The Post-Levator Aponeurosis Fat Pad.

PURPOSE: To examine the post-aponeurotic space and to confirm and define the presence of a post-aponeurosis fat-pad. METHODS: Experimental anatomic study. Nineteen-orbits from 10 freeze-preserved, unembalmed cadavers of caucasian subjects. In 12 orbits of 7 cadavers, a transconjunctival dissection of the everted upper eyelid was undertaken. Müller's muscle (MM) and conjunctiva were dissected as a composite flap exposing the posterior surface of the aponeurosis (LA) and the commencement of the levator palpebrae superioris (LPS) muscle. Anatomical localisation was agreed by 2 senior surgeons and an anatomist (VM). In the remaining 7 orbits a 1cm central upper eyelid wedge-excision was paraffin-embedded and studied histologically. RESULTS: Nineteen upper-eyelids from 10 freeze-preserved, unembalmed caucasian cadavers (5-male, 5-female, mean age 80.9; range 67-91 years) were studied. Of 12 eyelids of 7 cadavers, dissected and macroscopically evaluated, a fat-pad was identified in the post-aponeurotic space of all eyelids. Of these, 8 (66%) were predominantly diffuse. The remainder, mixed diffuse-discrete. All 4 of the latter category appeared multi-lobular. The fat-pad was seen to lie predominantly centro-medially, overlying MM, extending superiorly beyond the LA to lie posterior to LPS. Of the 7 upper eyelid wedge-excisions examined microscopically, a fat-pad was identified in all post-aponeurotic spaces, lying between 2 distinct tracts of smooth muscle. The anterior smooth muscle tract was intimately related to the posterior aspect of the LA, in keeping with the posterior smooth muscle layer of the aponeurosis. The posterior smooth muscle tract was in keeping with MM, thicker than the anterior layer, multi-layered and in 6 of 7 eyelids, interspersed with fat. CONCLUSIONS: We confirm and describe a distinct layer of fat in the post-aponeurotic space, consistently found between MM and the posterior smooth muscle layer of the aponeurosis. We refer to this as the post-aponeurosis fat-pad. These findings provide further anatomical detail for the surgeon undertaking blepharoptosis surgery, who may, in some cases, mistake the presence of fat in this space either for the pre-aponeurotic fat-pad, or for degenerative changes within MM that lies deep to it.

Blepharoptosis surgery in patients with myasthenia gravis.

BACKGROUND/AIMS: To review our approach of cautious surgical correction of blepharoptosis in patients with myasthenia gravis (MG) to minimise risk of exposure complications. METHODS: Retrospective case note review of 30 patients with symptomatic eyelid concerns despite appropriate medical treatment, who underwent eyelid surgery. The mean age at diagnosis was 47 years. 13/30 patients had systemic MG, 14/30 ocular MG and 3/30 congenital MG. The main outcome measures were improvement in eyelid height and/or position, duration of a successful postoperative result, need for further surgical intervention, and intraoperative or postoperative complications. RESULTS: 38 blepharoptosis procedures were performed on 23 patients. Mean age at time of surgery was 62 years, with an average follow-up of 29 months. 10 patients (16 eyelids) underwent anterior approach levator advancement, 4 patients (5 eyelids) posterior approach surgery and 8 patients (15 eyelids) brow suspension. One patient (2 eyelids) had tarsal switch surgery. An average improvement in eyelid height of 1.9 mm was achieved. Postoperative symptoms or signs of exposure keratopathy occurred in 17% of patients. This necessitated lid lowering in one eyelid of one patient. During follow-up, 37% of eyelids required further surgical intervention to improve the upper eyelid height, after an average of 19 months (range 0.5-49 months). CONCLUSIONS: Over a third of patients in our series required repeat surgery, which would be expected when the initial aim was to under-correct this group. In contrast to previous commentaries, the amount of eyelid excursion was not the main factor used to guide the surgical approach.

Periocular Scleromyxedema.

Scleromyxedema is characterized by cutaneous mucinosis and rarely presents to oculoplastic surgeons with bilateral upper and lower eyelid swelling. The authors present 2 case reports with a review of the literature and discuss the ophthalmic manifestations of scleromyxedema and the management of these cases. Both patients were in their early 50s and were fit and well prior to the presentation. They both presented with eyelid swelling and underlying nodularity of the subcutaneous tissue. The skin biopsy confirmed cutaneous manifestations of scleromyxedema. The systemic manifestations responded to intravenous and oral steroids and intravenous infusions of immunoglobulin. The ophthalmic manifestations were managed conservatively. Scleromyxedema is a rare connective tissue disease characterized by cutaneous mucinosis, extracutaneous manifestations, and monoclonal gammopathy. It rarely affects the eyelids and cornea. The authors would like to present 2 cases of periocular scleromyxedema.

