Thomas G. Hardy

Orbital volume augmentation with autologous micro-fat grafts.

Purpose: The purpose of this study is to present an alternative procedure for rehabilitation of the volume deficient anophthalmic or enophthalmic socket. Methods: A retrospective review of clinical and photographic records of 12 patients with either an anophthalmic or enophthalmic orbit (14 orbits) undergoing volume augmentation by micro-fat grafting, or lipostructure, as initially described by Coleman. Patients with orbital volume deficiency seen in the oculoplastic clinic at Chelsea and Westminster Hospital, London, UK, were invited to participate in the study. The technique is discussed in detail. Results: The volume of fat injected ranged from 0.8 mL to 4.5 mL (median, 3.05 mL) per orbit, with a median increase in exophthalmometry measurements (available in 9 patients) of 2 mm (range, 0–7.5 mm). Subjective improvement in cosmetic outcome was experienced in all patients. Repeat grafting was required in 1 patient, and will be required in another patient. There were no embolic complications. The procedure was well tolerated in all patients. Median follow-up was 14.5 months (range,12–30 months). Conclusions: Micro-fat grafting to the anophthalmic or enophthalmic socket appears to be a safe alternative technique for orbital volume enhancement. It has the advantages of avoiding alloplastic infectious complications, ease of technique, minimal donor site morbidity, acceptable graft take rate, low embolic complication rate, and good cosmetic outcome.

Enlargement of the Infraorbital Nerve: An Important Sign Associated with Orbital Reactive Lymphoid Hyperplasia or Immunoglobulin G4–Related Disease

OBJECTIVE To describe the clinical, histopathologic, and radiologic features of a recently identified cause for enlargement of the infraorbital canal. DESIGN Retrospective, noncomparative case series. PARTICIPANTS Consecutive patients were identified from the orbital databases at Moorfields Eye Hospital, London, England, and the Royal Victorian Eye and Ear Hospital, Melbourne, Australia. METHODS A retrospective, noncomparative review of the clinical case notes, radiology, and histopathology was performed. The English-language medical literature was reviewed for reports of enlargement of the infraorbital canal or nerve. MAIN OUTCOME MEASURES Extent of clinical and radiologic changes in patients with enlargement of the infraorbital canal. RESULTS A total of 14 patients (10 male) presented between the ages of 29 and 76 years with proptosis, eyelid swelling or a mass (10/14 cases), and periocular ache (5/14 cases). Clinical evidence of bilateral involvement was present in 6 of 14 patients. None had impairment of visual functions or facial sensation, but 4 of 14 patients had some reduction in ocular motility. Imaging showed a focal orbital mass in 10 of 14 patients (16/28 orbits), and all patients (22/28 orbits) had enlargement of some extraocular muscles. The infraorbital canal was enlarged in 20 of the 28 orbits, with associated ipsilateral orbital changes in 19 of 20 (all 14 patients) and ipsilateral maxillary sinus changes in 12 of 20 (11 patients). Biopsy-proven chronic orbital inflammation was present in all patients; this resembled reactive lymphoid hyperplasia (RLH) in 7 patients and immunoglobulin (Ig) G4-related sclerosing inflammation in 7 patients. When tested, serum IgG4 was elevated in 6 of 7 patients. Clinical or histologically proven enlargement of cervical lymph nodes was present in 7 of 14 patients. All patients responded well to systemic corticosteroid therapy, although some had a relapse upon withdrawal. One patient developed diffuse large B-cell lymphoma and subsequently leukemia, of which he later died 20 years after presentation. CONCLUSIONS Enlargement of the infraorbital nerve and canal is rare and strongly suggests a diagnosis of RLH or IgG4-related disease, especially in the presence of ipsilateral extraocular muscle enlargement, sinus disease, or focal orbital disease.

Silent Sinus Syndrome: A Mechanical Theory

We present the case of a progressive enophthalmos, 23 years after an orbital floor fracture treated conservatively. Imaging demonstrated orbital fat herniation into the pterygopalatine fossa and a collapse of the maxillary sinus walls. We hypothesise that some “silent sinus syndrome” might be caused by an aspiration phenomenon resulting from masticatory muscle movements.

