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12. UroPed (1583) > Pediatrics and Pregnancy > Flashcards

Pediatrics and Pregnancy Flashcards

1
Q

2 types of Cryptorchidism:

A
  1. Congenital – extrascrotal from time of birth
  2. Acquired – intrascrotal at birth but ascent subsequently
    1. Ascending
    2. Entrapped – after prior inguinal sx
    3. Retractile – vigorous cremasteric reflex
    4. Atrophic – volume loss after inguinal or testicular sx d/t prolonged position in extrascrotal location/developmental failure
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2
Q

Extrascrotal locations of UDT

A
  1. Prescrotal – above or at scrotal inlet
  2. Superficial Inguinal Pouch – distal and lateral to external ring, anterior to rectus muscle
  3. Prepubic – at external ring
  4. Canalicular – within the inguinal canal
  5. Ectopic – most commonly perineal, perirenal, femoral, peripenile, contralateral scrotal
  6. Abdominal – peeping through or proximal to internal inguinal ring, near bladder, iliac vessels, or kidney
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3
Q

What are some risk factors for UDT?

A
  1. Prematurity (15–30%, term 1-3%)
  2. Low Birth Weight (<900 g → 100%, 2700-3600 g → 3%)
  3. Genetic Susceptibility
  4. Maternal smoking
  5. Maternal ETOH consumption
  6. Maternal analgesic consumption
  7. Maternal Estrogen exposure
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4
Q

Why SHOULD you obtain GH at initial evaluation of UDT?

A

Guideline Statement 1

There are 2 phases of descent

  1. Transabdominal – 22-25 weeks GA (located at internal ring)
  2. Inguinoscrotal – androgen dependent – 25-30 weeks GA

**Higher in premature infants and low birth weight–adjusted GA important

**Unlikely to descend after 6 months

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5
Q

Who SHOULD be the first and regular evaluator of a male child’s testis?

A

Guideline Statement 2

PCP should palpate for quality and position at each well-child visit

** Approx. 70% palpable

**Of 30% not, 30% in inguinal scrotal area, 55% intra-abdominal, 15% vanishing/absent

**Spontaneous descent in first 6 mo, but may also “ascend” out of scrotum (acquired)

**Palpate testis–maintain in scrotal position for at least 30 seconds to fatigue cremaster muscle (differentiate retractile)

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6
Q

When SHOULD infants with UDT be referred? And why?

A

Guideline Statement 3

6 months (adjusted age) to Peds Uro

Impaired testicular growth, germ cell/leydig cell loss, decreased fertility possible, and cancer

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7
Q

What SHOULD be done for acquired UDT and when?

A

Guideline Statement 4

Refer after 6 mo (corrected for GA) to Peds Uro

**1-7% peaking at age 8

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8
Q

What MUST providers do for phenotypic male newborns with b/l nonpalpable testis?

A

Guideline Statement 5

Refer to Peds Uro/Peds Endocrine to evaluate for DSD

**Determine if palpable, if b/l non-palpable, may be genetic female (46XX) with CAH

**Failure to dx CAH can result in shock and electrolyte abnormalities

**17-hydroxyprogesterone, LH, FSH, testosterone, androstenedione, and Karyotype

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9
Q

What tests are done to evaluate when b/l non-palpable testis and phenotypic male

A
  1. 17-hydroxyprogesterone
  2. LH
  3. FSH
  4. Testosterone
  5. Androstenedione
  6. Karyotype
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10
Q

What imaging SHOULD be performed for UDT?

A

Guideline Statement 6

NONE

**US or other images with UDT prior to referral do not assist in decision making

**sx exploration (diagnostic lap or open for non-palpable)

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11
Q

What SHOULD providers assess for with UDT and penile anomalies? What type of anomalies are concerning?

A

Guideline Statement 7

DSD

Hypospadias or Micropenis (either unilateral or b/l)

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12
Q

What SHOULD be done in boys with b/l UDT w/o CAH?

A

Guideline Statement 8

Measure Mullerian Inhibiting Substance (MIS)/Anti- Mullerian Hormone (AMH) and CONSIDER hormone testing to eval for anorchia

  1. Inhibin b – undetectable
  2. FSH – elevated
  3. LH – elevated (Sertoli in response to endogenous FSH)
  4. Testosterone – low (Leydig cells in response to endogenous LH or exogenous hCG)

HCG stimulation test → elevation in FSH/LH → testosterone will be low

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13
Q

How often SHOULD provider assess retractile testis?

A

Guideline Statement 9

Annually

**Monitor for secondary ascent (2-45%)

**Hyperactive cremasteric muscle/reflex, foreshortened patent processus vaginalis, or entrapping adhesions

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14
Q

SHOULD providers supplement with hormonal therapy for UDT?

A

Guideline Statement 10

NO

*possible benefit to improve fertility rather than induce testicular descent–improved fertility index and sperm count – no long term data

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15
Q

When SHOULD specialists perform surgery for UDT?

A

Guideline Statement 11

In absence of spontaneous descent by 6 mo, perform w/in next 1 year (w/in first 18 mo life)

**No significant differences in fertility index for patients 1 yo or younger for UDT repair

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16
Q

What SHOULD be done in prepubertal boys with palpable UDT?

A

Guideline Statement 12

Inguinal or Scrotal Orchidopexy

**Best <18 mo, but likely fertility benefits before puberty

**Reduced cancer risk (2-6 fold risk reduction) prepubertal compared to post pubertal

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17
Q

Describe the 2 incision approach to orchidopexy:

A

*Inguinal and scrotal

  1. Inguinal incision between ASIS and pubic symphysis
  2. Dissect through abdominal layers to external oblique fascia
  3. Open external spermatic ring to expose inguinal canal
  4. Avoid ilioinguinal nerve
  5. Identify testis and divide gubernaculum
  6. Dissect patent processus vaginalis from spermatic cord and ligate at internal inguinal ring
  7. Mobilize cord and gain length, dissect tethering cremasteric fibers
  8. Scrotal portion–subdartos pouch, deliver and secure testis, avoid torsion on spermatic cord
  9. Close in layers with absorbable suture, void ilioinguinal nerve in closure

**Low lying testis, possible as single scrotal incision (when located distal to external inguinal ring)–high ligation of sac w/o opening of external spermatic fascia

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18
Q

What are risks associate with orchidopexy you would discuss with family?

A
  1. Infection
  2. Bleeding
  3. Damage to testicular vessels/atrophy of testis
  4. Damage to vas deferens
  5. Damage to ilioinguinal nerve (decreased sensation or pain to medial thigh, scrotum, and base of penis)
  6. Non-scrotal location
  7. Need to stage procedure
  8. Postoperative inguinal hernia
  9. Recurrence and need to revise in future
  10. Need for orchiectomy
  11. Testicular atrophy
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19
Q

What are some abnormalities you may notice during orchidopexy?

A
  1. Patent processus vaginalis (inguinal hernia)
  2. Abnormal fusion of epididymis and testis
  3. Looping vas deferens
  4. Small testicular size
  5. Epididymal atresia
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20
Q

What techniques can be used to gain length during orchidopexy?

A
  1. Fowler-Stephens: used for high intra-abdominal position (>2 cm above internal ring), division of testicular artery (internal spermatic vessels) to allow testis to survive on cremasteric/deferential blood supply (one stage vs. two stage)
  2. Prentiss maneuver: bring testis medial to inferior epigastric vessels
  3. Dissect spermatic vessels further along retroperitoneum
  4. Extend incision, open internal obligue musculature
  5. Secure testis as distal as possible and stage procedure
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21
Q

What do you do if you transect a vas deferns during an orchidopexy?

