Vascular malformations are difficult to treat and patients are affected psychologically and physically. The aim of the presentation is to discuss various treatment modalities available for management of venous malformations of Hand.

1. Management of
Venous
Malformations of
Hand
Dr.N.Mariappan
Dr.M.Srivignesh
Department of Hand Surgery,
SRI RAMACHANDRA
INSTITUTE OF HIGHER
EDUCATION AND
RESEARCH, CHENNAI

2. Clinical presentation
 22 years old male patient from West Bengal
presented with swelling of his right palm; right
middle and ring fingers since 2 years of age.
 Bluish discoloration of skin over the swelling
 No visible pulsation
 Reduces in size on hand elevation and reappears
on dependent position.
 Compressible / affected area is not warmer than the
surrounding areas / No thrill / No bruit on
auscultation.

3. Pre-operative
images
 The swelling extends from
the mid palm level to the
ulnar 3 fingers. Extends to
the TPX of ring and little
finger

4. Clinical diagnosis - venous malformation
Investigations
 X-ray of the right hand –Normal. No phlebolith.
 MRI angiogram of the right hand was done.
MRI Findings: The diagnosis was consistent with
venous malformation. Features of slow flow venous
malformation

5. MRI – Whole length of mid & ring finger and the proximal
phalanx of the little finger involved. Proximally it extends to
the distal edge of the flexor retinaculam

6. Plan-Excision of malformation of palm and mid finger
Possible complications:
 bleeding,
 necrosis and ulceration of the skin at suture line,
 neuropraxia and
 recurrence of the malformation
Discussed in detail and explained to the patient.

7. Surgical procedure
 General Anaesthesia
 Tourniquet control
 Loupe magnification
 INCISION:A zig-zag palmar incision with extension
to the ulnar border of PPX of mid finger and
extended till the pulp.

8. Operative findings
 Skin flaps were raised.
Extent :
 Proximally : Distal edge of carpal tunnel.
 Distal: Tip of middle finger
 The malformation was found to extend to the 2nd
and 3rd palmar lumbrical muscle level and involve
parts of the muscles.
 VM was surrounding the Neurovascular bundles
and flexor tendons to the middle, ring and little
fingers

9. VM of the
palm and
fingers

10. Surgical procedure
 Malformation encasing the NV bundles was carefully
dissected in the palm.
 The extensions into the lumbrical muscles were
secured with ligatures and excised partially.
 The venous malformation of palm ,middle finger and
PPX of ring finger were excised in toto.
 The wound was closed after hemostasis with drains.
 Dressing done and a dorsal POP slab was given.

11. Excision completed

12. Excised specimen

13. Follow up and mobilization of the Hand
 Drain tubes removed on POD 2
 Finger movements were normal. Edema of mid
finger.
 Normal sensation in all fingers except hyperesthesia
on the ulnar border of the middle finger.
 Patient was followed up till 6th POD and discharged
 Injection Sodium tetradecyl as sclerosant for ring
and little fingers in the next stage with a follow up
MRI.

14. Follow up – 8th post-operative day

15. Late Post –op

16. Full range of movements by
physiotherapy

17. Histopathology
 Predominantly fibro collagenous, fibromuscular and
adipose tissue with areas of dilated thick and thin
walled blood vessels few with occasional organizing
thrombus – consistent with Venous Malformation.

18. Venous Malformations of upper extremities and
Hand

19. Venous Malformations of the upper
extremities
Present at birth, VM are not always clinically evident
until later in life .Grow in concert with the child and
without spontaneous regression
VMs are sporadic and solitary. Greater than 5 cm
(56%), single (99%)
Multifocal, familial lesions: either glomuvenous
malformation (GVM) or cutaneo-mucosal venous
malformation (CMVM).

20. Venous
Malformation
Sporadic VMs
(94%),
Dominantly inherited
cutaneomucosal
VMs (1%)
Dominantly inherited
and non-inherited
glomuvenous
malformations (5%)
Classification (Vikula et al)

21. Distribution
47%
40%
13%
Percentage
Head & Neck
Extremity
Trunk

22. Types of VMs based
on angiographic
appearance
 Spongiform type
 Phlebectatic type
 Aneurysmatic type
 Reticular type

23. VMs of the Hand
 Almost all lesions involve
the skin, mucosa, or
subcutaneous tissue; 50%
also affect deeper
structures, such as
muscle, bone, joints, or
viscera.
 One third of the VM of
hand involve intrinsic
muscles of the hand.
 VMs involving muscle -
fibrosis, pain, and
disability.
Focal spongiform
VM

24. Venous Malformation
 Pain is the characteristic feature
 Bluish hue / normal skin
 Calcification due to repeated trauma with bleeding
leads to dystrophic calcification of the lesions
 Phleboliths could be palpated or seen in x-rays-
Pathognomonic of VM

25. Clinical features
 The primary morbidity of a venous malformation is
psychosocial, because most lesions affect the skin
and cause a deformity.
 The second most common complication associated
with venous malformation is pain, caused by
thrombosis and phlebolith formation.
 Stagnation within a venous malformation causes a
localized intravascular coagulopathy and
thrombosis.

