Ocular Considerations in Face Transplantation: Report of 2 Cases and Review of the Literature.

PURPOSE: Periorbital injuries are common in face transplantation (FT) candidates. It is therefore essential that the ophthalmologist play a central role in the multidisciplinary treatment of these patients. In this study, the authors perform a comprehensive review of all procedures involving periorbital components, provide an update for the ophthalmology community regarding the current state of the field, and present 2 cases. METHODS: A comprehensive review of the literature for all FT procedures including periorbital components was performed. The authors also present 2 patients who received FT including periorbital components for extensive facial disfigurement. One patient sustained high-energy avulsive ballistic injury and underwent a total face, double jaw, and tongue transplant in 2012. The second patient received a total face, eyelids, ears, and skeletal subunits transplant for extensive facial burns in 2015. RESULTS: Literature review demonstrated that 22 (54%) of the 41 patients undergoing FT received allografts containing periorbital components. Only 14 cases (64%) reported on the presence of ocular and periocular complications. The most common complications consisted of lower eyelid ectropion and lagophthalmos, and nearly all required revisional procedures. Both patients presented with significant periorbital scarring and demonstrated good visual acuity and aesthetic outcomes at postoperative follow up between 6 and 28 months. CONCLUSIONS: Face transplantation can address extensive facial and periorbital disfigurement with satisfactory functional and aesthetic outcomes. The majority of FT performed to date have included periorbital components, and postoperative ocular and periocular complications are common. It is critical for ophthalmologists to play a central role in the care of these patients.

Exposure rate of smooth surface tunnel porous polyethylene implants after enucleation.

PURPOSE: To determine the exposure rate after enucleation using the smooth surface tunnel (SST) and SST-EZ porous polyethylene (PP) orbital implants (Stryker Kalamazoo, MI, U.S.A.). METHODS: The authors performed an institutional review board-approved retrospective review of 150 consecutively placed SST and SST-EZ PP implants after enucleation. Patient records were reviewed for comorbidities, surgical details, follow-up length, and presence of implant exposure. All prior published studies on porous orbital implant insertion were reviewed, and the exposure rate for patients undergoing enucleation with primary implant insertion was studied for comparison. RESULTS: Of the 150 implants, 30 were excluded due to follow up of less than 8 weeks. Of the remaining 120 implants, 4 (3.3%) became exposed during the follow-up period (mean, 2.1 years), 2 of which were in children who underwent chemotherapy for retinoblastoma. The authors calculated a 7.1% exposure rate for all porous implant materials placed primarily after enucleation from 58 previously published studies. CONCLUSIONS: The SST and SST-EZ PP implants are well tolerated when placed at the time of enucleation. The theoretical advantage of a smooth anterior surface may indeed lead to a lower rate of exposure compared with standard unwrapped porous materials as demonstrated by the relatively low exposure rate using this implant.

Ocular manifestations of xeroderma pigmentosum: long-term follow-up highlights the role of DNA repair in protection from sun damage.

OBJECTIVE: Xeroderma pigmentosum (XP) is a rare autosomal recessive disease caused by mutations in DNA repair genes. Clinical manifestations of XP include mild to extreme sensitivity to ultraviolet radiation resulting in inflammation and neoplasia in sun-exposed areas of the skin, mucous membranes, and ocular surfaces. This report describes the ocular manifestations of XP in patients systematically evaluated in the Clinical Center at the National Institutes of Health. DESIGN: Retrospective observational case series. PARTICIPANTS: Eighty-seven participants, aged 1.3 to 63.4 years, referred to the National Eye Institute (NEI) for examination from 1964 to 2011. Eighty-three patients had XP, 3 patients had XP/Cockayne syndrome complex, and 1 patient had XP/trichothiodystrophy complex. METHODS: Complete age- and developmental stage-appropriate ophthalmic examination. MAIN OUTCOME MEASURES: Visual acuity; eyelid, ocular surface, and lens pathology; tear film and tear production measures; and cytologic analysis of conjunctival surface swabs. RESULTS: Of the 87 patients, 91% had at least 1 ocular abnormality. The most common abnormalities were conjunctivitis (51%), corneal neovascularization (44%), dry eye (38%), corneal scarring (26%), ectropion (25%), blepharitis (23%), conjunctival melanosis (20%), and cataracts (14%). Thirteen percent of patients had some degree of visual axis impingement, and 5% of patients had no light perception in 1 or both eyes. Ocular surface cancer or a history of ocular surface cancer was present in 10% of patients. Patients with an acute sunburning skin phenotype were less likely to develop conjunctival melanosis and ectropion but more likely to develop neoplastic ocular surface lesions than nonburning patients. Some patients also showed signs of limbal stem cell deficiency. CONCLUSIONS: Our longitudinal study reports the ocular status of the largest group of patients with XP systematically examined at 1 facility over an extended period of time. Structural eyelid abnormalities, neoplasms of the ocular surface and eyelids, tear film and tear production abnormalities, ocular surface disease and inflammation, and corneal abnormalities were present in this population. Burning and nonburning patients with XP exhibit different rates of important ophthalmologic findings, including neoplasia. In addition, ophthalmic characteristics can help refine diagnoses in the case of XP complex phenotypes. DNA repair plays a major role in protection of the eye from sunlight-induced damage.

