The role of OCT- angiography in predicting anatomical and functional recovery after endoscopic endonasal pituitary surgery: A 1-year longitudinal study.

PURPOSE: The purpose of this study was to investigate the changes in structural spectral-domain optical coherence tomography (SD-OCT), OCT Angiography (OCTA) parameters, and visual acuity, 1 year after endoscopic endonasal approach for the removal of an intra-suprasellar pituitary adenoma compressing optic chiasm and compare outcomes with 48 hours postoperative data. METHODS: Sixteen eyes of eight patients (4 males, 4 females, mean age 52 ± 11 years) were enrolled in this prospective study. The primary outcome was to evaluate the changes over time before and after surgery, analyzing the Best Corrected Visual Acuity (BCVA), Ganglion Cell Complex (GCC), Retinal Nerve Fiber Layer (RNFL) thicknesses, the retinal vessel density (VD) of Superficial Capillary Plexus (SCP), Deep Capillary Plexus (DCP), Radial Peripapillary Capillary (RPC) and the Foveal Avascular Zone (FAZ). The secondary outcome was to identify potential biomarkers that could predict visual acuity changes after 1-year follow-up. RESULTS: When comparing SD-OCT and OCTA measurements obtained after 1 year with those observed 48 hours after surgery, GCC and RNFL were significantly improved. After a significant reduction at 48 hours, GCC thickness showed a significant increase at 1 year after surgery (p = 0.007), while a significant restoration of RNFL thickness was found at 1 year (p = 0.005), as well as the VD of SCP, DCP, and RPC values. FAZ area did not change over time. BCVA significantly improved at each time after surgery (p = 0.037, p = 0.013). A statistically significant correlation was found between the preoperative BCVA, VD of SCP, DCP, RPC, and the postoperative BCVA at 1 year (p = 0.017, p = 0.029, p = 0.031, p = 0.023). CONCLUSION: SD-OCT and OCTA provide helpful information to identify the retinal structural and vascular improvements 1 year after surgery. OCTA parameters could serve as potential predictive markers for visual acuity recovery at long-term follow-up.

Retinal vascular density in multiple sclerosis: a 1-year follow-up.

BACKGROUND AND PURPOSE: Vascular pathology is increasingly acknowledged as a risk factor for multiple sclerosis (MS). Vascular density (VD) is reduced in the eyes of patients with MS on optical coherence tomography (OCT) angiography. We performed a 1-year prospective study to estimate VD variations over time and possible clinical correlates. METHODS: A total of 50 patients with MS underwent spectral domain-OCT and OCT angiography at baseline and after 1-year follow-up. Mixed-effect linear regression models were used to assess variations of each OCT measure and its relation to treatment and clinical outcomes. RESULTS: We observed an increase in parafovea VD (coefficient, 1.147; 95% confidence interval, 0.081-2.214; P = 0.035). Reduction in parafovea VD was associated with increase in Expanded Disability Status Scale score (coefficient, -0.969; 95% confidence interval, -1.732/-0.207; P = 0.013). CONCLUSIONS: Retinal VD can improve over time in MS, particularly in patients experiencing disease stability. Longer follow-up, inclusion of early MS cases and combination with conventional markers of MS severity (i.e. brain atrophy) are needed to better define VD as a potential new biomarker.

Spheno-orbital meningiomas: surgical approaches and outcome according to the intraorbital tumor extent.

OBJECT: This study proposes a topographical classification of spheno-orbital meningiomas. Its aim was to define whether the different intraorbital localizations require different surgical approaches and have different recurrence rates and outcomes. MATERIAL AND METHODS: Sixty patients with spheno-orbital meningiomas operated upon between 1983 and 2003 were reviewed. Four types were identified according to the extent of intraorbital tumor invasion: I: lateral or superolateral (15 cases); II: medial and inferomedial (8 cases); III: orbital apex (25 cases); IV: diffuse (12 cases). Three surgical approaches were used: lateral orbitotomy (15 cases with lateral or superolateral tumors), supraorbital-pterional approach (42 cases, including all 8 inferomedial cases, all 25 orbital apex cases, and 9 of 12 diffuse tumors), and a fronto-temporal-orbitozygomatic approach (only 3 cases with diffuse meningiomas and large-scale tumor invasion in the infratemporal fossa and cavernous sinus). RESULTS: Tumor removal was complete (Simpson grades I and II) in 40 cases, and incomplete in 20 (33.3%). There were two postoperative deaths (3.3%). A sufficient clinical follow-up was obtained in 52 cases. The clinical outcome was excellent in 26 patients (50%), good in 16 (30.8%), moderate in 6 (11.5%), and poor in 4 (7.7%). Twenty-two of 52 patients (42.3%) had tumor recurrence; however, 44 (84.6%) achieved tumor control after surgery alone through two or more operations. The recurrence rate was correlated with the Simpson grade of resection and the intraorbital tumor location. Significantly higher rates of recurrence were recorded for the orbital apex type (50%) and diffuse forms (60%), than for the inferomedial (28.5%) and superolateral forms (23%). CONCLUSIONS: Spheno-orbital meningiomas may be classified according to the location and extent of the intraorbital tumor invasion. The different localizations may require different surgical approaches, with different chances of complete removal. The location and extent of the intraorbital tumor results in different recurrence rates, lower for superolateral and inferomedial forms than for orbital apex and diffuse forms.

