Approach to child with generalized edema

1. Approach to child with generalized edema By : Ahmed Bahamid Pediatric resident @ Alsabeen hospital December, 9 th ,2012

2. History 19 months-old Yemeni boy from Dhamar C/O; Generalized body swelling 3 months

3. Cont. Hx. - History of present illness started 3 months earlier Gradual onset swelling 1 st in the eyelids (puffy eyes) & LL Progressive in course Seen in private clinics several times but no settled dx where made Ŕ by diuretics with temporary relief of edema.

4. Cont. Hx. The swelling eventually involve the entire body Face + abdomen + genetalia + LL Last 2 weeks Yellowish discoloration of the sclera Associated with low-grade fever

5. Review of systems Positive hx & Negative hx General; decreased activity, poor feeding, & Wt gain Skin; yellowish discoloration, itching of the scalp + hands + umbilicus, Cardiac; sweating and tiring with feeding, dyspnea started @ 3 months of age Respiratory; prolonged cough started @ 3 months of age and subsided with the start of recent complain

6. Cnot… GIT; anorexia, nausea, vomiting, No diarrhea with normal daily bowel motion and normal color. Genito-urinary; No difficulty with urination, No hematuria, No frothy urine , ONLY decreased urine output CNS; only irritability , NO abnormal movement, NO fits, or seizures, or weakness

7. Cnot… - Hematological; only pallor, NO hx of skin rash, bruises or bleeding - Musculoskeletal; No joint swelling or pain

8. PMHx No hx of similar attack Hx of fever with skin rash twice @ age of 3 months & 6 months Hx of prolonged cough since 3 months of age treated several times @ private clinics as chest infections but no admissions No hx of operations, trauma, allergy or ch. Medical diseases

9. Pregnancy & neonatal hx Product of FT, NSVD @ hospital. Pregnancy with antenatal care with no major problems No perinatal complications Average birth weight No cyanosis or jaundice, NO neonatal resuscitation or admissions

10. Nutritional hx Exclusive breast feeding in 1 st 3 months Bottle feeding started @ 4 months of age with adequate amount & concentration( fabimilk formula 1 & 2) besides breast feeding ( till 9 months) Formula changed to Nido milk & 10 months of age Weaning started @ 8 months of age with rice, cheese, & biscuits.

11. Immunization & developmental hx Immunization hx up-to-date except the last measles dose Developmental hx appropriate as his previous siblings (but motor development decreased markedly with the recent disease)

12. Family Hx 55y 33y 18y 17y 2y 14y 12y 11y 8y 19 m Father (DM & HTN) & smoker Mother ( 1 abortion, No still births 3 rd girl sibling died @ 2y of age from ch. GE + vomiting with rickets Other siblings healthy, no similar condition or renal disease in the family

13. Physical Exam Conscious, irritable, looked ill, mild RD Afebrile, pallor & jaundiced Generalized edema ( face + abdomen + LL + genitalia ) Vital signs Heart rate ( 116 bpm ) RR ( 48 cpm ) BP ( 80/40 mmHg ) Temp. ( 36.3C, axillary )

14. Cont. P/E - Growth Weight 11 kg on admission (50 th percentile) now 11.6 kg Length 77cm (10 th percentile) HC 48.5 cm (75 th percentile)

15. Pictures of the edema

16. Cont. P/E HEENT Head; Closed Ant. Fontanelle Eyes; yellowish sclera + pale conjunctiva, puffy eyes ENT; NAD Neck; diffuse swelling of soft tissues but no congested neck veins., no significant LN enlargement

17. Cont. P/E Chest : normal shape, good air entry bilaterally, normal vesicular breathing, no added sounds. CVS : not visible apex beat?? & barely palpable, S1 + S2+ distant heart sounds pulses: rapid weak pulses, equal Capillary refill 4 seconds with cold extremities

18. Cont. P/E - Abdomen: 1- inspection; distension, no scars or dilated veins, everted slit shape umbilicus 2- palpation; tense, no tenderness, wall edema, hepatomegaly (liver 12 cm BCM, span 15 cm) firm-to-hard in consistency, not tender, round border. 3- percussion: +ve shifting dullness & transmitted thrill. 4- auscultation: +ve bowel sounds

