APNEC Care of the Sick and Prematurely Born October 21, 2009 Renal - PowerPoint PPT Presentation

Loading...

PPT – APNEC Care of the Sick and Prematurely Born October 21, 2009 Renal PowerPoint presentation | free to download - id: 44cf2e-YjVmO



Loading


The Adobe Flash plugin is needed to view this content

Get the plugin now

View by Category
About This Presentation
Title:

APNEC Care of the Sick and Prematurely Born October 21, 2009 Renal

Description:

Fetal Anomaly GenitoUrinary ... 2009 Renal & Genitourinary Problems Slide 2 Renal Problems Genitourinary Problems Case of Baby A Physical Exam Neonatal ... – PowerPoint PPT presentation

Number of Views:176
Avg rating:3.0/5.0
Slides: 108
Provided by: Lis144
Category:

less

Write a Comment
User Comments (0)
Transcript and Presenter's Notes

Title: APNEC Care of the Sick and Prematurely Born October 21, 2009 Renal


1
APNECCare of the Sick and Prematurely
BornOctober 21, 2009Renal Genitourinary
Problems
  • Ravi Mangal Patel, MD
  • Fellow, Neonatology
  • Emory University School of Medicine

2
(No Transcript)
3
Renal Problems
  • Hypertension
  • Renal Masses
  • Renal
  • Juxtarenal
  • Renal Failure

4
Genitourinary Problems
  • Obstructive uropathies (PUV,UPJ,UVJ)
  • Vesicourethral reflux
  • Bladder exstrophy
  • Cloacal exstrophy
  • Prune Belly Syndrome
  • Ambiguous genitalia
  • Hypo/Epispadias

5
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

6
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)
  • 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

7
Neonatal Hypertension
  • Healthy newborns 0.2
  • Babies after NICU care 2.6
  • Infants with CLD up to 40
  • Worry about end organ damage
  • Cardiac Heart failure
  • Retinal Retinopathy
  • CNS Encephalopathy
  • Renal

Watkinson et al. Hypertension in the newborn
baby. Arch Dis Child Fetal Neonatal. 86F78-81,
2002
8
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.

9
Definition of HTN
  • AAP 2nd task force (70,000 American/British
    Children)
  • BP gt95 for age on three separate occasions
  • In newborn, only systolic values used
  • Term 95th Percentile
  • DOL 1 96mmHg
  • DOL 8-30 104mmHg
  • Term 99th Percentile
  • DOL 7-10 110-115mmHg

Watkinson et al. Hypertension in the newborn
baby. Arch Dis Child Fetal Neonatal. 86F78-81,
2002
10
HTN in premature infants
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 199980F38F42
11
Causes of Hypertension
Leigh M. Ettinger and Joseph T. Flynn.
Hypertension in the Neonate. NeoReviews
20023151.
12
Treatment of HTN
  • Treat at SBP gt99 or gt95 with end-organ
    involvement
  • Medications
  • Emergent use drips or IV medications
  • Nicardipine or Nitroprusside / Labetalol or
    Hydralazine
  • Non-emergent
  • B-blocker Propranolol most extensively used
  • Diuretic limited use in neonates for HTN
  • Calcium channel blocker Amlodipine, Nifedipine
  • ACE inhibitor Captopril

13
Leigh M. Ettinger and Joseph T. Flynn.
Hypertension in the Neonate. NeoReviews
20023151.
14
Back to our patient
Nitroprusside Drip Started
15
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)
  • 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

16
Differential of Abdominal Mass - Renal
  • Hydronephrosis
  • Cystic diseases of kidney
  • PCKD
  • MCDK
  • Renal vein thrombosis
  • Solid tumors of kidneys
  • Mesoblastic Nephroma
  • Wilms Tumor
  • Horseshoe or ectopic kidneys

17
Differential of Abdominal Mass - Juxtarenal
  • Neuroblastoma
  • Adrenal Hemorrhage
  • Pheochromocytoma
  • Teratoma
  • Sarcoma
  • Meconium Cyst

18
Lab Evaluation
  • Basic Metabolic Profile
  • Urinalysis
  • Urinary Electrolytes Renal Failure
  • Urine HVA/VMA Neuroblastoma
  • Teratoma - B-HCG AFP
  • Renin Renal Artery Stenosis
  • Urinary Catecholamines Pheochromcytoma

