APNEC
Care of the Sick and Prematurely Born
October 21, 2009
Renal & Genitourinary Problems
Ravi Mangal Patel, MD
Fellow, Neonatology
Emory University School of Medicine
Renal Problems
• Hypertension
• Renal Masses
– Renal
– Juxtarenal
• Renal Failure
Genitourinary Problems
• Obstructive uropathies (PUV,UPJ,UVJ)
• Vesicourethral reflux
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Bladder exstrophy
Cloacal exstrophy
Prune Belly Syndrome
Ambiguous genitalia
Hypo/Epispadias
Case of Baby A
• Almost 36 week female noted to have an
abdominal mass on delivery room exam
• Born to a 25 y/o G2P1
• Prenatal labs unremarkable
• Ultrasound on day of admission noted
polyhydramnios and “echogenic abnormality”
in area of abdomen
Physical Exam
• Temp 36.6 °C, Pulse 138, RR 58, BP 117/71, SpO2 97%
• Weight 2.4kg (~50%), Length 44 cm (25%), HC 32 cm (~50%)
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General: Near term female
HEENT: Normocephalic, non-dysmorphic.
Chest/Lung: CTAB, unlabored
CV: RRR, no murmur
Abdomen: moderately distended with mass in right quadrant
Genitourinary: Normal female
Skin: No lesions
Neuro: Normal
Extremities: Normal
Neonatal Hypertension
• Healthy newborns – 0.2%
• Babies after NICU care – 2.6%
• Infants with CLD – up to 40%
• Worry about end organ damage
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Cardiac – Heart failure
Retinal – Retinopathy
CNS – Encephalopathy
Renal
Watkinson et al. Hypertension in the newborn baby.
Arch Dis Child Fetal Neonatal. 86:F78-81, 2002
Getting the correct BP
• First reading frequently higher than third
• Nwanko et. al. recommends following protocol:
– check BP 1.5 hours after the last feeding or intervention
– Apply appropriately sized cuff
• 2/3 the length of the limb segment
• Defined size markers on cuffs
– Wait 15 minutes/until patient still
– obtaining three successive readings at 2-minute intervals.
Definition of HTN
• AAP 2nd task force (70,000 American/British Children)
– BP >95% for age on three separate occasions
– In newborn, only systolic values used
• Term 95th Percentile
– DOL #1
– DOL #8-30
96mmHg
104mmHg
• Term 99th Percentile
– DOL #7-10
110-115mmHg
Watkinson et al. Hypertension in the newborn baby.
Arch Dis Child Fetal Neonatal. 86:F78-81, 2002
HTN in premature infants
120
100
104
94
80
83
74
71
60
67
SBP (mmHg) at 97% Day 1
62
SBP (mmHg) at 97% Day 10
57
40
20
0
24 weeks
28 weeks
32 weeks
36 weeks
Northern Neonatal Nursing Initiative. Systolic blood pressure in babies of less than 32 weeks
gestation in the first year of life. Arch Dis Child Fetal Neonatal Ed 1999;80:F38–F42
Causes of Hypertension
Leigh M. Ettinger and Joseph T. Flynn. Hypertension in
the Neonate. NeoReviews 2002;3;151.
Treatment of HTN
• Treat at SBP >99% or >95% with end-organ
involvement
• Medications:
– Emergent – use drips or IV medications
• Nicardipine or Nitroprusside / Labetalol or Hydralazine
– Non-emergent
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B-blocker – Propranolol – most extensively used
Diuretic – limited use in neonates for HTN
Calcium channel blocker – Amlodipine, Nifedipine
ACE inhibitor – Captopril
Leigh M. Ettinger and Joseph T. Flynn. Hypertension
in the Neonate. NeoReviews 2002;3;151.
Back to our patient …
Nitroprusside Drip Started
Physical Exam
• Temp 36.6 °C, Pulse 138, RR 58, BP 117/71, SpO2 97%
• Weight 2.4kg (~50%), Length 44 cm (25%), HC 32 cm (~50%)
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•
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•
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General: Near term female
HEENT: Normocephalic, Non-dysmorphic.
