Urolithiasis. Syndrome of swollen scrotum. Pavlo Hoschynsky

Urolithiasis Introduction Urolithiasis is increasingly recognized in pediatric patients and is encountered in a variety of clinical settings. The wide geographic variation in the incidence of urolithiasis in childhood is related to climatic, dietary, and socioeconomic factors. Approximately 7% of urinary calculi occur in children younger than 16 years of age. Many children with stone disease have a metabolic abnormality. Revolutionary advances in the minimally invasive and noninvasive management of stone disease over the past 2 decades have greatly facilitated the ease with which stones are removed. Given the frequency with which stones recur, the development of a medical prophylactic program to prevent stone recurrences is desirable. The lifetime prevalence of kidney stone disease is estimated at 1% to 15%, with the probability of having a stone varying according to age, gender, race, and geographic location. Stone disease typically affects boys more co­mmonly as much as two to three times more frequently than females. Upper urinary tract stones occur more commonly in boys than girls by a ratio of 1.4:1 to 2.1:1.

Classification of stones Stone size: <5 mm, 5-10 mm, > 10-20 mm, > 20 mm.

Classification of stones Stone location: upper calyx, middle calyx or lower calyx, renal pelvis, upper ureter, middle ureter or distal ureter, urinary bladder.

Location of Renal stones

Classification of stones X-ray characteristics Radiopaque Poor radiopaque Radiolucent Calcium oxalate dehydrate Magnesium ammonium phosphate Uric acid Calcium oxalate monohydrate Apatite Ammonium urate Calcium phosphates Cystine Xanthine   2,8-dihydroxyadenine 'Drug-stones'

Stones classified according to their aetiology Non-infection stones Calcium oxalates Calcium phosphates Uric acid Infection stones Magnesium-ammonium-phosphate Apatite Ammonium urate Genetic causes Cystine Xanthine 2,8-dihydroxyadenine 'Drug stones'

Calcium oxalate monohydrates Calcium oxalate dihydrates

Uric acid

Struvite

Cystine

High risk stone formers General factors Early onset of urolithiasis in life (especially children and teenagers) Familial stone formation Brushite containing stones (calcium hydrogen phosphate; CaHP04. 2H20) Uric acid and urate containing stones Infection stones Solitary kidney (The solitary kidney itself does not have a particular increased risk of stone formation, but the prevention of a potential stone recurrence is of more importance)

High risk stone formers Diseases associated with stone formation Hyperparathyroidism Nephrocalcinosis Gastrointestinal diseases or disorders (i.e. jejuno-ileal bypass, intestinal resection, Crohn's disease,malabsorptive conditions) Sarcoidosis

High risk stone formers Genetically determined stone formation Cystinuria (type A, B, AB) Primary hyperoxaluria (PH) Renal tubular acidosis (RTA) type 1 2,8-dihydroxyadenine Xanthinuria Lesh-Nyhan-Syndrome Cystic fibrosis

High risk stone formers Anatomical and urodynamic abnormalities associated with stone formation Medullary sponge kidney (tubular ectasia) Ureteropelvic junction (UPJ) obstruction Calyceal diverticulum, calyceal cyst Ureteral stricture Vesico-uretero-renal reflux Horseshoe kidney Ureterocele Urinary diversion (via enteric hyperoxaluria) Neurogenic bladder dysfunction

Compounds that cause drug stones Active compounds crystallizing in urine • Allopurinol / oxypurinol • Amoxicillin / ampicillin • Ceftriaxone • Ciprofloxacin • Ephedrine • Indinavir • Magnesium trisilicate • Sulfonamide • Triamterene Substances impairing urine composition • Acetazolamide • Allopurinol • Aluminium magnesium hydroxide • Ascorbic acid • Calcium • Furosemide • Laxatives • Methoxyflurane • Vitamin D

Diagnostic steps in urolithiasis (UTI urinary tract infection, CT computed tomography, MR1 magnetic resonance imaging, PTH parathyroid hormone, pC02 partial pressure of carbon dioxide)

Fig. a,b. A 17-year-old girl with cystinuria Fig.a,b. A 17-year-old girl with cystinuria. a) Abdominal plain radiograph showing urolithiasis on the left, b) IVU showing hydronephrosis on the left due to urolithiasis

Fig. a,b. A 4-year-old boy with incomplete RTA and hyperoxaluria, a Sonogram of right kidney showing medullary nephrocalcinosis grade III (Dick et al. 1999). b Sonogram of bladder showing an ureteral stone on the right immediately before the ureterovesical junction

An 8-year-old boy with primary hyperparathy­roidism, hypercalciuria, and urinary tract infection. Ab­dominal plain radiograph showing a huge ureteral stone on the left immediately before the ureterovesical junction

Bilateral Ureteric Calculus in a patient presenting with Anuria Helical or Spiral CT provides 3D reconstruction. Helical refers to path the X ray follows on Gantry. These are rapidly performed and do not require contrast agents for reconstruction.

