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Part 24. Urologic disorders in infants and childhood

³Ú½¼ Á¤¸®

Part XXIV. Urologic Disorders In Infants And Children

Chapter 491. Congenital Anomalies & Dysgenesis Of The Kidney

; *»ýÈÄ 1ÀÏ ³» voidingÀÌ ¾øÀ¸¸é underlying anatomic abnormality °Ë»ç

; Ãâ»ý½Ã urine

 1) pH 5¡­7

 2) osmolality : 60¡­600mOsm/Kg (mmol/Kg) H2O

 3) many epithelial cell

 4) RBC : °£È¤ Á¸Àç

 5) *WBC : Ùí

 6) culture : sterile

 7) glucose & protein : trace amount

Renal agenesis(aplasia)

Bilateral Agenesis

; incidence : 1/,3000

; °á°ú : stillborn fetus

# ¡Ø96°´ Potter phenotype

    ; oligohydramnios¿¡ ÀÇÇØ ¹ß»ýÇϴ Ư¡ÀûÀÎ ÀÓ»óÁõ»ó

           - 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

     50¡­90%ÀÇ penetrance

     ´Ù¾çÇÑ expression

     anorectal, cariovascular, skeletal abnormality

 4. Áø´Ü

  1) maternal ultrasound (2nd trimester)

     : oligohyramnios

       bladderÀÇ nonvisualization

       absent kidney

 5. 1°³¿ù ³» deathÀÇ ¿øÀÎ

    : uremia or pulmonary insufficiency

Unilateral Renal Agenesis

; ´Ù¸¥ °Ë»çµµÁõ ¿ì¿¬È÷ ¹ß°ßµÇ´Â °æ¿ì°¡ ´ëºÎºÐ

; *single umbilical artery ÀÖÀ» ¶§ ¹Ýµå½Ã ÀǽÉ

; absence of ureter, ipsilateral bladder, hemitrigone

contralateral kidney : Ãâ»ýÈÄ compensatory hypertrophy

associated anomaly

1) genitourinary aystem : 40%

2) skeletal system : 30%

3) cardiovascular system & gastrointestinal system : °¢°¢ 15%

4) CNS & respiratory system : °¢°¢ 10%

incidence

1) 1/1,000 live births

; *male > female

; *left > right

¢¾ Mayer-Rokitansky Syndrome

    ; unilateral renal agenesis & vaginal atresia or agenesis

vas defensÀÇ agenesis, seminal vesicle cyst

Anomalies In Shape And Position

ascent & rotationÀÇ ºÒ¿ÏÀü  Fig 491-2

 ¡æ renal ectopia or nonrotation ¹ß»ý

# Horseshoe Kidney

    ; ½ÅÀåÀÇ lower poleÀÌ Á߽ɼ±¿¡¼­ À¶ÇÕ

    ; incidence - 1/500 birth

    ; *Turner syndromeÀÇ 7%

    ; *Wilm`s tumorÀÇ ¹ß»ýºóµµ°¡ 2¡­8¹è ´õ ³ô´Ù

Hypoplasia

definition : nephron¼ö°¡ °¨¼ÒÇÑ small kidney

; *not inherited

unilateral involvement

1) segmental hypoplasia or Ask-upmark kidney : ÀϺΠinvolve

2) entire involvement

3) 10¼¼ ¹Ì¸¸¿¡¼­ hypertensionÀ¯¹ßÇÏ´Â more common cause

bilateral involvement

1) chronic renal failureÀÇ Áõ»ó °¡Áü

2) 10¼¼ ¹Ì¸¸¿¡¼­ ESRFÀÇ ÁÖ¿øÀÎ

3) polyuria & polydypsia : common

4) oligomeganephronia(rare form) : nephron¼öÀÇ ÇöÀúÇÑ °¨¼Ò & ÇöÀúÇÑ hypertrophy

Dysplasia

1. definition

    : fetal kidneyÀÇ structural diffentiationÀÇ »ó´çÇÑ º¯È­·Î cyst, abnormal duct,

     undefferentiated mesenchyme, or cartilage°°Àº nonrenal element°¡ Á¸Àç

 2. ¿øÀÎ

  1) urinary tractÀÇ intrauterine obstruction

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

      ¢¡ º¸Åë bilateral & ESRF ¹ß»ý °¡´É

  2) multicystic dysplasia

   ¨ç unilateral or bilateral

   ¨è lower tractÀÇ developmental anomaly or contralateral normal kidney¿Í °ü·Ã

   ¨é unilateral form

      : nonfunctioning flank mass

        ¢¡ renal tissue°¡ °ÅÀÇ ¾ø°Å³ª ÀüÇô ¾ø´Â cystic mass

           hypertensionÀÌ »ý±â¸é Á¦°Å

   ¨ê bilateral form

     : chronic renal failure¿Í °ü·Ã ¢¡ ½ÉÇÑ °æ¿ì Potter syndrome¸¦ º¸ÀÓ

CHAPTER 492. Urinary Tract Infections

Prevalence And Etiology

# newbornÀÇ symptomatic UTI

    ; 1.4/1,000

# ¡ÚRisk Factors

    ; variable markedly with age & sex

    ; *uncircumcised male infant(<1yr)

    ; *female(>1yr)

    ; 7-11yr age

           - *¡ãcommon age group (2.5%)

    ; sexually active female : cystitis À§Çè Áõ°¡

    ; sexually active adolescent male & female : urethritis ¹ß»ý °¡´É

# Pathogens

    ; mostly colonic bacteria

    ; female

           - ¡ÚE. coli(75¡­90%) > 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 urethraÅëÇØ ħ¹ü

  - *ÀÌÈÄ : ascending infection

UTIÀÇ susceptibility¿¡ °ü¿©ÇÏ´Â factor

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

2) bacterial adherence¿¡ °ü¿©ÇÏ´Â factor : P blood grup phenotype

3) immunosuppression, diabetes, urinary tract obstruction,

   chronic granulomatous disease ½Ã Áõ°¡

infectionÀÇ severity¿¡ °ü¿©ÇÏ´Â factor

1) bacteriaÀÇ virulence

2) anatomic factors

   : ¡¤vesicoureteral reflux

        +¡æ vesical overdistention

       -+¡æ bladder wallÀÇ blood flow °¨¼Ò

        +¡æ infection¿¡ ´ëÇÑ bladderÀÇ natural resistance °¨¼Ò

     ¡¤obstruction

     ¡¤urinary stasis : bacterial multiplication

     ¡¤prisence of calculi

Acute Bacterial Cystitis

 1. Ư¡

    : mucosal congestion, edema¿Í petechiae, hemorrhage

      ¡æ detrusor muscle hyperactivity

      ¡æ bladderÀÇ functional capacity °¨¼Ò ¡æ vesicoureteral reflux

 2. cystitis cystica : chronic or recurrent infection ÀÖ´Â °æ¿ì ¹ß»ý

Acute Pyelonephritis

# ¡ÚP1 blood group

    ; VURÀÌ ¾ø¾îµµ ascending recurrent pyelonephritis

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

medulla¿Í pelvis¿¡ acute inflammatory cell infiltration ¡æ enlarged kidney

 ¡æ microabscess Çü¼º ¡æ renal scar Çü¼º : chronic pyelonephritis

 obstructionÀÖÀ¸¸é ´õ severe

 Áï°¢ÀûÀÎ Ä¡·á : ¿ÏÀü 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

  - tubuleÀÇ fibrosis & atrophy

; *cortex º¸´Ù medulla°¡ susceptible

    - high osmolality

           --> leukocyteÀÇ phagocytosis °¨¼Ò

# reflux nephropathy

    ; renal scars in VUR children

    ; no history of UTI

    ; *flat papillae¿¡¼­ ¹ß»ý

    ; *autoimmune response to Tamm-Horsfall protein

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

; *90% of chronic pyelonephritis

    - *VUR

¡Úreflux nephropathy or chronic pyelonephritis

    ; ¡ã cause of arterial hypertension in children

Urea-Spliting Organism : Proteus

; *stone formation

    - urea·ÎºÎÅÍ ammonia»ý¼º ¡æ alkaline urine Çü¼º ¡æ calcium phosphate, triple calcium, magnesium, ammonium phosphateÀÇ precipitation°¡´É ¡æ calculi Çü¼º ¡æ foreign body·Î ÀÛ¿ë ¡æ infection