Tumor infiltrating lymphocytes in uveal melanoma: a link with clinical behavior?

Experimental and clinical evidence indicate that immunological mechanisms might be important in the clinical course of uveal malignant melanoma (UMM). We analyzed the amount and phenotype of tumor infiltrating lymphocytes (TIL) and the expression of the apoptosis-inducing molecule Fas and its ligand, FasL, on tumor cells and TIL in a selected series of UMM with the aim to establish if a correlation between their expression and the clinical behavior of UMM exists. TIL phenotype and Fas/FasL expression were evaluated by immunohistochemistry in 61 cases of formalin-fixed, paraffin-embedded UMM. Results were compared with the follow-up data of patients. Most of the UMM showed a prevalence of CD8+ CD3+ T lymphocytes, or CD4+ and CD8+ cells in equal amounts. UMM showed a variable expression of FasL, ranging from 0 to > 40% of neoplastic cells. Fas was always expressed in TIL, although with a variable extent. A subgroup of UMM showed in TIL a strongly reduced or even absent expression of TCR zeta-chain, involved in activation of T-lymphocytes. This subgroup was characterized by a worse outcome. We hypothesized that an impaired cytotoxic immune response due to the loss of the zeta-chain expression plays a primary role in the biological course of UMM. Our results indicate that the overcoming of the impairment of TCR function may represent a prerequisite for the development of new therapeutic strategies for managing UMM, suggesting that elimination of tumor cells may be possible by activation of cytotoxic cells present within ocular melanomas.

[Intracorneal invasion of the ciliary body by melanoma]. / Invasion intracornéenne par un mélanome du corps ciliaire.

The authors report a case of a slow reduction of vision in the left eye of a 69-year-old woman. The slit-lamp examination of the eye showed a vascularized achromic subconjunctival mass close to the nasal limbus, invading the inferonasal corneal quadrant. At B-scan and standardized A-scan echography, the mass was shown to be localized to the ciliary body, with characteristics of a malignant melanoma. The patient did not want to undergo conservative treatment. Therefore, enucleation was performed and light microscopy revealed a pigmented malignant melanoma of the ciliary body with extrascleral growth and infiltration of the anterior third of the corneal stroma. No alteration of Bowman's membrane was present. The corneal infiltration, limited to the anterior third of the stroma, could be due to the fact that at this level the collagen fibers are disposed less regularly than those of the posterior two-thirds, with this different disposition responsible for the weak resistance opposed to the tumoral invasion.

Ophthalmic complications after surgery for nasal and sinus polyposis.

PURPOSE: To describe ophthalmic complications after nasal and sinus surgery. METHODS: Four cases with orbital complications were retrospectively selected from among more than 2000 cases of orbital pathologies. RESULTS: Motility disturbances due to extraocular muscle injury occurred in two patients after intranasal ethmoidectomy and in one patient after a Caldwell-Luc procedure. In the fourth case an orbital apex syndrome was noted after intranasal ethmoidectomies. CONCLUSIONS: Ophthalmic complications may occur after nasal and sinus surgery, even using an endoscopic procedure. Successful handling of these complications could be reached by on their early recognition and treatment.

Traumatic displacement of the globe into the ethmoid sinus.

PURPOSE: To describe displacement of the globe into the ethmoid sinus after an orbital trauma. METHOD: Case report. A 58-year-old man sustained trauma of the left eye and orbit, which resulted in displacement of the globe into the ethmoid sinus. One day after injury, surgery was performed to restore the intact globe into position within the orbit. RESULTS: After operation, the globe held in its anatomical site, and 10 months after surgery the visual acuity was 20/100, slight pallor of the optic disk was present, and there was no limitation of the horizontal and vertical ductions. CONCLUSIONS: To our knowledge, this is the first case of traumatic displacement of the globe into the ethmoid sinus with satisfactory restoration of normal globe position and preservation of vision.

Orbital dermis-fat graft using periumbilical tissue.