Bisphosphonate-induced osteonecrosis of the maxilla presenting as a cicatricial ectropion.

CASE REPORT: A 63-year-old gentleman, who was being treated with bisphosphonates for multiple myeloma, presented with a cicatricial ectropion of the lower eyelid, without exposure keratopathy. A CT scan demonstrated extensive destruction of bone with an infraorbital fracture surrounded by sclerotic bony changes. The patient was managed conservatively with discontinuation of bisphosphonate therapy and topical ocular lubricants. The patient's condition remained unchanged a year after this initial management.

Osteoradionecrosis of the exenterated orbit.

Osteoradionecrosis (ORN) occurs in an estimated 2% of head and neck-irradiated patients. It is seen most commonly in the mandible with other reported sites including the maxilla, temporal bone, clavicle, and vertebrae. It is defined as an area of exposed devitalised irradiated bone, with failure to heal during a period of at least 3 months, in the absence of local neoplastic disease. We report 2 cases of ORN following postoperative radiotherapy given to patients who had undergone an orbital exenteration. ORN can develop spontaneously in one-third of cases, although in the majority of patients, it is induced by secondary trauma. Radiotherapy induces an endarteritis in the small blood vessels of bone, thus favouring the generation of small thrombi that obliterate the vascular lumen and interrupt tissue perfusion. Likewise, irradiation impairs the function of osteoblasts, manifesting as osteopenia, with impairment of the repair and remodelling capacity of bone. Prior radiation exposure can thus decrease bone vascularity and injure its reserve reparative capacity. It is important to differentiate ORN from local recurrence of malignancy, bone metastasis, radiation-induced sarcoma, and infection. CT and MRI are effective diagnostic tools. Clinical management of ORN is complex and unsatisfactory. Treatment remains difficult, and prevention is paramount. A history of radiotherapy should alert clinicians to detect bone exposure or excessively prolonged socket healing. Early diagnosis with a high index of suspicion can achieve higher control rates with conservative management. Our case series reports a rare, previously unreported, but important complication of radiation therapy of the exenterated orbit.

Merkel cell carcinoma of the eyelid: management and prognosis.

IMPORTANCE: The literature on Merkel cell carcinoma (MCC) of the eyelid remains scarce, and there has yet to be a study using the most up-to-date TNM staging system for this rare but aggressive tumor. OBJECTIVE: To analyze the TNM stage, management, and outcomes of patients with MCC of the eyelid. DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series of 21 patients from 5 tertiary referral centers in the United Kingdom and Australia with primary MCC of the eyelid presenting at a median age of 77 years, with median follow-up of 54 months. Tumors were staged according to the American Joint Committee on Cancer, 7th edition, TNM criteria for eyelid carcinoma and MCC. MAIN OUTCOMES AND MEASURES: TNM stage, treatment modalities, and clinical outcome. RESULTS: The eyelid carcinoma TNM stages were T2aN0M0 for 5 patients, T2bN0M0 for 7 patients, T3aN0M0 for 4 patients, T3bN0M0 for 3 patients, T2bN1M0 for 1 patient, and T3aN1M0 for 1 patient. The MCC TNM stages were T1N0M0 for 12 patients, T2N0M0 for 7 patients, T1N1M0 for 1 patient, and T2N1M0 for 1 patient. One patient had a sentinel lymph node biopsy, and 8 patients underwent head/neck imaging. Eighteen patients underwent a wide local excision, 12 with a paraffin section and 6 with a frozen section. Two patients underwent Mohs surgery, 1 of whom required an orbital exenteration. Twelve patients (57%) received adjuvant radiotherapy, and 2 patients received chemotherapy. The local recurrence rate was 10%, the regional nodal recurrence rate was 10%, and the distant metastatic recurrence rate was 19%. The lowest T category tumor metastasizing to both regional nodes and distant locations was a T2a (eyelid TNM)/T1 (Merkel TNM) tumor measuring 8 mm. Two patients with T3a (eyelid TNM)/T2 (Merkel TNM) tumors died of metastatic MCC. CONCLUSIONS AND RELEVANCE: The majority of patients with MCC of the eyelid present with localized eyelid disease of T category T2 (eyelid TNM)/T1 (Merkel TNM). A wide local excision with margin control remains the mainstay of treatment, whereas the use of radiotherapy is institution specific. Tumors with a low T category are associated with regional nodal and distant metastatic disease. It may therefore be reasonable to consider a sentinel lymph node biopsy or strict regional lymph node surveillance for all MCCs of the eyelid, regardless of T category or size.

