Transitioning from a gold weight to an "enhanced" palpebral spring in the management of paralytic lagophthalmos secondary to facial nerve palsy.

PURPOSE: To analyze the outcomes of patients who transitioned from a weight to a spring. METHODS: Case series original investigation. Five consecutive patients undergoing "enhanced" palpebral spring insertion status post gold weight insertion were identified. Outcome measures included visual acuity, lagophthalmos, margin reflex distance 1, and corneal epithelial defects. Subjective outcomes included need for eye lubrication, closure and rapid blink, and symptoms of discomfort and dryness. This study was reviewed by an accredited Institutional Review Board (IRB) and granted exempt status according to federal regulations. RESULTS: Mean visual acuity post weight versus spring was 20/200 (logMAR 1.0, Std Dev. -0.6) and 20/25 (logMAR 0.1, Std Dev. -0.1), respectively (p = 0.0214, CI 0.231 to 1.670). Mean lagophthalmos post weight (3.5 mm, Std Dev. -2.8) versus post spring (0.2 mm, Std Dev. -0.5). Margin reflex distance 1 improved by a mean of 1.4 mm (gold wt -0.6, Std Dev. -0.5/spring -2.0, Std Dev. -0.4) (p = 0.0016, CI -1.773 to -0.977). Superficial punctate keratopathy was present in all patients with weights and present in no patients post transition (p = 0.0079). Post transition, three of five patients rarely required lubrication and experienced a more satisfactory blink rate. CONCLUSION: Patients with lagophthalmos due to cranial nerve seven palsy who have undergone gold weight placement but continue to exhibit objective and subjective corneal symptoms may have symptomatic improvement with the transition to a palpebral spring.

Orbital Myxofibrosarcoma: Case Report and Review of Literature.

Myxofibrosarcoma is rarely found in the orbit, let alone as a primary orbital tumor. With this having only been previously reported 5 times in the literature, there is a very limited understanding of its presentation and course. Typical management involves surgical excision with or without postoperative radiotherapy. Low-grade malignancy, although rare to metastasize, still has a high rate of local recurrence and with each recurrence increases the risk of metastatic potential. With this in mind, analysis of each case is critical in better understanding the disease, and this case adds to the current literature.

Diagnosis of optic nerve sheath meningioma during optic nerve sheath decompression.

Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.

Extreme Eyelid Lymphedema Associated With Rosacea (Morbihan Disease): Case Series, Literature Review, and Therapeutic Considerations.

PURPOSE: To describe severe lymphedema of the eyelids, known as Morbihan disease, a previously characterized but infrequently reported and poorly understood entity related to rosacea that features solid mid-facial and eyelid lymphedema. METHODS: Retrospective chart review, histopathologic and immunohistochemical analysis, and pertinent literature consideration. RESULTS: Five cases of Morbihan disease were identified. Histopathologic examination revealed pleomorphic perivascular and perilymphatic inflammation with profound lymphangiectasis and lymph stasis, thus suggesting elements of both rosacea and localized, chronic lymphedema. Multiple therapeutic interventions were performed including systemic anti-inflammatory therapy, surgical debulking, and corticosteroid injection. CONCLUSIONS: Extreme eyelid edema associated with characteristic skin changes and histopathologic findings represents an entity known as Morbihan disease which is rare and difficult to treat. While multiple modalities have been employed with variable results, future therapeutic considerations may include the use of targeted biologic agents.

Pediatric and adult vision restoration after optic nerve sheath decompression for idiopathic intracranial hypertension.

