Browse the corpus

Walk the Even Hospital Database by book and chapter — the raw source passages that ground Ask, DDx, and the rest.

15 passages

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

874 SECTION 12: Pediatrics uncomplicated UTI may be appropriate for outpatient care with oral antibiotics if they appear well, can tolerate oral medication, are not dehydrated, and are not immunocompromised. Outpatient IM or IV ceftriaxone (75 milligrams/kg) or gentamycin can be used as an alternative to hospitalization for patients who are unable to tolerate oral therapy but are otherwise nontoxic and well hydrated. Length of antimicrobial therapy should be 7 to 14 days with close pediatric follow-up to ensure appropriate antibiotic use and to consider the need for imaging. Adolescent girls (>13 years old) with UTI may be treated like adults with the option for a 3-day oral antibiotic regimen. Outpatient repeat urine testing as proof of cure is not routinely necessary or recommended. Of note, after confirmation of a febrile UTI in infants, parents or guardians should be counseled to bring back their child (ideally within 48 hours) for future febrile illnesses to ensure prompt medical evaluation and treatment of possible recurrent UTI. SPECIAL SITUATIONS  PEDIATRIC UTI WITH UROLITHIASIS Although uncommon, urolithiasis occurs in children, and the incidence is increasing and may be associated with the increasing prevalence of childhood obesity. 37-39 Children with symptoms suggestive of urolithiasis (colicky abdominal or flank pain, often radiating to the groin, and gross or microscopic hematuria) require imaging to evaluate for the possibil ity of stones (see Chapter 133, “ Acute Abdominal Pain in Infants and Children, ” and Chapter 137, “Renal Emergencies in Children”). While non–contrast-enhanced CT is the imaging modality of choice in adults, consideration of the potential long-term effects of radiation exposure must be weighed carefully, and renal US or MRI should be considered as alternatives. 40 In the setting of fever, pyuria, and an obstructing stone, initial treatment should be parenteral with inpatient management and consultation with pediatric urology. Additional urine studies may be obtained to identify predisposing factors and chemical composition of stones, which may guide future management. REFERENCES The complete reference list is available online at www.TintinalliEM.com. Pediatric Urologic and Gynecologic Disorders Deborah R. Liu SCROTUM Scrotal pain is one of the most common urologic emergencies seen in boys. Although many causes of scrotal pain may not require an immediate organ-preserving procedure, some causes can lead to rapid and permanent loss of testicular function without timely intervention. Thus, the clinician must identify patients who need emergent diagnostic and/or therapeutic procedures and those who need observation and reassurance.  TESTICULAR TORSION Consider testicular torsion in males with acute scrotal pain, because torsion is a urologic emergency. The estimated incidence of torsion in U.S. males younger than 18 years is 3.8 per 100,000 children. 1 Testicular torsion has a bimodal age presentation, with one peak in the immedi ate neonatal period and another peak during early puberty. Because the testicle of neonates with true prenatal torsion is not salvageable, many urologists agree that neonates can be taken to the operating room on a semi-elective basis when the infant is a few months of age to decrease the CHAPTER anesthesia risk.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

mmedi ate neonatal period and another peak during early puberty. Because the testicle of neonates with true prenatal torsion is not salvageable, many urologists agree that neonates can be taken to the operating room on a semi-elective basis when the infant is a few months of age to decrease the CHAPTER anesthesia risk. However, in neonates who experience postnatal torsion, the salvage rate is likely similar to adolescent testicular torsion, making early surgical detorsion a priority. The clinical distinction between pre natal and postnatal torsion, however, is sometimes difficult to elucidate. Most boys with testicular torsion present between 12 and 18 years of age. Classically, the pain is abrupt in onset and severe and is usually associated with nausea or vomiting. The testicle is extremely painful, and often the patient will walk with a wide-based gait to minimize the contact of the scrotum to the thigh. There may be a preceding history of a sports activity or even minor trauma to the area, which may lead the clinician to a misdiagnosis of traumatic injury. In some cases, the patient may recall episodes of previous scrotal pain that rapidly resolved without intervention, which may represent intermittent torsion with spontaneous detorsion. Patients with intermittent testicular pain should be referred for elective orchiopexy, as such patients with intermittent torsion are at risk for acute complete testicular torsion. Classic physical examination findings of acute testicular torsion include a swollen, tender, high-riding testis, with an abnormal transverse lie. There are often scrotal skin changes. Ipsilateral loss of the cremasteric reflex is often noted, but the presence of a normal cremasteric reflex does not rule out torsion. Doppler US is the diagnostic imaging study of choice,5 with radionuclide imaging a distant second. If the time to obtain diagnostic imaging may lead to delay of surgical intervention, advocate for emergent surgi cal exploration for highly suspected cases of torsion, rather than waiting for an imaging study to be completed. Time is especially critical if the duration of symptoms is <6 hours, as the salvage rate is excellent in such cases. Beyond 6 hours, the salvage rate becomes progressively worse, and after 48 hours of symptoms, the salvage rate is near zero. Patients presenting with equivocal signs of torsion may benefit from a Doppler US, which can visualize blood flow to the testis. In acute torsion, Dop pler demonstrates an enlarged testis with decreased or absent flow compared with the unaffected side. In patients with suspected intermittent torsion who have a normal Doppler US and resolution of pain, counsel the patient and family to seek medical attention immediately should the pain recur, and recommend urologic follow-up as an outpatient. Several recent studies have attempted to develop clinical predictors for acute torsion, with the hopes of decreasing time to surgical intervention for true cases of torsion, reducing the rate of negative explorations, and decreasing the number of unnecessary imaging studies. 4,6-9 The TWIST (Testicular Workup for Ischemia and Suspected Torsion) scor ing system was developed in 2013 to predict testicular torsion based on the following findings: testicular swelling (2 points), hard testicle (2 points), absent cremasteric reflex (1 point), nausea/vomiting (1 point), and high-riding testis (1 point). 8 This scoring system was retrospectively validated when used by urologists, and it was suggested that high-risk patients (score of ≥5) should have immediate surgical exploration, intermediate-risk patients (score of 3 or 4) should undergo Doppler US, and low-risk patients (score ≤2) do not require any testing.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

