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Downloaded from https://journals.lww.com/op-rs by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3POoLCqWplEcXgigPxl+hzaz2jeQzVWMuVYvAlO4WGcp1IqpYj8K7ag== on 05/15/2018 BRIEF REPORTS Two Cases of Periocular Cutaneous Angiosarcoma Damrong 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 8 previous reports of eyelid involvement. The authors report 2 further cases, one as a primary lesion and the other as a recurrence from a contiguous area. In both cases, the lesions appeared relatively inconspicuous, and their extent during micrographic excision was considerably larger than anticipated. Although wide surgical margins were obtained and adjuvant radiotherapy and chemotherapy was under- taken, one patient had died from distant metastasis, while the 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 or multifocal and most commonly involves the scalp and upper face in elderly whites. 2,3 They may present as flat or nodular lesions, may have surrounding edema, and their color may range 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 are predisposing factors for cutaneous AS, but only 10% of tumors have this association. No underlying genetic abnormality has been reported for cutaneous AS. AS involving the eyelid is 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 1 An 82-year-old white woman presented with a painless, erythematous, slightly elevated lesion in the left, medial upper eyelid, increasing in size over the previous 6 weeks. Also noted were small, irregular, poorly defined violaceous papules on the left nasal bridge and lower eyelid and cheek (Fig. 1A). An incisional biopsy of the upper eyelid showed oddly shaped vascular channels lined by malignant endothelial cells (Fig. 1B). A systemic workup found no metastases. She was treated by Mohs 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 forehead flap, and skin grafts was followed 6 weeks later by focal radiotherapy (Fig. 1D). Although no apparent recurrence de- veloped in the face, within 1 year, she developed a right pleural effusion from a pulmonary metastasis, followed by liver me- tastases. She died 6 months later. CASE 2 A 71-year-old white male was referred with a viola- ceous, painless maculo-papular lesion at the left medial upper eyelid (Fig. 2A). He had been diagnosed with angiosarcoma of the nasal bridge 4 years previously and treated with complete excision of the nose, reconstruction, and adjuvant radiotherapy. An incisional biopsy confirmed recurrent angiosarcoma (Fig. 2B). A systemic workup was negative, and the eyelid lesion was treated 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 †Pathology and 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, Canada V5Z 3N9. E-mail: [email protected] DOI: 10.1097/IOP.0b013e3181c0e180 FIG. 2. A, Violaceous, painless maculo-papular lesion in the left 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 recurrent poorly differentiated angiosarcoma showing a predominantly solid pattern with numerous vacuoles (hematoxylin-eosin). C, Excision with wide margins of left medial eyelid following biopsy-proven angiosarcoma. D, Reconstruction of left medial eyelid with Tenzel flap and cross-eyelid flap from lower eyelid and nasal reconstruction with axial flap. FIG. 1. A, Irregular, poorly defined violaceous papules on the left, medial upper eyelid and on the left nasal bridge and lower eyelid and cheek. B, Photomicrograph of eyelid skin showing infiltration of the dermis by cutaneous angiosarcoma com- posed of cords of atypical epithelioid cells, some of which form small lumens. The lesion stains for factor VIII-related antigen and also stained focally for CD31. (Antifactor VIII-related anti- gen, hematoxylin counterstain). C, Mohs excision with wide free-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

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

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    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 orsubcutaneous 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. Gündüz 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 withfrozen 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 byphotosensitivity, 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.

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

    368 © 2010 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

  • 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.

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

    © 2010 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. 369

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

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  • 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. Ozertürk 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 thecourse 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.

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  • 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 ethmoidalsinus. 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.

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  • 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.

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  • 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 andvascular 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.

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  • 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).

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  • 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. Källén 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.

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  • 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]ashington.edu

    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.

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  • 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).

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  • 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.

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  • R ecently, silicone prostheses have been used instead ofconventional acrylic ocular prostheses in anophthalmicpatients. 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).

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  • 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 EthmoiditisBülent 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, andlacrimal 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 Bülent Yazıcı, M.D.,

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

    DOI: 10.1097/IOP.0b013e3181cd5f7d

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  • 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 p