A
  1. Attempt to re-approximate ends of vas deferens with a fine non-absorbable suture
  2. Counsel family regarding injury and possibility of reduced fertility in future
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22
Q

What is differential dx for non-palpable testis?

A
  1. Abdominal or transinguinal (peeping) testis
  2. Complete atrophy (vanishing)
  3. Extra-abdominal but not palpable due to patient factors (anxiety, obesity, etc)
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23
Q

What should you do with a 6 mo boy with left UDT, noted at birth?

A
  1. EUA w/in 1 year
  2. Unlikely to descend
  3. If palpable or nubbin present in setting of contralateral hypertrophy → open exploration
  4. If remains non-palpable → diagnostic lap
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24
Q

What are the indications for treating an UDT with orchidopexy?

A
  1. Optimize testicular function and reduce fertility risk
  2. Facilitate dx of testicular malignancy with self exam after puberty
  3. Prevent inguinal hernia
  4. Prevent testicular torsion
  5. Cosmesis
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25
Q

What may you find for a non-palpable testis during diagnostic lap at inguinal ring?

A
  1. Norma appearing intra-abdominal testis
  2. Atrophic or abnormal appearing intra-abdominal testis
  3. Normal testicular vessels and vas deferens transversing internal ring
  4. Blind-ending testicular vessels and vas deferens
  5. Vas deferens with no vessels at internal ring
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26
Q

During diagnostic lap you find hypoplastic testis, normal contralateral, how do yo proceed?

A
  1. Orchiectomy
  2. Contralateral orchidopexy (not absolutely indicated but may be performed to secure solitary healthy testis)
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27
Q

What SHOULD providers counsel family on long-term risks associated with UDT?

A

Guideline Statement 16

  1. Testicular malignancy– (RR 2.75)increased risk at puberty, not eliminated by orchidopexy but allows for self-exam (counsel monthly) and screening and early detection
  2. Fertility–slight reduction in paternity rates (90% vs. 94%) due to germ cell loss, 65% for b/l UDT s/p sx
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28
Q

What SHOULD a provider do in a prepubertal boy with non-palpable testes?

A

Guideline Statement 13

  1. EUA to reassess palpability
  2. If non-palpable, diagnostic lap/open sx and abdominal orchidopexy, if indicated

**Favor MIS and goal to preserve primary blood supply if possible

**If 2 stage, ligation first, then repositioning (3-6 mo)

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29
Q

What SHOULD surgeon do during exploration of non-palpable testis?

A

Guideline Statement 14

Identify termination of testicular vessels

If vanishing, done

If nubbin, remove

*Beware long-looping vas

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30
Q

What patient and anatomic features indicate that a surgeon SHOULD perform orchiectomy at time of exploration for a UDT?

A

Guideline Statement 15

  1. Normal contralateral testis AND
  2. Short testicular vessels and vas deferens
  3. Dysmorphic or hypoplastic testis
  4. Post pubertal age
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31
Q

What is post operative f/up for orchidopexy?

A

1 and 6 mo check of size, consistency, and location

Annual testicular exam by PCP

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32
Q

What are success rates following orchidopexy?

A
  1. >95% for inguinal
  2. 70-90 % for 1-2 stage FS
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33
Q

What do you do for older boy who underwent orchidopexy as baby, now with faintly palpable testis?

A
  1. If palpable → observe (atrophy)
  2. If retracted → redo orchidopexy, or possible orchiectomy
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34
Q

Algorithm for Cryptorchidism (UDT)

A
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35
Q

Describe an index VUR patient?

A

4 yo with VUR and no evidence of BBD, who presents with febrile UTI and subsequent VUR on VCUG

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36
Q

Why is early detection and management of VUR important?

A

Renal preservation to prevent scarring (odds of renal scarring 2.8 x greater for children with pyelonephritis and VUR)

Guide management (worsened severity VUR correlated to more renal cortical abnormalities)

Prevention of febrile episodes/UTIs and hospitalizations

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37
Q

What affects can VUR have on a child? What follow up is important?

A

STANDARD

Overall health and renal function

Measurement of:

  1. Height
  2. Weight
  3. Blood pressure
  4. Serum creatinine (if b/l abnormalities found)
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38
Q

What lab evaluation is important for VUR?

A

RECOMMENDATION

UA for proteinuria and bacteriuria

If indicates infection → C&S

OPTION

Baseline GFR for future reference

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39
Q

What imaging is recommended for VUR?

A

RECOMMENDATION

Renal US to assess structure and function of upper tract

OPTION

DMSA (technetium-99m-labeled dimercaptosuccinic acid) to assess status of kidneys for scarring and function

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40
Q

Relationship between baseline UTI and DMSA renal cortical abnormalities

A
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41
Q

Rates of Resolution for VUR?

A

Grade 1: 80%

Grade 2: 68%

Grade 3: 45%

Grade 4/5: 17%

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42
Q

For VUR, what are OPTIONS for continuous antibiotic prophylaxis (CAP)?

A

OPTION

May be considered for child with hx UTI and VUR w/o BBD

OPTION

Observational management without CAP, and prompt initiation of abx for UTI, may be considered WITHOUT:

  1. BBD
  2. Recurrent febrile UTIs
  3. Renal Cortical Abnormalities

*Continuing use of CAP in children with Grade II - V seems prudent until more data are available

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43
Q

For children with VUR, to consider observation without continuous antibiotic prophylaxis what factors must be absent?

A

MUST BE WITHOUT

  1. BBD
  2. Recurrent febrile UTIs
  3. Renal Cortical Abnormalities
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44
Q

For ongoing monitoring of VUR, what child specific factors/labs are important to follow?

A

RECOMMENDATIONS:

General evaluation ANNUALLY

  1. BP
  2. Height
  3. Weight
  4. UA for proteinuria and bacteriuria, +/- C&S.

OPTION

The follow up interval is determined by the likelihood of resolution; for higher grade VUR, less likely and longer f/up necessary

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45
Q

For ongoing monitoring of VUR, what are the RECOMMENDATIONS for follow up imaging?

A

RECOMMENDATIONS:

  1. Imaging, including US and VCUG (radionucleotide cystogram or low dose fluoro, when available) EVERY 12-24 mo, with longer for those with less likelihood of resolution (higher grade 3-4, BBD, older age) to limit imaging studies *if observational approach is being used, f/up VCUG becomes an OPTION rather than RECOMMENDATION
  2. DMSA ONLY when US abnormal (greater concern for more scarring, i.e. breakthrough UTI, a higher grade of VUR, or elevated Cr)
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46
Q

For ongoing monitoring of VUR, what are the OPTIONS for follow up imaging?

A

OPTIONS:

  1. F/up VCUG after 1 year old in VUR grade 1-2 (observation)
  2. A single negative f/up VCUG establishes resolution
  3. Periodic upper tract imaging with renal US may be done to assess renal growth and presence of gross scarring
  4. DMSA to detect new scarring (esp. if febrile UTI)
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47
Q

What are symptoms of symptomatic breakthrough UTI in a child with VUR on CAP?

A
  1. fever
  2. dysuria
  3. frequency
  4. failure to thrive
  5. poor feeding
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48
Q

What is RECOMMENDED when child with VUR on CAP has BT-UTI? What factors influence treatment alternatives?

A

RECOMMENDATION

single episode → consider change in abx IF no evidence of pre-existing or new renal cortical abnormalities

change in therapy → consider open surgical ureteral re-implant or endoscopic injection bulking agents with curative intent

Factors: VUR grade, degree of renal scarring, BBD, and parental preferences

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49
Q

In VUR patients under observation who develop febrile UTI, next steps RECOMMENDATION?