point). 8 This scoring system was retrospectively validated when used by urologists, and it was suggested that high-risk patients (score of ≥5) should have immediate surgical exploration, intermediate-risk patients (score of 3 or 4) should undergo Doppler US, and low-risk patients (score ≤2) do not require any testing. 8 In 2016, the TWIST score was validated using non-urologists, but it was noted that the trained emergency medical technicians had different cutoffs for high risk (score of ≥6), intermediate risk (score of 1 to 5), and low risk (score of 0). 9 These studies have provided a more objective framework in the management of suspected torsion. To optimize timeliness and minimize unnecessary testing, advocate for immediate surgery in patients with highly suspected torsion, obtain emergent Doppler US for patients with equivocal signs of torsion, and consider no testing for patients with very low suspicion for torsion. Manual detorsion may be indicated for patients with torsion when there is no surgeon immediately available and when the duration of symptoms is too long for surgical salvage. Administer parenteral opioid analgesia, local anesthesia (infiltrating the spermatic cord near the external ring with lidocaine), or procedural sedation. Because the testis tends to torse in the medial direction, manual detorsion is accomplished by holding the testis between the thumb and index finger and rotating the testis in an outward direction toward the thigh (as if opening a book). However, the spermatic cord may be twisted >180 degrees, making it difficult to recognize how many times the testis should be outwardly rotated. Also, a very swollen hemiscrotum may make isolating the testis between two fingers challenging. The unsedated, older, verbal patient Tintinalli_Sec12_p0669-0996.indd 874 8/2/19 7:53 PM

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

the spermatic cord may be twisted >180 degrees, making it difficult to recognize how many times the testis should be outwardly rotated. Also, a very swollen hemiscrotum may make isolating the testis between two fingers challenging. The unsedated, older, verbal patient Tintinalli_Sec12_p0669-0996.indd 874 8/2/19 7:53 PM CHAPTER 136: Pediatric Urologic and Gynecologic Disorders 875 may be able to describe relatively immediate relief upon successful detorsion. Bedside or formal Doppler US may be useful in determining improvement of flow. The success rate of manual detorsion is quite variable and is not definitive therapy. Even after manual detorsion, patients need emergent surgical exploration to confirm complete detorsion and to perform bilateral orchidopexy.  TORSION OF TESTICULAR APPENDAGE The appendix testis and appendix epididymis are testicular embryologic remnants that can twist, resulting in venous congestion and subsequent infarction of the appendage. Torsion of a testicular appendage is most common in males between 7 and 12 years of age, although it can occur at any age. Typically, the patient’s symptoms are more insidious than true testicular torsion, with less severe pain and lack of systemic symptoms. Early in the course before scrotal edema and erythema develop, it may be possible to localize the point of tenderness to the upper pole of the testis or epididymis. In addition, one may observe the infarcted appendage through the scrotal skin (“blue dot sign”). If Doppler US is obtained, there should be normal testicular flow with a small hyperechoic region adjacent to the testis. Management consists of scrotal support, limita tion of activity, and oral analgesics (e.g., nonsteroidal anti-inflammatory drugs). If torsion of the testicular appendage is diagnosed early in its course, the pain may worsen before ultimate improvement due to ongoing inflammation, and this is an important point of counseling to prevent return to the ED.  EPIDIDYMITIS Epididymitis, or inflammation of the epididymis, is a common cause of scrotal pain in pre- and postpubertal boys that does not require surgical intervention. In a 2014 study of 252 patients with epididymitis present ing to an outpatient pediatric urology referral practice, the mean age at first presentation was 10.92 years, with the majority of cases occurring between 10 and 14 years. 10 In sexually active males, acute epididymitis may result from ascending urethral infection due to Chlamydia trachomatis or Neisseria gonorrhoeae. Epididymitis may also result from enterovirus or adenovirus infection. 11 Symptoms are insidious in onset, with dysuria, frequency, or fever. Prehn’s sign (relief of pain by elevating the scrotum) is not consistently reproducible in boys. Typically, the affected testis is mildly enlarged and tender with hemiscrotal erythema and swelling (Figure 136-1). Urinalysis may demonstrate pyuria and bacteriuria. Obtain urine culture and sensitivity, and in suspected sexual transmis sion, obtain specific testing for C. trachomatis and N. gonorrhoeae. Doppler US, if the diagnosis is in doubt, shows an enlarged epididy mis with increased blood flow, and normal blood flow to the testis. It is often difficult to differentiate torsion of a testicular appendage from FIGURE 136-1. Epididymo-orchitis. A 5-year-old boy with 1 day of left testicular pain and swelling, consistent with epididymo-orchitis. FIGURE 136-2. Hydrocele. A 3-month-old boy with bilateral hydroceles (left greater than right). epididymitis by US, but neither disorder requires surgical interven tion. Treatment of epididymitis is somewhat controversial, with some advocating analgesics and limited activity only and others supporting treatment with antibiotics.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

