The differential diagnosis for males who present to the ED with a swollen, erythematous penis includes phimosis, paraphimosis, balanitis, posthitis, hair tourniquet syndrome, and insect bite.1
Phimosis occurs when the distal prepuce is unable to be retracted over the glans penis. Normally, the prepuce cannot be retracted over the glans in infants and it should not be forced. With normal growth and stretching of the prepuce, it will become retractable in 90% of children by the age of 6 years. Local irritation or infection (balanoposthitis) can cause an abnormal constriction of the prepuce, preventing its ability to retract normally.
Phimosis may be noted on routine physical examination or may be reported by parents. Pain and swelling can occur with associated infections of the glans. Urinary stream in some cases may be diverted to one side or children may have hematuria.
Physical examination generally establishes the diagnosis. The EP will find a constricted distal prepuce that is unable to be retracted over the glans penis. Patients will sometimes have concomitant balanitis or balanoposthitis.
The diagnosis is established clinically; however, examination of the urine for UTI may be warranted in selected cases. If patients demonstrate signs of urinary tract obstruction, such as inability to urinate, abdominal fullness, or urinary frequency, then renal function studies should be obtained. Renal US may define the degree of obstruction.
As most cases are not a result of a pathologic condition but a result of normal growth and development, reassurance and an explanation of the natural course of this condition to parents is often all that is needed. Betamethasone valerate, 0.6% cream applied twice daily for 2 weeks, has been used to treat phimosis and may be prescribed in cases in which the phimosis is not anatomic but pathologic. Patients with recurrent balanitis, balanoposthitis, UTI, or obstruction should be referred to a urologist for circumcision.
Paraphimosis is a condition in which the foreskin or prepuce of the uncircumcised male is retracted over the glans and becomes trapped and unable to move back into its normal position (Fig. 82–6). Once the prepuce is retracted, its constriction proximal to the coronal sulcus causes venous congestion of the glans that further prevents reduction. Ischemic injury to the glans may ensue.
Paraphimosis: moderate edema of retracted foreskin, which is entrapped behind the coronal sulcus.
Patients have pain and edema of the distal penis and prepuce. If the child has not been circumcised, then a thorough examination to look for possible hair tourniquets and penile foreign bodies must follow.
The management of this condition focuses on retracting the prepuce over the head of the glans. Without anesthesia, most children poorly tolerate ice packs to the groin. The physician may place a penile block by injecting lidocaine 1% without epinephrine around the base of the penis. This will effectively reduce the pain. Ice packs can then be placed for 10 minutes after which manual reduction should be attempted (Fig. 82–7). The physician's index fingers are placed on the leading edge of the edematous foreskin, and the thumbs are placed on the glans. Thumb pressure is directed inward toward the body as the prepuce is pushed back over the glans. Successful reduction following compression of the glans and distal penis by wrapping with a small ace bandage starting distally and working toward the base of the penis has also been described.13 The Perth-Dundee method uses a 26-G needle to make multiple (up to 20) holes in the edematous foreskin, which is then manually compressed to express fluid, permitting reduction. As a last resort, it may be necessary to incise the constricting ring of tissue. General anesthesia may be required in young children. In most cases, subsequent circumcision is not necessary.13
Reduction of paraphimosis. (A) Manual compression of the glans and foreskin to reduce the edema of the foreskin. (B) Manual reduction of a paraphimosis. The thumbs push the glans proximally while the fingers provide countertraction to slip the phimotic ring over the glans.
Once reduction is complete, the prepuce should lie over the end of the glans and the urethral opening should not be visible. If retraction of the prepuce is successful and the child is able to urinate spontaneously, then the child can be discharged with urologic follow-up. If the prepuce cannot be retracted, then emergent urologic consultation is needed for surgical division of the phimotic ring, and eventually, circumcision.
Balanitis and Balanoposthitis
Balanitis (infection of the glans) and balanoposthitis (infection of the glans and foreskin) are more common in the uncircumcised male (approximately 6%) but may also be found in circumcised children (Fig. 82–8). It frequently presents during the preschool-age years and rarely prior to toilet training.
Balanoposthitis: note the erythema, localized edema, and significantly constricted preputial orifice of the distal penis.
Balanitis may be caused by entrapment of organisms under a poorly retractable foreskin. The etiology is often related to inadequate hygiene or chemical irritation from soaps or bubble baths, or persistent manual manipulation.1 Gram-negative or -positive bacterial organisms may be causative, and Group A β-hemolytic streptococcus has been implicated. Candidal infections are also associated with balanoposthitis in infants. In adolescents, sexually transmitted diseases must be considered as a cause. Chronic balanitis or phimosis may result in balanitis xerotica obliterans, a sclerotic disease of the prepuce noted histologically.
