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Biomedical subjects

L Koornneef

Publications and source records attributed to L Koornneef.

At least 19 recordsLinked to original sources

[Surgical rehabilitation in patients with Graves' ophthalmopathy; favorable results of orbital decompression via coronal approach].

Bilateral decompression of the orbit by a coronal approach was performed for cosmetic rehabilitation in 53 female patients with proptosis in the non-inflammatory stage after Graves' ophthalmopathy. Proptosis reduction averaged 5.9 mm Hertel value one year after surgery. All patients were satisfied with the cosmetic result obtained. Advantages of the technique used are: good access to all orbital walls facilitating adequate proptosis reduction; no visible scars after surgery; only very few complications. In our group there was no loss of visual acuity and the incidence of diplopia after surgery of only 4% is the lowest reported in the literature.

Adult

Recurrent hemangiopericytoma and brachytherapy.

A 42-year-old woman had two recurrences following excision of a hemangiopericytoma of the left orbit. A third local excision was followed by postoperative brachytherapy (55 Gy in 100 hours). One year later there is no sign of recurrence and the side-effects are minimal.

Adult

Metastatic tumors to the orbit--management and prognosis.

In a retrospective study the records of 34 consecutive cases with tumors metastatic to the orbit were reviewed with special attention to the management and prognosis of these patients. The primary tumor site was the breast in 20 patients, prostate in 5, kidney in 2 and skin in 2. The remaining 5 patients had other primary tumour sites. The symptoms of orbital metastasis preceded the detection of the primary tumor in 8 cases (24%). Twenty patients died after a mean interval of 25 months following the diagnosis of the orbital metastasis. This study gives a summary of the clinical features of these patients. With improved methods of treatment it was possible to obtain a relief of orbital symptoms in 24 patients (71%). Although the life expectancy of these patients remains poor, treatment can result in symptomatic relief and in an improvement in the quality of life, which is the main goal in the management of these patients.

Adult

Soluble IL-2 receptor levels in patients with Graves' ophthalmopathy.

In various autoimmune diseases circulating levels of soluble IL-2 receptor (sIL-2R) seem to be related to disease activity. Because reliable parameters of disease activity in Graves' ophthalmopathy are lacking, we measured sIL-2R levels in 47 patients with this disorder. The patients had Graves' disease, but no other immune-mediated diseases, had not yet received specific treatment for their ophthalmopathy and were euthyroid during the entire study period. Twenty-one of the 47 patients (45%) had sIL-2R values above the upper normal limit of 650 U/ml, as established in 20 healthy controls. There were no differences between patients with normal (median 469, range 280-644 U/ml) and elevated (median 946, range 678-1588 U/ml) sIL-2R levels regarding duration or severity of the eye disease (as assessed clinically from the total eye score). However, patients with severely enlarged eye muscles had higher sIL-2R values than patients with less severely enlarged eye muscles on CT scan. Patients with elevated sIL-2R tended to have a higher response rate (71%) to a 3-month course of prednisone, than those with normal levels (46%; P = 0.081). Since a successful outcome of prednisone treatment might be representative for disease activity, the elevated sIL-2R levels seem to reflect active inflammation. Although the practical relevance of this finding in individual patients is limited, it underscores the importance of cell-mediated immune responses in this thyroid-related eye disease.

Adult

Distribution of the nuclear thyroid-hormone receptor in extraocular and skeletal muscles.