A

RECOMMENDATION

Continuous Abx Prophylaxis

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50
Q

In VUR patients under observation who develop non-febrile UTI, what is an OPTION?

A

OPTION

Initiate CAP

*keep in mind that not all cases of pyelonephritis are febrile

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51
Q

What are resolution rates for surgical interventions for VUR? Open surgery and Endoscopic Single Injection of Bulking?

A

Open 98.1%

Endoscopic Bulking 83%

*Reported first year after intervention

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52
Q

What are some post operative VUR adverse events and their incidence?

A
  1. Obstruction → 0.4%
  2. Voiding disturbances → 4.2%
  3. Contralateral VUR (after unilateral treatment) → 9.6%
  4. New renocortical abnormalities → extreme variabilities in studies, study suggests can occur
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53
Q

What surgical OPTION may be used for VUR? RCTs have shown what trend in regards to febrile UTIs?

A

OPTION

Open and endoscopic methods

RCTs show a reduction in febrile UTIs in patient who undergo open surgical correction vs. CAP

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54
Q

Following resolution of VUR, either spontaneously or by surgical intervention, what is the general evaluation an when is it an OPTION vs. RECOMMENDATION?

A

OPTION

If normal kidneys by US or DMSA

BP, height, weight, UA (protein, UTI) → annually through adolescence

Longer interval of follow appropriate for higher grades VUR

RECOMMENDATION

If ABNORMAL kidneys by US or DMSA

MUST monitor BP, height, weight, UA (protein, UTI) → annually through adolescence

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55
Q

Following resolution of VUR, either spontaneously or by surgical intervention, what evaluation is a RECOMMENDATION with occurrence of febrile UTI?

A

RECOMMENDATION

BBD or recurrent VUR

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56
Q

What long-term concerns are RECOMMENDED for communication with a child at an appropriate age, after resolution or surgical correction of VUR?

A

RECOMMENDATION

  1. HTN (particularly during pregnancy)
  2. Renal function loss
  3. Recurrent UTI
  4. Familial VUR (in child’s siblings and offspring)
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57
Q

How is the index patient < 1 yo usually diagnosed wit primary VUR?

A
  1. Prenatal Hydronephrosis
  2. Occurrence of UTI
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58
Q

What are criteria used to determine the need for surgery for VUR?

A
  1. Persistent grade II-V VUR
  2. BT-UTI
  3. Noncompliance
  4. Deterioration of renal function
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59
Q

What is the RECOMMENDATION for CAP in a child < 1 yo and a history of febrile UTI with VUR?

A

RECOMMENDATION

CAP (greater morbidity from recurrent UTI in infants)

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60
Q

What is the RECOMMENDATION and the for CAP in a child < 1 yo WITHOUT a history of febrile UTI with VUR?

A

RECOMMENDATION

CAP if VUR grades III-V

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61
Q

What is the OPTION and the for CAP in a child < 1 yo WITHOUT a history of febrile UTI with VUR?

A

OPTION

May OFFER CAP

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62
Q

For baby boys < 1 yo with VUR what OPTION may be considered based on increased risk of UTIs?

A

OPTION

Circumcision

*Insufficient data to evaluate degree of risk and its duration

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63
Q

For prenatal hydronephrosis (PNH) what is recommended follow up?

A

Post natal RBUS 24-48 h after birth (what parents expect)

AND

Repeated in an additional 10-14 days as an outpatient

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64
Q

What is average incidence of VUR in patients with PNH?

A

Approximately 16% (ranges 13 - 23%)

Higher in females

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65
Q

In regards to SFU Grade 1 - 2 PNH what are a providers OPTIONS?

A

OPTIONS

An observational approach without screening for VUR (there is an unproven value of dx and tx VUR)

Prompt treatment of UTI

VCUG is an option to screen for VUR

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66
Q

In regards to SFU Grade 3 - 4 with PNH, what are is RECOMMENDED? WHY?

A

RECOMMENDATION

VCUG

High-grade hydronephrosis, hydroureter, abnormal bladder (late prenatal or postnatal), or development of UTI

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67
Q

Which post natal US findings correlate to which ddx, and why?

A

Isolated Hydronephrosis → UPJ or VUR

Ureteral dilation → Ectopic ureter, ureterocele, PUV (b/l)

Dilated bladder/bladder anomalies → ureterocele, PUV

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68
Q

You find hydronephrosis on postnatal US, bladder is normal. What are next steps?

A

Start CAP (until VUR excluded)

Infant follow up outpatient s/p VUCG and Renal Scan in ~2 weeks

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69
Q

What is recommendation for infant with UJPO?

A

Surgery → open dismembered pyeloplasty (changes/disparate differential function)

observe if renal function equal and good drainage, if hydro stable (not severe), no renal scan needed

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70
Q

What are different surgical options for open dismembered pyeloplasty in a child?

A
  1. Anderson-Hynes → standard method
    1. allows for excision of abnormal UPJ, renal pelvic reduction, transposition (if crossing vessel)
    2. contraindicated if long proximal stricture
    3. approach: anterior subcostal, flank, dorsal lumbotomy
      1. ID renal pelvis, expose UPJ
      2. transect and excise UPJ
      3. spatulate ureter laterally and excise redundant pelvis
      4. anastomosis performed using fine interrupted or running suture
      5. stent or PCN
      6. leave perirenal drain
  2. Foley Y-V plasty
    1. High inserting ureter or horseshoe kidney
    2. Contraindicated if crossing vessel or pelvic reduction
  3. Spiral flap (Culp-Deweerd)
    1. Pelvic reduction or long proximal ureteral stricture
    2. Contraindicated if crossing vessel or pelvic reduction
  4. Vertical flap (Scardino-Prince)
    1. Good for pelvic reduction or medium length ureteral stricture
    2. Contraindicated if crossing vessel
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71
Q

What would you advise a parent about robotic pyeloplasty for an infant?

A

Small working space limits use until at least 6 months old

Operative time and results are comparable for an experienced surgeon

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72
Q

What are the presenting signs and symptoms of UPJO in a child?

A
  1. Episodic flank pain
  2. Cyclical n/v
  3. Azotemia (unlikely if c/l normal kidney)
  4. Kidney stones
  5. Pyelonephritis
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73
Q

What is an indication for nephrectomy in child with UPJO?

A

Massive hydronephrosis with <15% differential fxn

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74
Q

Follow up after successful dismembered pyeloplasty?

A

RBUS 1-2 mo after PCN or stent removed

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75
Q

What do you do if hydronephrosis on RUS after pyeloplasty?

A
  1. Repeat RBUS 1-2 mo (common in early post operative period)
  2. If does not improve, MAG 3 to assess differential fxn and t½
  3. If >15% fxn, would repeat pyeloplasty, spiral/vertical procedure, or ureterocalicostomy
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76
Q

What are the causes of UPJO in a child?

A
  1. Intrinsic
    1. Attenuated and separated muscle fibers w/increased collagen at UPJ → insufficient emptying of pelvis
    2. Valvular mucosal folds
    3. Ureteral polyps
    4. Persistent fetal convolutions
  2. Extrinsic
    1. Crossing vessel over lower pole
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77
Q

What are GU associated anomalies with UPJO?

A
  1. Contralateral UPJO (10-40%)
  2. VUR (both c/l and i/l)
  3. MCDK
  4. Duplex system (UPJO)
  5. VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, radial and renal dysplasia)
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78
Q

In case of prenatal hydronephrosis, what questions should be asked about prior pregnancies?

A
  1. Maternal hypertension and preeclampsia
  2. Late fetal demise
  3. 2 vessel umbilical cord
  4. sex/chromosomal abnormalities (trisomy 21)
  5. prior miscarriages, especially recurrent
  6. prior fetal anomalies, including GU
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79
Q

What important questions should be asked about fetal US if prenatal hydronephrosis detected?

