Peter W. MacIntosh

Current trends in the management of thyroid eye disease.

Purpose of review The present review summarizes the body of literature concerning the medical and surgical treatment of thyroid eye disease (TED) from 1 January 2014 through 30 March 2015. Recent findings Corticosteroids continue to be the primary medical therapy for TED. Recent research has offered insight into potential differences between oral corticosteroid and intravenous corticosteroid treatment regimens in terms of efficacy and side-effect profiles. Steroid-sparing medications, for example, rituximab and others, are an area of active study. There has been renewed interest in the role of radiation therapy as a nonmedical treatment for TED with some promising data. The use of balanced orbital decompression techniques have become popular, although the data regarding postoperative diplopia are mixed, and ‘fat decompression’ offers an alternative or an augmentation to bony decompression. Stereotactic image guidance is a useful adjunct to orbital decompression surgery. Summary TED continues to be a difficult condition for the patient to cope with and for the clinician to treat, and recent research builds on the present foundation of knowledge and treatments, but unfortunately does not offer paradigm-shifting information at the present time.

Stereotactic microdebrider in deep lateral orbital decompression for patients with thyroid eye disease

Purpose: Stereotactic navigation systems have been used in neurosurgery and otolaryngology with great success. The current investigation illustrates the novel use of a microdebrider with built-in stereotactic guidance in a series of thyroid orbitopathy patients who underwent deep lateral orbital wall decompression surgery. Methods: A noncomparative, interventional, retrospective case series of patients who underwent deep lateral deep orbital wall decompression from 2006 to 2013 was conducted in accordance with Institutional Review Board policy and the Declaration of Helsinki. Patient demographics, indications for surgery, pre-, intra-, and postoperative findings along with complications were recorded. Results: One hundred eight deep lateral orbital decompression surgeries were performed in 69 patients using the Straightshot M4 Microdebrider with built-in stereotactic guidance (Medtronics). Seventy-eight cases were in women and 30 cases were in men. The average age was 50.4 years (SD = 11.9 years). Indications for surgery included proptosis, exposure keratopathy, or compressive optic neuropathy. No patient experienced intraoperative complications. Specifically, cerebrospinal fluid leak, visual loss, infection, or unanticipated inflammation were not encountered. The average postoperative follow-up time was 5.35 months. Mean reduction in proptosis was 3.72 mm (SD = 2.1). Visual acuity improved in 32.4% (35/108) of cases. Conclusions: This surgical instrument combines a single handpiece locator, microdebrider, irrigator, retractor, and suction device into one. It enhances anatomical localization during orbital decompression and, with an integrated tissue guard, may decrease the risk of injury to orbital soft tissues. Stereotactic navigation enhances the surgeon’s ability to determine the maximal limits of decompression in real time by confirming depth of bone removal and may potentially increase surgeons’ confidence in orbital decompression surgery.

Complications of Intralesional Bleomycin in the Treatment of Orbital Lymphatic Malformations

Abstract Purpose: Lymphatic malformations (LM) are associated with significant morbidity, and in the orbit, can lead to vision loss. Treatment of these lesions is complicated by their infiltrative nature, making surgical excision challenging. Bleomycin has been used since the 1970s for sclerosis of LM to reduce the need for or improve the success of surgical excision. This paper reviews the complications associated with intralesional bleomycin in the treatment of orbital LM. Methods: A comprehensive literature search was performed on PubMed. Thirty full-length English articles were reviewed. Results: The pathophysiology and imaging characteristic of LM were first reviewed. The head and neck literature was heavily cited given the dearth of specific orbital data. This review highlights the common techniques and dosages for injection, the overall success rate of this treatment, and its common side effects. The orbital data were then reviewed, and differentiated from the head and neck literature. Conclusion: Sclerotherapy of most LM with bleomycin appears to be safe and effective, but the lack of specific orbital data means we must extrapolate LM data from other fields to orbital LM with caution. Additional research is warranted, though the risk-benefit ratio remains unknown.

High grade neuroendocrine neoplasm of the antrum and orbit

Neuroendocrine malignancies-tumors characterized by the production of dense-core secretory granules-are most often encountered in the lungs and can also be found in extrapulmonary sites. Our patient had a primary neuroendocrine tumor of the antrum with an elusive cell of origin that secondarily invaded the inferior orbit. In the sinuses, neuroendocrine tumors may be confused with infectious sinusitis or squamous cell carcinoma. There are no known pathognomonic clinical or radiographic signs to distinguish these tumors from other conditions. Diagnosis depends on a biopsy with histopathologic and immunohistochemical analysis to identify biomarkers such as synaptophysin, chromogranin, CD56 and neuron specific enolase. Our patients tumor defied precise immunohistochemical characterization because of its primitive character and erratic biomarker expression. The diagnosis oscillated between a neuroendocrine carcinoma and an ectopic esthesioneuroblastoma grade IV-hence the use of the more generic nosologic category of neuroendocrine neoplasm without specifying a neuronal or epithelial origin. Data to guide management are limited, particularly in the ophthalmic literature, and derive from experience with tumors of the sinonasal compartments. In the present case of a sino-orbital high grade neuroendocrine neoplasm, regional lymph node metastases developed shortly after presentation. The tumor has responded well to chemotherapy and radiation, but recurrence is often encountered within 2 years in this class of neoplasms.