ureteral obstruction½ÃÀÇ infection

  : septicemia, pyelonephrois, renal & perirenal abscess formation

Xanthogranulomatous Pyelonephritis

1. Á¤ÀÇ : giant cell°ú foamy histiocyte°¡ ÀÖ´Â granulomatous inflammation

2. renal mass, acute or chronic infection À¯¹ß

3. predisposing factor

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

4. treatment : nephrectomy

Clinical Manifestation

; asymptomatic bacteriuria

    - mostly

# urinary tract infectionÀÇ 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 instabilityÀÇ Áõ»ó

# *hematuria

    ; *E. coli¿¡ ÀÇÇÑ hemorrhagic cystitis

# acute pyelonephritis

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

# chronic pyelonephritis

    ; asymptomaticÀÌ Òý

    ; 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

         ¨ç ÀÇÀÇ : single organismÀÌ 105 colonies/mL

           ¡æ UTI¿¡ 90% ÀÌ»óÀÇ specificity°¡Áü

         ¨è ¡Ø87,94 low colony³ª¿À´Â °æ¿ì

                   ; overhydration, recent bladder emptying, antibiotic therapy

    2) adhesive, sealed, sterile collection bag

    3) catheterized specimen

         ¨ç infant : 5F polyethylene feeding tube

         ¨è older children : 8F polyethylene feeding tube

    4) suprapubic puncture

         ¨ç full bladder ¸¸µé°í ½Ç½Ã

         ¨è 25 or 22 gauge needle »ç¿ë

         ¨é pubisÀÇ midline¿¡¼­ 1 finger-breadth »ó¹æ, 10¡­20¡Æ °¢µµ

         ¨ê ¡Ø87,94 false negative culture

                   ; unrecongnized antibiotic therapy, overhydration¿¡ ÀÇÇÑ dilution, antiseptic solutionÀÇ contamination

# urinalysis

    1) pyuria : ³ó´¢ ¾øÀ̵µ infection recur°¡´É

    2) microscopic hematuria : acute cystitis

    3) urinary casts : renal involvement

    4) alkaline pH : Proteus infection

# upper & lower UTIÀÇ °¨º°

    ; acute renal infection

           - leukocytosis, neutorphilia, ESR & CRP Áõ°¡

           - Ab-coated bacteriaÀÇ detection

           - single dose antibiotic therapy¿¡ ´ëÇÑ ¹ÝÀÀ

           - ´Ù¸¥ immunologic or biochemical test

                 / urineÀ» ³óÃàÇÏÁö ¸øÇÏ´Â °æ¿ì

                   / 30%¿¡¼­ ÀϽÃÀûÀÎ serum creatinine Áõ°¡

                   / sepsis ¶§¹®¿¡ febrile infection½Ã blood culture ÇÊ¿ä

    ; ¢¾Ç¥17-12(p722)

