Goldenhar syndrome (oculo-auriculo-vertebral dysplasia, OAVS) is a rare, congenital disease arising from the abnormal development of the first and second branchial arches.The incidence is between 1:3500 and 1:5600, with a male: female ratio of 3:2. The etiopathogenesis is multifactorial and dependent on genetic and environmental factors but there are still many unknown aspects.The classic features of Goldenhar syndrome include ocular anomalies - epibulbar dermoids, microphthalmia and coloboma, ENT features such as preauricular tragi, hearing loss, low implantation of the auricular pavilion, micrognathia, and vertebral anomalies such as scoliosis or hemivertebrae. The abnormalities are unilateral in 85% of the cases. Ocular features, especially bilateral dermoids are seen in 60% of the cases.The treatment varies with age and systemic associations, from mainly cosmetic, in uncomplicated cases, to complex reconstructive surgeries in severe cases. While the oculoplastic surgeon manages the oculo-palpebral defects, severe forms require a multidisciplinary approach. Treatment should be individualized, adapted to age, as well as to the extent and severity of the disease. The paper is based on the editorial team cases and experience.
Glaucoma is known as an optic neuropathy prone to progression that determines characteristic not only structural (loss of the ganglion cells as well as their axons) but also functional defects (visual field loss). Objective: To evaluate the possibility of applying ganglion cell complex analysis (GCC) in patients who associate ocular hypertension with tilted disc and marked peripapillary atrophy.Methods: In order to evaluate its components, GCC can be investigated using the Optical Coherence Tomography (OCT) revealing: ganglion cell layer (cells bodies), inner plexiform layer (dendrites and synapses), and nerve fiber layer (axons). Our study included 196 eyes divided into 3 groups: 52 diagnosed with primary open angle glaucoma (POAG), 63 with ocular hypertension (OH), and 81 healthy (normal) eyes (NE). All eyes were submitted to a complete ophthalmologic checkup that involved advanced optic nerve and GCC evaluation. Results: A positive statistically significant correlation was identified between the GCC thickness and the RNFL in all three categories taken into account: R=0,6, p<0,0001 for glaucoma group, R=0,66, p<0,0001 for OH group and R=0,46, p<0,0001 for normal group.Conclusions: GCC has been proved useful for the assessment of the retinal nerve fiber layer (RNFL) in eyes with OH that associate tilted disc or peripapillary atrophy where the optic disc edges might not be certainly determined by the OCT.
Glaucoma represents the main cause of irreversible blindness in the world and for this consideration, the interest in a quick and precise diagnosis and progression of the disease, prior to the appearance of irreversible damage, has been continuously rising. Glaucoma risk factors are already well known, but current studies reveal that it is necessary to make a proper analysis of the intraocular pressure (IOP) to obtain an accurate diagnosis, so we must take into consideration corneal properties that might affect IOP measurements. Starting from corneal geometrical properties represented by central corneal thickness (CCT) and continuing with biomechanical properties represented by corneal hysteresis (CH) and corneal resistance factor (CRF) we reviewed the value of investigating corneal properties in ocular hypertension (OH), primary open angle glaucoma (POAG) and normal tension glaucoma (NTG) patients. We can now say that CCT plays an important role in diagnosing glaucoma because it may mask the real value of the IOP and also, in setting the target for the IOP needed to stop disease progression. Also, CH is a factor that needs to be screened from the first consult of a glaucoma patient or suspect because it is correlated to the response to treatment, visual field (VF) and retinal nerve fiber layer (RNFL) progression and could anticipate the future evolution and patients prognosis. Both CCT and CH are factors that must be thought-about when we encounter a glaucoma suspect. CCT has a predictive role in OH and NTG patients, while CH has on the other hand a prognostic role in POAG, OH and NTG patients.
There are numerous articles on the discovery of this arterial polygon, its history being quite long, beginning with the Antiquity and up to the Modern Era. Making an analysis of the primary and secondary sources on this topic, the purpose of this article is to identify the significant moments of the discovery of this arterial polygon, which is an anatomical structure with great importance for neurologists and neurosurgeons. We will present the contributions to this topic from Renaissance and early Modern Era anatomists, such as Andreas Vesalius, Jean Fernel, Gabriel Fallopius, Giulio Cesare Casseri, Adriaan van den Spiegel, Johann Vesling, and Johann Jakob Wepfer von Schaffhausen. We also pointed out that the contribution of the famous British anatomist Sir Thomas Willis (1621–1675) was the most important one. He published De Cerebri Anatome , in 1664, in which he described the vascular arrangement laying at the base of the brain, accompanying it by the exquisite drawings of Christopher Wren. Thus, he demonstrated to the medical world that he had reached the greatest understanding of the structure and function of the circular arterial anastomosis. For this excellent discovery, his followers honored him by giving his name to this arterial circle.
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