Pediatrics and Pregnancy Flashcards
2 types of Cryptorchidism:
- Congenital – extrascrotal from time of birth
- Acquired – intrascrotal at birth but ascent subsequently
- Ascending
- Entrapped – after prior inguinal sx
- Retractile – vigorous cremasteric reflex
- Atrophic – volume loss after inguinal or testicular sx d/t prolonged position in extrascrotal location/developmental failure
Extrascrotal locations of UDT
- Prescrotal – above or at scrotal inlet
- Superficial Inguinal Pouch – distal and lateral to external ring, anterior to rectus muscle
- Prepubic – at external ring
- Canalicular – within the inguinal canal
- Ectopic – most commonly perineal, perirenal, femoral, peripenile, contralateral scrotal
- Abdominal – peeping through or proximal to internal inguinal ring, near bladder, iliac vessels, or kidney
What are some risk factors for UDT?
- Prematurity (15–30%, term 1-3%)
- Low Birth Weight (<900 g → 100%, 2700-3600 g → 3%)
- Genetic Susceptibility
- Maternal smoking
- Maternal ETOH consumption
- Maternal analgesic consumption
- Maternal Estrogen exposure
Why SHOULD you obtain GH at initial evaluation of UDT?
Guideline Statement 1
There are 2 phases of descent
- Transabdominal – 22-25 weeks GA (located at internal ring)
- Inguinoscrotal – androgen dependent – 25-30 weeks GA
**Higher in premature infants and low birth weight–adjusted GA important
**Unlikely to descend after 6 months
Who SHOULD be the first and regular evaluator of a male child’s testis?
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)
When SHOULD infants with UDT be referred? And why?
Guideline Statement 3
6 months (adjusted age) to Peds Uro
Impaired testicular growth, germ cell/leydig cell loss, decreased fertility possible, and cancer
What SHOULD be done for acquired UDT and when?
Guideline Statement 4
Refer after 6 mo (corrected for GA) to Peds Uro
**1-7% peaking at age 8
What MUST providers do for phenotypic male newborns with b/l nonpalpable testis?
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
What tests are done to evaluate when b/l non-palpable testis and phenotypic male
- 17-hydroxyprogesterone
- LH
- FSH
- Testosterone
- Androstenedione
- Karyotype
What imaging SHOULD be performed for UDT?
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)
What SHOULD providers assess for with UDT and penile anomalies? What type of anomalies are concerning?
Guideline Statement 7
DSD
Hypospadias or Micropenis (either unilateral or b/l)
What SHOULD be done in boys with b/l UDT w/o CAH?
Guideline Statement 8
Measure Mullerian Inhibiting Substance (MIS)/Anti- Mullerian Hormone (AMH) and CONSIDER hormone testing to eval for anorchia
- Inhibin b – undetectable
- FSH – elevated
- LH – elevated (Sertoli in response to endogenous FSH)
- Testosterone – low (Leydig cells in response to endogenous LH or exogenous hCG)
HCG stimulation test → elevation in FSH/LH → testosterone will be low
How often SHOULD provider assess retractile testis?
Guideline Statement 9
Annually
**Monitor for secondary ascent (2-45%)
**Hyperactive cremasteric muscle/reflex, foreshortened patent processus vaginalis, or entrapping adhesions
SHOULD providers supplement with hormonal therapy for UDT?
Guideline Statement 10
NO
*possible benefit to improve fertility rather than induce testicular descent–improved fertility index and sperm count – no long term data
When SHOULD specialists perform surgery for UDT?
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
What SHOULD be done in prepubertal boys with palpable UDT?
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
Describe the 2 incision approach to orchidopexy:
*Inguinal and scrotal
- Inguinal incision between ASIS and pubic symphysis
- Dissect through abdominal layers to external oblique fascia
- Open external spermatic ring to expose inguinal canal
- Avoid ilioinguinal nerve
- Identify testis and divide gubernaculum
- Dissect patent processus vaginalis from spermatic cord and ligate at internal inguinal ring
- Mobilize cord and gain length, dissect tethering cremasteric fibers
- Scrotal portion–subdartos pouch, deliver and secure testis, avoid torsion on spermatic cord
- 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
What are risks associate with orchidopexy you would discuss with family?
- Infection
- Bleeding
- Damage to testicular vessels/atrophy of testis
- Damage to vas deferens
- Damage to ilioinguinal nerve (decreased sensation or pain to medial thigh, scrotum, and base of penis)
- Non-scrotal location
- Need to stage procedure
- Postoperative inguinal hernia
- Recurrence and need to revise in future
- Need for orchiectomy
- Testicular atrophy
What are some abnormalities you may notice during orchidopexy?
