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Purpose: To compare two methods of measuring ocular torsion (the subjective Maddox rod [MR] test versus the objective disc-fovea angle [DFA] test) after macular translocation surgery., Methods: Ocular torsion was measured on consecutive patients after macular translocation at Duke University Eye Center between August 2001 and April 2002. Both MR and DFA measurements of torsion were made at the same clinic visit 4 to 8 weeks after the translocation surgery and again within 3 months after extraocular muscle surgery to decrease torsion. MR and DFA measurements were each performed by a separate examiner who was blinded to the results of the other method., Results: Thirty-five patients (35 eyes) were included for evaluation. Twenty-nine of these patients had intorsion measured by both MR and DFA after macular translocation but before extraocular muscle surgery (MR mean of 40.3 + 7.2 degrees v DFA mean of 47.0 + 7.9 degrees [P <.001]). The intrapatient reproducibility of the MR test was high (using four readings per session), with a mean coefficient variation of 4.8%. Twenty-five patients had residual torsion measured by both MR and DFA after extraocular muscle surgery (MR mean of 4.2 + 4.7 degrees v DFA of mean 4.8 + 7.0 degrees). There was good correlation between MR and DFA measurements of torsion (r(2) = 0.9)., Conclusions: DFA measurement correlates well with MR measurement of torsion in patients after full macular translocation. This study verifies the reproducibility of MR to measure large angles of torsion and offers DFA as a simple corroborative test for measuring ocular torsion in patients with poor vision or cooperation.

Background: To describe the clinical and laboratory features of Paget's disease of bone at the time of diagnosis., Patients and Method: This multicenter and retrospective study included 314 patients. Diagnosis was performed by means of characteristic radiological findings and typical bone scintigraphy. The variables analyzed included: epidemiological variables, cause of diagnosis, bone involvement, disease extension (Coutris index), complications, alkaline phosphate (AP) levels and disease activity (Renier index)., Results: There were 159 (50.5%) males and the mean age was 64.9 years (SD 12.6). Diagnosis was casual in 228 (72.6%) patients. Polyostotic involvement was detected in 201 (63.9%) patients. More common locations were pelvis, skull and lumbar spine. Sacrum was more frequently involved in men than in women (p < 0.05), whereas skull involvement was more common in women (p < 0.05). The number of bones involved was 3.1 SD 3 and the percentage of skeletal involvement was 8.7 % (SD 6.5). One hundred ninety eigth (63%) patients had complications. The disease was active in 242 (77%) patients with a mean AP value of 377 IU/L (SD 493); the activity of the disease measured by the Renier index was 34 (SD 46); the activity was greater in the skull and the humerus., Conclusions: Paget's disease of bone is usually asymptomatic. Polyostotic involvement and activity are common at the time of diagnosis. Knowledge of the extension and activity by means of a mathematical model may aid to make therapeutical decisions.

Aseptic diskitis is relatively common. Several rheumatic diseases involving the spine may have this complication. As this condition mimic infectious diskitis, it is important to recognize it. Clinically, it is characterised by vertebral pain of an inflammatory nature, occasionally accompanied by fever and an increase in the erythrocyte sedimentation rate. Radiologically, the decrease in the articular space and the irregularity of the vertebral plates are of particular importance. Although uncommon, it is necessary to bear in mind this possibility when the etiological search for a possible infectious diskitis proves fruitless. A MEDLINE (1986-2000) and PUBMED (1966-2000) search of relevant articles was performed. Descriptors used were aseptic diskitis, spondylodiskitis, pseudodiskitis and pseudoarthrosis.

The SAPHO syndrome is characterized by synovitis, acne, palmo-plantar pustulosis, hyperostosis and osteitis. SAPHO syndrome has been occasionally described in Spain. We present our experience of 16 cases with the SAPHO syndrome diagnosed between 1984-1995. The predominant clinical symptoms were: anterior thoracic pain (14 cases), sacroiliac pain (7 cases) and peripheral arthritis (2 cases). Cutaneous involvement was characterized by palmo-plantar pustulosis (8 cases) acne (3 cases) and psoriasis (2 cases). The histocompatibility antigen B27 was negative in all cases. A computerized tomographic study revealed involvement of sterno-costo-clavicular and manubriosternal joints (14 cases) and sacroiliitis (8 cases).

The AA. present a rarely encountered case combining a left recurrent palsy due to an sclerosis and hyperostosis of the first costoesternal joint, which improved after an antiinflammatory treatment.

Background: The Medical Technology Inc (MTI) Photoscreener is a new eccentric photoscreener that is being marketed as a device for the detection of amblyogenic factors in preverbal children. The purpose of this study was to evaluate the accuracy of the MTI Photoscreener in the target population of young, healthy children., Methods: One thousand and three healthy children between the age of 6 months and 59 months were photoscreened with the MTI Photoscreener. Nine hundred and forty nine children were included in the study and their results were compared with a complete ophthalmologic examination with cycloplegia., Results: The sensitivity of the MTI Photoscreener was determined to be 81.8% with a specificity of 90.6%. The overall agreement rate was 88.8%. The positive and negative predictive values were 68.9% and 95.2%, respectively. All cases of strabismus and media opacities were detected., Conclusion: The MTI Photoscreener is an accurate and reliable device designed to detect amblyogenic factors in young children. The camera offers promise as a useful mass-screening tool.