-2. Hydrocele. A 3-month-old boy with bilateral hydroceles (left greater than right). epididymitis by US, but neither disorder requires surgical interven tion. Treatment of epididymitis is somewhat controversial, with some advocating analgesics and limited activity only and others supporting treatment with antibiotics. In this era of antibiotic stewardship, consider selectively treating certain cases of epididymitis with empiric antibiot ics. In a 2011, retrospective, single-center, pediatric ED study of 140 patients, the incidence of positive urine culture in acute epididymitis was only 4.1%. The authors suggest empiric antibiotics for all young infants regardless of urinalysis results, as well as for all non–sexually active males with a positive urinalysis. 12 Sexually active males should be treated presumptively for C. trachomatis and N. gonorrhoeae.  HYDROCELE Hydrocele (Figure 136-2), the accumulation of fluid around the testis, is the most common cause of painless scrotal swelling in children. Parents often note intermittent swelling of one or both sides of the scrotum. This painless swelling may resolve when supine or sleeping and become more prominent when awake or crying. Hydrocele is termed noncommunicating, if there is residual but static swelling after the processus vaginalis has closed, or communicating, if swelling increases and decreases through a patent processus vaginalis. The diagnosis is confirmed by transillumination, whereby an otoscope or other light source is placed on the affected hemiscrotum and the hydrocele fluid is illuminated like a lantern; by contrast, a thickened scrotal wall or pure testicular enlargement will not transilluminate. Most simple hydroceles resorb by 18 to 24 months of age. A communicating hydrocele is often associated with inguinal hernia, and as long as the hernia is reducible, it does not require emergent surgical repair. Management of most hydroceles includes outpatient scrotal US with referral to urology.  VARICOCELE Varicocele is another cause of painless scrotal swelling and typically presents at the onset of puberty. It is due to abnormal dilation of sper matic cord veins, also known as the pampiniform plexus , due to faulty Tintinalli_Sec12_p0669-0996.indd 875 8/2/19 7:53 PM

