Part 24. Urologic disorders in infants and childhood

넬슨 정리

Part XXIV. Urologic Disorders In Infants And Children

Chapter 491. Congenital Anomalies & Dysgenesis Of The Kidney

; *생후 1 voiding 없으면 underlying anatomic abnormality 검사

; 출생시 urine

 1) pH 57

 2) osmolality : 60600mOsm/Kg (mmol/Kg) H2O

 3) many epithelial cell

 4) RBC : 간혹 존재

 5) *WBC :

 6) culture : sterile

 7) glucose & protein : trace amount

Renal agenesis(aplasia)

Bilateral Agenesis

; incidence : 1/,3000

; 결과 : stillborn fetus

# 96 Potter phenotype

    ; oligohydramnios 의해 발생하는 특징적인 임상증상

           - wide separated eyes

           - epicanthic folds

           - receding chin

           - limb anomalies

           - hypoplastic lungs

    ; causes

           - renal agenesis, cystic renal dysplasia, obstructive uropathy, infantile polycystic kidney, renal hypoplasia, medullary dysplasia

# *DDx of agnesis & aplasia

    ; aplasia

           - extreme form of dysplasia

           - *presence of nonfunctional tissue capping a normal or abnormal ureter

2) hereditary renal dysplasia

    : autosomal dominant

     5090% penetrance

     다양한 expression

     anorectal, cariovascular, skeletal abnormality

 4. 진단

  1) maternal ultrasound (2nd trimester)

     : oligohyramnios

       bladder nonvisualization

       absent kidney

 5. 1개월 death 원인

    : uremia or pulmonary insufficiency

Unilateral Renal Agenesis

; 다른 검사도증 우연히 발견되는 경우가 대부분

; *single umbilical artery 있을 반드시 의심

; absence of ureter, ipsilateral bladder, hemitrigone

contralateral kidney : 출생후 compensatory hypertrophy

associated anomaly

1) genitourinary aystem : 40%

2) skeletal system : 30%

3) cardiovascular system & gastrointestinal system : 각각 15%

4) CNS & respiratory system : 각각 10%

incidence

1) 1/1,000 live births

; *male > female

; *left > right

Mayer-Rokitansky Syndrome

    ; unilateral renal agenesis & vaginal atresia or agenesis

vas defens agenesis, seminal vesicle cyst

Anomalies In Shape And Position

ascent & rotation 불완전  Fig 491-2

  renal ectopia or nonrotation 발생

# Horseshoe Kidney

    ; 신장의 lower pole 중심선에서 융합

    ; incidence - 1/500 birth

    ; *Turner syndrome 7%

    ; *Wilm`s tumor 발생빈도가 28 높다

Hypoplasia

definition : nephron수가 감소한 small kidney

; *not inherited

unilateral involvement

1) segmental hypoplasia or Ask-upmark kidney : 일부 involve

2) entire involvement

3) 10 미만에서 hypertension유발하는 more common cause

bilateral involvement

1) chronic renal failure 증상 가짐

2) 10 미만에서 ESRF 주원인

3) polyuria & polydypsia : common

4) oligomeganephronia(rare form) : nephron수의 현저한 감소 & 현저한 hypertrophy

Dysplasia

1. definition

    : fetal kidney structural diffentiation 상당한 변화로 cyst, abnormal duct,

     undefferentiated mesenchyme, or cartilage같은 nonrenal element 존재

 2. 원인

  1) urinary tract intrauterine obstruction

     : prune-belly syndrome, ureterocele, urethral valve, ureteropelvic junction

      보통 bilateral & ESRF 발생 가능

  2) multicystic dysplasia

   unilateral or bilateral

   lower tract developmental anomaly or contralateral normal kidney 관련

   unilateral form

      : nonfunctioning flank mass

        renal tissue 거의 없거나 전혀 없는 cystic mass

           hypertension 생기면 제거

   bilateral form

     : chronic renal failure 관련 심한 경우 Potter syndrome 보임

CHAPTER 492. Urinary Tract Infections

Prevalence And Etiology

# newborn symptomatic UTI

    ; 1.4/1,000

# Risk Factors

    ; variable markedly with age & sex

    ; *uncircumcised male infant(<1yr)

    ; *female(>1yr)

    ; 7-11yr age

           - *common age group (2.5%)

    ; sexually active female : cystitis 위험 증가

    ; sexually active adolescent male & female : urethritis 발생 가능

# Pathogens

    ; mostly colonic bacteria

    ; female

           - E. coli(7590%) > Klebsiellla > Proteus

    ; male over 1yr

           - *Proteus=E. coli in some report

    - *G (+) cocci in another report

    ; *Staphylococcus saprophyticus

           - both sex

Pathogenesis And Pathology

; *in newborn

    - *bloodstream or urethra통해 침범

  - *이후 : ascending infection

UTI susceptibility 관여하는 factor

1) host factor : urethra cervical Ab (Ig A)

2) bacterial adherence 관여하는 factor : P blood grup phenotype

3) immunosuppression, diabetes, urinary tract obstruction,

   chronic granulomatous disease 증가

infection severity 관여하는 factor

1) bacteria virulence

2) anatomic factors

   : ·vesicoureteral reflux

        + vesical overdistention

       -+ bladder wall blood flow 감소

        + infection 대한 bladder natural resistance 감소

     ·obstruction

     ·urinary stasis : bacterial multiplication

     ·prisence of calculi

Acute Bacterial Cystitis

 1. 특징

    : mucosal congestion, edema petechiae, hemorrhage

      detrusor muscle hyperactivity

      bladder functional capacity 감소 vesicoureteral reflux

 2. cystitis cystica : chronic or recurrent infection 있는 경우 발생

Acute Pyelonephritis

# P1 blood group

    ; VUR 없어도 ascending recurrent pyelonephritis

    ; *E. coli - specific binding to P1 antigen of epithelial cell

medulla pelvis acute inflammatory cell infiltration enlarged kidney

  microabscess 형성 renal scar 형성 : chronic pyelonephritis

 obstruction있으면 severe

 즉각적인 치료 : 완전 healing

Chronic Pyelonephritis

; difficult to DDx

    - medullary cystic disease, ischemia, irradiation, analgesic abuse

* characteristic finding ; cortical scar with underlying calyceal deformity

; LM

    - pathy with glomerular fibrosis

    - interstitial chronic inflammation

  - tubule fibrosis & atrophy

; *cortex 보다 medulla susceptible

    - high osmolality

           --> leukocyte phagocytosis 감소

# reflux nephropathy

    ; renal scars in VUR children

    ; no history of UTI

    ; *flat papillae에서 발생

    ; *autoimmune response to Tamm-Horsfall protein

           --> play a role in development & progression of pyelonephritic scars

; *90% of chronic pyelonephritis

    - *VUR

reflux nephropathy or chronic pyelonephritis

    ; cause of arterial hypertension in children

Urea-Spliting Organism : Proteus

; *stone formation

    - urea로부터 ammonia생성 alkaline urine 형성 calcium phosphate, triple calcium, magnesium, ammonium phosphate precipitation가능 calculi 형성 foreign body 작용 infection