Differential Gene Expression Profiling of Orbital Adipose Tissue in Thyroid Orbitopathy

PURPOSE We aimed to determine differentially expressed genes relevant to orbital inflammation and orbital fat expansion in thyroid orbitopathy (TO) using microarray gene profiling in a case-control study. METHODS Human orbital adipose samples were obtained from individuals with active TO (n = 12), inactive TO (n = 21), and normal controls (n = 21). Gene expression profiles were examined using microarray analysis and were compared between active and inactive TO, and between active TO and normal controls. Top ranked differentially expressed genes were validated by real-time RT-PCR in an additional eight active TO, 13 inactive TO, and 11 normal controls and correlated with gene set enrichment analysis (GSEA) and molecular pathways analysis. RESULTS Seven hundred twenty-one probes (683 genes) and 806 probes (735 genes) were significantly differentially expressed in comparing active to inactive TO and in comparing active TO to healthy controls, respectively. All selected genes were confirmed to be differentially expressed by real-time RT-PCR. Multiple top ranked genes in active versus inactive TO comparison are overrepresented by immune and inflammatory response genes. They include defensins (DEFA1, DEFA1B, DEFA3), which were overexpressed by 3.05- to 4.14-fold and TIMD4 by 4.20-fold. Markers for adipogenesis were overexpressed including SCD, FADS1, and SCDP1. Gene set enrichment analysis revealed dysregulation of epigenetic signatures, T-cell activation, Th1 differentiation, defensin pathway, cell adhesion, cytoskeleton organization, apoptosis, cell cycling, and lipid metabolism in active TO. CONCLUSIONS Active TO is characterized by upregulation of genes involved in cell-mediated immune, innate immune, and inflammatory response and enhanced orbital adipogenesis. TIMD4, DEFA1, DEFA1B, and DEFA3 genes may be involved in the innate immune-mediated orbital inflammation in TO. Epigenetic mechanisms may play a role in the pathogenesis of TO.

Orbital compartment syndrome following aneurysm surgery.

Orbital compartment syndrome (OCS) is a rare cause of blindness following intracranial surgery. We report a patient with OCS following intracranial cerebrovascular surgery precipitated by severe straining. OCS occurred due to a rapid increase in intraorbital pressure within the rigid confines of the orbit causing hypoperfusion of critical neural structures, which resulted in visual loss and a complete external ophthalmoplegia. Treatment involved urgent surgical soft tissue decompression of the orbit, corticosteroids and osmotic agents. It is important to consider OCS as a cause of blindness in the neurosurgical postoperative setting as without rapid treatment this condition has a very poor prognosis.

The Anatomical Location and Laterality of Orbital Cavernous Haemangiomas

Abstract Purpose: To determine the anatomical location and laterality of orbital cavernous haemangiomas (OCH). Design: Retrospective case series. Methods: The records of 104 patients with OCH were analyzed. Main outcome measures: The anatomical location of each OCH defined by the location of a point at the centre of the lesion, and its laterality. Results: There were 104 patients included in the study. No patient had more than one lesion. Sixteen (15.4%) were located in the anterior third of the orbit, 74 (71.2%) were in the middle third, and 14 (13.5%) in the posterior third. In the middle third, 10 of 74 (13.5%) were extraconal and 64 intraconal (86.5%), with 30 of 64 (46.9%) middle third intraconal lesions lying lateral to the optic nerve. Of 104 lesions, 56 (53.8%) were left sided, showing a trend towards a predilection for the left side (p = 0.065). If data from other published series which included data on laterality is added to our own data and analysed, 270 of 468 (57.7%) OCH occurred in the left orbit (p < 0.005). Conclusions: OCH may occur at almost any location within the orbit. The commonest location is the middle third of the orbit, in the intraconal space lateral to the optic nerve. This may reflect an origin of these lesions from the arterial side of the circulation, as there are more small arteries in the intraconal space lateral to the optic nerve than in other locations. A predilection for the left orbit remains unexplained.

Solitary fibrous tumor of the orbit with intracranial involvement.

Solitary fibrous tumor of the orbit is a rare neoplasm that often follows a benign course. Additional involvement beyond the orbit is even rarer, having only been reported in 2 cases previously. The authors describe a 62-year-old patient with a primarily intracranial lesion including orbital extension which was debulked and initially diagnosed as meningioma. Two recurrences 3 and 4 years later disclosed a solitary fibrous tumor, with the initial histopathology being revised to the same diagnosis. This rare presentation of an orbital solitary fibrous tumor highlights the surgical challenge faced when the tumor straddles the cranio-orbital junction and demonstrates the potential for rapid regrowth after incomplete excision.

The natural history of orbital cavernous hemangiomas.

