Two Cases of Periocular Cutaneous Angiosarcoma · eyelid with Tenzel flap and cross-eyelid flap from lower eyelid and nasal reconstruction with axial flap. FIG. 1. A, Irregular, poorly

Dow

nloadedfrom

https://journals.lww.com

/op-rsby

BhDMf5ePH

Kav1zEoum1tQ

fN4a+kJLhEZgbsIH

o4XMi0hC

ywCX1AW

nYQp/IlQ

rHD3PO

oLCqW

plEcXgigPxl+hzaz2jeQzVW

MuVYvAlO

4WGcp1IqpYj8K7ag==

on05/15/2018

Downloadedfromhttps://journals.lww.com/op-rsbyBhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3POoLCqWplEcXgigPxl+hzaz2jeQzVWMuVYvAlO4WGcp1IqpYj8K7ag==on05/15/2018

BRIEF REPORTS

Two Cases of PeriocularCutaneous AngiosarcomaDamrong Wiwatwongwana, M.D.*, Valerie A. White, M.D.*†,and Peter J. Dolman, M.D., F.R.C.S.C.*

Abstract: Angiosarcoma is a rare malignancy with only 8previous reports of eyelid involvement. The authors report2 further cases, one as a primary lesion and the other as arecurrence from a contiguous area. In both cases, thelesions appeared relatively inconspicuous, and their extentduring micrographic excision was considerably larger thananticipated. Although wide surgical margins were obtainedand adjuvant radiotherapy and chemotherapy was under-taken, one patient had died from distant metastasis, whilethe second had distant cutaneous recurrences within 1 year.

A ngiosarcomas (AS) are uncommon, highly malignant, vas-cular endothelial tumors that typically arise in the skin or

subcutaneous tissue.1 The cutaneous variant may be solitary ormultifocal and most commonly involves the scalp and upperface in elderly whites.2,3 They may present as flat or nodularlesions, may have surrounding edema, and their color mayrange from bluish-red to skin tones. They are frequently mis-diagnosed as benign lesions, and their extent is often underes-timated. Chronic lymphedema and previous radiotherapy arepredisposing factors for cutaneous AS, but only 10% of tumorshave this association. No underlying genetic abnormality hasbeen reported for cutaneous AS. AS involving the eyelidis rare, and we could identify only 8 cases in the literature.2,4,5

We report 2 additional cases of AS involving the eyelid.

CASE 1An 82-year-old white woman presented with a painless,

erythematous, slightly elevated lesion in the left, medial uppereyelid, increasing in size over the previous 6 weeks. Also notedwere small, irregular, poorly defined violaceous papules on theleft nasal bridge and lower eyelid and cheek (Fig. 1A). Anincisional biopsy of the upper eyelid showed oddly shapedvascular channels lined by malignant endothelial cells (Fig. 1B). Asystemic workup found no metastases. She was treated byMohs excision with wide free margins involving all the multi-focal areas with a defect much larger than anticipated (Fig. 1C).Reconstruction with a cheek rotation flap, midline foreheadflap, and skin grafts was followed 6 weeks later by focalradiotherapy (Fig. 1D). Although no apparent recurrence de-veloped in the face, within 1 year, she developed a right pleuraleffusion from a pulmonary metastasis, followed by liver me-tastases. She died 6 months later.

CASE 2A 71-year-old white male was referred with a viola-

ceous, painless maculo-papular lesion at the left medial uppereyelid (Fig. 2A). He had been diagnosed with angiosarcoma of

the nasal bridge 4 years previously and treated with completeexcision of the nose, reconstruction, and adjuvant radiotherapy.An incisional biopsy confirmed recurrent angiosarcoma (Fig. 2B).A systemic workup was negative, and the eyelid lesion wastreated with wide excision using frozen section control (Fig. 2C),reconstruction with a modified Tenzel flap, and adjuvant radio-

Departments of *Ophthalmology and Visual Sciences and †Pathologyand Laboratory Medicine, University of British Columbia, Vancouver,British Columbia, Canada

Accepted for publication August 5, 2009.Address correspondence and reprint requests to Peter J. Dolman, M.D.,

Eye Care Centre, Section I, 2550 Willow Street, Vancouver, BC, CanadaV5Z 3N9. E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181c0e180

FIG. 2. A, Violaceous, painless maculo-papular lesion in theleft medial upper eyelid. This patient had a previous nasal exci-sion with reconstruction and radiotherapy for diagnosed angio-sarcoma 4 years earlier. B, Photomicrograph of eyelid skin(with epidermis on left) showing infiltration of dermis by recurrentpoorly differentiated angiosarcoma showing a predominantlysolid pattern with numerous vacuoles (hematoxylin-eosin).C, Excision with wide margins of left medial eyelid followingbiopsy-proven angiosarcoma. D, Reconstruction of left medialeyelid with Tenzel flap and cross-eyelid flap from lower eyelidand nasal reconstruction with axial flap.

FIG. 1. A, Irregular, poorly defined violaceous papules on theleft, medial upper eyelid and on the left nasal bridge and lowereyelid and cheek. B, Photomicrograph of eyelid skin showinginfiltration of the dermis by cutaneous angiosarcoma com-posed of cords of atypical epithelioid cells, some of which formsmall lumens. The lesion stains for factor VIII-related antigenand also stained focally for CD31. (Antifactor VIII-related anti-gen, hematoxylin counterstain). C, Mohs excision with widefree-margins shows a defect larger than clinically anticipated.D, Reconstruction with a variety of facial flaps.

Ophthal Plast Reconstr Surg, Vol. 26, No. 5, 2010 365

therapy. Over the following year, further nasal reconstructionwas performed (Fig. 2D). One year later, recurrent red patchesdeveloped at the tip of the new nose and in his left cheek, andbiopsy confirmed recurrent AS. This responded to 6 courses ofdoxorubicin and additional radiotherapy 3 months later. Fourmonths later, and 3 years after his initial eyelid biopsy, additionalred lesions erupted at the right cheek and left nasal bridge.

These cases highlight how AS may rarely involve theeyelid either as an initial or recurrent presentation. In bothpatients, the lesion appeared as a poorly defined violet maculo-papule, and its extent clinically was poorly defined with typicalmultifocal involvement. Pathologically, the lesions may bedifficult to diagnose as they are rare and may assume a varietyof forms ranging from near-normal dermal blood vessels withminimally atypia to a solid pattern as in Case 2. Once thediagnosis has been suspected, it may be confirmed by stainingfor vascular markers such as CD31, CD 34, factor VIII-relatedantigen, and with Ulex euopaeus-1 lectin. AS has a highpropensity for both local and distant metastases to lymph nodesand lungs with a 5-year actuarial survival rate reported from12% to 35%.1,3,6 Some reports suggest that a lesion less than 5cm carries a favorable prognosis.6 The current recommendedtherapy is controlled surgical excision with wide margins andradiotherapy, with chemotherapy using doxorubicin added forsystemic involvement. While our cases appeared smaller than 5cm, they were interpreted to have free margins on surgicalexcision and were treated with adjunctive radiotherapy (andadditional chemotherapy in the second case); one patient diedof metastasis within 2 years, and the other has had contiguousand multifocal recurrences over the subsequent 3 years.

REFERENCES1. Mark RJ, Poen JC, Tran LM, et al. Angiosarcoma. A report of 67

patients and a review of the literature. Cancer 1996;77:2400–6.2. Gunduz K, Shields JA, Shields CL. Cutaneous angiosarcoma with

eyelid involvement. Am J Ophthalmol 1998;125:870–1.3. Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and

scalp, prognosis and treatment. Cancer 1987;59:1046–57.4. Bray LC, Sullivan TJ, Whitehead K. Angiosarcoma of the eyelid.

Aust N Z J Ophthalmol 1995;23:69–72.5. Conway RM, Hammer T, Viestenz A, et al. Cutaneous angiosar-

coma of the eyelids. Br J Ophthalmol 2003;87:514–5.6. Maddox JC, Evans HL. Angiosarcoma of skin and soft tissue: a

study of forty-four cases. Cancer 1981;48:1907–21.

Neoadjuvant Chemotherapy inRecurrent Sebaceous Carcinoma ofEyelid With Orbital Invasion andRegional LymphadenopathyOmega Priyadarshini, M.S.*,Ghanashyam Biswas, M.D., D.M.†,Sutapa Biswas, M.D.‡, Rajesh Padhi, M.S.§,and Suryasnata Rath, M.S., F.R.C.S. (Glasg.)*

Abstract: A 35-year-old man presented with a recurrenttemporal conjunctival mass (25 � 12 mm) involving aboutsix-clock hours of the limbus in the left eye. The mass en-croached onto the temporal half of cornea and showed surfacekeratin, large intrinsic and feeder vessels. It infiltrated thedeep corneal stroma. There were no cells in the anteriorchamber. Ultrasound biomicroscopy confirmed infiltration ofdeep corneal stroma without intraocular invasion. Surgeryinvolved excision of the conjunctival component with 4-mm

margin, lamellar sclerectomy and a penetrating sclerokerato-plasty with 3 mm of healthy corneal margin. Cryotherapy(double-freeze-thaw) was done to the conjunctival margins.Histopathology showed it to be invasive sebaceous cell carci-noma. A thin layer of deep corneal stroma and all conjunctivalmargins were uninvolved. At thirty-six weeks after treatmentthe left eye recorded a visual acuity of finger counting at 1meter distance and no recurrence.

C urrent recommendations for eyelid sebaceous carcinomainclude Mohs’ micrographic surgery or excision with

frozen section control of margins and conjunctival map biopsy.1

Orbital invasion of sebaceous carcinoma occurs in 6% to 45%of cases and is associated with poor prognosis.1 Patients withorbital invasion are usually treated with orbital exenteration.1

We report a case of recurrent sebaceous carcinoma with orbitalinvasion, which showed a dramatic response to a single cycle ofneoadjuvant chemotherapy.

CASE REPORTA 64-year-old male presented with a recurrent fungat-

ing mass in the right lower eyelid. Histopathologic exami-nation of the slide from a prior surgical excision showed itto be sebaceous carcinoma with involved margins (Fig. 1A).Visual acuity could not be assessed in the right eye and was20/60 in the left eye. On examination, there was a largefungating mass involving the right lower eyelid. The masswas nontender and firm in consistency, with surface ulcer-ation and eyelash loss (Fig. 1B). The mass was palpable inthe superomedial orbit. Axial sections of CT of the orbitshowed a large homogenous lesion in the medial orbit withmild contrast enhancement. Indentation and lateral displace-ment of the eyeball were noted (Fig. 1C). Coronal sectionsshowed the mass to be inseparable from the eyeball. Systemicexamination showed a palpable enlarged submandibular node(AJCC TNM classification: T4N1M0). The anterior segmentand fundus details in the left eye were unremarkable except forsenile cataract. Fine needle aspiration from the submandibularnode confirmed tumor infiltration. The patient received onecycle of neoadjuvant chemotherapy with cisplatin 20 mg/m2/day over 2 hours and 5-fluorouracil 500 mg/m2/day over 6hours for 5 consecutive days.

He developed gastrointestinal toxicity in the form ofdiarrhea, oral mucositis, and electrolyte imbalance. Hemissed several subsequent visits. A follow-up visit 7 monthsafter neoadjuvant chemotherapy showed no residual tumoron clinical examination (Fig. 2B). Visual acuity was 20/70 inthe right eye and 20/25 in the left eye. The submandibularlymph node was enlarged. CT of the orbit showed a nonen-hancing irregular haze in the medial orbit (Fig. 2C). Thepatient was advised to have radiotherapy to the orbit andneck nodes. The patient did not receive radiation therapy andmissed several subsequent visits. Nine months later, he

*Ophthalmic Plastic surgery, Orbit and Ocular Oncology service, L.V.Prasad Eye Institute, Patia; †Medical Oncology Service, Sparsh Hospitalsand Critical Care, Saheednagar; ‡Pathology Service, Sparsh Hospitals andCritical Care, Saheednagar; and §Otorhinolaryngology Service, SparshHospitals and Critical Care, Saheednagar, Bhubaneswar, Orissa, India

Accepted for publication September 25, 2009.Supported by Hyderabad Eye Research Foundation, Hyderabad, Andhra

Pradesh, India.Address correspondence and reprint requests to Suryasnata Rath,

M.S., F.R.C.S. (Glasg.), Ophthalmic Plastic, Orbit and Ocular Oncologyservice, L.V. Prasad Eye Institute, Bhubaneswar, Orissa, India. E-mail:[email protected]

DOI: 10.1097/IOP.0b013e3181c32515

Brief Reports Ophthal Plast Reconstr Surg, Vol. 26, No. 5, 2010

366 © 2010 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

underwent cataract surgery in the left eye. A follow-up visit18 months after neoadjuvant chemotherapy showed no or-bital recurrence. A thorough systemic evaluation, includingan x-ray of the chest and ultrasound of the abdomen andpelvis, was normal. He underwent supra-omohyoid lymphnode dissection. Histopathologic sections showed only thesubmandibular node to have metastatic sebaceous carcinoma(Fig. 2A). Thirty months after neoadjuvant chemotherapy,the patient was alive and well with no local-regional orsystemic recurrence (AJCC TNM classification: T0N0M0).

COMMENTThe promising role of neoadjuvant chemotherapy in

down-staging solid tumors of the breast, head, and neck, andof esophageal-gastric tumors is extensively reported in theliterature.2– 4 A case of sebaceous carcinoma with favorableresponse to neoadjuvant chemotherapy was reported byMurthy et al.5 In their case, a 55-year-old woman received 3cycles of neoadjuvant chemotherapy followed by radiation,additional chemotherapy, and finally orbital exenteration fortumor control. In contrast, our case showed a dramatic

FIG. 1. A, Histopathology from the excision performed priorto presentation. This shows cells with finely vacuolated, foamycytoplasm, and nuclear atypia (hematoxylin-eosin) suggestiveof sebaceous carcinoma. B, Clinical photograph of the 64-year-old man who presented with a large fungating mass. Thisseemed to arise from the lower eyelid and involved the medialorbit. The mass was nontender and firm in consistency, withsurface ulceration and eyelash loss. C, Axial sections of CT ofthe orbit shows the large mass in the medial orbit. The masswas indenting and displacing the globe laterally.