ureteral obstruction시의 infection

  : septicemia, pyelonephrois, renal & perirenal abscess formation

Xanthogranulomatous Pyelonephritis

1. 정의 : giant cell foamy histiocyte 있는 granulomatous inflammation

2. renal mass, acute or chronic infection 유발

3. predisposing factor

   : renal calculi, obstruction, Proteus or E. coli infection

4. treatment : nephrectomy

Clinical Manifestation

; asymptomatic bacteriuria

    - mostly

# urinary tract infection symptom

    1) infancy

         ; *fever, weight loss, failure to thrive, N/V, diarrhea, jaundice

    2) children

       ; urinary frequency, pain durine micturition, urinary incontinence asso. /c urgency, bedwetting in a previously dry child, abdominal pain, foul smelling urine

# chronic or frequently recurrent cystitis

    ; daytime incontinence, bladder instability 증상

# *hematuria

    ; *E. coli 의한 hemorrhagic cystitis

# acute pyelonephritis

    ; *fever, chill, flank or abdominal pain, tenderness, enlarged kidney

# chronic pyelonephritis

    ; asymptomatic

    ; renal scar : arterial hypertension

# reflux nephropathy

  ; 15% of ESRF in childhood

# sepsis

    ; common in infecion & urinary tract obstruction

# *hyperammonemia wih CNS symptom

    ; *Proteus UTI and urinary stasis & obstruction

Laboratory Data

# culture

    1) toilet trained children ; midstream urine culture

         의의 : single organism 105 colonies/mL

           UTI 90% 이상의 specificity가짐

         87,94 low colony나오는 경우

                   ; overhydration, recent bladder emptying, antibiotic therapy

    2) adhesive, sealed, sterile collection bag

    3) catheterized specimen

         infant : 5F polyethylene feeding tube

         older children : 8F polyethylene feeding tube

    4) suprapubic puncture

         full bladder 만들고 실시

         25 or 22 gauge needle 사용

         pubis midline에서 1 finger-breadth 상방, 1020° 각도

         87,94 false negative culture

                   ; unrecongnized antibiotic therapy, overhydration 의한 dilution, antiseptic solution contamination

# urinalysis

    1) pyuria : 농뇨 없이도 infection recur가능

    2) microscopic hematuria : acute cystitis

    3) urinary casts : renal involvement

    4) alkaline pH : Proteus infection

# upper & lower UTI 감별

    ; acute renal infection

           - leukocytosis, neutorphilia, ESR & CRP 증가

           - Ab-coated bacteria detection

           - single dose antibiotic therapy 대한 반응

           - 다른 immunologic or biochemical test

                 / urine 농축하지 못하는 경우

                   / 30%에서 일시적인 serum creatinine 증가

                   / sepsis 때문에 febrile infection blood culture 필요

    ; ♥표17-12(p722)

Imaging Studies

# Renal Ultrasonography

    ; *Indication

           acute febrile infection hydronephrosis renal, perirenal abscess 감별

           antibiotic therapy 대한 반응이 즉각적이 않을

           severely ill & toxic child

           serum creatinine level 증가

# Technitium labeled 2,3dimercaptosuccinic acid(DMSA) or glucoheptanate renal scanning

    ; *acute pyelonephritis 진단이 불확실한 경우 유용

    ; parenchymal filling defect

           - pyelonephritis 진단 가능

         - chronic acute 감별은 불가능

# CT

    ; acute pyelonephritis *definitive diagnostic method

# voiding cystourethrography

    ; *acute infection치료후 3주째 reflux 평가위해 실시

    ; Indications

           - all males & females under 5yr with initial infection

           - older femaleat the time of second infection

    ; *female - radioisotopic VCUG

    ; male - radiographic VCUG

# VUR 존재한다면

           --> IVP with nephrotomography or radioisotope renal scanning 실시

    ; IVP

           - kidney size 평가

         - 가능한 calyceal blunting, ureteral dilatation, renal scarring detection

    ; DMSA or glucoheptanate radioisotopic renal scan

        - *renal scar detection

        - 장점

            / urography보다 sensitive

         / IV contrast media adverse effect

         / abdominal gas 인한 urography 해석 방해가 있는 경우

         / infant young child에게 특히 유용

Differential Diagnosis

1. external genitalia inflammation

   : yeast, pinworm 의한 vulvitis, vaginitis

     다른 agent cystits 감별

2. viral, chemical cystitis : history, urine culture

3. hypoplastic, dysplastic kidney, vascular accident 의한 small kidney

   chronic pyelonephritis 감별 : X-ray 유사

           + 보통 vesicoureteral reflux 존재

*4. acute hemorrhagic cystitis

    ; E. coli

    ; *adenovirus type 11 & 21

           - male

    - self-limited,

    - 대개 4 정도 지속

5. eosinophilic cystitis

 1) symptom

    : hematuria, ureteral dilatation

      eosinophilic mass 의한 bladder filling defect

Treatment

# acute cystitis pyelonephritis 안가게 prompt treatment 필요

; *symptom severe culteure 동시에 치료 실시

 1) trimethoprim-sulfamethoxazole

        - 710

           - effective aganist E. coli                                

 2) nitrofurantoin

           - 57mg/Kg/day, 34 divided dose

    - effective against Klebsiella-Enterbacter organism

 3) amoxicillin : 50mg/Kg/day

; symptom mild하거나 진단이 의심될 culture 치료 실시

; culture 결과가 불확실한 경우 재실시

  치료시작전에 catheterization or suprapubic aspiration이용해 2nd culture 실시

# pyelonephritis 생각되는 acute febrile infection

    1) cefataxime(100mg/Kg/day)