To compare presentations of idiopathic intracranial hypertension and efficacy of optic nerve sheath decompression between adult and pediatric patients, a retrospective cohort study was completed All idiopathic intracranial hypertension patients undergoing optic nerve sheath decompression by one surgeon between 1991 and 2012 were included. Pre-operative and post-operative visual fields, visual acuity, color vision, and optic nerve appearance were compared between adult and pediatric (<18 years) populations. Outcome measures included percentage of patients with complications or requiring subsequent interventions. Thirty-one adults (46 eyes) and eleven pediatric patients (18 eyes) underwent optic nerve sheath decompression for vision loss from idiopathic intracranial hypertension. Mean deviation on visual field, visual acuity, color vision, and optic nerve appearance significantly improved across all subjects. Pre-operative mean deviation was significantly worse in children compared to adults (p=0.043); there was no difference in mean deviation post-operatively (p=0.838). Significantly more pediatric eyes (6) presented with light perception only or no light perception than adult eyes (0) (p=0.001). Pre-operative color vision performance in children (19%) was significantly worse than in adults (46%) (p=0.026). Percentage of patients with complications or requiring subsequent interventions did not differ between groups. The consistent improvement after surgery and low rate of complications suggest optic nerve sheath decompression is safe and effective in managing vision loss due to adult and pediatric idiopathic intracranial hypertension. Given the advanced pre-operative visual deficits seen in children, one might consider a higher index of suspicion in diagnosing, and earlier surgical intervention in treating pediatric idiopathic intracranial hypertension.

Angiolipoma of the Orbit: A Rare Tumor in an Unusual Location.

Despite their relatively frequent occurrence on the trunk and extremities, lipomas rarely present in the orbit. Rarer still are variants of lipoma such as fibrolipoma, myxoid lipoma, and angiolipoma. The authors report a 66-year-old woman who presented with a large, slowly growing tumor of the forehead and orbit. The case presentation conforms to the tenets of the Declaration of Helsinki and is HIPAA compliant. Clinical and radiographic evidence suggested a lipomatous type of tumor, and excisional biopsy revealed adipose proliferation with numerous small vessels and fibrin thrombi consistent with angiolipoma. The tumor was completely excised without ophthalmic sequelae or recurrence in 6 months of follow up. To the authors' knowledge, this tumor represents only the second reported case of this type of highly vascularized lipomatous lesion within the orbit. This case is a novel entity in the differential diagnosis of orbital tumors and demonstrates the value of total excision in such cases.

Primary adnexal angiosarcoma masquerading as periorbital hematoma.

We report an elderly woman who was anticoagulated and presented with a recent history of right-sided orbital contusion and a periorbital hematoma without clinical or radiological evidence of focal mass or orbital involvement. She was initially treated conservatively. Continued progression of adnexal swelling and erythema prompted further investigation, however. There was no improvement with surgical drainage alone; biopsy revealed angiosarcoma. The discovery of this vascular tumor underscores the importance of a reconsideration of the diagnosis in the face of counterintuitive findings. Additionally, we emphasize the need to consider malignancy in the differential diagnosis of prolonged periorbital swelling, regardless of a history of recent trauma.

Nontraumatic subperiosteal bilateral orbital hemorrhages after mitral valve repair.

Nontraumatic subperiosteal orbital hemorrhages (NTSOHs) are rare events, but pose a grave risk of vision loss and must be evaluated carefully for optic nerve compromise. To the author's knowledge, only 2 cases of NTSOH after cardiovascular procedures have been reported. The authors report a unique case of bilateral NTSOH with optic neuropathy that developed after cardiothoracic surgery and reaccumulated after orbitotomy. The case presentation conforms to the tenets of the Declaration of Helsinki and is Health Insurance Portability and Accountability Act compliant. The etiology and management of this uncommon and dangerous entity will be discussed.

Linear IgA bullous dermatosis: an unusual cause of upper eyelid cicatricial entropion.

Chronic cicatrizing conjunctivitis is a relatively uncommon condition resulting in significant ophthalmic morbidity, including keratoconjunctivitis sicca, cicatricial entropion, trichiasis, corneal scarring, significant discomfort, and visual loss. The potential causes of cicatrizing conjunctivitis are varied and include commonly encountered entities such as ocular cicatricial pemphigoid, Stevens-Johnson syndrome, and trachoma and many more rare causes which are particularly difficult to diagnose and treat and may not be familiar to the ophthalmologist. The authors herein present a case of chronic cicatrizing conjunctivitis, cicatricial entropion, and trichiasis caused by a rare entity called linear IgA bullous dermatosis. The case presentation conforms to the tenets of the Declaration of Helsinki and is Health Insurance Portability and Accountability Act compliant. This chronic dermatosis has a varied presentation, and the ophthalmic manifestations in particular have been infrequently described. This case demonstrates the benefits of immunohistochemistry in diagnosis and the difficulties in medical and surgical management of linear IgA bullous dermatosis while underscoring the lifelong difficulties in managing chronic inflammatory conditions causing ocular cicatrization.