Evaluation for a suspected stone. (RBUS)-renal/bladder ultrasound

extracorporeal shockwave lithotripsy (ESWL) percutaneous nephrolithotomy -(PCNL)

Recommendations for pain relief during renal colic: -1st choice: treatment should be started with an NSAID(Diclophenac sodium, Indomethacin, Ibuprofen) -2nd choice: Hydromorphine(Pentazocine,Tramadol) -Diclofenac sodium is recommended to counteract recurrent pain after an episode of ureteral colic For septic patients with obstructing stones, the collecting system should be urgently decompressed, using either percutaneous drainage or ureteral stenting. Definitive treatment of the stone should be delayed until sepsis is resolved. Medical expulsive therapy Alpha-blockers (Tamsulosin, 0.4 mg, doxazosin,terazosin, alfuzosin and naftopidil) Calcium-channel blockers(nifedipine) Corticosteroids Chemolytic dissolution of stones: -Percutaneous irrigation chemolysis -Oral Chemolysis

Methods of percutaneous irrigation chemolysis Stone composition Refs. Irrigation solution Comments Struvite Carbon apatite 1-6 10% Hemiacidrin with pH 3.5-4 Suby's G Combination with Shockwave lithotripsy for staghorn stones Risk of cardiac arrest due to hypermagnesaemia Brushite 7 Hemiacidrin Suby's G Can be considered for residual fragments Cystine 8-13 Trihydroxymethyl- aminomethan (THAM; 0.3 or 0.6 mol/L) with pH range 8.5-9.0 N-acetylcysteine (200 mg/L) Takes significantly longer time than for uric acid stones Used for elimination of residual fragments Uric acid 10,14-18 Oral chemolysis is the preferred option

The figure shows a 12 month-old child treated with the Modulith SLK (Storz Medical AG, Kreuzlingen).

Operation: percutaneous nephrolithotomy ■ Rarely used in pediatric surgery ■ Utilize a nephroscope or ureteroscope ■ Extract with visualization ■ Break larger stones using ultrasonography Operation: open stone removal ■ Rarely necessary, only when urinary calculi are not amenable to ESWL or PL ■ Make an incision below the 12th rib ■ Expose the kidney and the ureter ■ Open the renal pelvis and extract the stone (or ureter in the case of a ureteral stone) ■ Wash the entire calyx system ■ Suture the pyelon or the ureter Postoperative care ■ Ureter drain for 2–5 days with an antegrade contrast X-ray before drain removal ■ Antibiotic therapy as prophylaxis in cases of vesicoureteral reflux ■ Urine culture once a month ■ Ultrasonography Prognosis ■ Stone recurrence is rare if urine is sterile and an obstruction does not occur

Medical treatment of recurrent stones

Scrotal Pain and Swelling Outline Embryology and anatomy Causes of Pain and Swelling Torsion, Epididymitis, Orchitis, Trauma History, Physical, Radiologic Exams, Labs Causes of Swelling Hydrocele, Varicocele, Spermatocele, Tumor, Idiopathic

Embryology Descent of testes at 32-40 wks gestation Descends within processes vaginalis Outpouching of peritoneal cavity Tunica vaginalis is potential space that remains after closure of process vaginalis

Anatomy Spermatic cord –testicular vessels, lymph, vas deferens Epididymis - sperm formed in testicle and undergo maturation, stored in lower portion Vas Deferens – muscular action propels sperm up and out during ejaculation Gubernaculum – fixation point for testicle to tunica vaginalis Tunica Vaginalis – potential space Encompasses anterior 2/3’s of testicle Tunica albuginea is inner layer opposing testis

Anatomy – Nuts and Bolts Posterior Anterior

Causes of Pain and Swelling Testicular torsion Torsion of appendix testis Epididymitis Trauma Orchitis and Others Swelling Hydrocele Varicocele Spermatocele Tumor

Torsion Inadequate fixation of testes to tunica vagnialis at gubernaculum Torsion around spermatic cord Venous compression to edema to ischemia

Epidemiology Accounts for 30% of all acute scrotal swelling Bimodal ages – neonatal (in utero) and pubertal ages 65% occur in ages 12-18yo Incidence 1 in 4000 in males <25yo Increased incidence in puberty due to inc weight of testes

Predisposing Anatomy Bell-clapper deformity Testicle lacks normal attachment at vaginalis Increased mobility Tranverse lie of testes Typically bilateral Prevalence 1/125

Torsion: Clinical Presentation Abrupt onset of pain – usually testicular, can be lower abdominal, inguinal Often < 12 hrs duration May follow exercise or minor trauma May awaken from sleep Cremasteric contraction with nocturnal stimulation in REM Up to 8% report testicular pain in past

