Priapism is a pathologic painful penile erection, unrelated to sexual stimulation, lasting more than 4 hours. There are three types of priapism: low flow (ischemic), high flow (nonischemic), and stuttering (recurrent). Ischemic priapism is due to venous occlusion, leading to compartment syndrome of the penis, and is a urologic emergency . Nonischemic, or high flow priapism, is due to unregulated cavernosal blood flow, often due to trauma or arteriocavernous fistula, and is usually painless. In this subtype, oxygenation is preserved and can be managed conservatively. Lastly, stuttering priapism is due to recurrent episodes of ischemic priapism, lasting less than 4 hours. It is often self-limited, and primarily occurs in patients with sickle cell disease.

Priapism is rare in pediatrics, although it may be underreported. It can occur at any age, even rarely, in the neonatal population. Sickle cell disease accounts for 65% of all episodes of priapism occurring in children. Up to 89% of men with sickle cell disease have reported at least one episode of priapism before the age of twenty. Patients present with painful rigidity of the corpus cavernosa and a soft glans ( Fig. 168.1 ). Depending on the duration of the episode, one may see sequelae of ischemia such as erythema, other color change, or gangrenous appearance.

Anatomy of the penis.

Reproduced with permission from: Field JJ, Vemulakonda VM, DeBaun MR. Diagnosis and management of priapism in sickle cell disease . www.uptodate.com/contents/diagnosis-and-management-of-priapism-in-sickle-cell-disease .

Priapism is a clinical diagnosis. The differential diagnosis of the underlying etiology is outlined in Table 168.1 . Laboratory testing can help determine an underlying etiology but is often not helpful in acute management. Complete blood count (CBC), reticulocyte count, and hemoglobin electrophoresis are useful in identifying undiagnosed sickle cell anemia. A cavernosal blood gas and color duplex ultrasonography can help differentiate the subtype of priapism.

Determination of subtype and underlying etiology guide management of priapism. Ischemic priapism requires time-sensitive intervention. Conservative measures may be trialed in patients presenting before 4 hours: physical exercise, urination, and cold packs (except in sickle cell patients in which this may worsen priapism). In patients presenting after 4 hours, or in whom conservative measures have failed, additional treatment includes hydration, pain control, and local anesthesia. This should be followed with intracavernous irrigation of saline and injection of sympathomimetics to achieve detumescence. Various case reports have shown detumescence using procedural sedation alone with either ketamine or nitrous oxide. ^, A dorsal penile block with or without a ring block should be performed prior to intracavernous injection, using no more than 4 mg/kg of lidocaine without epinephrine ( Fig. 168.2 ). After local anesthesia, corporal aspiration can be performed by using a 23- to 21-gauge butterfly needle in prepubescent males and a 19-gauge needle in adolescents; the needle should be inserted laterally, into the corpus cavernosum at 3 or 9 o’clock, avoiding the neurovascular bundle superiorly and the urethra inferiorly. Blood should be aspirated in 5 mL aliquots until it appears bright red followed by flush with 0.9% normal saline. A cavernosal blood gas can be sent using this sample. If detumescence is not achieved, the next step is intracavernosal injection of an alpha-adrenergic sympathomimetic agent, phenylephrine (100 mcg every 5 minutes, up to 1 hour. If these measures fail, the patient should be considered for emergent urologic intervention with surgical shunt placement.

Penile analgesia.

From: Davis JE, Silverman MA. Urologic procedures. In: Roberts JR, Custalow CB, editors. Roberts and Hedges’ Clinical Procedures in Emergency Medicine . Philadelphia: Elsevier Saunders; 2014: 1113-1154.

Contrary to ischemic priapism, management of stuttering and nonischemic priapism is not urgent, as many cases resolve spontaneously. Ultrasound may identify a fistula that may require arterial embolization or surgical ligation.

With successful detumescence, patients may be discharged home with close follow-up. Those who require ongoing injections of phenylephrine will likely need admission, especially if they have cardiac history. Patients who fail injection and require surgery should receive emergent urologic consultation and admission.