A
  1. Are kidneys appropriate size/echonenicity for GA?
  2. Was any prior urinary tract dilation related to bladder fullness?
  3. Any other structural/organ anomalies?
  4. Evidence of dilated posterior urethra with thick walled distended bladder (keyhole sign)?
  5. Were ureters visualized?
  6. Was 20 week structural sonogram normal?
  7. Has bladder been identified and shown full and empty?
  8. Is amniotic fluid index (AFI) normal for GA?
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80
Q

What family history is important for neonate or fetus with hydronephrosis?

A
  1. UTIs (febrile)
  2. GU anomalies or other major organs (i.e. VACTERAL)
  3. chronic CKD or ESRD or family hx of renal txp
  4. young onset HTN
  5. VUR
  6. Unexplained fevers
  7. Early death d/t respiratory distress
  8. Accessory nipples or ear tags
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81
Q

What labs would you order on neonate with prenatal hydronephrosis?

A
  1. CBC & diff
  2. BMP (1 week for true neonate Cr)
  3. UA
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82
Q

Describe post natal PE on baby with PNH?

A
  1. check for accessory nipples or ear tags
  2. umbilical abnormalities
  3. lower extremity deformations from oligohydramnios
  4. other congenital anomalies
  5. pulmonary distress (bell shaped chest, abnormal APGARS)
  6. distended bladder
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83
Q

If increased hydronephrosis on post natal US (especially b/l) or bladder distention, next step?

A

VCUG

*prior to d/c if b/l–absolute indication to r/o PUV

if unilateral or normal bladder should be prior to d/c but can be as outpatient as long as CAP started

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84
Q

What is appropriate CAP in neonate? What are there a/e?

A

Keflex or Amoxicillin

Loose stools

*C.Diff (watery stools) → peds GI → often probiotics

Rash

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85
Q

If neonates 10-12 day US show low risk UTD? Next steps

A

Consider repeat 3-4 months

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86
Q

Signs of UTI in neonate?

A
  1. May not have fever until 30 days
  2. Listelessness
  3. Excessive irritability
  4. Altered feeding habits
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87
Q

What should be done if PUV suspected or distended bladder?

A

RUS

VCUG

Trend Serum Cr

CAP

If cannot be done immediately, place 5 fr feeding tube in bladder

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88
Q

What anomalies could you look for on VCUG?

A
  1. VUR
  2. Bladder wall thickening with trabeculations and diverticuli
  3. Bladder neck hypertrophy
  4. A dilated posterior urethra with evidence of valves
89
Q

If PUV is diagnosed, what is recommended management?

A

TUR when creatinine has decreased <0.8-1.0 and infant ready for anesthesia

90
Q

What is follow up for PUV?

A

VCUG close post-op to assess complete valve resection and at 1 year (sooner if worsening hydronephrosis or pyelonephritis)

RUS and BMP (r/o metabolic acidosis and trend Cr)

Baseline DMSA (+/- most important with high grade VUR or dysmorphic kidneys)

91
Q

What describes a child with BBD?

A

Poorly characterized complex including

  1. urinary incontinence
  2. dysuria, perineal and penile pain
  3. UTIs
  4. frequency
  5. daytime wetting (+/- posturing to prevent wetting)
  6. infrequent voiding
  7. constipation or encoporesis
  8. abnormal LUTs storage or emptying
  9. high pressure voiding (failure to relax external sphincter or PF muscles)
  10. incomplete bladder emptying
92
Q

Why is BBD important in VUR? What is part of STANDARD?

A

STANDARD

Symptoms of BBD should be sought in initial evaluation (f/u, delayed voids, wetting, pain, holding, constipation)

Why?

Lower resolution rates of VUR (spontaneous and post-surgical–endoscopic)

Higher rates of UTI

Higher rates of post-operative complications

93
Q

What are treatment RECOMMENDATIONS for patients with BBD prior to any surgical intervention for VUR?

A

RECOMMENDATION

  1. Behavioral therapy
  2. biofeedback (>5 yo)
  3. anticholinergic meds
  4. alpha blockers
  5. bowel regimen
  6. regular follow up and modification PRN
94
Q

Regarding VUR and CAP, what is RECOMMENDED?

A

RECOMMENDATION

++ CAP

*Increased risk of infection and sequelae

95
Q

Incidence of VUR? Population? Twinning? Siblings?

A

1% general population

MUST Screen

100% identical twins

35-50% fraternal twins

27% (3-53%)Siblings

36% offspring

96
Q

What is RECOMMENDATION for sibling of child with VUR and renal scarring on US or UTI?

97
Q

What laboratory tests are necessary at time of hypospadias diagnosis in an otherwise normal neonate?

98
Q

When do you need to perform karyotype and hormonal studies in an infant with hypospadias? What additional tests as well?

A

Obtain: karyotype, testosterone, FSH, LH

  1. Perineal hypospadias
  2. UDT with proximal hypospadias
  3. Additional major congenital anomaly
  4. Concerns for DSD
99
Q

When are imaging studies considered in an infant with hypospadias? And which studies?

A

Concern for DSD

Syndromic hypospadias

Pelvic US and genitogram

100
Q

What are the recommendations for management for hypospadias in an otherwise healthy infant?

A
  1. Perform hypospadias repair at 6-12 mo (advocated by AAP).
    *Boys have significant phallic growth at ~3 mo d/t testosterone surge at 2 mo
  2. Because of cognitive, developmental, and emotional factors, repair should be completed by 15 mo
101
Q

What are risk factors for hypospadias?

A
  1. Heredity: 10% affected boys have affected relative
  2. Endocrine disrupters: natural or synthetic compounds (estrogen like) or anti-androgen effect
  3. Fetal endocrinopathies which alter testosterone production or its action
  4. > 200 syndromes
102
Q

What are location of hypospadias and incidence?

A
  1. Glandular/coronal: 70-80%
  2. Shaft 10-20%
  3. Penoscrotal 3-5%
  4. Perineal < 1 %
103
Q

When would you workup for DSD in a child with hypospadias?

A
  1. Newborn with severe hypospadias and non-palpable testis (1 or 2)
    1. Ambiguous genitalia, unilateral UDT, and a contralateral streak (mixed gonadal dysgenesis–MGD), 45X/46XY mosaicism
    2. Females with CAH (masculinized clitoris can look like severe chordee and proximal hypospadias
104
Q

What are key findings on GU exam?

A
  1. General appearance (facies)
  2. Abdomen
  3. Back (dimple, hair patch)
  4. Phallus, meatus (location, open), prepuce (dorsal hood), curvature (chordee), penile stretch length, testis (presence, size), development of scrotum
  5. Anus location
  6. Neuro
105
Q

When would one consider preoperative testosterone stimulation in relation to congenital penile anomalies?

A
  1. Small penises and/or small glans → increase penile and glans size
  2. Significant ventral chordee → reduce severity
  3. Re-operative hypospadias → increased size of penis and improve vascularity to tissues

Administration of topical or parenteral

4-6 weeks prior to surgery

IM given typically as 2-3 injections

106
Q

What are the desired outcomes of hypospadias surgery?

A
  1. Cosmetically normal phallus (circumcised or with foreskin reconstruction)
  2. Normal caliber neourethra and urethral meatus on glans
  3. Straight phallus
107
Q

Name complication of hypospadias repair?

A
  1. urethral stricture
  2. meatal stenosis
  3. urethrocutaneous fistula
  4. persistent chordee
  5. penile skin breakdown
  6. urethral diverticulum
108
Q

Key elements of hypospadias repair?