Torsion: Examination Edematous, tender, swollen Elevated from shortened spermatic cord Horizontal lie common (PPV 80%) Reactive hydrocele may be present Cremasteric reflex absent in nearly all (unreliable in <30mo old) (PPV 95%) Prehn’s sign elevation relieves pain in epididymitis and not torsion is unreliable

Intermittent Torsion Intermittent pain/swelling with rapid resolution (seconds to minutes) Long intervals between symptoms PE: testes with horizontal lie, mobile testes, bulkiness of spermatic cord (resolving edema) Often evaluation is normal – if suspicious need GU followup

Diagnosis – “Time is Testicle” Ideally -- prompt clinical diagnosis Imaging Color doppler – decreased intratesticular flow False + in large hydrocele, hematoma Sens 69-100% and Spec 77-100% Lower sensitivity in low flow pre-pubertal testes Nuclear Technetium-99 radioisotope scan Show testicular perfusion 30 min procedure time Sens and spec 97-100%

but dec flow w/in testis Acute torsion L testis Dec blood flow on L Late torsion on R Inc blood flow around but dec flow w/in testis

Images - Torsion Decreased echogenicity and size of right testicle Nuclear medicine scan shows "rim sign“ =no flow to testicle and swelling

Management Detorsion within 6hr = 100% viability Within 12-24 hrs = 20% viability After 24 hrs = 0% viability Surgical detorsion and orchiopexy if viable Contralateral exploration and fixation if bell-clapper deformity Orchiectomy if non-viable testicle Never delay surgery on assumption of nonviability as prolonged symptoms can represent periods of intermittent torsion

Intravaginal torsion with ischemia in a adolescent boy.

Manual Detorsion If presents before swelling Appropriate sedation In 2/3rds of cases testes torses medially, 1/3rd lateral Success if pain relief, testes lowers in scrotum Still need surgical fixation

Torsion: Special Considerations Adolescents may be embarrassed and not seek care until late in course Torsion 10x more likely in undescended testicle Suspicious if empty scrotum, inguinal pain/swelling Adult Emergency Physicians accurate in bedside US diagnoses with sens of 95% and specificity of 94% (missed 1 epididymitis, no torsion) Blavis M., Emergency Evaluation of Patients Presenting with A Cute Scrotum, Academy of Emergency Medicine. Jan 2001

Neonatal Torsion 70% prenatal, 30% post-natal Post-natal typically 7-10 days after birth Unrelated to gestation age, birth weight Post-natal presents in typical fashion Doppler U/S and radionucleotide scans less accurate with low blood flow in neonates Surgical intervention if post-natal Prenatal torsion presents with painless testicular swelling, rare testicular viability Rare intervention in prenatal torsion

Perinatal torsion

Torsion of Appendix Testis Small vestigial structure, remnant of Mullerium duct Pedunculated, 0.3cm long Other appendix structures Prepubertal estrogen may enlarge appendix and cause torsion

Torsion of Appendix Testis Peak age 3-13 yo (prepubertal) Sudden onset, pain less severe Classically, pain more often in abd or groin Non-tender testicle Tender mass at superior or inferior pole May be gangrenous, “blue-dot” (21% of cases) Normal cremasteric reflex, may have hydrocele Inc or normal flow by doppler U/S

Torsion of Appendix Testis Blue dot of gangrenous appendix testis

Testicular Appendages Appendix testis Appendix epididymis

Torsion of Appendix Testis Management supportive analgesics, scrotal support to relieve swelling Surgery for persistent pain no need for contralateral exploration

Epididymitis Inflammation of epididymis Subacute onset pain, swelling localized to epididymis, duration of days With time swelling and pain less localized Testis has normal vertical lie Systemic signs of infection inc WBC and CRP, fever + in 95% Cremasteric reflex preserved Urinary complaints: discharge/dysuria PPV 80%

Epididymitis Scrotum has overlying erythema, edema in 60% Normal vertical lie

Epididymitis Sexually active males Chlamydia > N. gonorrhea > E. coli Less commonly pseudomonas (elderly) and tuberculosis (renal TB) Young boys, adolescents often post-infectious (adenovirus) or anatomic Reflux of sterile urine through vas into epididymis 50-75% of prepubertal boys have anatomic cause by imaging

Etiologies of Epididymitis

Epididymitis Diagnosis Leukocytosis on UA in ~40% of patients PCR Chlamydia + in 50%, GC + in 20% of sexually active 95% febrile at presentation Doppler and Nuclear imaging show increased flow If hx consistent with STD, CDC recommends: Cx of urethral discharge, PCR for C and G Urine culture and UA Syphilis and HIV testing

Laboratory Adjuncts Studies of acute phase reactants: CRP, IL-1, IL-6 Documented epididymitis have 4 fold increase in CRP compared to testicular torsion PPV 94% and NPV 94% (inc 2 fold) Testicular tumor showed no increase in CRP Doehn C., Value of Acute Phase Proteins in the Differential Diagnosis of A Cute Scrotum, Journal of Urology. Feb 2001.

