Overview

Definition:
-Central venous catheter (CVC) thrombosis in pediatrics refers to the formation of a blood clot within or around a CVC, obstructing blood flow and potentially leading to catheter dysfunction or serious complications
-This can involve thrombus formation on the catheter surface, within the lumen, or extending into the central veins.
Epidemiology:
-The incidence of CVC-related thrombosis in pediatric patients varies widely, ranging from 2% to over 50%, depending on patient population (e.g., oncology, critical care), catheter type, duration of use, and diagnostic methods employed
-Prematurity, underlying malignancy, sepsis, and inherited thrombophilias are significant risk factors.
Clinical Significance:
-CVC thrombosis is a common complication in critically ill or chronically ill children requiring vascular access
-It can lead to loss of access, increased morbidity (pain, swelling, venous distension, post-thrombotic syndrome), catheter-related infections, and, in severe cases, pulmonary embolism or stroke
-Early recognition and appropriate management are crucial for optimizing patient outcomes and preserving venous capital.

Clinical Presentation

Symptoms:
-Signs of CVC thrombosis may include absent or sluggish blood return from the catheter
-Edema or swelling of the ipsilateral limb or face
-Engorgement of superficial veins in the affected area
-Pain or tenderness along the catheter tract or in the limb
-Fever or signs of local infection (erythema, purulence) may also be present, though not always directly due to thrombosis itself.
Signs:
-Physical examination may reveal a palpable venous cord, limb asymmetry in size, palpable lymphadenopathy, or a positive Homan's sign (though unreliable)
-Vital signs may be normal, but tachycardia or tachypnea could suggest a more widespread thrombotic event like PE
-A non-functioning or partially functioning catheter on aspiration is a key clinical clue.
Diagnostic Criteria:
-Diagnosis is typically made based on clinical suspicion combined with imaging
-While there are no formal diagnostic criteria for CVC thrombosis itself akin to DVT, a combination of the following is used: clinical signs and symptoms suggestive of thrombosis, and positive findings on vascular imaging
-Catheter tip cultures are essential to rule out catheter-related bloodstream infection (CRBSI).

Diagnostic Approach

History Taking:
-Key history points include duration of catheterization, catheter type and insertion site, underlying medical conditions (malignancy, cardiac disease, inflammatory conditions), history of previous thrombosis or thrombophilia, and recent immobility or trauma
-Inquire about any changes in catheter function or associated symptoms.
Physical Examination:
-A thorough physical examination should focus on the insertion site, the ipsilateral limb/extremity for edema, erythema, tenderness, and venous distension
-Palpate for venous cords
-Assess for signs of systemic illness or pulmonary embolism
-Examine the integrity of the skin over the catheter tract.
Investigations:
-The gold standard for diagnosis is ultrasound with Doppler of the suspected venous segment and the catheter tip
-Echocardiography may be used for suspected superior vena cava (SVC) or right atrial thrombus
-Laboratory investigations may include a complete blood count (CBC), coagulation profile (PT/INR, aPTT), D-dimer (less sensitive in pediatrics), and possibly thrombophilia workup if recurrent thrombosis is suspected
-Catheter tip cultures are crucial if infection is suspected.
Differential Diagnosis:
-Other causes of arm or leg swelling and pain in a child with a CVC include cellulitis, local trauma, allergic reaction to the catheter or infusate, superior vena cava syndrome (due to extrinsic compression), or venous stenosis (post-inflammatory or post-radiation)
-Differentiating between catheter-related thrombosis and CRBSI is critical.

Management

Initial Management:
-If CVC thrombosis is suspected and the catheter is functioning, attempts to aspirate sluggish blood or administer thrombolytics may be considered
-If the catheter is not essential, removal may be the first step, followed by anticoagulation
-If the catheter is essential for life-saving therapy, it may be left in situ while anticoagulation is initiated, with careful monitoring.
Medical Management:
-Anticoagulation is the cornerstone of management for confirmed CVC thrombosis
-In pediatric patients, unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH, e.g., enoxaparin) are commonly used
-LMWH is often preferred for its predictable pharmacokinetics and subcutaneous administration
-Dosing is typically weight-based and adjusted based on PTT (for UFH) or anti-Xa levels (for LMWH)
-Thrombolytic therapy with agents like alteplase (tPA) may be used for occluded catheters to restore patency, administered as a continuous infusion or dwell time
-Warfarin may be used for longer-term anticoagulation after initial heparin therapy, with target INR 2-3.
Surgical Management:
-Surgical intervention is rarely indicated for CVC thrombosis itself
-It may be considered in rare cases of extensive thrombosis causing severe limb ischemia, or for removal of large thrombi that cannot be managed with anticoagulation or thrombolysis
-Catheter removal with or without surgical thrombectomy may be an option.
Supportive Care:
-Supportive care includes pain management, elevation of the affected limb, and meticulous monitoring for signs of extension of thrombosis or complications like pulmonary embolism
-Compression stockings may be used once acute swelling subsides for post-thrombotic syndrome
-If CRBSI is present, prompt antibiotic therapy is initiated.

Complications

Early Complications:
-Catheter dysfunction (occlusion)
-Catheter-related bloodstream infection (CRBSI)
-Extension of thrombus into proximal veins
-Pulmonary embolism (PE)
-Swelling and pain in the affected limb.
Late Complications:
-Post-thrombotic syndrome (chronic venous insufficiency)
-Venous stenosis or occlusion
-Development of new thrombotic events
-Recurrence of thrombosis.
Prevention Strategies:
-Use of maximal sterile barrier precautions during insertion
-Optimal catheter tip placement
-Avoiding unnecessary dwell time
-Diligent catheter care and flushing protocols
-Prompt removal of catheters when no longer needed
-Careful selection of insertion sites to minimize risk to central veins
-Education of healthcare providers on CVC care.

Prognosis

Factors Affecting Prognosis:
-The prognosis depends on the extent of thrombosis, the presence of underlying thrombophilia, the speed and appropriateness of treatment, and the presence of complications such as PE or CRBSI
-Early diagnosis and treatment generally lead to better outcomes and preservation of venous function.
Outcomes:
-With prompt and appropriate anticoagulation and/or thrombolysis, most pediatric patients achieve resolution of catheter thrombosis and restoration of catheter function
-Complete resolution of thrombus may take weeks to months
-However, some may develop long-term sequelae like post-thrombotic syndrome.
Follow Up:
-Follow-up includes monitoring for signs and symptoms of recurrent thrombosis or post-thrombotic syndrome
-In cases with significant thrombophilia, prolonged anticoagulation and specialist hematology follow-up may be required
-Regular reassessment of vascular access needs is also important.

Key Points

Exam Focus:
-Remember that absent or sluggish blood return is the most common sign of CVC thrombosis
-LMWH is often the preferred initial anticoagulant in pediatrics due to its predictability
-D-dimer is less reliable in children
-Ultrasound with Doppler is the primary diagnostic modality.
Clinical Pearls:
-Always consider CVC thrombosis in a child with a central line presenting with unexplained limb swelling or pain
-Differentiate between catheter occlusion due to fibrin sheath vs
-intraluminal clot vs
-external compression
-Do not blindly remove a central line if it is essential for therapy
-assess for thrombosis and manage accordingly.
Common Mistakes:
-Delaying diagnosis by attributing symptoms solely to infection or catheter malfunction without imaging
-Inadequate anticoagulation dosing or duration
-Failure to consider underlying thrombophilias in recurrent cases
-Over-reliance on D-dimer for diagnosis in pediatric patients.