    2) ampicillin(100mg/Kg/day) with aminoglycosides

           ; gentamicin(3mg/Kg/day, 3 divided dose)

           ; ototoxicity & nephrotoxicity of aminoglycosieds

                   --> serum creatinine measurements(initial and daily)

    ; *Pseudomonas 특히 effective

    ; sodium bicarbonate 의한 *urine alkalinization으로 효과 증가

    3) bactrim

           ; Pseudomonas 제외한 G(-) effective

    4) *ciprofloxacin

           ; *18 세이상에서 resistant microorganism alernative agent

87 UTI 치료 planning

    ; 1주일후 urine culture 필요

           - urine sterility 확인

    ; asymptomatic이라도 12년간 3개월 간격으로 follow-up culture 실시

           - UTI predisposing factor없이도 recur

# anti-biotic prophylaxis

; agent

    - bactrim, nitrofurantoin

    - *1/3 dose once a day

; 89,93,95 Indication

  UTI frequent recurrence

  persistent vesicoureteral reflux

  persistent cystitis - incontinence, frequency, urgency

  neurogenic bladder

  urinary tract stasis & obstruction

  calculi

  reflux

# excellent long-term prognosis

main consequence of chronic renal damage by pyelonephritis

    ; arterial hypertension , renal insufficiency

8. infrequent voiding, constipation 대한 education

9. antibiotic therapy & surgical or percutaneous drainage

   : renal & perirenal abscess or obstructed urinary tract infection가진 경우

CHAPTER 493. Vesicoureteral Reflux

1. 원인 : ureterovesical junction valvular mechanism incompetence

2. why reflux harmful to kidney

  1) renal pelvis voiding higher vesical pressure 노출

    cf) normal renal pelvis pressure : 10 mmHg

  2) bacteria bladder로부터 kidney passage 용이

    intrarenal reflux scar 관여

     : intrarenal collecting system에서

       collecting tubule로의 urine reflux & bladder dilatation

       renal scar 생성에 impotant role

  megaureter-megacystic syndrome (fig 493-1)

       dilated ureter massive reflux

     incomplete bladder emptying

     bladder rapid return

     progressive bladder emptying

3.89 reflux nephropathy

  1) children young adult ESRF *1520%

  2) children hypertension 주요원인

Classification

Table 493-1

# Primary VUR

 *1) 원인 : UVJ congenital anomaly

        short intramural ureteral tunnel

            --> valvular mechanism efficiency 감소

        urteral orifice - lateral & cephalad direction

        trigone - underdeveloped

 2) Fig 493-2

# Cystic & Bladder Instability

    ; reflux precipitation or perpetuation

# Ureteral Duplication & Ureterocele

1) ureterocele

    ; *upper collecting system - obstruction

    ; lower collecting system - reflux or contralateral side reflux

2) 96 Duplicated system

  *reflux ; more common in lower ureter

  lower ureter

           ; *enter bladder higher & more laterally

           ; less competent vlave

    ureter divertivulum으로 들어가는 경우

           ; reflux more common

# Congenital Neurogenic Bladder

 1) 원인 : myelomeningocele, sacral agenesis

 2) reflux : 출생시 ⅓에서 발생 ½에서 posterior valve 발생

                               결국 ½이상에서 발생

# Intravesical Pressure 증가한 Reflux

 1) bladder outlet obstruction & vesical dysfunction 발생

 2) infection 없이도 severe renal damage 가능

# VUR Grade

   Fig. 493-5

1) base

   severity

   ureteral dilatation

      & calyceal deformity 정도

2) 의의 : prognosis & therapy

Natural History

1. reflux garde 증가에 따라 renal scarring, reflux nephropathy 확률 증가

2. intrarenal reflux scarring risk 증가

3. *grade I & II reflux

 1) no ureteral dilatation

 2) vesicoureteral region anatomy 거의 정상

 3) child 성장하면서 *80%에서 spontaneous하게 소실

4. grade III & IV reflux

 1) *spontaneous resolution ; 15%

5. familial tendency

  5 이하의 sibling 검사

Clinical Manifestation

1. 발견

 1) UTI evaluation

 2) voiding dysfunction, renal insufficiency, hypertension or 다른 UT 의심되는 pathology 실시하는 VCUG

Differential Diagnosis

1. history & radiograph

   : primary or secondary reflux 감별

 1) US : renal size

 2) isotopic parenchymal renal scan : scar rule out

2. *continued voiding dysfunction in spite of antibacterial prophylaxis

    ; anticholinergics 치료 or lower urinary tract urodynamic study

3. reflux, infection & voiding dysfunction가진 환자

   : voiding dysfunction infection 의해 생긴것인지 infection

     predisposing하는 reflux 원인인지 결정하기가 어렵다.

Evaluation

    : reflux diagnosis & grading 실시

 1. IVP & tomography : renal size, scar 여부 확인

 2. blood pressure

 3. baseline creatinine clearance

 4. cystoscopy

Treatment

Primary reflux & reflex ass. /c complete duplication

1. Grade I & II reflux

 1) spontaneous resolution

  ; infection 방지 & antibiotics prophylaxis(bactrim, nitrofurantoin

    ; long-term treatment

 2) 치료시작시 *매달 urine culture 실시

  ; *prophylaxis effective하면 3개월 간격으로 culture

 3) asymptomatic bacteriuria & reflux

    ; harmful

  ; *symptom 없어도 urine culture 중요

 4) radionulide VCUG ; *1 마다 실시

 5) renal US ; *1년마다 실시 renal growth 평가

 6) *radionuclide study spontaneous cessation

  ; *another study in 36mo before discontinued AB therapy

           --> confirm

 7) Indication of surgery

    ; failure of antibiotic prophylaxis

 8) *DMSA renal scan ; 치료의 마지막에 실시

    residual scar가진 경우 blood pressure long term follow up 필요

2. 89 Grade III reflux

 1) *new scar formation 의심시 periodic parenchymal scan

 2) *more than 50% of children ; surgery 실시

3. Grade IV & V(ass. /c significant ureteral dilatation & upper urinary tract change)

 1) *early surgery after a brief prophylaxis & reconfirmation

  ; esp. infant young child

    5세이하에서 scar 빈도 증가

  ; *warrant

           - reflux 의한 prenatal hydronephrosis가진 newborn

             --> 1세경 spontaneous improvement

Secondary reflux

1. *duplication 있는 경우 ; A 동일

2. *periureteral divertivulum 있는 경우 ; early surgery

3. severe cystitis 있는 경우 ; primary cause remove

4. iatrogenic ; surgery

Results Of Surgery

1. *Cohen operation ; 97% 성공률

2. antireflux surgery complication

 1) reflux persistence

 2) distal ureter obstruction

3. surgery 반대측 reflux 대개 자연 소실

다른 치료 방법

1. *endoscopic injection of poly tetrafluoroethylene(polytef) under ureteral orfice