26. Differential Diagnosis
Several conditions presenting as "blue
lesions": Differential diagnosis
Cutaneous angiosarcoma
Collateral venous network ,
Infantile hemangiomas ,
Dermal melanocytoses
Lymphatic malformations.
 The primary is lymphatic
malformation. Lymphatics arise
from veins embryologically.
 Venous malformation
(phlebectasia) may be a part of
combined malformation,
particularly lymphatic.

27. Laboratory tests
 D-dimer level : Biomarker for the diagnosis of VMs,
with high specificity but low sensitivity,
 40 % of patients with VMs have D-dimer levels
>0.5 mcg/mL and up to 25 % have levels
>1 mcg/mL.
 D-dimer levels are also helpful in differentiating
among variants of VMs & distinguishing VMs
from other vascular anomalies.
 D-dimer levels are normal in glomuvenous and
lymphatic malformations, Maffucci syndrome, and in
fast-flow lesions such as arteriovenous

28. Molecular and Genetic Diagnosis
Vascular Malformations Panel:
 is a 14 gene panel that includes assessment of non-
coding variants
 is ideal for patients with a clinical suspicion of
capillary, venous or arteriovenous vascular
malformations.
 is not ideal for patients with clinical suspicion of
lymphatic malformations

29. Investigations
 Plain X-ray of the hand
 Venous malformations that are present in deeper
locations are diagnosed on MRI and ultrasound
scan.
 Ultrasound : Differentiate vascular and lymphatic
malformations. (both classified as low-flow lesions).
 Contrast-enhanced CT to evaluate extent of
malformations , their complications such as acute
haemorrhage and confirms - calcification, thrombus
or concomitant lesions.

30. Non-invasive vascular investigations
 Whole body blood pool scintigraphy to detect 1.0
mL of abnormal blood pooling throughout the body –
In multifocal lesions assessment and for follow-up
 Trans-arterial lung perfusion scintigraphy to
identify micro-shunting arteriovenous malformation
 Radionuclide lymphoscintigraphy- only specific
test for assessment of the lymphatic system.
 Bone scanography

31. Invasive vascular investigations
 Direct percutaneous puncture with contrast
injection or phlebography (DPP) is the fine-needle
puncture of the VM with subsequent contrast
injection under fluoroscopy.
 DPP is the initial work-up of a VM prior to
sclerotherapy.
 It is the gold standard diagnostic tool
 Useful to confirm VM if required after alternative
imaging approaches have not yielded definitive
results, for treatment planning and ruling out

32. Invasive vascular investigations
 Ascending, descending, and/or segmental
venography or phlebography;
 Standard and/or selective arteriography;
 Percutaneous direct puncture angiography, ie,
arteriography or phlebography;
 Varicography or Lymphography.

33. ISSVA classification for vascular anomalies ©
(Approved at the 20th ISSVA Workshop, Melbourne, April 2014, last revision May 2018)
This classification is intended to evolve as our understanding of the biology and genetics
of vascular malformations and tumors continues to grow
Overview table
°defined as two or more vascular malformations found in one lesion
* high-flow lesions
A list of causal genes and related vascular anomalies is available in Appendix 2
The tumor or malformation nature or precise classification of some lesions is still unclear. These lesions
appear in a separate provisional list.
Vascular anomalies
Vascular tumors Vascular malformations
Simple Combined °
of major named
vessels
associated with
other anomalies
Benign
Locally aggressive or
borderline
Malignant
Capillary malformations
Lymphatic malformations
Venous malformations
Arteriovenous malformations*
Arteriovenous fistula*
CVM, CLM
LVM, CLVM
CAVM*
CLAVM*
others
See details See list