Complications of blepharoplasty.

This article provides a comprehensive discussion on the complications of blepharoplasty. We discuss the importance of preoperative counselling and surgical planning to provide the patient with a satisfactory outcome. Strategies are presented to manage common complications.

Hyaluronidase toxicity: a possible cause of postoperative periorbital inflammation.

BACKGROUND: Periorbital inflammation following regional anaesthesia is commonly attributed to hyaluronidase allergy. This case series suggests an alternative explanation in some patients. DESIGN: Retrospective case series. PARTICIPANTS: Seven patients presenting with postoperative non-infectious periorbital inflammation following peribulbar or sub-tenons anaesthesia, presenting at four different institutions, are described. METHODS: Data on patient demographics, operative data, clinical presentation, treatment and allergy testing were collected among the four institutions. MAIN OUTCOME MEASURES: Response to treatment and allergy testing were noted among the patients included in this study. RESULTS: Seven patients (five female) underwent uneventful phacoemulsification under a peribulbar or sub-tenon's block, all including hyaluronidase with concentrations ranging 50-250 IU/mL. The onset of inflammatory symptoms and signs varied from 12 h to 3 days after the surgery. The most common form of presentation was lid swelling and chemosis. Patients were treated with oral corticosteroids, with good clinical response. Four patients underwent skin prick and intradermal testing to the local anaesthetic used, and to the suspect and a control hyaluronidase batch. The results were all negative, excluding allergy as the aetiology of this toxic periorbital syndrome, in at least these four patients. CONCLUSION: Hyaluronidase toxicity, potentially related to concentration of hyaluronidase, may be a cause of postoperative periorbital inflammation after cataract surgery, rather than hypersensitivity.

Amyloidosis of the orbit and adnexae.

PURPOSE: To present a series of patients with orbital and adnexal amyloidosis and illustrate the diversity of disease and the challenges of managing such cases. METHODS: Descriptive case series of ten patients with biopsy proven amyloidosis involving the orbit, conjunctiva and eyelids. The presentation, clinical findings and management are discussed for each case. RESULTS: All patients had some form of eyelid abnormality or malposition. Presenting complaints included ptosis, epiphora and ocular discomfort. Other clinical findings included conjunctival lesions and proptosis. The majority of patients had localised amyloidosis and one patient had systemic disease. Conservative management included lubrication and the use of bandage contact lenses. Surgical management included debulking, ptosis or other lid surgery. CONCLUSION: Amyloidosis can present to an Occuloplastic clinic in a wide variety of ways. Definitive diagnosis is based on the histopathological findings. Management is often challenging. Multi-disciplinary team involvement is critical in view of its systemic associations.

Orbital lymphangiomas: a review of management strategies.

PURPOSE OF REVIEW: Orbital lymphangiomas present a difficult management problem for the ophthalmologist. This review offers a strategy for managing the condition. RECENT FINDINGS: There have been recent publications discussing the use of intralesional fibrin glue to aid dissection and intralesional sclerosant to shrink the lesion. We discuss the evidence for these treatments and present a case in which these treatments were combined. SUMMARY: This review highlights the classification and presents management options for orbital lymphangioma. We encourage a holistic and individualized approach to the patients, recognizing the variety of clinical manifestations that the condition can cause. In the event of surgical excision, we present the evidence for the use of intralesional fibrin glue and intralesional sclerosant, and describe using both in the same case.

Eyelid avulsion repair with bi-canalicular silicone stenting without medial canthal tendon reconstruction.

