Servelle–Martorell Syndrome (SMS)

Other names
Types
Causes
Symptoms and signs
Diagnostic tests
Non-pharmacological treatments
Drug treatments (medical therapies)
Dietary molecular supplements
Immunity-booster / regenerative / stem-cell drugs
Surgeries & procedures
Prevention
When to see a doctor urgently vs routinely
What to eat
FAQs

Servelle–Martorell syndrome is a very rare birth-present (congenital) vascular condition in which abnormally enlarged, twisty superficial veins grow through an arm or a leg and the bones in that limb stay small or thin. Doctors sometimes find no normal deep veins or missing venous valves, so blood tends to pool in the surface veins. Over years, the limb can look bulky on the outside because of the big surface veins and soft-tissue changes, yet the underlying bones are under-grown (hypoplastic) and the limb can be shorter or weaker. SMS is usually a venous malformation (a low-flow vascular anomaly). Arterial changes are uncommon. Most patients are best managed conservatively (e.g., compression, skin care); operations are considered only for selected complications. BioMed Central+3PMC+3Children’s Hospital Los Angeles+3

Servelle-Martorell syndrome (SMS) is a very rare, congenital (present from birth) vascular anomaly. It mainly involves slow-flow venous malformations in one limb together with under-growth of the bones and muscles of that limb (the limb is often shorter and thinner). Unlike better-known conditions such as Klippel-Trénaunay syndrome (which usually has limb overgrowth), SMS often shows limb hypotrophy/shortening plus phleboliths (small vein-stones) on X-ray. MRI and Doppler ultrasound confirm slow-flow venous channels and help plan care. Evidence is based on case reports and on broader research about venous malformations (VMs), because dedicated SMS trials are scarce. PMC+2Monarch Initiative+2 SMS belongs to the ISSVA family of vascular malformation syndromes (not tumors). Care is individualized, usually conservative first (compression, symptom control), with interventional radiology sclerotherapy, targeted medical therapy (e.g., sirolimus in selected complex VMs), and surgery reserved for specific problems. Multidisciplinary teams are best. PMC+1

Other names

Doctors and journals use several names for the same syndrome:

Servelle–Martorell angiodysplasia
Angio-osteohypotrophic (or angio-osteohypotrophic) syndrome
Phlebectatic osteohypoplastic angiodysplasia
All of these label a pattern of extensive venous malformations plus bony hypoplasia of the affected limb. Children’s Hospital Los Angeles+1

Unlike Klippel–Trénaunay syndrome (which often shows port-wine stains and overgrowth), SMS typically lacks a port-wine stain, shows diffuse venous malformations with phleboliths, no arteriovenous shunting, and the limb is often shorter due to bone under-development. Thoracic Key

Types

There is no official subtyping that all experts use, but clinicians often group cases in the following practical ways (to guide imaging and care):

By limb involved

Upper-limb–predominant SMS (classically described): prominent dilated superficial veins, hand/forearm muscle wasting, and hypoplastic small bones; sometimes extends to shoulder girdle. PMC
Lower-limb–predominant SMS: large superficial venous channels over the thigh/leg with bone hypoplasia, limb length discrepancy, and phleboliths on X-ray. Thoracic Key

By deep-vein anatomy

Absent or severely hypoplastic deep venous system / valve agenesis (blood diverts to superficial ectatic veins; higher pooling and bleeding risk). Children’s Hospital Los Angeles
Partially present deep venous system (some deep channels remain; still dominated by superficial venous malformations). Radiopaedia

By extent

Localized (one segment) vs Extensive (whole limb ± girdle). Extensive disease more often causes functional limits and orthopedic issues. PMC

By vessel mix

Purely venous malformation (most common).
Venous-predominant with minor arterial anomalies (uncommon). AHA Journals

Causes

For SMS no single proven, specific gene has been established. Experts think SMS arises from errors in vascular development before birth. However, venous malformations in general are often driven by somatic (post-zygotic) mutations in pathways such as TEK/TIE2 and PIK3CA, which over-activate PI3K–AKT–mTOR signaling; SMS likely shares biology with these venous malformations even if SMS-specific proof is limited. The list below combines SMS facts plus well-supported mechanisms from venous-malformation science that plausibly apply to SMS. Nature+3NCBI+3MDPI+3