34. Benign vascular tumors
Infantile hemangioma / Hemangioma of infancy see details
Congenital hemangioma GNAQ / GNA11
Rapidly involuting Non-involuting Partially involuting
(RICH) * (NICH) (PICH)
Tufted angioma * ° GNA14
Spindle-cell hemangioma IDH1 / IDH2
Epithelioid hemangioma FOS
Pyogenic granuloma (also known as lobular capillary hemangioma) BRAF / RAS / GNA14

35. Locally aggressive or borderline vascular tumors
Kaposiform hemangioendothelioma * ° GNA14
Retiform hemangioendothelioma
Papillary intralymphatic angioendothelioma (PILA), Dabska tumor
Composite hemangioendothelioma
Pseudomyogenic hemangioendothelioma FOSB
Polymorphous hemangioendothelioma
Hemangioendothelioma not otherwise specified
Kaposi sarcoma
Others
Malignant vascular tumors
Angiosarcoma (Post radiation) MYC
Epithelioid hemangioendothelioma CAMTA1 / TFE3
Others

36. Non-operative treatment
 VM is a benign condition.
 Non-problematic lesions can be observed.
 VM has greatest expansion during adolescence
due to pubertal hormones in pathogenesis.
 Women are advised to avoid estrogen-containing
OCP.
 Estrogen has more potent pro-angiogenic activity
than progesterone.

37. Non-operative treatment
 Compression: Tailored compression garments are
indicated for symptomatic and extensive VMs of the
extremities to reduce pain and risk of thrombosis.
 Compression is contraindicated in glomuvenous malformations
(GVMs), as it increases pain and compression is ineffective in
Maffucci syndrome.
 Use of graded elastic compression stockings or
sleeves (for a VM of the leg or arm) to prevent
swelling

38. Non-operative treatment
 Pain control — Persistent pain and lesions not
amenable to compression are treated with low
dose aspirin and/or NSAID.
 When pain is associated with localized intravascular
coagulopathy (LIC), low molecular weight heparin at
a dose of 100 anti-factor Xa units/kg/day is
introduced for 20 days, or longer if pain relapses.
 Low-dose aspirin to minimize the formation of
painful blood clots.

39. Non-operative treatment
 Coagulopathy control — Coagulation evaluation
with measurement of blood levels of D-dimer and
fibrinogen is ideal before starting surgical treatment.
 Patients with evidence of LIC (D-dimer
>0.5mcg/ml) may develop DIC with increased risk of
bleeding intra-op.
 Prophylactic treatment with LMWH at a dose of
100 anti-Xa/kg/day started 24 hours prior to any
surgical procedure and continued for a total of 5-
7days.

40. Management options for VMs
 Sclerotherapy: Under general anesthesia, in an
angiography suite with specialized X-ray and ultrasound
equipment.
 Endo venous laser therapy : Involves placing a diode
laser fibre through a needle or catheter.
 Venous embolization : Placement of coils or
embolization glue, via catheter into the VM to seal
communication between VM & circulating veins. Done
in combination with sclerotherapy or surgery.
 Surgical excision involves removing the abnormal
veins and the tissue around them.

41. Venous malformations have propensity for
recanalization and recurrence
The first-line treatment for a problematic venous
malformation is sclerotherapy, which generally is safer
and more effective than resection. Exceptions to this
rule include the following:
Small, well-localized lesions that can be easily excised
for cure
Glomuvenous malformations which respond less-
favorably to Sclerotherapy
Venous malformations involving the palmar aspect of
the hand (or adjacent to important structures)

42. Sclerotherapy- sclerosants used include
 Sodium tetradecyl sulfate,
 Ethanol,
 Polidocanol,
 Alcohol solution of zein,
 Bleomycin,
 Sodium morrhuate, or
 Ethanolamine oleate.
 Sclerotherapy using sclerosant foam

43. Sodium tetradecyl sulfate(1%)
For Cutaneous and smaller lesions:
Sodium tetradecyl sulfate(1%) – reduces pain and
malformation size.
Sodium tetradecyl sulfate (10 ml of 3% solution
mixed with 2 ml of ethiodized oil [Ethiodol] and 10
cc of air).

44. Ethanol as sclerosant
 Used for larger lesions-95% ethanol
 More destructive than sodium tetradecyl sulfate
 Potential for causing local and systemic
complications.
 Dosage : < 0.5 to 1 ml/kg (maximum of 30 to 60 ml).
 Can cause nerve damage
 Inject cautiously adjacent to important structures.

45. Complications of Sclerotherapy
Local Systemic
 Sclerotherapy- related necrosis
and infection after STS and ethanol
sclerotherapy.
 pulmonary embolism
 reversible cardiac arrest after
polidocanol sclerotherapy,
 fatal cardio-vascular collapse
during ethanol sclerotherapy, and
extensive tissue necrosis
 transient hemoglobinuria and
oliguria.

