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Biomedical subjects

H J Simonsz

Publications and source records attributed to H J Simonsz.

At least 19 recordsLinked to original sources
PubMed2006

The prenatal development of the human orbit.

During the 1970s, as part of his work for a doctor's thesis in which he described the development of the human orbit in great detail, the first author established the largest anatomical collection of embryonic and fetal orbits ever. Unfortunately, he died before the thesis could be finished. The thousands of sections have now been scanned at high resolution and made publicly available on the Internet at www.visible-orbit.org; 3-D reconstruction software is being developed. The Discussion and part of the 'Methods' section of this thesis are published in translation in this article. The conclusions of the first author at the time read as follows: (1) initially, the developing orbit is vaguely indicated by condensations in the mesenchymal connective tissue area; (2) in this connective tissue area, chondral, osseous and muscular structures develop and grow until, in the fully developed stage, the orbital content is surrounded by bony surfaces with a thin layer of connective tissue as periosteum, and by a muscle fragment; (3) the embryonic and early fetal phase, during which one can only speak of a 'regio orbitalis,' is followed by a period in which we can speak of a primordial orbit; (4) the phase of the primordial orbit extends until after birth; (5) the surface area of all orbital walls increases more or less linearly; (6) the 'musculus orbitalis Mülleri' occupies a special place in the orbital wall; (7) the so-called 'regio craniolateralis' is the primordium, which, in the fully developed stage, is occupied by the thick intersection of the frontolateral and the horizontal part of the frontal bone; (8) in the frontal plane, the shape of the primordial orbit, as well as that of the fully developed orbit, is more or less round; (9) the prenatal development of an eye socket is a complex event, characterized by changes in composition, shape and size of the orbital wall; and (10) the orbit can only be denoted by the term "eye socket" when it is fully developed. At the end of the thesis, he also presented the following postulates: (1) in the prenatal orbit, the development of the so-called 'periorbita' is at the forefront; (2) the mutual rotation of the orbital axes and the frontalization of the eyes from approx. 180 degrees in the early prenatal stages to approx. 50 degrees in adulthood do not seem to be caused by mechanical influences of the surrounding tissue; (3) the pterygopalatine fossa and the 'cavum cerebri' are not part of the orbit at any developmental stage; (4) in the prenatal skull, the inferior nasal concha, which forms part of the maxilla in the fully developed skull, is part of the 'capsula nasalis'; and (5) in order to achieve normal development of the eye socket in microphthalmus and anophthalmus, the normal orbital content should be restored.

Female↗
PubMed2005

[From gene to disease; incontinentia pigmenti and the NEMO-gene].

Incontinentia pigmenti (IP; MIM308310) is a rare neurocutaneous X-dominant inherited disorder. Besides skin and neurological abnormalities, there is also ophthalmologic and dental involvement. The first stage is characterised by inflammation and apoptosis of the skin and central nervous system. The first stage consists of vesicles and the second of verrucous elements; the third stage is characterised by hyperpigmentation while the fourth is characterised by slightly atrophic hypopigmentations. The skin abnormalities follow the lines of Blaschko. The disorder is observed almost exclusively in girls, but diseased boys are more seriously affected. The IP gene is localised on chromosome Xq28. Mutations in the NEMO-gene are responsible for IP. This gene codes for the nuclear factor-KB essential modulator protein (NEMO; synonym: inhibitor kappaB kinase (IKK)y). In the absence of serious neurological symptoms, the prognosis is not poor.

Female↗
PubMed2005

Mechanical properties and functional importance of pulley bands or 'faisseaux tendineux'.

INTRODUCTION: Connective tissue bands connect the horizontal rectus muscles at the level of the posterior pole to the orbital wall. These bands, referred to as pulley bands or faisseaux tendineux, purportedly act like springs to keep the rectus muscle bellies in place during eye movement out of the plane of the muscle. We examined the mechanical properties of these bands in human specimens obtained during surgery. In addition, we examined eye motility and stability of rectus muscles in a patient who had no functional pulley bands. METHODS: Exenterations were carried out on two patients with sebaceous gland carcinoma. Pulley bands were identified and force-elongation behavior was examined with a forceps and a force gauge. Stability of rectus muscles was examined in a patient with severe Crouzon's syndrome by orbital CT scans and during surgery under local, eye drop, anesthesia. RESULTS: The pulley bands showed leash-like mechanical behavior: they were slack over approximately 10mm and became taut when stretched further. In the patient with Crouzon's syndrome, both CT and observation of the muscle during surgery showed little sideways displacement of the muscle bellies in eye movement out of the plane of the muscle, despite the lack of functional pulley bands. DISCUSSION: The leash-like mechanical behavior of the pulley bands seems unsuited for stabilization of the muscle bellies. The patient with Crouzon's syndrome had relatively good eye motility and stable rectus muscle paths despite the lack of functional pulley bands.