Imaging Studies

# Renal Ultrasonography

    ; *Indication

           ¨ç acute febrile infection½Ã hydronephrosis¿Í renal, perirenal abscess¿Í °¨º°

           ¨è antibiotic therapy¿¡ ´ëÇÑ ¹ÝÀÀÀÌ Áï°¢ÀûÀÌ ¾ÊÀ» ¶§

           ¨é severely ill & toxic child

           ¨ê serum creatinine level Áõ°¡

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

    ; *acute pyelonephritisÀÇ Áø´ÜÀÌ ºÒÈ®½ÇÇÑ °æ¿ì À¯¿ë

    ; parenchymal filling defect

           - pyelonephritis Áø´Ü °¡´É

         - chronic°ú acute °¨º°Àº ºÒ°¡´É

# CT

    ; acute pyelonephritisÀÇ *definitive diagnostic method

# voiding cystourethrography

    ; *acute infectionÄ¡·áÈÄ ¾à 3ÁÖ° reflux Æò°¡À§ÇØ ½Ç½Ã

    ; ¢¾Indications

           - all males & females under 5yr with initial infection

           - older femaleat the time of second infection

    ; *female - radioisotopic VCUG

    ; male - radiographic VCUG

# ¡ÚVURÀÌ Á¸ÀçÇÑ´Ù¸é

           --> IVP with nephrotomography or radioisotope renal scanning ½Ç½Ã

    ; IVP

           - kidney size Æò°¡

         - °¡´ÉÇÑ calyceal blunting, ureteral dilatation, renal scarringÀÇ detection

    ; DMSA or glucoheptanate radioisotopic renal scan

        - *renal scarÀÇ detection

        - ÀåÁ¡

            / urographyº¸´Ù ´õ sensitive

         / IV contrast mediaÀÇ adverse effect Ùí

         / abdominal gas·Î ÀÎÇÑ urographyÀÇ ÇØ¼® ¹æÇذ¡ ÀÖ´Â °æ¿ì

         / infant¿Í young child¿¡°Ô ƯÈ÷ À¯¿ë

Differential Diagnosis

1. external genitaliaÀÇ inflammation

   : yeast, pinworm¿¡ ÀÇÇÑ vulvitis, vaginitis

     ´Ù¸¥ agent¿Í cystitsÀÇ °¨º°

2. viral, chemical cystitis : history, urine culture

3. hypoplastic, dysplastic kidney, vascular accident¿¡ ÀÇÇÑ small kidney¿Í

   chronic pyelonephritisÀÇ °¨º° : X-ray»ó À¯»ç

           +¡æ º¸Åë vesicoureteral reflux Á¸Àç

*4. acute hemorrhagic cystitis

    ; E. coli

    ; *adenovirus type 11 & 21

           - male¿¡ Òý

    - self-limited,

    - ´ë°³ 4ÀÏ Á¤µµ Áö¼Ó

5. eosinophilic cystitis

 1) symptom

    : hematuria, ureteral dilatation

      eosinophilic mass¿¡ ÀÇÇÑ bladderÀÇ filling defect

Treatment

# acute cystitis½Ã pyelonephritis ¾È°¡°Ô prompt treatment ÇÊ¿ä

; *symptomÀÌ severe ¡æ culteure¿Í µ¿½Ã¿¡ Ä¡·á ½Ç½Ã

 1) trimethoprim-sulfamethoxazole

        - 7¡­10ÀÏ

           - effective aganist E. coli                                

 2) nitrofurantoin

           - 5¡­7mg/Kg/day, 3¡­4 divided dose

    - effective against Klebsiella-Enterbacter organism

 3) amoxicillin : 50mg/Kg/day

; symptomÀÌ mildÇϰųª Áø´ÜÀÌ ÀÇ½ÉµÉ ¶§ culture ÈÄ Ä¡·á ½Ç½Ã

; culture °á°ú°¡ ºÒÈ®½ÇÇÑ °æ¿ì Àç½Ç½Ã

 ¡æ Ä¡·á½ÃÀÛÀü¿¡ catheterization or suprapubic aspirationÀÌ¿ëÇØ 2nd culture ½Ç½Ã

# pyelonephritis·Î »ý°¢µÇ´Â acute febrile infection ½Ã

    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¿¡ ƯÈ÷ effective

    ; sodium bicarbonate¿¡ ÀÇÇÑ *urine alkalinizationÀ¸·Î È¿°ú Áõ°¡

    3) bactrim

           ; Pseudomonas Á¦¿ÜÇÑ G(-)¿¡ effective

    4) *ciprofloxacin

           ; *18 ¼¼À̻󿡼­ resistant microorganismÀÇ alernative agent

¡Ø87 UTI Ä¡·á ÈÄ planning

    ; 1ÁÖÀÏÈÄ urine culture ÇÊ¿ä

           - urine sterility È®ÀÎ

    ; asymptomaticÀÌ¶óµµ 1¡­2³â°£ 3°³¿ù °£°ÝÀ¸·Î follow-up culture ½Ç½Ã

           - UTI´Â predisposing factor¾øÀ̵µ recur Òý

# anti-biotic prophylaxis

; agent

    - bactrim, nitrofurantoin

    - *1/3 dose once a day

; ¡Ø89,93,95 Indication

 ¨ç UTIÀÇ frequent recurrence

 ¨è persistent vesicoureteral reflux

 ¨é persistent cystitis - incontinence, frequency, urgency

 ¨ê neurogenic bladder

 ¨ë urinary tract stasis & obstruction

 ¨ì calculi

 ¨í reflux

# excellent long-term prognosis

¡Ú main consequence of chronic renal damage by pyelonephritis

    ; arterial hypertension , renal insufficiency

8. infrequent voiding, constipation¿¡ ´ëÇÑ education

9. antibiotic therapy & surgical or percutaneous drainage

   : renal & perirenal abscess or obstructed urinary tractÀÇ infection°¡Áø °æ¿ì

CHAPTER 493. Vesicoureteral Reflux

1. ¿øÀÎ : ureterovesical junctionÀÇ valvular mechanismÀÇ incompetence

2. why reflux harmful to kidney

  1) renal pelvis¸¦ voiding ½Ã higher vesical pressure¿¡ ³ëÃâ

    cf) normal renal pelvis pressure : 10 mmHg

  2) bacteria°¡ bladder·ÎºÎÅÍ kidney·Î passage°¡ ¿ëÀÌ

    ¨ç intrarenal refluxµµ scar¿¡ °ü¿©

     : intrarenal collecting system¿¡¼­

       collecting tubule·ÎÀÇ urine reflux & bladder dilatation

       ¡æ renal scar »ý¼º¿¡ impotant role

  ¨è megaureter-megacystic syndrome (fig 493-1)

     ¡æ  dilated ureter·Î massive reflux

     ¡æ incomplete bladder emptying

     ¡æ bladder·Î rapid return

     ¡æ progressive bladder emptying

3.¡Ø89 reflux nephropathy

  1) children°ú young adultÀÇ ESRFÀÇ *15¡­20%

  2) childrenÀÇ hypertensionÀÇ ÁÖ¿ä¿øÀÎ

Classification

Table 493-1

# Primary VUR

 *1) ¿øÀÎ : UVJÀÇ congenital anomaly

        ¨ç short intramural ureteral tunnel

            --> valvular mechanismÀÇ efficiency °¨¼Ò

        ¨è urteral orifice - lateral & cephalad direction

        ¨é 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) ¡Ø96 Duplicated system

  ¨ç *reflux ; more common in lower ureter

  ¨è lower ureter

           ; *enter bladder higher & more laterally

           ; less competent vlave

    ¨éureter°¡ divertivulumÀ¸·Î µé¾î°¡´Â °æ¿ì

           ; reflux more common

# Congenital Neurogenic Bladder

 1) ¿øÀÎ : myelomeningocele, sacral agenesis

 2) reflux : Ãâ»ý½Ã ¨÷¿¡¼­ ¹ß»ý ¡æ ¨ö¿¡¼­ posterior valve¿¡ ¹ß»ý

                               ¡æ °á±¹ ¨öÀ̻󿡼­ ¹ß»ý

# Intravesical Pressure°¡ Áõ°¡ÇÑ Reflux

 1) bladder outlet obstruction & vesical dysfunction ¹ß»ý

 2) infection ¾øÀ̵µ severe renal damage °¡´É

#¡Ú VUR Grade

   ¡ÚFig. 493-5

1) base

   ¨ç severity

   ¨è ureteral dilatation

      & calyceal deformityÀÇ Á¤µµ

2) ÀÇÀÇ : prognosis & therapy

Natural History

1. reflux garde¿¡ Áõ°¡¿¡ µû¶ó renal scarring, reflux nephropathy È®·ü Áõ°¡

2. intrarenal reflux½Ã scarringÀÇ risk Áõ°¡

3. *grade I & II reflux

 1) no ureteral dilatation

 2) vesicoureteral regionÀÇ anatomy °ÅÀÇ Á¤»ó

 3) child°¡ ¼ºÀåÇϸ鼭 *80%¿¡¼­ spontaneousÇÏ°Ô ¼Ò½Ç

4. grade III & IV reflux

 1) *spontaneous resolution ; 15%

5. familial tendency êó

  ¡Å 5¼¼ ÀÌÇÏÀÇ siblingµµ °Ë»ç

Clinical Manifestation

1. ¹ß°ß

 1) UTI evaluation ½Ã

 2) voiding dysfunction, renal insufficiency, hypertension or ´Ù¸¥ UTÀÇ ÀǽɵǴ pathology½Ã ½Ç½ÃÇÏ´Â VCUG ½Ã

Differential Diagnosis

1. history & radiograph

   : primary or secondary reflux °¨º°

 1) US : renal size

 2) isotopic parenchymal renal scan : scarÀÇ rule out

2. *continued voiding dysfunction in spite of antibacterial prophylaxis

    ; anticholinergics·Î Ä¡·á or lower urinary tractÀÇ urodynamic study

3. reflux, infection & voiding dysfunction°¡Áø ȯÀÚ

   : voiding dysfunctionÀÌ infection¿¡ ÀÇÇØ »ý±ä°ÍÀÎÁö infectionÀ»

     predisposingÇÏ´Â refluxÀÇ ¿øÀÎÀÎÁö °áÁ¤ÇϱⰡ ¾î·Æ´Ù.