Naphazoline as a confounder in the diagnosis of carotid artery dissection

Diagnosing Horner Syndrome can be difficult in the setting of an incomplete triad. A 27-year-old man presented with unilateral eyelid droop and intermittent ipsilateral headaches, having already seen 7 physicians. Physical examination revealed unilateral ptosis but no pupillary miosis or facial anhidrosis. Inspection of his clinical photographs revealed elevation of the ipsilateral lower eyelid, suggesting sympathetic dysfunction. On further questioning, he admitted to naphazoline dependence. Reexamination after ceasing the naphazoline unveiled the anisocoria. Vascular imaging subsequently revealed carotid dissection, and the patient was started on anticoagulant and antiplatelet therapy. The ptosis persisted after conjunctival Müllerectomy. External levator resection was recommended, but patient declined. This case underscores the importance of clinical photography, meticulous medical record review, and complete medication history including over-the-counter preparations. Clinicians should meticulously inspect the lower eyelid in cases of atypical blepharoptosis and consider the effects of eye drops when inspecting pupils for miosis.

Failed Cartilaginous Grafts in the Eyelid: A Retrospective Clinicopathological Analysis of 5 Cases

Purpose: To analyze the clinical and histopathologic features of 5 failed autologous cartilaginous grafts to the lower eyelids and to analyze the reasons for these failures. Methods: In this retrospective case series, the data collected included patient ages, reasons for and duration of cartilaginous graft implants, sources of cartilaginous grafts, and clinical and histopathologic findings at time of graft removal using hematoxylin and eosin, elastic, Alcian blue, and Masson trichrome staining for analysis of tissue alterations. Results: Five cartilaginous, posterior lamellar lower eyelid grafts were complicated by eyelid thickening or retraction, graft extrusion, and entropion. Histopathologic findings included segmentation of the original single implant, stripped of its perichondrium, due to “kerfing,” sometimes with overlapping of the segments and scar formation between the segments. In place of the perichondrium that had been removed during the preparation the graft implants, a fibrous pseudoperichondrial capsule had formed. Pyknotic nuclei in varying degrees were typically found in the center of the grafts, despite a high degree of preservation of the extracellular matrix (collagenous, elastic, and proteoglycan components). No evidence of inflammation, cartilaginous vascularization, or necrosis was identified in any graft. Conclusion: Despite minimal reactive processes, kerfing (partial thickness cuts made in the graft to increase its pliancy) may be partially responsible for graft migration, deformation, and surgical failure. The consequences were graft fragmentation and overlapping of the multiple fragments. Graft migration can be exacerbated if a posterior lamellar graft is used to correct an anterior lamellar deficiency. Interference with the overall architectural integrity of the graft and its extracellular matrix appears to play no role in failure, despite removal of the perichondrium. Mild to moderate degrees of chondrocytic dropout in the absence of necrosis and inflammation are probably attributable to the thick and coarsely textured collagen of the fibrous pseudoperichondrial capsule that may impede diffusion of nutrients into the center of the graft.

Posterior Vitreous Detachment Associated with Non-arteritic Ischaemic Optic Neuropathy

ABSTRACT It has been hypothesised that non-arteritic ischaemic optic neuropathy is caused by vitreous traction on the optic nerve. The authors recently took care of a patient who developed a posterior vitreous detachment shortly after she was diagnosed with non-arteritic ischaemic optic neuropathy. Her visual field spontaneously improved after the posterior vitreous detachment. This case report provides some evidence that non-arteritic ischaemic optic neuropathy, vitreous traction, and posterior vitreous detachment may be related.

A school of red herring

A 66-year-old man with chronic kidney disease presented with painless unilateral vision loss and bilateral optic disk swelling. Vision loss progressed in the affected eye and developed in the fellow eye. Evaluation for infectious, neoplastic, ischemic, and inflammatory disorders was unrevealing. High-dose corticosteroids appeared to stabilize his vision temporarily. Eventually he was diagnosed with papilledema in the setting of transverse venous sinus stenosis and dural arteriovenous fistula. His papilledema resolved, and his vision improved following stenting of the venous sinus stenosis and embolization of the fistula.

Pediatric Optic Nerve Meningioma: Diagnostic and Therapeutic Challenges

A 13-year-old female presented with left unilateral proptosis, blurry vision, and diplopia. Clinical examination showed left sided visual acuity of 20/50, limited extraocular movement, 5-mm proptosis, and optic disc edema. CT and MRI displayed a large, intraconal, well-demarcated soft tissue mass with inferotemporal displacement of the optic nerve. The imaging appearance was unusual and diagnosis remained uncertain. Histopathologic analysis of the biopsy specimen confirmed the diagnosis of atypical syncytial meningioma. The tumor cells were positive for both androgen and progesterone receptors and the Ki67 stain was positive (proliferation index of 8%). The patient was treated with proton beam radiation therapy (total dose 50.4 GyE) that suppressed tumor growth and has preserved visual acuity to date (20/40). Differential diagnosis and approaches to therapy are explored.

Multicentric myofibromatosis presenting as a large congenital eyelid myofibroma

Infantile myofibromatosis is a rare mesenchymal neoplasm that commonly involves the head and neck but rarely the eyelid. We report the case of a newborn boy referred for evaluation of a left eyelid lesion that occluded the visual axis. Urgent biopsy was performed to evaluate for malignancy. Histopathologic analysis demonstrated myofibroma. Although these lesions have been reported to regress spontaneously, debulking surgery was performed to prevent sensory or anisometropic amblyopia. Follow-up systemic evaluation revealed numerous subcutaneous and deep soft tissue lesions. There was no visceral involvement.