FIG. 2. A, Histopathology from the lymph node dissection onhigher magnification shows vaculated cytoplasm of the tumorcells suggestive of sebaceous carcinoma infiltration of the lymphnode. B, Clinical photograph of the man after neoadjuvant che-motherapy shows no residual mass. Cicatrization of the lower eye-lid is seen. The anterior segment in the right eye showed thepseudophakic status with a visual acuity of 20/70. C, Axial sectionof CT of the orbit obtained after neoadjuvant chemotherapyshows an irregular nonenhancing haze in the medial orbit.

Ophthal Plast Reconstr Surg, Vol. 26, No. 5, 2010 Brief Reports

© 2010 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. 367

response to a single cycle of neoadjuvant chemotherapy.Effective tumor control was achieved without further radi-ation or exenteration. In addition, useful vision was restoredafter treatment. Response to neoadjuvant chemotherapy al-beit in metastatic sebaceous carcinoma has been reported byEsmaeli et al.6 We believe that neoadjuvant chemotherapymay have a promising role to play in down-staging seba-ceous carcinoma invading the orbit. However, optimal dos-age and duration of treatment should be further studiedthrough experience shared in larger number of patients.

REFERENCES1. Cook BE Jr, Bartley GB. Treatment options and future prospects for

the management of eyelid malignancies: an evidence-based update.Ophthalmology 2001;108:2088–98; quiz 2099–100, 2121.

2. Teatini G, Meloni F, Bisail M, et al. Neoadjuvant chemotherapywith cisplatinum and 5-fluorouracil in advanced head and neckcancer. J Chemother 1990;2:394–6.

3. Ross PJ, Rao S, Cunningham D. Chemotherapy of oesophago-gastric cancer. Pathol Oncol Res 1998;4:87–95.

4. Faivre C, Rougier P, Ducreux M, et al. [5-fluorouracile and cisplati-num combination chemotherapy for metastatic squamous-cell analcancer.] Bull Cancer 1999;86:861–5.

5. Murthy R, Honavar SG, Burman S, et al. Neoadjuvant chemother-apy in the management of sebaceous gland carcinoma of the eyelidwith regional lymph node metastasis. Ophthal Plast Reconstr Surg2005;21:307–9.

6. Husain A, Blumenschein G, Esmaeli B. Treatment and outcomes formetastatic sebaceous cell carcinoma of the eyelid. Int J Dermatol2008;47:276–9.

Kindler Syndrome Causing SevereCicatricial EctropionGary J. Lelli, Jr., M.D.

Abstract: A 32-year-old female with Kindler syndromepresented with a 5-year history of lower eyelid malposition,corneal exposure, and recurrent erosions. Severe anteriorlamellar cicatricial changes were noted bilaterally, withbilateral lower eyelid ectropion and retraction. Tarsal ever-sion was noted on the left lower eyelid. The patient hadrepeatedly failed conservative treatments for keratopathyand was treated surgically, with resolution of corneal dis-ease and improved lower eyelid position. A review of Kin-dler syndrome is provided, geared toward the oculoplasticsurgeon who may participate in the care of these patients.

K indler syndrome is a rare autosomal recessive disorder caus-ing acral blisters in infancy and childhood, followed by

photosensitivity, diffuse cutaneous atrophy, poikiloderma, andacral keratoses.1 The disease has been noted to cause keratopathyand corneal ectasia, which in one case was severe enough torequire enucleation.2 Approximately 100 cases of the disease havebeen reported, but to the best of our knowledge, none in theoculoplastic literature describing the potential cicatricial eyelid

changes and subsequent treatment options. Herein, we report acase of Kindler syndrome with cicatricial ectropion requiringsurgical repair for treatment of exposure keratopathy.

CASE PRESENTATIONA 32-year-old female with Kindler syndrome, diagnosed

secondary to a history of acral blistering, poikiloderma, andphotosensitivity, and laboratory testing consistent with a mu-tation in the Kindlin-1 protein presented to the oculoplasticservice with recurrent corneal erosions, left more than right,and yellow discharge from each eye. These symptoms werepresent for 5 years and had been treated by numerous ophthal-mologists with a combination of artificial tears, ophthalmicsteroids, cyclosporine eye drops, and gels. She had been man-ually stretching her eyelids for over 1 year, without improve-ment, and had not undergone prior surgical intervention to hereyelids. Her ophthalmic examination was significant for 20/15vision in each eye with anterior lamellar cicatrization bilater-ally causing lower eyelid retraction and ectropion (Fig. 1). Theleft lower eyelid demonstrated tarsal eversion. Moderate (1�,modified Oxford scale) superficial punctate keratopathy waspresent bilaterally.

The patient was treated with staged (left side, followedby right side) surgical repair as follows. A lateral canthotomyand subciliary incision was made across the length of the lowereyelid. A skin-muscle flap was elevated inferiorly to the malareminence, releasing severe anterior lamellar cicatricial disease.After elevation of this flap, the eyelid was noted to be in goodapposition to the globe but with significant horizontal tarsoli-gamentous laxity. An inferior cantholysis was performed, atarsal strip fashioned and secured to the inner aspect of thelateral orbital rim with a nonabsorbable horizontal mattresssuture, giving the lower eyelid appropriate height, contour, andtension. The remaining skin defect was measured (11 mmvertically � 32 mm horizontally on the left side, 7 mm

Division of Oculoplastic Surgery, Department of Ophthalmology, WeillCornell Medical College, New York, New York, U.S.A.

Accepted for publication September 27, 2009.The author has no proprietary interest to disclose.Supported by Research to Prevent Blindness, New York, NY, U.S.A.Address correspondence and reprint requests to Gary J. Lelli, Jr., M.D.,

Division of Oculoplastic Surgery, Department of Ophthalmology, WeillCornell Medical College, 1305 York Avenue, New York, NY 10021, U.S.A.E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181c4dff6

FIG. 1. Preoperative photograph demonstrating bilateral (leftgreater than right) cicatricial ectropion and lower eyelid retrac-tion with lateral canthal dystopia in association with Kindlersyndrome. Tarsal ectropion is present on the left side. Poikilo-derma is also evident throughout the facial skin.



vertically � 32 mm horizontally on the right side), and aslightly oversized full-thickness skin graft was harvested fromthe retroauricular space and sutured in the lower eyelid anteriorlamellar defect. A bolster was placed over the graft for poste-rior pressure, and the lower eyelid was sewn on stretch to theupper eyelid and brow with multiple double-armed 4-0 silkmattress sutures. The stretch sutures were left in place for 5days postoperatively. The right lower eyelid was repaired inidentical fashion 1 month after the left-sided repair.

Postoperatively, the patient demonstrated improved eye-lid position with apposition to the globe and resolution ofkeratopathy and recurrent erosions (Fig. 2).

DISCUSSIONKindler syndrome was first reported by Kindler3 in 1954,

and since then, approximately 100 cases of this rare autosomalrecessive disorder have been published. The majority of thesereports are found in dermatological literature and do not com-ment extensively on the potential complications of the diseaseon the periocular tissues.

The largest series of patients with Kindler syndrome wasreported by Penagros et al.,4 describing 26 cases in 10 familiesfrom the Bocas del Toro province of Panama and alloweddiscovery of the KIND1 gene, located on chromosome 20p12.3,responsible for the disease. This series also demonstrated themost common clinical findings in the disease, namely skinblistering (seen in 100% of patients, usually within the firstdays of life), cutaneous atrophy (100%), poikiloderma (96%),and photosensitivity (92%). Mucosal involvement, frequentlyoral, urethral, or labial, is common but was not reported in theconjunctiva. Ectropion was reported in 10 patients (38%),although eye examinations were reportedly normal, except for1 patient with a posterior subcapsular cataract.

To the best of our knowledge, this is the first case reportto describe the anterior lamellar cicatricial ectropion that canoccur in Kindler syndrome and to discuss its surgical manage-ment. A brief review of the disease is relevant to the oculo-plastic surgeon, who will likely be involved in the care of

Kindler syndrome patients with eyelid malposition. The patientreported herein demonstrated reversal of exposure keratopathywithout additional erosions. The decision to treat this patientwith retroauricular skin grafts, as opposed to upper eyelid skingrafts, was prompted by the patient’s disease process and thepresence of diffuse cicatricial changes throughout her face.Because the disease is associated with lifelong diffuse cutane-ous atrophy and photosensitivity, there is concern that evenafter successful surgical repair, these patients may be at risk forrecurrent lower eyelid retraction and ectropion. Additionally, inmany patients with the disease, cicatricial ectropion maypresent earlier in life, further increasing the potential need foradditional revision surgeries with disease progression. Patientswith Kindler syndrome should be advised to avoid sun expo-sure, as this is known to worsen cutaneous scarring. Addition-ally, depending on the extent and severity of the cicatricialeyelid and midfacial changes, midface lifting may be an ap-propriate correlary to eyelid repair, especially in refractorycases. While conjunctival scarring was not present in thispatient, assessment for posterior lamellar scarring is importantin Kindler syndrome patients, as the disease frequently affectsother mucosal surfaces.

REFERENCES1. Yazdanfar A, Hashemi B. Kindler syndrome: report of three cases in

a family and a brief review. Int J Dermatol 2009;48:145–9.2. Satter EK. A presumptive case of Kindler syndrome with a new

clinical finding. Pediatr Dermatol 2008;25:646–8.3. Kindler T. Congenital poikiloderma with traumatic bulla formation

and progressive cutaneous atrophy. Br J Dermatol 1954;66:104–11.4. Penagros H, Jaen M, Sancho MT, et al. Kindler syndrome in native

Americans from Panama: report of 26 cases. Arch Dermatol 2004;140:939–44.

Eyelid Fistula Caused by aScleral BuckleOmar K. Ozgur, B.S.*, Sara P. Modjtahedi, M.D.*,and Lily Koo Lin, M.D.*

Abstract: Complications of scleral buckle procedures forretinal detachments are rather uncommon yet may result ina broad scope of problems. The authors report a case of achronic eyelid fistula caused by a scleral buckle. The patientwas an 81-year-old woman who presented with a nonheal-ing left upper eyelid wound that was repeatedly misdiag-nosed as a chalazion, but diagnostic workup revealed anextruded scleral buckle to be the cause. The patient wastreated surgically with removal of the scleral buckle andfull-thickness fistula repair and her wound healed well. Ascleral buckle can erode through conjunctiva and the fullthickness of an eyelid, causing an eyelid fistula and neces-sitate removal of the buckle.

*Ophthalmic Plastic and Orbital Surgery, University of California, DavisEye Center, Sacramento, California, U.S.A.

Accepted for publication October 21, 2009.The authors received no financial support nor have any proprietary

interests.Address correspondence and reprint requests to Lily Koo Lin, M.D., 4860

Y Street Ste 2400 Sacramento, CA 95817, U.S.A. E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181c78326

FIG. 2. Postoperative photograph (3 months after left side, 2months after right side) demonstrating improved lower eyelidand lateral canthal position bilaterally.



Scleral buckling material extrusion is a well-recognizedcomplication of scleral buckling surgery. We describe an

unusual presentation of an extruded solid silicone band causinga transpalpebral fistula and appearing clinically as a chroniceyelid ulceration rather than a frank eyelid perforation.

CASE REPORTAn 81-year-old Asian woman was referred by a general

ophthalmologist for an oculoplastics consultation for a 1-yearhistory of a chronic left upper eyelid nonhealing wound (Fig.A) that was unresponsive to antibiotic therapy. She describedher left eye as painful, red, and constantly tearing with clearand thick discharge. A prior eyelid biopsy showed nonspecificinflammatory changes. She had a wound repair with a full-thickness skin graft 8 months prior which healed, then returnedwith redness, tenderness, and pain. Her ocular history wassignificant for placement of a scleral buckle of the left eye fortreatment of retinal detachment 5 years prior. Additionally, shewas pseudophakic and status post blepharoplasty bilaterally.

Her medical history was significant for hypertension anddementia. A review of systems was otherwise negative. Shereported no known drug allergies. She denied smoking andalcohol use. Her family history was significant for retinaldetachments. Her medications included aspirin, memantine,calcium, and a multivitamin.

On examination, her best-corrected visual acuity was20/30 � 2 in the right eye, and no light perception in the left

eye. She had a left afferent pupillary defect. There was restric-tion of upgaze of the left eye, but the patient and her familywere unsure if that was a new problem. The external examina-tion was significant for erythema of a ptotic left upper eyelid,evidence of a skin graft medially, and a focal area of ulcerationwith overlying crust at the superior aspect of the eyelid, justunder the superior orbital rim (Fig. A). On attempted eversionof the left upper eyelid, there was adherence of the superiorconjunctiva to the eyelid, suggesting a possible full-thicknessulcerative defect of the eyelid that was communicating with theglobe. Furthermore, when the crust and ulcerative material wasremoved from the eyelid, the defect appeared to be nearly fullthickness. No madarosis was noted. There was ptosis of the leftupper eyelid with palpebral fissure width measuring 4 mm.Levator function was compromised in the left eyelid with poor(�4 mm) excursion. The slitlamp examination was notable forleft eye chemosis and conjunctival injection and pseudophakia.The funduscopic examination was notable for retinal and opticatrophy of the left eye.

