Phlegmasia Cerulea Dolens / Phlegmasia Alba Dolens
Overview
Phlegmasia เป็น severe form ของ DVT
เป็นภาวะ:
- limb-threatening
- life-threatening
เกิดจาก massive venous thrombosis โดยเฉพาะ:
- iliocaval
thrombosis
พบที่ lower extremity มากกว่า
upper extremity
Pathophysiology
Massive venous obstruction →
- severe
venous hypertension
- fluid
sequestration
- massive
edema
- impaired
microcirculation
- tissue
ischemia
progression:
DVT → phlegmasia alba
dolens → phlegmasia cerulea
dolens → venous gangrene
Types
1. Phlegmasia alba dolens
Collateral veins ยังพอ drainage ได้
Clinical triad:
- pain
- swelling
- pale/blanched
skin
ผิวยัง blanch ได้
2. Phlegmasia cerulea dolens
Collateral thrombosis ร่วมด้วย →
severe venous congestion
Clinical triad:
- severe
pain
- massive
swelling
- blue/cyanotic
nonblanching skin
3. Venous gangrene
microcirculatory collapse →
capillary thrombosis →
arterial compromise
นำไปสู่:
- tissue
necrosis
- compartment
syndrome
- limb
loss
Risk factors
Most common:
- malignancy
(~≥1/3)
Other associated conditions
- HIT
- antiphospholipid
syndrome
- trauma
- surgery
- pregnancy
- COVID-19
- femoral
venous catheter
- IVC
filter
- May-Thurner
syndrome
- congenital
venous anomalies
- hypercoagulable
states
Epidemiology pearls
- lower
extremity > upper extremity
- female
predominance (~5:1)
- left
leg more common
(สัมพันธ์กับ May-Thurner syndrome)
Clinical presentation
Symptoms
Classic:
- sudden
unilateral pain
- severe
swelling
Pain:
- severe
- constant
- out
of proportion
Progression
เริ่ม proximal →
ลามทั้ง extremity
อาจ progress:
- hours
→ days
Severe manifestations
- cyanosis
- sensory
loss
- weakness
- compartment
syndrome
- hypovolemic
shock
fluid sequestration อาจมากถึง:
Fluid sequestration approx 10 L
Physical examination
Early (alba)
- swollen
- tender
- pale/blanching
skin
Late (cerulea)
- tense
edema
- cyanotic
skin
- nonblanching
- absent
venous flow
Important vascular findings
ใช้ handheld Doppler ประเมิน:
- arterial
signals
- venous
signals
Progression pattern
Venous gangrene:
- venous
signals disappear first
- then
arterial signals lost
ต่างจาก arterial occlusion:
- venous
signals preserved initially
Shock findings
- tachycardia
- hypotension
- oliguria
Diagnosis
Clinical + duplex ultrasound
Supportive findings:
- massive
swelling
- severe
pain
- cyanosis
- venous
gangrene
Duplex ultrasound findings
- extensive
DVT
- noncompressible
veins
- absent/reduced
venous flow
ควรประเมิน:
- superficial
veins
- deep
veins
- arterial
flow
Iliocaval obstruction clues
Common femoral venous waveform:
- absent
respiratory variation
suggests proximal obstruction
Cross-sectional imaging
CT/MR:
- usually
not necessary before treatment
หาก clinical + duplex ชัดเจน
Differential diagnosis
Limb swelling
- cellulitis
- lymphedema
- compartment
syndrome
- uncomplicated
DVT
Acute limb ischemia
Arterial occlusion:
- pale
limb
- minimal
swelling
- delayed
venous filling
Phlegmasia:
- swollen
cyanotic limb
Treatment principles
Goals:
- stop
thrombus propagation
- restore
venous outflow
- reduce
venous hypertension
- preserve
limb
- prevent
shock
Supportive management
- absolute
bed rest
- aggressive
limb elevation (>30°)
- analgesia
- fluid
resuscitation
- monitor
rhabdomyolysis
- monitor
compartment syndrome
Anticoagulation
Start immediately
แม้ยังรอ ultrasound หาก suspicion
สูง
Preferred anticoagulant
IV UFH
เพราะ:
- rapid
titration
- short
half-life
- reversible
- suitable
for intervention
Duration
หลัง stabilize:
อย่างน้อย
Minimum anticoagulation duration = 3 months
Severity classification
Early phlegmasia
(Rutherford I; viable limb)
ยังมี arterial Doppler signal
Late phlegmasia
(Rutherford II; threatened limb)
ไม่มี arterial Doppler signal
Early phlegmasia treatment
เริ่ม:
- UFH
- limb
elevation
observe response ~12 hr
หากไม่ดีขึ้น:
→ venous intervention
Late phlegmasia treatment
ต้อง urgent intervention
เพราะ delay เพิ่ม:
- amputation
- mortality
Endovascular options
1. Mechanical thrombectomy (MT)
Preferred first approach
Advantages:
- rapid
clot removal
- faster
reperfusion
- shorter
ICU/hospital stay
2. Catheter-directed thrombolysis
ใช้:
- tPA
- urokinase
- streptokinase
often adjunct to MT
3. Surgical thrombectomy
ใช้เมื่อ:
- MT
unavailable
- failed
MT
- thrombolysis
contraindicated
After thrombectomy
หากมี venous stenosis:
- angioplasty/stenting
ถ้า stent:
- add
antiplatelet therapy
Fasciotomy
พิจารณาเมื่อ:
- compartment
syndrome
- reperfusion
injury
- venous
gangrene
Nonviable limb
→
amputation required
Complications
- pulmonary
embolism
- shock
- compartment
syndrome
- rhabdomyolysis
- venous
gangrene
- post-thrombotic
syndrome
- chronic
venous insufficiency
- limb
loss
Mortality & amputation
Mortality: 20%-66%
Amputation among survivors
Amputation rate 12%-50%
Major amputation:
- up
to 25%
Clinical pearls
- Phlegmasia
= severe iliocaval DVT with limb ischemia
- cyanotic
swollen painful limb = classic clue
- absent
arterial Doppler = threatened limb
- start
IV UFH immediately
- mechanical
thrombectomy preferred when available
- early
intervention improves limb salvage
- compartment
syndrome/reperfusion injury common
- mortality
และ amputation สูงมากหาก delayed
treatment
- malignancy
เป็น risk factor สำคัญที่สุด
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