Evaluation

    : refluxÀÇ diagnosis & gradingÈÄ ½Ç½Ã

 1. IVP & tomography : renal size, scar ¿©ºÎ È®ÀÎ

 2. blood pressure

 3. baseline creatinine clearance

 4. cystoscopy

Treatment

Primary reflux & reflex ass. /c complete duplication

1. ¢ÞGrade I & II reflux

 1) spontaneous resolutionÀÌ Òý

  ; infection ¹æÁö & antibiotics prophylaxis(bactrim, nitrofurantoin

    ; long-term treatment

 2) Ä¡·á½ÃÀ۽à *¸Å´Þ urine culture ½Ç½Ã

  ; *prophylaxis°¡ effectiveÇϸé 3°³¿ù °£°ÝÀ¸·Î culture

 3) asymptomatic bacteriuria & reflux

    ; harmful

  ; *symptomÀÌ ¾ø¾îµµ urine culture Áß¿ä

 4) radionulide VCUG ; *1³â ¸¶´Ù ½Ç½Ã

 5) renal US ; *1³â¸¶´Ù ½Ç½Ã ¡æ renal growth Æò°¡

 6) *radionuclide study»ó spontaneous cessation

  ; *another study in 3¡­6mo before discontinued AB therapy

           --> confirm

 7) ¢¾Indication of surgery

    ; failure of antibiotic prophylaxis

 8) *DMSA renal scan ; Ä¡·áÀÇ ¸¶Áö¸·¿¡ ½Ç½Ã

    ¡æ residual scar°¡Áø °æ¿ì blood pressureÀÇ long term follow up ÇÊ¿ä

2. ¡Ø89 Grade III reflux

 1) *new scar formation Àǽɽà periodic parenchymal scan

 2) *more than 50% of children ; surgery ½Ç½Ã

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

 1) *early surgery after a brief prophylaxis & reconfirmation

  ; esp. infant¿Í young child

    ¡ñ 5¼¼ÀÌÇÏ¿¡¼­ scar ºóµµ Áõ°¡

  ; *warrant

           - reflux¿¡ ÀÇÇÑ prenatal hydronephrosis°¡Áø newborn

             --> 1¼¼°æ spontaneous improvement

Secondary reflux

1. *duplication ÀÖ´Â °æ¿ì ; A¿Í µ¿ÀÏ

2. *periureteral divertivulum ÀÖ´Â °æ¿ì ; early surgery

3. severe cystitis ÀÖ´Â °æ¿ì ; primary causeÀÇ remove

4. iatrogenic ; surgery

Results Of Surgery

1. *Cohen operation ; 97%ÀÇ ¼º°ø·ü

2. antireflux surgeryÀÇ complication

 1) refluxÀÇ persistence

 2) distal ureterÀÇ obstruction

3. surgery ÈÄ ¹Ý´ëÃø reflux´Â ´ë°³ ÀÚ¿¬ ¼Ò½Ç

´Ù¸¥ Ä¡·á ¹æ¹ý

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

    ; ªÀº ±â°£ effective

2. *subureteric injection ; collagen, silicone particle

3. success rate ; 65-70%

¡Ø89 Cx of VUR

    ; UTI

    ; renal scar & atrophy

    ; hypertension(20%)

    ; CRF(reflux nephropathy)

Chapter 494 Obstruction of the urinary tract

; ¿øÀÎ - congenital(children¿¡¼­´Â 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

¨ç urethral obstruction in fetus

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

¨è Bilateral ureteral obstruction or urethral obstruction

    ; cause *oligohydroamnion and pulmonary hypoplasia

    ; severe obstructive uropathyÀÏ °æ¿ì ½Å»ý¾Æ¿¡¼­ Áï°¢ÀûÀÎ ¹ÝÀÀÀº renal damageÁ¤µµº¸´Ù pulmonary insufficiency¿¡ ´õ °ü°èµÈ´Ù.

¨é ±×¹Û¿¡ obstructionÀÌ reliefµÈ ÈÄ¿¡µµ polyuria, dilute urine, chronic acidosis(serum creatinine levelÀº Á¤»ó), hypertrophy & dilatation in the bladder and collecting system postobstructive diuresis°¡ »ý±æ ¼ö ÀÖ´Ù.

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 °í·Á

; *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 painÀÌ ÀÖ´Â all children¿¡°Ô¼­ intial study·Î abdominl US

Imaging Studies

1) US

    ¨ç screening method ¨è evaluate renal size, parenchymal thickness, ¨é determine ureteral dilatation, ¨ê bladder evaluation ±×·¯³ª acute ¶Ç´Â intermittent obst. ¶§´Â minimal dilatationµÇ¹Ç·Î misleadingÇÒ ¼ö ÀÖ´Ù.

2) DTPA

    ; gross estimate of different renal fuction dilatation

    ; renal pelvis¿¡ isotopeÀÖÀ» ¶§

    ¡æ Furosemide adm unobst ÀÖÀ» ¶§ : prompty excretes isotope, obst ÀÖÀ»    : no or slowly excrete

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

 

3) IVP

    ; preliminary radiography of the abdomen ¡æ calculi, spinal abnormalities,

       abdominal & intestinal gas patternÀ» º»´Ù.

  ; postvoiding film ¡æ residual bladder urineÀ» º»´Ù.

  ; obstruction side¿¡ radiopaque mediumÀÌ ³²¾ÆÀÖ´Ù.

  ; *intermitten obstruction

           - *painµîÀÌ À¯¹ßµÇ´Â acute phase¿¡ IVPÇÏ´Â°Ô °¡Àå À¯¿ëÇÏ´Ù.

Pressure Flow Studies (Whitaker test)

; percutaneous·Î renal pelvis¿¡ needle ³ÖÀº ÈÄ radiopaque dye perfusion ÈÄ renal pelvis¿Í bladder»çÀÌ ¾Ð·ÂÂ÷ ÃøÁ¤

    - *20cm of water ÀÌ»ó pr. grad½Ã obst.

Voiding cystourethrography

¸ðµç ureteral dilatation½Ã

¨ç VUR º¸±âÀ§ÇØ ½ÃÇà

¨è urethral obst, esp. post urethral valve

¨é infravesical obstruction palpable bladder

        ureth, catheterizationÀÌ Àß µÇÁö ¾ÊÀ¸¸é ureth strictureÀÇ½É obstructive ureth,           lesion other than valve.

Specific Type of Urinary tract Obstruction

Hydrocalycosis

¨ç infundibular obst.ÀÎÇÑ localized calyceal dilatation

¨è ¿øÀÎ ¦®developmental

        ¦±2¡Æto infl. process : exp. *Tbc

¨é cong. obst. due to stenosis or extrinsic vas. compr.½Ã Áõ»ó : pain(obstÀÇ surgical correction½Ã »ç¶óÁü)

¨ê Dx. : IVP

Obstruction of the UPJ

; ¡Ø93 ¡ã obstructive lesion in children

    - *¡ãoften by cong, stenosis of UPJ

    - others causes

           / ureteral kinks, fibrous band, aberrant vv.

; ¢ÀUPJ obstructionÀÇ °¡Àå ÈçÇÑ Áõ»óµé

  - 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 - Ãâ»ý 3ÀÏÀÌÈÄ ½Ç½Ã(oliguria°¡ dilatationÀ» maskingÇÒ ¼ö Àֱ⶧¹®)

           - ¡ÚÁ¤»óÀÌ¶óµµ 3 or 6 mo¶§ repeat

    ; mild to moderate hydronephrosis

           - observation & F/U

    ; marked dilatation, renal parenchymal thin

           - isotope renogram

           - normal renal fuctionÀ̸é F/U

                   / no improvement - *diuresis renogramÀ» 6-12mo

    ; ¢¾surgical repair Ix

       - involved kidney function decreased

           - bilateral involvement or solitary kidney

           - diminished overall renal function

           - palpable mass

¨ì Sx°¡Áø old children

  ¡æ IVP, ¸¸¾à IVP¿¡¼­ Àß ¾Èº¸Àδٸé US.