Congenital venous malformation of a limb (low-flow, structural error present from birth). Radiopaedia
Under-development (hypoplasia) of limb bones associated with nearby venous malformations. PMC
Absent or poorly formed deep veins/valves, forcing blood to superficial channels. Children’s Hospital Los Angeles
Extratruncular, diffuse venous malformations, a pattern typical of SMS limbs. Thoracic Key
Intra-osseous venous malformations that thin bone and predispose to deformity/fracture. Children’s Hospital Los Angeles
Somatic TEK/TIE2 pathway activation (established in venous malformations; likely relevant biology for SMS). MDPI
Somatic PIK3CA activation in venous malformations (drives PI3K–AKT–mTOR signaling, abnormal vessel growth). MDPI+1
PI3K–AKT–mTOR pathway over-signaling in venous endothelium (core disease circuit in VMs). Nature
Hemodynamic stasis from missing deep veins/valves, enlarging superficial veins over time. Children’s Hospital Los Angeles
Localized intravascular coagulopathy (LIC) within venous malformations (thrombin generation, pain, bleed risk). PubMed
Phlebolith formation (calcified thrombi) inside malformations, signaling chronic slow flow. Radiopaedia
Bone demineralization/osteopenia from adjacent malformations and chronic disuse/pain. Thoracic Key
Muscle hypotrophy in the affected segment due to chronic venous congestion and disuse. PMC
Micro-hemorrhage into soft tissues, adding bulk and tenderness. BioMed Central
Orthostatic pooling (occasionally severe hypotension reported if deep system is absent). Lippincott Journals
Limb length discrepancy driven by bone hypoplasia; the limb is often slightly shorter. Thoracic Key
Rare arterial anomalies (reported but uncommon) accompanying the venous malformation complex. AHA Journals
Genetic mosaicism (mutations in a patch of tissue only), a common pattern in venous malformations. BioMed Central
Growth-related triggers (puberty, growth spurts) that can unmask/progress VM symptoms. EMBO Press
Unknown/idiopathic factors—SMS remains extremely rare and not fully mapped genetically. Children’s Hospital Los Angeles

Symptoms and signs

Visible, snake-like superficial veins over an arm or leg since childhood or adolescence. PMC
Heaviness and aching in the limb that worsens after standing or activity. BioMed Central
Soft-tissue “bulk” from enlarged venous channels, yet the limb bones are under-grown. Radiopaedia
Limb length difference (often the affected limb is shorter). Thoracic Key
Muscle thinning (hypotrophy) and reduced grip or push strength in the involved limb. PMC
Swelling (edema) that fluctuates, especially after long standing. BioMed Central
Tender lumps or phleboliths you can feel in the malformation. PMC
Easy bruising or surface bleeding after minor injury to the malformation. BioMed Central
Skin changes overlying the malformation (blue-purple hue; sometimes thin skin). BioMed Central
Painful “flare-ups” due to small clots inside the malformation (localized thrombosis/LIC). PubMed
Limited range of motion from bulk, pain, or prior procedures. PMC
Pathologic or stress fractures in the hypoplastic bone (rare but reported). Oxford Academic
Functional limits (difficulty with fine motor tasks in hand cases; gait changes in leg cases). PMC
Orthostatic dizziness or low blood pressure in severe, extensive cases with absent deep veins. Lippincott Journals
Cosmetic concern and psychosocial stress from the visible veins and limb asymmetry. BioMed Central

Diagnostic tests

Big picture: Diagnosis relies on history + examination + imaging to map veins and bones. Physiologic tests and labs help stage complications (like clotting inside the malformation).

A) Physical examination

Inspection of limb contours and veins. The clinician looks for long, tortuous superficial channels, skin color changes, scars, and limb length difference. Visible venous “lakes” suggest low-flow malformation rather than varicose vein disease alone. Radiopaedia
Palpation for phleboliths and tenderness. Firm, small, rounded “pebble-like” foci inside a soft mass are classic for venous malformations; focal tenderness suggests local thrombosis/LIC. Radiopaedia+1
Circumference and length measurements. Tape and block measurements document asymmetry (bulk vs shortening) and track change over time. Thoracic Key
Functional testing (range, strength, gait/hand tasks). Records impact on daily life and helps plan therapy (e.g., compression, physiotherapy). BioMed Central

B) Manual bedside venous tests

These classic tests help distinguish superficial from deep system issues. In SMS, many patients lack deep veins/valves, so findings often favor superficial filling; imaging still decides.