The correlation between the occurrence of Graves' ophthalmopathy and Graves' hyperthyroidism may indicate a role for tri-iodothyronine (T3) hormone in the pathogenesis of Graves' ophthalmopathy. In Graves' ophthalmopathy the recti eye muscles are greatly enlarged whereas skeletal muscles seem unaffected. The distribution of the nuclear T3 receptor was studied in normal human and rat eye and skeletal muscles with immunohistochemistry using mouse (monoclonal) antibodies, and by in-situ hybridization for the detection of mRNA encoding the T3-receptor protein. Nuclear staining with T3-receptor antibodies was found in all types of tissues studied. Cytoplasmic staining occurred predominantly in the muscle fibres of the orbital layer of the eye muscles and was generally absent or very low in skeletal muscle fibres and hepatocytes. Immunostaining could be inhibited by preabsorbing the antibodies with bacterially expressed T3-receptor protein, implying specificity. The presence of nuclear and cytoplasmic hormone-free T3 receptor sites was indicated after preincubation of sections with T3 hormone; T3-receptor immunostaining decreased and T3-hormone staining increased. In-situ hybridization clearly revealed the presence of alpha-1 and beta-1 forms of the T3-receptor mRNA in liver, skeletal muscles, and orbital and intermediate layers of the eye muscles. The data demonstrate the presence of T3 hormone-receptor molecules in the extraocular and skeletal muscles. The different susceptibilities of these muscles to Graves' hyperthyroidism may relate to the quantitative differences in T3 hormone-receptor distribution.

Adolescent

Site-dependent effects of experimental hypo- and hyperthyroidism on resident macrophages in extraocular muscles of rats: a quantitative immunohistochemical study.

It has been suggested that the effects of dysthyroidism on resident immunocompetent cells of the extraocular muscles may play a role in the pathogenesis of Graves' ophthalmopathy. The distribution of such cells was therefore studied in extraocular muscles of rats that were made hyper- or hypothyroid by the oral administration of thyroxine or propylthiouracil respectively. Skeletal muscles were studied for comparison. The cell distributions were analysed in cryostat cross-sections subjected to a two-step immunoperoxidase method using well-characterized monoclonal antibodies against T cells, B cells, macrophages and MHC class II antigens. The extraocular muscles of control (euthyroid) rats contained numerous macrophages, fewer MHC-II positive cells and T cells and no B cells. Differences in the distribution of immunocompetent cells were found in control rats, between skeletal and extraocular muscles as well as within the various recti eye muscles. This particular tissue distribution resembles that previously reported for human extraocular and skeletal muscles. Quantitative analysis showed that experimental dysthyroidism only affected cell populations in the extraocular muscles. Significant effects on the number of macrophages were observed in the inferior rectus muscle of both hypo- and hyperthyroid rats, this was most pronounced in the orbital layer of the muscles. Both hyper- and hypothyroidism appear to affect local cell distributions in a tissue-specific manner. The presently observed site-dependent effects of dysthyroidism on local immunocompetent cell populations may have relevance for the differential involvement of muscular tissues in Graves' ophthalmopathy.

Animals

[Conservative therapy frequently indicated in blow-out fractures of the orbit].

The results of the conservative approach in the management of patients with orbital blow-out fractures were studied at the Orbital Centre, Amsterdam. In 54 out of 111 patients with a pure blow-out fracture, high resolution CT was performed. In these 54 patients with diplopia, clinical examination together with the analysis of direct multiplanar high resolution CT revealed four cases of impaired motility. These were oedema, oculomotor nerve palsy, enophthalmos and adhesions around the lower eye muscles and the orbital floor. Patients were immediately instructed to exercise eye movements thoroughly. If diplopia did not resolve, further management was decided on. In all patients with edema or oculomotor nerve palsy, diplopia resolved spontaneously. Surgery was necessary to reduce diplopia in the enophthalmos and adhesion groups. This study shows that eye movement exercises together with a 'wait and see' approach diminish the number of operations by 50% in patients with pure blow-out fractures. It also circumvents the iatrogenic adhesions between eye muscles and orbital floor, which are difficult to treat.

Combined Modality Therapy

[The surgical treatment of basal cell carcinoma of the eyelids].

