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

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

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. 86:F78-81, 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 >95% 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. 86:F78-81, 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 1999;80:F38–F42

11. Causes of Hypertension Leigh M. Ettinger and Joseph T. Flynn. Hypertension in the Neonate. NeoReviews 2002;3;151.

12. 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 • 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 2002;3;151.

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 • Wilm’s 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 (<6) • AFP 151,000 (<19,000) 139 105 12 112 4.1 1.1 23

20. Imaging Evaluation • Imaging • Start with plain film • Abdominal/Renal U/S • MRI • CT +/- contrast

23. Abdominal U/S

27. 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

28. Hydronephrosis

29. 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

30. 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

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 doesn’t 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

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 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

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. 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

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 (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)

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(< 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 > 1.5mg/dL