Chest/Lung: CTAB, unlabored
CV: RRR, no murmur
Abdomen: moderately distended with mass in right quadrant
Genitourinary: Normal female
Skin: No lesions
Neuro: Normal
Extremities: Normal
Differential of Abdominal Mass Renal
• Hydronephrosis
• Cystic diseases of kidney
– PCKD
– MCDK
• Renal vein thrombosis
• Solid tumors of kidneys
– Mesoblastic Nephroma
– Wilm’s Tumor
• Horseshoe or ectopic kidneys
Differential of Abdominal Mass Juxtarenal
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Neuroblastoma
Adrenal Hemorrhage
Pheochromocytoma
Teratoma
Sarcoma
Meconium Cyst
Lab Evaluation:
• Basic Metabolic Profile
• Urinalysis
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Urinary Electrolytes – Renal Failure
Urine HVA/VMA – Neuroblastoma
Teratoma - B-HCG & AFP
Renin – Renal Artery Stenosis
Urinary Catecholamines – Pheochromcytoma
Our Patient:
• BMP
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139 105 12
4.1 23 1.1
7.9
112
Urine HVA 20 (0-42)
Urine VMA 9 (0-27)
B-HCG 6.63 (<6)
AFP 151,000 (<19,000)
Imaging Evaluation
• Imaging
• Start with plain film
• Abdominal/Renal U/S
• MRI
• CT +/- contrast
Abdominal U/S
Differential of Abdominal Mass Renal
• Hydronephrosis
• Cystic diseases of kidney
– MCDK
– PKD
• Renal vein thrombosis
• Solid tumors of kidneys
– Mesoblastic Nephroma
– Wilm’s Tumor
• Horseshoe or ectopic kidneys
Hydronephrosis
Hydronephrosis
• Most common congenital condition on
prenatal ultrasound (1:500 to 1:700 deliveries)
• Causes are varied
– Physiologic (usually mild, up to 15%)
– UPJ or UVJ obstruction
– Vesicoureteral reflux
– Eagle-Barrett syndrome
– Posterior urethral valves
Hydronephrosis vs Pyelectasis
• 1 in 100 pregnancies w/ some evidence of dilation
• Hydronephrosis: Dilation of renal pelvis >1 cm
• Pyelectasis: Mild enlargement of renal pelvis 4-10
mm
– Often resolves
– F/u postnatal ultrasound
– May suggest increased T21 risk in older mom
Cystic Diseases of Kidneys
• Multicystic Dysplastic Kidney (MCDK)
• Polycystic Kidney Disease
– Autosomal Recessive
– Autosomal Dominant
Multicystic Dyplastic Kidney MCDK
• Most severe form of renal
dysplasia
• Multiple large cysts/ureteral
atresia
• Unilateral
• Sporadic (+/- VACTERL)
• Involutes over time
• Follow with serial U/S & remove
if doesn’t involute
• Urological testing on healthy
kidney
Polycystic Kidney Disease
• Genetic, with variable
expression
• Most commonly inherited
kidney disease
• Bilateral involvement
• Cysts may also be in liver
• Results in hypertension
‘Snowstorm’ appearance of
infantile polycystic disease
Polycystic Kidney Disease
Autosomal Recessive
‘Infantile’ PKD
Autosomal Dominant
Adult PKD
Frequency
1/40,000
1/10,000
Chromosome
6p21
16 (Codes for polycystin)
Diagnosis
Fetal U/S: large echogenic Rarely findings at birth
kidneys, oligohydramnios
Cysts
Kidneys  liver, as older
Large>2cms
Problems
Severe HTN, hepatic
HTN, renal insufficiency.
fibrosis, biliary dysgenesis, Worse as older
Potter’s Sequence
Kidney, liver, pancreas,
spleen. Variable size
Multicystic dysplastic kidney vs.