An extruded scleral buckle was high on the differentialas a cause of the eyelid wound, and a CT scan of the orbit wasordered to further evaluate the area because the eyelid could notbe everted or separated from the globe. An aerobic culture swabof the wound was taken and oral amoxicillin and clavulanate875/125 mg orally twice a day was started. At follow-up 1week later, her examination was unchanged. Her CT scandemonstrated the presence of a scleral buckle and was sugges-tive of an adherence to the eyelid (Fig. B). The Gram stain andculture showed no white blood cells and no growth of anyorganisms.

The patient was taken to the operating room for surgicalexploration with removal of the extruded scleral buckle, andfull-thickness resection of the eyelid fistula, debridement, andclosure of the wound. The scleral buckle, a solid siliconeencircling band, was found to have one end extruded throughthe conjunctiva and in communication with the full-thicknesseyelid wound, which incorporated palpebral conjunctiva, leva-tor, orbicularis, and skin. The solid scleral buckle measured2.5 � 0.2 � 0.1 cm. The procedure was performed without anycomplications. Postoperatively she did well, and the area ofulceration resolved, with mild improvement of her ptosis andlevator function and with improved full motility of the leftglobe. There was no recurrence of the lesion during 2 years offollow-up.

COMMENTScleral buckle placement is a common procedure in

retinal detachment repair. Rarely, complications may ariserequiring the removal of the buckle including scleral buckleexposure, extrusion, migration, intrusion, infection, chronicpain, inflammation, foreign body sensation, strabismus, diplo-pia, recurrent subconjunctival hemorrhage, macular distortion,impingement of the optic nerve, swelling of buckle elements,granuloma, sudden vision loss, and cutaneous extrusion.1

Hilton and Wallyn2 describes 600 cases of retinal detachmentrepairs with scleral buckles in which 23 (3.8%) were removedbecause of infection, foreign body sensation, recurrent conjunc-tival hemorrhages, impingement on the optic nerve, or maculardistortion. On diagnosis of infection, immediate removal of thebuckle is necessary to prevent further complications.3

Extrusion and intrusion of scleral buckling material arerecognized complications,4–6 yet our patient is unusual in thather eyelid findings were repeatedly misdiagnosed as a chala-zion, despite having undergone an eyelid biopsy to rule outmore ominous pathology. It took over 1 year before an accurate

A, An 81-year-old woman with left upper eyelid erythema anda focal nonhealing area of ulceration and depression for 1 year. B,An axial CT showing anterior displacement of the scleral bucklewith thickening of the overlying preseptal soft tissue.



diagnosis was made and her condition was treated. We found 2other reports in the literature of similar instances in which asilicone sponge implant caused an eyelid fistula.4,5 Our casediffers in that the sponge material in the other reports hadvisibly perforated through the eyelid skin. Our case involved asolid silicone band that eroded through the palpebral tissuesand gave the appearance of an ulcerative lesion on the eyelidrather than an obvious perforation. There are a few theories ofthe pathophysiology of an eyelid fistula formation. Accordingto the mechanic theory, a dislocated silicone implant providestension and friction against layers of the eyelid, which eventu-ally becomes fragile enough for perforation.5 Another theory isrelated to aging changes of Tenon capsule in the elderly inwhich the capsule becomes more atrophic and provides lesssupport to prevent extrusion of the implant.5,6

REFERENCES1. Covert DJ, Wirostko WJ, Han DP, et al. Risk factors for scleral

buckle removal: a matched, case-control study. Am J Ophthalmol2008;146:434–9.

2. Hilton GF, Wallyn RH. The removal of scleral buckles. ArchOphthalmol 1978;96:2061–3.

3. Hadden OB. Infection after retinal detachment surgery. Aust N Z JOphthalmol 1986;14:69–73.

4. Winward KE, Johnson MW, Kronish JW. Transpalpebral extrusionof a silicone sponge exoplant. Br J Ophthalmol 1991;75:499–500.

5. Ozerturk Y, Bardak Y, Durmus M. An unusual complication of retinalreattachment surgery. Opthalmic Surg Lasers 1999;30:483–4.

6. Russo CE, Ruiz RS. Silicone sponge rejection. Early and latecomplications in retinal detachment surgery. Arch Ophthalmol1971;85:647–50.

Ocular Bacillary Angiomatosis in anImmunocompromised ManMeltzer A. Murray, M.D.*, Katherine J. Zamecki, M.D.*,Joseph Paskowski, M.D.*, and Gary J. Lelli, Jr., M.D.†

Abstract: An immunocompromised man presented with aninflammatory eyelid lesion. Biopsy was performed; histopa-thology and special staining confirmed a diagnosis of bacillaryangiomatosis. The man was treated with oral erythromycin,and the lesion resolved. The etiologic agents of bacillary angi-omatosis are Bartonella henselae and Bartonella quintana,Gram-negative coccobacilli. The organisms stain positivelywith the Warthin-Starry silver stain. Lesions can be cutaneousor visceral and have been commonly described in immuno-compromised patients. Histopathologic examination of lesionsreveals angiogenesis and cellular proliferation. Bacillary angi-omatosis can be treated with oral antibiotics.

A 54-year-old Hispanic man infected with HIV developedprogressive swelling of his left lower eyelid over the

course of several weeks. He had been taking acyclovir for

genital herpes and had been recently treated for central nervoussystem toxoplasmosis.

On examination, there was a firm, 7 mm � 7 mm,nontender, erythematous nodule on the left lower eyelid andtarsal conjunctiva causing inferior scleral show from masseffect (Fig. 1A and B). The cornea and bulbar conjunctivaappeared uninvolved, and there was no evidence of intraocularinflammation. Vision and the remainder of the examinationwere normal. There were no additional skin lesions. The initialclinical impression was chalazion. The patient was treatedconservatively with topical bacitracin ointment, warm com-presses, and oral antibiotics without improvement in the lesion.A transconjunctival biopsy of the lesion was performed underlocal anesthesia. Subsequent to the establishment of the patho-logic diagnosis, treatment with 500 mg oral erythromycin twicedaily and gentamicin drops was instituted. The lesion was notedto resolve over 6 months. The patient’s general conditionultimately deteriorated due to central nervous system toxoplas-mosis. He was lost to follow-up on returning to his nativecountry.

Histologic examination of hematoxylin-eosin–stainedsections revealed a highly vascular lesion composed of slit-likeand focally dilated blood vessels lined by plump endothelialcells. The intervening stroma contained a variety of inflamma-tory cells with predominantly neutrophils (Fig. 2). There weregranular basophilic deposits comprised of pleomorphic bacilli(Fig. 3). The organisms stained positively with the Warthin-Starry silver stain. Gram (Brown-Brenn) and acid-fast stainsfailed to reveal organisms. Based on the histopathologic find-ings, the diagnosis of bacillary angiomatosis (BA) was made.

DISCUSSIONThe first report of the entity that has become known as

BA was made by Stoler et al.1 in 1983 at which time they used

*Department of Ophthalmology, Mount Sinai School of Medicine; and†Weill Cornell Department of Ophthalmology, New York PresbyterianHospital, New York, New York, U.S.A.

Accepted for publication October 30, 2009.The authors have no proprietary interest to disclose.Supported, in part, by Research to Prevent Blindness.Address correspondence and reprint requests to Katherine J. Zamecki,

M.D., Department of Ophthalmology, Mount Sinai School of Medicine, 1Gustave L. Levy Place Box 1183, New York, New York 10029, U.S.A.E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181cff605

FIG. 1. A, External color photograph of the lower eyelidmass. B, Mass as it appeared on the palpebral conjunctiva.

FIG. 2. Stroma of the lesion containing inflammatory cells,mainly neutrophils.



the term “atypical subcutaneous infection associated with ac-quired immunodeficiency.” Subsequent investigators describedpapular skin lesions clinically resembling Kaposi sarcoma,which showed suppuration and masses of coccobacillary or-ganisms that were Gram-negative and silver positive with theWarthin-Starry stain. LeBoit et al.2 initially called the processan epithelioid hemangioma-like vascular proliferation, consid-ering it a manifestation of cat-scratch disease. Subsequently,this pseudoneoplastic lesion has become known as BA.

The etiologic agents of BA are currently classified in thegenus Bartonella. The organism was initially classified as Roch-alimaea henselae and later named Bartonella henselae, which isalso considered the major agent of cat-scratch disease.3 B.henselae and B. quintana are currently designated as the organ-isms responsible for BA. Bartonella is a Gram-negative rod. Thedomestic cat is thought to be a significant reservoir with transmis-sion to humans via a fecal-oral route.

While typically affecting the skin of immunocompro-mised individuals as solitary or multiple lesions, BA has beenreported to involve several extracutaneous sites, including thesomatic soft tissues, liver and biliary systems, lung, bone, heart,brain, and oral mucosa. Systemic dissemination of organismscan result in bacteremia, sepsis, and death.4 In immunocompe-tent patients, the lesions may spontaneously regress.

The florid neovascularization characteristic of BA maylead to an erroneous diagnosis of Kaposi sarcoma, angiosar-coma, or pyogenic granuloma, conditions that merit widelydivergent clinical management. The angiogenesis and cellularproliferation of BA have been studied by electron microscopyand immunohistochemistry and found to represent a floridgrowth of endothelial cells. BA responds well and promptly tooral erythromycin and other antibiotics. Biopsy of an accessiblelesion, using silver impregnation of the organisms, should yieldthe correct diagnosis and permit appropriate therapy, whichusually consists of at least 4 months of oral antibiotics.

BA affecting the orbit or conjunctiva is a rare entity.There are only a few reports in the literature of such lesions.5,6

However, in the correct patient population, BA should remainon the differential diagnosis list. If diagnosed, it is responsiveto antibiotic therapy.

REFERENCES1. Stoler MH, Bonfiglio TA, Steigbigel RT, Pereira M. An atypical

subcutaneous infection associated with acquired immunodeficiencysyndrome. Am J Clin Path 1983;80:714–8.

2. LeBoit PE, Berger TG, Egbert BM, et al. Epithelioid hemangioma-

like vascular proliferation in AIDS: manifestation of cat-scracthdisease bacillus infection? Lancet 1988;1:960–3.

3. Adal KA, Cockerell CJ, Petie WA. Cat-scratch disease, bacillaryangiomatosis and other syndromes due to Rochalimea. N EnglJ Med 1994;330:1509–15.

4. Kemper CA, Lombard CM, Deresinski SC, Tompkins LS. Visceralbacillary epithelioid angiomatosis: possible manifestations of dis-seminated cat-scratch diease in the immunocompromised host: areport of two cases. Am J Med 1990;89:216–22.

5. Tsai PS, DeAngelis DD, Spencer WH, et al. Bacillary angiomatosisof the anterior orbit, eyelid, and conjunctiva. Am J Ophthal 2002;134:433–5.

6. Edmonson BC, Morris WR, Osborn FD. Bacillary angiomatosiswith cytomegalovirus and mycobacterial infections of the palpebralconjunctiva in a patient with AIDS. Ophthal Plast Reconstr Surg2004;20:168–70.

The Ethmoidal Sinus Roof:Anatomical Relationships With theIntracranial CavityYasuhiro Takahashi, M.D., Ph.D.*,Hirohiko Kakizaki, M.D., Ph.D.*,Takashi Nakano, M.D., Ph.D.†, Ken Asamoto, M.D., Ph.D.†,Dinesh Selva, M.B.B.S. (Hons), F.R.A.C.S., F.R.A.N.Z.C.O.‡,and Igal Leibovitch, M.D.§

Abstract: A detailed understanding of the relationship be-tween the ethmoidal sinus and the intracranial cavity isessential to prevent intracranial penetration during orbitalsurgery. The authors analyzed 10 postmortem orbits withtheir adjacent skull bases of 5 Asian cadavers (3 males and2 females; mean age of 80 years at death). After removingall orbital contents, skull and brain, the medial orbital wall,ethmoidal cells, and ethmoidal roof were also removed.From the intracranial cavity view, the ethmoidal roof wassituated just lateral to the cribriform plate. From theorbital view, the location of the roof was close to thesuperior border of the medial orbital wall. These anatom-ical observations may be useful to prevent intracranialpenetration and cerebrospinal fluid leakage during medialorbital wall decompression.

M edial orbital wall decompression is a common operationthat is considered safe when performed in the ethmoidal

sinus. Nevertheless, it may still be complicated by intracranialpenetration and cerebrospinal fluid (CSF) leakage when sur-gery extends beyond this sinus.1 Therefore, understanding theprecise anatomy of the ethmoidal sinus and its close relation-ship with the intracranial cavity is essential to achieve a safemedial decompression.2

The general anatomy of the ethmoidal sinus and struc-tures surrounding it is well documented.1,3 The medial wall of

Departments of *Ophthalmology and †Anatomy, Aichi Medical Univer-sity, Nagakute, Aichi, Japan; ‡South Australian Institute of Ophthalmologyand Discipline of Ophthalmology and Visual Sciences, University of Ad-elaide, South Australia, Australia; and §Division of Oculoplastic and OrbitalSurgery, Department of Ophthalmology, Tel-Aviv Medical Center, Tel-Aviv University, Tel-Aviv, Israel

Accepted for publication October 30, 2009.The authors have no financial interest related to this manuscript.Supported by Zaidanhojin Aikeikai, Aichi, Japan.Address correspondence and reprint requests to Hirohiko Kakizaki, M.D.,

Ph.D., Department of Ophthalmology, Aichi Medical University, Nagakute,Aichi 480-1195, Japan. E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181c94e81

FIG. 3. Granular basophilic deposits composed of pleomor-phic bacilli.



the ethmoidal sinus is also the lateral wall of the nasal cavity,and the nasal septum is the medial border of the intranasalspace. The superior border of the intranasal space is thecribriform plate of the ethmoidal bone. The ethmoidal sinusroof is thin and separates the sinus from the intracranial cavity.The ethmoidal sinus reaches to the cribriform plate only aroundthe superomedial corner of the sinus.

