External limiting membrane and visual outcome in macular hole repair: spectral domain OCT analysis.

PURPOSE: To investigate the relationship between postoperative visual acuity and integrity of the external limiting membrane (ELM) and inner segment-outer segment (IS-OS) junction layers, using spectral domain optical coherence tomography (SD-OCT), in eyes with macular holes (MHs) following surgical repair. METHODS: Medical charts of MH-operated cases were retrospectively identified and reviewed. The primary outcome measures were best-corrected visual acuity (BCVA) and the status of the ELM and IS-OS lines, using SD-OCT, at 6 weeks and 6 months postoperatively. RESULTS: Sixty-two eyes of 62 patients were included. At 6 weeks following surgery, out of 56 (90.3%) eyes with successful MH closure: 0 eyes showed the combination of disrupted ELM and continuous IS-OS layers; 7 eyes (12.5%) demonstrated continuity of both ELM and IS-OS (ELM(c)/IS-OS(c) group); 29 eyes (51.8%) had continuous ELM with discontinuous IS-OS layers (ELM(c)/IS-OS(d) group); and 20 eyes (35.7%) had discontinuities in both the layers (ELM(d)/IS-OS(d) group). The ELM(d)/IS-OS(d) group had the lowest visual gain at 6 months (P = 0.03). At 6 months, a restoration of the integrity of IS-OS layer was observed in 51.7% eyes in the ELM(c)/IS-OS(d) group and in 5% in the ELM(d)/IS-OS(d) group (P = 0.001). CONCLUSIONS: When both ELM and IS-OS layers showed disruptions 6 weeks postoperatively, a significantly worse BCVA was measured at 6 months, compared with the eyes with only IS-OS disruptions, detected 6 weeks following surgery. The integrity of the ELM layer appears to be a critical factor for the restoration of the photoreceptor layer and for predicting a successful visual outcome following MH repair.

Correlation between fluorescein angiography and spectral-domain optical coherence tomography in the diagnosis of cystoid macular edema.

AIMS: To compare the ability to detect cystoid macular edema (CME) and its late complications between spectral-domain optical coherence tomography (SD-OCT) and fluorescein angiography (FA). METHODS: Retrospective, observational, case series. 85 Eyes who had FA and SD-OCT performed on the same day at first visit and/or at follow-up visits were included. FA and SD-OCT images were evaluated for the evidences associated with CME and other structural changes of macula. FA and SD-OCT images were then superimposed to determine the relationships of diagnostic features between the two images. Main outcome measure was the correlation between FA and SD-OCT findings of macula in patients with CME. RESULTS: The common causes of CME in our study were retinal vein occlusion (RVO, 63%), diabetic retinopathy (DR, 21.18%) and posterior uveitis (3.53%). CME associated with RVO, age-related macular degeneration and DR were missed by FA in 18.52%, 33.33% and 33.33% of cases, respectively. Subretinal fluid was undetectable by FA in 54.55%, which mainly were in the RVO group. SD-OCT gave earlier CME diagnosis than FA in three (3.53%) eyes. Residual CME at follow-up visits were missed by FA in one (1.18%) eye. Late complications of long-standing CME (secondary macular hole (two eyes), secondary subretinal fluid (five eyes), retinal pigment epithelium detachment (one eye) and photoreceptor atrophy (one eye)) were detectable only by SD-OCT. CONCLUSIONS: SD-OCT demonstrated greater sensitivity than FA in detecting CME, particularly those associated with RVO, DR and age-related macular degeneration. SD-OCT was also more sensitive than FA for detecting subretinal fluid and late complications of long-standing CME.

New patterns of retinal collateral circulation are exposed by a retinal functional imager (RFI).