Polycystic kidney disease
MCDK
More common - 1/4000
Unilateral
Sporadic
Unilateral mass
Check urinary tract (90% w/ other
GU anomalies)
PKD
AD - 1/10000, AR - 1/40000
Bilateral
Genetic
HTN/ renal insufficiency/
oliguria/ Family hx
Check liver, spleen, pancreas
Renal Vein Thrombosis
Flank mass, hematuria, and renal failure:
• Usually w/in first 3 DOL, dx RUS w/ doppler
• Risk factors: Maternal diabetes, dehydration, sepsis,
hypovolemia, DIC, polycythemia, hypercoagulable
state
• Conservative, non-operative mgmt
• Thrombocytopenia (consumptive)
• Most frequent vascular condition
Renal Vein Thrombosis
(flank mass can be felt from congestion)
Tumors of Neonate
• Renal:
– Mesoblastic Nephroma
– Less common:
• Wilms (aka nephroblastoma)
• Clear cell
• Rhabdoid Tumor
• Adrenal:
– Pheochromocytoma (medulla of adrenals)
– Neuroblastoma
Mesoblastic Nephroma
• Most common renal neoplasm in 1st year of life
• Described by Bolande et al in 1967 (Pediatrics
1967;40:272)
• 60% are diagnosed before 6 months
• Neonatal tumors: 1:27,000
– Of which renal tumors are 7%
• Accounts for 3-6% of renal tumors in childhood and
is most frequent benign renal tumor
• Nephrectomy is generally curative
– chemotherapy for incomplete resection, infrequent local
recurrences and rare pulmonary mets
Mesoblastic Nephroma
• Can be seen with polyhydramnios (from
excessive fetal urine production)
• Peak age at presentation: 3 months
– Usually w/ large, palpable abdominal mass
• Males = females
Wilm’s Tumor
• Common tumor, but rarely diagnosed in the
first month of life
– 80% diagnosed between 1 and 5 years of age
• 15% associated with other syndromes
– WAGR
– Hemihypertrophy
– Beckwith-Wiedemann
• Most have good prognosis and are cured with
primary nephrectomy
Ectopic Kidney
• Kidney not located in usual position
• 1 in 1,000 births, but only about one in 10 of these
are ever diagnosed; up to 10% bilateral
Most common:
Horseshoe Kidney
Unilateral renal agenesis
Pelvic kidney
(Left kidney more likely to be abnormal)
Ectopic Kidney
• Function is generally normal initially, but…
• Abnormal position leads to obstruction in 50%
of ectopic kidneys
• Increased risk UTI, kidney stones, VUR
• Frequently associated with abnormalities of
other organ systems (uterine, cardiac, skeletal)
Ectopic Kidney Locations
Ectopic Kidney
(simple renal ectopia)
Horseshoe Kidney
• Most common renal fusion anomaly (1:400)
• As kidneys rise from pelvic area they fuse at lower
pole (90%)
• 33% without symptoms, although associated w/
Turner Syndrome, Trisomy 18
• Commonly present with UTI
• Diagnosis: Renal Ultrasound, VCUG
• Supportive Management, prophylactic antibiotics,
surgical intervention (stones)
Horseshoe Kidney
Patient course
• Patient underwent exploratory laparotomy
and resection of mass
• Findings: Pararenal immature teratoma with
no malignancy identified
Mass and Right Kidney
Post-op Course
• Developed oliguria (< 1cc/kg/hr) and increasing
creatinine post-operatively
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Admit
POD #0
POD #1
POD #2
POD #3
12 / 1.1
11 / 1.0
15 / 1.4
18 / 1.9
22 / 2.3
Consensus definition of
acute renal failure:
serum creatinine > 1.5mg/dL
Post-op Renal U/S
Pre-op
Post-op
Differential of Acute Renal Failure
• Prerenal
• Intrinsic
• Obstructive
Annabelle N. Chua and Minnie M. Sarwal.
Acute Renal Failure Management in the Neonate.
NeoReviews 2005;6;e369-e376
Prerenal vs Intrinsic Renal
Obtain Urine Na/Cr
• Intrinsic
– FeNa >2.5%
– RFI >3
Calculations
RFI = (Urine Na / Urine Cr ) x100
FeNa (%) =
(Urine Na / Serum Na)
(Urine Cr / Serum Cr)
x100
• Prerenal
– FeNa <2.5%
– RFI <3
Annabelle N. Chua and Minnie M. Sarwal.
Acute Renal Failure Management in the Neonate.