Although the ethmoidal area anatomy was previouslystudied, the clinical interpretation may vary between differentsurgical subspecialties. From the point of view of the orbitalsurgeon performing medial orbital decompressions, it is impor-tant to be familiar with the location of the ethmoidal roof asseen from the orbit or from the intracranial cavity. The purposeof our study was, therefore, to examine the relationships be-tween the ethmoidal sinus and the intracranial cavity and toemphasize the implications to the orbital surgeon.

MATERIALS AND METHODSTen postmortem orbits with their adjacent skull bases of 5

Asian cadavers (3 males and 2 females; mean age, 80 years; range,68–89 years at death) were studied. All cadavers did not have any

history of orbital surgeries, ear-nose-throat surgeries, and neurosurgicaloperations. All cadavers were registered with Aichi Medical Univer-sity, and proper consents and approvals were obtained prior to their use.All methods for securing human tissues were humane and compliedwith the tenets of the Declaration of Helsinki.

In this study, we defined the ethmoidal roof as the part betweenthe lateral border of the cribriform plate and the superior border of themedial orbital wall or the frontal sinus exit.

After removing the orbital contents, skull and brain, the medialorbital wall, ethmoidal cells, and mucosa were also removed. Forexamining the relationships between the ethmoidal sinus and theintracranial cavity, the ethmoidal roof was removed using chisels anda punch. Photographs were taken with a digital camera (CAMEDIAC-8080 Wide Zoom; Olympus, Tokyo, Japan).

RESULTSFrom the intracranial cavity view, the location of the ethmoidal

sinus roof could not be determined before removing the roof (Fig. A).After removing the bony roof, it was shown to be situated just lateralto the cribriform plate. Hence, the ethmoidal roof was not part of thecribriform plate (Fig. B). From the orbital view, the ethmoidal roof was

A, Intracranial cavity view before removing the ethmoidal roof. The location of the ethmoidal sinus roof could not be determined.B, Intracranial cavity view after removing the ethmoidal roof. The right ethmoidal roof is seen to be situated just lateral to the cribri-form plate. The periosteum on the cribriform plate is removed. C, Transorbital view, right side. In the anterior part, the ethmoidalroof is adjacent to the superior border of the medial orbital wall through the frontal sinus, but in the posterior part, the roof directlyattaches to the medial orbital wall. Dotted line borders the frontal sinus area in the orbit. D, Transorbital view, right side. The eth-moidal roof is obviously close from the superior border of the medial orbital wall. The purple dots above the bone defect correspondto the area of the cribriform plate.



obviously close to the superior border of the medial orbital wall. In theanterior part, the ethmoidal roof was adjacent to the superior border ofthe medial orbital wall through the frontal sinus (Fig. C), but in theposterior part, the roof was directly attached to the medial orbital wall(Fig. C, D). These anatomical findings were demonstrated in all 10orbits analyzed.

DISCUSSIONThe ethmoidal sinus is an important anatomical structure

that is frequently encountered by otolaryngologists, neurosur-geons, and orbital surgeons. In this study, we demonstrated theposition of the ethmoidal roof in relation to the cribriform plateand the intracranial cavity. Recognizing this position is impor-tant for orbital surgeons to avoid CSF leakage during medialorbital wall decompressions.

CSF leakage4 is an uncommon but severe complicationof medial orbital wall decompression. It is thought to beinduced by rotational movements that transmit forces to thecribriform plate, leading to fractures, followed by CSF rhinor-rhea.4 As the cribriform plate is not part of the wall of theethmoidal sinus and only attaches to the superomedial corner ofthe sinus, the fractures causing CSF rhinorrhea should includeboth the ethmoidal roof and the cribriform plate. Because only2 of 3 patients with CSF leakage have symptomatic rhinor-rhea,4 the rest of the patients may have only an ethmoidal rooffracture and an intact cribriform plate. In these cases, othersymptoms, such as headache, meningitis, and pneumocepha-lus,4 should raise the possibility of CSF.

The fact that the ethmoidal roof is made of thin cortexbone4 similar to the posterior border of the deep lateral orbitalwall,5 and the anatomical relations described in our study,points to the importance of avoiding strong rotational forcesduring decompression surgery to reduce the risk of fractures inthe ethmoidal roof and the cribriform plate, followed by un-controlled CSF leakage.

REFERENCES1. McMinn RMH, Hutchings RT. A Colour Atlas of Human Anatomy.

London, United Kingdom: Wolfe Medical Publications Ltd., 1977.2. O’Malley MR, Meyer DR. Transconjunctival fat removal com-

bined with conservative medial wall/floor orbital decompressionfor Graves orbitopathy. Ophthal Plast Reconstr Surg 2009;25:206 –10.

3. Kikawada T. 3-D Visualization of the Paranasal Sinuses: Anatomy andEndoscopic Surgery. Tokyo, Japan: Kanehara & Co., 2002:1–38.

4. Badilla J, Dolman PJ. Cerebrospinal fluid leaks complicating orbitalor oculoplastic surgery. Arch Ophthalmol 2007;125:1631–4.

5. Kakizaki H, Nakano T, Asamoto K, Iwaki M. Posterior border of thedeep lateral orbital wall—appearance, width, and distance from theorbital rim. Ophthal Plast Reconstr Surg 2008;24:262–5.

Congenital BlepharoptosisCo-occurring With VATER AssociationAkihiro Ichinose, M.D.*, Koji Nomura, M.D.†,Hideki Murakami, M.D.*, and Shinya Tahara, M.D.*

Abstract: VATER association is the tendency for 5 specificanomalies (vertebral and vascular anomalies [V], anal atresia[A], esophageal atresia and/or tracheoesophageal fistula [TE],and radial and renal anomalies [R]) to occur together in oneindividual. Recently, malformations and abnormalities, otherthan those of diagnostic criteria of VATER association, havebeen considered significant for clarifying the nature ofVATER association and for establishing guidelines for the

treatment of infants with VATER association because they aresupposed to be determinants of prognosis. Malformationsassociated with the eye have, however, scarcely been high-lighted in VATER association, although several occurrenceshave been reported. The authors describe the first case ofcongenital blepharoptosis co-occurring in a patient withVATER association. The co-occurrence of several malformationsin the ipsilateral face and hand of the patient were indicative oftheir arising from the congeneric pathogenesis. Surgical repairof the blepharoptosis was carried out by eyebrow suspensionwith fascia lata when the patient was 5 years old; a favorableoutcome was achieved in both function and aesthetics.

T he VATER association has been described as theco-occurrence of major malformations: vertebral and

vascular anomalies (V), anal atresia (A), esophageal atresiaand/or tracheoesophageal fistula (TE), and radial and renalanomalies (R); a co-occurrence of 3 of the 5 malformationsconstitutes the diagnostic criteria.1 Although the nature and thepathogenesis of VATER association remain unknown in the ma-jority of cases,2 numerous etiologies are known to be responsible,such as teratogenic exposures3 and genetic disorders.4 Recently,malformations and abnormalities, other than those of diagnosticcriteria in VATER association, have been considered significantfor clarification of the nature of VATER association and otherdisorders with multiple malformations,2,5,6 especially those re-sponsible for mental retardation or other medical or developmentalproblems that have been highlighted for establishing guidelines fortreatment of infants with VATER association. Described here, forthe first time, is the case of congenital blepharoptosis co-occurringin a patient with VATER association.

CASE REPORTA 5-year-old boy was referred to our unit for treatment of

congenital blepharoptosis in the right eye (Fig. 1). The diagnosisof VATER association had been established at birth because of the

*Department of Plastic Surgery, Kobe University, Graduate School ofMedicine; and †Department of Ophthalmology, Hyogo Prefectural KobeChildren’s Hospital, Kobe, Japan

Accepted for publication November 7, 2009.Presented in part at the 51th Annual Meeting of Japan Society of Plastic

and Reconstructive Surgery, Nagoya, April 9–11, 2008.Address correspondence and reprint requests to Akihiro Ichinose, M.D.,

Department of Plastic Surgery, Kobe University, Graduate School of Med-icine, 7-5-1, Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan. E-mail:[email protected]

DOI: 10.1097/IOP.0b013e3181c9fdf0

FIG. 1. The patient suffered from congenital blepharoptosiswith an ipsilateral anomalous long eyebrow.



presence of lateral curvature (vertebral anomaly), esophageal atre-sia, and the absence of a right thumb (radial anomaly). The only childborn of healthy nonconsanguineous parents, he was of short statureand with hypotrophy (�2 standard deviation), weight 1,680 g, height42 cm; his psychomotor development was normal. The right eyebrowwas anomalously long, and right microtia and alopecia of the righttemple were noted. CT showed no anomaly in cranial or facial bones,and no other malformation was detected. At 1 month of life, he hadundergone surgery for esophageal atresia. At 3 years of life, pollici-zation of his right index finger had been carried out.

The blepharoptosis was surgically repaired at the age of5 years. Preoperatively, the margin-to-reflex distance was �1mm in the right eye and 3 mm in the left eye; excursion of theupper eyelids was 3 mm in the right eye and 14 mm in the lefteye; visus was 0.63 and 0.8; and the optic nerve and retinaswere normal. Eyebrow suspension with autogenous fascia latawas selected because the function of the levator muscle was poorwhile that of the frontal muscle was excellent. Two strips (width,2 mm; length, 40 mm) of fascia lata were removed from the thigh.They were transferred in 2 tunnels made through the retroorbicu-laris fibroadipose layer from an incision near the margin ofthe upper eyelid to an incision 5 mm superior to the eyebrow (Fig.2). The lower ends of both strips were fixed to the tarsal plate, andthe upper ends were fixed to the subcutaneous tissue superior tothe eyebrow. The shape and the height of the upper eyelid werealso checked by physically pressing the eyebrow upward. Under-correction is preferred during operation because the fascia latatends to shorten several weeks postoperatively.

Postoperatively, the eyelid height increased, reached itsmaximum height (margin-to-reflex distance: 3 mm), andachieved symmetry with the other eyelid 3 months postopera-tively; no changes were observed thereafter (Fig. 3A–C). Anatural shape and contour of the eyelid was obtained withoutlagophthalmos or conspicuous eyelid lag in the down gaze.

DISCUSSIONVATER association is named for the tendency of 5 specific

anomalies to occur together in a single individual more commonlythan would be expected.1 It is considered to arise from abnormal-ities during mesodermal differentiation1,3,4; however, its natureand pathogenesis remain unknown. Recently, several malforma-tions and abnormalities, other than those of diagnostic criteria inVATER association, have been observed. First, some patients withVATER association are reported to have overlapping features of

many disorders with multiple malformations.7–10 Clarifying thenature of VATER association and other disorders with multiplemalformations is a challenging task.2 Second, some investigatorssuspect that the malformations and abnormalities, other than thecriteria of VATER association, might be determinants of theprognosis.6 Long-term follow-up would be conducive to deter-mine what features of VATER association or of features other thanthose of VATER association would put an individual at risk ofmental retardation or other medical or developmental problemsand would contribute to determine guidelines for the treatment ofinfants with VATER association.

Malformations around the eye have scarcely been high-lighted in VATER association; however, it is anticipated that thefrequency is, in fact, not low according to recent reports.5,6 Morethan a few occurrences have been observed, such as aniridia,5

optic disc coloboma,6 retinal anomaly,6 and strabismus.6 Suchnonfatal malformations, which had been considered less impor-tant, seem to become a more crucial issue to the patients ofVATER association who now have demonstrated a tendency to

FIG. 2. Two strips (width, 2 mm; length, 40 mm) of autoge-nous fascia lata were transferred in 2 tunnels of the right eyelidand were made deep to the orbicularis oculi between the smallincisions near the eyelid margin and the suprabrow. One endof both strips was fixed to the tarsal plate, and the other endwas fixed to the subcutaneous tissue of the suprabrow.

FIG. 3. Nine months after correction by the eyebrow suspen-sion: straight gaze (A), down gaze (B), and closed eyes (C).



live longer life spans. Malformations related to vision are signif-icant, especially that they are critical to the quality of life andcritical for mental development. Severe congenital blepharoptosisnot only impairs visual development but also significantly affectsfacial appearance. To the best of our knowledge, congenitalblepharoptosis co-occurring with VATER association has notbeen reported to date. We cannot conclude that blepharoptosis isinvolved with VATER association from the patient data, althoughthe co-occurrence of several malformations in the ipsilateral faceand hand of the patient is indicative of their arising from thecongeneric pathogenesis. Relevance of VATER association andmany co-occurring minor malformations may be determined whenthe nature and pathogenesis of VATER association is illuminated.

In our case, the corrected blepharoptosis successfullyachieved by eyebrow suspension at this age provided prospectsfor developing visual functions. The eyebrow suspension aes-thetically yielded good result as well. The method describedhere is a good choice for the individual presenting with severeblepharoptosis but with excellent function of frontal muscle.Long-term observation of the development of vision and ofmental faculties should be included in the follow-up of patients.

REFERENCES1. Quan L, Smith DW. The VATER association. Vertebral defects, Anal

atresia, T-E fistula with esophageal atresia, Radial and Renal dyspla-sia: a spectrum of associated defects. J Pediatr 1973;82:104–7.

2. Kallen K, Mastroiacovo P, Castilla EE, et al. VATER non-randomassociation of congenital malformations: study based on data fromfour malformation registers. Am J Med Genet 2001;101:26–32.

3. Merlob P, Naor N. Drug induced VATER association: is dibenz-epin a possible cause? J Med Genet 1994;31:423.

4. Reardon W, Zhou XP, Eng C. A novel germline mutation of thePTEN gene in a patient with macrocephaly, ventricular dilatation,and features of VATER association. J Med Genet 2001;38:820–3.

5. Kawano T, Wang C, Hotta Y, et al. Three novel mutations of the PAX6gene in Japanese aniridia patients. J Hum Genet 2007;52:571–4.