At the Orbital Centre, Amsterdam, a retrospective study of treatment of eyelid basal cell carcinomas was carried out. Between 1981 and 1989 46 patients were referred to our centre. Of these, 31 patients had a histologically proven basal cell carcinoma of the eyelid and underwent a radical excision. Follow-up varied from 11 to 94 months. No recurrences were observed. Functional and cosmetic results were good. Radical excision is compared with other treatment modalities and we conclude that radical excision has the highest success rate. Follow-up is always recommended, however.

Adult

Potential antigen-presenting cells in normal extraocular muscles demonstrated with double immunoenzyme staining.

Antigen-presenting cells are of crucial importance for the initiation and regulation of regional immune responses. In a previous study, indirect morphological evidence that morphologically normal human orbital tissues contain HLA-DR-positive macrophages, which may represent antigen-presenting cells, has ben obtained. In the present study, these cells were characterized in detail using double immunoenzyme staining techniques with monoclonal antibodies directed against several well-characterized monocyte/macrophage markers and against HLA-DR gene products. The orbital muscular tissues appear to contain numerous HLA-DR, monocyte/macrophage marker double-stained cells, which are considered to be potential antigen-presenting cells. The cells are widely distributed in the connective tissue of all the orbital muscular tissues studied and consist of several subsets with different phenotypes. Furthermore, site-specific differences were shown between recti muscles and the levator/Müller's muscles with respect to the distribution of HLA-DR and one monocyte/macrophage marker (OKM5). Many of the orbital antigen-presenting cells appear to be of the dendritic type and are considered to be of major importance in regulating local orbital immunity.

Aged

Results of radiotherapy in patients with stage I orbital non-Hodgkin's lymphoma.

This paper describes the results of radiotherapy in early stage orbital non-Hodgkin's lymphoma. From 1970 to 1985, 33 orbital localizations in 30 patients were treated. The total dose applied ranged from 21 to 57 Gy (2 Gy per fraction), two-thirds of all patients received a dose of 40 Gy. The complete-response rate was 94% and the 10 years actuarial survival was 90%; no significant difference in survival was observed between patients with low grade or intermediate grade lymphoma. No local recurrence was detected during follow up and 20% of the patients developed generalized disease. Two optic nerve neuropathies and three retinopathies were observed in five patients, four of these occurred at a dose level of less than 43 Gy. Keratitis occurred in 58% of the patients treated, a sicca syndrome in 30% and cataract of different grades in 58% of the patients treated. Although local control was excellent, severe complications were observed in 13% of the patients who received a dose of less than 43 Gy.

Dose-Response Relationship, Radiation

Intradiploic epidermoid cysts of the bony orbit.

Epidermoid cysts originating within the diploic space of the bony orbit are rare. The authors retrospectively studied four patients with an intradiploic epidermoid cyst of the orbital bones to investigate the clinical and the computed tomographic (CT) findings. The clinical presentation was dependent on the location of the slowly expanding epidermoid cyst. The sphenoid bone was involved in three patients and the frontal bone in one patient. Proptosis caused by intraorbital extension of the mass was the most common presenting sign. The findings on high-resolution CT scans appeared to be specific for intradiploic epidermoid cysts. The typical sclerotic margin, which is diagnostic of intradiploic epidermoid cysts, can be demonstrated by CT scans with a bone window setting.

Adolescent

Primary and secondary implants in the anophthalmic orbit. Preoperative and postoperative computed tomographic appearance.

Sagittal computed tomographic (CT) scans of 10 anophthalmic orbits with a primary implant, an acrylic ball covered with donor sclera, and 10 anophthalmic orbits before and after insertion of a secondary implant were studied. For each patient, the scans of the anophthalmic and contralateral normal orbit were compared. In the anophthalmic orbits, the anatomy was optimally restored in those with a primary implant, and suboptimal results were achieved in the orbits with a secondary implant. The greatest differences were noticed in the orbits without an implant. Differences were noticed for the position and length of the superior muscle complex and the inferior rectus muscle, the position of the upper eyelid, the depth of the superior sulcus, and the backward tilt of the prosthesis. The authors conclude that the rotatory displacement of the orbital contents after enucleation, which explains the sequelae of the anophthalmic orbit, is grossly circumvented by the insertion of an implant. In achieving this effect, primary implants are better than secondary ones.

