Unicoronal Synostosis Research Articles

Endoscope-Assisted "Extended" Suturectomy for Unicoronal Craniosynostosis: A Technical Note.

Since the initial description of endoscopically assisted suture craniectomy followed by orthotic helmet therapy by Barone and Jimenez,1 many craniofacial teams have shown acceptance of this technique, including our center.2,3 The original technique described by Jimenez and Barone4 for unicoronal craniosynostosis involved a 2-cm incision on the scalp midway between the anterior fontanelle and pterion, followed by endoscopically assisted removal of a 5-mm sleeve of fused coronal suture between the above two points. They also mention removal of the bone at the wing of sphenoid during the suturectomy over the medial frontosphenoid suture region to liberate the orbit. We have, in principle, adopted the above technique in our practice, but have noted some difficulty in locating the pterion accurately as a part of the lateral dissection. The extent of medial dissection is straightforward, mainly because of the presence of the anterior fontanelle. The pterion, on the other hand, is typically pulled toward the superolateral angle of the orbit and vice versa as a part of the deformity described in unicoronal craniosynostosis. We believe that a fused frontosphenoidal suture in addition to the fused coronal suture is the reason for this phenomenon (Fig. 1). An interesting study by Rogers and Mulliken5 revealed that in 36 of 51 computed tomographic scans of unicoronal synostosis patients, there were additional sutures involved in the basilar coronal ring. More specifically, they demonstrated synostosis of the lateral 50 percent of the frontosphenoidal suture, extending to the middle orbital roof in 25 to 75 percent of cases. This feature was progressive as the age of the baby increased from 3 to 5 months old and almost total by 5 months of age.Fig. 1.: Comparison of the location of the pterion on the affected and unaffected sides (black arrows) in a 4-month-old girl with right unicoronal craniosynostosis. The pterion is pulled toward the superolateral orbit. Also, note the fused frontosphenoidal suture on the right side (blue arrow) compared to the patent frontosphenoidal suture on the unaffected left side (green arrow).Our endeavor is to operate on the child as soon as possible (i.e., within 3 months of age), but our cohort has more than half the number of cases in the age group of 3 to 5 months. Hence, it is our practice to extend the suturectomy along the lateral frontosphenoidal suture, all the way up to the superolateral angle of the orbit (also including the frontozygomatic suture in the process). This gives us a buffer to counter the restrictive force of the frontosphenoidal suture fusion happening between 3 to 5 months of age and also spares us the obligation to find the pterion accurately. The dissection proceeds behind the ridge of the sphenoid wing and then turns anteriorly to include the frontosphenoidal suture and frontozygomatic suture (Fig. 2), in the process truly liberating the orbit. We think this is a practically simpler endpoint compared to the pterion at the level of the lateral canthus, as described by the pioneers.Fig. 2.: Postoperative scan demonstrating the “extended” suturectomy to include the frontosphenoidal suture up to the superolateral orbital margin.The impact of this modification (“extended” suturectomy) on the eventual outcome should be validated by long-term follow-up studies. We recommend this simple technical refinement of extending the suturectomy until the superolateral orbit to any surgeon adopting the minimally invasive methods (orthotics or springs) of craniosynostosis correction. DISCLOSURE None of the authors has a financial interest in any of the products or devices mentioned in this article.

Long-term ophthalmic outcomes in 120 children with unilateral coronal synostosis: a retrospective look over 20 years

Prior studies comparing ophthalmic outcomes after treating unicoronal synostosis (UCS) by early endoscopic strip craniectomy (ESC) vs later fronto-orbital advancement (FOA) were modest in sample size, lacked consistent age adjustment and were contested. We report long-term age-adjusted ophthalmic outcomes for a large cohort after nonrandomized treatment by one of these two options.

Virtual Coordinate System in Unicoronal Synostosis.

