phlebology 2014 christou

8/20/2019 Phlebology 2014 Christou

http://slidepdf.com/reader/full/phlebology-2014-christou 1/5

Original article

Non-involuting congenital haemangioma of theeyelid: successful treatment with fluroscopicultrasound guided sclerotherapy and

surgical excision

E Christou* and K Parsi†

*Department of Dermatology, Sydney Children’s Hospital, Randwick, New South Wales, Australia; †Department of Dermatology, St Vincent’s Hospital, Sydney, New South Wales, Australia

AbstractWe present a case of non-involuting congenital haemangioma (NICH) of the right eyelidwhich was present at birth as a purpuric macule but increased in size to cause significantobstruction of vision. At four years of age the lesion was treated with fluroscopicultrasound-guided sclerotherapy using 0.5% sodium tetradecyl suphate foam andsurgically debulked 16 days later. Histopathology was negative for glucose transporter-1stain confirming the diagnosis. The residual segments were subsequently treated in threefurther sessions of sclerotherapy in the ensuing three years. This treatment approachresulted in a good cosmetic and functional outcome with no associated complications. Toour knowledge, this is the first published case of a histologically confirmed NICH treatedprimarily with sclerotherapy.

IntroductionNon-involuting congenital haemangiomas (NICH)are rare vascular tumours that are present at birthand classically remain stable over time showingno signs of regression or spontaneous involution,1

However they have been described to worsen.2

Treatment options for lesions involving sites of crucial cosmetic and functional significance have been limited to long-term observation and conser-vative management. Given the vascular natureof these tumours and the significant risk of bleed-ing, surgical excision of such lesions as a single

treatment modality is often not a safe or viableapproach. Furthermore, undefined clinical bordersand anatomical extension would render completeexcision of such vascular tumours impossible.Interventional treatment options are limited as

embolization with microparticles may result inend-organ ischaemia, while embolization withagents such as absolute alcohol may result in sig-nificant tissue necrosis, nerve damage and thrombotic complications. Here, we present a case of anorbital NICH that was successfully treated withfluoroscopic ultrasound guided sclerotherapy(FUGS) and surgical excision.

Case report

A 19-month-old woman presented with a vascular birthmark on her right upper eyelid. The lesionwas first noticed at birth as a dusky red macule(Figure 1a) but subsequently enlarged (Figures 1band c). Valsalva manoeuvres such as crying madethe lesion appear more prominent. There wasno history of haemorrhage, thrombosis or ulcera-tion. Magnetic resonance imaging (MRI) showed alobulated soft tissue mass measuring 1 × 3 cmdemonstrating intense contrast enhancement.A computerized tomography (CT) scan performedat 19 months of age showed an intraorbital

Correspondence: E Christou MBBS (hons), BHsc,

Dermatology, Sydney Children’s Hospital, Randwick,

New South Wales 2031, Australia.

Email: [email protected]

Accepted 23 October 2012

DOI: 10.1258/phleb.2012.012116:4–8.Phlebology 2014;29

by guest on February 29, 2016phl.sagepub.comDownloaded from

mailto:[email protected]

http://phl.sagepub.com/




mailto:[email protected]



extension to the anterolateral aspect of the rightglobe approaching the lacrimal gland. Venogramdemonstrated the lesion to be limited to the uppereyelid with drainage mostly involving the lateralsuperficial temporal vein rather than an intraorbitalpathway. Duplex ultrasound showed a soft tissuemass within the right lateral upper eyelid(Figure 2a) demonstrating pulsatile flow. The masshad typical B-mode features of a soft tissue tumour

as against an arterio-venous (AV) malformation.Furthermore, there were no feeding arteries ordraining veins typical of an AVM. These findingswere consistent with the diagnosis of a vasculartumour (Figure 2). Preoperative thrombophiliascreening showed no mutations in factor V or pro-thrombin gene and no coagulation factor inhibitordeficiencies. A homozygous status for methylene-tetrahydrofolate reductase (MTHFR) polymorph-ism was detected, however the fasting serumhomocysteine level was within the normal range(3 m mol/L, 5– 12).

