Project description:Head shape assessments in children with metopic synostosis are a relevant outcome measure in addition to functional measures, such as neurocognitive outcomes, behavioral outcomes, and visual functioning outcomes. However, consensus on head shape assessments in children with metopic synostosis is lacking. The aim of this study is to develop a reproducible and reliable suture-specific photo score that can be used for cross-center comparison of phenotypical severity of metopic synostosis and evaluation of esthetic outcome of treatment later in childhood. We conducted a retrospective study among nonsyndromic metopic synostosis patients aged <18 years. Preoperative and postoperative photosets of patients with metopic synostosis from 6 expert centers were included. The photo score was discussed in the group of expert craniofacial plastic surgeons and pediatric neurosurgeons. Interrater reliability was determined with modified weighted Fleiss' kappa and intraclass correlation coefficients. Correlation between individual photo score items with overall phenotype was assessed using Spearman correlation analyses. The metopic synostosis photo score contained the following items: "wedging of the forehead", "hypotelorism", "temporal hollowing", "biparietal widening,"and an assessment of "overall phenotype". Items were scored on a 4-point ordinal scale ranging from normal to severe. We found moderate interrater reliability for all items, but substantial agreement for the summed scores. Correlation with overall phenotype was lowest for biparietal widening. To conclude, although agreement on individual photo score items was suboptimal, the agreement on the summed score was substantial, which indicates there is consensus on the overall severity of the metopic synostosis phenotype.
Project description:Metopic craniosynostosis is a common growth disturbance in the infant cranium, second only to sagittal synostosis. Presenting symptoms are usually of a clinical nature and are defined by an angular forehead, retruded lateral brow, bitemporal narrowing, and a broad-based occiput. These changes create the pathognomonic trigonocephalic cranial shape. Aesthetic in nature, these morphological changes do not constitute the only developmental issues faced by children who present with this malady. Recent studies and anecdotal evidence have also demonstrated that children who present with metopic synostosis may face issues with respect to intellectual and/or psychological development. The authors present an elegant approach to the surgical reconstruction of the trigonocephalic cranium using an in situ bandeau approach.
Project description:Greig cephalopolysyndactyly syndrome (GCPS) is a multiple congenital malformation characterised by limb and craniofacial anomalies, caused by heterozygous mutation or deletion of GLI3. We report four boys and a girl who were presented with trigonocephaly due to metopic synostosis, in association with pre- and post-axial polydactyly and cutaneous syndactyly of hands and feet. Two cases had additional sagittal synostosis. None had a family history of similar features. In all five children, the diagnosis of GCPS was confirmed by molecular analysis of GLI3 (two had intragenic mutations and three had complete gene deletions detected on array comparative genomic hybridisation), thus highlighting the importance of trigonocephaly or overt metopic or sagittal synostosis as a distinct presenting feature of GCPS. These observations confirm and extend a recently proposed association of intragenic GLI3 mutations with metopic synostosis; moreover, the three individuals with complete deletion of GLI3 were previously considered to have Carpenter syndrome, highlighting an important source of diagnostic confusion.
Project description:Objective assessment of head shape has been an elusive goal in the management of craniosynostosis patients. Clinical judgment, craniometric indices, and computed tomography scans are the primary means through which a surgeon assesses this patient population. The purpose of this study was to examine and discuss the utility of the STARscanner for evaluation of surgical outcomes in metopic synostosis patients. A retrospective chart review of patients with metopic synostosis who underwent fronto-orbital advancement with pre- and postoperative STARscanner imaging at Texas Children's Hospital was performed. Two patients were identified and evaluation and discussion of the data produced by the STARscanner was undertaken. A novel symmetry index created by the authors, called the anterior-posterior volume ratio (APVR), was discussed for use in metopic synostosis patients. The postoperative growth metrics demonstrated an interval increase compared with the preoperative data. The anterior symmetry ratio, posterior symmetry ratio, overall symmetry ratio, cranial vault volumes, cranial vault asymmetry index, and cephalic ratio were not found to be useful in evaluating resolution of dysmorphology after fronto-orbital advancement in metopic synostosis. The APVR does not characterize dysmorphology, but may help show degree of expansion of the anterior cranial vault after fronto-orbital advancement. The STARscanner imaging device does not appear to have significant utility in characterizing head shape for surgical outcomes assessment in metopic synostosis. The minor utility of this device may be that it is a safe and fast way to derive growth parameters for both short-term and long-term follow-up of cranial vault remodeling.