Phimosis is a clinical diagnosis and defined as the inability to fully retract the prepuce beyond the glans penis. Parents or patients may describe a “balloon appearance” of the foreskin with urination. The majority of phimosis cases are physiologic. The ability to retract the prepuce increases with age, with 50% resolving by age one and 89% by age three. Phimosis is pathologic when it leads to balanoposthitis, urinary tract infections (UTI), or urinary retention.

Asymptomatic patients do not require further workup, but those with pain on urination should have a urinalysis (UA) and culture to evaluate for urinary tract infection.

Management of phimosis is conservative in most cases, but ongoing issues may require circumcision for definitive treatment. There is no general consensus among urologists on the care of phimosis. Parents should be instructed on general hygiene measures of the uncircumcised penis and may practice gentle retraction beginning at 2 years of age. If there are minor recurrent issues with hygiene, infection, or urination, a course of 1% topical hydrocortisone or 0.1% topical triamcinolone applied BID for up to 12 weeks have similar rates of phimosis resolution.

Those with chronic UTIs, balanoposthitis, or issues with urination should be referred to urology for circumcision consideration. Patients who are able to urinate and have a reassuring physical examination can be managed as outpatients with primary care or urologic follow-up.

Paraphimosis is a urologic emergency, wherein the foreskin of the penis is trapped behind the glans at the corona, resulting in engorgement, venous congestion, and vascular compromise. If left untreated, venous congestion and arterial compromise can lead to distal ischemia and even necrosis of the penis.

History may reveal a recent penile examination, Foley catheter placement, parental attempts at hygiene, trauma, or recent sexual intercourse. This occurs more commonly in the uncircumcised male, but circumcised individuals who have excessive foreskin can develop paraphimosis. On examination, patients present with painful swelling of the penis with foreskin visibly retracted behind the glans. Any discoloration may indicate ischemia and compromised blood supply ( Fig. 168.3 ).

Paraphimosis in a 4-year-old uncircumcised boy.

Courtesy Dr. Marianne Gausche-Hill.

The diagnosis is clinical, based on history and physical examination, and there is no role for blood work or imaging. The penis should be inspected for hair tourniquet, which can mimic symptoms of paraphimosis.

Management is directed at replacing the foreskin back over the glans penis. In uncomplicated cases, the foreskin can be manually reduced. Ice packs are useful in decreasing edema but could worsen already compromised penile arterial flow. With manual reduction, gentle pressure is applied for 5 to 10 minutes, either by hand or with compression bandages. After applying pressure to reduce the edema, the provider should use two thumbs to gently press the glans, while pulling the foreskin into place ( Fig. 168.4 ). Osmotic methods with sugar or 20% mannitol-soaked gauze can reduce edema prior to manual reduction.

Paraphimosis reduction.

Courtesy P.P. Kelalis.

If manual reduction is not successful, analgesia with dorsal penile block and procedural sedation may be required to facilitate the procedure. If unsuccessful, a dorsal slit may be required, using two clamps at the foreskin at 12 o’clock followed by an incision to release the constriction. Ultimately, surgical circumcision by a urologist may be required.

Patients with uncomplicated cases that are manually reduced can be discharged home with outpatient follow-up. The foreskin should be inspected for any abrasions, which should be treated with topical bacitracin if found. Patients should not retract the foreskin for a week to avoid recurrence. Patients should be evaluated for need for definitive treatment with circumcision by urology. Patients with ischemia or necrosis should be admitted for pain control, antibiotics, and further care by urology.

Balanoposthitis is the inflammation of both the glans (balanitis) and foreskin (posthitis) of the penis, and is most common in young uncircumcised males. In pediatrics, the majority of cases are due to poor hygiene, with accumulated sebaceous material leading to bacterial or fungal overgrowth; however, there are also other infectious (fungal, bacterial, HPV, STIs) and noninfectious (contact dermatitis, chemical irritant, trauma) etiologies to consider.