    ; 짧은 기간 effective

2. *subureteric injection ; collagen, silicone particle

3. success rate ; 65-70%

89 Cx of VUR

    ; UTI

    ; renal scar & atrophy

    ; hypertension(20%)

    ; CRF(reflux nephropathy)

Chapter 494 Obstruction of the urinary tract

; 원인 - congenital(children에서는 M/C), trauma, neoplasma, calculi, inflamatory process, surgical procedure

Etiology

Table 494-1

# High grade ureteral obstruction of early onset in fetal life

    ; result in renal dysplasia

           - from multicystic kidney to cortical dysplasia

# Chronic ureteral obstraction

    ; result in hypertrophy and later dilatation of ureter and upper collecting system

Clinical manifestation

urethral obstruction in fetus

    ; result in patent urachus, urethral dilatation, ureteral dilatation, VUR, urinary extravasation (urinoma, urinary ascites)

Bilateral ureteral obstruction or urethral obstruction

    ; cause *oligohydroamnion and pulmonary hypoplasia

    ; severe obstructive uropathy 경우 신생아에서 즉각적인 반응은 renal damage정도보다 pulmonary insufficiency 관계된다.

그밖에 obstruction relief 후에도 polyuria, dilute urine, chronic acidosis(serum creatinine level 정상), hypertrophy & dilatation in the bladder and collecting system postobstructive diuresis 생길 있다.

Diagnosis

; often silent

; in newborn

    - palpable abdominal mass due to hydronephrotic kidney

    - *patent urachus

    - *ascites(due to intraperitoneal urinary extravasation)

; prenatally diagnosed by US

    - oligohydroamnios & pulmonary hypoplasia 고려

; *Infection and sepsis

    - maybe first indication of obstructive lesion of urinary tract

    - renal US for all children with acute stage of febrile UTI

; obstructive renal insufficiency

    - *failure to thrive, vomitting diarrhea, other nonspecific symptom

; *overflow urinary incontinence, or poor urinary stream

    - *in older children

    - infravesical obstruction

; flank or abdominal pain with N/V in acute obstruction

; silent or vague abdominal or typical flack pain with increased fluid intake in chronic obstruction

    --> *abdominal pain 있는 all children에게서 intial study abdominl US

Imaging Studies

1) US

    screening method evaluate renal size, parenchymal thickness, determine ureteral dilatation, bladder evaluation 그러나 acute 또는 intermittent obst. 때는 minimal dilatation되므로 misleading 있다.

2) DTPA

    ; gross estimate of different renal fuction dilatation

    ; renal pelvis isotope있을

    Furosemide adm unobst 있을 : prompty excretes isotope, obst 있을    : no or slowly excrete

       isotopic renography with iodine labeled Hippuran : more accurate but exposed        kidney radiation

 

3) IVP

    ; preliminary radiography of the abdomen calculi, spinal abnormalities,

       abdominal & intestinal gas pattern 본다.

  ; postvoiding film residual bladder urine 본다.

  ; obstruction side radiopaque medium 남아있다.

  ; *intermitten obstruction

           - *pain등이 유발되는 acute phase IVP하는게 가장 유용하다.

Pressure Flow Studies (Whitaker test)

; percutaneous renal pelvis needle 넣은 radiopaque dye perfusion renal pelvis bladder사이 압력차 측정

    - *20cm of water 이상 pr. grad obst.

Voiding cystourethrography

모든 ureteral dilatation

VUR 보기위해 시행

urethral obst, esp. post urethral valve

infravesical obstruction palpable bladder

        ureth, catheterization 되지 않으면 ureth stricture의심 obstructive ureth,           lesion other than valve.

Specific Type of Urinary tract Obstruction

Hydrocalycosis

infundibular obst.인한 localized calyceal dilatation

원인 developmental

        2°to infl. process : exp. *Tbc

cong. obst. due to stenosis or extrinsic vas. compr. 증상 : pain(obst surgical correction 사라짐)

Dx. : IVP

Obstruction of the UPJ

; 93 obstructive lesion in children

    - *often by cong, stenosis of UPJ

    - others causes

           / ureteral kinks, fibrous band, aberrant vv.

; UPJ obstruction 가장 흔한 증상들

  - fetal hydronephrosis in maternal ultrasonography

  - palpable renal mass in newborn

  - abd, flank or back pain

  - febrile UTI

  - hematuria after minimal trauma

; *bilateral (20%)

# Unilateral hydronephrosis in the fetus (normal contralat. kidney, normal AF)

    ; not indication for prenatal finding

    ; *US - 출생 3일이후 실시(oliguria dilatation masking 있기때문)

           - ★정상이라도 3 or 6 mo repeat

    ; mild to moderate hydronephrosis

           - observation & F/U

    ; marked dilatation, renal parenchymal thin

           - isotope renogram

           - normal renal fuction이면 F/U

                   / no improvement - *diuresis renogram 6-12mo

    ; surgical repair Ix

       - involved kidney function decreased

           - bilateral involvement or solitary kidney

           - diminished overall renal function

           - palpable mass

Sx가진 old children

  IVP, 만약 IVP에서 안보인다면 US.

D/Dx

    i) megacalycosis

    pelvis, ureteric dilatation없이 congenital non-obstructive calyceal dilatation

    ii) VUR with marked dilated & kinking of the ureter

    iii) midureteral, distal ureteral obst.