In recent years, several patients with microcephaly, lymphedema and chorioretinal dysplasia have been described. We have studied two additional patients with similar findings. The question of whether microcephaly with lymphedema and microcephaly with chorioretinal dysplasia and lymphedema are distinct entities remains unanswered. Identification of other patients in the future may provide additional information.

Five cases of oral rhabdomyosarcoma in childhood are reported. Four of these children, who were all aged between 3 and 14 years, were girls. In all patients, combined therapy was undertaken which included tumour resection, radiation and chemotherapy. Problems in treatment and prognosis are discussed, and the clinical, histomorphological and ultrastructural findings are described.

Morphometry was employed on different entities of chronic myeloproliferative diseases (CMPD) and reactive lesions in addition to normal control specimens. The entities studied included: (1) inflammatory reactions of the bone marrow (so-called myelitis in chronic rheumatoid arthritis), (2) chronic granulocytic leukemia (CGL), (3) agnogenic myeloid metaplasia in an early hypercellular stage (so-called chronic megakaryocytic-granulocytic myelosis, CMGM), (4) agnogenic myeloid metaplasia in an advanced fibrosclerotic stage or osteomyelofibrosis/sclerosis (MF/OMS), (5) polycythemia vera (P. vera), (6) reactive thrombocytosis (TH, as a sequel of miscellaneous conditions) and (7) primary (idiopathic, essential) thrombocythemia (PTH). Evaluation was done on plastic-embedded semithin sections with a constant thickness of 3 micron in approximately 20 cases of each group of CMPD. The following parameters were determined: (1) density distributions of the megakaryocyte and non-megakaryocyte compartments, (2) arrangement of megakaryopoiesis in the bone marrow space (i.e., inhomogeneity or clustering) and (3) the fine structure of megakaryocytes in PTH, with a quantitative analysis of the nuclear morphology by circular deviation and contour factors. The megakaryocyte morphology was closely related to a facultative or obligatory increase of the platelet count in these various entities of CMPD and was separable into two major categories: (1) controls, CGL and myelitis versus (2) CMGM, MF/OMS, P. vera, TH and PTH. These two categories were distinguishable by the prominence of megakaryopoiesis in the bone marrow as well as the elevated platelet counts in the periphery. Moreover, in comparison with CMGM and MF/OMS, PTH was characterized by an apparently normal maturation and a conspicuous polyploidization of megakaryocytes according to the nuclear morphology, which was similar to that of P. vera. Our results suggest that PTH presents a monolinear growth of the megakaryopoiesis in the same way as CGL exhibits a monolinear proliferation of the neutrophilic granulopoiesis. This is in contrast to the mixed cellularity of both the megakaryocyte and granulocyte lineage in CMGM and MF/OMS.

Morphometric evaluation was performed on semi-thin sections of core biopsies of the bone marrow and included 20 cases of each group of diseases besides control specimens. (i) Hyperergic myelitis of rheumatic origin. (ii) Chronic granulocytic leukaemia (CGL). (iii) Polycythaemia vera (P. vera). (iv) Chronic megakaryocytic-granulocytic myelosis (CMGM). (v) Myelofibrosis or osteomyelosclerosis (MF/OMS). The following classification of megakaryopoiesis was applied: normal megakaryocytes; giant forms; microforms; intussusceptions; cytoplasmic fragments; naked nuclei. The density distribution shows an increase of megakaryocyte number in those 5 different marrow disorders, ranging from about 13/mm2 in the normal sample up to 65 cells/mm2 in MF/OMS. Microforms are most frequently encountered in CGL, whereas giant megakaryocytes, intussusceptions and many cytoplasmic fragments characterize P. vera, CMGM and MF/OMS. Our measurements suggests 3 distinct categories of bone marrow lesions with corresponding alterations of the megakaryopoiesis: (i) myelitis and CGL; (ii) P. vera; (iii) CMGM and MF/OMS.

A case of rectal carcinoid tumor in a 54 year old female patient is reported. An endoscopic resection of an elevated high rectal lesion was done after performed a well established radiologic and endoscopic diagnosis. The tumor was localized in the submucosa of the rectum. Microscopically, neoplastic cells were mainly arranged in nests and trabecular structures within thin fibrous stroma. Mitotic figures and pleomorphism cellular were uncommon. Histochemically the argyrophil stain of Grimelius were positive but the Masson-Fontana silver stain were negative. At the ultrastructural level neurosecretory granules were found in the cytoplasma, varied in shape and size, and 150-400 nm in longest axis.