Failure of radiation therapy in orbital xanthogranuloma.

PURPOSE: The efficacy of radiation therapy in orbital xanthogranuloma in patients who fail medical therapy is unclear. The purpose of this study was to ascertain its effectiveness. METHODS: The records of 11 cases were reviewed retrospectively for histopathologic findings, age, gender, site of involvement, clinical manifestations, and outcomes of treatment. The case histories of the 4 patients treated with radiation, all of whom had failed medical treatment, were described. RESULTS: Of 11 patients, 5 were female, and all were white. The age range at the time of presentation was 25 to 85 years. Nine patients had bilateral involvement. Five patients, all of whom had bilateral disease, had systemic manifestations or autoimmune disease thought to be related to their orbital disease. In general, patients treated with systemic corticosteroids had at least a partial response of their lesion. However, none of the 4 patients treated with orbital radiation (3 of whom had not responded to steroid treatment and 1 of whom had responded only to high-dose steroids) experienced improvement, and at least 3 experienced exacerbation of their disease. The histologic features before treatment in all cases were similar and consistent with xanthogranuloma. CONCLUSION: Orbital xanthogranuloma may be a unilateral or bilateral condition. Particularly when bilateral, it may be associated with similar lesions elsewhere or with systemic autoimmune disorders. The results of this study suggest that fractionated radiotherapy not only may be ineffective but also may exacerbate the progression of the orbital lesions in patients who do not respond to medical therapy or who are steroid dependent on intolerable doses of medication.

Asymptomatic conjunctival mucosa-associated lymphoid tissue-type lymphoma with presumed intraocular involvement.

PURPOSE: To report a case of conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma with presumed intraocular involvement. METHODS: Observational case report. RESULTS: A 73-year-old white man presented for a routine eye examination and was found to have a salmon-colored bulbar conjunctival mass of the left eye. Ultrasound showed a low-reflective mass with diffuse thickening of the ciliary body and choroid. Immunohistochemistry and flow cytometry of an incisional biopsy specimen suggested a polyclonal lesion. Treatment with topical steroids yielded no clinical improvement, and excisional biopsy was performed. A diagnosis of MALT lymphoma was made after polymerase chain reaction (PCR) analysis of the immunoglobulin heavy chain (IgH) locus revealed a clonal B-cell population. CONCLUSIONS: Conjunctival MALT lymphoma can present without symptoms and can extend intraocularly. PCR analysis of the IgH locus can identify lesion clonality when immunohistochemistry and flow cytometry fail to do so.

The Usage of a Conjunctival Flap to Improve Retention of Boston Type 1 Keratoprosthesis in Severe Ocular Surface Disease.

PURPOSE: The Boston keratoprostheses type 1 devices (KPro) are utilized in cases unfavorable to penetrating keratoplasty. The prognosis remains guarded in cases of ocular surface disease due to risk of tissue necrosis. We describe a novel surgical approach using a conjunctival flap with a delayed opening to improve retention. METHODS: In three patients with advanced cicatrizing conjunctivitis, a Type 1 keratoprosthesis was stabilized using a full tarsal conjunctival flap. Three months postoperatively, an opening was created in the flap overlying the optical portion of the device. RESULTS: All patients had no device related complications over a mean follow-up period of 17.7 months (range 15-21 months) and vision remained excellent at better than 20/200 for all patients. CONCLUSIONS: Utilization of a tarsal flap either primarily as part of a two stage modified technique or secondarily in cases of tissue necrosis and impending device extrusion might maximize retention of the type 1 KPro.