AIMS: To describe different patterns of retinal collateral circulation observed in normal subjects and in patients with a variety of ocular diseases during studies with the retinal functional imager (RFI). METHODS: Normal subjects and patients with various ocular diseases underwent retinal blood flow imaging using the retinal functional imager. RESULTS: Twenty-eight eyes of 21 patients constituted the study population (eight eyes of six normal subjects and 20 eyes of 15 patients with different ocular diseases). Four patterns of retinal collateral circulation have been recognised: (1) looped collateral pattern-this arterovenous anastomotic vessel is characterised by a link between the endings of the adjacent artery and vein, and has an appearance of a loop; (2) vertical collateral pattern-this anastomotic vessel is characterised by a connection between superior and inferior vascular systems (arterial or venous), and in its course this collateral vessel crosses the horizontal raphe; (3) H-shaped collateral pattern-this arterovenous anastomotic vessel is characterised by a connection between two adjacent vessels (artery and vein), but, unlike anastomotic vessel of the "looped" pattern, this vessel connects the middle parts of the vessels and not the endings of these vessels; (4) cilioretinal-retinal collateral pattern. This anastomotic vessel is characterised by a link between the cilioretinal artery and retinal arterial circulation. CONCLUSIONS: The findings of this study shed a different light on the retinal circulation and have demonstrated new patterns of retinal collateral circulation evident from RFI imaging. These patterns appear to be present in normal subjects as well as in patients with various ocular diseases.

A comparison between microperimetry and standard achromatic perimetry of the central visual field in eyes with glaucomatous paracentral visual-field defects.

BACKGROUND AND AIMS: The aim was to correlate retinal sensitivity as determined by scanning laser ophthalmoscope microperimetry (SLO-MP) in glaucomatous eyes with paracentral visual field (VF) defects detected by standard automated perimetry (SAP). METHODS: Twenty eyes with glaucomatous optic neuropathy and an SAP VF defect involving the central 16 test points (at least one point with p<1% in the 24-2 VF) were enrolled. Eyes with diseases other than glaucoma were excluded. All patients underwent SLO-MP and SAP of the central 10 degrees . Results from each eye were divided into four quadrants for analysis. Normal and abnormal quadrants by SAP were compared with the corresponding normal and abnormal quadrants by SLO-MP. Regression analysis was used to correlate the mean threshold values (dB) of SLO-MP and SAP in each quadrant. Macular optical coherence tomography (OCT) was performed when there was a disagreement between functional tests. RESULTS: The mean age and VF mean deviation were 60.8 (13.4) years and -7.3 (6.1) dB, respectively. There was a significant correlation between SLO-MP and SAP results in all quadrants (r(2)> or =0.68, p<0.001). All abnormal SAP quadrants had a corresponding abnormal SLO-MP quadrant. However, 21% of the normal SAP quadrants had an abnormal corresponding microperimetry result; a corresponding significant reduction in total macular thickness measured by OCT was present in 75% of these quadrants. CONCLUSIONS: Macular sensitivity evaluated by SLO-MP correlates significantly with SAP paracentral VF defects. SLO-MP detected retinal sensitivity reduction in areas of OCT structural damage with normal SAP and suggests that subtle paracentral functional deficits may be present in many more eyes with established glaucoma than generally assumed.

Evaluation of central serous retinopathy with en face optical coherence tomography.

BACKGROUND: The diagnosis of idiopathic central serous retinopathy (CSR) is usually based on biomicroscopy and fluorescein angiography (FA). The optical coherence tomography (OCT) ophthalmoscope produces en face OCT scans (OCT C-scans) and provides additional information not readily available by conventional imaging techniques. The authors describe the characteristic features observed in patients with a clinical diagnosis of CSR using the OCT ophthalmoscope. METHODS: 38 eyes with a clinical diagnosis of CSR, seen at the Academic Medical Centre (Amsterdam, Netherlands) and the New York Eye and Ear Infirmary (New York, USA) between August 2002 and March 2004, were evaluated with standard digital FA and scanned with the OCT ophthalmoscope. RESULTS: Nine of 38 eyes had no serous neurosensory detachment (inactive CSR) when scanned with the OCT ophthalmoscope. Characteristics for active CSR (n=29) were large neurosensory detachment (23/29), subretinal hyper-reflective depoits (20/29), and pigment epithelial detachment (15/29). One third of the patients, either active or inactive, had multiple small pigment epithelial detachments located both within and outside the neurosensory detachment. CONCLUSION: The OCT ophthalmoscope provides complementary morphological information on patients with CSR. The presence of more diffuse retinal pigment epithelium (RPE) changes lends further support to the concept that CSR is a diffuse rather than localised RPE anomaly.

Three-dimensional ultrasound for the measurement of choroidal melanomas.