6. Wheeler PG, Weaver DD. Adults with VATER association: long-term prognosis. Am J Med Genet A 2005;138:212–7.

7. Hall BD. Choanal atresia and associated multiple anomalies. J Pe-diatr 1979;95:395–8.

8. Khoury MJ, Cordero JF, Greenberg F, et al. A population study ofthe VACTERL association: evidence for its etiologic heterogene-ity. Pediatrics 1983;71:815–20.

9. Holt M, Oram S. Familial heart disease with skeletal malforma-tions. Br Heart J 1960;22:236–42.

10. Feingold M, Hall BD, Lacassie Y, Martinez-Frias ML. Syndromeof microcephaly, facial and hand abnormalities, tracheoesophagealfistula, duodenal atresia, and developmental delay. Am J MedGenet 1997;69:245–9.

Hypertrichosis of the Upper CheekArea Associated With TravoprostTreatment of GlaucomaSantiago Ortiz-Perez, M.D.,and Jane M. Olver, F.R.C.Ophth.

Abstract: Travoprost is a prostaglandin analog used in thetreatment of open-angle glaucoma. This drug is safe andefficacious and has a low incidence of systemic and localside effects. Common local side effects are conjunctivalhyperemia, iris pigmentation, and hypertrichosis of theeyelashes. The authors present a case of a patient whodeveloped marked hypertrichosis of the cheek vellus 3months after starting treatment with travoprost.

CASE REPORTWe report an 80-year-old white female with a medical

history of anxiety treated with diazepam. During routine oph-thalmologic examination, diagnosis of open-angle glaucomawas made, and treatment with daily topical travoprost 0.004%(Travatan; Alcon Laboratories, Fort Worth, TX, U.S.A.) wasinitiated. The drug was well tolerated during the first weeks.After 3 months of follow-up, intraocular pressure (IOP) waswithin normal range in both eyes. The standard static auto-mated perimetry, optical coherence tomography, and clinicalexamination remained stable. Trichomegaly with mild ptosis ofthe eyelashes in both eyes was evident. Although this sideeffect was expected, she also complained of increased growthof the vellus hairs on her upper cheek (Fig. 1). We notedmarked hypertrichosis in the region of the upper cheek malareminence, including greater thickness and length of the cheekhair, and an increase in the hair pigmentation (Fig. 2). Tra-voprost treatment was suspended, and a nonprostaglandinanalog IOP-reducing agent was started. At last follow-up, 6months later, the hypertrichosis has disappeared, and theeyelashes have returned to a normal length, showing thatthese side effects are reversible after stopping the treatment(Fig. 3).

DISCUSSIONProstaglandin analogs are some of the most recent

additions to the list of ocular hypotensive medications avail-able for the treatment of glaucoma. The 3 commercial drugsat present are latanoprost 0.005% (Xalatan; Pharmacia &UpJohn, Kalamazoo, MI, U.S.A.), bimatoprost 0.03% (Lu-migan; Allergan, Irvine, CA, U.S.A.), and travoprost. Themain mechanism of action of these agents in the reduction ofIOP has been shown to be increased uveoscleral outflow ofaqueous humor.1

In clinical use, prostanoids are known to cause iris andeyelid pigmentation, eyelash growth, and conjunctival hyperemia.In general, the incidence of iris and eyelash pigmentation increaseswith the treatment period and differs depending on the iris, skin,and hair color. This may explain why the incidence differs in

Oculoplastic and Orbital Service, Western Eye Hospital and the EyeDepartment, Charing Cross, London, United Kingdom

Accepted for publication November 9, 2009.None of the authors disclose any type of financial interest related to the

manuscript.Address correspondence and reprint requests to Jane M. Olver, F.R.C.Ophth.,

Western Eye Hospital, Marylebone Road, London NW1 5QH, United Kingdom.E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181cbfbdd

FIG. 1. Appearance of 80-year-old woman after 3 months oftreatment with travoprost. Note trichomegaly with ptosis ofthe eyelashes in both eyes.



different countries and the psychological impact that these sideeffects can cause in the patients. Furthermore, it has been reportedthat prostaglandin analogs can affect not only the eyelashes butalso the adjacent adnexal hair.2–5

The prostaglandin E2 and prostaglandin F2a have beendescribed as possible modulators of hair growth. Topical use wasreported to increase human hair growth and terminal hair counts.Minoxidil, a potent trichogenic agent, was found to enhanceprostaglandin endoperoxide synthase-1 activity, suggesting a linkbetween prostaglandin synthesis and hair growth. Furthermore,topical prostaglandin analogs used in the treatment of glaucomahave been reported to cause eyelashes growth. Nevertheless, thiseffect occurs only in the application area of the drug, proving thatprostaglandins work in a paracrine way naturally.2,6

Our patient presented with hypertrichosis not only of theeyelashes but also of the cheek hair. This event has a moresignificant aesthetic impact than the eyelashes growth, espe-cially in women, and it can be due to unnoticed drops in thisarea or by applying too many drops in the eye, resulting inspillage over the cheek.

Most glaucomatous patients are old, sometimes withvisual impairment due to glaucoma and other possible pathol-ogies. Sometimes, they are unaided in administering the drops.It is common for these patients to put the drops outside theeye or to use an excessive amount. This unnoticed use ofprostaglandin analogs over the skin of the face by thepatients can cause hypertrichosis and a marked change intheir appearance.

With the widespread and increasing use of topical prosta-glandin analogs, physicians must be aware of this possible sideeffect. Patients must be informed about this effect, which can bereversible but also can cause a considerable amount of discomfortto them.

REFERENCES1. Susanna R Jr, Medeiros FA. The pros and cons of different prosta-

noids in the medical management of glaucoma. Curr Opin Ophthal-mol 2001;12:149–56.

2. Johnstone MA, Albert DM. Prostaglandin-induced hair growth.Surv Ophthalmol 2002;47(suppl 1):185–202.

3. Elgin U, Batman A, Berker N, Ilhan B. The comparison of eyelashlengthening effect of latanoprost therapy in adults and children. EurJ Ophthalmol 2006;16:247–50.

4. Centofanti M, Oddone F, Chimenti S, et al. Prevention of derma-tologic side effects of bimatoprost 0.03% topical therapy. Am JOphthalmol 2006;142:1059–60.

5. Doshi M, Edward DP, Osmanovic S. Clinical course of bimatoprost-induced periocular skin changes in Caucasians. Ophthalmology2006;113:1961–7.

6. Colombe L, Vindrios A, Michelet JF, Bernard BA. Prostaglandinmetabolism in human hair follicle. Exp Dermatol 2007;16:762–9.

Large Particle Hyaluronic Acid Gelfor the Treatment of Lower EyelidRetraction Associated WithRadiation-Induced LipoatrophyJeffrey L. Peckinpaugh, M.D., Harsha S. Reddy, M.D.,and Robert N. Tower, M.D.

Abstract: A 42-year-old female with a remote history of aleft maxillary sinus tumor treated with excision and radia-tion therapy was referred for dry eye symptoms. Ophthal-mic examination revealed left-sided exposure keratopathy,lagophthalmos, lower eyelid retraction, and fat atrophy ofinferior periorbital tissue with associated hollowing. Largeparticle hyaluronic acid gel was injected to expand andreinforce the lower eyelid. After treatment, there was sig-nificant improvement in lagophthalmos, inferior scleralshow, and periorbital hollowing. Excellent symmetry withthe fellow eye was achieved. The patient reported markedlyreduced dry eye symptoms. No adverse side effects wereobserved. The use of large particle hyaluronic acid gelshows promise as a novel nonsurgical therapy in the manage-ment of lower eyelid retraction associated with radiation-induced lipoatrophy. Additionally, large particle hyaluronicacid gel may provide better soft tissue expansion and retentionthan small particle hyaluronic acid gel, increasing the longev-ity of treatment.

L ower eyelid retraction with associated lagophthalmos andexposure keratopathy can result from multiple etiologies.

These include thyroid-associated orbitopathy, trauma, age-related involution, and postsurgical changes, most often fol-lowing blepharoplasty. Less commonly, retraction may resultfrom external beam radiation of paranasal masses, which re-sults in periorbital lipoatrophy and loss of vertical eyelidvolume.1 Standard surgical treatments for lower eyelid retrac-tion include posterior lamellar spacers, skin grafts, and myo-cutaneous advancement flaps. However, these procedures risk

Department of Ophthalmology, University of Washington, Seattle,Washington, U.S.A.

Accepted for publication November 11, 2009.Address correspondence and reprint requests to Robert N. Tower,

M.D., Department of Ophthalmology, University of Washington, HMCBox 359608, 325 9th Avenue, Seattle, WA 98104-2499, U.S.A. E-mail:[email protected]

DOI: 10.1097/IOP.0b013e3181cc85b0

FIG. 2. A, Hypertrichosis in the region of the upper cheekmalar eminence (black arrow). B, Note the increase of thick-ness, length, and pigmentation of the hair (black arrow).

FIG. 3. A and B, Six months after stopping the treatment, thehypertrichosis has disappeared. Also note that the eyelashes areof less length.



complications, including fibrosis, eyelid contraction, tissue re-jection, and the need for further surgery.2,3 A nonsurgicalalternative using small particle hyaluronic acid gel injectionshas previously been described by Goldberg et al.4 and Goldbergand Fiaschetti.5 We describe a similar technique using recentlyavailable large particle hyaluronic acid gel for a novel indica-tion, the treatment of lower eyelid retraction associated withradiation-induced lipoatrophy.

CASE REPORTA 42-year-old African American female was referred for

lagophthalmos, lower eyelid retraction, and exposure keratopa-thy of her left eye. Her medical history was significant for a leftmaxillary sinus neuroblastoma treated 18 years prior withexcision, chemotherapy, and local radiation. She reported oc-ular irritation and increasing difficulty in closing her left eyeover the past several years. She was using artificial tears withminimal relief of her symptoms.

External examination of the left eye was notable forlower eyelid retraction with moderate inferior periorbital hol-lowing. No enophthalmos or periorbital scarring was noted.Slit-lamp examination showed inferior corneal punctate epi-theliopathy in the left eye. The right eye examination wasnormal. External measurements were obtained (Table). Stan-dard pretreatment external color digital photographs weretaken (Fig. 1A, B).

A diagnosis of lower eyelid retraction with associatedlagophthalmos and exposure keratopathy from suspectedradiation-induced lipoatrophy was made. After full discus-sion of all options with the patient, we decided to proceedwith injection of large particle hyaluronic acid gel tissuefiller (Perlane, Medicis Corporation, Scottsdale, AZ, U.S.A.)to treat her lower eyelid retraction and periorbital hollowing.

Using a 30-gauge needle, a total of 1 cc of hyaluronic acidgel was injected in small amounts across the lower eyelid in2 planes as shown in Figure 2.

The first plane was just posterior to the orbicularismuscle within the substance of the suborbicularis oculus fat.Several fine multilevel injections, releasing the gel while with-drawing the needle with each injection, were given as previ-ously described.4 A second preperiosteal fill plane was createdby a series of deep injections with the needle bevel downwardalong the inferior orbital rim.

Two weeks after treatment, there was no inferior scleralshow, less than 1 mm of lagophthalmos, improvement ofinferior periorbital hollowing, and improved symmetry (Fig.1C, D). Her exposure keratopathy and ocular comfort signifi-cantly improved. On phone discussion 11 months postopera-tively, she reported sustained improvement in dry eye symp-toms and appearance of her left lower eyelid and cheek lastingfor 5 to 6 months with slow deterioration of the treatmenteffects thereafter. She reported no adverse reaction or compli-cations from the injection.

DISCUSSIONSuccessful treatment with small particle hyaluronic acid

gel of lower eyelid retraction has been described by Goldberget al.4 and Goldberg and Fiaschetti.5 They reported a meanreduction of inferior scleral show of 1.0 mm and filler longevityof 3 to 4 months, with an average interval of 3.6 monthsbetween the first and repeat injection. This case demonstratessimilar efficacy of large particle hyaluronic acid for the treat-ment of lower eyelid retraction, especially in the reduction ofinferior scleral show and lagophthalmos (2.0 mm each). Wereport good functional and cosmetic success for the treatment

Preoperative external measurements

Measurement, mm Right eye Left eye

Margin reflex distance 1 4 4Inferior scleral show 0 2Lagophthalmos 0 3Palpebral fissure height 9 12

FIG. 1. A and B, pre-procedure. C and D, post-procedure.

FIG. 2. First plane with multiple threadlike injections posterior toorbicularis within suborbicularis oculus fat (grey lines) and secondplane deep with preperiosteal fill along the orbital rim (purple dots).



of inferior hollowing and lower eyelid retraction associatedwith radiation-induced lipoatrophy, a previously undescribedindication for hyaluronic acid fillers. External beam radiationtargeting orbital and periorbital tumors is known to causedegenerative changes to periocular skin and soft tissue, whichpose therapeutic challenges.1 Advantages of this method in-clude minimal invasiveness in poor surgical candidates, poten-tial reversibility, and the ability to adjust eyelid position withadditional applications.6

We report an adequate treatment effect for approxi-mately 5 to 6 months in comparison with 3 to 4 monthsdescribed with small particle materials. Theoretically, largeparticle hyaluronic acid will retain better soft tissue expansionand prolonged duration. This may enable permanent tissueresponses that reduce the frequency or volume of subsequentfiller injections, although no definitive evidence exists cur-rently. Further longitudinal studies with larger sample sizes areneeded to better define the frequency of maintenance injectionsand potential long-term complications.

REFERENCES1. Durkin SR, Roos D, Higgs B, et al. Ophthalmic and adnexal complications

of radiotherapy. Acta Ophthalmol Scand 2007;85:240–50.2. Patel BC, Patipa M, Anderson RL, McLeish W. Management of

post-blepharoplasty lower eyelid retraction with hard palate graftsand lateral tarsal strip. Plast Reconstr Surg 1997;99:1251–60.