Patients present with localized pain, erythema, and sometimes dysuria due to local irritation. Penile discharge, rash, or lymphadenopathy raises suspicion for a sexually transmitted infection, such as Neisseria gonorrhea or Chlamydia , whereas oral ulcerations or arthralgias may point to a rheumatologic etiology, such as psoriasis or lichen sclerosis. The foreskin should be assessed for concomitant phimosis or paraphimosis.

The diagnosis of balanoposthitis is clinical, based on history and physical examination. Additional evaluation for patients with dysuria, discharge, extragenital findings, or recurrent balanitis may include UA, STI testing, or glucose (to assess for diabetes in recurrent candidal balanitis).

Management of balanoposthitis is mainly supportive and directed at adequate hygiene measures. Parents and teenagers should be educated on hygiene and care of the uncircumcised penis. Teenagers should also be provided with education on safe sex practices. Treatment should include coverage for both bacterial and fungal overgrowth, with topical bacitracin and topical nystatin (or clotrimazole), respectively. Inflammation can be managed with 0.5% hydrocortisone cream twice daily. In the event of overlying mild cellulitis, patients should be prescribed cephalexin, 25 to 50 mg/kg/day in divided doses for seven days.

We recommend that children who are unable to urinate, have signs of systemic illness (e.g., fever), or evidence of more than a mild cellulitis be admitted for IV antibiotics and receive consultation from a urologist.

At times a controversial topic, circumcision rates are approximately 38% worldwide. The most recent American Academy of Pediatrics Task Force Recommendations in 2012 concluded that there are lifelong benefits to circumcision that outweigh the risks of the procedure itself. The rate of adverse events from circumcision is low at 1% to 4%.

The major postoperative circumcision complication is bleeding, which may reveal an underlying coagulopathy. Coagulants such as topical thrombin and local pressure should be applied to the circumcised area; patients with a bleeding diathesis may warrant a hemoglobin check and coagulopathy studies, as well as fresh frozen plasma (FFP) or factor replacement. Urgent urologic consultation may be required.

Other immediate postoperative complaints include pain, concern for infection, or extremely rarely, a more severe injury to the penis. Pain usually self-resolves and should not require intervention; if a child is older than 6 months, nonsteroidal antiinflammatory drugs (NSAIDs) or acetaminophen may be trialed. As the circumcision dressing can contribute to urinary retention, patients are predisposed to a urinary tract infection. Those with symptoms should have a UA sent. Systemic infection is uncommon, but in the neonatal period may require a full septic workup with blood work, urine, and cerebrospinal fluid (CSF) studies. Delayed presentation may indicate postoperative adhesions or meatal stenosis. Adhesions or meatal stenosis require outpatient urology follow-up and potential repeat circumcision at a later date.

Penile injuries due to entrapment or hair tourniquet may present from childhood to adolescence and can result in neurovascular damage. Metallic rings used to improve sexual activity are placed by the patient, whereas zippers or hair are the result of accidental entrapment. These objects create damage by reducing vascular supply to the penis and can become a urologic emergency.

The patient will present with pain and swelling of penis. In the event of a hair tourniquet, the hair itself may be difficult to visualize. This does not require any lab testing or imaging, unless there is concern for underlying urethral injury, in which case a retrograde urethrogram may be required along with urology consult.

Management depends on the offending agent. Nonmetallic objects can be cut off with ring cutters or a saw, while using cooling agents to avoid burns to the skin. Hair can be removed using a hair removal cream or by direct visualization and cutting the hair. Depending on the amount of edema, one may need to apply ice or compression before attempting removal. Pain control using a penile block will facilitate manipulation and removal of an object.

In the event of zipper entrapment, the zipper should be removed to free the foreskin from entrapment. There are 5 common methods to remove a zipper from the genitals: cutting the median bar with bone or wire cutters ( Fig. 168.5 ); using a screwdriver to separate the teeth; application of mineral oil as lubricant; lateral compression of the fastener with pliers; and removal of the teeth with trauma sheers. The cloth of the zipper should be cut close to the zipper teeth, which should release the underlying skin. Upon release, the underlying tissue may be damaged. If these techniques are unsuccessful, urology should be consulted for surgical intervention with circumcision as the last option.