Dermis-fat grafts are currently used in orbital reconstruction in a variety of procedures. The most frequent harvesting site is the gluteal area. However, we encountered some patients with anophthalmic socket who wished to avoid a visible scar on the buttock. In this article, we describe the effort to offer the patient an alternative donor site. Of the last 36 patients with anophthalmic socket who needed a dermal fat implant, 11 wished to avoid a visible scar on the buttock. To satisfy their requests we have endeavored to harvest the dermis graft from the periumbilical area. The rate of absorption, the motility, and the satisfaction of the patients were used as outcome measures and were analyzed carefully. Of 11 patients, 4 were women and 7 were men. The ages of these patients ranged from 24 to 56. The maximum follow-up was 137 months and the minimum 22 months, with a mean follow-up of 79 months. Some degree of absorption of the graft developed in one patient who had a severe absorption and required further operation. Of 11 cases, there were 7 with excellent motility, 3 with good motility, and 1 not evaluated. The motility was measured with the final prosthesis. The results for all patients were satisfactory. The periumbilical area has sufficient concentration of subdermal fat, and it is a relatively hair-free region as the lateral quadrant of the buttock. This area is a suitable alternative donor site of dermal fat implant for anophthalmic socket, especially in young women.

Radiation late effects in children treated for orbital rhabdomyosarcoma.

BACKGROUND AND PURPOSE: The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects. PATIENTS AND METHODS: Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples. All but three patients, who received 45, 54 and 55 Gy respectively, have been treated by immediate radiation at the dose of 60 Gy, delivered in 2 Gy fractions, five times per week, by cobalt 60 megavoltage equipment. Combined chemotherapy using vincristine and vincristine plus dactinomycin on alternate weeks was also administered as part of induction therapy. RESULTS: An overall survival rate of 94.7% was registered. In our patients the majority of radiation late effects were paid by orbit and ocular adnexa. Side effects to lens and ocular structures were fewer and of low grade. CONCLUSIONS: Radiation therapy is still essential for local control of orbital rhabdomyosarcoma, however radiation side effects have to be carefully considered together with the therapeutic goal to be obtained.

Morphometric analysis of AgNORs in uveal malignant melanoma.

OBJECTIVE: To define the value of silver-stained nucleolar organizer regions (AgNOR) in the evaluation of uveal malignant melanomas (MM). STUDY DESIGN: Morphometric analysis of AgNORs was performed on 30 uveal MM of spindle A, B, epithelioid and mixed cell types. Findings were compared with cytotype, largest tumor dimension (LTD) and clinical outcome. RESULTS: AgNOR mean perimeter (MP) was 3.76 microns (SD 1.21, range 1.79-8.51 microns) in A, 5.05 microns (SD 1.96, range 1.79-16.41 microns) in B and 6.15 microns (SD 3.86, range 1.79-33.80 microns) in epithelioid MM; mean area (MA) was 0.86 micron 2 (SD 0.60, range 0.20-2.78 microns 2) in A, 1.58 microns 2 (SD 1.11, range 0.20-9.63 microns 2) in B and 2.34 microns 2 (SD 2.10, range 0.20-15.27 microns 2) in epithelioid MM; the aspect ratio (AR) was 3.10 (SD 0.19, range 2.90-3.35) in A, 5.80 (SD 2.07, range 3.77-9.32) in B and 12.22 (SD 2.84, range 8.11-15.81) in epithelioid MM. From comparing MA and RA with follow-up, it seemed that MM with a good prognosis (spindle A and a subgroup of B tumors, B1) exhibited the lowest MA and AR values, while MM with poorer clinical behavior (epithelioid and a second subgroup of spindle B MM, B2) showed the highest. No correlation was found between AgNORs and LTD. CONCLUSION: AgNORs may contribute to the prognostic evaluation of uveal MM.

Orbital dirofilariasis.

PURPOSE: To warn the ophthalmologist to consider orbital dirofilariasis in the differential diagnosis of inflammatory masses of the orbit. PATIENTS: Two cases with non-specific swelling and pain of the orbit who had a initial diagnosis of "inflammatory pseudotumor of the orbit". RESULTS: These are two new cases of orbital dirofilariasis. CONCLUSION: In the ophthalmic literature, the highest incidence of ocular dirofilariasis is reported in Italy. Since both these patients had no history of travel in endemic areas of Dirofilaria, this parasitic infection is presumably not rare in Italy and is probably underdiagnosed.

Warthin tumor of the lacrimal gland.