Doppler Epididymitis Left Epididymitis Inc blood flow in and around left testis

Epididymitis Treatment Sexually active treat with Ceftriaxone/Doxycycline or Ofloxacin Pre-pubertal boys Treat for co-existing UTI if present Symptomatic tx with NASIDs, rest Referral all to GU for studies to rule out VUR, post urethral valves, duplications Negative culture has 100% NPV for anomaly

Orchitis Inflammation/infection of testicle Spread from epididymitis, Swelling pain tenderness, erythema and shininess to overlying skin Spread from epididymitis, hematogenous, post-viral Viral: Mumps, coxsackie, echovirus, parvovirus Bacterial: Brucellosis

Mumps Orchitis Extremely rare if vaccinated 20-30% of pts with mumps, 70% unilateral, rare before puberty Presents 4-6 days after mumps parotitis Impaired fertility in 15%, inc risk if bilateral

Trauma Result of testicular compression against the pubis bone, from direct blow, or straddle injuries Extent depends on location of rupture Tunica albuginea ruptures (inner layer of tuncia vaginalis) allows intratesticular hematoma to rupture into hematocele Rupture of tunica vaginalis allow blood to collect under scrotal wall causing scrotal hematoma Doppler often sufficient to assess extent Surgery for uncertain dx, tunica albuginea rupture, compromised doppler flow

Testicular Hematoma Blood as a filling defect in testis

Other Causes of Pain Incarcerated inguinal hernia Henoch-Schonlein Purpura Vasculitis of testicular vessels Rarely presents with only scrotal pain Referred pain Retrocecal appendix, urolithiasis, lumbar/sacral nerve injury Non specific scrotal pain Minimal pain, nl exam – return immediately for inc symptoms

Scrotal Swelling Hydrocele Varicocele Spermatocele Testicular Cancer

Hydrocele Fluid accumulation in potential space of tunica vaginalis May be primary from patent PV or secondary to torsion/epididymitis

Hydrocele Transilluminating anterior cystic mass

Hydrocele Mass increases in size during day or with crying and decreases at night if communicating If non-communicating and <1 yo follow If communicating (enlarging), scrotum tense (may impair blood flow) requires repair Unlikely to close spontaneously and predisposes to hernia

Varicocele Collection dilated veins in pampiniform plexus surrounding spermatic cord More common on left side R vein direct to IVC L vein acute angle to renal vein ~20% of all adolescent males

Varicocele Often asymptomatic or c/o dull ache/fullness upon standing Spermatic cord has ‘bag of worms’ appearance that increased with standing/valsalva If prepubertal, rapidly enlarging, or persists in supine position rule out IVC obstruction Most management conservatively Surgery if affected testis < unaffected testis volume

Spermatocele Painless sperm containing cyst of testis, epipdidymis Distinct mass from testis on exam Transilluminates Do not affect fertility Surgery for pain relief only

Testicular Cancer Painless mass Most common solid tumor in 15-30 yo males 20% of all cancers in this group Painless mass Rapidly growing germ cell tumors may cause hemorrhage and infarction Present as firm mass Typically do not transilluminate Diagnostic imaging with U/S initially

Acute Idiopathic Scrotal Edema Scrotal skin red and tender underlying testis normal no hydrocele Erythema extends off scrotum onto perineum Empiric tx, cause unknown Antihistamine, steroids Resolves w/in 48-72hrs

Conclusions Clinical history and careful exam are key factors in formulating accurate differential Imaging and labs useful adjuncts in unclear cases U/S superior to nuclear imaging if time essential TIME IS TESTICLE Early surgical intervention and GU involvement Swelling without pain, usually less time sensitive diagnostically

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References Ciftci, AO. Clinical Predictors for Diff. Diagnosis of Acute Scrotum, European J. of Ped. Surgery. Oct 2004. Blavis M., Emergency Evaluation of Patients Presenting with Acute Scrotum, Academy of Emergency Medicine. Jan 2001 Doehn C., Value of Acute Phase Proteins in the Differential Diagnosis of Acute Scrotum, Journal of Urology. Feb 2001. Kaplan G., Scrotal Swelling in Children. Pediatrics in Review. Sep 2000. Luzzi GA. Acute Epididymitis. BJU International. May 2001. Fleisher G, Ludwig S, Henretig F. Textbook of Pediatric Emergency Medicine. 2006.