NeoReviews 2005;6;e369-e376
Serum BUN
Serum Creatinine
Patient CXR
Weight Trends
5
4.5
4.3
4.06
Kilograms
4
3.5
3.22
3
2.5
2
2.4
Birth
1wk
2wk
3wk
Dialysis - Indications
• Indications for dialysis:
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Fluid Overload
Hyperkalemia/Electrolyte Disturbances
Severe metabolic acidosis
Catabolism/Malnutrition
Symptomatic uremia
Drug toxicity (renally excreted)
Marsha M. Lee, Annabelle N. Chua and Peter D. Yorgin
Neonatal Peritoneal Dialysis. NeoReviews 2005;6;e384-e391
Patient Course
Peritoneal Dialysis Started
7
6
5
4
3
2
Serum Creatinine
Genitourinary Malformations
Genitourinary Problems
• Obstructive uropathies (PUV,UPJ,UVJ)
• Vesicourethral reflux
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Bladder exstrophy
Cloacal exstrophy
Ambiguous genitalia
Hypo/Epispadias
Urachal Anomalies
Obstructive Uropathies
• Posterior Urethral Valves (PUV)
• Ureteropelvic Junction Obstruction (UPJ)
• Ureterovesical Junction Obstruction (UVJ)
– Ureterocele
Posterior Urethral Valves
Posterior Urethral Valves
• Most common cause of obstructive uropathy
in males
– Does not affect females
• Incidence: 1/5000-1/8000
• Cause: congenital membrane which (partially)
obstructs urethra
Posterior Urethral Valves
• Clinical presentation:
– bilateral flank masses (hydronephrosis)
– distended bladder
– poor urinary stream (+/- dribbling)
• Diagnostic test: VCUG
• Therapeutic Goal: Preserve renal function,
avoid renal failure
– 30% at risk for progressive renal insufficiency
PUV
Posterior Urethral valves
• Immediate: Place Foley, Decompress Bladder
• Surgical therapy: Ablation of valves, urinary
diversion
– Fetal therapy w/ vesicoamniotic shunt
• Severe cases can present with Potter’s Sequence
– Oligohydramnios, pulmonary hypoplasia, uterine molding
Posterior Urethral Valves
Obstruction at urethra
Urinary bladder enlarges
Ureters become dilated & tortuous (hydroureter)
Back pressure in the collecting systems
Compromised development of renal parenchyma
Renal failure
Posterior Urethral Valves
Hydronephrosis
Dilated ureters
Thickened
trabeculated
bladder
Dilated proximal urethra
UPJ Obstruction
UPJ Obstruction
(Ureteropelvic)
• Most common cause of congenital
hydronephrosis
• Cause: Abnormal muscle development at UPJ
• Males > Females
• Can be associated with other congenital
abnormalities (e.g. VATER)
UPJ Obstruction
(Ureteropelvic Junction)
• Clinical/Prenatal Presentation:
– Prenatal: Hydronephrosis (unilateral)
– Post-natal: Renal mass (hydronephrosis) or workup
afterurinary tract infection
• Diagnosis: Obstructive pattern on diuretic
enhanced radionucleotide scan
UPJ Obstruction - Imaging
Radionucleotide Scan
MR Urogram
UPJ Obstruction – Surgical Repair
• Pyeloplasty - involves removing the blockage,
and reconnecting the ureter to the renal pelvis.
– Stent may be left across the pyeloplasty or a nephrostomy
may be left above the repair to decompress the kidney
UVJ Obstruction
(Ureterovesical Junction)
UVJ Obstruction
(Ureterovesical Junction)
• Second most common cause of hydronephrosis
• Cause: Deficient development of uereter or a
ureterocele
• Do not have dilated ureter in UPJ obstruction:
UPJ Obstruction
UVJ Obstruction
UVJ Obstruction
(Ureterovesical Junction)
• Clinical/Prenatal Presentation:
– Prenatal: Hydronephrosis (+/- Hydroureter)
– Post-natal: Renal mass (hydronephrosis) or workup
after urinary tract infection
• Diagnosis: Renal Ultrasound, Radionucleotide
Scan, VCUG
• Treatment:
– Surgical resection of obstruction
– May require stent placement, ~10% recurrence
Ureterocele
(outpouching of ureter as it enters bladder)
Vesicoureteral Reflux (VUR)
Mild
Moderate
Severe
Vesicoureteral Reflux (VUR)
• Accounts for 25-30% of antenatal
hydronephrosis & 1% of newborns
• Most (75%) children outgrow this during
childhood
• Presentation: UTI, hydronephrosis
– Up to 30% with other GU anomalies
– Males present earlier due to shorter ureters but
girls 2x more likely to have reflux
– 3x greater in whites > blacks
Vesicoureteral Reflux (VUR)
• Concern: increased risk of UTI, renal scarring
in 30-60% of Grade IV and V Reflux
• Meidcal Treatment:
– Antibiotic prophylaxis (controversial)
• Amoxicillin for < 2mo
• TMP-SMX or Nitrofurantoin QHS
– Surveillance ultrasounds
– VCUG
• Surgical Treatment
– open vs endoscopic - DEFLUX
VUR - DEFLUX
VUR - Monitoring
• Ismaili at al., prospective study of 43 patients
• At 2 years:
– Mild VUR spontaneously resolved in 91 percent
with low-grade reflux (grades I to III).