Project description:This is a case of a 67-year-old female with a history of Graves' disease and associated thyroid eye disease who presented to the emergency room after falling and hitting her head. Subsequently, she noted periorbital air on the left side when blowing her nose. A CT scan in the emergency room revealed a left orbital floor fracture and decompression of the left orbit. While nonsurgical orbital decompression in the setting of Graves' eye disease has been reported in several instances, spontaneous ethmoid bone remodeling or fracture has been the most cited mechanism, with rarer instances of true spontaneous orbital floor fracture. Furthermore, there exist only two known case reports of traumatic orbital floor fractures with subsequent orbital decompression in the setting of thyroid eye disease. In only one of the two known cases, therapeutic effect was established. However, no significant follow-up period was noted. This case represents a rare traumatic orbital floor fracture resulting in orbital decompression with demonstrably improved exophthalmos. It is the first to include definitive follow-up both before and after operative management of the contralateral orbit.
Project description:PurposeThe orbital apex is the narrowest part of the orbit, housing the link between the intracranial cavity and orbit. Knowledge of orbital apex anatomy is crucial to selecting a surgical approach and reducing the risk of complications. Our purpose is to summarize current knowledge on surgical anatomy and attempt to reach a consensus on definition of the orbital apex.MethodsThe online databases of Embase, the Cochrane library, Web of Science and PubMed (MEDLINE) were queried in a comprehensive bibliographic search on the (surgical) anatomy of the orbital apex and consisted of a combination of two subjects, using indexed terms and free text: "Orbital Apex" and "Orbital Anatomy."ResultsA total of 114 relevant papers were included in this review. Numerous anatomical variations are described in the literature. Variations of the optic canal include duplication (0.64%) and keyhole anomaly (2.65%). Variations in pneumatization of the anterior clinoid process were unilateral in almost 10%, bilateral in 9%, and normal in 72%. A rare variant of the superior orbital fissure (SOF) is Warwick's foramen, which appears as if the lowest portion of the SOF was separated from the main fissure by a transverse bony bridge.ConclusionThe definition of the orbital apex varies in the literature, and further research would most likely identify additional variations. A universal definition reporting these variations and pathology and imaging findings is essential for determining the optimal surgical approach to the orbital apex.
Project description:PurposeTo determine the safety and effectiveness of orbital decompression for thyroid eye disease (TED) in our unit. To put this in the context of previously published literature.Patients and methodsA retrospective case review of all patients undergoing orbital decompression for TED under the care of one orbital surgeon (SMS) between January 2009 and December 2015. A systematic literature review of orbital decompression for TED.ResultsWithin the reviewed period, 93 orbits of 55 patients underwent decompression surgery for TED. There were 61 lateral (single) wall decompressions, 17 medial one-and-a-half wall, 11 two-and-a-half wall, 2 balanced two wall, and 2 orbital fat only decompressions. For the lateral (single) wall decompressions, mean reduction in exophthalmometry (95% confidence interval (CI) was 4.2 mm (3.7-4.8), for the medial one-and-a-half walls it was 2.9 mm (2.1-3.7), and for the two-and-a-half walls it was 7.6 mm (5.8-9.4). The most common complications were temporary postoperative numbness (29% of lateral decompressions, 17% of other bony decompressions, OR 0.50, 95% CI 0.12-2.11) and new postoperative diplopia (9% of lateral decompressions, 39% of other bony decompressions, OR 6.8, 95% CI 1. 5-30.9). Systematic literature searching showed reduction in exophthalmometry for lateral wall surgery of 3.6-4.8 mm, with new diplopia 0-38% and postoperative numbness 12-50%. For other bony decompressions, reduction in exophthalmometry was 2.5-8.0 mm with new diplopia 0-45% and postoperative numbness up to 52%.ConclusionDiffering approaches to orbital decompression exist. If the correct type of surgery is chosen, then safe, adequate surgical outcomes can be achieved.