Optimizing Reconstruction with Periorbital Transplantation: Clinical Indications and Anatomic Considerations.

UNLABELLED: Complex periorbital subunit reconstruction is challenging because the goals of effective reconstruction vary from one individual to another. The purpose of this article is to explore the indications and anatomic feasibility of periorbital transplantation by reviewing our institutional repository of facial injury. METHODS: Institutional review board approval was obtained at the R Adams Cowley Shock Trauma Center for a retrospective chart review conducted on patients with periorbital defects. Patient history, facial defects, visual acuity, and periorbital function were critically reviewed to identify indications for periorbital or total face (incorporating the periorbital subunit) vascularized composite allotransplantation. Cadaveric allograft harvest was then designed and performed for specific patient defects to determine anatomic feasibility. Disease conditions not captured by our patient population warranting consideration were reviewed. RESULTS: A total of 7 facial or periorbital transplant candidates representing 6 different etiologies were selected as suitable indications for periorbital transplantation. Etiologies included trauma, burn, animal attack, and tumor, whereas proposed transplants included isolated periorbital and total face transplants. Allograft recovery was successfully completed in 4 periorbital subunits and 1 full face. Dual vascular supply was achieved in 5 of 6 periorbital subunits (superficial temporal and facial vessels). CONCLUSIONS: Transplantation of isolated periorbital structures or full face transplantation including periorbital structures is technically feasible. The goal of periorbital transplantation is to re-establish protective mechanisms of the eye, to prevent deterioration of visual acuity, and to optimize aesthetic outcomes. Criteria necessary for candidate selection and allograft design are identified by periorbital defect, periorbital function, ophthalmologic evaluation, and defect etiology.

Eyelid transplantation: lessons from a total face transplant and the importance of blink.

BACKGROUND: Despite inclusion of periorbital structures in facial transplants, critical assessment of posttransplantation short- and long-term periorbital function has not been reported. The purpose of this article is to report recovery of ocular and periorbital function, with critical appraisal of posttransplant blink in the setting of revision surgery. METHODS: Prospective ocular and periorbital functional assessments were completed at multiple time points in a patient undergoing facial transplantation and subsequent revision operations. Function was evaluated using clinical ocular examinations, visual acuity assessments, photography, and video at various intervals from preoperative baseline to 13.5 months after transplantation. During this period, revision operations involving periorbital structures were performed at 6 and 9 months after transplantation. RESULTS: Before transplantation, volitional blink was 100 percent in both eyes. Involuntary blink was 40 percent in the right eye and 90 percent in the left eye, with occasional full closure. Following face transplantation, voluntary blink was preserved, partial skin sensation was present, and involuntary blink improved to 70 percent in the right eye and 100 percent in the left eye. Following revision surgery, visual acuity and voluntary and involuntary blink were impaired. By 7.5 months after revision, improvement comparable to the pretransplantation assessment was observed. CONCLUSIONS: Adherence to principles of blink preservation is critical in periorbital transplantation. Involuntary blink is essential for preserving vision, and can be improved after transplantation. Revision surgery may temporarily impair advances made with initial allotransplantation. A comprehensive understanding of ocular biomechanics and function is invaluable to the reconstructive surgeon performing facial transplantation involving periorbital structures and posttransplant revision operations. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Simple outpatient postoperative analgesia using an orbital catheter after enucleation.

