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.

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.

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.

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.

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.

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.

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.

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 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.

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.

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.

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.

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.

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.

Difference in response to botulinum toxin type A treatment between patients with benign essential blepharospasm and hemifacial spasm.

BACKGROUND: Botulinum toxin (BTX) is the first-line treatment in managing benign essential blepharospasm (BEB) and hemifacial spasm (HFS). We wished to assess the difference in duration of effect and the number of BTX treatments required to treat patients with BEB and HFS. METHODS: A prospective study of patients attending the BTX clinic in Manchester Royal Eye Hospital over 6 months. All treatments were administered by a single experienced ophthalmologist. A questionnaire was completed for each patient. In patients with BEB where the BTX was injected bilaterally, one side was randomized to compare with HFS patients. Patient demographics, cumulative dose of BTX, duration of BTX effect with patient satisfaction and the number of previous BTX injections were recorded. RESULTS: Sixty-four patients were included in the study. The mean age was 60.8 years. Among them, 30 patients had BEB and 34 had HFS. Patients with HFS received a lower mean dose of BTX than patients with BEB (12.23 units vs. 16.2 units). The patients with HFS had a longer duration of effect than patients with BEB, with fewer BTX treatments. Of all patients, 90% with HFS and BEB were satisfied with the effect of their last BTX injection. Three unsatisfied patients in the BEB group were referred on for surgical management of their disorder. CONCLUSIONS: We have shown that patients with BEB have a shorter duration of effect with BTX and require more frequent BTX treatments than patients with HFS, highlighting that facial dystonias in patients with BEB is more challenging to manage.

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.

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.