Solitary fibrous tumor of the orbit in a 1-year-old patient: a case report.

Solitary fibrous tumors (SFTs) are rare neoplasms of mesenchymal origin. While the mean age of presentation is 58 years old, we report the case of the youngest documented patient with an SFT of the orbit. A 13-month-old child was evaluated for eyelid asymmetry and then referred to the oculoplastic service. On examination, a soft tissue mass of the right inferomedial orbit was observed. MRI demonstrated a well-circumscribed, extraocular lesion in the inferomedial right orbit, potentially fibrous in nature. Excision was performed without complications. Pathologic examination demonstrated fibrous tissue proliferation with a staghorn vascular pattern, as well as benign fibrous cells with tapering nuclei and abundant pericellular reticulin. Immunohistochemistry (IHC) demonstrated that the cells stain diffusely positive for CD34 and vimentin. With the MRI findings, pathology, and IHC, the diagnosis was confirmed to be SFT. SFTs of the orbit, although rare, may occur in the pediatric population.

A spectroscopic approach to measuring meibum lipid composition and conformation in donors with SjÓ§gren's syndrome.

Patients with SjÓ§gren's syndrome (SS) have dry eye associated with meibomian gland dysfunction (MGD). The meibum from donors with dry eye due to MGD but without SS (MMGD) presents with lower levels of cholesteryl ester, less straight chains, and more ordered hydrocarbon chains compared with meibum from donors without MGD (Mn). The aim of the current study was to compare the composition and hydrocarbon chain conformation of meibum from donors with Sjögren's syndrome (Mss) to Mn and MMGD. Meibum was expressed from patients with SS using an ILUX instrument (Alcon Inc., Fort Worth TX). All of the nine meibum donors with SS were female. Meibum composition was characterized using 1H-NMR and meibum hydrocarbon chain conformation was measured using fourier transform infrared spectroscopy. Meibum from every donor with SS measured contained a significantly (P < 0.01) higher cholesteryl ester/wax ester ratio and more straight chains compared with donors without SS or dry eye. None of the nine phase transitional parameters were significantly different, P > 0.05, for Mss compared with Mn. Nor was the CH3/CH2 band height ratio used to estimate the number of hydrocarbon CH3 and CH2 moieties different, P = 0.22, for Mss compared with Mn. In conclusion, the compositional differences between Mss compared with Mn did not result in differences in any of the nine meibum lipid phase transitional parameters measured. The compositional differences observed between Mss and Mn could be markers for or contribute to SS as the differences could lead to tear film lipid packing differences other than conformational differences.

Post-operative epiphora following the transcutaneous medial canthal incision.

PURPOSE: The safety profile of the transcutaneous medial canthal incision for access to the medial orbit is assessed with a focus on the risk of post-operative iatrogenic epiphora. METHODS: A retrospective chart review of patients undergoing medial orbitotomy via the transcutaneous medial canthal incision was performed. Patients with a minimum of 3 months of follow-up were included and post-operative complications were assessed and characterized. RESULTS: One-hundred-fifty patients were included in the study. A total of 4 complications were identified, including one each of the following: nasolacrimal duct obstruction, hypertrophic scar, suture granuloma and soft tissue infection. Only the nasolacrimal duct obstruction required surgical intervention. DISCUSSION: Access to the medial orbit has been achieved through a variety of approaches, each with their own benefits and risk profile. The transcaruncular approach has increased in usage as a means to avoid a visible cutaneous scar and decrease the risk of iatrogenic epiphora, however, there are specific patients who may have relative contraindications to this approach. The current study demonstrates the low risk profile of the transcutaneous medial canthal incision, specifically the minimal risk of iatrogenic damage to the nasolacrimal outflow system. This approach is another useful tool which orbit surgeons should be familiar with to offer as an option to patients requiring medial orbitotomy.

Combined medial canthopexy and lateral tarsal strip for floppy eyelid syndrome.