3. Sullivan SA, Dailey RA. Graft contraction: a comparison of acel-lular dermis versus hard palate mucosa in lower eyelid surgery.Ophthal Plast Reconstr Surg 2003;19:14–24.

4. Goldberg RA, Lee S, Jayasundera T, et al. Treatment of lower eyelidretraction by expansion of the lower eyelid with hyaluronic acid gel.Ophthal Plast Reconstr Surg 2007;23:343–8.

5. Goldberg RA, Fiaschetti D. Filling the periorbital hollows withhyaluronic acid gel: initial experience with 244 injections. OphthalPlast Reconstr Surg 2006;22:335–41; discussion 341–3.

6. Soparkar CN, Patrinely JR, Tschen J. Erasing restylane. OphthalPlast Reconst Surg 2004;20:317–8.

Enhanced Enlargement of SiliconeOcular Prostheses Achieved bySilicone Gel (MED-361)Carrie Lynn Morris, M.D.*, Matt Singer†,and James C. Fleming, M.D.*

Abstract: Recently, silicone prostheses have been used in-stead of acrylic ocular prostheses. The authors sought toassess the ability of silicone gel to facilitate the growth of anocular silicone prosthesis and to determine the best appli-cation modality to achieve maximal possible growth. Twoocular prostheses with similar baseline dimensions andcontour were used for comparison of growth environment.One prosthesis was placed in an eyelid mold, while the otherwas placed in an open Petri dish. Silicone gel drops (MED-361; NuSil Technology, Carpinteria, CA, USA) were ad-

ministered 3 times daily to both prostheses . Over thecourse of 41 days, an equal amount of enlargement oc-curred in all dimensions in both prostheses. Enlargement ofa silicone prosthesis can be achieved by various modes ofapplication of topical silicone gel.

*Department of Ophthalmology, Hamilton Eye Institute, University ofTennessee–Memphis; and †Silicone Ocular Prosthetics Inc, Memphis, Tennes-see, U.S.A.

Accepted for publication November 11, 2009.Oral presentation at 2008 ASOPRS Fall Symposium, New Orleans, LA,

U.S.A., Abstract Presentation.The authors have no financial or proprietary interest to disclose.Address correspondence and reprint requests to Carrie Lynn Morris,

M.D., Washington University, Campus Box 8096, 660 South Euclid Ave-nue, St. Louis, MO 63110, U.S.A. E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181cff9e3

FIG. 2. The gelatin and glycerin eyelid mold where prosthesisA was encased during silicone gel drop application (A). Theplastic Petri dish is shown on the right where prosthesis B wasplaced during intervals of silicone gel direct application (B).

FIG. 1. A, Specimen A (top), housed in a gelatin model tomimic the epithelium of orbital fornices, was mounted on a1-mm grid to standardize measurements and digital imaging atbaseline. Baseline comparison of width and height of specimenB (bottom) placed in a synthetic Petri dish for storage. B, Base-line comparison on 1-mm grid of Specimen A (top) and Speci-men B (bottom) in thickness.



R ecently, silicone prostheses have been used instead ofconventional acrylic ocular prostheses in anophthalmic

patients. These offer an alternative to an acrylic prosthesis, whichmay rarely cause an allergic tissue reaction.1,2 While refining thesilicone prosthesis design, patients were instructed to use siliconegel drops to lubricate the prosthesis in an effort to maintain apolished appearance. Serendipitously, it was observed that thesilicone prosthesis began to enlarge with administration of thesilicone gel drops. Given this information, we postulated that itmight be possible to intentionally enlarge a silicone prosthesis byapplication of silicone gel drops. If one could controllably manage

the growth of ocular or orbital prostheses, then socket expansionof fornices could be achieved.

Therefore, a prospective, comparison, nonblinded trial wasdesigned to evaluate whether growth of a silicone prosthesis could beachieved by application of silicone gel drops. The best applicationmodality, drop form versus direct topical lubrication, was explored.

METHODSTwo silicone ocular prostheses with similar initial dimensions

and contour (25 mm � 20.50 mm � 11.50 mm [prosthesis A]) and (25

FIG. 3. A, Specimen A at baseline measures 25 mm � 20.5 mm at width and height, respectively (top). Specimen A is pictured atday 41, showing a 2-mm increase in width and 1-mm increase in height, measuring 27 mm � 21.5 mm, respectively (bottom). Thered registration lines help reveal the exact dimensions on the graphs. Each square on the grid is 1 mm � 1 mm. B, Specimen A isshown to note a baseline thickness of 11.5 mm (top). On day 41, a 1-mm enlargement in thickness was noted of 12.5 mm (bottom).C, Specimen B was pictured on grid for comparison at baseline (top). Specimen B measures 25 mm � 21 mm, width and height,respectively. Specimen B grew an equivalent amount as specimen A, a 2-mm increase in width and 1-mm increase in height (27mm � 22 mm) was observed, respectively (bottom). D, Specimen B is measured to be 12-mm thick at baseline (top). On day 41, asimilar growth of 1 mm in thickness was observed for specimen B to be 13 mm (bottom).



mm � 21 mm � 12 mm [prosthesis B]) were used for comparison ofgrowth environment (Fig. 1). Prosthesis A was placed in an orbitalmold to mimic the epithelium of the bulbar conjunctiva and palpebralconjunctival fornices and eyelids. The mold was created using 300-bloom gelatin with glycerin material, which is commonly used intheatrical masks and molds because it has properties similar to humanskin. Prosthesis B of the same initial size was placed within a plasticPetri dish (Fig. 2). Prosthesis volumes were not measured.

Two drops (0.05 ml) of 350 cps silicone oil gel (MED-361;NuSil Technology, Carpinteria, CA, U.S.A.)3 were placed on pros-thesis A while it was within the eyelid encasement 3 times a day atregularly spaced intervals. Two drops (0.05 ml) of 350 cps silicone oil gelwere used to lubricate prosthesis B by direct placement with a finger.Digital photographs were taken at baseline and twice per week on amillimeter grid to assess enlargement in all dimensions (thickness, width,and height). Enlargement of each prosthesis was compared with eachprosthesis’ baseline dimensions and between each prosthesis.

RESULTSAfter 41 days of silicone oil gel administration to the prostheses, a

2 mm (8%) increase in width occurred in both prosthesis A and B (25–27mm) compared with baseline parameters (Fig. 3A, B, respectively). Thiswas a 0.05 mm/day rate of growth in terms of width. A 1-mm enlargementin height and thickness of each prosthesis A (height: 20.5–21.5 mm;thickness: 11.5–12.5 mm) and prosthesis B (height: 21–22 mm; thickness:12–13 mm) was observed (Fig. 3A–D). This was a 0.025 mm/day rate ofgrowth in regard to both thickness and height. Prosthesis A had a 4.9%increase in height versus 4.8% for prosthesis B. Prosthesis A became 8.7%thicker compared with prosthesis B, which became 8.3% thicker byapplying silicone gel in various forms. A continuous, steady, linear growthpattern occurred in all dimensions for both prosthesis A and B during thesilicone gel application.

DISCUSSIONEnlargement of silicone prosthesis is achieved by place-

ment of topical 350 cps silicone gel in drop form or directapplication. Use of 350 cps silicone gel may increase the sizeof a silicone prosthesis. We observed a relatively linear growthpattern of both prostheses in all dimensions with continuedadministration of silicone oil. One may extrapolate these data andpostulate that growth would have indeed continued. Siliconepolymers are thought to lay over each other in a scaffolding-likefashion, achieving enlargement.

As expected, we noted that the orbital mold wasstretched by prosthesis A. In addition, it became increasinglydifficult to set the prostheses in a grid position due to theirgrowth. These expansile forces noted may not correlate to invivo conditions induced by a contracted socket or phimoticeyelids of congenital anophthalmos. While the gelatin moldclosely mimics the epithelium of human skin, it does not havethe dynamic nature of a socket or includes contractile forces,nor does it have the advantage of elastic malleable properties ofepithelium and dermis, which may allow for socket molding.Limitations of this study include a small sample size, applica-tion and measurement error, and lack of volume measurement.

Future study goals include: (1) continued administrationof silicone drops until a plateau in growth is reached, (2)cessation of drop administration to assess whether regressionoccurs, (3) volume analysis, (4) comparison of silicone gel anda control material, (e.g., normal saline or water), and (5) humantrials involving microophthalmic and anophthalmic patients.

Silicone gel application may not be ideal for every patientin need of socket expansion due to lack of controlled growth inone dimension, but it may serve as a noninvasive alternative forthose in need of overall enlargement of the socket.

REFERENCES1. Weaver RE, Goebel WM. Reactions to acrylic resin dental prosthe-

ses. J Prosthet Dent 1980;43:138–42.2. Bozkurt B, Akyuek N, Irkec M, et al. Immunohistochemical find-

ings in prosthesis-associated giant papillary conjunctivitis. ClinExperiment Ophthalmol 2007;35:535–40.

3. NuSil Silicone Technology, MED-361, Carpinteria, CA, U.S.A.,Product Profile, March 2007.

Pseudodacryocystitis andNasolacrimal Duct ObstructionSecondary to EthmoiditisBulent Yazıcı, M.D.*, and Zeynep Yazıcı, M.D.†

Abstract: A 23-year-old woman presented with clinicalsymptoms suggestive of acute dacryocystitis. She had nohistory of epiphora, and her lacrimal drainage system waspatent on irrigation. CT findings were consistent with an-terior ethmoiditis and maxillary sinusitis. Although thesymptoms responded to antibiotic treatment, they recurred2 times within the following 5 months. During the lastepisode, a complete obstruction of the nasolacrimal ductdeveloped. The condition did not recur after externaldacryocystorhinostomy and anterior ethmoidectomy dur-ing a follow-up of 21 months. Ethmoiditis may rarely causea localized infection in the lacrimal sac region mimickingdacryocystitis. Recurrent infections may progress to com-plete obstruction of the nasolacrimal duct.

T he orbital soft tissues are separated from the anteriorethmoid sinuses by thin bones, lamina papyracea, and

lacrimal bone. Because of this close anatomical relationship,ethmoiditis may cause several orbital infections. Anterior eth-moiditis may rarely lead to a localized infection in the lacrimalsac region mimicking dacryocystitis (“pseudodacryocystitis”).1

We describe an adult patient with anterior ethmoiditis mani-festing as recurrent pseudodacryocystitis. In this case, therepeated perisaccal infections progressed to complete obstruc-tion of the nasolacrimal duct, which was different from theprevious cases.

CASE REPORTA 23-year-old woman was referred with a diagnosis of

recurrent acute dacryocystitis. On examination, a tender, ery-thematous, fluctuant swelling was noted in the lacrimal sacregion under the right medial canthal tendon. Digital pressureover the swelling did not produce any discharge through thepuncta. She had experienced a similar attack once previously.She denied any history of tearing. We performed lacrimalirrigation through the upper lacrimal canaliculus, after whichthe fluid could pass easily in the nasopharynx without reflux.The ocular examination findings were otherwise normal, andshe did not have a history of a systemic disease.

Departments of *Ophthalmology and †Radiology, Uludag University,Bursa, Turkey

Accepted for publication November 11, 2009.Presented at the 18th Annual Meeting of European Society of Ophthalmic

Plastic and Reconstructive Surgery, September 14–16, 2000, Paris, France.Address correspondence and reprint requests to Bulent Yazıcı, M.D.,

Department of Ophthalmology Uludag University, 16059 Bursa, Turkey.E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181cd5f7d



Digital subtraction macrodacryocystogram showed thepatency of the lacrimal drainage pathway, but a slight extrinsiccompression of the nasolacrimal sac and duct was noted later-ally (Fig. 1A). No diverticula formation or filling defect, whichcould be due to a dacryolith or tumor, was seen. CT dacryo-cystography showed a soft tissue mass in the right medialcanthal region and opacification of the anterior ethmoid sinus(Fig. 2). Mucosal thickening and air-fluid level were also notedin the right maxillary sinus. Extrinsic compression of the massled to a thinning of the column of contrast media in both sacand nasolacrimal duct. A culture of the purulent materialaspirated from the perisaccal mass yielded Staphylococcusaureus. Based on these findings, a diagnosis of pseudodacryo-cystitis arising from anterior ethmoiditis was made.

The symptoms disappeared with treatment with peroralamoxicillin clavulanate and nasal decongestant spray. How-ever, the same clinical picture recurred 2 times within thefollowing 5 months. During the last episode, the patient re-ported constant tearing in her right eye. There was no fluidpassage on lacrimal irrigation. Dacryocystography demon-strated complete obstruction at the junction between the lacri-mal sac and the nasolacrimal duct (Fig. 1B).

External dacryocystorhinostomy and anterior ethmoid-ectomy were performed. Macroscopically, the lacrimal boneseemed to be intact at the time of surgery. No foreign body wasnoted in the sac. The histopathologic examination showednonspecific inflammation, vascular congestion, and edema inthe sac wall. No complication or recurrence of epiphora devel-oped during a postsurgical follow-up period of 21 months.

DISCUSSIONAcute dacryocystitis is mostly secondary to acquired

idiopathic nasolacrimal duct obstruction. Other conditions thatcan cause acute dacryocystitis are lacrimal sac tumors, sacdacryoliths, diverticuli, and ethmoidal mucocele.2 There arefew reports of pseudodacryocystitis secondary to anterior eth-moiditis in the literature. Remulla et al.1 described 2 pediatricpatients who were 4 and 11 years old. In these cases, ethmoid-

itis recurred several times, which led to abscess formation inthe sac region masquerading as chronic dacryocystitis. One ofthese children was successfully treated with systemic antibiot-ics and the other with external ethmoidectomy plus an antibi-otic. Although these cases had epiphora during the acuteexacerbations, they did not progress to obstruction of thenasolacrimal duct during the follow-up period. Shvero et al.3

reported a case of ethmoiditis in an adult who presented withbilateral fistulae above the medial canthus, with no clinicalsigns of nasolacrimal duct obstruction.

