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Intraoperative view of the patient. (Above, Left) The frontal bone and the frontal bar/lateral orbital rim bones on the operating table prior to advancement. (Above, Right) Partial bilateral orbital decompression prior to orbital periosteal scoring. (Below, Left) Frontal bone after fixation showing frontal advancement. (Below-Right) Orbital periosteal scoring resulting in decompression of the orbital content showing protrusion of orbital fat content into the orbital volumes.
Source publication
Early closure of cranial sutures results in various types of cranial vault deformities, named craniosynostosis. Although mostly associated with syndromic cases, bony orbit deformities such as exorbitism can be seen with various types of craniosynostosis. This condition can be associated with papilledema and besides its effect on the patient's appea...
Similar publications
In syndromic craniosynostosis, the relation between the supraorbital area and the frontal bone is not good, and it is not possible to reform this area with 1-block advancement. To avoid this problem, the frontal bone is separated from the fronto-orbital bandeau, each is reshaped and remodeled separately, and then both are reattached. The retrusion...
Background:. Multidirectional cranial distraction osteogenesis (MCDO) is a procedure of ours developed earlier for treating craniosynostosis. However, the numerous bone flaps led to prolonged operative time and occasional bone detachment from dura. We have since simplified the osteotomy design. In treating sagittal synostosis, required bone flaps h...
Patients with syndromic craniosynostosis often require a large amount of cranial expansion to avoid intracranial hypertension, but the surgical procedure remains controversial. A patient of severe syndromic craniosynostosis with multiple bony defects and anomalous venous drainage at the occipital region was treated by multidirectional cranial distr...
In this review, we discuss in detail our current procedure for treating craniosynostosis using multidirectional cranial distraction osteogenesis (MCDO). The MCDO method allows all phenotypes of skull deformity to be reshaped by distraction osteogenesis, except in patients who are 5 months of age or younger and patients with posterior cranial vault...
Citations
... Hypertelorism describes an abnormally increased distance between the two orbits due to skeletal anomaly [3]. Exorbitism indicates a narrowing of the orbit due to a smaller bony orbital volume, which precludes the orbit to contain normal orbital tissue volume determining proptosis [1,4]. Proptosis can be associated with papilledema and, besides its esthetic effect, may cause ocular problems such as subluxation of the globe, lagophthalmos or keratitis resulting in corneal ulcers and, in the most severe cases, loss of vision [5]. ...
Purpose
Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation.
Methods
High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the “major” and “minor” sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index.
Results
Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2 infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p<0.05).
Conclusions
Our data show that FGFR2 mutation together with posterior coronal branch synostotic involvement has a synergic effect in causing a more severe degree of orbital proptosis.
A 34-year-old man with Basedow disease consulted us to treat disfiguring proptosis. On examination, the patient showed right lower eyelid retraction and right inferior rectus muscle enlargement, which were consistent with thyroid eye disease. Hertel's exophthalmometric examination demonstrated proptosis of 25.5 and 24.0 mm on the right and left sides, respectively. Computed tomography (CT) images revealed absence of the sphenoid door jamb. To correct both exorbitism and exophthalmos, the patient underwent bilateral "shallow" decompression of the lateral and medial orbital walls with orbital fat removal under general anesthesia. The authors removed 2.5 and 1.0 mL of orbital fat from the inferolateral quadrant of the right and left orbits, respectively. At 3 months postoperatively, based on the measurements of the axial globe position on sagittal CT images, postoperative proptosis reductions of 7.5 and 6.5 mm were observed on the right and left sides, respectively.