¨í D/Dx

    i) megacalycosis

    pelvis, ureteric dilatation¾øÀÌ 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

¨î Tx. surgical pyeloplasty

¨ï Follow up ½Ã diuresis renography

Midureteral Obstruction

¨ç 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 ß¾ Rt.ureter°¡ 3rd lumbar vert. level¿¡¼­ medially deviated.

¨è ÀÌ¿ÜÀÇ¿øÀΠ    ¦® retroperitoneal tumor

                  ¦· fibrosis by surgery. infl. process (CGP)

                  ¦± radiation

Ureteral ectopia

¨ç mesonephric duct migration path ñé ¾î´À°÷¿¡³ª ¹ß»ý°¡´É

¨è drain single collecting system

    ; commonly belongs to upper moiety of duplicated collecting system

¨é ureteral orifice of upper collecting syst.

    ; always caudal to ureteral orifice of lower collecting system

¨ê male

    i) usually *single ectopic ureter

    ii) bladder neck, urethra above external sphinncter seminal vesicle, vas deferensµîÀ¸·Î À¯ÀÔ

    iii) high grade obstruction, UTI or epididymitis Sx.

    iv) ¹Ý´ëÃøÀÌ normal½Ã nephroureterectomy

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

¨ë 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 µ¿¹Ý

Ureterocele

; congenital cystic dilatation of the distal ureter ¡æ bladder³»·Î protrusionµÇ¾î pin point

; ¿© ¡µ³²

; simple ureterocele

    ¥¡) non-duplicated collecting system orifice´Â Á¦À§Ä¡(bladder³»¿¡ êó)

    ¥¢) IVP ß¾

           ; varying degree of ureteral & calyceal dilatation, round filling defect in bladder delayed film¿¡ ureter°¡ cleanred visibleÇϰųª & filling of contrast mediaÀÏ ¼öµµ ÀÖ´Ù.

    ¥£) Tx.

    transurethral incision of uretercele (¶§·Î VUR ÃÊ·¡ÇÏ¿© ureteral reimplant ÈÄ¿¡ ʦ)

    open excision of ureterocele & reimplartation

    upper tract dilatation ¾ø´Â small ureterocele½Ã No Tx.¡æ pr. flow study & diuresis

; ureteral duplication µ¿¹Ý

    - ÁÖ·Î *upper renal moiety¿¡¼­ drainÇÏ´Â ureter°¡ ectopic ureterocele Òý

    - upper renal moiety : poor fuction, cong.obst.À¸·Î dysplastic

    - lower renal moiety : frequently reflux

    - ectopic ureterocle may extend submucosally into post. urethra

           / ´ëºÎºÐ unilateral

; UTI

; bladder neck obst.

; urinary retention, reflux µîÀÇ ¾ç»óÀ» º¸ÀÓ

; IVP

    ¨Í large filling defect in bladder

    ¨Î characteristic finding of duplication

    ¦® upper collectin syst. - poor or absent function.

    ¦± lower collecting syst. - caudal displacement

           ¡æ "drooping lily app"

; Tx

    ¨Í excision of upper collecting system : partial nephrectomy & ureterectomy

    ¨Î small ectopic ureterocele with ipsilateral duplicated ureter¿¡ low or no reflux½Ã : No Tx.

    ¨Ï large ureterocele with ipsilateral lower ureter¿¡ high grade reflux½Ã : excision of ureterocele & reimplantation of remaining ureter £« partial upper moiet

    ¨Ð obstructing, ureteroceleÀ» °¡Áø acutely ill, septic infant½Ã involved collecting systemÀÇ drainage by transureterally or by percut. nephrostomy°¡ ÇÊ¿äÇÔ

Megaureter

¨ç dilated ureter

¨è careful Hx. PE. voiding cystourography µîÀÌ 2¡Æmegaureter¿ÍÀÇ DDxÀ§ÇØ Áß¿ä

¨é º¸Åë distal ureter°¡ more dilatedµÇ¾î ÀÖ°í bladder¿ÍÀÇ junction¿¡¼­ °©ÀÚ±â

   tapperingµÈ´Ù.

      ¨ê obst. megaureter¿Í nonobst. megaureter DDxÀ§ÇØ dieuresis renogram press

          flow study µî ÇØ¼­ obstÀ̸é opÇØÁØ´Ù.

Table 494-2

¡Ø91½½ Prune-Belly Syndrome

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

; 1/40,000

; *97% male

¢Þ Characteristics

  - deficient abdominal muscle

  - undescended testes

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

         / ureter & upper partÀÇ 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.(£«)

; Ä¡·á

    - obstructionÀÌ ¾øÀ¸¸é UTI¿¹¹æÀÌ Ä¡·áÀÇ ¸ñÇ¥´Ù.

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

    - orchiopexy : 1¼¼À̳»

; ¿¹ÈÄ

    - ¡Úpulmonary & renal dysplasiaÁ¤µµ¿¡ ´Þ·ÈÀ½.

    - 1/3Àº »ç»êµÇ°Å³ª Ãâ»ýÈÄ ¼ö°³¿ù ³»¿¡ pulmonary CxÀ¸·Î Á×À½

  - Àå±â »ýÁ¸ÀÚÀÇ °æ¿ì 1/2°¡ CRF·Î °¡¸ç ÀÌ °æ¿ì  KTPÈÄÀÇ °æ°ú´Â ¾çÈ£ÇÏ´Ù.

Bladder Neck Obstruction

¨ç ¿øÀÎ

 2¡Æto ectopic ureterocele.

 bladder calculi, prostatic tumor(rhabdomyosarcoma)

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

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

¨é 1¡Æbladder neck obst is exceptional in males

¡Ø94 Posterior Urethral Valves

; urethral valve ñé *¡ãcommon type

; sail-shaped membrances with eccentric opening

    - *verumontanum¿¡¼­ ½ÃÀÛÇÏ¿© 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 ¡è 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 ÈÄ ¨Í percut. nephrostomy

           ¨Î renal analysis stableÇÑ ÈÄ evalutation & Tx

# incontinence°¡ ÁÖ¼ÒÀÎ °æ¿ì

    ; primary valve ablation

# *VUR

    ; expectant Tx. with AB therapy

           --> *relux°¡ 1 yr ¡è & kidney function ¡é

           --> surgical correction

# *Tx.ÈÄ¿¡µµ urinary incontinence 50%

    ¥¡) surgical damage of sphincter

    ¥¢) dilatation of prostatic urethra

    ¥£) poor bladder compliance

    ¥¤) polyuria from renal damage

    --> *³ªÀ̵é¸é (exp. after puberty)È£Àü

; *Px in newborn

    - depend on pul.hypoplasia & renal function recovery potential

    - neonateÀÌÈÄ¿¡ 1/3¿¡¼­ ¾î´À Á¤µµÀÇ Renal insuff.ÀÖ¾î renal transplantation ¿äÇÔ

Urethral Stricture

¨ç male

    ¥¡) congenital : rare

  ¥¢) urethral trauma

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

       ; accidental (straddling injury, pelvic fracture)

    ¥£) Sx.