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

ent scrotal US with referral to urology.  VARICOCELE Varicocele is another cause of painless scrotal swelling and typically presents at the onset of puberty. It is due to abnormal dilation of sper matic cord veins, also known as the pampiniform plexus , due to faulty Tintinalli_Sec12_p0669-0996.indd 875 8/2/19 7:53 PM 876 SECTION 12: Pediatrics valvular venous return. Most varicoceles occur on the left, possibly due in part to the acute angle of confluence with the left renal vein and resulting higher venous pressure. Classically the mass of enlarged veins can be palpated superior and posterior to the testis (“bag of worms”) and is more prominent when standing or with the Valsalva maneuver; even large varicoceles may be missed in the supine position. Varicoceles are managed on an outpatient basis by urology. The implications of possible subfertility should be discussed in the urologist’s office.  INTRASCROTAL TUMORS Intrascrotal tumors are uncommon in young children; however, testicular tumors are the most common solid tumor among adolescent males.13 The testicular or paratesticular tumor usually presents as a painless, firm, unilateral scrotal mass. Evaluation includes serum α-fetoprotein and tumor β-human chorionic gonadotropin levels, scrotal US, and urgent urologic consultation. DISORDERS OF THE PENIS A swollen, red, or painful penis can usually be categorized as a disorder of the foreskin or of the shaft of the penis. The most common abnor malities of the foreskin are phimosis, paraphimosis, and balanoposthitis. Penile shaft disorders occur less commonly and include priapism, tourniquet syndrome, and zipper injury.  PHIMOSIS Phimosis is caused by stenosis of the distal aspect of the foreskin, preventing retraction of the foreskin over the glans. There may be a history of ballooning of the foreskin during urination, with dribbling of entrapped urine after voiding is complete (Figure 136-3). Most uncircumcised infants have normal, physiologic phimosis. Nearly all cases of physiologic phimosis spontaneously resolve by 5 years of age and rarely require treatment other than daily cleaning while bathing. If a patient has persistent phimosis beyond school age and the parent desires treatment, topical steroid cream can be effective. Several studies have shown that application of a medium-potency topical steroid twice daily for 4 to 8 weeks can be a safe and effective treatment for phimosis. 14-16 Acquired cases of phimosis may be secondary to recurrent balanoposthitis, poor hygiene, or forcible retraction of the foreskin. Acquired cases are often refractory to medical management and may ultimately require circumcision. One of the few true emergencies related to phimosis occurs when the foreskin is nearly completely sealed off, causing acute urinary retention. Such cases may require dilation of the foreskin under procedural sedation or dorsal penile block to place a Foley catheter.  PARAPHIMOSIS Paraphimosis is a true urologic emergency. This occurs when a tight ring of phimotic foreskin is retracted proximal to the glans and becomes trapped in that position. Subsequent impairment of venous and lym phatic drainage causes progressive swelling of the glans and foreskin. If the paraphimosis is not promptly reduced, arterial blood supply becomes compromised, and the glans may necrose. Symptoms of paraphimosis are pain, erythema, and swelling of the shaft and glans, distal to the constricting ring of foreskin (Figure 136-4). The area of the shaft proximal to the constriction appears normal. Because delay in reduction will lead to worsening edema resulting in a more difficult manual reduction, paraphimosis should be reduced as soon as possible. Mild paraphimosis may be manually reduced without the need for sedation or analgesia.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

ure 136-4). The area of the shaft proximal to the constriction appears normal. Because delay in reduction will lead to worsening edema resulting in a more difficult manual reduction, paraphimosis should be reduced as soon as possible. Mild paraphimosis may be manually reduced without the need for sedation or analgesia. More difficult cases will require either a dorsal penile nerve block or procedural sedation, depending on the age and degree of cooperativeness of the patient. The dorsal penile nerve provides most of the somatosensory innervation to the shaft and glans penis. The dorsal penile block (Figure 136-5) is useful for minor painful procedures of the penis, such as paraphimosis reduction, dorsal slit procedure, or zipper entrapment release. Using a 25- or 27-gauge needle, inject lidocaine hydrochloride without epinephrine into the base of penis, at the junction between the penis and the suprapubic skin, off the midline to avoid the superficial dorsal vein. Inject the lidocaine just deep to the Buck fascia, which is located 3 to 5 mm beneath the skin. A slight “pop” is usually felt as the needle passes through the fascial layer. Aspirate before injecting the lidocaine, because the dorsal arteries and veins are within close proximity to the nerve. Depending on the size of the child, between 1 and 5 mL of lidocaine should be used. Half of the volume is injected at the 10 o’ clock position, with the other half injected at the 2 o’ clock position. Another technique involves injecting only once at the midline through the Buck fascia, with injection of the full volume directed toward each direction after negative aspiration of blood. Like most nerve blocks, optimal analgesia is achieved after 5 minutes. Once analgesia is achieved either by dorsal nerve block or procedural sedation, manual reduction of the paraphimosis may be attempted. To decrease penile edema, it is often helpful to use a bag of ice (for 3-minute increments to avoid cold injury) or manual compression before FIGURE 136-3. Phimosis in a toddler leading to ballooning of foreskin caused by entrapped urine. FIGURE 136-4. A 12-year-old boy with paraphimosis for more than 24 hours. Tintinalli_Sec12_p0669-0996.indd 876 8/2/19 7:53 PM

