APPROACH TO CHILD WITH GENERALIZED EDEMA

By : Ahmed BahamidPediatric resident @ Alsabeen

hospital

December, 9th,2012

History

- 19 months-old Yemeni boy from Dhamar

- C/O;- Generalized body swelling 3

months

Cont. Hx.

- History of present illness started- 3 months earlier- Gradual onset swelling- 1st 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.

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

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

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

Cnot…

- Hematological; only pallor, NO hx of skin rash, bruises or bleeding

- Musculoskeletal; No joint swelling or pain

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

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-

Nutritional hx

- Exclusive breast feeding in 1st 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.

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)

Family Hx

55y

33y

18y

17y

2y

14y

12y

11y

8y19 m

Father (DM & HTN) & smokerMother ( 1 abortion, No still births3rd girl sibling died @ 2y of age from ch. GE + vomiting with ricketsOther siblings healthy, no similar condition or renal disease in the family

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)

Cont. P/E

- Growth • Weight 11 kg on admission (50th

percentile) now 11.6 kg• Length 77cm (10th percentile)• HC 48.5 cm (75th percentile)

Pictures of the edema

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

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

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

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

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

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)

Cont… D/Dx

3- cardiac - CCF - constrictive pericarditis - restrictive cardiomyopathy - tricuspid valve disease4- others - veno-occlusive disease - Budd-Chiari syndrome - superior vena cava thrombosis - cystic fibrosis

Investigations

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

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

cont… investigations

- Urine analysis: Normal

- Chest X-ray: globular cardiac shadow enlargement

-

D/Dx

1- cardiac - CCF - restrictive cardiomyopathy - constrictive pericarditis2- hepatic - ch. Active hepatitis (viral infection) - metabolic ( Gaucher disease, Nieman-pick

disease, Wilson disease, GSD type IV) - chronic liver failure - malignancy (primary/secondary)

Cont… D/Dx

3- Renal - Nephrotic syndrome - Acute GN

4- others - veno-occlusive disease - Budd-Chiari syndrome - superior vena cava thrombosis - cystic fibrosis

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

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

D/Dx

Cardiac 1- restrictive cardiomyopathy?2- constrictive pericarditis?

Echocardiography

Picture of restrictive cardiomyopathy with congestive heart failure

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

Approach to a child with edema

Definition & background Pathophysiology Causes Clinical approach investigations Management of edema

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.

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)

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.

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

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.

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

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

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

3- Increased capillary leakage Insect bite, trauma, allergy,

sepsis, & angio-edema Vasculitis (anaphylactoid

purpura, SLE, dermatomyositis, polyarteritis nodosa, scleroderma, & Kawasaki disease)

4- Impaired lymphatic flow Lymphatic obstruction (tumor),

congenital lymphedema. Milroy disease in newborn Wuchereria bancrofti infection Post-surgical & post irradiation

5- Impaired venous flow Hepatic venous outflow obstruction,

superior/inferior vena cava obstruction

6- Others Myxedema, Hydrops fetalis, drugs

like NSAIDs, steroids, vasodilators etc…

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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

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

Investigations

A- Urine dipstick & microscopy Proteinuria, hematuria, & casts are

indicative of renal disease

B- RFT Raised serum urea & creatinine are

indicative of renal disease

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

D- LFT Hypoalbuminemia in the absence of

circulatory overload suggests hypoproteinemic states

Hyperbilirubinemia and elevated liver enzymes suggests liver disease

E- Chest X-ray and ECG Cardiomegaly with prominent

perihilar vascular markings/upper lobe diversion and left ventricular hypertrophy confirms intravascular fluid overload

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

Ascitic fluid analysisSAAG > 1.1 gm/dl SAAG < 1.1gm/dl

Liver cirrhosisVeno-oclusive dis.Fulminant hepatic failureCardiac ascitesMixed ascitesLiver metastasis

Nephrotic syndromeTBNutritionalCollagen 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

Management

* General measures1- Dietary management Na restriction to 2gm/m2/day Fluid restriction to 2/3 of maintenance

depending on the severity of edema2- Diuretics therapy3- Bed rest4- Specific therapy according to the cause

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.

Thank you for your attention and patience