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sinusitis, orbital cellulitis, or dental abscess. Often these patients will present with
a history of dental or facial pain, sinus congestion, erythema, or fever. A history
of environmental exposure should lead to the diagnosis of other common causes
of localized swelling including sunburn, frostbite, and plant-induced dermatitis
(poison ivy). Although rarely seen, pit viper envenomation may cause rapid onset
of painful swelling at the site of injury (see Chapter 90 Environmental
Emergencies, Radiological Emergencies, Bites and Stings ). Occasionally, an
infant will present with unexplained, localized swelling of an extremity that has
been present since birth. In this situation, the possibility of an injury secondary to
birth trauma should be explored. Less commonly, congenital lymphedema
(Milroy disease), Turner syndrome (bilateral leg edema), and Noonan syndrome
(pedal edema) should be considered. Meige disease (lymphedema praecox) is a
hereditary disorder that also results in lymphedema, but patients will present later
in childhood or around puberty, usually with swelling of the feet or lower legs.

FIGURE 25.1 Edema in children. GI, gastrointestinal; SIADH, syndrome of inappropriate
secretion of antidiuretic hormone.


Sickle cell anemia may cause swollen and painful digits in young children,
referred to as dactylitis (see Chapter 93 Hematologic Emergencies ).
Thrombophlebitis or deep venous thrombosis rarely occurs in the prepubertal
child but may affect adolescents; inherited hypercoagulable states, weightlifting,
indwelling catheters, and the use of oral contraceptive pills predispose patients to
this condition. Evaluation of these patients should include an ultrasound of the
venous system of the affected limb and a thorough laboratory evaluation.
Superior vena cava syndrome is a medical emergency caused by obstruction of
blood flow through the vessel, resulting from compression from a tumor,
thrombosis, or neoplastic invasion. This usually presents with shortness of breath
and swelling to the head, neck, or upper extremities, often with some degree of
cyanosis or plethora.

GENERALIZED EDEMA
Generalized edema, with an otherwise normal examination, occurs most
commonly in patients with renal disease, particularly nephrotic syndrome (see
Chapter 100 Renal and Electrolyte Emergencies ). The initial diagnosis is based
on significant proteinuria (3+ or >200 mg/dL on a urinalysis). A urinalysis should
therefore be included early in the evaluation of any pediatric patient presenting
with generalized edema. The presence or absence of urine red blood cells, white
blood cells, or casts in the urine, along with further laboratory testing including
chemistries, albumin, total protein, complement, and triglyceride levels may help
to confirm the diagnosis. Various factors, including the presence of hypertension
or significant fluid collections in the pleural or peritoneal spaces, must be
considered to determine the appropriate initial management of these patients.
Other forms of renal disease or vasculitis, including glomerulonephritis,
hemolytic uremic syndrome, or Henoch–Schönlein purpura (HSP) may cause
generalized edema. In the child with HSP, the swelling primarily affects the lower
extremities, where the purpuric rash predominates, or is isolated to specific joints
when arthritis is present. The purpuric rash, despite normal platelet count and
coagulation studies (consistent with a vasculitis), is usually, but not universally,
present.
The evaluation of the child presenting with generalized edema must also
include a complete and thorough cardiovascular examination. Patients with CHF,
pericarditis, myocarditis, or cardiomyopathy may present with edema, but these
children will often have additional signs and symptoms. An edematous child
presenting with a gallop, tachycardia, tachypnea, inspiratory crackles, or


hepatomegaly should be evaluated for cardiac disease (see Chapter 86 Cardiac
Emergencies ).
In an edematous patient with a normal cardiac examination and no proteinuria,

further evaluation should include a search for hepatic and other gastrointestinal
diseases, as well as other forms of vasculitis. Patients with protein-losing
enteropathy, from milk protein allergy, celiac disease, giardiasis, primary
intestinal lymphangiectasia (Waldmann disease) or inflammatory bowel disease,
can present with generalized edema with few other physical examination
findings. These patients may have significant protein loss through the GI tract and
will often present with hypoalbuminemia. An initial laboratory evaluation,
including liver function tests, electrolytes, erythrocyte sedimentation rate, creactive protein and measurement of total protein and albumin, may reveal
abnormalities. However, further evaluation, including more specific blood, urine,
and stool testing, is often required to definitively diagnose the etiology of edema
in this subset of patients.
As noted throughout this chapter, generalized edema may be a sign of a serious
underlying disease. However, less serious conditions may be causative as well.
Certain medications (oral contraceptive pills, corticosteroids, lithium,
nonsteroidal anti-inflammatory agents, calcium channel blockers, and others) may
cause some patients to become edematous. This swelling usually resolves when
the drug is discontinued. Cyclical edema related to menstruation occurs
frequently in young women. The etiology of this edema is likely hormonally
mediated, although the exact mechanisms are unclear. Pregnancy may result in
edema as well.
In conclusion, it is important to remember that a complete history and physical
examination of the patient with either localized or generalized edema may be
enough to arrive at a likely diagnosis. It is of particular importance to focus on the
renal, cardiovascular, and gastrointestinal systems when searching for an etiology
for generalized edema. Commonly, patients presenting with symptoms of
localized edema will have an allergic, traumatic, or infectious etiology and, with
appropriate management, will have resolution of their symptoms without serious
sequelae.
Suggested Readings and Key References
Braamskamp MJ, Dolman KM, Tabbers MM. Clinical practice. Protein-losing

enteropathy in children. Eur J Pediatr 2010;169:1179–1185.
Downie ML, Gallibois C, Parekh RS, et al. Nephrotic syndrome in infants and
children: pathophysiology and management. Paediatr Int Child Health


2017;37(4):248–258.
Farkas H, Martinez-Saguer I, Bork K, et al. International consensus on the
diagnosis and management of pediatric patients with hereditary angioedema
with C1 inhibitor deficiency. Allergy 2017;72(2):300–313.
Hsu DT, Pearson GD. Heart failure in children: Part I: history, etiology, and
pathophysiology. Circ Heart Fail 2009;2:63–70.
Katz BZ. Epstein-Barr virus infections. In: Long SS, Prober CG, Fischer M, eds.
Principles and Practice of Pediatric Infectious Disease . 5th ed. New York:
Churchill Livingstone; 2018:1088–1095.