A case report of ultrasonographic findings in bilateral diffuse Uveal melanocytic proliferation.

BACKGROUND: To report undescribed characteristics of patients with bilateral diffuse uveal melanocytic proliferation (BDUMP) on ultrasound biomicroscopy (UBM) and high-frequency B-scan ultrasonography. CASE PRESENTATION: Two of four participants presented with worsening bilateral vision after previously diagnosed primary pulmonary or ovarian carcinoma. The other two patients were diagnosed with lung carcinoma after presentation with BDUMP. All patients had ciliary body nevi-like lesion in combination with iris or ciliary body cysts, and uveal thickening on UBM. Focally elevated choroidal nevi-like lesion and exudative retinal detachment with choroidal thickening were detected with B-scan ultrasonography. CONCLUSIONS: Our case series demonstrates the uveal characteristics of patients with BDUMP based on high-frequency B-scan ultrasonography and UBM. Ultrasonographic findings are crucial in the diagnosis of BDUMP because it is occult in nature.

Case report: imaging and treatment of ophthalmic manifestations in oculodentodigital dysplasia.

BACKGROUND: Diagnostic and surgical management of severe chronic angle- closure glaucoma secondary to ciliary body cysts can be difficult to manage in a patient with oculodentodigital dysplasia. CASE PRESENTATION: A 6-year old girl with oculodentodigital dysplasia, with progressive chronic angle- closure glaucoma secondary to ciliary body cysts presented to our clinic. The initial examination revealed counting fingers vision in the left eye. Intraocular pressure (IOP), as assessed by tonopen, was 31 mm Hg. Ultrasound biomicroscopy revealed ciliary body cysts in the left eye, and gonioscopy confirmed chronic angle closure. A tube shunt was placed to control the elevated IOP. A year after her tube shunt placement in the left eye, ultrasound biomiscropy was performed on her right eye and showed no ciliary body cysts. Gonioscopy in the right eye revealed an open angle to the ciliary body band. Subsequent serial gonioscopy every 3 months showed gradual narrowing of the right eye angle and finally three-and-a-half years after tube placement of the left eye, her right eye IOP became uncontrolled with medications alone and a tube shunt was similarly placed in the right eye. Intraoperative ultrasound biomicroscopy performed at the time of the right eye tube shunt revealed extensive ciliary body cysts in the right eye. Her IOP in both eyes have been well controlled since the placement of tube shunts. CONCLUSIONS: This is one of the first reported cases of severe chronic angle- closure glaucoma secondary to ciliary body cysts in a patient with oculodentodigital dysplasia. We believe that early screening for ciliary body cysts is important in patients with oculodentodigital dysplasia.

Quantitative measurements of the ciliary body in eyes with malignant glaucoma after trabeculectomy using ultrasound biomicroscopy.

PURPOSE: To evaluate and compare the structural differences of the ciliary body in eyes with and without malignant glaucoma. DESIGN: Cross-sectional study. PARTICIPANTS: Twenty-seven consecutive patients diagnosed with malignant glaucoma in 1 eye after trabeculectomy were recruited. They were all originally diagnosed with primary angle closure (PAC) or PAC glaucoma (PACG). Twenty-seven PAC/PACG eyes of 27 patients who had undergone uneventful trabeculectomy in the same period were also recruited. They were comparable with the fellow eyes of the malignant glaucoma patients in terms of surgical type, glaucoma type, and stage. METHODS: A-scan ultrasonography and ultrasound biomicroscopy measurements were performed on the eyes with malignant glaucoma, the fellow eyes of the patients with malignant glaucoma, and the matched eyes. MAIN OUTCOME MEASUREMENTS: Ciliary body parameters included maximum ciliary body thickness (CBTmax), ciliary body thickness at the point of the scleral spur (CBT0) and 1000 µm from the scleral spur (CBT1000), anterior placement of the ciliary body (APCB), and the trabecular-ciliary process angle (TCA). Biometric measurements including axial length, central anterior chamber depth (ACD), pupil diameter (PD), anterior chamber width, and lens vault (LV) were also recorded. RESULTS: Average CBTmax were 0.545±0.088 (mean ± standard deviation), 0.855±0.170, and 0.960±0.127 mm in eyes with malignant glaucoma, their fellow eyes, and the matched eyes, respectively. Average APCB were 0.860±0.176, 0.608±0.219, and 0.427±0.139 mm, respectively. Average TCA were 18.49±4.12, 41.79±17.27, and 48.53±10.38 degrees, respectively. The CBTmax, CBT0, CBT1000, and TCA were smaller, whereas APCB was larger in eyes with malignant glaucoma compared with their fellow eyes (P < 0.01). The fellow eyes had larger APCB and smaller CBTmax and CBT0 than the matched eyes (P < 0.05). The ACD, anterior chamber width, and PD were smaller, whereas LV was larger in eyes with malignant glaucoma compared with their fellow eyes (P < 0.05). No differences were found in the ACD, anterior chamber width, PD, or LV between the fellow eyes of malignant glaucoma and matched eyes (P > 0.1). CONCLUSIONS: The ciliary bodies were thinner and more anteriorly rotated in eyes with malignant glaucoma as well as in their fellow eyes, which may be the predisposing factor for malignant glaucoma.