The patient was regularly observed for thenext two years and serial measurements andphotographs were taken. At four years of age, thelesion had grown in bulk to interfere with theability of the patient to open the right eye. Afterextensive counselling with parents that included acomprehensive discussion of all possible outcomesand potential for serious complications such as blindness and stroke, a decision was made to

proceed with treatment to preserve eyesightfunction (Figure 3).All procedures were performed under general

anaesthesia and following aseptic protocols. Inthe first treatment, the lesion was cannulatedunder ultrasound guidance and contrast agent(Iohexol obtained as OMNIPAQUE, GE Healthcare,Rydalmere, NSW, Australia) was introduced forfluoroscopic visualization (Figure 4). Sodium tetra-decyl sulphate (STS, obtained as FIBRO-VEIN 3%,Australian Medical & Scientific, Artarmon, NSW,Australia) at 0.5% was used as the sclerosing

Figure 1 Gradual enlargement of the lesion of right orbit before surgery and at follow up. (a) Day 1,(b) 17 months, (c) four years, (d) preoperative and (e) 11 months postoperative

5:4–8Phlebology 2014;29

E Christou and K Parsi. Non-involuting congenital haemangioma of the eyelid Original article








agent. The liquid sclerosant was converted into amicrofoam with a liquid:gas ratio of 1 + 4 usingroom air as the foaming gas, a three-way tap andtwo 0.5 micron filters. A total of 0.5 mL of micro-foam was injected under ultrasound guidance andmonitored by fluoroscopy until the foam wasobserved to approach the draining vessels. Fourmilligrams of intravenous dexamethasone wasadministered to minimize postoperative orbitaloedema. Compression dressings were applied andmaintained for 24 hours postoperatively. Prophylac-

tic anticoagulation was provided as the procedurelocation was deemed high risk for poor outcome if extended thrombosis formed at the site. Enoxaparin20 mg was administered subcutaneously on the dayof the procedure and continued until clinical andultrasound follow-up two days later. Ultrasoundshowed no vascular flow within the lesion whilethe surrounding vasculature showed normal flow.A surgical debulking operation was performed 16days after the procedure to benefit from improvedhaemostasis obtained following embolization. Thisinvolved a superior eyelid mid-crease excision and

Figure 2 (a) B-mode ultrasound image of the right orbit showing a soft tissue mass within the right lateral upper eyelid.(b) Doppler ultrasound demonstrating high flow within the soft tissue mass

Figure 3 Fluoroscopy following the injection of the contrast agent showing the extent and drainage of the lesion

Figure 4 (a) Irregularly outlined dilated vascular spaces lined by flattened endothelium (H&E 100). (b) negative glucosetransporter-1 stain

6 :4–8Phlebology 2014;29

Original article E Christou and K Parsi. Non-involuting congenital haemangioma of the eyelid








removal of tissue and overlying residual skin. Thelevator muscle had been laterally displaced by thelesion but was able to be excised from thesurrounding postseptal structures. Histopatho-logy showed a lobular structure consistent with ahaemangioma (Figure 4a). Glucose transporter-1

(GLUT-1) staining was negative on the lesionalendothelial cells confirming the diagnosis of NICH(Figure 4b).

Follow-up 10 months after the procedure revealeda well-healed upper eyelid scar within tarsal fold,residual 2 – 3 mm of ptosis of eyelid and normalvisual examination (Figure 1e). However, ultra-sound and MRI imaging showed two small residuallesions within the lateral upper eyelid. The residualsegments were further treated with 0.5% STSmicrofoam on three occasions in the followingthree years. These procedures included bothultrasound-guided and direct vision sclerotherapy but did not require fluoroscopic monitoring. Clinicaland ultrasound follow-up 12 months after the lastprocedure showed no evidence of tumoural recur-rence although a cutaneous telangiectatic com-ponent remained visible.

