The higher incidence of UTI occurs only during the first year of life, and beyond that time, circumcision has not been shown to decrease infections. Circumcision should be discussed with the family of any male infant with a UTI or vesicoureteral reflux, although suppressive antibiotics for the first year of life are an option. Disadvantages of neonatal circumcision include meatitis and meatal stenosis because the protective covering of the prepuce is lost. In addition, a boy may develop chordee or concealed penis, and the procedure is not without discomfort.

Circumcision is usually performed in one of two ways. Clamp or bell circumcision is reserved for infants younger than a few months of age. A freehand technique by dorsal slit or sleeve method is preferred for older children. It is important to recognize the features of a normal and abnormal penis. Children with chordee, hypospadias, and buried or webbed penises should not undergo routine neonatal circumcision. The clamp and bell devices commonly used in neonatal circumcision were designed for use on a normal penis and when applied inappropriately can cause injury to the urethra or yield a poor cosmetic result. The prepuce may be an important source of tissue for later reconstruction. No patient who may need future hypospadias repair should undergo routine circumcision. Be wary of the child described as having a “natural circumcision.” Usually these are boys with a prominent dorsal hooded foreskin and a subtle distal hypospadias, and a hypoplastic corpus spongiosum. Whenever a circumcision is performed, the surgeon must carefully examine the underlying glans and meatus for occult hypospadias with normal foreskin.

Stenosis of the urethral meatus usually occurs years after neonatal circumcision. The delicate meatus is susceptible to inflammation from local trauma such as from rubbing against a diaper or underwear. Meatitis progresses to cicatrix formation, with narrowing of the orifice. Deflected urinary stream and prolonged voiding times are typically noted by parents after a child is toilet trained. The classic history is that the urine stream deflects upward or sprays. A fine, forceful stream may emanate from a pinpoint meatus. Irritative voiding symptoms, such as dysuria and urgency, rarely occur without a concomitant UTI. Isolated meatal stenosis without infection does not require radiographic assessment of the urinary tract or cystoscopy. When stenosis is suspected, the meatus can be calibrated with small catheters or sounds. If stenosis is not severe, parents can be taught to perform daily obturation with a feeding tube or the tip of an ophthalmic ointment tube. Significant stenosis should be surgically corrected by a ventral incision, followed by a 3-month program of daily calibration of the urethra with a catheter, to ensure adequate opening.

Priapism is the involuntary and prolonged erection of the corpora cavernosa. Recurrent and prolonged bouts of priapism can lead to corporal fibrosis and impotence. The causes of priapism can be categorized into two broad groups: low flow or high flow. The majority of patients have low-flow priapism. Decreased venous outflow leads
to increased intracavernous pressure and subsequent decreased arterial inflow. The venoocclusion leads to stasis of blood, hypoxia, and acidosis. The glans penis and spongiosum are flaccid. The most common low-flow situation in pediatric patients occurs with sickle cell disease or trait; about 5% of these patients experience priapism. The erection occurs as part of a diffuse sickle crisis or as an isolated event. Sickle crisis is managed by transfusion to reduce the hemoglobin S level, as well as oxygenation, hydration, alkalinization, and pain control. Aspiration with irrigation of the corporal bodies with an α-adrenergic agent can also be tried, but is usually unsuccessful in sickle cell patients unless systemic management of the crisis has been also started. Phenylephrine, 100 to 200 μg, can be used; patients need careful monitoring, particularly those with a history of cardiovascular disease. When conservative management fails, surgical construction of a shunt connecting the corpora cavernosa to the spongiosum may be effective temporarily; ultimately, however, these close. Recurrent bouts of priapism can result in impotence due to the repetitive pressure damage to the corporal bodies.

Leukemia is another cause of childhood priapism. Although chronic granulocytic leukemia accounts for only a small percentage of pediatric leukemias, one-half of all leukemic priapism occurs in these patients. Leukemic cells appear to cause microvascular occlusion within the corporal bodies, but other possible factors include leukemic infiltration of the sacral nerves or central nervous system as well as abdominal and pelvic venous obstruction. Treatment is directed at lowering the circulating leukocyte count. Shunts may be necessary for refractory cases. Other etiologies for priapism in the child include blunt perineal trauma, spinal cord injury, medications (tricyclic antidepressants, phenothiazines, hydralazine, prazosin, guanethidine, heparin, and cocaine), and retroperitoneal fibrosarcomas. As with sickle cell disease and leukemia, treatment is aimed at the underlying disease process. Fat emulsions of 20% infused as part of total parenteral nutrition (TPN) have been associated with priapism. It is believed that the fat emulsion increases blood coagulability, distorts erythrocytes, and, promotes adhesion and clumping. Antidepressants, such as trazadone, and antipsychotics, such as chlorpromazine have also been associated with priapism.

High flow priapism is caused by unrestrained arterial inflow. Because the problem is not one of venoocclusion, there is no hypoxia and acidosis. Almost all cases of high-flow priapism are due to penile and perineal trauma. An injury to the cavernosal artery results in a cavernosus-to-corporal body fistula. The key diagnostic finding in high-flow priapism is the discovery of well-oxygenated red blood upon corporal aspiration. In contrast to the flaccidity found in low-flow priapism, the glans and spongiosum in high-flow priapism is engorged with blood and is quite firm.