19
Our Patient
7.9
  • BMP
  • Urine HVA 20 (0-42)
  • Urine VMA 9 (0-27)
  • B-HCG 6.63 (lt6)
  • AFP 151,000 (lt19,000)

139
105
12
112
4.1
1.1
23
20
Imaging Evaluation
  • Imaging
  • Start with plain film
  • Abdominal/Renal U/S
  • MRI
  • CT /- contrast

21
(No Transcript)
22
(No Transcript)
23
Abdominal U/S
24
(No Transcript)
25
(No Transcript)
26
(No Transcript)
27
Differential of Abdominal Mass - Renal
  • Hydronephrosis
  • Cystic diseases of kidney
  • MCDK
  • PKD
  • Renal vein thrombosis
  • Solid tumors of kidneys
  • Mesoblastic Nephroma
  • Wilms Tumor
  • Horseshoe or ectopic kidneys

28
Hydronephrosis
29
Hydronephrosis
  • Most common congenital condition on prenatal
    ultrasound (1500 to 1700 deliveries)
  • Causes are varied
  • Physiologic (usually mild, up to 15)
  • UPJ or UVJ obstruction
  • Vesicoureteral reflux
  • Eagle-Barrett syndrome
  • Posterior urethral valves

30
Hydronephrosis vs Pyelectasis
  • 1 in 100 pregnancies w/ some evidence of dilation
  • Hydronephrosis Dilation of renal pelvis gt1 cm
  • Pyelectasis Mild enlargement of renal pelvis
    4-10 mm
  • Often resolves
  • F/u postnatal ultrasound
  • May suggest increased T21 risk in older mom

31
Cystic Diseases of Kidneys
  • Multicystic Dysplastic Kidney (MCDK)
  • Polycystic Kidney Disease
  • Autosomal Recessive
  • Autosomal Dominant

32
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 doesnt
    involute
  • Urological testing on healthy kidney

33
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
34
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 kidneys, oligohydramnios Rarely findings at birth
Cysts Kidneys ? liver, as older Largegt2cms Kidney, liver, pancreas, spleen. Variable size
Problems Severe HTN, hepatic fibrosis, biliary dysgenesis, Potters Sequence HTN, renal insufficiency. Worse as older
35
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

36
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

37
Renal Vein Thrombosis(flank mass can be felt
from congestion)
38
Tumors of Neonate
  • Renal
  • Mesoblastic Nephroma
  • Less common
  • Wilms (aka nephroblastoma)
  • Clear cell
  • Rhabdoid Tumor
  • Adrenal
  • Pheochromocytoma (medulla of adrenals)
  • Neuroblastoma

39
Mesoblastic Nephroma
  • Most common renal neoplasm in 1st year of life
  • Described by Bolande et al in 1967 (Pediatrics
    196740272)
  • 60 are diagnosed before 6 months
  • Neonatal tumors 127,000
  • 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

40
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

41
Wilms 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

42
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)

43
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)

44
Ectopic Kidney Locations
45
Ectopic Kidney(simple renal ectopia)
46
Horseshoe Kidney
  • Most common renal fusion anomaly (1400)
  • 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)

47
Horseshoe Kidney
48
Patient course
  • Patient underwent exploratory laparotomy and
    resection of mass
  • Findings Pararenal immature teratoma with no
    malignancy identified

49
Mass and Right Kidney
50
Post-op Course
  • Developed oliguria (lt 1cc/kg/hr) and increasing
    creatinine post-operatively
  • Admit 12 / 1.1
  • POD 0 11 / 1.0
  • POD 1 15 / 1.4
  • POD 2 18 / 1.9
  • POD 3 22 / 2.3

Consensus definition of acute renal failure
serum creatinine gt 1.5mg/dL
51
Post-op Renal U/S
  • Pre-op
  • Post-op

52
Differential of Acute Renal Failure
  • Prerenal
  • Intrinsic
  • Obstructive

Annabelle N. Chua and Minnie M. Sarwal. Acute
Renal Failure Management in the
Neonate. NeoReviews 20056e369-e376
53
Prerenal vs Intrinsic Renal
  • Obtain Urine Na/Cr
  • Calculations
  • Intrinsic
  • FeNa gt2.5
  • RFI gt3
  • Prerenal
  • FeNa lt2.5
  • RFI lt3
  • RFI (Urine Na / Urine Cr ) x100
  • FeNa ()