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

l to use a bag of ice (for 3-minute increments to avoid cold injury) or manual compression before FIGURE 136-3. Phimosis in a toddler leading to ballooning of foreskin caused by entrapped urine. FIGURE 136-4. A 12-year-old boy with paraphimosis for more than 24 hours. Tintinalli_Sec12_p0669-0996.indd 876 8/2/19 7:53 PM CHAPTER 136: Pediatric Urologic and Gynecologic Disorders 877 attempting reduction. Squeezing the glans and swollen foreskin using one’s palm or a compression dressing for 5 minutes usually decreases the edema to allow successful manual reduction. The most common technique for manual reduction involves placing both thumbs over the glans, with both index fingers and long fingers surrounding the trapped foreskin. One pushes the glans back into the foreskin while pulling the foreskin back into normal position. This may require a few minutes of constant pressure before the glans slips through the paraphimotic ring (Figure 136-6). Manual reduction may fail if there is extreme swelling of the fore skin and glans from prolonged paraphimosis. Emergent urologic consultation is necessary for such cases. Although more invasive procedures are ideally done by a surgeon, the emergency physician may need to perform such procedures if necrosis is imminent. One commonly used technique involves using a 21-gauge needle to make multiple punctures in the foreskin followed by gentle compression, thus draining some of the edema. Manual reduction can then be attempted again. A dorsal slit procedure may be necessary if other attempts at reduction fail. This involves making a vertical incision over the constricting ring to release the paraphimosis. All cases of paraphimosis, whether simple or complicated, require follow-up with a urologist to assess healing and the need for circumcision.  BALANOPOSTHITIS Balanitis (cellulitis of the glans), posthitis (cellulitis of the foreskin), and balanoposthitis (cellulitis of the glans and foreskin) are common diagnoses in young males. Poor hygiene and phimosis predispose children to such infections ( Figure 136-7). On examination, the glans, the foreskin, or both the glans and foreskin are swollen, tender, and edematous. In most cases, empiric treatment with oral antibiotics with a first-generation cephalosporin and warm soaks are sufficient. In cases in which there is an associated erythematous papular rash with satellite lesions, antifungal cream may also be indicated.  PRIAPISM Priapism is a prolonged, unwanted erection not associated with sexual stimulation. Low-flow (venous) and high-flow (arterial) priapism are managed differently. High-flow (nonischemic) priapism is generally due to an arteriove nous fistula from trauma (i.e., lacerated cavernous artery shunting blood into the cavernous bodies). This can lead to a persistent partial or full erection for days to weeks but is generally not painful. Because of the continuous inflow of arterial blood, ischemia or impotence does not occur. Therefore, high-flow priapism is not a true urologic emergency. Most cases are treated conservatively, and only a few cases require angioembolization of the lacerated artery. FIGURE 136-5. Dorsal penile block. Local anesthesia infiltration around the base of the penis. Black dots represent the locations of the skin wheals. [Reproduced with permission from Reichman EF, Simon RR: Emergency Medicine Procedures, Copyright © 2004, The McGraw-Hill Companies, Inc. All rights reserved. Section 10. Genitourinary Procedures, Chapter 125, Anesthesia of the Penis, Testicle, and Epididymis, Figure 125-3.] FIGURE 136-7. A 4-year-old boy with posthitis and phimosis. FIGURE 136-6. Manual reduction of paraphimosis.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

ncy Medicine Procedures, Copyright © 2004, The McGraw-Hill Companies, Inc. All rights reserved. Section 10. Genitourinary Procedures, Chapter 125, Anesthesia of the Penis, Testicle, and Epididymis, Figure 125-3.] FIGURE 136-7. A 4-year-old boy with posthitis and phimosis. FIGURE 136-6. Manual reduction of paraphimosis. After appropriate analgesia is administered, place both thumbs over the glans, with both index and long fingers surround ing the trapped foreskin. The foreskin can then be reduced by placing pressure on the glans. [Reproduced with permission from Reichman EF, Simon RR: Emergency Medicine Procedures , Copyright © 2004, The McGraw-Hill Companies, Inc., Figure 129-4.] Tintinalli_Sec12_p0669-0996.indd 877 8/2/19 7:53 PM