Discussion

We report the use of FUGS followed by surgicalexcision as a therapeutic option in the managementof a histologically-confirmed NICH. As against

infantile haemangiomas, these congenital tumoursare present at birth, fail to spontaneously involuteand do not stain with GLUT-1.3 Although thelesion demonstrated high flow, we excluded anarteriovenous malformation (AVM) based on sono-graphic and MRI characteristics. MRI with T1and T2 weighted imaging and angiography/venography is the investigation of choice in thediagnosis of vascular anomalies. AVM usually pre-sents with dilated fast flow vascular channelswhile slow flow vascular malformations appear bright on T2 weighted or fluid sensitive sequences.

Here, AVM was excluded by multiple modalities(ultrasound, MRI and venography) based on theabsence of dilated vascular channels, feedingarteries or draining veins and the presence of alobulated soft tissue mass typical of haemangiomas.AVM is an important differential diagnosis forNICH and similarly poses a challenging manage-ment option.

This rare case of NICH at a site of functionaland cosmetic significance highlights the possibilityof successful treatment planned and delivered ina staged fashion. Options for management of

NICH are limited. Surgical approach alone may becomplicated by incomplete excision as wellas intraoperative and postoperative bleeding.1,4

Other described treatment options include endo-vascular particulate embolization, tissue cauteriza-tion, vascular laser therapy and cryotherapy to

control recurrent episodes of oral bleeding.5

Sclerotherapy has been commonly used followingincomplete surgery to treat venous and lymphaticmalformations. By contrast, vascular tumours areusually not treated with embolization or sclerother-apy due to risk of tissue necrosis, nerve injury,intra-arterial injection, venous or arterial thrombo-sis, occlusion of distant vessels due to embolizationand end-organ necrosis. A literature search revealeda small number of patients with haemangiomaswho were treated with the combination of sclero-therapy followed by surgery,6 and a case of NICHtreated with embolization with polyvinyl alcoholand placement of a platinum minicoil prior to surgi-cal excision.7 The target lesion in this case wassituated at a high risk anatomical site.

Venous drainage of the orbit occurs through ananastomozing network of internal and externalsystems and involves the superior and inferiorophthalmic veins. The major drainage of thelateral eyelids is via the superficial temporal veinwhile the angular and facial veins drain themedial aspect. The angular vein communicateswith the superior and inferior ophthalmic veinswhich in turn drain into the cavernous sinus.

Hence, cavernous sinus thrombosis and strokewould be serious potential complications of sclero-therapy at this site. Other significant potential com-plications would include retinal vein thrombosisand blindness as well as ischaemic or transientneurological complications due to gas embolism.A recent case report highlights such potential com-plications when percutaneous sclerotherapy treat-ment to a glabellar haemangioma led to a defectof no light perception following retinal artery andposterior cillary artery occlusion.8

To reduce the risk of thrombotic complications,

we screened and excluded significant thrombo-philic abnormalities in the preoperative work-up.The homozygous mutation in MTHFR was notassociated with an increased serum level of homo-cysteine. Furthermore, venography demonstrateda primarily lateral rather than intra-orbital venousdrainage.

To further reduce the risk of thrombosis, wechose a 0.5% concentration of STS in the foamformat. This anionic surfactant is commonly usedin the treatment of varicose veins but is also usedfor treatment of venous malformations.4,9,10 Lower

7:4–8Phlebology 2014;29

E Christou and K Parsi. Non-involuting congenital haemangioma of the eyelid Original article








concentrations of detergent sclerosants (,0.1%) areshown to induce platelet activation with a net pro-thrombotic effect whereas higher concentrationsdemonstrate antithrombotic and antifibrinolyticproperties due to lysis of platelets, destruction of clotting factors and plasminogen.11 The foam

format is preferred to the liquid as foam displacesthe intra-vascular blood, reducing the de-activationof sclerosants due to protein-binding and increasingthe potency of these agents.12

In addition to these preventive measures,prophylactic anticoagulation was provided for48 hours after discharge and full ambulation wasencouraged immediately after each procedure. Noanticoagulation was provided in the subsequenttreatments. There is currently no evidence-basedconsensus with respect to indications and durationof prophylactic anticoagulation in the treatment of vascular anomalies. To avoid the risk of phlebitisin the postoperative period, running, bike ridingand excessive exertion was discouraged for thefirst two postoperative weeks.