- Patent processus vaginalis (inguinal hernia)
- Abnormal fusion of epididymis and testis
- Looping vas deferens
- Small testicular size
- Epididymal atresia
What techniques can be used to gain length during orchidopexy?
- 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)
- Prentiss maneuver: bring testis medial to inferior epigastric vessels
- Dissect spermatic vessels further along retroperitoneum
- Extend incision, open internal obligue musculature
- Secure testis as distal as possible and stage procedure
What do you do if you transect a vas deferns during an orchidopexy?
- Attempt to re-approximate ends of vas deferens with a fine non-absorbable suture
- Counsel family regarding injury and possibility of reduced fertility in future
What is differential dx for non-palpable testis?
- Abdominal or transinguinal (peeping) testis
- Complete atrophy (vanishing)
- Extra-abdominal but not palpable due to patient factors (anxiety, obesity, etc)
What should you do with a 6 mo boy with left UDT, noted at birth?
- EUA w/in 1 year
- Unlikely to descend
- If palpable or nubbin present in setting of contralateral hypertrophy → open exploration
- If remains non-palpable → diagnostic lap
What are the indications for treating an UDT with orchidopexy?
- Optimize testicular function and reduce fertility risk
- Facilitate dx of testicular malignancy with self exam after puberty
- Prevent inguinal hernia
- Prevent testicular torsion
- Cosmesis
What may you find for a non-palpable testis during diagnostic lap at inguinal ring?
- Norma appearing intra-abdominal testis
- Atrophic or abnormal appearing intra-abdominal testis
- Normal testicular vessels and vas deferens transversing internal ring
- Blind-ending testicular vessels and vas deferens
- Vas deferens with no vessels at internal ring
During diagnostic lap you find hypoplastic testis, normal contralateral, how do yo proceed?
- Orchiectomy
- Contralateral orchidopexy (not absolutely indicated but may be performed to secure solitary healthy testis)
What SHOULD providers counsel family on long-term risks associated with UDT?
Guideline Statement 16
- 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
- Fertility–slight reduction in paternity rates (90% vs. 94%) due to germ cell loss, 65% for b/l UDT s/p sx
What SHOULD a provider do in a prepubertal boy with non-palpable testes?
Guideline Statement 13
- EUA to reassess palpability
- 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)
What SHOULD surgeon do during exploration of non-palpable testis?
Guideline Statement 14
Identify termination of testicular vessels
If vanishing, done
If nubbin, remove
*Beware long-looping vas
What patient and anatomic features indicate that a surgeon SHOULD perform orchiectomy at time of exploration for a UDT?
Guideline Statement 15
- Normal contralateral testis AND
- Short testicular vessels and vas deferens
- Dysmorphic or hypoplastic testis
- Post pubertal age
What is post operative f/up for orchidopexy?
1 and 6 mo check of size, consistency, and location
Annual testicular exam by PCP
What are success rates following orchidopexy?
- >95% for inguinal
- 70-90 % for 1-2 stage FS
What do you do for older boy who underwent orchidopexy as baby, now with faintly palpable testis?
- If palpable → observe (atrophy)
- If retracted → redo orchidopexy, or possible orchiectomy
Algorithm for Cryptorchidism (UDT)
Describe an index VUR patient?
4 yo with VUR and no evidence of BBD, who presents with febrile UTI and subsequent VUR on VCUG
Why is early detection and management of VUR important?
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
What affects can VUR have on a child? What follow up is important?
STANDARD
Overall health and renal function
Measurement of:
- Height
- Weight
- Blood pressure
- Serum creatinine (if b/l abnormalities found)
What lab evaluation is important for VUR?
RECOMMENDATION
UA for proteinuria and bacteriuria
If indicates infection → C&S
OPTION
Baseline GFR for future reference
What imaging is recommended for VUR?
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
Relationship between baseline UTI and DMSA renal cortical abnormalities
Rates of Resolution for VUR?
Grade 1: 80%
Grade 2: 68%
Grade 3: 45%
Grade 4/5: 17%
For VUR, what are OPTIONS for continuous antibiotic prophylaxis (CAP)?
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:
- BBD
- Recurrent febrile UTIs
- Renal Cortical Abnormalities
*Continuing use of CAP in children with Grade II - V seems prudent until more data are available
For children with VUR, to consider observation without continuous antibiotic prophylaxis what factors must be absent?
MUST BE WITHOUT
- BBD
- Recurrent febrile UTIs
- Renal Cortical Abnormalities
For ongoing monitoring of VUR, what child specific factors/labs are important to follow?