PURPOSE: To describe the eyelid condition known as "floppy eyelid syndrome" and to present the results of our experience treating patients with floppy eyelid syndrome. METHODS: A retrospective analysis of 16 patients from 2009 to 2013 who underwent combined medial canthopexy and lateral tarsal strip by two surgeons (HBL and WRN) for the treatment of floppy eyelid syndrome (FES). Age, gender, presence of obstructive sleep apnea (OSA), papillary conjunctivitis, punctate keratopathy and lash ptosis were recorded pre-operatively. Outcomes assessed included improvement in upper eyelid laxity, operative complications, post-operative symptomatic relief and delayed recurrence of FES. RESULTS: A total of 18 procedures (on 36 eyelids) were performed on 16 patients over the 4-year period. All patients (18/18) had relief of symptoms and good functional results, defined as improvement in lid laxity and resolution of symptoms. Average follow up was 124days. 8 of 16 patients (50%) had a pre-existing diagnosis of OSA. The remaining 8 patients were referred for sleep study and 2 were subsequently diagnosed with OSA. Two patients experienced some degree of FES recurrence without return of symptoms. There was one complication reported in which a partial dehiscence of the lateral canthal tendon occurred which did not require operative revision. CONCLUSIONS: Combined medial canthopexy and lateral tarsal strip are a safe and effective technique for the treatment of floppy eyelid syndrome. There is a strong association of FES and OSA and it is important to have any patient diagnosed with FES evaluated for OSA.

Nodular Fasciitis of the Orbit.

A 13-month-old boy was presented with new onset proptosis of the right eye. CT scan and MRI showed an enhancing mass in the right superior orbit with local bone remodeling and erosion. A craniotomy was performed for biopsy and sub-total resection. Histopathology and immunohistochemistry confirmed the lesion to be nodular fasciitis. Nodular fasciitis lesions are classically found in the anterior ocular adnexa, especially in pediatric patients. This is the first reported case of nodular fasciitis arising in the posterior orbit of a child younger than 16.

Foreign Body Granuloma Following Orbital Reconstruction with Porous Polyethylene.

Porous polyethylene is commonly used in the orbit for fracture repair and anophthalmic reconstruction; it reportedly has a good safety profile and integrates well into host tissue. Foreign body reaction to porous polyethylene has been reported in facial tissue, but to our knowledge, not in the orbit. The authors report 2 cases of foreign body inflammatory giant cell reaction in patients who underwent orbital fracture repairs with porous polyethylene implants.

Orbital cellulitis and corneal ulcer due to Cedecea: First reported case and review of the literature.

Cedecea is a gram-negative bacterium from the family Enterobacteriaceae, rarely associated with human infection. We report the first case of an orbital cellulitis and corneal ulcer due to Cedecea in a patient who sustained a motor vehicle accident and was then found to have a retained wooden orbital foreign body.

Recession and Extirpation of the Lower Eyelid Retractors for Paralytic Lagophthalmos.

PURPOSE: To describe a technique to correct lower eyelid malposition and lagophthalmos due to facial nerve palsy. METHODS: Chart review was performed and identified 13 patients with facial nerve palsy, who presented with paralytic eyelid malposition and were treated with recession and extirpation of the lower eyelid retractors between September 2012 and March 2014 by 1 surgeon (HBL). RESULTS: A total of 12 eyelids in 11 patients met inclusion criteria. Mean preoperative MRD2 was 10 mm (range, 9.0-12.0 mm) and the mean postoperative MRD2 was 7 mm (range, 5.0-9.0 mm). The MRD2 improved an average of 3.0 mm in each patient (range, 2.0-4.0 mm). Patients had an average of 6.9 mm (range, 4.0-10.0 mm) of lagophthalmos preoperatively, which improved to 2.1 mm (range, 0.0-4 mm). The amount of lagophthalmos improved an average of 4.8 mm in the patients. There were no complications encountered in the patients. All patients had a subjective improvement in ocular comfort. CONCLUSIONS: The authors' surgical technique is effective in addressing lower eyelid malposition and ocular surface disease in paralytic lagophthalmos.

CD56 negative extranodal NK/T-cell lymphoma of the orbit mimicking orbital cellulitis.

INTRODUCTION: To report a case of Extranodal NK/T-cell lymphoma of the orbit mimicking orbital cellulitis. CASE DESCRIPTION: A 52-year-old healthy male presented to our institution after 3 months of treatment for sinusitis with antibiotics and steroids. The patient was transferred due to the presence of an "orbital abscess" on CT with orbital signs that not responding to antibiotics. Clinical examination was significant for decreased vision in the affected orbit of 20/50, a trace RAPD OS, elevated IOP of 30 OS, proptosis and grossly decreased motility with diplopia, periorbital edema and chemosis. Dilated funded exam was unremarkable. CT imaging demonstrated a left sided pan-sinusitis, a medial "orbital process" with proptosis and erosion of the cribiform plate. The patient was taken for an emergent orbital exploration for histopathologic diagnosis. Intraorbital and sinus biopsy was consistent with extranodal NK/T-cell lymphoma, with extension into the skull base and left orbital space. The patient was started on radiation therapy followed by chemotherapy. COMMENTS: The authors demonstrate how the acute presentation of an aggressive extranodal NK/T-cell lymphoma can present in a similar fashion as orbital cellulitis. Additionally, the case highlights that a unilateral pansinusitis with involvement of the skull base and orbit is likely due an aggressive malignant process in an immune competent patient.