Adolescent

Classification of the eye changes of Graves' disease.

Classification of the eye changes of Graves' disease may have clinical use in the description of the present eye state, in the assessment of treatment results, and in the choice of therapy. Requirements for any classification system should include simplicity, clinical nature (i.e., easily carried out by any physician equipped with ordinary noninvasive techniques), reproducibility and meaningfulness (i.e., objective and of clinical relevance to the patient). The NOSPECS system (although it can be improved) is suitable for pure description. It is not very helpful in the assessment of treatment results. Therapeutic outcome probably is best described by measuring quantitatively changes in visual acuity, eye muscle motility, and proptosis. Such an approach, however, does not solve the problem of whether or not the overall outcome is a success in the patient with improvement in one aspect but worsening in another item. A recently developed clinical activity score may be helpful in the selection of therapy, especially for deciding whether or not immunosuppressive therapy should be instituted.

Graves Disease

Prosthesis motility with and without intraorbital implants in the anophthalmic socket.

Ocular prosthesis motility was measured and compared in 15 patients with a primary baseball implant after enucleation of an eye, in 11 patients with a secondary baseball implant, in 12 patients with an Allen implant, and in 11 patients without any intraorbital implant. In all patients a noticeable lag of movement of the prosthetic eye was measured: in the extreme directions of gaze the excursions of the prosthesis were far less in comparison with the contralateral normal eye. For normal eye movement round the primary position of gaze, however, the prosthesis motility in the primary baseball and Allen implant group appeared to be sufficient to give a lifelike appearance. The average motility of the prostheses in these two groups did not differ. The motility in the secondary baseball group and in the group without an implant was evidently worse. In the last group the prosthesis motility was most impaired. We conclude that the insertion of an implant, even when inserted some time after the enucleation (a secondary implant), improves the motility of the prosthesis markedly. We recommend the primary baseball implant as the correction of choice after enucleation.

Adolescent

Conjunctival cysts in anophthalmic orbits.

Five out of 149 patients (3%) who received an intraorbital implant to prevent or treat the disfiguring symptoms associated with the postenucleation socket syndrome developed intraorbital conjunctival cysts. All five patients had received a secondary implant two 14 months previously. After excision of the cysts four patients required additional surgery for lack of conjunctiva and/or recurrent cyst formation. The clinical findings, mechanism of development, and management of this rare but serious complication of socket surgery are described.

Adult

Lid lengthening by sclera interposition for eyelid retraction in Graves' ophthalmopathy.

The efficacy of scleral grafts for eyelid lengthening in patients with thyroid related upper and/or lower lid retraction was evaluated in 62 consecutive patients with Graves' ophthalmopathy who underwent lid surgery in the last 3.5 years. Seventy-eight upper and 30 lower lids were lengthened by scleral interposition. A good or acceptable result was achieved in 50% of all operated upper lids after one procedure. This percentage increased to 75% after a second and to 77% after a third procedure. Persistent temporal retraction and nasal overcorrection were the major complications. In lower lid lengthening the success percentage was 90% after one operation. We conclude that scleral grafting for upper eyelid lengthening has no distinct advantage in comparison with other lengthening techniques. Scleral implants to lengthen lower lids are very effective.

Adult

Fine needle aspiration biopsy in orbital tumours.

Fine needle aspiration biopsy (FNAB) was performed in 46 patients with an orbital mass. Positive cytological identification was made in 43 biopsy specimens. In 26 cases with histopathological control the accuracy was 81%. In experienced hands FNAB is safe and appears to be a valuable tool in establishing a diagnosis of malignancy in orbital tumours.

Adult