Edema
René VanDeVoorde
INTRODUCTION
Edema refers to the clinical condition in which an excessive amount of fluid accumulates in the extravascular interstitial space of the body. The balance of competing Starling forces (hydrostatic and oncotic pressure) between plasma and the interstitium normally favors a small net movement of fluid from the capillary lumen into the interstitial space. This fluid is then collected by the lymphatic system and returned to the venous system via the thoracic duct. Edema occurs when these different forces become unbalanced either from alterations to capillary hemodynamics (change in hydrostatic or oncotic pressure gradients across the capillary wall), an increase in capillary wall permeability, or impaired lymphatic function. Edema may be localized or generalized. Localized edema is found in diseases of the lymphatic system, with venous obstruction, and in conditions associated with increased permeability of the capillary wall (e.g., infection, burns, trauma, and allergic reactions). Allergic reaction is the most common cause of localized edema in childhood. Generalized edema usually develops secondary to hypoalbuminemia (the main source of plasma oncotic pressure) or increased hydrostatic pressure from sodium and water retention. Minimal-change nephrotic syndrome (MCNS), although uncommon in and of itself with an annual incidence of 2-7 cases per 100,000 children, accounts for most cases of generalized edema. Severe generalized edema is referred to as anasarca. Additionally, localized edema may be seen in the abdomen (ascites) or chest (pulmonary edema or pleural effusions), but are typically associated with generalized edema.
DIFFERENTIAL DIAGNOSIS LIST
A variety of diseases may cause localized or generalized edema, with some contributing to both. They may be classified according to the pathophysiologic mechanism, although for localized edema the location of the edema may be more helpful as an approach to the diagnosis.
Localized Edema (Represent Distinctive Areas of Involvement)
Increased Capillary Permeability
Allergic reaction (insect bites, contact dermatitis, drug or food allergy)
Local trauma
Cellulitis
Angioedema (hereditary, ACE inhibitor induced)
Vasculitis (Henoch-Schönlein purpura – scalp, Dermatomyositis – eyelids, Kawasaki disease – hands or feet)
Hypothyroidism (pre-tibial myxedema, periorbital)
Epstein-Barr virus (bilateral upper eyelids, i.e., Hoagland’s sign)
Increased Plasma Hydrostatic Pressure
Venous thrombosis
Dactylitis of sickle cell anemia (hands or feet)
Extrinsic venous compression (tumor, lymphadenopathy)
Cirrhosis (ascites, bilateral lower extremities)
Impaired Lymphatic Drainage
Regional lymph node trauma or lymphadenitis
Lymphedema Praecox (lower extremities – 4 times more likely than upper extremities)
Turner or Noonan syndrome (neck, dorsal hands, or feet)
Filariasis
Milroy’s disease
Autoimmune involvement of lymphatics (sarcoidosis, Juvenile Rheumatoid Arthritis, Crohn’s)
Generalized Edema
Increased Capillary Permeability
Sepsis
Burns
Serum Sickness
Systemic Inflammatory Response Syndrome
Hypothyroidism (myxedema)
Other infections (Scarlet Fever, Rocky Mountain Spotted Fever, Roseola)
Decreased Plasma Oncotic Pressure (Hypoproteinemia)
Nephrotic syndrome
Hepatic failure (hepatitis, congenital fibrosis, cystic fibrosis, metabolic disorders)
Protein-losing enteropathy (milk protein allergy, Celiac disease, Menetrier’s disease, inflammatory bowel disease, Fontan physiology)
Protein-calorie malnutrition (kwashiorkor)
Severe anemia (hemolytic anemia) Beriberi
Increased Hydrostatic Pressure
Congestive heart failure
Cirrhosis
Renal failure
Glomerulonephritis (Postinfectious, hereditary, IgA Nephropathy, Henoch-Schönlein, Membranoproliferative)
Medications (vasodilators, corticosteroids)
Excessive iatrogenic intravenous fluid
APPROACH TO THE PATIENT WITH EDEMA
The general approach to children with edema is to first determine the character of the edema, localized versus generalized, as their subsequent etiologies are usually quite different. Localized edema can be unilateral or even fixed (not extending further) in distribution, whereas generalized edema would not. Generalized edema
often is dependent in nature, involving those areas affected by gravity most. This is typically the lower legs and feet in ambulatory children but may include the sacral area in nonambulating children. Generalized edema may also involve tissues that are easily distensible such as the eyelids, but also areas less easily examined like the scrotum or labia. However, localized edema may also be dependent, especially if it involves the lower extremity or scalp, or involve distensible tissues.
often is dependent in nature, involving those areas affected by gravity most. This is typically the lower legs and feet in ambulatory children but may include the sacral area in nonambulating children. Generalized edema may also involve tissues that are easily distensible such as the eyelids, but also areas less easily examined like the scrotum or labia. However, localized edema may also be dependent, especially if it involves the lower extremity or scalp, or involve distensible tissues.
As both localized and generalized edema can be associated with life-threatening conditions, the immediate evaluation should always be to insure that the patient is stable from a cardiorespiratory standpoint before proceeding. Localized facial edema could concurrently involve the airway while patients with generalized edema could have pulmonary edema and/or cardiac compromise, all potential medical emergencies. An initial inquiry into how the patient is breathing while assessing their respiratory effort (tachypnea, retractions), breath sounds (stridor, wheezing, rales), and heart rate (tachycardia) and heart sounds (S3 or S4 gallop) should be completed before proceeding with any extended work-up.
Localized Edema (Figure 31-1)
As allergic reactions, infection, and trauma are the most common causes of localized edema, symptom history and associated findings should first focus on these possibilities. Edema is often acute in onset in these cases with a history of antecedent trauma, either direct to the soft tissue (trauma), causing a break in skin integrity (cellulitis), or from an insect bite. Pruritis is often associated with allergic reactions while erythema or fever may be seen with cellulitis. The age of onset may also be helpful, as congenital causes of localized edema are fairly limited in scope (birth trauma, Milroy’s disease, Turner, or Noonan syndrome) while lymphedema praecox and hereditary angioedema tend to develop or worsen closer to puberty. A history of recurrent swelling episodes, even if they include different areas of localization, is suggestive of hereditary angioedema, especially if accompanied by recurrent abdominal pain episodes.