Functional Brain Imaging During Extra-Ocular Light Stimulation in Anophthalmic and Sighted Participants: No Evidence for Extra-Ocular Photosensitive Receptors.

Light plays a critical role in regulating physiology and behavior, including both visual and non-visual responses. In mammals, loss of both eyes abolishes all of these responses, demonstrating that the photoreceptors involved are exclusively ocular. By contrast, many non-mammalian species possess extra-ocular photoreceptors located in the pineal complex and deep brain. Whilst there have been suggestions of extra-ocular photoreception in mammals, including man, evidence for these photoreceptors is limited. One approach to objectively determine the presence of such receptors is to measure brain responses to light using functional magnetic resonance imaging (fMRI). Moreover, by using participants who are clinically anophthalmic (congenital and acquired), it is possible to investigate potential light detection in the absence of the retina. Here we scanned participants with anophthalmia and sighted participants in 4 different conditions; the first 3 conditions had a bright light source applied to the following locations: behind the right ear ("ear"), just below the nasal bridge and between the eyes ("head"), and at the right popliteal fossa ("knee"). In the fourth and final scan, the light source was switched off so that there was no light stimulus. All participants were scanned in a completely dark room. No consistent brain activity was detected during any of the light conditions in either sighted controls or anophthalmic participants. Thus, we do not provide any evidence for the presence of extraocular photoreceptors modulating human brain activity, despite recent evidence for gene transcription that may occur as a result of these photoreceptors.

The Effect of Congenital and Acquired Bilateral Anophthalmia on Brain Structure.

The aim of this study was to compare the pattern of changes in brain structure resulting from congenital and acquired bilateral anophthalmia. Brain structure was investigated using 3T magnetic resonance imaging (MRI) in Oxford (congenital) or Manchester (acquired). T1-weighted structural and diffusion-weighted scans were acquired from people with anophthalmia and sighted control participants. Differences in grey matter between the groups were quantified using voxel-based morphometry and differences in white matter microstructure using tract-based spatial statistics. Quantification of optic nerve volume and cortical thickness in visual regions was also performed in all groups. The optic nerve was reduced in volume in both anophthalmic populations, but to a greater extent in the congenital group and anophthalmia acquired at an early age. A similar pattern was found for the white matter microstructure throughout the occipitotemporal regions of the brain, suggesting a greater reduction of integrity with increasing duration of anophthalmia. In contrast, grey matter volume changes differed between the two groups, with the acquired anophthalmia group showing a decrease in the calcarine sulcus, corresponding to the area that would have been peripheral primary visual cortex. In contrast, the acquired anophthalmia group showed a decrease in grey matter volume in the calcarine sulcus corresponding to the area that would have been peripheral primary visual cortex. There are both qualitative and quantitative differences in structural brain changes in congenital and acquired anophthalmia, indicating differential effects of development and degeneration.

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.

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.

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.

Management of cicatricial ectropion secondary to pyoderma gangrenosum.

Pyoderma gangrenosum is a rare condition with few cases of eyelid involvement reported in the literature. Pathergy is a well-recognised phenomenon that can trigger this condition. Pyoderma gangrenosum should be considered in cases of progressive cribriform cicatrisation where there is a history of antecedent trauma. Surgical management of a resultant ectropion may be challenging as a result of aggressive scarring and the risk of provoking a recurrence. We report a case of pyoderma gangrenosum causing a cicatricial ectropion, and discuss the underlying aetiology of iatrogenic incitement, and its implications for surgical management.

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.

A reliable surgical technique for the management of growing fractures of the frontal bone involving the orbit.

PURPOSE: Growing fractures involving the orbit occur infrequently. Due to the risk of neurological trauma and the ophthalmic sequelae of progressive intraorbital expansion of the fracture, surgical management is indicated. There is no accepted standard in the approach to the surgical management of these patients. The aim of this paper is to describe a reliable technique for the repair of growing fractures and discuss the long-term outcome. METHODS: We present two cases in which a technique using Norian CRS® (Craniofacial Repair System) fast set putty over a Medpor® scaffold was used to repair a growing fracture. RESULTS: This technique provides a secure repair with a good functional and cosmetic outcome. CONCLUSIONS: Growing fractures occur in the paediatric population so it is important to ensure that the repair does not restrict or adversely affect future bone growth. One case has been followed-up for over 10 years and has maintained normal growth.

A case of a spontaneous intraorbital arteriovenous fistula: clinico-radiological findings and treatment by transvenous embolisation via the superior ophthalmic vein.

A 72-year-old male presented with progressive right axial proptosis and red eye. Catheter angiography demonstrated an intraorbital arteriovenous fistula (IAVF) distal to the central retinal artery (CRA). Transvenous embolisation following direct surgical exposure of the superior ophthalmic vein (SOV) resulted in rapid resolution of his symptoms and signs. Transvenous embolisation via the SOV is a safe, effective alternative to transarterial embolisation for treating spontaneous IAVF where transarterial embolisation poses a risk of CRA occlusion.

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.

Acute dacryocystitis in a 2-year old child caused by pantoea.

A previously healthy 23-month-old girl was admitted for the management of an acute unilateral dacryocystitis following accidental contact with dog faeces. No periocular trauma was reported. Microbiological investigation showed a multiresistant strain of Pantoea species to be the responsible pathogen. The infection responded to a course of oral Clindamycin and Ciprofloxacin, in combination with Chloramphenicol eye drops. This is the first report of an acute dacryocystitis sustained by this microorganism.

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.