19. Cont. P/E Genetalia : scrotal swelling with +ve transillumination Back : pitting sacral edema CNS ; NAD LL ; petting edema, level just below the knee LN ; no significant LN enlargement MSS ; no joint swelling or tenderness

20. Summary 19 months-old-boy Tired and sweating on feeding started @ 3 months of age Recurrent chest infection started @ 3 months of age Swelling started periorbital & in LL, then became generalized (last 3 months) Jaundice & low-grade fever (last 2 weeks) O/E; looks ill, mild RD, generalized edema + huge hepatomegaly + ascites + pallor + mild jaundice

21. Differential diagnosis 1- Renal - Nephrotic syndrome - Acute GN 2- Hepatic - ch. Active hepatitis (viral infection) - metabolic ( Gaucher disease, Nieman-pick disease, Wilson disease, GSD type IV) - chronic liver failure - malignancy (primary/secondary)

22. Cont… D/Dx 3- cardiac - CCF - constrictive pericarditis - restrictive cardiomyopathy - tricuspid valve disease 4- others - veno-occlusive disease - Budd-Chiari syndrome - superior vena cava thrombosis - cystic fibrosis

23. Investigations CBC; - Hb% 7.2 g/dl - PCV 22 - WBC 12.8 - Neut 50 % - Lymph 42 % - Mono 4 % - Eosin 4 % - Platelets 134,000

24. cont… investigations CRP: +ve (2+) RFT: (N) urea 16 mg/dl, creatinine 0.6 mg/dl LFT: T.protein 5.1 g/dl, albumin 2.7 g/dl, TSB 6.7 mg/dl, SGOT 72 U/L RBS: 78mg/dl Electrolytes: Na 112 mmol/l, K 5.2 mmol/l, Ca 6.7

25. cont… investigations Urine analysis: Normal Chest X-ray: globular cardiac shadow enlargement -

26. D/Dx 1- cardiac - CCF - restrictive cardiomyopathy - constrictive pericarditis 2- hepatic - ch. Active hepatitis (viral infection) - metabolic ( Gaucher disease, Nieman-pick disease, Wilson disease, GSD type IV) - chronic liver failure - malignancy (primary/secondary)

27. Cont… D/Dx 3- Renal - Nephrotic syndrome - Acute GN 4- others - veno-occlusive disease - Budd-Chiari syndrome - superior vena cava thrombosis - cystic fibrosis

28. Abdominal U/S: - Marked hepatomegaly, smooth surface, no focal lesion Signs of dilated IVC & hepatic veins Bilateral pleural effusion Partial collapse of Rt. Lower lobe Marked pericardial effusion Marked ascites

29. Abdominal CT- scan Markedly enlarged liver Retrograde filling of dilated IVC & hepatic veins, with no signs of thrombotic changes or obstructing agent, reflecting passive hepatic congestion related to cardiac cause Large amount of ascites Prominent dilatation of both atrium with relatively small ventricles & mild to moderate Rt. Sided pleural effusion

30. D/Dx Cardiac 1- restrictive cardiomyopathy? 2- constrictive pericarditis?

31. Echocardiography Picture of restrictive cardiomyopathy with congestive heart failure

32. Treatment & hospital course Ampicillin , IV 500mg QID Captopril, oral, 6.25 mg BID Lasix, IV, 10 mg BID Vitamin K, IV, 5mg single dose Definitive treatment: heart transplantation

33. Approach to a child with edema Definition & background Pathophysiology Causes Clinical approach investigations Management of edema

34. Definition and background of edema Accumulation of excess interstitial fluid and could be localized or generalized.   Edema results from either excess salt & water retention or from increased transfer of fluid across the capillary membranes.   Understanding of the Pathophysiology of edema is important in the clinical approach and management of this condition in children.

35. Cont. definition and background… Distribution: 1- Anasarca ; gross, generalized edema with profound subcutaneous tissue swelling. 2- Localized edema; does not reflect a sustained impairment in the ability to maintain normal Na balance. 3- Special forms of fluid collections in the different body cavities Hydrothorax (in pleural cavity) Hydropericardium (in pericardial cavity) Ascites (in peritoneal cavity)

36. Pathophysiology Generalized edema can arise via two different processes; Reduced intravascular volume leading to Na & water retention → under-filling edema   Na & water retention secondary to expanded plasma & intracellular tissue fluid volume accompanied by lack of natriuresis → over-filling edema.