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

long fingers surround ing the trapped foreskin. The foreskin can then be reduced by placing pressure on the glans. [Reproduced with permission from Reichman EF, Simon RR: Emergency Medicine Procedures , Copyright © 2004, The McGraw-Hill Companies, Inc., Figure 129-4.] Tintinalli_Sec12_p0669-0996.indd 877 8/2/19 7:53 PM 878 SECTION 12: Pediatrics Low-flow (ischemic) priapism is caused by sludging of red blood cells, leading to impaired venous drainage, venous congestion, and ischemia. In children, the most common cause of priapism is sickle cell disease. 17 Other less common causes in children include illicit drugs (cocaine and cannabis), antidepressants, antipsychotics, and leukemia (presenting with extreme hyperleukocytosis). 17 Low-flow priapism causes a very rigid and extremely painful erection. The type of priapism can usually be identified by history and physi cal examination. Doppler US can distinguish the type of priapism, with low-flow priapism showing decreased or no blood flow in the cavernosal arteries. The most reliable method, however, involves testing aspirated blood from the corpus cavernosum for blood gas analysis. Aspiration of the corpus cavernosum should be done only by an experienced urolo gist. Blood from low-flow priapism will be dark in color, with a partial pressure of oxygen (P o 2) <30 mm Hg, a partial pressure of carbon dioxide (Pco 2) >60 mm Hg, and a pH <7.25. Cavernous blood gas from high-flow priapism is bright red in color with numeric values similar to normal arterial blood. Without a history of pelvic, genital, or perineal trauma, nearly all priapism is low flow and usually secondary to sickle cell crisis. Priapism can occur in all forms of sickle cell disease, including sickle hemoglobin C and the sickle thalassemias. Priapism affects 30% of all males with sickle cell disease, 18 with most reporting repetitive painful episodes of prolonged erections.19 Such recurrent episodes are termed “stuttering” priapism and are unpredictable and of variable duration. Obtain a history including the duration of symptoms and any pre cipitating events (i.e., medications or illicit drugs). When low-flow priapism lasts for >4 hours, the risk for permanent damage leading to impotence is significant and requires emergency urology consultation. While waiting for the urologist, administer IV fluids, opioid analgesics, and supplemental oxygen, and maintain the patient as NPO (nothing by mouth) for possible procedural sedation or operative management. If sickle cell disease is the underlying cause, treat with IV venous hydration at 1.5 times maintenance rates and consider red blood cell exchange transfusion (see Chapter 143, “Sickle Cell Disease in Chil dren”). Prolonged priapism requires concurrent aggressive urologic management with corporeal aspiration and irrigation, intracavernous injection of a sympathomimetic drug (such as phenylephrine or epi nephrine), or, potentially, surgical shunting as a last resort. Ketamine is an established detumescent 17 and should be preferentially considered for patients requiring procedural sedation prior to corporeal aspiration or irrigation.  TOURNIQUET SYNDROME OF THE PENIS First reported in the literature in 1832, Reinisch and colleagues coined the term hair-thread tourniquet syndrome in 1988, when they described six cases of young infants with digit strangulation. 20 In a 2004 review, among the 90 cases of tourniquet syndrome found in the literature, toes were affected in 47%, penis in 25%, fingers in 20%, clitoris in 6%, and labia in 2%. Tourniquet syndrome of the penis presents with penile redness, swelling, and pain. Occasionally, the presenting sign is irritability of unknown cause, so a thorough history and physical are crucial to identify a tourniquet syndrome.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

es were affected in 47%, penis in 25%, fingers in 20%, clitoris in 6%, and labia in 2%. Tourniquet syndrome of the penis presents with penile redness, swelling, and pain. Occasionally, the presenting sign is irritability of unknown cause, so a thorough history and physical are crucial to identify a tourniquet syndrome. On physical examination, the area of the penis distal to the strangulation is erythematous, edematous, and tender. Edema often obscures the hair or thread itself. Treatment includes cutting the hair or thread if visualized, or using a depilatory agent, such as Nair ® . Depilatory creams will not work on synthetic fibers, however, and if unable to remove the constriction, urologic consultation is necessary. Damage from the tourniquet can range from mild penile edema, to glandular disfigurement, urethral transaction, and even penile amputation. 22 Although most cases are unintentional, they can also result from abuse.  ZIPPER INJURY Penile zipper entrapment is most often seen in school-age boys, most commonly when not wearing underpants. The patient’s shaft, foreskin, FIGURE 136-8. A 4-year-old boy with (A) penile zipper entrapment and (B) post-zipper removal after cutting the median bar. or glans becomes entrapped between the locked teeth of the zipper or within the fastener itself ( Figure 136-8). If the skin is trapped between the teeth of the zipper, cut the cloth between the locked teeth, and separate the teeth of the zipper. However, if the skin is caught within the fastener of the zipper, releasing the skin is more difficult. There are several methods described in the literature for zipper release. The most commonly used method is to use sturdy wire cutters or bone cutters to cut the median bar of the zipper (Figure 136-9). This requires a special tool, which may not be available, and in some cases, the angle of the zipper and type of zipper preclude easy access to the median bar. Other methods include using a mini hacksaw to cut the median bar, 23 dousing the area with liberal amounts of mineral oil and Tintinalli_Sec12_p0669-0996.indd 878 8/2/19 7:54 PM