PURPOSE: To describe a case of Warthin tumor involving the lacrimal gland. METHODS: Case report. The patient underwent a lateral orbitotomy to remove a nodular lesion involving the lacrimal gland. The specimen was submitted for histopathologic examination. RESULTS: The lesion was characterized by epithelial columnar cells arranged in solid nests or lining cystic spaces containing an exudative fluid and by pronounced lymphoid infiltrate with focal follicular formation. CONCLUSIONS: To our knowledge, this is the first case of Warthin tumor involving the lacrimal gland. Management requires complete excision of the lesion.

Fine needle aspiration (FNA) biopsy of orbital masses: a critical review of 51 cases.

FNA biopsy of 51 orbital masses is critically reviewed. Aspiration was performed with a 23 G needle inserted by an ophthalmologist; the smears were prepared by a cytologist. Forty-two cases (83%) were correctly diagnosed as benign or malignant either with (68%) or without (15%) correct specification of the histology. There were two false-negative and seven inadequate cases. Immunocytochemical stains were performed in five cases using the following antibodies: L26 (Pan B), UCHL1 (Pan T), kappa and lambda immunoglobulin light chains (three cases) in order to distinguish inflammatory pseudotumours from low-grade non-Hodgkin's lymphomas. In two cases we used CAM 5.2 (a monoclonal cytokeratin cocktail) and vimentin to ascertain the epithelial origin of two metastatic tumours. In five other cases cytospins were not adequately cellular for immunocytochemistry. Insufficient material and one false-negative sample were obtained from very fibrotic lesions or from posteriorly located lesions. The results are discussed and compared with other series reported in the literature. Orbital FNA biopsy may be considered a useful tool in the diagnostic approach to orbital masses in which the relatively high number of inadequate aspirations is offset by a low cost-benefit ratio.

Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy.

Primary maxillary localization of Ewing's sarcoma is unusual. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. We report here two new cases of Ewing's sarcoma localized to facial bones, successfully treated by local high dosage radiotherapy and systemic chemotherapy. Our experience suggests that, especially for particular sites not suitable to radical surgery, radiation therapy can represent an effective tool to achieve local control of the tumor.

Dermoid cysts: 16-year survey.

A total of 145 patients with orbital dermoid cysts examined at the Orbital Clinic, Institute of Ophthalmology, School of Medicine, University of Naples "Federico II" over a period of 16 years were reviewed. The orbital cysts were classified as exophytic and endophytic, according to their site of attachment in relation to the orbital rims. This classification can explain the different natural history of these lesions. The exophytic cysts growing externally are discovered in childhood, whereas the endophytic ones are discovered later in life when they produce bone damage, with or without invasion of the adjacent structures.

Orbital dermoid cysts located within the lateral rectus muscle.

BACKGROUND: Two patients, a 13-year-old girl and a 31-year-old man, had an orbital dermoid tumor located in the lateral rectus muscle. This unusual location of dermoid tumors has not been reported previously. METHODS: Computed tomography and magnetic resonance imaging showed a cystic mass within the belly of the lateral rectus in each patient. Surgical excision through a lateral orbitotomy showed a well-circumscribed mass surrounded by lateral rectus fibers. No periorbital attachment was noted. RESULTS: Results of histopathologic evaluations showed a dermoid cyst. Postoperatively, the diplopia and proptosis resolved. In one patient, an unusual subconjunctival deposition of fat droplets was seen. CONCLUSION: The findings in patients demonstrate an unusual presentation of dermoid cysts and make an addition to the differential diagnosis of enlarged extraocular muscles.

An unusual orbital lesion: hepatoma metastatic to the orbit.

The authors describe a case of histologically proven orbital metastasis from a hepatic carcinoma in an 85-year-old patient with rapid onset of proptosis and visual loss. The clinical diagnosis of orbital metastatic lesion was established by standardized echography and CT scan. To the best of our knowledge, this is only the third histologically proven case of hepatoma metastatic to the orbit.

Optic nerve involvement in acute lymphoblastic leukemia.

Leukemic infiltration of the optic nerve is rare. We describe the diagnostic assessment and the outcome in two adult patients suffering from acute lymphoblastic leukemia with T phenotype. In both cases the leukemic involvement of the eye was observed as an isolated extramedullary relapse followed after several months by hematological relapse. CT and MRI scans were negative, while an A-scan echography of the eye clarified the diagnosis. Early radiotherapy caused recovery of visual acuity in one case. A-scan echography is the most sensitive investigation for the early recognition of ocular localization in acute leukemias.

Comparison between echographic and histological findings in choroidal malignant melanomas.

Echography is crucial in making a diagnosis of malignant melanoma when ocular media are opaque. Even with clear ocular media, standardized echography is important to confirm or to rule out a malignant melanoma in the eye. In this study the reflectivity in 27 cases of malignant melanomas has been evaluated and compared to cell type, pigmentation and number and size of blood vessels. No significant correlations have been found.