37. Cont. Pathophysiology… Mechanism of under-filling edema Initiated with ↑↑ glomerular permeability to albumin → albuminuria → hypoalbuminemia → ↓↓ plasma oncotic pressure → movement of water from intravascular space to the interstitium. The contracted intravascular volume→↑↑ RAA activity +↑↑ SNS activity + ADH release These factors→ water & Na retention→ further ↓↓ plasma oncotic pressure→ setting up a vicious circle

38. Cont. Pathophysiology… Mechanism of over-filling edema Resulting from expanded extracellular volume that results from primary renal Na retention, possibly secondary to the renal damage. In over-filling edema the RAA system & SNS & ADH secretion are depressed .

39. Causes of edema Causes of edema according to physiological changes: Increased hydrostatic pressure Decreased plasma oncotic pressure (hypoproteinemic states) Increased capillary leakage Impaired lymphatic flow Impaired venous flow

40. Cont. Causes of edema according to physiological changes… 1- Increased hydrostatic pressure Acute nephritis syndrome Acute tubular necrosis Cardiac failure-low output (CCF) Cardiac failure-high output (hyperthyroidism, anemia, beriberi) Arteriovenous fistula Acute and chronic renal failure Constrictive Pericarditis & restrictive cardiomyopathy

41. 2- Decreased plasma oncotic pressure (hypoproteinemic states) Nephrotic syndrome Chronic liver failure, autoimmune hepatitis, fulminant hepatic failure Protein losing enteropathy Protein caloric malnutrition Severe burns

42. 3- Increased capillary leakage Insect bite, trauma, allergy, sepsis, & angio-edema Vasculitis (anaphylactoid purpura, SLE, dermatomyositis, polyarteritis nodosa, scleroderma, & Kawasaki disease)

43. 4- Impaired lymphatic flow Lymphatic obstruction (tumor), congenital lymphedema. Milroy disease in newborn Wuchereria bancrofti infection Post-surgical & post irradiation

44. 5- Impaired venous flow Hepatic venous outflow obstruction, superior/inferior vena cava obstruction 6- Others Myxedema, Hydrops fetalis, drugs like NSAIDs, steroids, vasodilators etc…

45. Clinical approach Confirm edema Assess distribution of edema: generalized VS localized edema Detailed history and physical examination to assess severity, associated complications, and underlying cause of edema.

46. Clinical approach cont… Assess distribution of edema generalized VS localized edema In generalized edema look for pretibial, sacral, scrotal, vulval edema other than periorbital edema and ascites.

47. Clinical approach cont… Localized edema Hx. Of trauma, insect bite, or infection Peripheral lymphedema in female newborn  to exclude Turner’s syndrome Acute edema of the face and neck  to exclude superior vena cava obstruction syndrome.

48. Clinical approach cont… B- Generalized edema 1- Renal disease (most common cause in children) Rapid onset edema, puffiness around the eyes, gross hematuria, oliguria, hypertension, cardiomegaly, pulmonary edema to suggest acute glomerulonephritis. Frothy urine suggests nephrotic syndrome. Absence of circulatory congestion differentiates nephrotic syndrome from nephritic syndrome.

49. Renal disease cont… Signs and symptoms of chronic insufficiency such as anemia, growth retardation, and uremic symptoms such as nausea and vomiting. Exclude secondary causes such as post-infectious glomerulonephritis (history of throat or skin infection in recent past), SLE, Henoch Schonlein purpura (skin rash & joint pain). Look for symptoms of hypertensive encephalopathy (headache, irritability, confusion, altered sleep pattern, & convulsion).

50. 2- Liver disease Ask for hx of fever, anorexia, vomiting, abdominal pain, progressive jaundice, fetor hepaticus, bleeding manifestations, clay color stool, black tarry stool, hematemesis, pruritis & abdominal distension. Stigmata of chronic liver disease such as palmar erythema, clubbing & spider naviae. HSM with gross ascites in the absence of jaundice to exclude portal vein thrombosis. Previous operation scar such as Kasai porto-enterostomy.