# newborn /c renal mass D/Dx UPJ obst

    i) multicystic renal dysplasia

    ii) solid renal tumor

    iii) renal vein thrombosis

Tx. surgical pyeloplasty

Follow up diuresis renography

Midureteral Obstruction

Cause

) cong. ureteral stenosis or ureteral valve in midureter

) partially obstructed retrocaval ureter

) circumcaval ureter

    ; Rt. anomalous development of vena cava with persistent ventral infrarenal subcardinal veins

    ; IVP Rt.ureter 3rd lumbar vert. level에서 medially deviated.

이외의원인     retroperitoneal tumor

                  fibrosis by surgery. infl. process (CGP)

                  radiation

Ureteral ectopia

mesonephric duct migration path 어느곳에나 발생가능

drain single collecting system

    ; commonly belongs to upper moiety of duplicated collecting system

ureteral orifice of upper collecting syst.

    ; always caudal to ureteral orifice of lower collecting system

male

    i) usually *single ectopic ureter

    ii) bladder neck, urethra above external sphinncter seminal vesicle, vas deferens등으로 유입

    iii) high grade obstruction, UTI or epididymitis Sx.

    iv) 반대측이 normal nephroureterectomy

    v) bilateral single ectopic ureter unilateral case with functioning involved kid. reimplantation

female

    i) *ass. /c duplication

    ii) ureter of upper collecting system

           --> bladder neck, urethra at or above the level of sphincter 

           ; upper pole nephroureterectomy

    iii) ureter --> vestibule, vagina, uterus

           ; urinary incontinence, vaginal discharge

    iv) Dx

        ; IVP, US, endoscopy with high degree of suspicion

    v) bilateral simple ectopic ureter

           ; *bladder hypoplasia 동반

Ureterocele

; congenital cystic dilatation of the distal ureter bladder내로 protrusion되어 pin point

; 〉남

; simple ureterocele

    ) non-duplicated collecting system orifice 제위치(bladder내에 )

    ) IVP

           ; varying degree of ureteral & calyceal dilatation, round filling defect in bladder delayed film ureter cleanred visible하거나 & filling of contrast media 수도 있다.

    ) Tx.

    transurethral incision of uretercele (때로 VUR 초래하여 ureteral reimplant 후에 )

    open excision of ureterocele & reimplartation

    upper tract dilatation 없는 small ureterocele No Tx. pr. flow study & diuresis

; ureteral duplication 동반

    - 주로 *upper renal moiety에서 drain하는 ureter ectopic ureterocele

    - upper renal moiety : poor fuction, cong.obst.으로 dysplastic

    - lower renal moiety : frequently reflux

    - ectopic ureterocle may extend submucosally into post. urethra

           / 대부분 unilateral

; UTI

; bladder neck obst.

; urinary retention, reflux 등의 양상을 보임

; IVP

    large filling defect in bladder

    characteristic finding of duplication

    upper collectin syst. - poor or absent function.

    lower collecting syst. - caudal displacement

           "drooping lily app"

; Tx

    excision of upper collecting system : partial nephrectomy & ureterectomy

    small ectopic ureterocele with ipsilateral duplicated ureter low or no reflux : No Tx.

    large ureterocele with ipsilateral lower ureter high grade reflux : excision of ureterocele & reimplantation of remaining ureter partial upper moiet

    obstructing, ureterocele 가진 acutely ill, septic infant involved collecting system drainage by transureterally or by percut. nephrostomy 필요함

Megaureter

dilated ureter

careful Hx. PE. voiding cystourography 등이 2°megaureter와의 DDx위해 중요

보통 distal ureter more dilated되어 있고 bladder와의 junction에서 갑자기

   tappering된다.

      obst. megaureter nonobst. megaureter DDx위해 dieuresis renogram press

          flow study 해서 obst이면 op해준다.

Table 494-2

91 Prune-Belly Syndrome

(=abdominal muscle deffciency syndrome =Eagle -Barrett syndrome)

; 1/40,000

; *97% male

Characteristics

  - deficient abdominal muscle

  - undescended testes

  - urinary tr. abnormality result from urethral obstruction in fetal life

         / ureter & upper part mass dilatation, large bladder with patent urachus, VUR, urachal diverticulum, dilated prostatic urethra, hypoplastic prostate, dysplastic kidney, megalourethra

; *oligohydramnios, pul. hypoplasia - frequent Cx

; bowel malrotation c universal mesentery

; cardiac anomaly (10%)

; musculoskeletal anomaly (more than 50%)

; 1/4 - at birth urethral obst.()

; 치료

    - obstruction 없으면 UTI예방이 치료의 목표다.

  - obst.. () / temporary drainage & later reconstructive surgery

    - orchiopexy : 1세이내

; 예후

    - pulmonary & renal dysplasia정도에 달렸음.

    - 1/3 사산되거나 출생후 수개월 내에 pulmonary Cx으로 죽음

  - 장기 생존자의 경우 1/2 CRF 가며 경우  KTP후의 경과는 양호하다.

Bladder Neck Obstruction

원인

 2°to ectopic ureterocele.

 bladder calculi, prostatic tumor(rhabdomyosarcoma)

 post. ureth. valve ; functional disturbance rare neurogenic bladder dysfuction   

Sx. : difficulty voiding, urinary retention, UTI bladder distention with overflow incontinence

1°bladder neck obst is exceptional in males

94 Posterior Urethral Valves

; urethral valve *common type

; sail-shaped membrances with eccentric opening

    - *verumontanum에서 시작하여 distally extend

           --> attach to anterolateral wall of urethra

; dilated prostatic urethra, detrusor m. & bladder neck hypertrophy

    --> VUR

; oligohydroamnios, pul. hypoplasia

# prenatally diagnosed case

    ; severe obstruction

           --> prenatal bladder decompression by percutaneous vesicoamniotic shunt or open fetal surgery

    ; *worse prognosis than detected after birth

# severe case

  ; *failure to thrive due to uremia

    ; *sepsis due to UTI

# lesser severe case

    ; *difficulty in maintaining urinary continence

Diagnosis

    ; VCUG

           - prostatic urethral dilatation with transverse linear filling defect

           - VUR : 2/3

Treatment

# serum Cr - normal or return to normal

    ; primary ablation of valve through transurethral approach

  ; by temporary vesicostomy if insufficient urethral caliber

# serum Cr - high or despite bladder drainage

    ; suspect 2ndary ureteral obstruction, irreversible renal damage, renal dysplasia

    ; upper tract drainage

           - cutaneous pyelostomy, high ureterostomy 

# no improvement of renal function

    ; reconstructive surgery at early age

# septic & uremic Pt

    ; electrolite imbalance

    ; Abt percut. nephrostomy

           renal analysis stable evalutation & Tx

# incontinence 주소인 경우

    ; primary valve ablation

# *VUR

    ; expectant Tx. with AB therapy

           --> *relux 1 yr & kidney function

           --> surgical correction

# *Tx.후에도 urinary incontinence 50%

    ) surgical damage of sphincter

    ) dilatation of prostatic urethra

    ) poor bladder compliance

    ) polyuria from renal damage

    --> *나이들면 (exp. after puberty)호전

; *Px in newborn

    - depend on pul.hypoplasia & renal function recovery potential

    - neonate이후에 1/3에서 어느 정도의 Renal insuff.있어 renal transplantation 요함

Urethral Stricture

male

    ) congenital : rare

  ) urethral trauma

           ; iatrogenic (cath, endoscopic, ureth, reconstr.)

