Urolithiasis. Syndrome of swollen scrotum

Urolithiasis. Syndrome of swollen scrotum.Pavlo Hoschynsky

Urolithiasis IntroductionUrolithiasis 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 co mmonly 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 stonesCalcium oxalatesCalcium phosphatesUric acidInfection stonesMagnesium-ammonium-phosphateApatiteAmmonium urateGenetic causesCystineXanthine2,8-dihydroxyadenine'Drug stones'

Calcium oxalate monohydrates

Calcium oxalate dihydrates

Uric acid

Struvite

Cystine

High risk stone formersGeneral 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 theprevention 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 stonesActive 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. 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 hyperparathy roidism, hypercalciuria, and urinary tract infection. Ab dominal plain radiograph showing a huge ureteral stone on the left immediately before the ureterovesical junction

Bilateral Ureteric Calculus in a patient presenting with Anuria 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 ofureteral 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 therapyAlpha-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 CommentsStruviteCarbon apatite

1-6 10% Hemiacidrin with pH 3.5-4Suby's G

Combination with Shockwavelithotripsy for staghorn stonesRisk of cardiac arrest due tohypermagnesaemia

Brushite 7 HemiacidrinSuby's G

Can be considered forresidual fragments

Cystine 8-13 Trihydroxymethyl- aminomethan(THAM; 0.3 or 0.6 mol/L) with pH range8.5-9.0N-acetylcysteine (200 mg/L)

Takes significantly longertime than for uric acid stonesUsed for elimination ofresidual fragments

Uric acid 10,14-18 Trihydroxymethyl- aminomethan(THAM; 0.3 or 0.6 mol/L) with pH range8.5-9.0

Oral chemolysis is thepreferred 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 ultrasonographyOperation: open stone removal■ Rarely necessary, only when urinary calculi are not amenable to ESWLor 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 aureteral stone)■ Wash the entire calyx system■ Suture the pyelon or the ureterPostoperative care■ Ureter drain for 2–5 days with an antegrade contrast X-ray before drainremoval■ Antibiotic therapy as prophylaxis in cases of vesicoureteral reflux■ Urine culture once a month■ UltrasonographyPrognosis■ Stone recurrence is rare if urine is sterile and an obstruction does notoccur

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

AnteriorPosterior

Causes of Pain and Swelling

Pain 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 deformityTesticle lacks normal

attachment at vaginalis Increased mobilityTranverse lie of testesTypically bilateralPrevalence 1/125

Torsion: Clinical Presentation

Abrupt onset of pain – usually testicular, can be lower abdominal, inguinalOften < 12 hrs durationMay follow exercise or minor traumaMay 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%

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

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 AppendagesAppendix 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 malesChlamydia > 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 boysTreat for co-existing UTI if presentSymptomatic tx with NASIDs, restReferral 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 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

Most common solid tumor in 15-30 yo males20% of all cancers in this group

Painless massRapidly growing germ cell tumors may cause

hemorrhage and infarctionPresent as firm massTypically 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

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.