Our patient required three treatments over threeyears. Given the vascular nature of these lesions,complete occlusion or removal is not possible in asingle procedure and regular clinical and ultra-sound follow-up is required to assess recurrence.The patient will be followed with annual clinicaland ultrasound review.

ConclusionIn summary, we report the use of fluoroscopicultrasound-guided sclerotherapy followed by sur-gical excision as a therapeutic option in the manage-ment of NICH. This rare case highlights thetechnical difficulties in treating such vascularlesions in high-risk anatomical sites, and describesa practical method to treat such lesions. Thechoice of the sclerozing or embolizing agent, its con-centration and composition and other treatmentvariables may influence the outcome and requirecareful consideration.13,14 Such high-risk procedures

should only be undertaken with full knowledge andadequate experience in the selected interventionaltechnique.

References

1 Matulich J, Wood G, Sugo E. Case of non-involuting con-genital haemangioma. Aus J Dermatol 2005;46:165–8

2 Enjolras O, Mulliken JB, Boon LM, et al. Non-involutingcongenital hemangioma: a rare cutaneous vascularanomaly. Plast Reconstr Surg 2001;107:1647

3 North PE, Waner M, James CA, Mizeracki A, Frieden IJ,Mihm MC Jr. Congenital nonprogressive hemangioma:a distinct clinicopathologic entity unlike infantilehemangioma. Arch Dermatol 2001;137:1607–20

4 Jin YB, Lin XX, Ma G, et al. Non-involuting congenitalhemangioma: a study for diagnosis and treatment.[Chinese] Zhonghua Zheng Xing Wai Ke Za Zhi 2009;25:189–93

5 Selehian S, Gemmete JJ, Kasten S, Edwards SP. Novelapplication of percutaneous cryotherapy for the treat-ment of recurrent oral bleeding from a noninvolutingcongenital hemangioma involving the right buccalspace and maxillary tuberosity. Cardiovasc InterventRadiol 2011;34(Suppl. 2):S277– 81

6 O’Donovan JC, Donaldson JS, Morello FP, Pensler JM,

Vogelzang RL, Bauer B. Symptomatic haemangiomasand venous malformations in infants, children andyoung adults: treatment with percutaneous injectionof sodium tetradecyl sulfate. Am J Roentgenol1997;169:723–9

7 Ooi KG, Wenderoth JD, Francis IC, Wilcsek GA. Selectiveembolization and resection of a large noninvoluting con-genital hemangioma of the lower eyelid. Ophthal PlastReconstr Surg 2009;25:111–4

8 Matso T, Fujiwara H, Gobara H, Mimura H, Kanazawa S.Central retinal and posterior ciliary artery occlusionafter intralesional injection of sclerosant to glabellar sub-cutaneous hemangioma. Cardiovasc Intervent Radiol2009;32:341–6

9 Parsi K, Kossard S. Multiple hereditary glomangiomas:

successful treatment with sclerotherapy. Aust J Dermatol2002;43:43

10 Lee BB, Bergan J, Gloviczki P, et al. Diagnosis and treat-ment of venous malformations: consensus documentof the International Union of Phlebology (IUP)-2009.Int Angiol 2009;28:434–51

11 Parsi K, Exner T, Low J, Ma DDF, Joseph JE. In vitroeffects of detergent sclerosants on clot formation andfibrinolysis. Eur J Vasc Endovasc Surg 2010;41:267–77

12 Parsi K, Exner T, Connor DE, Herbert A, Ma DDF, Joseph JE. The Lytic effects of detergent sclerosants onerythrocytes, platelets, endothelial cells and microparti-cles are attenuated by albumin and other plasma com-ponents In vitro. Eur J Vasc Endovasc Surg 2008;36:216–23

13 Cavezzi A, Parsi K. Complications of foam sclero-therapy. Phlebology 2012;27(Suppl. 1):46– 5114 Parsi K. Paradoxical embolism, stroke and sclerotherapy.

Phlebology 2012;27(Suppl. 4):147– 67

8 :4–8Phlebology 2014;29

Original article E Christou and K Parsi. Non-involuting congenital haemangioma of the eyelid






phlebology 2014 christou

Documents