       ; accidental (straddling injury, pelvic fracture)

    ) Sx.

           ; bladder instability hematuria dysuria

    ) Dx

           ; voiding film of IVP RGCU, endoscope

    ) Tx

           ; short - endoscopic dilatation & internal urethrostomy

           ; long - urethroplasty

female

    ; true is exceptional

    ; urethral ring urethral obst. & UTI 초래

Anterior Urethral Valve & Urethral Diverticula In Male

동반

Dx : VCUG

diverticula dilatation of Cowper gland & duct 생각

Male Urethral Meatal Stenosis

; 4 이하 - 8F , 10세이상 - 10F

    true meatal stenosis

; neonatal circumcision glans ammoniacal dermatitis 인해 발생

; hypospadia 시도

; Tx. : meatoplasty

Chapter. 495. Anomalies of the Bladder

Bladder Extrophy

; 빈도

- 1/40,000 birth.

- boys girls

Clinical Manifestations

mesoderm cloacal membrane으로 caphaled extension 안되어

classic 경우

bladder protrusion from the abd. wall

exposure of bladder mucosa

downward displacement of umbilicus

wide separated public rami

separated recti m.

ant. displaced anus with rectal prolapse

: complete epispadia with wide & shallow scrotum undescended testes inguinal hernia

         female : epispadia, clitoral duplication wide separation of labia

         broad based gait : wide separated public rami때문

untreated 경우

) total urinary incontinence

) bladder Cancer , esp. adenoCa.

Treatment

; 출생 직후 bladder silastic shield 다른 적당한 plastic dressing 해서 desication of bladder 막는다.

; *48hr이내 closure of the exstrophied bladder

    - before permanant changes in the bladder walls

    - flexibility of pelvic joint 있을때 시행

; initial op. 목적

    ) precise closure of the bladder & prostatic urethra in male

    ) elongation of the urethral plate & penis

    ) closure of the abd. wall

; 대부분 후에 VUR동반

; epispadia operation

    - 1-2yr

; Tx of incontinence by bladder neck reconstruction

    - *after rectal control

Prognosis

치료후에 upper urinary tract에서 15%이하의 deterioration. 70% 이상의 continence 획득한다.

이밖에 artificial sphincter 만들거나 cytoplasty, abdominal stoma, ureterosigmy 등을 필요에 따라 해주기도 하나 이런 op ch. pyelonephritis, urinary tract damage 전해질 불균형, 장기간 경과 carcinoma 이행 등의 위험률이 있다.

Other Exstrophy Anomalies

cloacal exstrophy

  severe abn. of colon, rectrum, short bowel

epispadia

   distal epispadia : reconstruction of urethra & penis

Bladder Diverticula

; *대개 UVJ 호발 VUR

원인

congenital

commonly asoc. distal ureth. obst. neurogenic bladder dysf.

Tx.

    ; small : none

    ; large ineff. voiding, residual urine stasis. UTI excision

   [urachal anomaly] : male fe

Chaper 496. Neurogenic Bladder

Etiology

; congenital

; meningomyelocele, lipomeningocele, sacral agenesis, other spinal abnormalities

; acquired & traumatic

    - less frequent

    - cerebral palsy, CNS tumor & their treatment pelvic op. (imperforated anus, sacrococygeal, teratoma)

; two important consequences

    - *upper tract deterecration, urinary incontinence

Renal Damage

; dys-synergia

    --> functional obstruction of bladder outlet

    --> high intravesical pressures

    --> bladder muscles hypertrophy, trabeculation

    --> VUR

    --> deterioration of upper tracts

; VUR

    - 30% of neonate with myelomeningoceles, later 20%

    - *more severe than primary reflux

; infection

    - compounds problems

; neurologic anomaly 가진 Pt 모두가 neurogenic bladder 있는 것은 아니므로 urodynamic study(cytometrography sphincter electromyography)하여 R/O시켜야 .

Urinary incontinence

; 원인

    total or partial denervation of sph.

    bladder hyperreflexia or poor bladder compliance

    chronic urinary retention

3) Tx

Neurogenic bladder 가진 환자 치료의 목적

           protection of upper urinary tract

           eventually providing continence

newborn with low intravesical pr. & No VUR

          expectant therapy

           US & radioisotopic cystogram f-up

           recurrent UTI prolonged antibiotic therapy

           urodynamic study at 6 mo. repeat

VUR(+), elevated intravesical pr. (grade &)

         Antibiotics prophylaxis intermittent catheterization

         anticholinergic drug(oxybutynin 0.4mg/kg/day Sig 1/2)p.o 40%에서 호전

         severe reflux 있을 경우 corrective surgery

   incontinence Tx

       ) sphincter tone 충분하다면 4hr 간격으로 intermittent catheterization

       ) 78 yr. old who have adequate manual dexterity intermittent self                  catheterization

       ) intermittent catheterization 동안 antibacterial prophylaxis 있다.

     bladder capacity compliance 충분. but urethral resistance 낮을

            implanatation of artificial sphincter

     Enterocytoplasty

     ) Tx : neurogenic bladder bladder exstrophy, post urethral valve bladder

         capacity 줄이는 질환

      ) Rt. or Lt. colon 이나 ileum 끼어 넣음

      ) 경우 intermittent cath   colon G(-) bacteria 나오므로 Sx 있을 때만

          UTI Tx

    ) hypermetabolic acidosis 있으므로 Tx (enteric mucosal surface urine 접촉하는 부분에서 ammonium, choloride. H+이온 흡수, K+ loss하므로)

    ) 이런 Cx 막기 위해 ch. renal insufficiency 환자에게는 colon 대신 gastric segment 사용하기도 .