A
  1. Correction of penile curvature
  2. Urethroplasty
  3. Interposition of tissue barrier between neourethra and skin closure
  4. Glansplasty
  5. Skin closure including circumcision or foreskin reconstruction
109
Q

Describe a chordee repair:

A
  1. Deglove down to penopubic and penoscrotal junction, artificial erection
  2. If curvature <30%, dorsal plication in midline (avoid NVB at 11:00 and 1:00)
  3. After plication or degloving, if >30% residual curvature, mobilize urethral plate off corporal bodies and repeat artificial erection
  4. If curvature persists, transect urethral plate off corporal bodies at corona or junction of normal urethra, make transverse incision through tunica albuginea ventral at point of max curvature in corporal bodies form 3:00-9:00 position, then graft with dermis, small intestinal submucosa, or tunica vaginalis flap
110
Q

Urethroplasty options for primary hypospadias:

A
  1. Distal (shaft into glans)
    1. Tubularized incised plate (TIP)
      1. Thiersch-Duplay
      2. Mathieu (flip-flap)
  2. Proximal (penoscrotal to mid-shaft)
    1. TIP
    2. Preputial skin flap
      1. Onlay
      2. Koyanagi
    3. 2 stage repair (2nd stage 6-12 mo later)
      1. Byars’ prepucial flap
      2. Buccal graft
111
Q

What flaps can be used to cover neourethra to minimize fistula formation?

A
  1. Local flap → periurethral dartos fascia
  2. Rotation of dorsal or preputial dartos
  3. Tunica vaginalis (from scrotum)
112
Q

What do you do for a post hypospadias urethrocutaneous fistula?

A

Wait 6 mo to allow in-growth of vascular supply and full resolution of edema → correct

*longer urethroplasty, high rate

*MC at junction of native urethra and neourethra

113
Q

What do you do for a post hypospadias urethral stricture?

A

Urethral dilation, DVIU, or urethroplasty

*Occurs at proximal or distal ends of anastomosis

114
Q

What do you do for a post hypospadias meatal stenosis?

A

Trial of steroid cream, then meatoplasty if persists

115
Q

What do you do for a post hypospadias urethral diverticulum?

A

Correct distal obstruction and excise redundant skin, or consider completely resecting skin used for urethra and performing buccal staged urethroplasty

*Due to distal obstruction or elastic skin used at initial repair

116
Q

Key physical exam finding when evaluating neonate for DSD?

A
  1. Penile length and diameter (stretched)
  2. Severity of curvature
  3. Location of urethral meatus
  4. Palpability and size of testis
  5. General size and appearance of clitoris (severe clitoromegaly → stretched length and width taken
  6. Location of meatus in relationship to vaginal orifice
  7. Present of urethral and vaginal opening (high testosterone in utero → fuse vagina/urethra (urogenital sinus)
  8. Appearance of labia → fusion, anterior placement, hyperpigmentation, absence of labia minora
  9. Uterus may be palpable in rectum (cord like structure)
117
Q

When should evaluation for DSD proceed?

A
  1. Overt genital ambiguity
  2. Female genitalia with enlarge clitoris, posterior labial fusion, or an inguinal/labial mass
  3. Male genitalia with b/l UDT, micropenis, perineal hypospadias, or hypospadias with non-palpable testis
118
Q

What labs do you send when concerned for CAH in neonate? What do you do if abnormal?

A

BMP

Resuscitate infant with IV fluids, correct electrolytes

Start glucocorticoid and mineralocorticoid

Call neonatology, endocrine, medical genetics, psych

119
Q

What imaging studies do you order when concerned for DSD and CAH?

A

Abdominal and pelvic US

Look for uterus, gonads

*possible adrenal US in experienced hands

120
Q

What causes CAH?

A

3 enzymatic defects: 21-hydroxylase (95%) (17-OH progesterone → 11 deoxycortisol), 11B-hydroxylase (virilization and HTN)

MC with salt wasting and virilization (75%)

Sxs: n/v, dehydration, progressive weight loss, hyperkalemia, shock

121
Q

Timing, workup, and concerns for surgery for genital reconstruction for babies with CAH?

A
  1. 2-6 mo age (recently debatable)
  2. preoperative genitogram → length of confluence of common urogenital sinus and the urethra
  3. stress dose corticosteroid perioperatively
122
Q

What is mixed gonadal dysgenesis (MGD)?

A

Second most common type of DSD

  1. Unilateral testis (typically UDT)
  2. An intrabdominal streak
  3. Persistent mullerian duct structures
  4. Varying degrees of virilization of external genitalia
123
Q

What are common types of DSD?

A
  1. CAH → MC (CAH 21-hydroxylase, 11b-hydroxylate)
  2. MGD [46,XY, 45,XO/46,XY]
  3. Ovotesticular DSD (formerly hermaphroditism) → both testis and ovaries or ovotestis (controversy)
  4. Androgen Sensitivity Syndromes → abnormalities in androgen receptor function (un- or under- virilized);
    1. complete androgen insensitivity (testicular feminization → 46 XY → b/l UDT, female appearing genitalia, absence of mullerian features
    2. partial - incomplete virilization→hypospadias, b/l UDT, rudimentary Wolffian features
  5. Disorder of Androgen Action→disorder of 5a-reductase→normal formation of testis and male ducts; however no conversion to DHT→no normal external genitalia (ambiguous or severe hypo)
  6. Persistent Mullerian Duct Syndrome→46XY, normal male external genitalia with uni- or bi- UDT and fallopian tubes, a uterus, and upper vagina which drains into prostatic utricle
    1. Abnormality in gene and production of MIS
124
Q

When is ppx gonadectomy or removal of streak gonads recommended?

A

Patients raised as girls with Y genetic material

125
Q

Common gonadal tumors associated with DSD?

A

Gonadoblastoma and dysgerminoma

126
Q

Steroid Biosynthetic Pathway

127
Q

How would you obtain a urine sample on infant with infection?

A
  1. CIC
  2. SP aspiration if unable to CIC
  3. Bag collection (high rate contamination)
128
Q

How do you calculate bladder capacity in child?

A
  1. Age: Age (in years) + 2 = urine capacity in oz (30mL/oz)
  2. Weight in infancy: Weight (kg) x 7 = urine capacity
129
Q

How do you treat child with febrile UTI? What imaging studies do you order?

A

IVF and IV broad spectrum abx

RBUS

130
Q

In a duplex system what usually happens to moieties? What does a cystic mass in bladder represent? What does it mean when bladder is distended as well?

A

Upper pole obstructs

Lower pole refluxes

Ureterocele

Ureterocele can prolapse into urethra and obstructing (can be seen as congested intralabial mass)

131
Q

Treatment of obstructing ureterocele in an baby girl?

A
  1. Transurethral incision
  2. PCN to obstructed moiety
132
Q

Describe transurethral incision of ureterocele

A
  1. 9-11 Fr resectoscope or peds cystoscope (7-10 Fr) with Bugbee electrode, angle wire, or holmium laser
  2. Make 2-4 mm transverse incision at lowest point within bladder to ensure drainage (visualize Jet)
  3. If extends into urethra, make second incision at lowest point within urethra
133
Q

Incidence of ipsilateral VUR after ureterocele incision?

A

0-50%, ectopic ureteroceles have highest rate of de novo VUR

VUR much more common to lower pole moiety

134
Q

What imaging to follow up ureterocele incision?

A

VCUG and nuclear renogram while on ppx abx

+/- RBUS to confirm adequate decompression

135
Q

Nonfunctioning moiety of duplex system with hydronephrosis s/p ureterocele incision, options?