RECOMMENDATIONS:
General evaluation ANNUALLY
- BP
- Height
- Weight
- 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
For ongoing monitoring of VUR, what are the RECOMMENDATIONS for follow up imaging?
RECOMMENDATIONS:
- 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
- DMSA ONLY when US abnormal (greater concern for more scarring, i.e. breakthrough UTI, a higher grade of VUR, or elevated Cr)
For ongoing monitoring of VUR, what are the OPTIONS for follow up imaging?
OPTIONS:
- F/up VCUG after 1 year old in VUR grade 1-2 (observation)
- A single negative f/up VCUG establishes resolution
- Periodic upper tract imaging with renal US may be done to assess renal growth and presence of gross scarring
- DMSA to detect new scarring (esp. if febrile UTI)
What are symptoms of symptomatic breakthrough UTI in a child with VUR on CAP?
- fever
- dysuria
- frequency
- failure to thrive
- poor feeding
What is RECOMMENDED when child with VUR on CAP has BT-UTI? What factors influence treatment alternatives?
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
In VUR patients under observation who develop febrile UTI, next steps RECOMMENDATION?
RECOMMENDATION
Continuous Abx Prophylaxis
In VUR patients under observation who develop non-febrile UTI, what is an OPTION?
OPTION
Initiate CAP
*keep in mind that not all cases of pyelonephritis are febrile
What are resolution rates for surgical interventions for VUR? Open surgery and Endoscopic Single Injection of Bulking?
Open 98.1%
Endoscopic Bulking 83%
*Reported first year after intervention
What are some post operative VUR adverse events and their incidence?
- Obstruction → 0.4%
- Voiding disturbances → 4.2%
- Contralateral VUR (after unilateral treatment) → 9.6%
- New renocortical abnormalities → extreme variabilities in studies, study suggests can occur
What surgical OPTION may be used for VUR? RCTs have shown what trend in regards to febrile UTIs?
OPTION
Open and endoscopic methods
RCTs show a reduction in febrile UTIs in patient who undergo open surgical correction vs. CAP
Following resolution of VUR, either spontaneously or by surgical intervention, what is the general evaluation an when is it an OPTION vs. RECOMMENDATION?
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
Following resolution of VUR, either spontaneously or by surgical intervention, what evaluation is a RECOMMENDATION with occurrence of febrile UTI?
RECOMMENDATION
BBD or recurrent VUR
What long-term concerns are RECOMMENDED for communication with a child at an appropriate age, after resolution or surgical correction of VUR?
RECOMMENDATION
- HTN (particularly during pregnancy)
- Renal function loss
- Recurrent UTI
- Familial VUR (in child’s siblings and offspring)
How is the index patient < 1 yo usually diagnosed wit primary VUR?
- Prenatal Hydronephrosis
- Occurrence of UTI
What are criteria used to determine the need for surgery for VUR?
- Persistent grade II-V VUR
- BT-UTI
- Noncompliance
- Deterioration of renal function
What is the RECOMMENDATION for CAP in a child < 1 yo and a history of febrile UTI with VUR?
RECOMMENDATION
CAP (greater morbidity from recurrent UTI in infants)
What is the RECOMMENDATION and the for CAP in a child < 1 yo WITHOUT a history of febrile UTI with VUR?
RECOMMENDATION
CAP if VUR grades III-V
What is the OPTION and the for CAP in a child < 1 yo WITHOUT a history of febrile UTI with VUR?
OPTION
May OFFER CAP
For baby boys < 1 yo with VUR what OPTION may be considered based on increased risk of UTIs?
OPTION
Circumcision
*Insufficient data to evaluate degree of risk and its duration
For prenatal hydronephrosis (PNH) what is recommended follow up?
Post natal RBUS 24-48 h after birth (what parents expect)
AND
Repeated in an additional 10-14 days as an outpatient
What is average incidence of VUR in patients with PNH?
Approximately 16% (ranges 13 - 23%)
Higher in females
In regards to SFU Grade 1 - 2 PNH what are a providers OPTIONS?
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
In regards to SFU Grade 3 - 4 with PNH, what are is RECOMMENDED? WHY?
RECOMMENDATION
VCUG
High-grade hydronephrosis, hydroureter, abnormal bladder (late prenatal or postnatal), or development of UTI
Which post natal US findings correlate to which ddx, and why?
Isolated Hydronephrosis → UPJ or VUR
Ureteral dilation → Ectopic ureter, ureterocele, PUV (b/l)
Dilated bladder/bladder anomalies → ureterocele, PUV
You find hydronephrosis on postnatal US, bladder is normal. What are next steps?