Orbital abscess from dacryocystitis caused by Morganella morganii.

A 22-year-old female with multiple developmental abnormalities stemming from cardiofaciocutaneous syndrome presented with a recurrent orbital abscess 2 years after orbitotomy with drainage of an abscess of presumed hematogenous-origin. During careful intraoperative examination the abscess was seen to directly extend from the lacrimal sac. Cultures were taken and grew Morganella morganii, a Gram negative rod uncommon in ocular and periocular infections. To the author's knowledge, this microorganism has been reported in only one previous case of orbital abscess and underscores the need for organism identification and antibiotic sensitivity analysis in cases of orbital abscess, particularly those with extension from dacryocystitis.

Simplified Müller's muscle-conjunctival resection internal ptosis repair.

PURPOSE: To describe and evaluate a modified technique for blepharoptosis repair via Müller's muscle-conjunctival resection. METHODS: An observational case series is reported. Records of 29 consecutive patients (42 eyelids) with blepharoptosis undergoing internal ptosis repair between January 2008 and December 2010 were reviewed including detailed preoperative and postoperative evaluations, eyelid measurements, surgical outcome, and complications. The procedure performed was a modified Müller's muscle-conjunctival resection with simple measurements, no traction sutures, a double-opposing mattress suture, and single external knot. Main outcome measures included success of ptosis repair surgery defined by improvement in margin reflex distance, symmetry of upper eyelid position, and incidence of complications. RESULTS: The described modified Müller's muscle-conjunctival resection procedure achieved improvement in eyelid position in all patients with mean margin reflex distance change of 2.3 mm (p < 0.001). No patient experienced significant complications, and all procedures resulted in objective eyelid symmetry (within 1 mm) and satisfactory cosmetic appearance. CONCLUSIONS: The procedure described is an efficient, safe, and successful technique for internal blepharoptosis repair.

Inflammatory modulators and biologic agents in the treatment of idiopathic orbital inflammation.

PURPOSE OF REVIEW: To review the diagnosis and treatment of idiopathic orbital inflammation (IOI), with an emphasis on the development and use of inflammatory mediators and biologic agents. RECENT FINDINGS: Use of targeted biologic immunomodulatory therapy is becoming widespread and proving effective against many inflammatory and autoimmune conditions. Although corticosteroids remain the mainstay of care for IOI, their use is fraught with complications and side-effects, suggesting the need for novel therapies. Evidence for the successful implementation of nonsteroidal inflammatory mediators in IOI is accumulating. SUMMARY: The treatment of IOI with traditional methods, particularly corticosteroids, is often inadequate and accompanied by recurrences, medication dependence, and intolerability. Many of these issues may be avoided with the use of existing and novel pharmaceutical agents targeting specific inflammatory mediators. The literature on these agents and their use in IOI is in its infancy but shows tremendous promise in the treatment of this often-recalcitrant illness.

Visual improvement after optic nerve sheath decompression in a case of congenital hydrocephalus and persistent visual loss despite intracranial pressure correction via shunting.

Among the sequelae of persistent raised intracranial pressure (ICP) are ophthalmologic signs and symptoms, including cranial nerve palsies, visual field deficits, papilledema, and vision loss. Elevated pressure within the optic nerve sheath may not be relieved by shunt procedures, which can decrease generalized ICP. The authors present a case of acute visual loss in the setting of chronic hydrocephalus and multiple shunt revisions. Despite shunt correction resolving systemic symptoms of raised ICP, this child had persistent visual loss. Bilateral optic nerve sheath decompression was performed, and the visual acuity improved over the next 3 days. This case highlights the importance of routine ophthalmologic examination in patients with hydrocephalus and shunts and demonstrates the utility of optic nerve sheath decompression as a surgical intervention when shunting alone does not resolve visual loss.