Several studies have suggested a relationship betweensinonasal disorders and acquired idiopathic nasolacrimal ductobstruction.4–6 In their CT study, Kallman et al.4 reported asignificantly higher incidence of ethmoidal opacification, aggernasi cell opacification, and nasal septal deviation in patientswith nasolacrimal duct obstruction compared with control sub-jects. Francis et al.6 reported anterior ethmoidal soft tissueopacification in 15 of 107 cases of dacryostenosis on preoper-

FIG. 1. A, Right digital subtraction dacryocystography later-ally shows a patent lacrimal drainage system and some ex-trinsic compression of the nasolacrimal sac and duct laterally(arrows). B, Dacryocystogram taken 12 days after the lastrecurrence of ethmoiditis shows a complete obstruction atthe nasolacrimal duct and sac junction.

FIG. 2. A, CT dacryocystography scan shows opacification ofthe right anterior ethmoid air cells with a soft tissue mass inthe right medial canthal region. The column of contrast mediain the right lacrimal sac (arrow) is thin compared with the leftone. B, The contrast column in the distal part of the nasolacri-mal duct (arrow) appears to be of the same thickness as thoseon the other side. Sinusitis is seen in the right maxillary sinus.



ative CT evaluation. However, detailed clinical informationwas not given about this subgroup of patients.

Anterior ethmoid air cells extend beyond the sutures ofthe ethmoid bone in most normal orbits. In 93%, the cells areanatomically adjacent to the lacrimal sac fossa, with 40%entering the frontal process of the maxilla.7 In our patient too,the ethmoid cells extended to the level of the lacrimal sac inboth sides. Ethmoidal sinus infections may gain access to theorbit by direct extension through the thin ethmoidal or lacrimalbones, congenital bony dehiscence, or infected thromboembolialong the valveless venous connections between the orbit andparanasal sinuses.8 Recent clinicopathologic findings indicatethat osteitis of the sinus walls may be responsible for both thespread of the local sinusitis and the cases refractory to medicalor surgical treatments.9 The periosteal sheets surrounding thelacrimal sac circumferentially, and possibly the lacrimal fascia,prevent the lacrimal sac from orbital infection in most cases.However, as the small dehiscences or holes in the lacrimalfascia allow the transmission of infection from the sac to theother tissues of the orbit, the reverse of this is also possible.10

In the present case, the direct compression of the local abscessto the sac and nasolacrimal duct, extension of the inflammationfrom the adjacent sinusitis and perisaccal infection to thenasolacrimal duct or invasion of microorganisms in the duct, ora combination of these factors could play a role in the devel-opment of lacrimal obstruction. During episodes of pseudo-dacryocystitis, defects may develop in the lacrimal fascialbarriers secondary to stretching and inflammation, which mayallow passage of the microorganisms to the sac.

In patients who present with clinical features of acutedacryocystitis and patent lacrimal drainage system on irriga-tion, the differential diagnosis should include pseudodacryo-cystitis due to ethmoiditis. In these patients, the infection mayrecur despite systemic antibiotic treatment. Early ethmoidec-tomy should be considered to prevent recurrence of the infec-tion and progression to nasolacrimal duct obstruction.

REFERENCES1. Remulla HD, Rubin PA, Shore JW, Cunningham MJ. Pseudo-

dacryocystitis arising from anterior ethmoiditis. Ophthal PlastReconstr Surg 1995;11:165–8.

2. Hurwitz JJ. Disease of the sac and duct. In: Hurwitz JJ, ed. TheLacrimal System. Philadelphia, PA: Raven Press, 1996:117–38.

3. Shvero J, Hadar T, Freinmesser R. Fistulae in the inner canthusassociated with ethmoiditis. J Laryngol Otol 1992;106:1076–8.

4. Kallman JE, Foster JA, Wulc AE, et al. Computed tomography inlacrimal outflow obstruction. Ophthalmology 1997;104:676–82.

5. Annamalai S, Kumar NA, Madkour MB, et al. An associationbetween acquired epiphora and the signs and symptoms of chronicrhinosinusitis: a prospective case-control study. Am J Rhinol 2003;17:111–4.

6. Francis IC, Kappagoda MB, Cole IE, et al. Computed tomographyof the lacrimal drainage system: retrospective study of 107 cases ofdacryostenosis. Ophthal Plast Reconstr Surg 1999;15:217–26.

7. Blaylock WK, Moore CA, Linberg JV. Anterior ethmoid anatomy facil-itates dacryocystorhinostomy. Arch Ophthalmol 1990;108:1774–7.

8. Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis oforbital complications in acute sinusitis. Laryngoscope 1970;80:1414–28.

9. Lee JT, Kennedy DW, Palmer JN, et al. The incidence of concur-rent osteitis in patients with chronic rhinosinusitis: a clinicopath-ological study. Am J Rhinol 2006;20:278–82.

10. Shinohara H, Taniguchi Y, Kominami R, et al. The lacrimal fasciaredefined. Clin Anat 2001;14:401–5.

Presumed Primary OrbitalNeuroblastoma in a 20-Month-Old FemaleNanfei Zhang, M.D., and Lily Koo Lin, M.D.

Abstract: Neuroblastoma is the most common malignantdisease of childhood, and it often arises from either theadrenal gland or along the sympathetic chain. The au-thors report a case of a 20-month-old female with apresumed primary neuroblastoma of the orbit. Radiolog-ically, the tumor showed a brightly enhancing masslesion of the left side with intraorbital, temporal extraor-bital, transsphenoid, and intracranial components. His-topathologically, the tumor was composed of small roundblue cells. Immunohistochemical staining was positivefor neuron-specific enolase. To the best of the authors’knowledge, primary orbital neuroblastoma has only beenpreviously reported once in children, although it hasbeen reported twice in adults.

N euroblastoma is the most common extracranial solidmalignancy of childhood. The tumor is thought to arise

from the neural crest and can occur in the adrenal gland oranywhere along the sympathetic chain in the retroperitoneumor mediastinum. Secondary orbital involvement occurs in ap-proximately 20% of patients, and it is sometimes the initialmanifestation of tumor.1

CASE REPORTA 20-month-old female presented for a work-up of

child abuse with a 2-week history of periorbital ecchymosisand swelling of her left eye. There was an inconsistenthistory of left eye trauma against a sharp object. On eyeexamination, she was able to fix and follow with each eye.The left eye showed significant proptosis with resistance toretropulsion and limitation of motility in superior and lateralgazes (Fig. A). The pupils were equally round and werereactive to light. No relative afferent pupillary defect waspresent. Intraocular pressures were 16 mm Hg OD and 22mm Hg OS. Subconjunctival hemorrhage was present supe-ronasally and inferotemporally in the left eye. In addition,diffuse punctate epithelial keratopathy was present in theinterpalpebral fissure area, consistent with chronic exposurekeratopathy due to the lagophthalmos resulting from thedegree of proptosis.

The MRI of the orbit and brain showed a brightlyenhancing mass of the left side with intraorbital, temporalextraorbital, transsphenoid, and intracranial components. Therewas enhancement of the dura extending over the lateral aspectof the left frontal lobe and proptosis of the left globe withdownward and medial deviation of the muscle cone. Themass in the coronal plane measured approximately 3.4 cm inhorizontal and vertical dimensions (Fig. B). The patientunderwent a lateral orbitotomy for biopsy of the most easilyaccessible mass.

Department of Ophthalmology, University of California, Davis, Sacra-mento, California, U.S.A.

Accepted for publication November 21, 2009.No authors have any proprietary or conflicting interests to disclose.Address correspondence and reprint requests to Lily Koo Lin, M.D.,

Department of Ophthalmology, University of California, Davis, 4860 YStreet, Ste 2400, Sacramento, CA 95817, U.S.A. E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181cc8598



The hematoxylin-eosin stain revealed sheets and nests ofsmall blue cells with high nuclear-to-cytoplasmic ratios andhyperchromatic nuclei infiltrating through bone and skeletalmuscle (Fig. C). Immunostained slides revealed positive cyto-plasmic staining for neuron-specific enolase, consistent withneuroblastoma (Fig. D).

A bone marrow biopsy showed abnormal hypocellularbone with trilineage hematopoiesis and small clusters of for-eign cells, consistent with metastatic neuroblastoma. Peripheralblood smear showed mild normocytic anemia and mild leftshift in the granulocytes.

Abdominal and chest CT failed to reveal any othermasses. Urine homovanillic acid and vanillylmandelic acidlevels were within normal limits. Meta-iodobenzylguanidinescintiscan scan and bone scan were negative for sites ofmetastasis.

Due to the unfavorable factors, including age at diag-nosis, clinical stage 4 disease, and histopathologic classifi-cation in the unfavorable histology group,2 the patient wastreated according to the high-risk protocol. Subtotal resec-tion of the tumor was performed by neurosurgery withremoval of orbital roof, including dura, sphenoid bone,orbital groove, supraorbital rim, lateral orbit, zygoma, andthe soft tissue tumor involving the orbit and temporalismuscle. Pathology specimens again demonstrated neuroblas-toma cells of poorly differentiated subtype and a failure toidentify a primary tumor site. The patient received 5 courses

of chemotherapy consisting of cisplatin, etoposide, vincris-tine, cyclophosphamide, doxorubicin, and mesna. Repeatbone marrow biopsy was free of malignant cells. She under-went autologous stem cell bone marrow transplantation. At12-month follow-up, no primary site other than the orbit hasbeen identified. She continues to be followed by the pediat-ric hematology and oncology, neurosurgery, and ophthal-mology services.

DISCUSSIONLike other cases of neuroblastoma in the literature, the

initial focus of the work-up was suspected child abuse. Theteam had not recognized the concerning degree of proptosisuntil it was brought to their attention by the ophthalmologyservice. Even then, there was reluctance in the team to drop thechild abuse work-up. Once the results of the MRI, whichophthalmology had ordered, were available, the managementtook a different turn.

The intriguing aspect of this case was the primary sitebeing the orbit in a 20-month-old female. The bone marrowbiopsy showed malignant cells consistent with neuroblas-toma, but no other primary site other than the orbit couldbe identified through CT chest, CT abdomen, meta-iodobenzylguanidine scintiscan scan, and bone scan duringher follow-up period of 1 year. This tumor did not secretehigh levels of vanillylmandelic acid or homovanillic acid,

A, The appearance of the patient at 20 months of age with left-sided proptosis and characteristic periorbital ecchymosis. B, A coro-nal T1-weighted fat-suppressed postgadolinium magnetic resonance image of the brain through the orbits reveals a brightly enhanc-ing mass with intraorbital, left lateral extraorbital, transsphenoid, and intracranial components. There is a dural reaction extendingover the lateral aspect of the left frontal lobe. There is proptosis of the left globe with downward and medial deviation of the musclecone. The mass in the coronal plane measures approximately 3.4 cm in side-to-side dimension and roughly the same in vertical di-mension. C, Microscopic appearance of the tumor at the time of first left orbitotomy. The tissue is infiltrated with sheets and nests ofsmall round blue cells with high nuclear-to-cytoplasmic ratio and hyperchromatic nuclei. There are no areas of necrosis, and scantneurophil stroma is identified. Well-formed rosettes (the Homer Wright rosettes) are not present (hematoxylin-eosin, �500). D,Neuron-specific enolase stain of the neuroblastoma cells in the tissue (�100).



which might have made the search for primary site difficult.Although primary orbital neuroblastoma has been reportedin adults twice3,4 and in a child once,5 to the best of ourknowledge, this is the first report in the U.S. literature ofsuch a tumor in this age group.

Our case reinforces the important role of the oph-thalmologist in recognizing proptosis, which thwartedsignificant delay in diagnosis in this case, and in monitor-ing response to treatment and managing ophthalmiccomplications.

ACKNOWLEDGMENTSThe authors thank Dr. Jaime Campbell at UCDMC for

her help in analyzing, interpreting, and illustrating the histologysection.

REFERENCES1. Weinstein JL, Katzenstein HM, Cohn SL. Advances in the diagnosis

and treatment of neuroblastoma. Oncologist 2003;8:278–92.2. Shimada H, Ambros IM, Dehner LP, et al. The International

Neuroblastoma Pathology Classification (the Shimada system).Cancer 1999;86:364–72.

3. Bullock JD, Goldberg SH, Rakes SM, et al. Primary orbital neuro-blastoma. Arch Ophthalmol 1989;107:1031–3.

4. Jakobiec FA, Klepach GL, Crissman JD, Spoor TC. Primary differen-tiated neuroblastoma of the orbit. Ophthalmology 1987;94:255–66.

5. Mirzai H, Baser EF, Tansug N, et al. Primary orbital neuroblastomain a neonate. Indian J Ophthalmol 2006;54:206–8.

Isolated Cavernous Hemangiomaof ConjunctivaArchana Malik, M.S.*, Soniya Bhala, M.S.*,Sudesh K. Arya, M.S.*, Subina Narang, M.S.*,Rajpal P. S. Punia, M.D.†, and Sunandan Sood, M.S.*

Abstract: A 70-year-old Indian female presented with alobulated, violet-colored mass in the left eye involving themedial bulbar conjunctiva and caruncle. It was smooth,vascular, compressible, and freely mobile. It was neitherreducible nor pulsatile and did not cause any proptosis ordystopia. Any syndromic associations and orbital extensionof the lesion were ruled out. Excisional biopsy was per-formed, and histopathologic examination confirmed thediagnosis of cavernous hemangioma of conjunctiva involv-ing the caruncle.

H emangioma is a developmental malformation of bloodvessels, rather than a true tumor, and is an example of a

hamartoma.1 It may be capillary, venous (cavernous), or arte-rial. Cavernous hemangioma can appear at any age, consists ofmultiplicity of venous channels of varying caliber, shows notendency to involution, and may become larger and trouble-some by bleeding after remaining stationary for years. It ismostly seen in the orbit and eyelid and is relatively uncommon

in the conjunctiva and caruncle. Because of this rarity, aninteresting clinical report of this condition is reported.