           ; ¨Í bladder instability ¨Î hematuria ¨Ï dysuria

    ¥¤) Dx

           ; voiding film of IVP RGCU, endoscope

    ¥¥) Tx

           ; short - endoscopic dilatation & internal urethrostomy

           ; long - urethroplasty

¨è female

    ; true is exceptional

    ; urethral ring ¡æ urethral obst. & UTI ÃÊ·¡

Anterior Urethral Valve & Urethral Diverticula In Male

¨ç µÎ °³ µ¿¹Ý ʦ

¨è Dx : VCUG

¨é diverticula´Â dilatation of Cowper gland & duct·Î »ý°¢

Male Urethral Meatal Stenosis

; 4¼¼ ÀÌÇÏ - 8F ¡é, 10¼¼ÀÌ»ó - 10F ¡é

    ¡æ true meatal stenosis

; neonatal circumcision ÈÄ glansÀÇ ammoniacal dermatitis ÀÎÇØ ¹ß»ý

; hypospadia ½Ãµµ ʦ

; Tx. : meatoplasty

Chapter. 495. Anomalies of the Bladder

Bladder Extrophy

; ºóµµ

- 1/40,000 birth.

- boys ¡µgirls

Clinical Manifestations

¨ç mesodermÀÌ cloacal membraneÀ¸·Î caphaled extension ¾ÈµÇ¾î õó

¨è classic °æ¿ì

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 rami¶§¹®

¨é untreated °æ¿ì

¥¡) total urinary incontinence

¥¢) bladder Cancer ¡è, esp. adenoCa.

Treatment

; Ãâ»ý Á÷ÈÄ bladder¸¦ silastic shield³ª ´Ù¸¥ Àû´çÇÑ plastic dressingÀ» ÇØ¼­ desication of bladder¸¦ ¸·´Â´Ù.

; *48hrÀ̳» closure of the exstrophied bladder

    - before permanant changes in the bladder walls

    - flexibility of pelvic joint ÀÖÀ»¶§ ½ÃÇà

; initial op. ¸ñÀû

    ¥¡) precise closure of the bladder & prostatic urethra in male

    ¥¢) elongation of the urethral plate & penis

    ¥£) closure of the abd. wall

; ´ëºÎºÐ ÈÄ¿¡ VURµ¿¹Ý

; epispadia operation

    - 1-2yr

; Tx of incontinence by bladder neck reconstruction

    - *after rectal control

Prognosis

¨ç Ä¡·áÈÄ¿¡ upper urinary tract¿¡¼­ 15%ÀÌÇÏÀÇ deterioration. 70% ÀÌ»óÀÇ continence¸¦ ȹµæÇÑ´Ù.

¨è À̹ۿ¡ artificial sphincter¸¦ ¸¸µé°Å³ª cytoplasty, abdominal stoma, ureterosigmy µîÀ» Çʿ信 µû¶ó ÇØÁֱ⵵ Çϳª ÀÌ·± op´Â ch. pyelonephritis, urinary tract damage ÀüÇØÁú ºÒ±ÕÇü, Àå±â°£ °æ°ú ÈÄ carcinoma ÀÌÇà µîÀÇ À§Çè·üÀÌ ÀÖ´Ù.

Other Exstrophy Anomalies

¨ç cloacal exstrophy

  severe abn. of colon, rectrum, short bowel

¨è epispadia

   distal epispadia : reconstruction of urethra & penis

Bladder Diverticula

; *´ë°³ UVJ¿¡ È£¹ß ¡æ VUR

¨ç ¿øÀÎ

¦® congenital

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

¨è Tx.

    ; small : none

    ; large ¡æ ineff. voiding, residual urine stasis. UTI½Ã excision

   [urachal anomaly] : male ¡µfe

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)

; ¡Útwo ¡ãimportant 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 °¡Áø PtÀÇ ¸ðµÎ°¡ neurogenic bladder ÀÖ´Â °ÍÀº ¾Æ´Ï¹Ç·Î urodynamic study(cytometrography sphincter electromyography)ÇÏ¿© R/O½ÃÄÑ¾ß ÇÔ.

Urinary incontinence

; ¿øÀÎ

    ¨Í total or partial denervation of sph.

    ¨Î bladder hyperreflexia or poor bladder compliance

    ¨Ï chronic urinary retention

3) Tx

¨ç Neurogenic bladder °¡Áø ȯÀÚ Ä¡·áÀÇ ¸ñÀû

           protection of upper urinary tract

           eventually providing continence

¨è newborn with low intravesical pr. & No VUR

          expectant therapy

           US & radioisotopic cystogram f-up

           recurrent UTI½Ã prolonged antibiotic therapy

           urodynamic study at 6 mo.¿¡ repeat

¨é VUR(+), elevated intravesical pr. (grade ¥°&¥±)

         ¦® Antibiotics prophylaxis intermittent catheterization

         ¦² anticholinergic drug(oxybutynin 0.4mg/kg/day Sig 1/2)p.o½Ã 40%¿¡¼­ È£Àü

         ¦± severe reflux ÀÖÀ» °æ¿ì corrective surgery

   incontinence Tx

       ¥¡) sphincter tone ÃæºÐÇÏ´Ù¸é ¡æ 4hr °£°ÝÀ¸·Î intermittent catheterization

       ¥¢) 7¡­8 yr. old who have adequate manual dexterity ¡æ intermittent self                  catheterization

       ¥£) intermittent catheterization µ¿¾È antibacterial prophylaxis ¾µ ¼ö ÀÖ´Ù.

     ¨ë bladder capacity¿Í compliance´Â ÃæºÐ. but urethral resistance°¡ ³·À» ¶§

            implanatation of artificial sphincter

     ¨ì Enterocytoplasty

     ¥¡) Tx : neurogenic bladder bladder exstrophy, post urethral valve µî bladder

         capacity¸¦ ÁÙÀÌ´Â Áúȯ

      ¥¢) Rt. or Lt. colon À̳ª ileumÀ» ³¢¾î ³ÖÀ½

      ¥£) ÀÌ °æ¿ì intermittent cathÇÒ ¶§  colonÀÇ G(-) bacteria ³ª¿À¹Ç·Î Sx ÀÖÀ» ¶§¸¸

          UTI Tx

    ¥¤) hypermetabolic acidosis ¿Ã ¼ö ÀÖÀ¸¹Ç·Î Tx (enteric mucosal surface ñé urine°ú Á¢ÃËÇÏ´Â ºÎºÐ¿¡¼­ ammonium, choloride. H+À̿ Èí¼ö, K+ lossÇϹǷÎ)

    ¥¥) ÀÌ·± Cx ¸·±â À§ÇØ ch. renal insufficiency ȯÀÚ¿¡°Ô´Â colon ´ë½Å gastric segment¸¦ »ç¿ëÇϱ⵵ ÇÔ.

    ¥¦) CxÀ¸·Î perforation, peritonitis, potential for malignancy µîÀÌ ÀÖÀ½.

Chaper 497. Voiding dysfunction

Nocturnal Enuresis

¨ç 5¼¼ childÀÇ 20%

    ; ±× ÀÌÈÄ ÇØ¸¶´Ù 15% °¨¼ÒÇÏ¿© adult¿¡¼­´Â 1%ÀÌÇÏ

¨è ¿øÀÎ

    ; *delayed maturation of the cortical mechanism

    ; alteration of the circadian rhythm of ADH secretion

¨é     primary ;  night-time continence period (-)

           secondary ; formely dry child¿¡¼­ emotionally disruptive event ÈÄ¿¡ ¹ß»ý

¨ê male(3¹è) ¡µfemale

¨ë FHxÀÖ´Ù.