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

pecial tool, which may not be available, and in some cases, the angle of the zipper and type of zipper preclude easy access to the median bar. Other methods include using a mini hacksaw to cut the median bar, 23 dousing the area with liberal amounts of mineral oil and Tintinalli_Sec12_p0669-0996.indd 878 8/2/19 7:54 PM CHAPTER 136: Pediatric Urologic and Gynecologic Disorders 879 then freeing the entrapped skin with gentle traction,24 or twisting a small flat-head screwdriver between the two faceplates of the zipper to widen the gap and allow release of the tissue.25 The age and degree of cooperativeness of the patient will determine type of analgesia and/or sedation needed. The patient may need oral analgesics, IV analgesics, or a dorsal penile block with or without pro cedural sedation. If all attempts fail at bedside zipper release, consult the urologist for removal under general anesthesia in the operating room. FEMALE GYNECOLOGIC PROBLEMS Postmenarchal and sexually active adolescent gynecologic problems are covered in Section 11, “Obstetrics and Gynecology. ” One of the most challenging physical examinations to perform is the gynecologic examination of the young female. Provide extra care and attention to create a nonthreatening environment to obtain a thorough, less anxietyprovoking examination. Tell the child that the parent approves the examination and that he or she will remain in the room. Tell the child why the examination is necessary and that everything will be explained to her beforehand. It is helpful to have the parent stand near the head of the bed while holding the child’s hand. Never forcibly restrain a child for a gynecologic examination. If the examination is difficult, procedural sedation or an examination under general anesthesia is an alternative approach. Regardless of practitioner gender, obtain third-party assistance (such as a nurse or social worker). The first position in which to examine the young female external genitalia is the frog-legged position ( Figure 136-10), with the child lying supine (or near-supine on a parent’s lap). Spread the child’s knees apart. Sometimes, this position can be aided with the soles of the feet brought together. The vestibule and hymen can be seen by gently pressing the labia majora laterally and posteriorly. Examine the child in the knee-chest position (Figure 136-11) for visualization of the perianal area and the outer vaginal vault. Ask the child to position herself on the examination table on her hands and knees, like a baby crawling. With her parent standing at the head of the bed, ask the child to put her head down onto her hands, with her elbows resting on the examination table. Apply gentle lateral and upward traction over the buttocks and labia majora to inspect the vaginal vault.  LABIAL ADHESIONS Labial adhesions (Figure 136-12) are fusions of the labia minora, most commonly seen in infant and preschool-aged girls. The exact cause is unknown, although they are thought to be related to a girl’s low level of estrogen, which predisposes the epithelium to irritation. Irritation may be due to poor hygiene, harsh soaps, bubble baths, or minor trauma. Re-epithelialization occurs as a response to irritation, forming the labial adhesions. The adhesions appear as a flat, connected surface, inferior to the cli toris, with a thin, vertical raphe. Adhesions may extend from the clitoris over the entire introitus, or they can be partial with some perforations. Labial adhesions are often asymptomatic and may be discovered on routine examination or when obtaining a catheterized urine specimen, although occasionally the child may complain of dysuria. Most labial adhesions resolve spontaneously during puberty, or topical estrogen cream can be applied over the area twice a day for 2 to 4 weeks.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

s are often asymptomatic and may be discovered on routine examination or when obtaining a catheterized urine specimen, although occasionally the child may complain of dysuria. Most labial adhesions resolve spontaneously during puberty, or topical estrogen cream can be applied over the area twice a day for 2 to 4 weeks. Topical estrogen may cause transient hyperpigmentation of the area, and pro longed use may induce reversible secondary sexual characteristics. Once Teeth Sliding piece Finger grip Front plate Median bar FIGURE 136-9. Parts of zipper are identified for penile zipper release. The most com mon method is to cut the median bar, thus releasing the entrapped skin. [Reproduced with permission from Reichman EF, Simon RR: Emergency Medicine Procedures, Copyright © 2004, McGraw-Hill, Inc., New York, Figure 131-1.] FIGURE 136-10. Girl lying in frog-legged position with knees spread apart and feet together. This position is best for examining the vestibule and hymen. FIGURE 136-11. Girl in knee-chest position with elbows resting on examination table. This position is best for examining the perianal area and the vaginal vault. Tintinalli_Sec12_p0669-0996.indd 879 8/2/19 7:55 PM