Start CAP (until VUR excluded)
Infant follow up outpatient s/p VUCG and Renal Scan in ~2 weeks
What is recommendation for infant with UJPO?
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
What are different surgical options for open dismembered pyeloplasty in a child?
- Anderson-Hynes → standard method
- allows for excision of abnormal UPJ, renal pelvic reduction, transposition (if crossing vessel)
- contraindicated if long proximal stricture
- approach: anterior subcostal, flank, dorsal lumbotomy
- ID renal pelvis, expose UPJ
- transect and excise UPJ
- spatulate ureter laterally and excise redundant pelvis
- anastomosis performed using fine interrupted or running suture
- stent or PCN
- leave perirenal drain
- Foley Y-V plasty
- High inserting ureter or horseshoe kidney
- Contraindicated if crossing vessel or pelvic reduction
- Spiral flap (Culp-Deweerd)
- Pelvic reduction or long proximal ureteral stricture
- Contraindicated if crossing vessel or pelvic reduction
- Vertical flap (Scardino-Prince)
- Good for pelvic reduction or medium length ureteral stricture
- Contraindicated if crossing vessel
What would you advise a parent about robotic pyeloplasty for an infant?
Small working space limits use until at least 6 months old
Operative time and results are comparable for an experienced surgeon
What are the presenting signs and symptoms of UPJO in a child?
- Episodic flank pain
- Cyclical n/v
- Azotemia (unlikely if c/l normal kidney)
- Kidney stones
- Pyelonephritis
What is an indication for nephrectomy in child with UPJO?
Massive hydronephrosis with <15% differential fxn
Follow up after successful dismembered pyeloplasty?
RBUS 1-2 mo after PCN or stent removed
What do you do if hydronephrosis on RUS after pyeloplasty?
- Repeat RBUS 1-2 mo (common in early post operative period)
- If does not improve, MAG 3 to assess differential fxn and t½
- If >15% fxn, would repeat pyeloplasty, spiral/vertical procedure, or ureterocalicostomy
What are the causes of UPJO in a child?
- Intrinsic
- Attenuated and separated muscle fibers w/increased collagen at UPJ → insufficient emptying of pelvis
- Valvular mucosal folds
- Ureteral polyps
- Persistent fetal convolutions
- Extrinsic
- Crossing vessel over lower pole
What are GU associated anomalies with UPJO?
- Contralateral UPJO (10-40%)
- VUR (both c/l and i/l)
- MCDK
- Duplex system (UPJO)
- VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, radial and renal dysplasia)
In case of prenatal hydronephrosis, what questions should be asked about prior pregnancies?
- Maternal hypertension and preeclampsia
- Late fetal demise
- 2 vessel umbilical cord
- sex/chromosomal abnormalities (trisomy 21)
- prior miscarriages, especially recurrent
- prior fetal anomalies, including GU
What important questions should be asked about fetal US if prenatal hydronephrosis detected?
- Are kidneys appropriate size/echonenicity for GA?
- Was any prior urinary tract dilation related to bladder fullness?
- Any other structural/organ anomalies?
- Evidence of dilated posterior urethra with thick walled distended bladder (keyhole sign)?
- Were ureters visualized?
- Was 20 week structural sonogram normal?
- Has bladder been identified and shown full and empty?
- Is amniotic fluid index (AFI) normal for GA?
What family history is important for neonate or fetus with hydronephrosis?
- UTIs (febrile)
- GU anomalies or other major organs (i.e. VACTERAL)
- chronic CKD or ESRD or family hx of renal txp
- young onset HTN
- VUR
- Unexplained fevers
- Early death d/t respiratory distress
- Accessory nipples or ear tags
What labs would you order on neonate with prenatal hydronephrosis?
- CBC & diff
- BMP (1 week for true neonate Cr)
- UA
Describe post natal PE on baby with PNH?
- check for accessory nipples or ear tags
- umbilical abnormalities
- lower extremity deformations from oligohydramnios
- other congenital anomalies
- pulmonary distress (bell shaped chest, abnormal APGARS)
- distended bladder
If increased hydronephrosis on post natal US (especially b/l) or bladder distention, next step?
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
What is appropriate CAP in neonate? What are there a/e?
Keflex or Amoxicillin
Loose stools
*C.Diff (watery stools) → peds GI → often probiotics
Rash
If neonates 10-12 day US show low risk UTD? Next steps
Consider repeat 3-4 months
Signs of UTI in neonate?
- May not have fever until 30 days
- Listelessness
- Excessive irritability
- Altered feeding habits
What should be done if PUV suspected or distended bladder?
RUS
VCUG
Trend Serum Cr
CAP
If cannot be done immediately, place 5 fr feeding tube in bladder