¨ì neurologic & spinal abnormality, fluid intack, urinary output

¨í urinalysis

    ; Bacteriuria

    ; specific gravity & osmolarity after overnight fast

¨î daytime sx. (-). P/E, UA, urine culture normal½Ã ¡æ No further evaluation

    needed

¨ï Tx

        ¥¡) reassurance. self - limited, eliminate punitive measure

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

             not successful

       ¥£) imipramine drug ²÷À¸¸é ´ë°³ recur

       ¥¤) desamino - D - arginine vasopressin : 50%

       ¥¥) Tx. of conditioning with alarm.

Unstable Bladder

¨ç voiding dysfunction not related to neurologic abNl or dys Fx

¨è Sx : frequency, urgency, blader pain¾øÀÌ diurnal urinary incontine

¨é Study

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

¨ê Fx

    UT pathology¾øÀ» ¶§ oxybutynic & other anticholinergic

Non-Neurogenic Neurogenic Bladder

¨ç non neurogenic destrusor/sphincter dys°ú °°Àº ¸»

¨è neurologic abnormal ¾øÀÌ voiding ½Ã ext sphincter°¡ relax(-)

¨é Hx»ó UTI Constipation Hx ÀÖ´Ù.

¨ê P/E»ó

VUR trabeculated bladder, decreased urine flow rate intermittent pain

¨ë toilet training°ú °ü·Ã

¨ì Tx

         ¥¡) treatment of reflux & antibiotics prophylaxis

         ¥¢) behavioral modefication & encouragement of relaxation during voiding

         ¥£) Buifeedback

         ¥¤) intermittent catheterization & anticholinergic drug

         ¥¥) diazerpam

¨í Px : poor

Infrequent Voiding

¨ç UTI¿Í µ¿¹ÝµÈ micturition ¶§ common disorder

¨è girls¿¡ Òý, FHx°¡ ÀÖ´Ù.

¨é ¿øÀÎÀº behavioral

¨ê Tx : antiotics prophylaxis.

               encourage to frequently void

               complete emptying by double voiding

Other Cause Of Incontinence In Females

table 497-1

1) Ureteral ectopia

¨ç ´ë°³ female¿¡¼­ duplicated collection system°ú µ¿¹Ý

¨è Nl voiding pattern º¸À̸鼭 constant dribbing of urine

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

¨ê Tx : partial nephroureterectomy

Chapter 498. Anomalies of the penis and urethra

Hypospadiasis

¨ç ºóµµ : 1/500 newborns.

     ¨è mildestÇÑ °æ¿ì uretheral meatus°¡ glansÀÇ vent·Î openÇÏ´Â °Í¿¡¼­ºÎÅÍ severeÇÒ    ¼ö·Ï penis°¡ ventral·Î curvedµÇ¸é¼­ penile urethra°¡ ª¾ÆÁø´Ù. (chordeeµ¿¹ÝµÇ¾î¼­)

¨é meatusÀÇ opening : ventral aspect of glans, penoscrotal juction, perineum

¨ê 10%¿¡¼­ undescended testes µ¿¹Ý

¨ë Ä¡·áÀÇ ¸ñÀû mild case : cosmatic

                     severe case : ¼­¼­ voidingÇÒ ¼ö ÀÖ°Ô

                     future Nl sexual function

                     avoid psychogenic consequence

     ¨ì ideal age for repair´Â controversialÇÏÁö¸¸ ÇöÀç 18mo.ÀÌÀü¿¡ ½ÃÇà ¡æ toilet              training(1¼¼Àü)¿¡ ½Ç½Ã newborn period¿¡ ½ÃÀÛ (routine circum ÇÇÇÒ °Í)

Agenesis and Micropenis

A. agenesis : rareÇÏ¿© anorectal and rectal anomalies¿Í µ¿¹Ý ¸¹ÀÌ ÇÔ.

B. Micropenis

             normal NB penic : 3.5¡¾0.7cm

             primary

             secondary testicular failure after complete morphogenesis

              anencephaly

              pituitary agenesis

              Kallmann, Noonan, Prader - Willi synd

              rudimentary testes

              dwarfism

              materna hormone administration

    Tx. : ¨ç hormone stimulation

         ¨è rearing as female with later genital reconstruction

Phimosis & Paraphimosis

   phimosis : prepuce°¡ retractionµÉ ³ªÀÌ(3¼¼)°¡ Áö³ªµµ retractionµÇÁö ¾Ê´Â °ÍÀ¸·Î inflamationÀÇ sequal·Î µÇ´Â °Í°ú congenital·Î µÇ´Â °ÍÀÌ ÀÖ´Ù.

   Paraphimosis : phimotic preuce°¡ coronal sulcus µÚ·Î retractµÇ¾î reduceµÉ ¼ö ¾øÀ» ¶§, ÀÌ ¶§ edema severe pain È£¼ÒÇϸç Ãʱ⠹߰ßÇϸé heavy sedationÀ̳ª Àü½Å¸¶Ãë ½ÃŲ ÈÄ lubricationÇØ¼­ foreskinÀ» reduce½ÃŲ´Ù.

Chapter 499. Disorders and Anomalies of the Scrotal Contents

Undescended Testes

Andescended & Ectopic testes

1) Cryptorchidism

¨ç true undescended testes : normal path of descent µû¶ó Á¸Àç

   processus vaginalis : patent

        ectopec testes : inguinal canal ÅëÇØ complete descent ÇßÀ¸³ª 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(2000¡­2500g) - 17%

    ; 900gm¡é - 100%

# 7th mo. of gestation½Ã testicular descent õó

# *spontaneous testicular descent dose not occur after 1yr

# Complication

         ¥¡) infertility in adulthood

         ¥¢) tumor development : 20¡­44% ¡è risk

         ¥£) associated hernia

         ¥¤) torsion of the cryptorchid testis

         ¥¥) psychologic effects of an empty scrotum

# bilateral cryptorchidism : 30%

     ¨ë undescended testis´Â ¿ø·¡ Á¶Á÷ÇÐÀûÀ¸·Î´Â Á¤»óÀ̳ª failure of developement and      atrophy°¡ ÀϾ 3rd or 4th decade of life¿¡¼­´Â maligrant testcular°¡ µÉ È®·üÀÌ       20¡­44% Áõ°¡µÈ´Ù. ÀÌ Áß¿¡¼­ M/C tumor´Â Seminoma(60%)ÀÌ´Ù. (Á¤»ó testes¿¡       ¼­´Â 30%)

     ¨ì true undescended testes¿¡¼­ indirect inguinal hernia°¡ ¹Ýµå½Ã ³ªÅ¸³ª¸ç À̹ۿ¡

        torsion infarctionÀÌ µ¿¹ÝµÉ ¼ö ÀÖ´Ù.

Treatment

# unilataterl cryptorchid testes

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

# bilateral

    ; palpable testis

           - unilateral °ú µ¿ÀÏÇÏ°Ô Ä¡·á

    ; not palpable testes

           - *hCG stimulation test

                   / testosterone¡è½Ã abd. exploration & orchoipexy

                   / negative responseÀÏ ¼öµµ ÀÖ´Ù

      ¥£) HCG or LHRH

            surgical Tx. replacement ¸øÇÔ

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

Retractile Testes

   exaggerated cremasteric reflex·Î inguinal canal³»·Î retract

   puberty ¶§ scrotumÀ¸·Î ³»·Á¿È

   No Cx

Abscent testes

   non-palpable testesÀÇ 200%

¦®congenital : quite rare

¦±acquired : vascular accident

Torsion Of The Testis Or Appendices

¨ç 6»ì ÀÌÇÏ¿¡¼­ acute scrotal pain swellingÀ» È£¼ÒÇϴ ȯ¾ÆÀÇ 40%¿¡¼­ testicular         torsion ÀÌ´Ù.