Release of zipper entrapment by cutting of the median bar.

From: Snyder HM III, et al. Genitourinary trauma. In: Fleisher GR, Ludwig S, editors. Textbook of Pediatric Emergency Medicine , ed 4. Philadelphia: Lippincott Williams & Wilkins; 2000.

Most of these cases do not require surgical intervention and can be discharged home after the removal of the offending agent.

Acute epididymitis is pain and swelling of the epididymis lasting less than 6 weeks, whereas orchitis is pain and swelling of the entire testicle. The etiology of the inflammation in epididymitis is due to infectious and noninfectious causes. Infectious causes vary by age of presentation. The most common cause in prepubescent males is viral infections. Adolescents may get epididymitis from N. gonorrhoeae or C. trachomatis ; other bacterial causes in this age group may be related to structural anomalies of the urinary tract. Noninfectious causes may be due to a vasculitis (e.g., in Henoch-Schönlein purpura, see later), chemical irritation, or trauma. Orchitis is usually the result of bacterial or viral infection and is classically associated with mumps.

Patients present with scrotal pain and swelling, with tenderness on physical examination. Patients may complain of nausea, vomiting, or referred abdominal pain. Urethral discharge may indicate a sexually transmitted infection (STI). Cremasteric reflexes should be intact, and one may see relief of pain with scrotal elevation (Prehn sign), although these elements do not rule out testicular torsion. Those with mumps may also present with viral symptoms, as well as lymphadenopathy and parotid gland swelling. Differential diagnosis should include epididymitis, orchitis, testicular torsion and torsion of the appendix testis, inguinal hernia, hydrocele, and varicocele.

Though the diagnoses of epididymitis and orchitis are clinical, UA and culture should be obtained to evaluate for pyuria. STI testing should be considered for all adolescents and those who are sexually active. Ultrasound (US) should be obtained in cases where testicular torsion is considered possible. US may show increased vascular flow to the epididymis. Patients with bacterial orchitis may rarely get a scrotal abscess, which would also be detected on ultrasound.

Management of epididymitis and orchitis includes supportive care with analgesia, ice packs, and scrotal elevation. If there is concern for gonococcal STI, patients should be empirically treated with 500 mg IM ceftriaxone for patients <150 kg. If there is concern for chlamydial STI, treatment is with either doxycycline 100 mg PO BID for 7 days or azithromycin 1 g PO once. Those with UA evidence of UTI should be treated for a complicated UTI according to local sensitivity patterns for E. coli . Scrotal abscess should receive antibiotics and consultation with urology.

Most patients with uncomplicated epididymitis or orchitis can be discharged home with outpatient follow-up.

Testicular torsion is a urologic emergency associated with acute onset of scrotal pain. Testicular torsion should always be considered in acute scrotal pain, as delayed diagnosis can lead to loss of viability of testicular tissue and potentially loss of spermatogenesis. Testicular torsion can occur at any age, but in particular, has peaks in the neonatal and adolescent periods. Testicular salvage rates depend on time to presentation, as well as time to surgical intervention, with increased orchiectomy rates when blood flow is not restored within 6 hours of symptom onset.

Testicular torsion can occur within the tunica vaginalis (i.e., intravaginally), constricting arterial blood flow, or within the scrotum (i.e., extravaginally). If there is inadequate fixation of the testicle, in which the tunica vaginalis completely covers the testis superior to the spermatic cord, this “bell clapper” deformity predisposes to torsion ( Fig. 168.6 ). Other risk factors include cryptorchidism, trauma, familial history, or prior episodes.

Anatomy of testicular torsion.

From: Snyder HM III, et al. Pain—scrotal. In: Fleisher GR, Ludwig S, editors. Textbook of Pediatric Emergency Medicine , ed 4. Philadelphia: Lippincott Williams & Wilkins; 2000.