    ) Cx으로 perforation, peritonitis, potential for malignancy 등이 있음.

Chaper 497. Voiding dysfunction

Nocturnal Enuresis

5 child 20%

    ; 이후 해마다 15% 감소하여 adult에서는 1%이하

원인

    ; *delayed maturation of the cortical mechanism

    ; alteration of the circadian rhythm of ADH secretion

    primary ;  night-time continence period (-)

           secondary ; formely dry child에서 emotionally disruptive event 후에 발생

male(3) female

FHx있다.

neurologic & spinal abnormality, fluid intack, urinary output

urinalysis

    ; Bacteriuria

    ; specific gravity & osmolarity after overnight fast

daytime sx. (-). P/E, UA, urine culture normal No further evaluation

    needed

Tx

        ) reassurance. self - limited, eliminate punitive measure

        ) fluid restriction, midnight awakening systems of reward & punishment :

             not successful

       ) imipramine drug 끊으면 대개 recur

       ) desamino - D - arginine vasopressin : 50%

       ) Tx. of conditioning with alarm.

Unstable Bladder

voiding dysfunction not related to neurologic abNl or dys Fx

Sx : frequency, urgency, blader pain없이 diurnal urinary incontine

Study

    Hx P/E UA, urine culture, uroflowmeter, abdo U/S, male - VCUG

Fx

    UT pathology없을 oxybutynic & other anticholinergic

Non-Neurogenic Neurogenic Bladder

non neurogenic destrusor/sphincter dys 같은

neurologic abnormal 없이 voiding ext sphincter relax(-)

Hx UTI Constipation Hx 있다.

P/E

VUR trabeculated bladder, decreased urine flow rate intermittent pain

toilet training 관련

Tx

         ) treatment of reflux & antibiotics prophylaxis

         ) behavioral modefication & encouragement of relaxation during voiding

         ) Buifeedback

         ) intermittent catheterization & anticholinergic drug

         ) diazerpam

Px : poor

Infrequent Voiding

UTI 동반된 micturition common disorder

girls , FHx 있다.

원인은 behavioral

Tx : antiotics prophylaxis.

               encourage to frequently void

               complete emptying by double voiding

Other Cause Of Incontinence In Females

table 497-1

1) Ureteral ectopia

대개 female에서 duplicated collection system 동반

Nl voiding pattern 보이면서 constant dribbing of urine

Dx : IVP, US, CT of kid, Exam under anesthesia for ectopic ureteral orifice in vestibula or vagina

Tx : partial nephroureterectomy

Chapter 498. Anomalies of the penis and urethra

Hypospadiasis

빈도 : 1/500 newborns.

     mildest 경우 uretheral meatus glans vent open하는 것에서부터 severe    수록 penis ventral curved되면서 penile urethra 짧아진다. (chordee동반되어서)

meatus opening : ventral aspect of glans, penoscrotal juction, perineum

10%에서 undescended testes 동반

치료의 목적 mild case : cosmatic

                     severe case : 서서 voiding 있게

                     future Nl sexual function

                     avoid psychogenic consequence

     ideal age for repair controversial하지만 현재 18mo.이전에 시행 toilet              training(1세전) 실시 newborn period 시작 (routine circum 피할 )

Agenesis and Micropenis

A. agenesis : rare하여 anorectal and rectal anomalies 동반 많이 .

B. Micropenis

             normal NB penic : 3.5±0.7cm

             primary

             secondary testicular failure after complete morphogenesis

              anencephaly

              pituitary agenesis

              Kallmann, Noonan, Prader - Willi synd

              rudimentary testes

              dwarfism

              materna hormone administration

    Tx. : hormone stimulation

         rearing as female with later genital reconstruction

Phimosis & Paraphimosis

   phimosis : prepuce retraction 나이(3) 지나도 retraction되지 않는 것으로 inflamation sequal 되는 것과 congenital 되는 것이 있다.

   Paraphimosis : phimotic preuce coronal sulcus 뒤로 retract되어 reduce 없을 , edema severe pain 호소하며 초기 발견하면 heavy sedation이나 전신마취 시킨 lubrication해서 foreskin reduce시킨다.

Chapter 499. Disorders and Anomalies of the Scrotal Contents

Undescended Testes

Andescended & Ectopic testes

1) Cryptorchidism

true undescended testes : normal path of descent 따라 존재

   processus vaginalis : patent

        ectopec testes : inguinal canal 통해 complete descent 했으나 subcut location

                        end up

         M/C :  lat to the ext. inguinal ring below subcut. fascia

# incidence      

    ; 0.7% of children after 1yr and adults

    ; 3.4% in newborn

    ; prematurity(20002500g) - 17%

    ; 900gm - 100%

# 7th mo. of gestation testicular descent

# *spontaneous testicular descent dose not occur after 1yr

# Complication

         ) infertility in adulthood

         ) tumor development : 2044% risk

         ) associated hernia

         ) torsion of the cryptorchid testis

         ) psychologic effects of an empty scrotum

# bilateral cryptorchidism : 30%

     undescended testis 원래 조직학적으로는 정상이나 failure of developement and      atrophy 일어나 3rd or 4th decade of life에서는 maligrant testcular 확률이       2044% 증가된다. 중에서 M/C tumor Seminoma(60%)이다. (정상 testes       서는 30%)

     true undescended testes에서 indirect inguinal hernia 반드시 나타나며 이밖에

        torsion infarction 동반될 있다.

Treatment

# unilataterl cryptorchid testes

    ; *orchiopexy during early in the 2nd yr. of life

# bilateral

    ; palpable testis

           - unilateral 동일하게 치료

    ; not palpable testes

           - *hCG stimulation test

                   / testosterone↑시 abd. exploration & orchoipexy

                   / negative response 수도 있다

      ) HCG or LHRH

            surgical Tx. replacement 못함

            1,000u im.3 times weekly for 3 wk testes하강 (-), orchiopexy

Retractile Testes

   exaggerated cremasteric reflex inguinal canal내로 retract

   puberty scrotum으로 내려옴

   No Cx

Abscent testes

   non-palpable testes 200%

congenital : quite rare

acquired : vascular accident

Torsion Of The Testis Or Appendices

6 이하에서 acute scrotal pain swelling 호소하는 환아의 40%에서 testicular         torsion 이다.