¨è ¿øÀÎ ¡æ abnl. fixation of the testis to the scrotal envelope

¨é Sx ¡æ acute scrotal pain, tenderness, swelling absent cremasteric reflex

¨ê DDx

¥¡) incarcerated hernia ¡æ ÀÌ ¶§´Â inguinal area¿¡ swelling ÀÖ´Ù.

      ¥¢) torsion of one of the 0testicular or epididymal appendicies ¡æ ÀÌ ¶§´Â pain

          swelling Àû°í testisÀ§¿¡ blue dot º¸À̰í local tenderness

¥£) epididymitis :    13ÀÌÈÄ¿¡´Â ¹Ýµå½Ã R/O

       18¼¼ ÀÌ»ó½Ã M/C cause of scrotal pain & swelling

       ÀÌ ¶§ UA°¡ abnormal

¨ë Áø´Ü¹ý : ultrasonogram, color doppler, ultrasound, isotope scan

¨ì Tx

   prompt surgical exploration 6hÀü¿¡ retorsion½Ã 90%¡è¿¡¼­ survival of gonad contrlateral testes´Â scrotum¿¡ fixation

Varicocele

; pampaiform venous dilatation due to valvular incompetence of spermatic vein

; *predominantly Lt side

    - bilateral 10%

; rare before 10yr, adult 15%

¨é subfertility, decreased size of testis ʦ

¨ê large½Ã painful, fixed varicocele : retreoperitoneal tumorÀǽÉ

¨ë surgery : pain relieve, normal testis development ¸ñÀû

¨ì varicocelÀÇ early correction°ú fetility¿¡ ´ëÇÑ °ü°è´Â ¾ÆÁ÷ ¹àÇôÁöÁö ¾Ê¾ÒÀ½.

Hydrocele

¨ç tunica vaginalis¿¡ fluid accumulation transillumination (+)

¨è small hydroceleÀº 1yr³» ¼Ò½Ç

¨é communicating hydrocele (vary in amount)½Ã´Â indirect inguinal herniaó·³ 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

 

   ¨ç Trauma·Î ÀÎÇÑ accidental injuryÀÏ °æ¿ì skeletalÀ̳ª CNS µî ´Ù¸¥ °÷Àº trauma¿Í    µ¿¹ÝµÉ °æ¿ì°¡ ¸¹°í ¸¸¾à kid¸¸ Trauma ¹Þ¾Ò´Ù¸é ¼±ÇàÇÏ´Â kid anomaly°¡ ÀÖÀ» È®·ü    ÀÌ ¸¹´Ù. (horseshoe kid, renal ectopia, hydronephrosis)

¨è Evaluation of Pt.

¥¡) bladder cath. : except bleeding from urethra

         urethral injury½Ã retrograde urogram

        Tx. : suprapublic cystostomy drainage until resorption of hematoma ¡æ                       urethroplasty

     

 

      Cx. :  erectile impotence  urethral stricture  urinary incontinence

¥¢) cystography

¥£) IVP

¥¤) renal angiogram, CT

¨é Renal injury ºÐ·ù

minor - parenchymal ¼Õ»ó, ´ëºÎºÐ ÀÌ°Í op. ÇÊ¿ä¾ø´Ù.

major - collecting system, renal pedicle ¼Õ»ó

¨ê infant & child¿¡¼­ Kidney¿¡ injury È£¹ßÀÌÀ¯

   proportionally greater size

   less sturdy fatty & fascial envelope

   greater flexibility of overlying lower ribs

Chapter 501. Urinary Lithiasis

¨ç ±âÈÄ, À½½Ä, »çȸ °æÁ¦Àû ¿ä¼Ò¿Í ¿¬°üµÇ¹Ç·Î geographic variationÀÌ ÀÖ´Ù.

¨è Sx. ¼ø¼­

¥¡) gross or microscopic hematuria

¥¢) abd. pain

¥£) flank or back pain

¥¤) UTI sx

¨é plain abd. ß¾

¥¡) faintly seen ¨Í cystine stone ¨Î infectious stone(Struvite)

¥¢) radiolucent stone

  ¨Í uric acid ¨Î 2.8-dihydroxyadenine ¨Ï xanthine calculi ¡æ US, IVP, CT·Î Áø´Ü.

¥£) complete functional & radiographic study 1/4¿¡¼­ VUR(+)

¥¤) chemical & crystallographic analysis

¨ê laboratory tests suggested to evaluate urolithiasis

    table 501-1

¨ë classification of urolithiasis

    table 501-2

Calcium Stones

; calcium oxalate

    - *¡ãcommon urinary caculi

¨ç ¿øÀÎ

         ¥¡) idiopathic urolithiasis

         ¥¢) associated with hyperclacemia

              ¨Í hyperparathyroidism

              ¨Î sarcoidosis

              ¨Ï immobilization

              ¨Ð hypervitaminosis D

              ¨Ñ idiopathic, isolated form

         ¥£) normocalcemic hypercalciuria

             furosemide adm.

             uncontrolled distal RTA

             TPN, alkalosis

             idiopathic

             renal tubular calcium leak¡æ 2ndary hyperparathyroidism.

                                     ¡æ hyperabsorption of calcium

            (primary intestinal hyperabsorption of calcium)

¨è urinary calcium measurement

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

          urinary Ca/creatinine ration ¡µ0.25mg/mg

¨é calcium oxalate stone

          small bowel ds. & malabsorption

           ¡æ intestinal hyperoxaluria 1¡Æhyperoxaluria (type 1 or 2)

Cystinuria

dibasic amino acids(cystine, ornithine arginine, lysine,)ÀÇ transportÀÇ inborn error½Ã

¡æ excessive urinary excretion of products

¡æ formation of calculi

Struvite Stones

urea splitting organism

(¨ç Proteus, occasionally ¨è Klebsiella ¨é E. coli ¨ê Pseudomonas) ¡æ urine alkalinazation,       excessive ¡æ magnesium ammonium phosphate (struvite), calcium phosphate

       aluminum phosphate Ä§Âø

Uric Acid Stones

¨ç ´ë°³ hyperuricosuria with or without hyperuricemia

            radioluscent stone

           persistent acid urine & urate crystalluri½Ã ÀǽÉ

      ¨è ¿øÀÎ

        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 ¹æÁö

¨ç adequate state of hydration & diuresis

¨è alteration of pH

        ¥¡) cystine, uric acid pH 7.5ÀÌ»óÀ¸·Î alkalinazation½Ã solubility ¡è

        ¥¢) struvite½Ã acidic urine

 

¨é 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

¨ê ¥¡) cellulose phosphate ¡æ primary absorptive hypercalciuria

         ¥¢) pyridoxine ¡æ hyperoxaluria

         ¥£) salts of phosphate, citrate, magnessium ¡æ calcium oxalate solubility Áõ°¡

# surgical Tx

    ; *ÃÖ±Ù¿¡´Â °ÅÀÇ ÇÏÁö ¾ÊÀ½

    ; obstruction of collecting system, pain, bleeding, perpetuating infectionÀÌ ÀÖÀ»¶§ ½Ç½Ã

# endoscopical or percutaneous access to kid

# extracorporeal shock wave lithotripsy

    ; *renal & ureteral stone ¸ðµÎ °¡´É

    ; *succes rate - more than 75%