– In patients with high-grade reflux (grades IV and
V), VUR resolved in only 2 of 11 patients.
Ismaili K, Hall M, et. al. Primary vesicoureteral reflux detected in
neonates with a history of fetal renal pelvis dilatation: A prospective
clinical and imaging study. J Pediatr. 2006 Feb;148(2):222-227.
Genitourinary Malformations
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Bladder exstrophy
Cloacal exstrophy
Ambiguous genitalia
Hypo/Epispadias
Urachal Anomalies
Bladder Exstrophy
Bladder Exstrophy
• Spectrum of severity
– Small defect can result in epispadias
– large defect can result in exposure of posterior
bladder wall
• Occurs in 1 in 30,000 births
• Exposed bladder mucosa is edematous and
friable
Bladder Exstrophy
• Moist, fine-mesh gauze or vaseline gauze to
cover exposed bladder
• Antibiotics
• Renal ultrasound
• Transfer infant to surgical center
– Want surgical correction by 48-72 hours of life
– Sacroiliac joints are still pliable and the pelvis can
be “molded” to allow better approximation of the
pubic rami
• IVP of limited utility b/c of poor concentrating
ability of neonatal kidney
Bladder Exstrophy - Surgery
• Turn-in of the bladder to preserve bladder
function
• Symphysis pubis is approximated
• Iliac osteotomies aren’t necessary if repair is
within 48 HOL (bones are still pliable from
circulating maternal estrogens)
• Epispadiac urethra is reconstructed later
Bladder Exstrophy
Cloacal Exstrophy
Cloacal Exstrophy
• Very rare: 1 in 200,000 births
• Sporadic occurrence
• Complex of GI and GU anomalies:
– Imperforate anus
– Exstrophy of the bladder
– Omphalocele
– Vesicointestinal fistula
– Frequently w/ prolapse of bowel thru the fistula
on bladder mucosa
Cloacal Exstrophy – Preop
• Cover exposed mucosa (vaseline/saline gauze)
and/or plastic wrap to minimize heat loss
• Gender assignment / diagnosis
– Karyotype / FISH
– Controversial regarding assignment
• NG suction to relieve partial intestinal
obstruction.
– Stool frequently excreted through a vesicointestinal fistula that is often partially obstructed
Cloacal Exstrophy - Operative
• Survival 80% - mortality due to sepsis and
bowel obstruction
• Prompt surgery to separate fecal and urinary
streams
• Bladder can be closed during initial procedure
if baby stable
• Subsequent procedures to reduce the number
of stomas and create genitalia
Cloacal Exstrophy
• Multiple Long term problems:
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Psychosocial / Gender Identification
Recurrent UTIs
Bowel/Bladder Incontinence
Sexual function in later life
Ambiguous Genitalia
Ambiguous Genitalia - CAH
• CAH is the most common diagnosis in virilized XX
infants
• Presentation
– Hypoglycemia, vomiting, diarrhea, hypovolemia,
hyponatremia with hyperkalemia, and shock
• Management
– Monitor electrolytes, glucose
– 17-OHP high (Newborn screen / Lab Studies)
• Treatment: glucocorticoids (hydrocortisone)
• May require mineralocorticoids in salt-wasters
CAH – Multiple Types
Hypo/Epispadias
Hypospadias
Hypospadias
• Incidence: up to 1:250 births
• Associations: undescended testicles (DSD),
inguinal hernias
• Management: recognition, avoidance of
circumcision
• Surgery: usually by 2 years of life
Epispadias
• Incidence: 1:10,000 to 1:20,000
• Associations: Bladder exstrophy, VUR (40%)
• Management: recognition, avoidance of
circumcision
• Surgery: multistaged reconstruction
Epispadias
Urachal abnormalities
• Patent urachus:
– Connection remains between allantois and fetal
bladder
– Drainage/urine leakage through umbilicus
– Surgically remove @ 2 mo
• Urachal sinus:
– Drains to umbilicus
– Intermittent drainage from umbilicus (may be
serous or serosanguinous
• Urachal cyst:
– Fluid-filled structure occurring in between the
two obliterated ends of the urachus (distal end)
Urachal abnormalities
The End