Brodie–Trendelenburg test. Assesses rapid superficial refilling when standing; very sensitive for superficial/perforator reflux (less specific). PMC
Perthes (modified) test. With a thigh tourniquet, walking should empty superficial veins if deep veins are patent; failure suggests deep system absence/obstruction. JAMA Network
Tap (Schwartz) and cough impulse tests. Detect transmitted wave along a refluxing superficial vein; supportive, not definitive. PubMed
Hand-held Doppler at bedside. Screens for flow direction/reflux before formal duplex imaging. PubMed

C) Laboratory and pathology tests

D-dimer (often elevated in venous malformations due to LIC). High values warn of thrombotic activity and pain risk. PMC
Fibrinogen (can be low in severe LIC), a bleeding-risk flag that guides anticoagulation in some patients. PubMed
CBC (complete blood count). Checks anemia (if recurrent bleeding) and platelets (rarely low in complex vascular-anomaly coagulopathies). JAMA Network
Coagulation panel (PT/INR, aPTT). Baseline before procedures; helps detect broader coagulation disturbance in large/active lesions. JAMA Network
Targeted genetic testing (lesional tissue) for VM genes (e.g., TEK/TIE2, PIK3CA) when results would alter care. Confirms VM biology and can open targeted-therapy options in research/selected settings. MDPI+1

D) Electrodiagnostic / physiologic venous tests

Air plethysmography (APG). Quantifies calf pump function, reflux, and outflow obstruction; useful adjunct to duplex in complex venous disease. PMC
Photoplethysmography (PPG). Non-invasive skin sensor estimates refill times; a sensitive screen for reflux (less specific). PubMed
Foot venous pressure (invasive hemodynamics, selected centers). Measures pressure fall on exercise; maps severity of venous outflow problems. Journal of Vascular Surgery

E) Imaging tests

Duplex ultrasound. First-line map of superficial channels, flow direction, thrombi, and any residual deep veins; low-flow pattern supports venous malformation. Radiopaedia
Plain radiographs of the limb. Show bone hypoplasia/osteopenia and phleboliths (round calcifications) that strongly suggest venous malformation. Thoracic Key+1
MRI/MR venography. Best overview of extent and depth (skin, muscle, bone, intra-osseous channels), and confirms low-flow characteristics; critical for surgical/IR planning. PMC
Catheter venography / CT venography (selected). Defines anatomy when interventions are planned or when deep venous absence/variants must be proven. JVS Venous

Non-pharmacological treatments

Because SMS is ultra-rare, these measures come from high-quality guidance on slow-flow venous malformations in general. Your team will tailor them to lesion size, location, and any LIC.

