Oculocerebrocutaneous Syndrome (Delleman Syndrome): A Case with a Novel Presentation of Orbital Involvement

Oculocerebrocutaneous syndrome (OCCS), also known as Delleman syndrome (DS), is a rare congenital anomaly featuring focal skin defects, orbital anomalies, and central nervous system malformations. Diagnosis of Delleman syndrome is based on the triad of eye, central nervous system (CNS), and cutaneous defects and confirmed by magnetic resonance imaging. A 23-day-old girl was referred to our department for brain imaging. The infant had multiple cutaneous appendages on the right side of her face. There also was a fleshy mass measuring about 12 mm over her right eye. Brain MRI demonstrated the evidence of colpocephaly, agenesis of the corpus callosum, nodular subependymal heterotopias adjacent to the right lateral ventricle, aplasia of the cerebellar vermis, hypoplasia of the right cerebellar hemisphere, and widening of CSF space in the posterior fossa. There was also an exophytic skin lesion on her right cheek, measuring about 13 × 12 mm in size. In the orbital MRI, there was a mixed cystic solid mass measuring about 25 × 20 mm in her right orbital cavity. The orbital content was abnormal and suggestive of rudimentary orbit. Considering the findings, diagnosis of oculocerebrocutaneous syndrome (Delleman syndrome) was established for the patient. Because of the variations in orbital and CNS manifestations, all patients with clinical suspicion of DS should be assessed by brain and orbital MRI and managed by a pediatric neurologist and ophthalmologist.

The orbital content of bars: the origin of ‘non-x1-tree’, bar-supporting orbits

ABSTRACT Recently, many orbital studies in barred galaxy potentials have revealed the existence of orbits that are not trapped around x1-tree orbits, but could be potentially appropriate building blocks for bars. These findings question the uniqueness of the x1 family as the standard paradigm of orbital motion in galactic bars. The main goal of this paper is to investigate the role that such orbits could play in shaping the morphology of bars. We trace the morphological patterns appearing in the face-on and edge-on views of the non-periodic orbits presented in these studies and we show that they are introduced in the system by second type (‘deuxième genre’) bifurcations of x1. For this purpose, we use a typical 3D Ferrers bar model and follow the radial and vertical bifurcations of the x1 family considered as being mul-periodic, with mul = 2, 3, 5. The variation of the stability indices of x1 in the mul = 2, 3 cases gives us also the 4- and 6-periodic orbits, respectively. We tabulate these orbits including all information necessary to assess their role as appropriate building blocks. We discuss their stability and their extent, as well as their size and morphological evolution, as a function of energy. We conclude that even the most important of the mul-periodic orbits presented in Tables 2–5 are less appropriate building blocks for bars than the families of the x1-tree at the same energy.

Cáncer palatosinusal t4a/b: extensión de las resecciones oncológicas

Background: the problems of reconstructive surgery for the midface are variable and can be very complex. The anatomical proximity of the midface to the orbit, base of the skull and maxillary sinuses is a challenge for the surgeon who must perform a curative resection. Objective: The aim of this presentation is to report the survival rate and disease-free interval in T4a and T4b neoplasms of the palate and paranasal sinuses consecutively resected over a 30-year period. Material and methods: Surgery was extended to the orbit in 85.2%, the skull in 8.3%, the neck in 18.7% and the parotid gland in 7.3%. Soft tissue reconstruction was performed using free flaps in 32.5%, muscle flaps in 21.6%, local flaps in 20.2%, musculocutaneous flaps in 14.7% and skin flaps in 11.3%. Results: Local and general complications were reported, and 4/203 patients (2%) died. At 5 years, overall survival was 62.5% and disease-free survival was 53%. Univariate analysis revealed that lack of previous treatment was significantly associated with recurrence and squamous cell carcinoma was a predictor of survival. Conclusions: The indication of exenteration is mandatory in the presence of ophthalmoplegia or involvement of the orbital content. Survival at 5 years is acceptable, considering the advanced stages of the disease. In some patients, previous treatments were

Computerized Dynamic Posturography for Evaluating Dizziness in Patients with Scarred Orbital Content

Our objective was to assess the capability of computerized dynamic posturography (CDP) to evaluate dizziness in blind patients who lack corneoretinal potential. We performed a retrospective analysis of 2 young blind subjects with intraorbital scarring who complained of dizziness and underwent CDP. Tests were carried out at our center for balance disorders using the NeuroCom's EquiTest System version 4.0. CDP demonstrated vestibular dysfunction and revealed it to be severe. We conclude that CDP can serve as a potentially reliable tool for assessing dizziness in blind patients who have no corneoretinal potential.

Ophthalmic comlications of functional endoscopic sinus surgery

Functional endoscopic sinus surgery (FESS) is an effective and safe surgical technique, which revolutionized the surgical management of nasal cavity and paranasal sinus diseases. The intimate connection between paranasal sinuses and the orbit places the orbital content at a risk of injury during sinus surgery, especially that of ethmoid sinuses. The orbit, the optic nerve, extraocular muscles and the lacrimal drainage system could be damaged during FESS. The risk of injury correlates to anatomical variations, degree and severity of disease, previous procedure results, and surgical experience. Ophthalmic complications can vary in severity from minor ones, such as localized hematomas, to extremely dangerous, such as optic nerve injury, that could lead to complete blindness. In order to minimize the risk of such complications, it is necessary to consider probable anatomic variations of paranasal sinuses and orbit, which are to be detected by CT scan before surgery.

Concealed d-wave pairs in the s± condensate of iron-based superconductors

A central question in iron-based superconductivity is the mechanism by which the paired electrons minimize their strong mutual Coulomb repulsion. In most unconventional superconductors, Coulomb repulsion is minimized through the formation of higher angular momentum Cooper pairs, with Fermi surface nodes in the pair wavefunction. The apparent absence of such nodes in the iron-based superconductors has led to a belief they form an s-wave (s±) singlet state, which changes sign between the electron and hole pockets. However, the multiorbital nature of these systems opens an alternative possibility. Here, we propose a new class of s± state containing a condensate of d-wave Cooper pairs, concealed by their entanglement with the iron orbitals. By combining the d-wave (L=2) motion of the pairs with the internal angular momenta I=2 of the iron orbitals to make a singlet (J=L+I=0), an s± superconductor with a nontrivial topology is formed. This scenario allows us to understand the development of octet nodes in potassium-doped Ba1−x KXFe2As2 as a reconfiguration of the orbital and internal angular momentum into a high spin (J=L+I=4) state; the reverse transition under pressure into a fully gapped state can then be interpreted as a return to the low-spin singlet. The formation of orbitally entangled pairs is predicted to give rise to a shift in the orbital content at the Fermi surface, which can be tested via laser-based angle-resolved photoemission spectroscopy.

Implant Retained Orbital Prosthesis – Case Report/ Implantatno retinirana orbitalna proteza – prikaz slučaja

SUMMARY Orbital defects after tumor resection (exenteration of orbital content) have been traditionally reconstructed with adhesive-retained craniofacial prostheses, also known as epistheses. The breakthrough in rehabilitation of facial defects with implant-retained prostheses has come with development of modern silicones (vynilpolysiloxane) and bone anchorage called osseointegration. Craniofacial implant technology offers improved reconstructive options to patients. This paper describes therapeutical procedure on a patient who received craniofacial implant-retained orbital prosthesis after orbital exenteration. The patient reported excellent prosthesis handling and stability