Aged↗
PubMed2005

Final report of the early vs. late infantile strabismus surgery study (ELISSS), a controlled, prospective, multicenter study.

BACKGROUND: The optimal age for surgery for infantile esotropia is controversial. Proponents of early surgery believe that further loss of binocular vision can be prevented by early surgery, a minority believes that binocular vision can even be restored by early surgery. The ELISSS compared early with late surgery in a prospective, controlled, non-randomized, multicenter trial. METHODS: Fifty-eight clinics recruited children aged 6-18 months for the study. Each clinic operated all eligible children either 'early', i.e. at age 6-24 months, or 'late', i.e. at age 32-60 months. At baseline the angle of strabismus, refraction, degree of amblyopia and limitation of abduction were assessed. Intermediate examinations took place every six months. Children were evaluated at age six in the presence of independent observers. Primary endpoints were (i) level of binocular vision, (ii) manifest angle of strabismus at distance and (iii) remaining amblyopia. Secondary endpoints were number of operations, vertical strabismus, angle at near and the influence of surgical technique. RESULTS: A total of 231 children were recruited for early and 301 for late surgery. Age at entry examination was 11.1 months (SD 3.7 months) in the early group and 10.9 (SD 3.7) months in the late group. Refraction, amblyopia and limitation of abduction were distributed equally in the early and late groups, but the angle of strabismus was slightly larger in the early group. Dropout-rates were 26.0% in the early and 22.3% in the late group. At age six, 13.5% of the early vs. 3.9% of the late group recognized the Titmus Housefly; 3.0% of the early and 3.9% of the late group had stereopsis beyond Titmus Housefly. No significant difference was found for angle of strabismus. 35.1% of the early group and 34.8% of the late group did not have an angle between 0 degrees and 10 degrees , the thresholds set for re-operation. For ratio of the visual acuities (remaining amblyopia) there was a small but significant advantage for the early group. There was hardly any correlation between the baseline parameters and the primary endpoints. Children scheduled for early surgery had first been operated at 20 (SD 8.4) months, but 8.2% had not been operated at age six. Children scheduled for late surgery had been operated at 49.1 (SD 12.7) months, but 20.1% had not been operated at age six. The number of operations per child was 1.18 (SD 0.67) in the early and 0.99 (SD 0.64) in the late group. Age at recruitment, age that strabismus reportedly had started and refraction at entry examination were similar among operated and non-operated children. Only the angle of strabismus at entry predicted, to some extent, whether a child had been operated at age six. DISCUSSION: Children operated early had better gross stereopsis at age six as compared to children operated late. They had been operated more frequently, however, and a substantial number of children in both groups had not been operated at all.

Depth Perception↗
PubMed2004

Amblyopia & Strabismus Questionnaire: design and initial validation.

Literature on the benefits of screening and treatment of amblyopia emphasizes the costs of insufficiently treated amblyopic patients who lose their better eye. However, patients with insufficiently treated amblyopia and strabismus who do not lose their better eye only experience a slight decrease in their quality of life, and such patients occur more frequently. We designed and validated a questionnaire for amblyopia and strabismus in order to assess the decrease in quality of life of such patients. Complaints were collected from outpatients and reduced to five domains, pertaining to distance estimation, visual disorientation, problems with social contacts and cosmetic problems, diplopia, and fear of losing the better eye. For these domains 26 questions were formulated that constitute the Amblyopia & Strabismus Questionnaire (A&SQ). The A&SQ, the National Eye Institute Visual Function Questionnaire-25 (VFQ-25) and the Short Form-12 Health Survey (SF-12) were presented to 53 healthy controls, 68 outpatients and a cohort of 174 outpatients born between 1962 and 1972 and treated between 1968 and 1974 for amblyopia and strabismus. The quality of life was best in healthy controls and worst in current outpatients, not only on the SF-12 and the VFQ-25 but also on the A&SQ, demonstrating an acceptable construct validity of the A&SQ. The decrease in the quality of life as measured by the A&SQ was most outspoken in our outpatient group of amblyopia and strabismus patients, less in the cohort that had been treated 30 years previously and least in the healthy controls, demonstrating an acceptable discriminatory validity of the A&SQ.

Adult↗
PubMed2002

A preliminary report about the relation between visual acuity increase and compliance in patching therapy for amblyopia.