Medical-grade compression garments (stockings/sleeves).
Purpose: reduce venous pooling, swelling, aching, and risk of superficial thrombophlebitis.
How it works: external pressure reduces venous diameter, improves valve coaptation where present, increases flow velocity, and limits edema. Evidence supports routine use in low-flow malformations and after sclerotherapy. Classes I–II may already reduce VM volume on MRI. Wear daily, especially during standing/walking; replace every 6 months. PubMed+2Annals of Vascular Surgery+2
Intermittent limb elevation.
Purpose: symptom relief for heaviness and swelling; helps venous return.
How it works: gravity-assisted drainage reduces venous pressure and interstitial edema; use at rest and overnight.
Physiotherapy & graded activity.
Purpose: maintain joint range, strength, balance, and gait symmetry; prevent deconditioning and back pain from limb-length difference.
How it works: targeted strengthening and stretching improve muscle pump function, aiding venous return; gait training reduces compensatory overload. Orthopedic reviews on limb growth problems in vascular anomalies support conservative rehab first. Lippincott Journals
Aquatic therapy.
Purpose: low-impact strengthening with natural hydrostatic compression that often eases pain.
How it works: water pressure acts like gentle 360° compression; buoyancy reduces joint load while enabling active calf/forearm “venous pumps.”
Custom orthotics, heel lifts, and shoe adaptation.
Purpose: compensate for leg-length discrepancy (LLD), improve pelvic alignment, reduce back/hip/knee strain.
How it works: adding lift to the shorter limb levels the pelvis; consensus treats LLD ≥2 cm to prevent biomechanical problems. POSNA+1
Skin care & ulcer prevention.
Purpose: protect fragile, venous-stretched skin from trauma/infection.
How it works: daily emollients, careful nail/foot care, prompt care of cuts; compression plus hygiene lowers cellulitis risk.
Travel & immobility precautions.
Purpose: lower VTE risk on long trips/prolonged sitting.
How it works: stand/walk every 1–2 h, do calf pumps, hydrate; consider compression stockings on flights; LMWH only for high-risk travelers after medical advice. Aspirin is not recommended for travel VTE prevention. CDC Travelers’ Health+2PMC+2
Weight management & general fitness.
Purpose: reduce venous hypertension and joint stress, improve endurance.
How it works: aerobic activity supports calf pump; healthy weight reduces ambulatory venous pressure.
Pain self-management & pacing (mind-body).
Purpose: reduce flare–rest cycles and catastrophizing; improve function.
How it works: pacing, relaxation/breathing, cognitive strategies help chronic pain coping (adjunct to medical therapy).
Heat avoidance on the affected limb.
Purpose: lessen venous dilation and throbbing.
How it works: heat expands veins; prefer cool or lukewarm baths; avoid saunas/hot packs on lesion.
Protective sleeves/padding for sport or work.
Purpose: prevent knocks and superficial bleeding in superficial VMs.
How it works: shock-absorbing pads over clusters; combine with compression.
Lymphedema-style care if mixed components.
Purpose: manage swelling when VM coexists with lymphatic malformation.
How it works: modified manual drainage and bandaging by experienced therapists.
Multidisciplinary clinic follow-up.
Purpose: coordinate imaging, hematology (LIC), interventional radiology, orthopedic needs, and rehab.
How it works: ISSVA-aligned pathways reduce misdiagnosis and streamline sequence (conservative → sclerotherapy/targeted therapy → selective surgery). PMC+1
Activity modifications (smart load).
Purpose: keep moving without provoking venous congestion.
How it works: frequent micro-breaks, avoiding static standing; cycling and swimming favored over high-impact running if painful.
Pregnancy & hormonal planning.
Purpose: anticipate volume/LIC changes; plan compression and anticoagulation if indicated.
How it works: specialist counseling; estrogen raises VTE risk—discuss contraception options with hematology if LIC present. CDC
Peri-procedure clotting plans.
Purpose: avoid LIC-to-DIC worsening during big dental/orthopedic procedures.
How it works: pre-op labs (D-dimer/fibrinogen), LMWH bridging in high-risk, compression post-op per hematology guidance. JAMA Network
Education on red-flags.
Purpose: early recognition of DVT/PE and severe bleeding.
How it works: teach urgent symptoms (new one-sided swelling/pain; sudden breathlessness, chest pain, hemoptysis). CDC
Psychological support.
Purpose: body-image concerns, chronic pain, fatigue, and uncertainty are common.
How it works: counseling and peer support reduce distress; improves adherence.
Workstation & school adjustments.
Purpose: limit prolonged dependent limb position; allow movement/compression use.
How it works: sit–stand options, scheduled walk/stretch breaks.
Smoking cessation & alcohol moderation.
Purpose: better microvascular/skin health and healing.
How it works: reduces endothelial irritation and edema; supports safer procedures.

Drug treatments (medical therapies)

Doses below are typical starting points or ranges used in vascular-anomaly practice; your specialist personalizes dosing based on age, weight, labs, drug levels, and LIC status. Many uses are off-label for VMs/SMS.