OBJECTIVE: To evaluate the reliability of 3-dimensional ultrasound (3D-US) for the measurement of choroidal melanomas. DESIGN: Retrospective case series. PARTICIPANTS: Forty-two consecutive cases of choroidal melanoma imaged with 3D-US. METHODS: Tumor measurements obtained with ophthalmoscopy, transillumination, standard ultrasound techniques, 3D-US, and pathological studies. Tumor diameters, heights, and volumes were compared. Our 3D-US tumor measurement techniques were tested for intraobserver and interscan reproducibility. RESULTS: Fifty 3D-US images were studied. The 3D-US tumor measurements were found to be reproducible (height coefficient of variation [CV] < or = 3%; diameter CV < or = 9.7%; volume CV < or = 13.2%). There was significant correlation with the usual methods of tumor measurement (diameter r = 0.76; height r > or = 0.98). Significant differences were found between measurements at pathological examination, as compared with both 2-dimensional and 3D-US height measurements (range, 0.73-0.83 mm). This finding was thought to be due to specimen shrinkage. Three-dimensional ultrasound was found to be at least as reproducible as clinical examination and standard ultrasound techniques used for measurement of diameter and height of choroidal melanomas. It was our impression that the 3D-US volume measurements accounted for the geometry of the tumor better than volume estimates calculated from basal area and tumor height. CONCLUSIONS: Three-dimensional ultrasound measurements of choroidal melanoma were reproducible, correlated well with other tumor measurement techniques, and can be used for measurement of choroidal melanomas.

Glaucoma drainage tube kink after pars plana insertion.

PURPOSE: To describe focal obstruction of drainage tubes by kinking at the scleral entry site after pars plana insertion. METHODS: Case study. RESULTS: Three eyes of three patients underwent uncomplicated placement of a Baerveldt implant into the vitreous cavity at the time of pars plana vitrectomy. Intraocular pressure remained increased after the procedure without evidence of flow. Surgical exploration and modification of the tube placement resulted in immediate intraocular pressure reduction. Compression of the tube at the scleral entry site was confirmed intraoperatively in all eyes by ultrasound biomicroscopy. CONCLUSION: Kinking of the tube at its scleral entry site should be recognized as a possible cause of increased intraocular pressure without bleb formation after pars plana insertion of a glaucoma drainage implant.

Diode-light transillumination for ophthalmic plaque localization around juxtapapillary choroidal melanomas.

PURPOSE: An evaluation of plaque-mounted diode-light transillumination (DLT) for localization of episcleral plaques beneath juxtapapillary tumors. METHODS AND MATERIALS: Two patients scheduled for radiotherapy for juxtapapillary melanomas were offered DLT as an additional method of ophthalmic plaque localization. Plaques were constructed by affixing 4 non-heat producing, light-emitting diodes with their apertures flush with the episcleral outer surface of the plaque's rim. Bio-implantable epoxy was used to encapsulate the electronic components. Then the plaques were loaded with 103Pd seeds. After the eye-plaques were sewn to the episclera covering the base of the intraocular tumors; the diode-lights were illuminated, viewed and recorded. Photodocumentation of the relative position of the 4 lights around tumor's base was obtained in both cases. RESULTS: Digital images of plaque-mounted diode retro-transillumination were obtained. No evidence of diode-light toxicity was noted. Both tumors were found to be covered by the ophthalmic plaques. CONCLUSION: Juxtapapillary tumors are often difficult or impossible to visualize with standard transillumination techniques and have been associated with poor local control rates. We have developed plaque-mounted DLT in an effort to improve ophthalmic plaque localization. Retrobulbar transillumination was viewed by indirect ophthalmoscopy and recorded with video-imaging. This technique provides unique photographic documentation of episcleral plaque localization beneath juxtapapillary tumors.

Relapse of acute lymphoblastic leukemia in pregnancy: survival following chemoirradiation and autologous transfer of interleukin-2-activated stem cells.