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

on with knees spread apart and feet together. This position is best for examining the vestibule and hymen. FIGURE 136-11. Girl in knee-chest position with elbows resting on examination table. This position is best for examining the perianal area and the vaginal vault. Tintinalli_Sec12_p0669-0996.indd 879 8/2/19 7:55 PM 880 SECTION 12: Pediatrics lysis of the adhesions occurs, the parent should apply petroleum jelly to the area for another 2 to 3 weeks to maintain labial separation. Do not manually separate adhesions, as they will likely recur.  VAGINAL DISCHARGE During the first 2 to 3 weeks of life, many infant girls have normal physiologic vaginal discharge. The leukorrhea is thin, slippery, and clear or white in color. Among older infants and young girls, there are two common causes of vaginal discharge: vaginal foreign body and vulvovaginitis. Also, consider sexual abuse with infection from N. gonorrhoeae , C. trachomatis, or Trichomonas as a possibility. Vaginal foreign bodies present with foul-smelling vaginal discharge, which can be slightly bloody. The patient may complain of voiding symptoms secondary to local irritation. Symptoms are often present for a long period of time before presentation to a physician. The most common vaginal foreign body in prepubertal females is toilet paper. Examination of the patient in knee-chest position (see Figure 136-11) is the best method to visualize the vaginal vault and possible foreign body. If the foreign body is readily seen, it can be removed using forceps or warm water vaginal lavage. If bedside removal is not possible, the patient may require examination and removal under procedural sedation or general anesthesia. Vulvovaginitis is a very common cause of vaginal discharge, pain, and pruritus. Most cases are not associated with any specific organism, but irritation is secondary to poor hygiene, wiping back to front after urination or defecation, bubble baths, tight clothing or underwear, or perfumed bathing products. Treatment for such cases includes proper hygiene habits and eliminating offending agents. Among bacterial causes of vaginitis in the prepubertal female, the most common are group A β-hemolytic Streptococcus, Staphylococcus aureus, Escherichia coli, and Shigella. Candida vaginitis is not common among prepubertal females because of the alkaline pH of the vagina. Vaginal bleeding is another common complaint in the pediatric ED. In the first 2 to 3 weeks of life, as maternal hormonal levels wane, the newborn female may experience sloughing of her endometrium with subsequent vaginal bleeding. This form of nonpathologic bleeding is always self-limited and requires no treatment other than reassurance to the parents. In children, urethral prolapse occurs more commonly in prepubertal black females between the ages of 2 and 10 years old. The complaint is usually painless blood spotting on the underwear, although some patients may also experience mild irritation with voiding. The mucosa of the distal urethra prolapses outward beyond the meatus, causing venous congestion of the prolapsed tissue. Prolapse appears as a red-purple, doughnut-shaped mass with a central dimple. If the diagnosis is in doubt, pass a urinary catheter through the central opening for confirmation. The treatment of choice is sitz baths and topical estrogen cream for 2 weeks. Constipation may exacerbate the prolapse if the child strains with defecation, so providing a stool softener is often helpful. If con servative medical management fails, or if the prolapsed tissue becomes necrotic, the tissue may have to be surgically reduced and/or removed. Fortunately, most straddle injuries cause only minor superficial abrasions, lacerations, or hematomas of the perineum. Such cases can be treated with supportive care and sitz baths.

contenttextbook· 136 Pediatric Urologic and Gynecologic Disorders· item 137· p.919–925

vative medical management fails, or if the prolapsed tissue becomes necrotic, the tissue may have to be surgically reduced and/or removed. Fortunately, most straddle injuries cause only minor superficial abrasions, lacerations, or hematomas of the perineum. Such cases can be treated with supportive care and sitz baths. Pain with voiding can be relieved by allowing the child to urinate in a bathtub of warm water. More significant injuries, such as an expanding hematoma, a laceration beyond the superficial layers, a wound that continues to actively bleed, any rectal bleeding, or inability to urinate, require immediate gyneco logic evaluation. In addition, sexual abuse or assault should always be considered as a possible cause of genital injury and should be reported to the appropriate state agency as mandated by law. The vast majority of vaginal bleeding in adolescents is caused by dysfunctional uterine bleeding. Irregular menses with or without prolonged bleeding is especially common in females during the first year after menarche, due to the high number of anovulatory cycles. Dysfunctional uterine bleeding is best managed initially with a trial of a combined estrogen-progestin pill. The estrogen stops the bleeding, and the progestin stabilizes the endometrium. There are multiple forms of oral contraceptive pills that would be appropriate as first-line therapy. For further discussion, see Chapter 96, “ Abnormal Uterine Bleeding. ”  IMPERFORATE HYMEN Cases of imperforate hymen ( Figure 136-13) typically present to the ED as a teenage female with chronic, vague abdominal pain, who has secondary sexual characteristics, yet is still “premenarchal. ” Physical examination reveals a bluish bulging membrane covering the introi tus, representing accumulated menstrual blood and hematocolpos. If the hematocolpos is large, the patient may have symptoms of urinary urgency, frequency, or dysuria. Treatment in adolescents is urgent surgical repair, but in asymptomatic infants and young girls, surgery is performed on an elective basis. REFERENCES The complete reference list is available online at www.TintinalliEM.com. FIGURE 136-13. Teenage female with imperforate hymen (with Foley catheter in place). FIGURE 136-12. Eight-month-old girl who was noted to have labial adhesions when a catheterized urine specimen was ordered in the ED for a fever workup. Tintinalli_Sec12_p0669-0996.indd 880 8/2/19 7:56 PM