Sirolimus (mTOR inhibitor).
Class/dose/time: mTOR inhibitor; common pediatric start 0.8 mg/m² twice daily with trough target ~10–15 ng/mL; adults often 1–2 mg/day, titrated; taken long-term with lab/level monitoring.
Purpose: reduce pain, swelling, bleeding, and lesion activity in complex slow-flow malformations; improve LIC markers.
Mechanism: inhibits PI3K/AKT/mTOR signaling that drives abnormal endothelial growth and coagulation activation in VMs.
Side-effects: mouth ulcers, high lipids, cytopenias, edema, rash, infection risk; needs vaccination review and contraception counseling. RCTs and prospective studies support efficacy for slow-flow malformations. Pediatric Radiology Handbook+2JAMA Network+2
Everolimus (mTOR inhibitor).
Class/dose/time: mTOR inhibitor; adults often 5–10 mg once daily (titrate to target troughs); course months–years.
Purpose: alternative when sirolimus not tolerated/available.
Mechanism/AE: similar to sirolimus (stomatitis, hyperlipidemia, cytopenias); drug level and lipid/CBC monitoring needed. Evidence extrapolated from vascular-anomaly cohorts using mTOR blockade. Journal of Vascular Surgery
Enoxaparin (LMWH).
Class/dose/time: anticoagulant; prophylactic dosing commonly used for LIC-related pain/thrombophlebitis (e.g., adults 40 mg SC daily or ~0.5 mg/kg daily; higher if treatment dosing is indicated) for 5–7 days during flares or longer if persistent LIC.
Purpose: relieve pain from microthrombosis and lower D-dimer; prevent LIC aggravation and procedure-related DIC in extensive VMs.
Mechanism: potentiates antithrombin to reduce thrombin generation within the malformation.
Side-effects: bruising/bleeding; injection-site pain; dose-adjust in renal impairment. Landmark studies identify LMWH as first-line pharmacologic treatment for painful LIC in VMs. PMC+1
Rivaroxaban (DOAC).
Class/dose/time: factor Xa inhibitor; real-world VM/LIC regimens often 10–20 mg once daily (off-label) in adults; duration individualized.
Purpose: alternative to LMWH for chronic LIC pain with elevated D-dimer or for peri-procedural control when injections are impractical.
Mechanism: directly blocks Xa, reducing thrombin generation inside VMs.
Side-effects: bleeding, drug interactions (CYP3A4/P-gp). Emerging series support symptom and D-dimer improvement in VMs. ScienceDirect
Dabigatran (DOAC).
Class/dose/time: direct thrombin inhibitor; adult regimens commonly 110–150 mg twice daily (off-label for LIC); adjust in renal disease.
Purpose: alternative oral anticoagulant when Xa inhibitors or LMWH unsuitable.
Mechanism: blocks thrombin; lowers microthrombi within lesions.
Side-effects: bleeding, dyspepsia; reversal available (idarucizumab). Small studies show pain and LIC marker improvements. PubMed+1
Acetaminophen (Paracetamol).
Class/dose/time: non-opioid analgesic; 500–1,000 mg every 6–8 h PRN (max 3 g/day in most adults).
Purpose: first-line for background pain without affecting clotting.
Mechanism: central COX modulation.
Side-effects: liver toxicity with overdose/alcohol. (General analgesic guidance; preferred when anticoagulated.)
NSAIDs (e.g., Ibuprofen, Naproxen).
Class/dose/time: anti-inflammatory analgesics; ibuprofen 200–400 mg q6–8 h PRN; naproxen 220 mg q8–12 h.
Purpose: short-term flare pain if not on anticoagulation and with normal renal/GI risk.
Mechanism: COX inhibition reduces inflammation.
Side-effects: gastric bleeding, kidney effects; avoid/limit if on LMWH/DOAC or if bleeding risk is high.
Topical lidocaine (patch/gel).
Class/dose/time: local anesthetic; 5% patch up to 12 h/day.
Purpose: focal neuropathic-like pain over superficial clusters.