AIMS: To evaluate the functional and cosmetic success rate of combined canalicular laceration and eyelid avulsion repairs using the bi-canalicular Crawford stent, without the concomitant placement of a medial traction suture to repair medial canthal tendon (posterior limb) avulsion. METHODS: Retrospective, non-comparative consecutive case series of 40 consecutive patients with traumatic eyelid avulsion injuries associated with canalicular laceration from 1997 to 2003 who underwent surgical repair using the bi-canalicular Crawford stent were included. All patients underwent surgical repair of the canalicular laceration under general anaesthesia using the bi-canalicular stent. Meticulous anastomosis of the torn canaliculus was undertaken. No attempt was made to suture the avulsed medial canthal tendon (posterior limb) to the periosteum of the posterior lacrimal crest. RESULTS: Blunt trauma was the most common mechanism of injury and the inferior canaliculus was most commonly involved. Of the 37 patients who attended postoperative follow-up, 24 patients had no subjective symptoms of epiphora. Minimal, mild and moderate epiphora was present in seven, five and one patient respectively. Thirty-three patients had excellent cosmetic repositioning of the lid; two developed medial ectropia and a further two patients had lid margin notching but good lid position. Eight patients had premature stent loss. CONCLUSIONS: Bi-canalicular stenting achieved excellent cosmetic results in eyelid avulsion injuries, by facilitating adequate tissue realignment without the need for a posterior lacrimal crest fixation suture. Good functional results were achieved and were comparable with previous studies.

The use of soft tissue expanders in periocular reconstruction: a case series.

PURPOSE: To describe the use of soft tissue expanders for periocular reconstruction in the presence of limited local tissue availability. METHODS: A retrospective case series of 5 patients who attended the Manchester Royal Eye Hospital. They presented a reconstructive challenge due either to the presence of periocular scarring or to a marked loss or limitation of normal local skin for reconstruction. The soft tissue expander (SOE-3624 device) was inserted under the skin and subcutaneous tissue at a site selected to provide the desired additional tissue. Over periods of time varying from 6 to 16 weeks, the soft tissue expander was gradually inflated with injections of sterile saline in the outpatient clinic. As soon as the desired amount of additional skin and subcutaneous tissue had been obtained, reconstructive surgery was undertaken. Functional and cosmetic results were the main outcomes of the intervention. RESULTS: Four of the patients had the device inserted in the lower eyelid or cheek area and one patient had the device inserted above the eyebrow. No episode of tissue necrosis occurred in our patients. All the devices were tolerated by the patients and were removed without any complications. The implant migrated in one patient, limiting the reconstructive surgery, and one patient had a lateral canthal dehiscence following reconstruction. All patients thought that the surgery had improved their cosmesis. No patient required any additional surgical procedures. CONCLUSIONS: The authors conclude that the SOE-3624 tissue expander represents a good option in providing additional tissue for periocular reconstruction, particularly in younger patients. It was well tolerated and effective in this series.

A case of acute compressive optic neuropathy secondary to aneurysmal bone cyst formation in fibrous dysplasia.

We report a 19-year-old female with craniofacial fibrous dysplasia who had presented with rapidly progressive visual loss in her right eye secondary to aneurysmal bone cyst formation. Craniotomy with drainage and curettage of this bone cyst resulted in progressive improvement in visual acuity that returned to normal 5 months post-operatively.

The surgical management and outcomes for spheno-orbital meningiomas: a 7-year review of multi-disciplinary practice.

AIMS/PURPOSE: Spheno-orbital meningiomas account for 9% of all adult intracranial meningiomas. Complete resection is extremely difficult with this condition. We report on our experience in the surgical management of spheno-orbital meningiomas. METHODS: A retrospective review was performed of all patients with spheno-orbital meningiomas who underwent joint neurosurgical and ophthalmic procedures between January 2000 and December 2007. Radiological findings, presenting clinical signs, indications for surgery, surgical approach, histopathological findings, surgical complications and post-operative results were recorded. RESULTS: Twelve patients were included in the study. Visual function deterioration was the main indication for surgery. Six patients underwent an optic canal decompression along with their initial tumour resection and all 12 patients underwent an extensive lateral orbital wall decompression. Post-operatively 6 patients had reduced proptosis. Two patients had an improvement in their visual acuity, 5 patients had a stable visual acuity and 5 patients had a progressive deterioration in visual acuity following surgery. Six patients maintained a stable visual field. Cranial nerve palsy was the commonest post-operative complication. Three patients required postoperative fractionated radiotherapy. Three patients required further surgery. CONCLUSION: Spheno-orbital meningiomas are difficult tumours to manage. Surgical resection can reduce the degree of proptosis and stabilise visual function in patients with failing vision, although sustained improvement is difficult to achieve if the tumour behaves in an aggressive manner. The risk of post-operative visual loss is considerable, either due to surgery or tumour progression. Outcomes from surgical decompression may not necessarily be better than the natural history of these tumours.

Epithelial inclusion cyst associated with a porous polyethylene orbital floor implant.