46. Grades of complications - Clavien–Dindo
classification
Based on further treatment needed to manage the
complication.
Conservatively manageable - grades I and II,
Surgical, radiological, or endoscopic intervention-
requiring complications - grade III,
Life-threatening - grade IV, and fatal grade V
complications

47. Other treatment modalities
 Argon and Yttrium-aluminum-Garnet (YAG) laser
therapy for smaller lesions
 Neodymium yttrium-aluminium-garnet (Nd:YAG)
laser for venous lesions are small, located in difficult
anatomical situations, and have not responded to
other treatments with good control of VMs.
 It is usually administered via a flexible optic cable
and causes the photocoagulation of blood vessel
tissue
 Newer anti-angiogenesis drugs in future

48. Indications for surgery for VMs in
general
Surgery is rarely first-line therapy- considered in
select situations
(1) to ligate efferent veins to improve the results with
sclerotherapy,
(2) to remove residual VM after sclerotherapy,
(3) to remove lesion resistant to sclerotherapy, or
(4) localized lesion amenable to complete excision

49. Resection is less favorable than sclerotherapy because
The entire lesion can rarely be removed.
Excision may cause a worse deformity than the
malformation.
 Recurrence is likely, since abnormal channels
adjacent to the lesion are not treated.
The risk of blood loss and iatrogenic injury is high.

50. Hand VMs -Indications for surgery
 Pain, Swelling, Deformity and Localized venous
malformations
Staged Excision restricted by anatomical
considerations,
begin distally and progress proximally.
Subtotal resection :A) to reduce bulk B)Improving the
contour C) Better function

51. Resection should be considered for
Small, well-localized venous malformations that can
be completely removed
Persistent symptoms after completion of
sclerotherapy (patent channels are not accessible
for further injection)

52. Lesions involving the palmar aspect of
the hand:
 are best treated surgically, because if sclerotherapy
is performed first, fibrosis may prohibit later
operative intervention and would increase the
difficulty and risks of the procedure.

53. Diseases and conditions involve various
types of VMs.
 Glomovenous malformations contain nerve cells and cause
the malformations to become hardened and tense. These
types of malformations can be inherited and often occur in
multiple places.
 Blue rubber bleb nevus syndrome involves numerous
rubbery lesions that can appear both externally and internally.
 Lesions in the stomach or gastrointestinal tract can cause
severe abdominal pain and bleeding; we remove these
surgically.
 Maffucci's syndrome leads to VMs and bony growths called
enchondromas. These can result in serious deformities that
may worsen with age and become malignant.

54. Targeted agents
 The mammalian target of rapamycin (mTOR)
inhibitor Sirolimus has emerged as a promising
targeted therapy for VMs.
 Sirolimus decreased the proliferation of endothelial
cells and inhibited the excessive activation of AKT,
which is responsible for smooth muscle deficiency .
 D-dimers and fibrinogen levels improved, and MRI
images showed significant decrease in volume after
12 months of treatment

55. Differential Diagnosis
Two newly introduced conditions involve the upper
extremities are
 Fibro-adipose vascular anomaly (FAVA)
 CLOVES:
Congenital Lipomatous Overgrowth
Vascular malformations
Epidermal nevi
Skeletal anomalies

56. Take home Messages
 Venous malformations (VMs) are slow-flow
vascular malformations.
 They consist of dilated and dysfunctional veins
that are deficient in smooth muscle cells.
 VMs are mostly sporadically, rarely inherited in
autosomal dominant fashion.
 Blood stagnation and localized intravascular
coagulation, are reflected by elevated blood levels
of D-dimer (>0.5 mcg/mL) and normal or low
fibrinogen.

57. Take home messages
 VMs typically manifest as a light to dark blue skin
discoloration overlying a soft, compressible,
subcutaneous mass.
 Diagnosis of VM is clinical.
 USG and MRI can confirm the diagnosis and assess
the VM extent.

58. Take home messages
 The management requires an interdisciplinary team
including
◦ Dermatologist
◦ Interventional radiologist,
◦ Haematologist,
◦ Plastic or vascular surgeon
 Treatment is individualized - include supportive therapies
◦ Compression,
◦ Medical management of coagulopathy, and pain control
◦ Sclerotherapy
◦ Surgery or combination.
 Sirolimus (mTOR inhibitor) - promising treatment for complex
VMs.

59. Thank you very much
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