Patients with testicular torsion usually present with acute onset unilateral scrotal pain and edema, often associated with nausea and vomiting. Pain may radiate to the abdomen, and children may be embarrassed to note genital complaints; thus, even without a testicular complaint, a complete GU examination should be performed. Patients may have a high-riding, transverse, swollen testicle, with possible discoloration, and lack of cremasteric reflex on the affected side, though a normal cremasteric reflex does not rule out torsion.

Differential diagnosis includes torsion of the testicular appendage, which presents similarly, but usually occurs in prepubescent males with a more indolent course and less intense pain at the superior pole of the testis. Other considerations include orchitis, epididymitis, trauma, hydrocele, varicocele, inguinal hernias, or testicular tumors.

Testicular torsion is a clinical diagnosis, but scrotal ultrasound with Doppler can confirm the diagnosis when the testis is torsed. A UA may show hematuria or leukocytosis, which would be more indicative of epididymitis or orchitis. Doppler ultrasound may show decreased perfusion to the testicle or twisting of the spermatic cord.

Urology should be consulted emergently for definitive surgical management. Once testicular torsion is confirmed, management requires surgical detorsion on the affected side, with either orchiopexy or orchiectomy if the testicle is not viable. Time to the operating room is directly related to testicular salvage, with less than 6 hours as most optimal.

In the event of surgical delay, a bedside manual detorsion under analgesia can be attempted after discussion with urology. Because most torsion occurs medially, the testicle is rotated in an “open book” fashion, laterally until detorsion has occurred. However, a small percentage of cases do torse laterally, so this maneuver could worsen symptoms in those cases. If manual detorsion fails, surgical management is warranted. For suspected testicular torsion that has detorsed and has normal findings or high flow (i.e., from reperfusion) on ultrasound, we recommend urology be consulted and surgery be considered to prevent recurrence.

Painless scrotal swelling in children may occur secondary to a hydrocele or varicocele. A hydrocele occurs when peritoneal fluid accumulates within the tunica vaginalis; hydroceles are either communicating or noncommunicating with the peritoneal cavity. Hydroceles commonly occur in newborns and will spontaneously resorb by age one. However, in older children, hydroceles may occur secondary to epididymitis, orchitis, torsion, or tumor. A varicocele is a collection of dilated veins in the pampiniform plexus, which surrounds the spermatic cord, and occurs in about 15% of adolescent males. Primary varicoceles occur spontaneously, while secondary varicoceles occur due to some obstruction of venous outflow of the plexus. Most varicoceles are left-sided due to the 90-degree angle of drainage that occurs from the left spermatic vein to the left renal drain. A right-sided varicocele raises concern for intra-abdominal mass.

If a hydrocele is communicating, painless scrotal swelling may increase when the patient performs a Valsalva maneuver. Transillumination will reveal clear fluid surrounding the testicle. Although varicoceles will also cause painless scrotal swelling, examination will reveal a “bag of worms” consistency on palpation, and a varicocele does not transilluminate. The differential for painless scrotal swelling includes hydrocele, varicocele, spermatocele, cyst, inguinal hernia, or testicular tumor. If there is pain, the differential includes reactive hydrocele secondary to epididymitis, orchitis, torsion, or a tumor.

If there is concern for testicular tumor, a CBC, lactate dehydrogenase (LDH), and uric acid would be reasonable. An ultrasound of the testes will be helpful to evaluate for torsion or mass. For right-sided varicoceles or those of sudden onset, we recommend patients undergo imaging with ultrasound with Doppler, CT, or MRI to evaluate for inferior vena cava thrombus, renal vein thrombosis, or abdominal mass.

Hydroceles in newborns can be managed expectantly as most will resorb on their own. Outside the newborn period, there is moderate evidence that surgical correction of varicocele leads to improved sperm concentration and testicular volume. Patients may be referred to urology for conservative management versus surgical repair with ligation or embolization; in the absence of an abdominal or testicular mass, these cases may be managed as outpatients.