A 50-year-old man presented with reduced vision, hyperglobus, and an inferior orbital mass 3(1/2) years after the repair of an orbital floor fracture using a porous polyethylene (Medpor) implant. On funduscopy, the sclera was markedly indented. This was associated with retinal striae and a retinal pigment epithelial disturbance involving the macula. CT revealed a large inferior orbital cystic mass displacing the globe. The cyst was excised and the orbital implant was removed. Histologic findings were consistent with a respiratory epithelial inclusion cyst. Postoperatively, the vision improved and the hyperglobus resolved. The retinal striae and pigmentary disturbance persisted. Late capsule-related complications, such as epithelial cyst formation and intracapsular hemorrhage, have been reported in association with nonporous materials only. This case demonstrates that epithelial cyst formation may occur in association with a porous orbital floor implant and result in visual impairment.

A novel approach to the management of severe facial disfigurement in neurofibromatosis type 1.

A 12-year-old girl with neurofibromatosis type 1 underwent subtotal anterior exenteration of a blind, unsightly, buphthalmic eye and orbital/eyelid plexiform neurofibroma. Simultaneously, a channeled Medpor orbital implant was positioned to reduce a meningoencephalocele resulting from severe sphenoid wing dysgenesis. Two-stage osseointegration was subsequently performed to permit secure fitting of an orbital prosthesis. This patient's cosmesis and social interaction were improved markedly with this surgical approach for disfiguring ocular, eyelid, and orbital disease due to neurofibromatosis type 1.

Use of the pericranial flap in medial canthal reconstruction: another application for this versatile flap.

PURPOSE: To describe the use and outcomes of a versatile surgical technique in the reconstruction of deep soft tissue and bony defects of the medial canthus. METHODS: A retrospective review of consecutive cases requiring reconstruction of medial canthal defects involving loss of periosteum or bone by a median forehead pericranial flap and full-thickness skin grafting in a tertiary referral hospital setting. Two techniques were used: an open technique, using a midline forehead incision; and an endoscopic technique, using 2 incisions behind the hairline. RESULTS: Twenty-one cases were identified: 19 open and 2 endoscopic. The average length of follow-up was 13 months (range, 6-50 months). Ten cases required additional oculoplastic procedures including local periosteal flaps and mucous membrane grafts. Two cases (10%) had complete flap failure; one of these was caused by infection. Five (24%) had partial (< 50%) skin graft necrosis. Two cases (10%) have required further surgery. CONCLUSIONS: Our experience shows the pericranial flap to be versatile, robust, and easy to manipulate, offering advantages over alternative techniques when used for the repair of deep medial canthal defects. It is a valuable reconstructive technique that can yield good cosmetic and functional results.

Review of patients with basal cell nevus syndrome.

PURPOSE: To review patients with basal cell nevus syndrome (BCNS), documenting presentation, referrals, treatment patterns, and associated morbidity. METHODS: Cross-sectional review and retrospective data collection of 39 patients with BCNS. Patients from the BCNS support group were invited to be examined. Demographics, presenting features, associated pathologies, and treatment modalities were recorded. Demographic data, age at presentation, age at diagnosis, spectrum of ophthalmic and periocular disease, treatment modalities used, and periocular deformities developed were reviewed. RESULTS: Thirty-nine patients were included with age range of 5 to 72 years. Presenting clinical features included odontogenic keratocyst in 17 patients and basal cell carcinoma in 13 patients; less common presentations were with congenital malformations (n = 2), with ophthalmic associations (n = 3), and at genetic counseling (n = 4). Seventeen of the 39 patients confirmed a parental diagnosis of BCNS. Basal cell carcinoma developed in 18 of the 28 patients before the age of 30, confirming the reported early age of onset. Periocular basal cell carcinoma was reported in 24 of 39 patients (61%), with recurrent disease reported in 17 of these 24 (71%), despite a variety of treatment modalities used. Associated ophthalmic features were multiple eyelid cysts (15 patients), strabismus (9 patients), myopia (5 patients), hyperopia (7 patients), cataracts (5 patients), myelinated nerve fibers (3 patients), amblyopia (3 patients), and nystagmus and iris transillumination defects (2 patients each). All patients were involved in multidisciplinary medical care. CONCLUSIONS: Patients with BCNS frequently have ophthalmic manifestations, particularly periocular basal cell carcinoma. Multidisciplinary care is essential in the care of the patient with BCNS. Early diagnosis of BCNS may allow for skin protection and surveillance at an earlier age.