PURPOSE: The aim of this study was to establish a relation between visual acuity increase and compliance in children who have been prescribed patching therapy for their amblyopic eye. METHODS AND MATERIALS: In 14 new amblyopic children (mean age 4.3 +/- 1.9 years) compliance was measured electronically during one week, six months after starting patching therapy, with an Occlusion Dose Monitor (ODM), distributed through house visits. The children were diagnosed with anisometropia (5), strabismus (4) and anisometropia and strabismus (5). The degree of amblyopia was expressed as the ratio between the acuity of the amblyopic eye and the acuity of the good eye. Satisfactory increase in acuity was assessed by means of the following three criteria: acuity amblyopic eye / acuity good eye >75%, acuity exceeding 0.5 E-chart, three lines LogMAR acuity increase. RESULTS: Fourteen reliable recordings were obtained, which showed that children who did not patch, or were patched inconsistently, did not reach satisfactory acuity increase. CONCLUSION: There is indeed a statistically significant relation between acuity increase and measured compliance.

Amblyopia↗
PubMed2001

Preliminary report: prescription of prism-glasses by the Measurement and Correction Method of H.-J. Haase or by conventional orthoptic examination: a multicenter, randomized, double-blind, cross-over study.

In a multicenter, randomized, double-blind, cross-over study in the Netherlands, the effectiveness of (prism-)glasses prescribed by the Measurement and Correction Method of H.-J. Haase (MKH) was compared to that of glasses prescribed by conventional orthoptic examination. Nine pairs of MKH-optometrists and orthoptists recruited patients who primarily presented with asthenopia, and each prescribed the patient (prism-)glasses. A questionnaire for asthenopia was developed that rated headache and tired eyes as 0-7 days per week and none-light-medium-severe, respectively. Light sensitivity, problems with focusing, near-work problems and burning eyes were each rated as: never-occasionally-often-always. A patient was eligible if he scored 'medium', 'often' or '5 days a week' twice; or 'medium' (etc.) once and 'light' (etc.) twice. Controls, in contrast to the patients, typically answered 'none' or 'never' to half of the complaints, but 37% of them would have passed the admission criteria. Among other criteria were: 18 to 40 years of age, horizontal angle < 4 degrees, vertical < 1.7 degrees, acuity > or = 0.8, stereopsis threshold disparity < 120". Seventy-two patients fulfilled all criteria and returned sufficient questionnaires. They wore the first glasses for six weeks, were without glasses for two weeks, and then wore the second glasses for six weeks. At the start, halfway and at the end of each 6-week period, questionnaires were filled out; 97% were returned. Only 19 of the orthoptists' glasses contained prisms (14 horizontal, 5 vertical; horizontal average of all glasses 0.49 PD, vertical 0.05 PD). Five of the orthoptists' glasses were plano. All MKH glasses contained prisms, 53 of 72 both horizontal and vertical, 18 only horizontal and one only vertical (horizontal average of all glasses 2.83 PD, vertical 0.79 PD). The starting levels of complaints were high and both glasses improved complaints dramatically. The starting levels were lower, but not significantly, in the second 6-week period and improvement was less outspoken. Because of these differences, the two periods had to be evaluated separately. The primary outcome of the study was defined as the difference between the effect of the MKH glasses and that of the orthoptists' glasses in the first and second 6-week periods. For problems with focusing, in the first 6-week period, and for tired eyes, in the second 6-week period, the difference exceeded the difference that had been defined as clinically significant (one day per week less headache or half the distance light-medium or half the distance occasionally-often), but it did not reach statistical significance. The statistical power was approximately 0.7 for demonstrating this clinically significant difference. Statistical significance was not reached in multivariate repeated measure ANOVA either. Forty-four patients preferred to keep the MKH glasses, 25 the orthoptists' glasses, including one plano. It is striking that 25% of the patients did not prefer the glasses that, according to the questionnaire, improved their complaints the most. A year after the study, the questionnaire was sent again to all patients: The levels of complaints after a year were similar to those at the end of the second 6-week period, whether they had preferred the MKH or the orthoptists' glasses, and were similar to the levels in controls. The most conspicuous finding was that both glasses improved the complaints dramatically. Apart from the prisms, other reasons could be: spherical and cylindrical correction, improved wearing comfort of the frame, placebo effect, Hawthorne effect and regression to the mean.

Adolescent↗
PubMed2000

The optimal stimulus to elicit suppression in small-angle convergent strabismus.