Acute idiopathic scrotal edema (AISE) is a rare diagnosis characterized by acute scrotal erythema, swelling, and tenderness. It is a benign, self-limiting condition with an unclear etiology and is a diagnosis of exclusion. AISE generally affects prepubertal boys, though can affect males into adulthood.

Physical examination shows swelling, induration, and erythema of a unilateral testicle, which may or may not be tender. Diagnosis is via ultrasound and demonstrates edematous thickening and increased vascularity of the scrotal wall, but normal-appearing testes.

Once more insidious causes have been ruled out, AISE generally resolves within a few days with NSAIDs and scrotal support.

An inguinal hernia is a common cause of scrotal swelling in males and labial swelling in females, with indirect hernias accounting for just under 5% of full-term infants. Risk increases with prematurity, low birth weight, and male gender. In males, an indirect hernia results from failed closure of the processus vaginalis, and usually contains intestines. In females, a hernia results from the failed closure of the canal of Nuck, and more often contains ovary rather than bowel. The greatest risk is with incarceration (inability to reduce the herniated contents), as well as strangulation (vascular compromise), of the herniated contents.

Patients present with intermittent bulge of the inguinal region into the scrotum or labia, or a persistent bulge consistent with incarceration. Patients with a reducible bulge usually have no complaints of pain, whereas those with incarceration may have a significant amount of pain. The patient may also experience vomiting and diffuse abdominal pain from intestinal obstruction.

Differential diagnosis should include hydrocele, varicocele, testicular/ovarian torsion, or malignancy. The diagnosis is often clinical, but ultrasound with Doppler can assist in identifying herniated contents as well as ovarian flow in females.

Ultimately, surgery is needed for definitive repair. As long as the patient is clinically stable, manual reduction should be attempted with appropriate analgesia, by applying traction to the proximal inguinal canal with one hand and gentle pressure to the distal canal towards the abdomen with the other hand for up to 5 minutes. Incarcerated hernias with strangulation, as well as canal of Nuck hernias containing ovary, have high risk for bowel ischemia or ovarian torsion without prompt surgical intervention. In the event of painless, reducible hernias without incarceration or strangulation, patients can be referred for surgery and outpatient evaluation.

Although a small percentage of overall pediatric malignancies, testicular tumors are the most common solid organ tumor in adolescents, comprising 14% of all adolescent cancer. Patients with cryptorchidism, prior testicular cancer on the contralateral side, or family history of cancer are all at increased risk. Most testicular cancers are teratomas, carcinomas, yolk sac tumors, Leydig cell tumors, or Sertoli cell tumors; however, lymphoma and leukemia can also metastasize to the testicle.

Patients present with painless unilateral testicular swelling or mass. Some may describe an aching or pulling sensation, or abdominal pain. There may be a reactive hydrocele present, but the testis does not transilluminate with an underlying mass. Examination may demonstrate lymphadenopathy, hepatosplenomegaly, or gynecomastia.

Diagnostic evaluation should include a CBC with differential, chemistry panel, LDH, uric acid, human chorionic gonadotropin (HCG), UA, and ultrasound. Labs are obtained to evaluate for cell line suppression, tumor lysis syndrome, or end-organ damage. Chest x-ray should be performed to rule out mediastinal mass with testicular metastasis. Tumor markers such as HCG, alpha fetal protein (AFP), luteinizing hormone (LH), and follicular stimulating hormone (FSH) can be sent and are helpful to the oncologist for further delineation of the underlying subtype. An ultrasound is necessary to identify solid tumor versus a cystic structure.

Once the workup is complete, pediatric oncology should be consulted for further management, including CT chest, abdomen, and pelvis for staging purposes, orchiectomy for histologic evaluation, and possibly retroperitoneal lymph node dissection or chemotherapy. A testicular biopsy should not be performed due to concern for possible tumor seeding. Initial management may be inpatient, with long term outpatient follow-up.