Mechanism: sodium-channel blockade in nociceptors.
Side-effects: local rash/numbness.
Gabapentin/Pregabalin (neuropathic pain modulators).
Class/dose/time: gabapentin starting 100–300 mg at night, titrate; pregabalin 25–75 mg bid.
Purpose: nerve-type pain from tissue distortion.
Mechanism: α2δ calcium-channel modulation lowers neuronal excitability.
Side-effects: sedation, dizziness, weight gain.
Short antibiotic courses for cellulitis.
Class/dose/time: e.g., cephalexin 500 mg q6h × 5–7 days, per local guidance.
Purpose: treat skin infection over VM that can worsen swelling and LIC.
Mechanism: eradicates skin bacteria.
Side-effects: GI upset, allergy.
Proton-pump inhibitor (PPI) gastroprotection when NSAIDs required.
Class/dose/time: omeprazole 20 mg daily while NSAIDs used.
Purpose: lower GI bleeding risk.
Mechanism: acid suppression.
Side-effects: headache, rare hypomagnesemia.
Alpelisib (PI3K inhibitor) — only when a proven PIK3CA-related overgrowth spectrum (PROS) component coexists.
Class/dose/time: PI3K-α inhibitor; adults 250 mg once daily; pediatrics lower weight-based dosing; FDA-approved for PROS, not for typical SMS.
Purpose: targeted therapy for lesions driven by PIK3CA mutations (PROS subset), sometimes overlapping with complex combined malformations.
Mechanism: inhibits overactive PI3K-α signaling to reduce tissue overgrowth/vascular lesion activity.
Side-effects: hyperglycemia, diarrhea, rash; needs glucose monitoring and expert oversight. U.S. Food and Drug Administration+1
Low-dose opioids (short term only, if severe flares).
Class/dose/time: e.g., tramadol 25–50 mg PRN for brief periods.
Purpose: rescue analgesia while definitive VM care (compression/sclerotherapy) proceeds.
Mechanism: μ-opioid plus monoamine effects.
Side-effects: sedation, nausea, dependence; avoid long-term use.
Statins (selected adults with dyslipidemia on mTOR therapy).
Class/dose/time: per lipid profile.
Purpose: treat sirolimus-induced hyperlipidemia and reduce cardiovascular risk.
Mechanism: HMG-CoA reductase inhibition.
Side-effects: myalgia, liver enzyme rise; watch drug interactions.
Antihistamines (itch/urticarial flares over superficial VM).
Class/dose/time: cetirizine 10 mg daily PRN.
Purpose: symptom relief when skin over VM is irritable.
Mechanism: H1 blockade.
Side-effects: drowsiness (older agents).
Topical steroid for eczematous skin over compression areas (short courses).
Class/dose/time: hydrocortisone 1% bid for 5–7 days.
Purpose: treat dermatitis from garments/adhesives.
Side-effects: skin thinning if overused.
Vitamin D (if deficient).
Class/dose/time: cholecalciferol per local protocols.
Purpose: bone health in hypotrophic limb at risk of osteopenia from disuse.
Mechanism: improves calcium/phosphate balance.
Side-effects: hypercalcemia if over-supplemented.
Iron therapy (if chronic microbleeding or anemia).
Class/dose/time: ferrous sulfate 325 mg (65 mg elemental) qod.
Purpose: correct iron-deficiency anemia from oozing VM.
Side-effects: GI upset; check ferritin/TSAT.
Mouth-ulcer prophylaxis during mTOR therapy.
Class/dose/time: steroid mouthwashes (e.g., dexamethasone 0.5 mg/5 mL swish), non-alcoholic rinses.
Purpose: reduce aphthous-like stomatitis.
Side-effects: oral candidiasis if overused.
Vaccinations & infection prophylaxis planning on mTOR.
Class/dose/time: keep inactivated vaccines up to date before/while on therapy; avoid live vaccines while immunosuppressed.
Purpose/mechanism: lower infection risk while on sirolimus/everolimus; follow national immunization advice and specialty guidance. APS Journal