A modified ultrasound-guided surgical technique for the management of the uveal effusion syndrome in patients with normal axial length and scleral thickness.

PURPOSE: The purpose of this study was to describe a modified surgical technique for the management of the uveal effusion syndrome (UES). METHODS: A consecutive interventional case series of six eyes with UES is reported. The diagnosis of the UES was based on detailed ophthalmic examination, fluorescein angiography, B-scan ultrasonography, biometry, and magnetic resonance imaging. All eyes underwent an ultrasound-guided placement of the sclerostomies subjacent to the area of maximal choroidal swelling using a scleral punch without scleral flaps or vortex vein decompression. RESULTS: All patients were men with a mean age of 53 years. The mean postoperative follow-up was 16.25 months. Five eyes had normal axial lengths (22.54-23.05 mm) by ultrasound and normal sclera thickness on magnetic resonance imaging. One eye had a shorter axial length (21.65 mm) and mild scleral thickening on magnetic resonance imaging. All six eyes had anterior peripheral choroidal swelling. Three eyes had associated serous retinal detachment, and three eyes had acute appositional angles. After surgery, five eyes had total resolution of the peripheral choroidal swelling and retinal detachment or normalization of the angle. One eye had partial resolution of the retinal detachment. Of the three eyes with retinal detachment, two eyes experienced improvement in visual acuity after surgery. No complications were noted. CONCLUSION: This modified ultrasound-guided surgical technique for sclerostomy placement seems to be effective in the management of the UES, including eyes with normal axial length and scleral thickness, a subset of the UES that has been previously reported not to respond to surgery.

Is preoperative ciliary body and iris anatomical configuration a predictor of malignant glaucoma development?

BACKGROUND: To investigate anatomical configuration of ciliary body and iris using ultrasound biomicroscopy as a predictor of malignant glaucoma development. DESIGN: Retrospective study in a tertiary care hospital. PARTICIPANTS: Cohort of 31 consecutive patients diagnosed with post-surgical malignant glaucoma. METHODS: Anterior chamber angle, iris and ciliary body configuration of involved eyes that had ultrasound biomicroscopy evaluation prior to the malignant glaucoma onset were evaluated. In cases with no presurgical ultrasound biomicroscopy exam of the involved eye, images from the fellow eye (imaged within 6 months) were analysed. MAIN OUTCOME MEASURES: Qualitative parameters. RESULTS: Thirty-one eyes (31 patients) had confirmed malignant glaucoma between 1996 and 2008. Most patients were women (65%) and had an anatomical narrow angle or angle-closure glaucoma (77%). Mean intraocular pressure at diagnosis was 30.4 ± 13.5 mmHg. The most common operation was trabeculectomy with mitomycin C (55%, 17/31 eyes), combined (3/17) or not (14/17) with cataract extraction and intraocular lens implantation. Among these 31 cases, we were able to evaluate the ultrasound biomicroscopy images of 13 patients (13 eyes) including involved eyes imaged prior to the malignant glaucoma onset or eligible fellow eyes. A narrow angle with or without iridotrabecular contact was found in all eyes. A large and/or anteriorly positioned ciliary body associated with an iris root angulating forward and centrally, revealing a plateau iris configuration, was noticed in 85% (11/13) of these eyes. CONCLUSION: Identification of plateau iris configuration by ultrasound biomicroscopy should be considered as a possible predictor of post-operative malignant glaucoma development.

Ciliary body cysts in neurofibromatosis: a new coexistence?