51. 3- Cardiac disease Symptoms of CCF such as decreased effort tolerance, orthopnea, paroxysmal nocturnal dyspnea in older children and poor weight gain, feeding difficulties, excessive sweating, bluish episodes and respiratory distress in infants. Signs of cardiomegaly, gallop rhythm, precordial pulge, pallor, cool extremities, elevated JVP, weak pulse, pulsus paradoxus, murmur, displaced apex beat, tender hepatomegaly, & lung crepitations. Assess for underlying cause such as structural heart disease, cardiomyopathy & myocarditis. Edema in cardiac disease often denotes a late sign in small children.

52. 4- Protein losing enteropathy Hx of chronic diarrhea, steatorrhea, foul stools, FTT, repeated infections & redcurrant abdominal pain. Detailed dietary history for possible cow milk allergy and gluten hypersensitivity Assess for complications of anemia, malnutrition and vitamin deficiency This condition should be considered in every case of unexplained edema (even without diarrhea) especially when it is associated with hypoproteinemia.

53. 5- Protein energy malnutrition (Kwashiorkor) Hx of anorexia, lethargy, diarrhea, vomiting, FTT, susceptibility to infections, night blindness, inadequate or inappropriate dietary hx especially prolonged lack of protein. In examination; growth parameters, pallor, apathy, irritability, skin changes, hair changes, & signs & symptoms of micronutrient deficiency.

54. 6- Allergic reactions Edema usually mild, commonly periorbital. Hx of allergen exposure such as medications, animal dander, food preservatives and coloring. Associated rashes such as urticarial. Assess for Steven-Johnson reaction.

55. Generalized edema Circulatory overload ? No Yes Proteinuria ? Proteinuria , hematuria? Yes No Yes No Acute GN Cardiac disease Nephrotic syndrome Stigmata of ch. Liver dis.? Yes No Chronic liver dis. Ch. diarrhea? Protein losing enteropathy

56. Investigations A- Urine dipstick & microscopy Proteinuria, hematuria, & casts are indicative of renal disease B- RFT Raised serum urea & creatinine are indicative of renal disease

57. C- Full blood count Normochromic Normocytic anemia suggest chronic disease Hypochromic microcytic anemia suggest IDA from occult GIT bleeding e.g. cow’s milk allergy Megaloblastic anemia suggests B12 and folate deficiency from small bowel disease

58. D- LFT Hypoalbuminemia in the absence of circulatory overload suggests hypoproteinemic states Hyperbilirubinemia and elevated liver enzymes suggests liver disease

59. E- Chest X-ray and ECG Cardiomegaly with prominent perihilar vascular markings/upper lobe diversion and left ventricular hypertrophy confirms intravascular fluid overload

60. N.B if these basic investigations do not reveal the cause of edema, further investigations may have to be done: - Echocardiography - Serum-ascites albumin gradient (SAAG) - CT scan or MRI abdomen

61. Ascitic fluid analysis SAAG > 1.1 gm/dl SAAG < 1.1gm/dl Liver cirrhosis Veno-oclusive dis. Fulminant hepatic failure Cardiac ascites Mixed ascites Liver metastasis Nephrotic syndrome TB Nutritional Collagen vascular dis. High SAAG, normal protein  Budd chiari synd. & constrictive pericarditis High SAAG, low protein  liver cirrhosis Low SAAG, low protein  nephrotic syndrome, TB, nutritional Low SAAG, normal protein  chylus ascites, pancreatic ascites

62. Management * General measures 1- Dietary management Na restriction to 2gm/m2/day Fluid restriction to 2/3 of maintenance depending on the severity of edema 2- Diuretics therapy 3- Bed rest 4- Specific therapy according to the cause

63. Points to remember Edema more in the morning and subsiding by evening is suggestive of renal edema Ascites to start with, followed by edema may suggest a possibility of hepatic failure Nutritional history combined with anthropometry, vitamin & mineral deficiency signs, points to the diagnosis of nutrition deficiency states like kwashiorkor Edema in the dependant part associated with tachypnea and abnormal findings in the heart suggests the diagnosis of cardiovascular diseases.

64. Thank you for your attention and patience