Dietary molecular supplements

No supplement shrinks an SMS lesion. A few venoactive plant extracts (used in chronic venous disease) may ease heaviness/edema for some people. Always check for interactions if you take LMWH/DOACs because several agents have antiplatelet/anticoagulant effects.

Micronized purified flavonoid fraction (MPFF: diosmin 90% + hesperidin 10%).
Dose: common regimens 500–1000 mg/day in divided doses.
Function/mechanism: improves venous tone, reduces leukocyte adhesion and inflammation, and may reduce edema and leg symptoms in chronic venous disease. Evidence is for CVD, not VMs; consider as a symptom adjunct only. PMC+1
Horse-chestnut seed extract (aescin).
Dose: standardized extract often 50 mg aescin twice daily.
Function/mechanism: venoactive; reduces capillary leakage and edema; RCTs in chronic venous insufficiency show symptom improvement. Avoid if on anticoagulants unless your clinician agrees. Raw seeds are toxic. Cochrane Library
Pycnogenol® (French maritime pine bark).
Dose: 100–300 mg/day in studies.
Function/mechanism: antioxidant/venoactive effects; trials in venous insufficiency show reductions in swelling and heaviness; quality of evidence varies. Interaction caution with anticoagulation. PMC
Vitamin D (if deficient).
Dose: personalized to blood levels.
Function/mechanism: supports bone health in a hypotrophic limb with altered loading.
Omega-3 (EPA/DHA).
Dose: often 1 g/day EPA+DHA.
Function/mechanism: anti-inflammatory; mild antiplatelet effect—do not add without discussing anticoagulation status.
Rutin/oxerutins.
Dose: product-specific (e.g., 500–1000 mg/day).
Function/mechanism: venoactive bioflavonoids; some CVD symptom benefit; watch bruising risk with anticoagulants.
Gotu kola (Centella asiatica) extracts.
Dose: product-standardized.
Function/mechanism: proposed microcirculatory benefits in CVD; evidence low-to-moderate; avoid with liver disease.
Quercetin-rich citrus bioflavonoids.
Mechanism: anti-oxidative/anti-inflammatory; symptom adjunct only; theoretical interaction with anticoagulants.
Magnesium (for cramps if present).
Mechanism: neuromuscular relaxation; use only for documented cramps; avoid in renal failure.
Protein-adequate balanced diet.
Function: supports skin healing and rehab; aim for sufficient protein and micronutrients; avoid extreme diets that promote dehydration or constipation.

Immunity-booster / regenerative / stem-cell drugs

Transparent answer: There are no approved or guideline-supported “immune-booster,” regenerative, or stem-cell drugs for Servelle-Martorell syndrome or for venous malformations. Care relies on compression, sclerotherapy, targeted agents like sirolimus in selected cases, cautious anticoagulation for LIC, and selective surgery. Using unproven “stem-cell” or “immune-boosting” products can cause harm and may interact with necessary anticoagulation. If you see clinics advertising these for VMs/SMS, seek a second opinion at a vascular-anomalies center. (See ISSVA-aligned reviews and guidelines.) PMC+2SpringerLink+2

Surgeries & procedures

Image-guided percutaneous sclerotherapy (interventional radiology).
What: Injection of a sclerosant (e.g., polidocanol foam, sodium tetradecyl sulfate, bleomycin, doxycycline, ethanol in expert hands) directly into the VM, often in multiple sessions.
Why: first-line procedure to shrink symptomatic lesions, reduce pain/bleeding, and make later surgery easier. Systematic reviews show good symptom control with low major-complication rates; agent choice balances efficacy vs safety. Neurointervention+2Annals of Vascular Surgery+2
Combined sclerotherapy then surgical excision/debulking.
What: pre-op sclerotherapy to reduce lesion flow/size, followed by limited surgical removal of residual bulky tissue or well-bounded VM.
Why: improves resectability, reduces blood loss, and can provide durable contour/function improvement in select cases. PMC
Debulking or staged resection for refractory bulk/pain.
What: partial removal of symptomatic VM tissue when sclerotherapy alone is insufficient.
Why: symptom control; radical cure is rarely the goal; recurrence possible; done in experienced centers. PMC
Endoscopic/minimally invasive resections (selected sites).
What: endoscopic removal for better cosmetic results in certain subcutaneous lesions.
Why: smaller scars, shorter healing in selected anatomy. Lippincott Journals
Orthopedic options for leg-length discrepancy (LLD).
What: shoe lifts/orthotics for mild LLD; consider epiphysiodesis (slowing the longer leg) in growing children with significant predicted LLD; lengthening is rarely considered due to bleeding risk and lesion biology.
Why: improve gait and spine alignment; decisions are individualized with orthopedics. POSNA

Prevention

Wear correctly fitted compression most waking hours when on your feet. PubMed
Move often: calf/forearm pumps, walking breaks; avoid long standing.
Elevate the limb during rest; sleep with slight elevation.
Protect the skin; moisturize; treat cuts early to avoid cellulitis.
Plan travel (hydration, aisle seats, movement; compression; high-risk travelers may need LMWH after clinician advice). CDC Travelers’ Health
Tell surgeons/dentists you have a VM; arrange pre-op coagulation plan if extensive disease/LIC. JAMA Network
Healthy weight and fitness to lower venous pressure.
Heat moderation (no hot tubs/saunas on affected limb).
Pregnancy & hormones: specialist counseling; avoid estrogen if VTE risk is high. CDC
Know red-flags of DVT/PE and seek urgent care if they appear (new unilateral swelling/pain; sudden breathlessness, chest pain, coughing blood). CDC