PURPOSE: Neurofibromatosis 1 (NF1) is an autosomal dominant, multisystem disorder that also effects the eye. Herein, we aimed to investigate the posterior iris surface and ciliary body morphology of NF1 patients by ultrasonic biomicroscopy (UB). MATERIALS & METHODS: Nine consecutive unrelated subjects with NF1, and as a control group 25 healthy subjects, were included in the study. All patients underwent ophthalmic examination including visual acuity testing, slit-lamp biomicroscopy, tonometry, gonioscopy (Schaffer classification), and dilated ophthalmoscopy, UB. RESULTS: Mean age was 35.1 ± 16.2 (range, 11-57) and 34.5 ± 15.6 (range, 9-60) for NF1 and control groups respectively (p>0.05). Lisch nodules were present in 16 of 18 eyes (88.8%) in NF1 group. Fundoscopic examination of the control group and 15 eyes of NF1 (83.3%) patients was normal, whereas hypoplastic and tilted optic nerve were present in three eyes, and temporally-located bone-spicule-like lesions was present in one eye of the NF1 group. UB revealed ciliary body cyst in 77.7% (14/18) of the eyes among NF1 group, and 8% (4/50) among control group (p<0.05). The mean size of the cysts were 520 ± 191 µ (range, 220-860 µ) and 495 ± 231 µ (range, 300-830 µ) at NF1 and control groups, respectively. Gonioscopic evaluation revealed that 55% of the NF1 patients have an unoccludable anterior chamber angle (Grade 3 or 4), 45% occludable angle (Grade 1 or 2), and 78% irregular pigment patches. However, occludable angle rate was just 4% in the control group, and none of the patients had irregular pigment patches. CONCLUSION: The coexistence of ciliary body cysts and NF1, and the effect of these cysts in the eye should be enlightened with further studies.

Low intraocular pressure resulting from ciliary body detachment in patients with myotonic dystrophy.

PURPOSE: To investigate why myotonic dystrophy type 1 (DM1) patients have low intraocular pressure (IOP). DESIGN: Prospective, comparative case series. PARTICIPANTS: One hundred two eyes of 51 patients with DM1 (age range, 21-64 years) and 44 eyes of 22 healthy subjects of similar age (21-64 years). METHODS: All participants underwent IOP measurement with Goldmann applanation tonometry and an in vivo examination of the ciliary body with a 35-MHz high-resolution B-scan. The findings were compared between the 2 groups. In both groups, only patients with no history of ocular trauma or surgery were included. The differences were evaluated using the unpaired Student t test. MAIN OUTCOME MEASUREMENTS: Intraocular pressure, central corneal thickness (CCT), and echographic evidence of ciliary body detachment. RESULTS: The mean ± standard deviation (SD) IOP in patients with DM1 was 10.9 ± 3.1 mmHg and that in the control patients was 15.4 ± 2.2 mmHg, a difference that reached significance (P<0.01). The mean ± SD CCT (measured at the pupillary center) was 574.4 ± 37.9 µm in the patients with DM1 and 557.8 ± 39.2 µm in the controls (P = 0.02). Detachment of the ciliary body was identified in all DM1 subjects. Size was variable and the detachment involved 1 or more quadrants. The number of quadrants affected by the detachment was not correlated with the IOP (R(2) = 0.088) or the size of the CTG expansion. No detachments were found in the healthy controls. CONCLUSIONS: Detachment of the ciliary body may explain the low IOP values in patients with DM1. The finding of a ciliary body detachment in an individual who has not had recent eye surgery or trauma raises the possibility of a DM1 diagnosis.

[Ultrasonographic visualization of ciliary pars plana cysts].

On a basis of detailed analysis of ultrasound biomicroscopy (UBM) scanograms of 617 patients (823 eyes) ciliary pars plana cysts were found in 8.9% (55 patients, 66 eyes). The main types of ciliary pars plana cyst were revealed: solitary isolated and multiple (isolated and confluent). The results of the study confirmed topographic and morphologic correlation of ciliary pars plana cysts and peripheral retinoschisis. Authors demonstrated the value of UBM for visualization not ciliary pars plana cysts only but oral and peripheral retinal degenerative changes as well, that can lead to retinoschisis and retinal detachment.

Ciliary Body Mass as a Feature of Ocular Sarcoidosis.