Corrigendum to "Ophthalmic considerations in patients with pfeiffer syndrome." [Am. J. Ophthalmol. Case Reports (2) 2016 1-3].

[This corrects the article DOI: 10.1016/j.ajoc.2016.04.001.].

Ophthalmic considerations in patients with Pfeiffer syndrome.

PURPOSE: We report here a newborn male infant with striking features consistent with severe Pfeiffer syndrome type II, including cloverleaf skull deformity with pansynostosis, extreme proptosis, upper extremity contractures, broad big toes and thumbs with varus deviation and genetic mutation in the FGFR2 gene. The authors review the ophthalmic complications in Pfeiffer syndrome and discuss the unique surgical strategies used for obtaining adequate corneal coverage in these unique patients. OBSERVATIONS: Ophthalmic considerations in Type 2 Pfeiffer Syndrome include vision loss secondary to increased intracranial pressure, and extreme proptosis as a result of orbitostenosis and midfacial retrusion. Our patient has undergone multiple ophthalmic/oculoplastic, neurosurgical, and midfacial surgeries as a result of corneal deterioration due to extreme exorbitism. CONCLUSIONS AND IMPORTANCE: It is important for ophthalmologists to be aware of the ophthalmic complications associated with patients with craniosynostosis syndromes. Our case identifies the importance of close communication between ophthalmology and plastic reconstructive surgery to help formulate the most successful plan in treating corneal decompensation and proptosis in Pfeiffer Syndrome patients.

Pigmented epithelioid melanocytoid tumor of the ocular adnexa.

An 8-year-old girl with a history of microphthalmia in the right eye presented with a left medial upper eyelid mass with a dark blue-green nodule that could be seen through the skin but did not appear to involve the overlying epidermis. A biopsy demonstrated a pigmented epithelioid melanocytoid tumor with rare mitoses arising in association with a congenital nevus and positive margins. Due to the pathological findings, the patient underwent excision of the tumor with 5-mm margins and a sentinel lymph node biopsy. The re-excision of the upper eyelid margins demonstrated residual benign congenital melanocytic nevus, but did not reveal residual melanocytic lesion. The parotid sentinel node biopsy revealed benign and cytologically mature nevus nests in the capsule and septa, but there was no evidence of involvement with the pigmented epithelioid melanocytoid tumor. The patient underwent successful eyelid reconstruction and had no evidence of recurrence or metastasis after 2 years.

Regulation of oxidative stress by the anti-aging hormone klotho.

klotho is an aging suppressor gene and extends life span when overexpressed in mice. Klotho protein was recently demonstrated to function as a hormone that inhibits insulin/insulin-like growth factor-1 (IGF-1) signaling. Here we show that Klotho protein increases resistance to oxidative stress at the cellular and organismal level in mammals. Klotho protein activates the FoxO forkhead transcription factors that are negatively regulated by insulin/IGF-1 signaling, thereby inducing expression of manganese superoxide dismutase. This in turn facilitates removal of reactive oxygen species and confers oxidative stress resistance. Thus, Klotho-induced inhibition of insulin/IGF-1 signaling is associated with increased resistance to oxidative stress, which potentially contributes to the anti-aging properties of klotho.

Suppression of aging in mice by the hormone Klotho.

A defect in Klotho gene expression in mice accelerates the degeneration of multiple age-sensitive traits. Here, we show that overexpression of Klotho in mice extends life span. Klotho protein functions as a circulating hormone that binds to a cell-surface receptor and represses intracellular signals of insulin and insulin-like growth factor 1 (IGF1), an evolutionarily conserved mechanism for extending life span. Alleviation of aging-like phenotypes in Klotho-deficient mice was observed by perturbing insulin and IGF1 signaling, suggesting that Klotho-mediated inhibition of insulin and IGF1 signaling contributes to its anti-aging properties. Klotho protein may function as an anti-aging hormone in mammals.