A
  1. Heminephrectomy with subtotal ureterectomy (dilated non-functioning moiety)
  2. Observation with serial US (document resolution of hydro and decompression of ureterocele)
  3. Common sheath re-implant, excision of decompressed ureterocele, posterior bladder wall reconstruction or marsupialization of ureterocele to bladder mucosa
136
Q

Discuss surgical technique for heminephrectomy and subtotal ureterectomy in a child?

A
  1. Flank position, extraperitoneal exposure
  2. Upper pole hemi with partial ureterectomy, careful to ligate only vessels feeding upper pole
  3. Distal stump left open if not ligated if no VUR
137
Q

How long does it take for a newborn to void?

A

Usually within 24 h

138
Q

What is a normal creatinine level for healthy newborn?

A

<0.4 after 3-5 days (prior reflects maternal)

139
Q

Hold old should a child be when ordering a nuclear renogram?

A

Best to wait 3 mo (prior neonatal kidney functionally immature and not as accurate)

140
Q

What does it mean if serum creatinine level does NOT decrease to less than 1 by 1 year old?

A

high risk of ESRD if does NOT decrease < 1

141
Q

What is ddx of febrile UTI (pyelonephritis) in an infant with prenatal hydronephrosis?

A
  1. VUR
  2. UVJ obstruction
  3. Ureterocele with obstructed moiety (hydronephrotic segment - usually UP)
  4. Ectopic ureter with hydronephrosis
142
Q

In a duplex system with severe dilation of upper pole, what is ddx concerning for?

A

Ectopic ureter or ureterocele

143
Q

If concerned for ectopic ureter or ureterocele and normal bladder with dilated ureter on US, what other tests can be performed?

A

VCUG

Nuclear renogram

144
Q

What is Meyer Weigert rule?

A

When 2 ureteral buds incorporating into UGS, most inferior bud (lower pole) incorporated first and migrates more lateral and superior(prone to VUR), the upper pole ureter is higher and incorporated later and more medial and inferior (prone to obstruction)

145
Q

Why is a left common sheath reimplant recommended for a duplex system with function of both moieties?

A

separation of duplex ureters can lead to injury of longitudinal blood supply between ureters and compromise ureters

146
Q

Key surgical principals for ureteral reimplant?

A
  1. Adequate mobilization of distal ureter → tension free, preserve blood supply
  2. Creation of generous submucosal tunnel → 5 x width of ureter in length
  3. Manipulate ureter to correct position in submucosal tunnel to prevent obstruction, stricture, persistent VUR
  4. Careful handling to prevent hematuria or bladder spasms
147
Q

Unique complication of extravesical ureteral reimplant?

A
  1. Transient urinary retention (20%) with b/l repairs
    * Thought to be due to excessive electrocautery during ureteral mobilization and injury to pelvic plexus nerves just 2 cm distal and medial to UVJ
148
Q

Post surgical ureteral reimplant f/up?

A
  1. Continue CAP for several months
  2. RBUS 1-2 mo to r/o obstruction
  3. Post operative VCUG is not required if doing well and grade I-IV, grade V may require
  4. Annual height, weight, BP, and UA for protein and infection, and creatinine through adolescnce
  5. Good bowel and bladder habits
149
Q

Ddx of continuous leakage in a 5 yo girl?

A
  1. BBD with urinary incontinence
  2. Neurogenic bladder
  3. Ectopic ureter
150
Q

How do you evaluate for ectopic ureter?

A
  1. RBUS - often normal (occasional UP hydro)
  2. EUA (cystovaginoscopy +/- injection of methylene blue)
  3. MR urogram (best) – look for for UP, dilated ureteral path
151
Q

Tx options for ectopic ureter with continuous incontinence?

A
  1. Upper to lower UU (either in lower ureter - small inguinal incision or proximal near pelvis)
  2. Common sheath reimplant
  3. Upper pole heminephrectomy and subtotal ureterectomy
152
Q

What is initial workup for newborn with abdominal mass?

A

UA/micro

BMP

RBUS

153
Q

What is ddx for neonate with abdominal mass and US with cystic kidneys and hydronephrosis?

A
  1. Multicystic dysplastic kidney
  2. UPJO
  3. VUR
154
Q

What would next steps be for infant with abdominal mass and US showing multiple cysts and mild hydronephrosis?

A

VCUG and nuclear

CAP started (Amox of Keflex, do NOT give child <2 mo Bactrim (kernicterus) or Macrobid (hemolytic anemia)

155
Q

What are the different types of nuclear renograms?

A
  1. MAG3: secreted proximal tubule, focuses on uptake and drainage
  2. DTPA: filtered in glomerulus, measures GFR (not w/renal insufficiency)–less anatomic detail, not usually used in children
  3. DMSA: assess cortical scarring and function
156
Q

How do you manage MCDK?

A

Observe

Operate if respiratory distress or difficulty feeding d/t mass effect

157
Q

General recommendations for child under 1 year old with VUR?

A

CAP Grade III-IV or febrile UTI

158
Q

What are general recommendations for child over 1 years old with VUR?

A
  1. BBD → CAP
  2. CAP option if BBD absent and hx of UTI
159
Q

What are absolute indications for surgical intervention for VUR? What types of surgery are considered?

A

Endoscopic bulking

Ureteral re-implant

  1. Medical noncompliance with CAP
  2. Breakthrough UTI on CAP
  3. Progresive scarring
  4. Associated UVJ abnormality
160
Q

Goals of intevention for VUR?

A
  1. Reduce episodes of pyelo
  2. Maximize renal growth and prevent future scarring
  3. Reduce risk of HTN and ESRD
161
Q

Describe techniques for ureteral reimplant:

A
  1. Intravesical
  • Politano-Leadbetter: creates new ureteral hiatus more cephalad and new submucosal tunnel in orthotopic location (longer tunnel)
  • Glenn-Anderson: original hiatus with advancement of ureter distal towards bladder neck (shorter tunnel)
  • Cohen: ureteral tunnel directed laterally across trigonal towards contralateral orifice (small bladder or posterior bladder wall/bn surgery done)
  1. Extravesical
    * Lich-Gregoir: advances ureter distally with lengthening of tunnel made by muscular trough for ureter
162
Q

What is RECOMMENDED postoperative f/up for ureteral reimplant?

A
  1. RBUS 4-6 weeks after to r/o obstruction
  2. Continue CAP 3-6 mo with OPTION of performing VCUG to confirm resolution of VUR
163
Q

Complications of ureteral reimplant?

A
  1. Recurrent UTI
  2. Contralateral VUR
  3. Persistent VUR
  4. Kinking of ureter (related to j-hooking when bladder full/intermittent obstruction)
  5. UVJ obstruction
  6. Bladder diverticulum
  7. Urine Leak
  8. Urinary retention (b/l extravesical approach)
164
Q

VUR sibling incidence?

165
Q

When is screening of sibling of VUR patient indicated? How?

A

Cortical abnormality, size asymmetry, hx of UTI

Radionucleotide cystogram or VCUG

166
Q

Describe grades of reflux classification of VUR?

A
  1. Reflux to ureter
  2. Reflux to ureter and collecting system, no dilation
  3. Mild dilation of renal pelvis and calyces
  4. Moderate dilation of renal pelvis and calyces
  5. Severe dilation of renal pelvic and calyces with ureteral tortuosity
167
Q

Normal submucosal tunnel to ureteral diameter ratio?

168
Q

Primary causes of VUR?

A
  1. Short intramural ureter (submucosal length <5:1)
  2. Absence of detrusor backing
  3. lateral displacement of UO
  4. Abnormal configuration of UO
169
Q

Secondary causes of VUR?

A
  1. NGB
  2. BOO
  3. Paraureteral diverticulum
  4. Cystitis
  5. Detrusor instability
  6. Duplex system +/- ureterocele
170
Q

What are of kidney most susceptible to scarring?