Four cases of relapse of acute lymphoblastic leukemia (ALL) in pregnancy have been reported previously. During the past 2 decades, ALL has become curable in a majority of children, many of whom have entered their reproductive years. Thus, additional occurrences of relapsing ALL during pregnancy can be anticipated. We present the fifth case in the English-language medical literature of recurrent ALL in pregnancy. A 20-year-old woman with ALL experienced a relapse during the third trimester of her first pregnancy. Reinduction therapy was started with vincristine and prednisone and the baby was delivered 3 weeks later. Umbilical cord blood was collected and stored. The patient then received intensive chemotherapy with whole body radiotherapy and autologous peripheral blood stem cell rescue. The ALL has been in second remission for 22 months. Our patient is the only current survivor of a relapse of ALL during pregnancy. In addition, the collection of umbilical cord blood from a pregnant woman with leukemia has not been reported previously.

Plaque-mounted diode-light transillumination for localization around intraocular tumors.

OBJECTIVE: To evaluate the usefulness of plaque-mounted diode-light transillumination (DLT) for the localization of episcleral plaques around intraocular tumors. METHODS: A clinical case series was performed to create, evaluate, and modify diode-light plaque construction, application, and imaging. Eight patients with choroidal melanoma were offered DLT as an additional method of ophthalmic plaque localization. Plaques were constructed by affixing non-heat-producing, light-emitting diodes with their apertures flush with the episcleral outer surface of the rim of the plaque. A bioimplantable epoxy was used to encapsulate the electronic components. Radioactive DLT eye plaques were sewn to the episclera to cover the base of the intraocular tumors; then diode lights were illuminated, viewed, and recorded. Thus, DLT was used to photographically document the relative position of the eye plaque covering the tumor base. The use of DLT also permitted a subjective evaluation of the contact (plaque contact) of each light with the sclera. RESULTS: Still and video images of plaque-mounted diode retro-transillumination were obtained, and no evidence of toxic effects of diode light were noted. CONCLUSIONS: Small posterior melanomas are difficult to visualize with standard transillumination techniques and are associated with poor local control. To improve and document plaque placement, we developed plaque-mounted diode lights for retrobulbar transillumination. This technique provides unique photographic documentation of episcleral plaque localization beneath intraocular tumors.

Three-dimensional ultrasonography of choroidal melanoma: extrascleral extension.

PURPOSE: To describe the results of three-dimensional ultrasonography used to evaluate extrascleral extension of a choroidal melanoma. METHODS: Case report. The three-dimensional ultrasound system uses a 10-MHz B-mode transducer combined with a motorized rotating holder. The system acquires 180 sequential images that are stored and processed to create a three-dimensional block of the region of interest. RESULTS: Unique coronal and oblique perspectives were obtained from interactive manipulation of the three-dimensional reconstruction. Examination of the three-dimensional image allowed us to detect the transscleral uveal-orbital connection. Extrascleral melanomatous extension was confirmed on histopathologic examination. CONCLUSION: Three-dimensional ultrasonography is a promising imaging technique for evaluating melanomatous extrascleral extension.

Three-dimensional ultrasonography of choroidal melanoma: localization of radioactive eye plaques.

OBJECTIVE: To evaluate the use of 3-dimensional (3D) ultrasonography for the localization of episcleral eye plaques during the treatment of choroidal melanomas. METHODS: A series of 13 patients with choroidal melanoma were treated with radioactive palladium 103 seeds affixed into gold eye plaques. During surgery, 3D ultrasonography was performed with a commercially available system to evaluate the relative position of radioactive plaques secured beneath their intraocular tumors. This system consists of an automated, rotating, handheld, B-scan ultrasonographic probe operating at 10 MHz, a personal computer, and 3D imaging software. RESULTS: We measured the margins of the plaque extending beyond the tumor and the distance between the radioactive seeds and the tumor apex. We also evaluated the relationship between the plaque edge, the episclera, and the tumor's edges. While the plaques were well centered over the tumor in all cases, the plaque margins around the tumor were found to be variably sized. When comparing measurements taken at the time of plaque insertion with those taken at the time of plaque removal, we noted changes in the apical tumor height and in plaque centration. In the 1 patient with a juxtapapillary tumor, the posterior margin of the plaque was found to be displaced away from the sclera, or "tilted." CONCLUSIONS: Three-dimensional ultrasonography offers a new method for ophthalmic plaque localization. Unique perspectives can be visualized through the use of computer-aided 3D reconstructions that permit the assessment of the relative position of the plaque to the optic nerve and the measurement of the distance between the in vivo radioactive seed and the tumor apex. Our experience suggests that when compared with 2-dimensional ultrasonography, 3D ultrasonography offers new capabilities that can be used to improve plaque placement and radiation dose calculations.