PURPOSE: To determine the optimal stimulus duration as well as the most appropriate luminance profile to elicit suppression in small-angle convergent strabismus. METHODS: In 10 subjects with small-angle convergent strabismus, using a device allowing binocular viewing and peripheral fusion, we determined what the optimal stimulus would be to elicit suppression. Three control subjects were also included in the study. Stimuli were shown randomly in the central 3 degrees of the visual field of either eye. Stimulus durations were varied in seven steps from 50 to 1000 ms and three luminance-time profiles were used: square wave, triangle and half-sinus, thus yielding 21 different stimuli. The peak light intensity was the same for all stimuli. RESULTS: Suppression, defined as the difference in the threshold sensitivities under monocular vs. binocular viewing, was found with our test device in five of the ten subjects, and ranged between 3 and 33 dB. Suppression was deepest with triangular or half-sinusoidal stimuli of 400 ms duration. Square wave stimuli elicited the smallest amount of suppression. CONCLUSION: Stimuli with a gradual increment and decrement, like triangular or half-sinusoidal stimuli, with a duration of 400 ms are the most effective to elicit suppression

Adolescent↗
PubMed2000

Perinatally ruptured dermoid cyst presenting as congenital oculomotor palsy.

An eight-month-old girl presented with congenital exotropia and latent nystagmus. Further evaluation revealed congenital ptosis of the left eye and restriction of the elevation, depression and adduction of the left eye. A diagnosis of congenital oculomotor palsy was made. At the age of three months she had been examined by the neurologist because of retarded psychomotor development. All laboratory investigations were normal. At the age of eight months, a CT scan of the brain and orbit was found to be normal. The patient was treated for amblyopia. At the age of five, strabismus surgery was performed, and a large fibrous tumor encapsulating the superior and lateral rectus muscle was found. A biopsy was taken and pathology showed fibrous tissue containing a hair. Based on the clinical history, the diagnosis of a perinatally ruptured orbital dermoid cyst was made. Review of the previous CT and an additional CT showed enlargement of the left lateral orbital wall with a notch in the lateral wall, indicative of a dermoid cyst.

Blepharoptosis↗
PubMed1999

Quantitative visual fields under binocular viewing conditions in primary and consecutive divergent strabismus.

BACKGROUND: Although there have been a number of studies on the size of the suppression scotoma in divergent strabismus, there have been no reports on the full extent (i.e. size as well as depth) of this scotoma. METHODS: Binocular static perimetry was used to measure suppression scotomas in five patients with primary divergent strabismus and ten patients with consecutive divergent strabismus. Four control subjects were also included in the study. With two modified Friedmann visual field analysers, we measured the visual field of both eyes under monocular and binocular viewing conditions. The objective angle of squint ranged from 3 degrees to 25 degrees. Best corrected visual acuity was at least 0.4, but mostly 1.0 in both eyes. RESULTS: All subjects had normal visual fields for each eye under monocular viewing conditions. In 12 of the 15 subjects, we found a large area of suppression encompassing the projection of the fixation point as well as that of the fovea in the non-fixating eye under binocular viewing conditions. In 2 of these 12 patients, one with primary and one with consecutive divergent strabismus, the area of suppression was located nasally to the position of the fovea in the field of the non-fixating eye (nasal hemisuppression). In another two patients with divergent squint combined with vertical deviation, a small fixation-point suppression scotoma was found. The depth of suppression ranged from 3 dB to 16 dB. In one subject only, no suppression was found. CONCLUSIONS: Our findings indicate that the shape of the suppression scotoma is not related to the origin of divergent strabismus or to the angle of squint. Our results also indicate that the critical age for the development of suppression in divergent squint might be up to 14 years.

Adolescent↗
PubMed1999

Electronic monitoring of treatment compliance in patching for amblyopia.

We developed a coinsized occlusion dose monitor (ODM) to measure compliance with patch-wearing during the treatment of amblyopia objectively. It measures the temperature difference between the front and back of the ODM every 2-5 minutes by means of two thermistors. The data is stored in EEPROM memory and read out after recording for a week by connecting it to a PC. The ODM measures 35x23x4 mm and weighs 6 g. The back of the ODM is glued to the front of the amblyopia patch with double-sided Scotch tape. When the patch with the ODM is on the eye, the temperature at the back of the ODM is higher than at the front. Compliance is being studied in children taking part in a large amblyopia cohort study. The parents were instructed during home visits every three months to put the ODM on the patch. After a week, the ODM was collected and read out. Although the parents knew that a recording was being made, compliance was mediocre in many cases. Children were patched infrequently, for 5 minutes only, for long periods on the last days of the recording, at night, etc. Diaries detailing patch time were unreliable.

Amblyopia↗