Purpose: Orbital cavernous hemangiomas (OCH) often present as incidentally found asymptomatic lesions, and it has been reported that such lesions do not grow. The authors’ own experience has been different. They describe the natural history of OCH in a cohort with asymptomatic incidentally found lesions and compare them with patients with symptomatic OCH to identify any characteristics that might predict those which enlarge and produce symptoms. Methods: This was a retrospective comparative case series from 3 tertiary referral orbital centers in Australia. There were 104 patients with OCH, 31 of whom presented with incidentally found lesions. For patients with incidental lesions, only those with a minimum follow-up period of 12 months were included. Outcome measures included demographic features, clinical features at presentation and during follow up, anatomical location of the OCH, change in maximal linear dimensions of the OCH over time, follow-up period, and requirement for treatment in the follow-up period. Results: There were 104 patients included in the study. Thirty-one had an asymptomatic, incidental OCH on imaging performed for other indications. Seventy-nine patients had treatment, and 11 of these had presented with an incidental, asymptomatic OCH that enlarged and produced symptoms or new clinical findings. In the 20 other patients with incidentally found OCH, there was no or minimal change in the follow-up period of 1.2 to 20 years (mean 5.8 years, standard deviation [SD] 4.6 years). None of the factors analyzed in the study were able to predict those patients with an incidental OCH that enlarged and required treatment. Patients with incidental lesions that did not grow over several years did not change over much longer periods of time. When considering all patients with OCH, both incidentally diagnosed and symptomatic, there were 4 factors that predicted whether someone with an OCH would require treatment. These were maximal linear dimension, male gender, extraconal location (increased risk of requiring treatment), and being an incidental finding (decreased risk of requiring treatment). Conclusions: OCH may present as an incidental finding. A significant number will grow over time and require treatment. The authors could not identify any factor that would predict those lesions that enlarge over time and require treatment. If an incidental OCH does not change over several years, it is very unlikely to do so in longer periods of follow up.

Atypical clinical presentation of isolated extraocular muscle sarcoid.

A case of painless isolated extraocular muscle sarcoid and a literature review are presented. The atypical features in our case include a lack of overt inflammatory symptoms and signs, such as pain, ptosis, and diplopia. The presentation of minimal symptoms without improvement warrant a biopsy to establish the diagnosis and to administer appropriate treatment in order to prevent potential long-term complications from sarcoidosis.

Infratemporal inflammatory myofibroblastic tumour with orbital extension.

Inflammatory myofibroblastic tumour (IMT) is a rare distinctive neoplasm of intermediate biological potential with a predilection for the abdominopelvic region and lung of children and young adults. It is histologically composed of spindle cells (myofibroblasts) in a myxoid to collagenous stroma with a prominent inflammatory infiltrate composed primarily of plasma cells and lymphocytes. Its pathogenesis is controversial. Arising most commonly in the lungs, only two cases of histopathologically confirmed IMT originating from the infratemporal and pterygopalatine fossae have been documented in the literature. Neither had orbital involvement. We now report the first case of IMT originating from the infratemporal fossa with orbital extension in a previously well 31‐year‐old woman. The patient presented with a 5‐month history of intermittent right‐sided headaches, progressive trismus and right lower lid swelling. She then developed right proptosis, diplopia and paraesthesia in the ophthalmic and maxillary divisions of her trigeminal nerve. Magnetic resonance imaging showed a soft tissue mass occupying most of the right infratemporal fossa with extension into pterygopalatine fossa and orbit. Provisional diagnosis from an open biopsy was nodular fasciitis. She underwent surgical debulking of the infratemporal fossa and lateral orbit through a coronal and trans‐oral approach with trans‐zygomatic access via total zygomatic osteotomy. Review of intraoperative specimens revised the diagnosis to IMT. Further management included systemic corticosteroids and adjuvant radiotherapy.Inflammatory myofibroblastic tumour (IMT) is a rare distinctive neoplasm of intermediate biological potential with a predilection for the abdominopelvic region and lung of children and young adults. It is histologically composed of spindle cells (myofibroblasts) in a myxoid to collagenous stroma with a prominent inflammatory infiltrate composed primarily of plasma cells and lymphocytes. Its pathogenesis is controversial. Arising most commonly in the lungs, only two cases of histopathologically confirmed IMT originating from the infratemporal and pterygopalatine fossae have been documented in the literature. Neither had orbital involvement. We now report the first case of IMT originating from the infratemporal fossa with orbital extension in a previously well 31-year-old woman. The patient presented with a 5-month history of intermittent right-sided headaches, progressive trismus and right lower lid swelling. She then developed right proptosis, diplopia and paraesthesia in the ophthalmic and maxillary divisions of her trigeminal nerve. Magnetic resonance imaging showed a soft tissue mass occupying most of the right infratemporal fossa with extension into pterygopalatine fossa and orbit. Provisional diagnosis from an open biopsy was nodular fasciitis. She underwent surgical debulking of the infratemporal fossa and lateral orbit through a coronal and trans-oral approach with trans-zygomatic access via total zygomatic osteotomy. Review of intraoperative specimens revised the diagnosis to IMT. Further management included systemic corticosteroids and adjuvant radiotherapy.