Signs and symptoms include swelling, erythema, penile discharge, dysuria, bleeding, and, rarely, ulceration of the glans. Phimosis can occur but is uncommon. A careful examination of the base of the penis should be performed to look for a strand of hair that may cause strangulation and edema.
Balanitis is diagnosed clinically. In selected cases, the clinician may wish to obtain a urinalysis and send cultures of the penile discharge.
Local care with soaks (Sitz baths), gentle cleaning of the foreskin sulcus, and topical antibiotic ointment are recommended. Application of 0.5% hydrocortisone cream may be helpful.1 The addition of oral antibiotics, such as cephalexin, for 5 to 7 days, may be reserved for the more severe cases. The presence of a purulent discharge, fiery-red erythema, and moist exudate might suggest a streptococcal etiology.1 Urethral discharge should prompt STD cultures. Patients should be followed within 2 days to assure that symptoms have resolved. Children with repeated episodes may be referred to a urologist for elective circumcision.
Priapism is a prolonged (over 4 hours), painful erection unrelated to sexual stimulation. It is relatively uncommon in childhood except in patients with sickle cell disease. In this group, priapism occurs in up to 10% of patients. Priapism is rare in the neonatal period where polycythemia and trauma are presumed etiologies.
Pathophysiology of priapism can be divided into two mechanisms:
Low-flow or ischemic mechanism: there is little or no cavernous blood flow, and the penis is painful and tender to palpation; it is a urologic emergency seen in sickle cell disease and polycythemia
High-flow or engorgement mechanism: not ischemic, the penis is typically not fully rigid or painful; preceeding trauma is the most common etiology.14,15
Either mechanism results in engorgement of the corpora cavernosum with a flaccid corpora spongiosum and glans. This engorgement leads to inflammation, increased stasis of cavernous blood, deoxygenation, acidosis, further sludging, thrombosis, fibrosis, and impotence if unrelieved. Drugs of abuse such as alcohol, marijuana, cocaine, and amphetamines have also been implicated. Fortunately, most cases of priapism can be treated medically and do not result in impotence.
Factors that may precipitate priapism in patients with sickle cell anemia are infection, trauma, acidosis, hypoxia, sexual intercourse, and masturbation. Other etiologies of priapism include trauma (genital, pelvic, or perineal, such as a straddle injury), drugs (antihypertensives and vasoactive agents, anticoagulants, antidepressants or psychoactives, and alprostadil), leukemia, Kawasaki's disease, and polycythemia. When used as directed, phosphodiesterase-5 inhibitors commonly used for erectile dysfunction (such as sildenafil and tadalafil) have rarely been associated with priapism.14
Patients often have delayed presentation, possibly a result of embarrassment. Patients are noted on physical examination to have an erect penis, which is firm on the dorsal surface (corpora cavernosum) and soft on the ventral surface (corpora spongiosum) and glans. Patients should be asked about previous history of priapism and its treatment, duration and degree of pain, and placement of urethral and penile foreign bodies. The bladder should be palpated for signs of urinary retention, which can be easily relieved by placement of a urinary catheter.
Priapism is a clinical diagnosis based on physical examination. However, the physician may wish to order a complete blood count, looking for evidence of leukemia or anemia, and a sickle cell preparation and hemoglobin electrophoresis. Renal function studies are indicated if there has been significant urinary retention. If patients have suffered perineal trauma, a retrograde cystourethrogram may be indicated. Color Doppler US may be used to determine if the priapism is secondary to a low-flow or a high-flow state.
Treatment is based on the presumed etiology. Providing oxygen, hydration, and analgesics to patients with sickle cell disease may alleviate the priapism. If not, an exchange transfusion in consultation with a hematologist is considered. Patients with leukemia may receive hydration and analgesics and appropriate treatment for their cancer. A urinary catheter should be inserted to relieve bladder distension. Once medical management is initiated, patients should be admitted for observation.
Urologic consultation is recommended in all cases. The timing of surgical management of priapism is controversial. Although some clinicians prefer to attempt medical management initially, the American Urological Association recommends that surgical treatment be initiated concurrent with systemic treatment for ischemic priapism.14 Initial intervention uses therapeutic aspiration with or without irrigation, followed by intracavernous injection of a sympathomimetic vasoconstrictor such as phenylephrine. If unsuccessful, surgical shunting of blood from the cavernosum to the spongiosum or the glans may be performed.14 Intravenous ketamine hydrochloride has been used to treat priapism in the newborn. Parenteral vasodilators, including hydralazine or terbutaline (0.25 to 0.5 mg IV every 4 hours), have been used to treat priapism with varying success. Aspiration and sympathomimetic agents are not used for nonischemic priapism emergently.14