원인 abnl. fixation of the testis to the scrotal envelope

Sx acute scrotal pain, tenderness, swelling absent cremasteric reflex

DDx

) incarcerated hernia 때는 inguinal area swelling 있다.

      ) torsion of one of the 0testicular or epididymal appendicies 때는 pain

          swelling 적고 testis위에 blue dot 보이고 local tenderness

) epididymitis :    13이후에는 반드시 R/O

       18 이상시 M/C cause of scrotal pain & swelling

       UA abnormal

진단법 : ultrasonogram, color doppler, ultrasound, isotope scan

Tx

   prompt surgical exploration 6h전에 retorsion 90%↑에서 survival of gonad contrlateral testes scrotum fixation

Varicocele

; pampaiform venous dilatation due to valvular incompetence of spermatic vein

; *predominantly Lt side

    - bilateral 10%

; rare before 10yr, adult 15%

subfertility, decreased size of testis

large painful, fixed varicocele : retreoperitoneal tumor의심

surgery : pain relieve, normal testis development 목적

varicocel early correction fetility 대한 관계는 아직 밝혀지지 않았음.

Hydrocele

tunica vaginalis fluid accumulation transillumination (+)

small hydrocele 1yr 소실

communicating hydrocele (vary in amount)시는 indirect inguinal hernia처럼 Tx.

Epididymitis

   acute scrotal pain & swelling

   cong. anomaly of wolffian duct (ectopic ureter entering the vas.)

   UA : pyurea gonococcus Chlamydia

   Tx. : bed rest antibiotics

Chapter 500. Trauma to the Genitourinary Tract

 

   Trauma 인한 accidental injury 경우 skeletal이나 CNS 다른 곳은 trauma    동반될 경우가 많고 만약 kid Trauma 받았다면 선행하는 kid anomaly 있을 확률    많다. (horseshoe kid, renal ectopia, hydronephrosis)

Evaluation of Pt.

) bladder cath. : except bleeding from urethra

         urethral injury retrograde urogram

        Tx. : suprapublic cystostomy drainage until resorption of hematoma                        urethroplasty

     

 

      Cx. :  erectile impotence  urethral stricture  urinary incontinence

) cystography

) IVP

) renal angiogram, CT

Renal injury 분류

minor - parenchymal 손상, 대부분 이것 op. 필요없다.

major - collecting system, renal pedicle 손상

infant & child에서 Kidney injury 호발이유

   proportionally greater size

   less sturdy fatty & fascial envelope

   greater flexibility of overlying lower ribs

Chapter 501. Urinary Lithiasis

기후, 음식, 사회 경제적 요소와 연관되므로 geographic variation 있다.

Sx. 순서

) gross or microscopic hematuria

) abd. pain

) flank or back pain

) UTI sx

plain abd.

) faintly seen cystine stone infectious stone(Struvite)

) radiolucent stone

  uric acid 2.8-dihydroxyadenine xanthine calculi US, IVP, CT 진단.

) complete functional & radiographic study 1/4에서 VUR(+)

) chemical & crystallographic analysis

laboratory tests suggested to evaluate urolithiasis

    table 501-1

classification of urolithiasis

    table 501-2

Calcium Stones

; calcium oxalate

    - *common urinary caculi

원인

         ) idiopathic urolithiasis

         ) associated with hyperclacemia

              hyperparathyroidism

              sarcoidosis

              immobilization

              hypervitaminosis D

              idiopathic, isolated form

         ) normocalcemic hypercalciuria

             furosemide adm.

             uncontrolled distal RTA

             TPN, alkalosis

             idiopathic

             renal tubular calcium leak 2ndary hyperparathyroidism.

                                     hyperabsorption of calcium

            (primary intestinal hyperabsorption of calcium)

urinary calcium measurement

          upper limit of normal : 4mg/kg/24h

          urinary Ca/creatinine ration 0.25mg/mg

calcium oxalate stone

          small bowel ds. & malabsorption

           intestinal hyperoxaluria 1°hyperoxaluria (type 1 or 2)

Cystinuria

dibasic amino acids(cystine, ornithine arginine, lysine,) transport inborn error

excessive urinary excretion of products

formation of calculi

Struvite Stones

urea splitting organism

( Proteus, occasionally Klebsiella E. coli Pseudomonas) urine alkalinazation,       excessive magnesium ammonium phosphate (struvite), calcium phosphate

       aluminum phosphate 침착

Uric Acid Stones

대개 hyperuricosuria with or without hyperuricemia

            radioluscent stone

           persistent acid urine & urate crystalluri 의심

      원인

        inborn error of purine metabolism

        Lesch - Nyhan. syndrome

        G-6PD-deficiency

        short-bowel synd. (esp. ileostomy)

        chronic dehydration & acidosis

        tumor & myeloprolif. ds.

        cf. 2.8. dihydroxyadenine lithiasis

          adenine phosphoribosyltransferase def.

Treatment

# 2 prospectives

    ; underlying metabolic disorder Tx.

           - infection, predisposing anatomic factor

    ; Tx of complication associated with stone itselfs

           - infection & obstruction

recurrence 방지

adequate state of hydration & diuresis

alteration of pH

        ) cystine, uric acid pH 7.5이상으로 alkalinazation solubility

        ) struvite acidic urine

 

therapy for underlying 0metalbolic disorder

        ) thiazide : primary renal hypercalciurla

        ) RTA Tx.

        ) allopurinol(uric acid stone. 2.8 - dihydroxvadenosine)

        ) D-penicallamine (cystine, hemicystine)

        ) N-acetyl cystine - cystine stone

) cellulose phosphate primary absorptive hypercalciuria

         ) pyridoxine hyperoxaluria

         ) salts of phosphate, citrate, magnessium calcium oxalate solubility 증가

# surgical Tx

    ; *최근에는 거의 하지 않음

    ; obstruction of collecting system, pain, bleeding, perpetuating infection 있을때 실시

# endoscopical or percutaneous access to kid

# extracorporeal shock wave lithotripsy

    ; *renal & ureteral stone 모두 가능

    ; *succes rate - more than 75%