CASE REPORTA 70-year-old female presented with a painless mass in

the left eye medially since 5 years. It increased gradually to itspresent size and was constant over the past 2 years. There wasno change in color and no bleeding from the mass. Cosmesiswas the only concern.

On examination, a smooth, lobulated, oval, violet-coloredmass measuring 5 � 4 � 3 mm appeared to involve both thecaruncle and bulbar conjunctiva (Fig. 1). It was soft, vascu-lar, compressible, and freely mobile. The swelling wasneither reducible nor pulsatile. Adjacent eyelid margin wasslightly ulcerated due to pressure effect of the mass. Therewas no proptosis or dystopia, thereby ruling out any orbitalextensions. Extraocular movements were normal. Visualacuity was 20/200 in both eyes. Anterior segment examina-tion revealed grade IV nuclear sclerosis in both eyes, andfundus was within normal limits.

Cardiovascular and dermatologic examination was nor-mal, which ruled out any syndromic association. Hemogramwas normal. Contrast-enhanced CT of the orbits and skull andultrasound of the abdomen were normal.

A clinical diagnosis of caruncular vascular lesion wasmade, and excisional biopsy was performed. The growth wasexcised in toto under local anesthesia and sent for histopatho-logic examination. No posterior extension of the mass wasnoticed perioperatively. Histopathology confirmed the diagno-sis of cavernous hemangioma of bulbar conjunctiva involvingthe caruncle. Epidermis showed stratified squamous mucosa-covered tissue containing sebaceous gland, suggesting carun-cular involvement, and subepithelial tissue showed dilatedvascular channels, some filled with blood, along with thepresence of moderate mixed inflammatory cell infiltrate (Fig.2). The postoperative period was uneventful. The ulcerated area

Departments of *Ophthalmology and †Pathology, Government MedicalCollege and Hospital, Chandigarh, India

Accepted for publication November 21, 2009.The authors do not have any proprietary or financial interest in any

procedure or product mentioned in this manuscript.Address correspondence and reprint requests to Archana Malik, M.S.,

Government Medical College and Hospital, Sector 32, Chandigarh, India.E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181cd6307

FIG. 1. A round, vascular, freely mobile, compressible massarising from the bulbar conjunctiva and involving the caruncleis shown.



on the lids healed. The patient underwent cataract surgery 2months later with good gain in vision. Since then, the patienthas been followed up with no recurrence at 1 year of follow-up,and her cosmetic appearance is normal.

DISCUSSIONCavernous hemangioma is the most common benign

orbital tumor in adults.2 Eyelid is a common site, but conjunc-tival cavernous hemangioma is a rarity, and to our knowledge,no case report of cavernous hemangioma of the caruncle isreported. In a series of 1,643 conjunctival tumors, less than 1%were cavernous hemangiomas.3 Conjunctival cavernous hem-angioma arises often from the conjunctival vessels and rarelyfrom the scleral, muscular, or orbital vessels. It is asymptomaticand stationary for many years but gives poor cosmetic appear-ance and sometimes bleeds, giving rise to bloody tears.

The present case was asymptomatic, except for thecosmetic factor. Total excision was possible, and histopatho-logic examination confirmed the diagnosis. There was no re-currence on 1 year of follow-up.

Only 3 cases of isolated cavernous hemangioma of theconjunctiva have been reported. Rao et al.4 reported a similarcase that was confirmed on excisional biopsy, although carun-cular involvement has not been commented upon. A youngmale with a bulbar cavernous hemangioma was reported byKoo et al.5 Another report of isolated pedunculated hemangi-oma of the conjunctiva exists in literature.6 A case of bluerubber bleb nevus syndrome was noted in an infant who hadorbital, cutaneous, conjunctival, retinal, and intestinal cavern-ous hemangiomas.7 This is uncommon in adults.

Our patient had isolated cavernous hemangioma of thecaruncle and conjunctiva, without orbital involvement. To ourknowledge, no reports of caruncular cavernous hemangiomahave been reported. Orbital involvement should be ruled out toconfirm the extent of the lesion. Treatment in patients withorbital involvement includes orbitotomy or transconjunctivalapproach, and treatment in patients with isolated caruncularinvolvement includes excisional biopsy.

REFERENCES1. Jagger J, Henderson H. Bailey & Love’s Short Practice of Surgery.

25th ed. London, United Kingdom: Edward Arnold, 2008:645–9.

2. Shields JA, Bakewell B, Augburger JJ, Flanagan JC. Classificationand incidence of space-occupying lesions of the orbit. A survey of645 cases. Arch Ophthalmol 1984;102:1606–11.

3. Shields JA. Vasculogenic tumors and malformations. In: Shields JA,ed. Diagnosis and Management of Orbital Tumors. Philadelphia,PA: WB Saunders, 1989:134–8.

4. Rao MR, Patankar VL, Reddy V. Cavernous hemangioma of con-junctiva (a case report). Indian J Ophthalmol 1989;37:37–8.

5. Koo BS, Kang DU, Kim IT. A case of cavernous hemangioma of thebulbar conjunctiva. J Korean Ophthalmol Soc 1989;30:649–51.

6. Prasad GN. Pedunculated hemangioma from the conjunctiva. IndianJ Ophthalmol 1971;19:182.

7. Crompton JL, Taylor D. Ocular lesions in the blue rubber blebnaevus syndrome. Br J Ophthalmol 1981;65:133–7.

Effects of Reflected CO2 LaserEnergy on Operative Field Materials:Risks to Patients and OperatingRoom PersonnelMatthew A. Hammons, M.D.*, Nicholas A. Ramey, M.D.†,Sandra Stinnett, Dr.P.H.†, and Julie A. Woodward, M.D.†

Abstract: This study investigates the effects of specularlyand diffusely reflected CO2 laser energy on operative fieldmaterials. The CO2 laser was reflected off the sandblastedand polished surfaces of an eyelid plate and a wet cottongauze pad. The laser was aimed at a surgical glove, opera-tive gown, laser safety goggles, and endotracheal tube, eachpositioned 5 cm, 10 cm, and 15 cm from the plate. Primaryoutcomes were time to initial effect and description ofresult. There was no reflection off wet gauze and no effecton the gauze itself. When reflected off sandblasted andpolished surfaces, the laser created a hole in the glove anda flame in the surgical gown. When targeting safety gogglesand an endotracheal tube, the laser created a surface divotat short distances and surface irregularity at 15 cm. Whilethe CO2 laser is an excellent surgical instrument, reflectedlaser energy can affect operative field materials.

T he CO2 laser has been used in multiple surgical specialtiesfor decades, including oculoplastic, plastic, dermatologic,

and gynecologic surgeries. Advantages of laser use includeimproved intraoperative hemostasis, clearer view of relevant anat-omy, decreased operating time, and improved appearance in theimmediate postoperative period.1 The safety of CO2 laser formedical procedures has been extensively explored.2–5

Instruments are made “laser safe” by altering their sur-faces through sandblasting, ebonization, and anodization.Driver and Taylor6 compared the power densities of reflectionsoff samples of stainless steel that were polished, roughened,and/or chemically blackened. Polished steel reflected mostenergy, followed by blackened surface, then shot-blasted sur-face, and finally a combination of blackened and shot-blasted

*North Texas Ophthalmic Plastic Surgery, Fort Worth, Texas; and †DukeUniversity Eye Center, Durham, North Carolina, U.S.A.

Accepted for publication November 29, 2009.Presented at the Association for Research in Vision and Ophthalmology,

Fort Lauderdale, FL, U.S.A., May 2002 and the American Society ofOphthalmic Plastic and Reconstructive Surgery Fall Meeting, Key Largo,FL, U.S.A., November 2007.

Address correspondence and reprint requests to Nicholas A. Ramey,M.D., Duke University Medical Center, Box 3802, Durham, NC 27710,U.S.A. E-mail: [email protected]

DOI: 10.1097/IOP.0b013e3181db5498

FIG. 2. Photomicrograph showing epidermis-lined tissue withsebaceous glands. Dermis shows dilated caverns filled withRBCs (hematoxylin-eosin, �40).



surface.6 This study investigates effects of CO2 laser reflectionsoff a “laser-safe” surgical instrument on several operative fieldmaterials.

METHODSA Sharplan 40c CO2 laser (Lumenis, Inc., Santa Clara,

CA, U.S.A.) was set at the typical 6.0 W in continuous-wavemode for incisional surgery. The hand piece was fixed at 30°from the normal of the following reflective surfaces: wet cottongauze, polished handle and sandblasted working area of a usedbut sterilized Jaeger eyelid plate (Oculo-Plastik Inc., Montreal,Canada). A Jaeger eyelid plate is generally used to protectocular/periocular tissues from misdirected direct laser energy.The instrument was inspected under 2.5� magnification aftereach trial to assess for surface changes. The laser was kept in

pinpoint focus on the reflective surface. Target materials in-cluded a surgical glove, gown, CO2 safety goggles (OD6 at200–360 nm, 10,600 nm), and endotracheal tube, each fixed at5 cm, 10 cm, and 15 cm from the reflective surface. For eachmaterial, the time to observable change and a description of thechange were recorded for 3 trials at each test distance. Analysisof variance techniques were used to assess the significance ofthe variation of the time to effect off the different reflectivesurfaces.

RESULTSNo change was observed on the surface of the wet gauze

after 30 seconds of continuous laser firing. No alterations in thesandblasted or polished surfaces of the Jaeger plate wereobserved throughout the experiment.

For all surfaces except the goggles, the polished reflec-tive surface created visible changes more quickly (Table). Nochange was appreciable after 15 seconds of continuous laserfiring for both the goggles and endotracheal tube at 15 cm.Neither reflective surface created a consistently larger defect.Effects of the CO2 laser energy on target materials are dis-played in the Figure.

DISCUSSIONThis study demonstrates a clear risk of damage to oper-

ative field materials from reflected laser energy off both thesandblasted and the polished surface of Jaeger eyelid plate. At5 cm from a “laser-safe” reflective surface, the reflected beamburned a hole in a surgical glove in less than 2 seconds of laserfiring. The time to initial damage was shorter with the polishedreflective surface for all materials but the goggles at 10 cm.Initial changes were difficult to visualize on the goggles’ clearsurface. This difficulty may have contributed to observer errorin timing. Future investigations may use thermometric moni-toring to minimize this error. Because no damage to or reflec-tion from dampened gauze was noted after more than 30

Results of analysis of variance comparison of time toinitial visible change in target material with laserreflected off the polished versus the sandblasted surface

Targetmaterial

Distance(cm)

Mean time toinitial change

(seconds)

Difference pPolishedsurface

Sandblastedsurface

Glove 5 1.09 1.64 0.55 1.00010 1.88 3.95 2.07 0.00415 5.39 7.50 2.11 0.003

Surgicalgown

5 0.73 1.25 0.52 1.00010 1.31 3.02 1.71 0.02615 2.83 9.24 6.41 �0.001

Goggles 5 1.37 2.54 1.17 0.70210 4.64 4.44 �0.020 1.000

Endotrachealtube

5 1.00 1.11 0.11 1.00010 3.10 12.31 9.21 �0.001

A, Surgical glove with full-thickness holes from the CO2 laser. B, Surgical gown material with full-thickness holes and charred areasfrom the CO2 laser. C, Plastic safety goggles with divots on the surface from the CO2 laser. D, An endotracheal tube showing a largesurface divot from the CO2 laser.



seconds of continuous laser firing, we recommend replacing theJaeger plate with a nonspecular wooden tongue blade wrappedin damp gauze. This implement may be used to protect thecornea, globe, or any underlying or adjacent tissues during anyprocedure that involves laser energy.

No correlation was found between reflective surface anddamage size. The surface of the Jaeger eyelid plate was curved,which could create unpredictable diffuse reflections and ex-plain variability in defect size. This effect was minimized byaiming the laser at a fixed location on both the polished andsandblasted surfaces. The curvature of target materials couldaffect defect size. The gown and gloves were stretched to createflat target surfaces, while the goggles and endotracheal tubewere both curved. Although care was taken to orient targetsperpendicular to the angle of incidence, any variation couldlead to differences in the angle of impact and thus in the size ofthe created defect. These curved surfaces may be flattened tominimize this effect in future studies.

Our experiment assessed the effect of reflected CO2 laserenergy on common operative field materials. Although “laser-

safe” instruments should be used during CO2 laser procedures,surgeons and support staff should be aware that laser energyreflected from these instruments may still damage operativefield materials and personnel. This study supports the use of atongue blade wrapped in damp gauze to prevent such damage.

REFERENCES1. Baker SS, Muenzler WS, Small RG, Leonard JE. Carbon dioxide

laser blepharoplasty. Ophthalmology 1984;91:238–44.2. Sliney DH. Laser safety. Lasers Surg Med 1995;16:215–25.3. Rohrich RJ, Gyimesi IM, Clark P, Burns AJ. CO2 laser safety

considerations in facial skin resurfacing. Plast Reconstr Surg 1997;100:1285–90.

4. Hirshman CA, Smith J. Indirect ignitioin of the endotracheal tubeduring carbon dioxide laser surgery. Arch Otolaryngol 1980;106:639–41.

5. Wald D, Michelow BJ, Guyuron B, Gibb AA. Fire hazards and CO2laser resurfacing. Plast Reconstr Surg 1998;101:185–8.

6. Driver I, Taylor C. The effect of surface finish on the reflection ofCO2 laser beams from specula. Phys Med Biol 1987;32:227–35.



Documents

Two Cases of Periocular Cutaneous Angiosarcoma · eyelid with Tenzel flap and cross-eyelid flap from lower eyelid and nasal reconstruction with axial flap. FIG. 1. A, Irregular, poorly