OBJECTIVE: To evaluate an indwelling orbital catheter, placed at enucleation, for repeatable delivery of local anesthetic on an outpatient basis. METHODS: A retrospective, noncomparative, case series medical record review was performed of patients undergoing enucleation and receiving an indwelling orbital pain-control catheter at surgery by us from January 1, 1998, through December 31, 2001. Medical records were reviewed for hospitalization status postoperatively. Medical records of those patients treated on an outpatient basis were reviewed for patient and family comments about ease of use of the pain-control catheter and the degree of pain control and for any complications associated with catheter use. The main outcome measures included documented patient and family comments and physician medical record notes about catheter use and complications. RESULTS: Of the 85 patients, 67 were treated on an outpatient basis. The other 18 patients required a postoperative hospital admission for unassociated medical problems. Of the 67 patients, 58 (87%) reported using the catheter at home at least once. Of these 58 patients, 10 reported mild discomfort with catheter use, but in no case did the patient discontinue catheter use because of discomfort. All patients using the catheter reported pain relief lasting from 1(1/2) to 4 hours. No postoperative complications associated with catheter placement were observed. CONCLUSION: The orbital pain-control catheter allows a caregiver to easily and repeatedly deliver local anesthetic to the operative site following enucleation, resulting in effective postoperative analgesia while the patient recovers at home.

LASIK, blepharoplasty, and dry eyes.

Because LASIK and blepharoplasty both may cause dry eye symptoms, the possible cumulative effect of these surgeries is a serious consideration. The author discusses the physiological mechanisms in these procedures that can lead to dry eye and provides practical guidelines for evaluating patients. (Aesthetic Surg J 2002;22:382-383.).

Primary mucinous carcinoma of the periocular region: successful management with local resections over 30 years.

Primary mucinous carcinoma of the skin is a rare malignant neoplasm, often with periocular involvement, believed to originate from eccrine sweat glands. It is slow growing and locally destructive, at times forming tumour satellites. We present a case with six local recurrences treated with surgical resections over a period of 30 years. We have not been able to find longer follow-up in the literature, and believe this case may offer insight into the management of these uniquely indolent malignancies.

Total face, double jaw, and tongue transplantation: an evolutionary concept.

BACKGROUND: The central face high-energy avulsive injury has been frequently encountered and predictably managed at the R Adams Cowley Shock Trauma Center. However, despite significant surgical advances and multiple surgical procedures, the ultimate outcome continues to reveal an inanimate, insensate, and suboptimal aesthetic result. METHODS: To effectively address this challenging deformity, a comprehensive multidisciplinary approach was devised. The strategy involved the foundation of a basic science laboratory, the cultivation of a supportive institutional clinical environment, the innovative application of technologies, cadaveric simulations, a real-time clinical rehearsal, and an informed and willing recipient who had the characteristic deformity. RESULTS: After institutional review board and organ procurement organization approval, a total face, double jaw, and tongue transplantation was performed on a 37-year-old man with a central face high-energy avulsive ballistic injury. CONCLUSIONS: This facial transplant represents the most comprehensive transplant performed to date. Through a systematic approach and clinical adherence to fundamental principles of aesthetic surgery, craniofacial surgery, and microsurgery and the innovative application of technologies, restoration of human appearance and function for individuals with a devastating composite disfigurement is now a reality. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Secondary infection affecting one of two simultaneously placed orbital wall implants.

A 10-year-old boy experienced traumatic injuries to the right bony orbit, which were repaired with a nylon foil and a barrier porous polyethylene orbital implant. Three years after surgery, he presented with maxillary and ethmoid sinusitis with infection of the nylon foil plate. This plate was removed, yielding complete recovery.

Smooth nylon foil (SupraFOIL) orbital implants in orbital fractures: a case series of 181 patients.

PURPOSE: The SupraFOIL smooth nylon foil implant is a polyamide sheet available in varying thicknesses that has been used in orbital reconstruction following trauma. The authors report their experience with smooth nylon foil implants in the repair of orbital fractures. METHODS: A retrospective chart review of patients having undergone repair of orbital fractures between January 1, 1995 and December 31, 2003 was conducted. The Wilcoxon test was used to compare cases with complications and those with no complications. RESULTS: Of 282 orbits that underwent fracture repair with alloplastic material, 87 were excluded because non-nylon foil implants were used alone or in combination with nylon foil to repair the orbital fracture. An additional 14 fractures were excluded because of insufficient clinical data or lack of follow-up, for a total of 181 orbits that underwent orbital fracture repair with only nylon foil. A transconjunctival approach was used in 98% cases, and all 181 implants were fixated with at least 1 titanium screw. Average follow-up was 362.8 days. The overall complication rate was 1.7%. One patient had an acute postoperative orbital hemorrhage (0.6% acute complication rate). Two patients had late orbital infections, 683 days and 984 days following repair (1.1% late complication rate). CONCLUSIONS: The authors found the smooth nylon foil implant to be safe and effective in orbital fracture repair. The lower rate of complications the authors observed compared with previously reported series may be related to implant fixation. A titanium screw secured just posterior to the orbital rim may decrease long-term complications by providing more stability than implants left without fixation.