A

Upper and lower poles

*compound papilla with large open collecting ducts (intrarenal reflux of urine)

171
Q

What is ddx of child with abdominal mass on exam?

A
  1. Wilms’ tumor
  2. Congenital mesoblastic nephroma (MC renal tumor of infants, 3 subtypes: classic, cellular, mixed, tx RNx and adjuvant chemo for cellular type or incomplete resection)
  3. Neuroblastoma (not renal mass but can present as abdominal mass)
  4. Clear cell sarcoma (3-5% childhood renal tumors, no associated syndromes, unicentric/unilateral, assoc. brain/bone mets)
  5. Rhabdoid tumor (2% childhood renal tumors, median age <16 mo, hypercalcemia, brain mets, poor prognosis, very sick child)
  6. RCC
  7. Angiomyolipoma
172
Q

Next step after dx renal mass on US?

173
Q

Renal mass on US and CT (heterogenous)? What studies would you order?

A
  1. CBC (plt) and CMP
  2. If concerns for intracaval extension (MRI if US cannot exclude)
  3. Chest CT
  4. Cranial CT or MRI (if clear cell sarcoma or rhabdoid tumor)
  5. Skeletal survey bones can (if pathology confirms clear cells sarcoma or RCC)
174
Q

What congenital anomalies are associated with Wilms’ tumor?

A
  1. WT1 gene: 11p13 (non-overgrowth syndromes)
  • WAGR (wilms’, aniridia, genital, MR) → 30% risk
  • Denys-Drash (male pseudohermaphroditism, renal mesangial sclerosis, wilms’)
  1. WT2: 11p15 (overgrowth syndromes)
  • Beckwith-Wiedemann (hemihypertrophy, macroglossia, visceromegaly, omphalocele) → 5% risk
  • Hemihypertrophy
  • Perlman (visceromegaly, macrosomia, polyhydramnios, facies)
  • Sotos (macrocephaly, MR)
175
Q

What are some clinical features/presentation of Wilms’ tumor?

A
  1. Most children otherwise healthy, present with only mild sxs
  2. Palpable mass → 90%
  3. Hematuria → 25%
  4. HTN → 25%
  5. Girls = Boys
  6. Median Age 3.5 yo
176
Q

MC site of metastasis for WT?

A
  1. Lung
  2. Regional LN
    Liver
177
Q

How do you define stage for WT?

A

Surgery

Radical Nx

178
Q

Approach to RNx in WT child?

A
  1. Transperitoneal approach
  2. Inspect and palpate all 4 quadrants for extension (liver, nodes, peritoneal seeding)
  3. Inspect contralateral (no longer necessary if MRI/CT done preop)
  4. Palpate IVC and RV for thrombosis
  5. Complete nephrectomy w/o tumor spillage
  6. Sample suspicious nodes (RPLND as indicated)
179
Q

When is neoadjuvant chemotherapy recommended in WT?

A

B/L

Tumors too large to excise safely

IVC involvement above hepatic veins

180
Q

What are the two types of WT histology?

A
  1. Favorable: intrarenal pseudocapsule (compressing adjacent normal parenchyma), class triphasic components (blastemal, epithelia, and stromal)
  2. Unfavorable: anaplasia (nuclear enlargement, hyperchromasia, abnormal mitotic figures), associated with resistance to chemotherapy and poor prognosis (even if stage I)
181
Q

What are stages of WT?

A
  1. Confined to kidney, completely resected, no intra-op spillage
  2. Extracapsular penetration, completely resected, may have renal sinus extension or vessel invasion
  3. Residual tumor in abdomen (nodes, spillage, peritoneal implants, incomplete resection)
  4. Hematogenous metastases to liver/lung/bone/brain
  5. Bilateral at dx
182
Q

What are risk factors for WT recurrence?

A
  1. spillage
  2. unfavorable histology
  3. incomplete excision
  4. failure to sample LN can result in understaging
183
Q

After RNx what is treatment of WT by stage?

A
  1. Favorable histology
  • Stage I & II: Vincristine and Dactinomycin
  • Stabe III & IV: Vincristine, Dactinomycin, Doxorubicin, Radiation
  1. Unfavorable histology (anaplastic)
  • Stage I: Vincristine, Dactinomycin, Doxyorubicin, Radiation
  • Stage II-IV: Vincristine, Dactinomycin, Doxyorubicin, Cysclophosphamide, Etoposide, and Radiation
184
Q

How do you treat stage V WT?

A
  1. Neoadjuvant chemotherapy
  2. Sx therapy of each kidney individually
    1. RNx, PNx, wedge, enucleation
    2. Address less affected side first
185
Q

What are major a/e of chemotherapy agents used in WT?

A

Dactinomycin–myelosuppression

Vincristine–neurotoxicity

Doxorubicin–cardiotoxicity

Etoposide–myelosuppression

186
Q

What are major a/e of XRT for WT?

A

Scoliosis (if vertebral column adjacent to renal fossa radiated)

Radiation pneumonitis

Nephritis

Hepatitis

187
Q

What should children with WT be screened for?

A

Aniridia

Hemihypertrophy

Beckwith-Wiedemann (4-7% risk)

Denys-Drash (recommended b/l RNx prior to txp)

188
Q

f/up of WT patient post RNx?

A

RBUS q3 mo until 7 yo

189
Q

What is DDX of severe b/l prenatal hydronephrosis and bladder wall thickening?

A
  1. PUV
  2. Prune belly syndrome
  3. VUR
  4. NGB
  5. Urethral atresia
190
Q

What is mgmt of b/l prenatal hydronephrosis?

A
  1. Observation, collab with MFM
  2. Discuss with MFM regarding timing of delivery
  3. Consider check AF lecithin-sphingomyelin ratio (fetal lung maturity >2, consider early delivery)
191
Q

Indications for prenatal placement of vesicoamniotic shunt?

A

US with urinary tract obstruction:

  1. Singleton pregnancy w/o other major organ anomalies
  2. oligohydramnios or anhydramnios
  3. CVS or Amnio with 46XY (normal karyotype)
  4. <25 weeks GA
  5. Favorable fetal urine parameters 2-3 separate aspirations over 5-7 days

*Na <100, Ca <8, Osmo <210, B-2 microglobulin <4, Protein <20

192
Q

What is next step, baby boy in respiratory distress with bladder palpable to level of umbilicus?

A

Place feeding tube/catheter

Unable to, what next?

SP aspiration (temporize)

Vesicostomy in OR or at bedside if unstable and in NICU (anesthesia support)

193
Q

Key elements of cutaneous vesicostomy?

A

Adequately mobilize dome of bladder and resect urachal remnant

secure bladder to fascia

create large stoma through skin and fascia (at least 22 Fr)

194
Q

What is etiology of abdominal ascites in setting of PUV?

A

Urinary due to high intraluminal pressures in urinary tract, leads to urine extravasation across renal fornix

(significant could consider paracentesis but good bladder drainage allows extravasation site to heal)

195
Q

What are the type of PUV characterized by Dr. Hugh Hampton Young?

A
  1. Type 1 (95%) → leaflets at distal aspect of veromontanum an fan out to fuse in midline just proximal to external sphincter
  2. Type 2 → non-obstructing tissue secondary to distal urethral obstruction (no longer referred to as valves)
  3. Type 3 (5%) → membrane distal to veromontanum lying transversely across urethra with small perforation in center, windsock in bulbous urethra with voiding
196
Q

What study would you perform prior to surgery for PUV?