When to see a doctor urgently vs routinely

Urgent/emergency now: sudden one-sided limb swelling/pain, bluish discoloration, shortness of breath, chest pain on breathing, coughing blood, fainting, unstoppable bleeding from the lesion, fever with spreading skin redness. These can signal DVT/PE or serious infection. CDC
Soon (within days): new or worsening pain, repeated clots in the lesion (tender cords), ulcer that isn’t healing, rapidly enlarging lump, problems with compression fit, or new numbness.
Routine follow-up: periodic review with a vascular-anomalies team (imaging, LIC labs if extensive disease), orthotics check, and rehab progress. PMC

What to eat

Do aim for a balanced plate: lean proteins, colorful vegetables, high-fiber whole grains—supports skin and muscle recovery.
Do stay well-hydrated; dehydration thickens blood and worsens swelling.
Do favor foods rich in vitamin C (citrus, berries) and protein (fish/eggs/beans) to support wound repair.
Do include potassium-rich produce (bananas, leafy greens) to balance sodium and help fluid balance (unless restricted).
Don’t overdo salt; excess sodium draws fluid into tissues and increases edema.
Don’t binge alcohol; it worsens dehydration and bleeding risk.
Do maintain a healthy weight; every kilo less reduces venous load during standing.
Do discuss fish-oil, ginkgo, garlic, and high-dose vitamin E with your clinician if you’re on LMWH/DOACs—these can increase bleeding.
Do time fiber/fluids to avoid constipation (straining increases venous pressure).
Do take vitamin D only if low, as advised; avoid megadoses without testing.

FAQs

Is SMS the same as Klippel-Trénaunay or Parkes-Weber?
No. SMS typically has limb under-growth with venous malformations; KTS often shows overgrowth and capillary stain; Parkes-Weber has fast-flow AV shunts. PMC
What causes SMS?
The exact cause is unclear. It is a congenital vascular malformation syndrome; genetics are still being explored. Care follows VM principles. PMC
Will compression cure it?
No, but it controls symptoms and may reduce lesion volume and swelling. MDPI
When is sirolimus used?
In symptomatic, complicated slow-flow malformations (pain, bleeding, LIC, functional limits) after conservative steps; managed by specialists with drug-level monitoring. Pediatric Radiology Handbook
Is sirolimus lifelong?
Duration varies; months to years. Teams sometimes taper if symptoms and labs stabilize. Journal of Vascular Surgery
Are DOACs safe for LIC?
Early studies suggest rivaroxaban/dabigatran can help some patients; decisions weigh bleeding risk and are individualized. LMWH remains first-line for painful LIC flares. ScienceDirect+1
Does sclerotherapy hurt?
It’s an image-guided day-procedure; pain and swelling for several days are common. Multiple sessions may be needed. Overall safety is favorable when done in experienced centers. Neurointervention
What sclerosant is “best”?
No single best agent. Ethanol is powerful but riskier; bleomycin/polidocanol/STS are gentler with good safety; choice depends on lesion anatomy and center expertise. Neurointervention+1
Can surgery cure SMS?
Cure is uncommon. Surgery is typically debulking for bulk/pain or resection after sclerotherapy for discrete parts. Recurrence can occur. PMC
Should I worry about blood clots?
LIC within the malformation is common; your team may check D-dimer/fibrinogen and treat flares with LMWH. Know DVT/PE red-flags. JAMA Network+1
Are “stem-cell” or “immune-boosting” shots helpful?
No—not recommended or approved for VMs/SMS. Seek evidence-based care. SpringerLink
Can pregnancy make SMS worse?
VM symptoms may fluctuate with hormonal/volume changes; plan pregnancy with your team, use compression, and discuss clotting plans. CDC
What imaging is best?
MRI with contrast and Doppler ultrasound map lesion extent/flow; they guide treatment strategy. PMC
How often should I follow up?
At least annually in stable cases; more often if symptoms change, new clots, or before/after procedures—ideally in a vascular-anomalies clinic. PMC
What’s the outlook?
Most people manage well with conservative care and selective procedures; symptoms are chronic but controllable. Outcome varies by lesion size/location and presence of LIC. PMC

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