Importance: Ocular sarcoidosis may be the primary and only clinical manifestation of sarcoidosis, and irreversible loss of visual acuity may occur from delayed treatment. Thus, early diagnosis is imperative to preserve visual acuity. Establishing the diagnosis of sarcoidosis in cases manifesting only in the eye may require a diagnostic biopsy, which may prove challenging in the absence of available ocular surface, lacrimal gland, and systemic tissue for biopsy. Objective: To describe diffuse or segmental ciliary body mass as a clinical sign and tissue source for biopsy in the diagnosis of ocular sarcoidosis. Design, Setting, and Participants: This case series captures 3 cases from a university-based ocular oncology and uveitis practice. All 3 patients presented with uveitis and a diffuse or segmental ciliary body mass, which was identified via ultrasonographic biomicroscopy. Main Outcomes and Measures: Clinical, ancillary, and histopathological ocular findings in the 3 patients. Results: The patients were a 52-year-old white woman, a 42-year-old African American man, and an 81-year-old white man. Two individuals had unilateral involvement, and 1 had bilateral involvement. All 3 individuals presented with uveitis. Diffuse, circumferential ciliary body masses were observed in 2 patients. A localized, nodular ciliary body mass on the temporal quadrant with superior and inferior extensions was observed in 1 patient. Moderate to high internal reflectivity was observed on ultrasonographic biomicroscopy in all 3 individuals. Biopsies of the ciliary body masses were diagnosed as sarcoidosis in all 3 patients. Conclusions and Relevance: These findings support that diffuse or segmental ciliary body mass may serve as a clinical sign and tissue source for biopsy in the diagnosis of ocular sarcoidosis, although the frequency of this cannot be determined from 3 cases. To our knowledge, such cases all demonstrate a uveitic component; whether a ciliary body mass caused by sarcoidosis can occur in the absence of uveitis remains unknown.

Transient Myopia Secondary to Supraciliary Effusion: Unusual Complication After an Uneventful Prolene Gonioscopy-assisted Transluminal Trabeculotomy.

PURPOSE: The purpose of this study was to report 2 cases experiencing a transient decrease in their vision due to the development of acute myopia after an uneventful prolene gonioscopy-assisted transluminal trabeculotomy (GATT) surgery. METHODS: A 52-year-old woman with primary open-angle glaucoma and an 8-year-old boy with juvenile glaucoma underwent uneventful GATT surgery. RESULTS: On the postoperative first day, both patients had mild shallow anterior chamber and intraocular pressures measured as 19 and 16 mm Hg, respectively. Both patients had myopia measured as -4.5 and -6.0 D, respectively. Fundus examination was unremarkable in the first patient, whereas it showed bilateral optic disc cupping and retinal nerve fiber layer atrophy in the second patient. In the first patient, supraciliary effusion was detected by using ultrasound biomicroscopy. Ultrasound biomicroscopy could not be performed in the second patient, but ocular ultrasonography revealed a thin layer of suprachoroidal fluid. Myopia was completely regressed, and visual acuity increased within 1 week of the follow-up in both patients. At the sixth month of the follow-up, intraocular pressures in both patients were under control without any antiglaucoma medications. CONCLUSION: Transient decrease in vision after the GATT surgery might occur secondary to supraciliary effusion leading to acute, transient myopia.

Intravitreal triamcinolone reduces the morphologic changes of ciliary body after pars plana vitrectomy for retinal vascular diseases.

PURPOSE: To investigate the early postoperative changes in ciliary body after pars plana vitrectomy (PPV) for retinal vascular disease and to determine whether intravitreal triamcinolone acetonide (IVTA) affects postvitrectomy changes of the ciliary body. DESIGN: Prospective interventional case-control study. METHODS: We recruited 46 patients who underwent uncomplicated PPV for retinal vascular disease (35 with proliferative diabetic retinopathy (PDR) and 11 with branch retinal vein occlusion) and divided the patients into two groups according to the use of IVTA at the end of the PPV. The morphologic changes of the anterior segments were measured by ultrasound biomicroscopy (UBM) one day before and one day, two days, three days, five days, two weeks, one month, and two months after the PPV. The main outcome measures were the thickness and area of the ciliary body, the frequency of supraciliary effusions (SEs), angle-opening, and anterior chamber depth (ACD). We compared the UBM parameters between the two groups. RESULTS: The thickness and area of the ciliary body significantly increased from day 1 to day 5 postoperatively. Nineteen of 46 eyes had SEs that were frequently associated with divergent alterations of postoperative intraocular pressure (IOP). The angle-opening and ACD were significantly decreased and dependent upon the findings of ciliary body thickness and SEs. The degree of the morphologic changes of the ciliary body and the frequency of SEs were significantly lower and of shorter duration in the IVTA group compared to the non-IVTA group. CONCLUSIONS: The PPV for retinal vascular disease induces the morphologic changes of the ciliary body associated with postoperative abnormalities in IOP during the early postoperative period. Administration of IVTA at the end of the PPV effectively reduces or shortens the duration of the postvitrectomy changes of the ciliary body.