Project description:The goals of operative treatment for unilateral coronal synostosis (UCS) are to improve appearance and allow unrestricted brain growth. However, for severe unilateral premature closure of the coronal suture, existing methods do not address the compression of the brain or expand the volume of the skull cavity. We report our retrospective experience with bilateral fronto-orbital advancement combined with cranial vault release using a free-floating bone flap (CVR + FFBF) technique and the resulting changes in the anterior cranial vault asymmetry index (ACVAI) and intracranial volume. Twenty patients with UCS who underwent bilateral fronto-orbital advancement combined with CVR + FFBF technique from April 2014 to May 2019 were included. Surgical efficacy was evaluated by the ACVAI and intracranial volume before the operation, 1 week after the operation, and at the last follow-up (average 19.8 months; range, 12 to 40 months). The measurement data are presented as the mean ± standard deviation and were statistically analyzed by t-test. The ACVAI was 9.07%±3.55% before the operation, 3.56%±3.42% 1 week after the operation, and 3.13%±2.41% at the last follow-up. The ACVAI 1 week after the operation was significantly lower than that before the operation (t=4.827, P<0.001). There was no significant difference between the ACVAI 1 week after the operation and at the last follow-up (t=0.660, P=0.517). The intracranial volume was 1,027.85±112.25 mL in patients before the operation and 1,131.92±161.71 mL in the normal control group, which was a statistically significant difference (t=2.364, P=0.023). The intracranial volume significantly increased 1 week after surgery: 1,081.62±111.10 mL (t=8.703, P<0.001), and this trend continued at the last follow-up (1,386.90±119.30 mL) similarly to the normal control group (1,438.22±89.28 mL). At the last follow-up, there was no significant difference between the two groups (t=1.540, P=0.132). For the treatment of UCS, bilateral fronto-orbital advancement combined with CVR + FFBF technique offers functional and cosmetic outcomes in terms of intracranial volume expansion and fronto-orbital symmetry.
Project description:PurposeLittle is known about the way orbital fat slides and/or deforms during eye movements. We compared two deformation algorithms from a sequence of MRI volumes to visualize this complex behavior.MethodsTime-dependent deformation data were derived from motion-MRI volumes using Lucas and Kanade Optical Flow (LK3D) and nonrigid registration (B-splines) deformation algorithms. We compared how these two algorithms performed regarding sliding and deformation in three critical areas: the sclera-fat interface, how the optic nerve moves through the fat, and how the fat is squeezed out under the tendon of a relaxing rectus muscle. The efficacy was validated using identified tissue markers such as the lens and blood vessels in the fat.ResultsFat immediately behind the eye followed eye rotation by approximately one-half. This was best visualized using the B-splines technique as it showed less ripping of tissue and less distortion. Orbital fat flowed around the optic nerve during eye rotation. In this case, LK3D provided better visualization as it allowed orbital fat tissue to split. The resolution was insufficient to visualize fat being squeezed out between tendon and sclera.ConclusionB-splines performs better in tracking structures such as the lens, while LK3D allows fat tissue to split as should happen as the optic nerve slides through the fat. Orbital fat follows eye rotation by one-half and flows around the optic nerve during eye rotation.Translational relevanceVisualizing orbital fat deformation and sliding offers the opportunity to accurately locate a region of cicatrization and permit an individualized surgical plan.