A

VCUG (dx dilated urethra, valves, thickened bladder, often VUR, diverticuli)

MAG 3

197
Q

Describe PUV ablation:

A

Cystoscopically with bugbee, resectoscope with hook or cold knife, or holmium laser

Incise valve at 5, 7, and 12

198
Q

b/l hydro persists after PUV ablation, next steps?

A

Observe

Repeat nuclear renogram or Whitaker test to assess UVJ

199
Q

Former PUV ablation, b/l hydro, recurrent UTIs and difficulty toilet training, next steps?

A
  1. VCUG
  2. If no valves, UDS
  3. If VUR, normal UDS, consider anti-reflux surgery if UTIs on CAP
  4. If no VUR, abnormal UDS, tx based on UDS results
    1. Younger boys have detrusor hyperreflexia and decreased size and compliance
    2. Older boys can have myogenic failure
    3. May require AC, alpha blockers, CIC, continuous nocturnal drainage, timed voiding, or combo
    4. A small % require augmentation and creation of continent catheterizable channel to lower Pdet
200
Q

What is valve bladder syndrome?

A

Constellation: bladder dysfunction (intrinsic) → deterioration of upper tracts → incontinence, poor sensation → high volumes, poor compliance

201
Q

Ddx of flank pain both urologic and non-urologic?

A
  1. Nephrolithiasis
  2. Pyelonephritis
  3. Appendicitis
  4. Cholecystitis
  5. Diverticulitis
  6. Placental Disorders
202
Q

When using US for flank pain, describe key elements that are helpful in determining obstruction?

A

Renal US (increased sensitivity if doppler used → the velocity of intrarenal blood flow can be quantified from resistive index (RI = PSV - EDV/PSV)

RI > 0.7 sens. 70-92% and spec 83-98% for acute obstruction

An inter-renal difference in RI of 0.06 AND asymmetric absence of ureteric jet supports the diagnosis

Endovaginal US (UVJ stones)

203
Q

What is physiologic hydronephrosis of pregnancy caused by?

A

Common 80-90% pregnancies

  1. Progesterone mediated smooth muscle relaxation
  2. Mechanical compression by ovarian veins and the uterus (R>L)
    • Dilation rare below pelvic brim
  3. Increased blood volume, renal perfusion, and urine production
204
Q

What imaging studies could be used for diagnosis of nephrolithiasis in pregnancy?

A
  1. Renal US → initial (no radiation), accuracy increased by doppler or vaginal US
  2. Limited IVP → second line, scout, 30 sec and 20 min, rad dose 0.20-0.25 rads to fetus
  3. Low dose CT → PPV 95.8% when combined with US, <15 mGy, discuss radiation
  4. Renal nuclear scan → obstruction but limited anatomic detail, radioactive iodine crosses placenta, can affect fetal thyroid > 10-12 weeks GA
  5. MRI → no radiation, sens for hydronephrosis, doesn’t visualize stones, costly and time consuming
205
Q

Risk of radiation to a fetus?

A
  1. Cell death and teratogenic effects:
    1. Teratogenic at 100-200 rad
    2. Greatest damage to CNS 8-15 GA
    3. Growth restriction, microcephaly, MR (as low as 20 rad)
    4. ACOG, no increased risk <50 mGy or 5 Rad
  2. Carcinogenesis
    1. Unknown but small
    2. ACOG 1-2 rad fetal, increase risk leukemia 1.5-20 x
  3. 1 Gy = 100 rad
206
Q

Options for ureteral stone in pregnancy?

A
  1. MET if hemodynamically stable, no signs infection, 80% chance of passage, IVF, abx, pain control, alpha blocker (limited data)
  2. Proceed with stent, PCN, or URS
207
Q

What the the STRONG recommendation in pregnancy for failure of MET?

A

Guideline Statement 56

In pregnant patients with ureteral stones, clinicians may offer URS to patients who fail observation.

Ureteral stent and nephrostomy tube are alternative options with frequent stent or tube changes usually being necessary.

(Index Patient 15) Strong Recommendation; Evidence Level Grade C

208
Q

What are safe abx during pregnancy?

A

PCN

Cephalosporins (ineffective against enterococcus)

Clindamycin (group b strep and PCN allergy)

Aminoglycosides (poss ototox and neprhotox, used with Amp for pyelo)

Sulfa: 1st trimester (teratogenicity), 3rd trimester (kernicterus/hyperbilirubinemia)

TMP: 1st trimester NTD

FQ: NO (tendons)

Tetracycline: NO (deiscoleration)

209
Q

What is first line for pyelonephritis in pregnancy?

A

Amp + Gent

Cephalosporins

95% women respond w/in 72 h

Tx for 2 weeks, transition to oral once sensitivities

Consider suppressive 50-100 nitrofurantoin until delivery

210
Q

Complications of untreated pyelonephritis in pregnancy?

A
  1. Fetal prematurity
  2. Low birth weight
  3. Premature labor
  4. Fetal mortality
211
Q

In young boy with acute scrotum, what are other important questions to ask about pts hx?

A

abdominal or flank pain?
when did you last eat or drink?
gross hematuria?
hx of scrotal trauma?
previous episodes?
dysuria or voiding sxs?
any bleeding problems, use of ibuprofen?
if prior sx, how did they handle anesthesia?

212
Q

what are important elements of PE for acute scrotum in young boy?

A

penis
perineum
scrotum (lie, location, cremasterics, swelling, tenderness, high riding, erythema)
abdominal/flank tenderness and pain
bladder fullness/discomfort
tanner stage

213
Q

what is ddx of acute scrotum?

A

epididymitis/orchitis
hernia/hydrocele
insect bite
testicular torsion
testis tumor
torsion of appendix testis or epididymis
trauma
varicocele

214
Q

what are appropriate diagnostic test for the acute scrotum?

A

scrotal US with doppler
UA, UCX if indicated
CBC

215
Q

risks of surgical exploration for acute scrotum?

A

anesthesia
bleeding/scrotal hematoma
chronic orchalgia
suture reaction/scarring
wound infection
loss of testis if deemed non-viable

216
Q

Describe b/l orchidopexy:

A
  1. scrotal or midline incision
  2. open dartos fascia on affected side, deliver testis, wrap in warm saline-soaked gauze
  3. open dartos contralateral side, deliver testis
  4. probe tunica vaginalis to r/o patent processus
  5. 2-3 point fixation of tunica albuguinea to adjacent dartos with permanent or LT absorbable (PDS or Prolene)
  6. re-evaluate affected side, possible doppler to assess flow, or incise tunica albuguinea to assess for bleeding
  7. pexy if viable, orchiectomy if not (call family to discuss)
  8. ensure meticulous hemostasis
  9. close wound in layers
  10. scrotal support
  11. limit contact sports 2 weeks, until pain free
  12. sport cup in long term
  13. wound check 3-4 weeks after
  14. discharge from clinic if doing well
217
Q

DDX for 4 yo boy with flank pain, n/v, left hydronephrosis and a cystic dilation w/in bladder lumen? Name 2 and treatment options

A

Obstructing ureterocele – incision

Megaureter –extrinsic compression of posterior bladder wall → stent → temporary distal cutaneous ureterostomy → definitive tapered ureteral reimplant

218
Q

Important principles of tapered ureteral reimplant:

A
  1. Create a smooth transition from proximal ureter to the tapered segment
  2. can do over 10-12 Fr Foley
  3. be mindful of devascularization when choosing ureteral segment to excise
  4. Mucosa to mucosa apposition
  5. 5:1 tunnel to ureteral diameter for an anti-refluxing repair
  6. Ureteral stent
  7. Foley
  8. Abx ppx
219
Q

What are of kidney most susceptible to scarring?

A

Upper and lower poles

*compound papilla with large open collecting ducts (intrarenal reflux of urine)

12. UroPed (1583) (13 decks)

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