Ultrasound biomicroscopy after vitrectomy in eyes with normal intraocular pressure and in eyes with chronic hypotony.

PURPOSE: To analyze the ultrasound biomicroscopy (UBM) features of eyes with chronic hypotony after pars plana vitrectomy (PPV) and compare them with the UBM features of eyes with normal intraocular pressure (IOP) after PPV. METHODS: This comparative (nonrandomized) interventional study included 64 eyes of 64 patients who underwent PPV. Group 1 included 20 eyes with less complex vitreoretinal diseases (VRD) that presented with normal IOP after PPV. Group 2 included 44 eyes with severe proliferative vitreoretinopathy (PVR) managed with PPV and silicone oil tamponade that presented with complete retina reattachment and chronic ocular hypotony. UBM was performed to study the anterior segment, ciliary body (CB), and peripheral retina. The UBM findings of the two groups were compared. RESULTS: In Group 1, 19/20 eyes presented with no CB alterations detected by UBM. In Group 2, 43/44 eyes presented with CB alterations that included tractional CB detachment (n=16); exudative CB detachment (n=11); tractional CB detachment/CB atrophy (n=7); CB hypotrophy (n=5); tractional CB detachment/exudative CB detachment (n=3); and CB edema (n=1). There was a strong relationship between IOP and CB findings revealed by UBM. CONCLUSIONS: Eyes with hypotony following PPV have CB abnormalities that can be detected by UBM. These CB alterations were not found in eyes with normal IOP after PPV in this series.

A case report on the change of the refractive power after a blunt trauma.

PURPOSE: To determine the pathogenesis of transient myopia after blunt eye trauma. METHODS: In one patient, the refraction of both eyes (the left eye was injured, but the right eye was not) was measured with an autorefractometer. The cycloplegic refraction was measured at the early stage of trauma and again 3 months after the blunt eye injury. The angle and depth of the anterior chamber, the ciliary body, and the choroids were examined by ultrasound biomicroscopy (UBM) over 3 months. The depth of the anterior chamber, the thickness of the lens, and the axial length were measured by A-scan ultrasonography in both eyes. During the 3 months after the injury, we made comparisons between the menifest and the cycloplegic refractions, the depths of anterior chambers, the thickness of the lenses, the axial lengths, and the UBM-determined appearances of the angles and depths of the anterior chambers, the ciliary bodies, and the choroids in both eyes. RESULTS: We suspect that the depth reduction in the anterior chamber, the increase in anterior to posterior lens diameter, and the edema in the ciliary body are all related to the change in the refractive power following the blunt trauma. CONCLUSIONS: Ultrasound biomicroscopy (UBM) and ultrasonography of the anterior segment in the eye may be helpful to diagnose and confirm changes in the refractive power that occur after trauma.

Cyclodialysis cleft secondary to removal of an anterior chamber phakic intraocular lens.

We present a patient who had decreased visual acuity and hypotony in the left eye 2 months after removal of an anterior chamber phakic intraocular lens (pIOL). Gonioscopy demonstrated a cyclodialysis cleft at the 6 o'clock position in the region of the IOL footplate, which was confirmed by ultrasound biomicroscopy. A cyclodialysis cleft formation is one possible complication of pIOL removal. Careful gonioscopy evaluation before removal of pIOLs should be mandatory to assess the amount of fibrosis and the presence of synechia between the IOL and the surrounding tissues.

Multiple bilateral iridociliary cysts: ultrasound biomicroscopy and clinical characteristics.

BACKGROUND: To describe the utility of ultrasound biomicroscopy in diagnosing multiple bilateral iridociliary cysts and to determine the clinical significance of this condition. METHODS: A retrospective review of 73 patients referred for ultrasound biomicroscopy who were found to have bilateral iridociliary cysts. RESULTS: Seventy-three patients were identified who demonstrated 2 or more cysts in 1 eye and at least 1 in the other with ultrasound biomicroscopy. A solitary iris elevation was the most common reason for referral (84.9%). There were 44 patients for whom sufficient follow-up data were available, with a mean follow-up of 40.4 months. Clinical sequelae included 1 case of sectoral cataract (2.3%) and 4 cases of cyst-related glaucoma (9.1%). INTERPRETATION: Multiple bilateral iridociliary cysts is a common condition with occasional clinical significance. Ultrasound biomicroscopy is a valuable technique in diagnosing this condition. Patients who have significant angle compromise should be followed and treated as required for glaucoma.