Personal computer-based 3-dimensional ultrasound biomicroscopy of the anterior segment.

OBJECTIVE: To develop a practical, inexpensive system for 3-dimensional ultrasound biomicroscopic imaging of the anterior segment with a commercially available high-frequency ultrasound imager and a personal computer. METHODS: Sequential, high-frequency, ultrasound biomicroscopic images of the anterior segment were obtained with a motorized scanning control arm designed in our imaging laboratory. Images were acquired by a personal computer-based video capture device. Ultrasound slice data were then reconstructed as 3-dimensional volumetric images by a personal computer and commercially available software. RESULTS: Four 3-dimensional visualization formats were developed to enhance the clinical utility of high-frequency ultrasound. Rotational animation sequences were created that detailed the extent and anatomy of a filtering bleb, intraocular lens subluxation, focal angle closure from an iridociliary cyst, intraocular foreign bodies, and an iris tumor. CONCLUSIONS: Three-dimensional, high-frequency ultrasound of the anterior segment enhances our ability to visualize spatial relationships between adjacent anatomic structures. The low cost and ease of use of this system make widespread clinical application practical.

Human cyclic neutropenia transferred by allogeneic bone marrow grafting.

Human cyclic neutropenia shows many features in common with the animal model of cyclic neutropenia in grey collie dogs. Until now, however, evidence was lacking that cyclic neutropenia in man as in the dog is caused by a defect in a transplantable hematopoietic stem cell. A patient is presented who, while undergoing bone marrow transplantation as treatment for acute lymphoblastic leukemia in relapse, acquired cyclic neutropenia from her histocompatible sibling donor.

Acute lymphoblastic leukemia: the significance of nuclear convolutions.

Because of the significance attributed by some investigators to the presence of cells with convoluted nuclei in lymphoblastic lymphoma, and the absence of any information on the presence and significance of such cells in acute lymphoblastic leukemia (ALL), we reviewed the blood, bone marrow films, and clinical records of 101 children with ALL. We attempted to determine whether leukemic cells with convoluted nuclei can be recognized in such films, and if so, whether this observation has clinical significance. In fifty-seven of the patients the leukemic cells had convoluted nuclei. For the purposes of this study, our patients were divided into three groups: Group I, 44 patients without cells having convoluted nuclei: Group II, 30 patients with 10% or fewer convoluted nucleus cells (CNC); and Group III, 27 patients with more than 10% CNC. Clinical comparison of the three groups with respect to age and sex distribution, physical and hematologic findings at presentation, response to therapy and survival showed no significant differences. Patients with a mediastinal mass had a significantly shorter survival compared to those without a mediastinal mass, regardless of the presence or absence of CNC (p = 0.0001). Our results indicate that the nuclear convolutions can easily be recognized in blood or bone marrow films of patients with ALL, and that their presence has no provable clinical significance.

A family with acute leukemia, hypoplastic anemia and cerebellar ataxia: association with bone marrow C-monosomy.

The eldest brother in a sibship of five children died of acute myelogenous leukemia at 10 years of age. The second and third eldest brothers died of hypoplastic anemia at ages five and nine years, respectively. A surviving 6 year old brother, the proband of the study, has abnormalities that suggest a preleukemic state: mild pancytopenia, platelet dysfunction, immunodeficiency, and bone marrow hypoplasia with approximately 18 per cent blast forms. His 17 year old sister has a mild normochromic normocytic anemia. Cytogenetic studies revealed C-group monosomy in the bone marrows of the proband and the third brother (45, XY, -C); band studies demonstrated that a No. 8 chromosome was missing in the proband (45, XY, -8). At least four of the siblings and their father had cerebellar ataxia, and evidence of a small cerebellum at autopsy examination or by computerized axial tomography. The disorder in this family has major features of two autosomal recessive preleukemic diseases, ataxia-telangiectasia and Fanconi's anemia. However, these and other inherited conditions were excluded by clinical or laboratory criteria, and no environmental causes of the familial disorder were found. The constellation of abnormalities in the family may constitute a new genetic syndrome.