Use of porous polyethylene with embedded titanium in orbital reconstruction: a review of 106 patients.

PURPOSE: To evaluate our initial experience with a porous polyethylene implant with embedded titanium in orbital reconstruction. METHODS: A retrospective review of the charts was performed for patients receiving at least 1 orbital porous polyethylene implant with embedded titanium from October 2004 through April 2006. Patient demographics, implant type and size, method of fixation, postoperative complications, and length of follow-up were recorded. RESULTS: One-hundred six patients received at least one embedded titanium implant (80 men, 26 women). Age ranged from 3 years to 85 years (mean, 31 years). Indications included orbital fracture repair (102 patients), correction of enophthalmos (3 patients), and reconstruction after tumor resection (1 patient). The average floor implant used was 22.3 mm wide by 27.9 mm long. The average medial wall implant was 13.6 mm tall by 22 mm long. Implants were fixated with 1 or 2 microscrews in 75 patients (70.8%) and placed without fixation in 31 patients (29.2%). Four postoperative complications were noted: a retrobulbar hemorrhage on postoperative day 3 that resolved without sequelae, a vertical overcorrection requiring removal of the implant, a transient oculomotor disturbance, and a screw placed in proximity to the infraorbital nerve canal resulting in hypesthesia and chronic pain that required removal of the screw. In the 3 complications potentially related to the implant, the embedded titanium allowed postoperative implant visualization and guided subsequent management. No implant extrusions or postoperative infections were noted. CONCLUSIONS: Porous polyethylene implants with embedded titanium provide a new alternative to alloplastic implant materials for orbital reconstruction with a profile that combines several advantages of porous polyethylene and titanium implants.

Effect of 0.5% apraclonidine on ptosis in Horner syndrome.

To demonstrate the effect of apraclonidine on anisocoria and ptosis in Horner syndrome, one drop of 0.5% apraclonidine was instilled in both eyes of 3 patients who presented with acute Horner syndrome, and the effect on ptosis and anisocoria was documented. As reported in the literature, one drop of 0.5% apraclonidine reverses the anisocoria of Horner syndrome. In addition, 0.5% apraclonidine leads to a complete resolution of the ptosis associated with Horner syndrome, a finding reported once in the literature. Apraclonidine is a safe and readily available alternative to cocaine for the diagnosis of Horner syndrome.

New corticosteroid-eluting porous polyethylene implant for the management of lower eyelid retraction: a pilot study.

PURPOSE: Lower eyelid retraction after trauma presents a challenging management problem. We postulated that a porous polyethylene (pPE) eyelid spacer coated with a polyvinyl alcohol (PVA) and triamcinolone acetonide (TA) matrix could deliver corticosteroid locally over extended periods and modulate inflammation and scar formation. We designed a pPE corticosteroid-eluting implant and evaluated its characteristics in vitro and in vivo. METHODS: The release characteristics of pPE implants coated with a PVA/TA matrix of low, intermediate, and high doses of TA were studied in vitro. The implants were then placed in the posterior lamella of lower eyelids of Dutch Belted rabbits for 12 weeks. Clinical events were recorded and eyelids were examined for gross and histologic features, including capsular thickness and degree of vascularity, fibrovascular ingrowth, and inflammatory response. RESULTS: In vitro, implants coated with the intermediate and high doses of TA released the drug at a steady rate for at least 78 days. In rabbits, the PVA and PVA/TA coating prevented fibrovascular ingrowth, except where breaks in the PVA/TA coat were present. Implants with PVA/TA coating demonstrated less inflammation and capsule vascularity. An inverse correlation between TA dose and capsule thickness was noted. CONCLUSIONS: We describe a novel drug-release pPE eyelid implant. The corticosteroid-eluting implant demonstrated antiangiogenic and anti-inflammatory properties, which could prove beneficial in the treatment of lower eyelid retraction.