(Urine Na / Serum Na)
x100
(Urine Cr / Serum Cr)
Annabelle N. Chua and Minnie M. Sarwal. Acute
Renal Failure Management in the
Neonate. NeoReviews 20056e369-e376
54
Serum BUN
55
Serum Creatinine
56
Patient CXR
57
Weight Trends
1wk
2wk
3wk
Birth
58
Dialysis - Indications
  • Indications for dialysis
  • 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
20056e384-e391
59
Patient Course
Peritoneal Dialysis Started
7
6
5
4
3
Serum Creatinine
2
60
(No Transcript)
61
(No Transcript)
62
Genitourinary Malformations
63
Genitourinary Problems
  • Obstructive uropathies (PUV,UPJ,UVJ)
  • Vesicourethral reflux
  • Bladder exstrophy
  • Cloacal exstrophy
  • Ambiguous genitalia
  • Hypo/Epispadias
  • Urachal Anomalies

64
Obstructive Uropathies
  • Posterior Urethral Valves (PUV)
  • Ureteropelvic Junction Obstruction (UPJ)
  • Ureterovesical Junction Obstruction (UVJ)
  • Ureterocele

65
Posterior Urethral Valves
66
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

67
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

68
PUV
69
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 Potters Sequence
  • Oligohydramnios, pulmonary hypoplasia, uterine
    molding

70
Posterior Urethral Valves
71
Posterior Urethral Valves
Hydronephrosis
Dilated ureters
Thickened trabeculated bladder
Dilated proximal urethra
72
UPJ Obstruction
73
UPJ Obstruction(Ureteropelvic)
  • Most common cause of congenital hydronephrosis
  • Cause Abnormal muscle development at UPJ
  • Males gt Females
  • Can be associated with other congenital
    abnormalities (e.g. VATER)

74
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

75
UPJ Obstruction - Imaging
  • Radionucleotide Scan
  • MR Urogram

76
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

77
UVJ Obstruction(Ureterovesical Junction)
78
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
79
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

80
Ureterocele(outpouching of ureter as it enters
bladder)
81
Vesicoureteral Reflux (VUR)
Mild
Moderate
Severe
82
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 gt blacks

83
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 lt 2mo
  • TMP-SMX or Nitrofurantoin QHS
  • Surveillance ultrasounds
  • VCUG
  • Surgical Treatment
  • open vs endoscopic - DEFLUX

84
VUR - DEFLUX
85
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
Feb148(2)222-227.
86
Genitourinary Malformations
  • Bladder exstrophy
  • Cloacal exstrophy
  • Ambiguous genitalia
  • Hypo/Epispadias
  • Urachal Anomalies

87
Bladder Exstrophy
88
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

89
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

90
Bladder Exstrophy - Surgery
  • Turn-in of the bladder to preserve bladder
    function
  • Symphysis pubis is approximated
  • Iliac osteotomies arent necessary if repair is
    within 48 HOL (bones are still pliable from
    circulating maternal estrogens)
  • Epispadiac urethra is reconstructed later

91
Bladder Exstrophy
92
Cloacal Exstrophy
93
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

94
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
    vesico-intestinal fistula that is often partially
    obstructed

95
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

96
Cloacal Exstrophy
  • Multiple Long term problems
  • Psychosocial / Gender Identification
  • Recurrent UTIs
  • Bowel/Bladder Incontinence
  • Sexual function in later life

97
Ambiguous Genitalia
98
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

99
CAH Multiple Types
100
Hypo/Epispadias
101
Hypospadias
102
Hypospadias
  • Incidence up to 1250 births
  • Associations undescended testicles (DSD),
    inguinal hernias
  • Management recognition, avoidance of
    circumcision
  • Surgery usually by 2 years of life

103
Epispadias
  • Incidence 110,000 to 120,000
  • Associations Bladder exstrophy, VUR (40)
  • Management recognition, avoidance of
    circumcision
  • Surgery multistaged reconstruction

104
Epispadias
105
Urachal abnormalities
  • Patent urachus
  • Connection remains between allantois and fetal
    bladder
  • Drainage/urine leakage through umbilicus
  • Surgically remove _at_ 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)

106
Urachal abnormalities
107
The End
About PowerShow.com