Background:We propose a landmark-based, virtual coordinate system, specifically designed for assessment of asymmetrical craniofacial anatomy associated with unicoronal synostosis.Method:CT scans of 33 patients with nonsyndromic unicoronal synostosis were included. Proposed mid-sagittal plane was compared with commonly used sagittal planes: (1) nasion, sella, and basion (N-S-BA); (2) midplane of bilateral frontozygomatic sutures (midFZ); and (3) the skull gravity center plane, to evaluate reliability and validity in the assessments of the anterior and posterior skull base.Results:The proposed midplane is similar to the midFZ plane in describing the direction of the anterior skull base. However, it has less bias than the N-S-BA (P < 0.001), and the gravity center planes (P < 0.001). The proposed midplane measures the direction of the posterior skull base plane, similar to the midFZ and gravity center planes, but it has less measurement deviation than the N-S-BA plane (P < 0.001). The most protrusive point on the frontal bone in unicoronal patients is contralateral to the fused suture and distant from the mid-sagittal plane by 13.93 ± 4.01 mm. In addition, it is more anteriorly positioned, by 5.32 mm (P < 0.001), when compared with the corresponding point on the synostotic side. The uppermost point of the supraorbital rim on the synostotic side is cephalic to that of the contralateral side by 4.09 mm (P < 0.001).Conclusions:Prioritized orientation of an averaged Frankfort horizontal plane, followed by the location of the mid-sagittal and coronal planes, can generate a reliable and valid coordinate framework for the assessment of asymmetric skull shape in unicoronal synostosis.

Rotation flap distraction osteogenesis for unicoronal synostosis.

Unicoronal craniosynostosis is notoriously difficult to treat, with long-term studies demonstrating high rates of relapse and the need for reoperation using open fronto-orbital advancement. Applying the principles of distraction osteogenesis to cranial vault remodeling has demonstrated promising short-term results that compare favorably with traditional methods, with simultaneous correction of both frontofacial and endocranial morphology, along with significant increases in intracranial volume. Here, the authors demonstrate their technique for rotation flap distraction osteogenesis in the treatment of unicoronal synostosis and provide case examples.The video can be found here: https://vimeo.com/519505008.

Long-term ophthalmic outcomes in 120 children with unilateral coronal synostosis: a 20-year retrospective analysis

Prior studies comparing ophthalmic outcomes after treating unicoronal synostosis (UCS) by early endoscopic strip craniectomy (ESC) versus later fronto-orbital advancement (FOA) are modest in sample size, or lack consistent age adjustment. We report long-term, age-adjusted ophthalmic outcomes for a large cohort after nonrandomized treatment by one of these two options. The following data was retrieved from a retrospective review of the medical records of patients with treated UCS born since 2000: cycloplegic refractions, sensorimotor examinations, and strabismus procedures before craniofacial repair and postoperatively at approximately 18 and 60months of age. V-pattern strabismus was graded as mild (absent or+1/-1 oblique dysfunction) versus moderate-to-severe (≥+2/-2 oblique dysfunction or left to right vertical alignment change of ≥20Δ or ocular torticollis >15°). A total of 120 infants were included: 60 treated by FOA and 60 by ESC. By the late examination, aniso-astigmatism was present in 72% of FOA-treated patients and 46% of ESC-treated patients (P<0.0001). By late examination, the age-adjusted odds ratio of moderate-to-severe V-pattern strabismus after treatment by FOA versus ESC was 2.65 (95% CI, 1.37-6.28; P=0.02); strabismus surgery was performed in 26 infants treated by FOA compared with 13 treated by ESC (OR=2.8; P=0.02). Amblyopia developed in 60% of FOA-treated patients compared with 35% of those treated by ESC (OR 3.0; 95% CI, 1.3-6.7; P=0.02). Our age-adjusted ophthalmic results confirm better long-term outcomes after treatment of USC by endoscopic strip craniectomy. Recognition and referral of affected infants by the earliest months of life facilitates the opportunity for endoscopic repair.

Morphologic Severity of Craniosynostosis: Implications for Speech and Neurodevelopment

Background: Single-suture craniosynostosis (SSC) can be associated with neurodevelopmental deficits. We examined the correlation between morphologic severity and incidence of speech-language or psychological concerns. Methods: In 62 patients (33 sagittal, 17 metopic, and 12 unicoronal), morphologic severity was determined via preoperative computed tomography (CT). Severity metrics for sagittal, metopic, and unicoronal synostosis were adjusted cephalic index (aCI), interfrontal angle (IFA), and anterior cranial fossa area ratio (ACFR), respectively. Speech-language and psychological concerns were assessed at age ≥4.5 years and defined as recommendation for therapy or monitoring. Results: Mean assessment age was 5.7 years; 32% had a speech-language concern and 44% had a psychological concern; 44% had neither. Sagittal: Mean aCI of those with a speech-language concern (0.62) and those without (0.62) were equivalent ( P = .580), as were mean aCI of those with a psychological concern (0.62) and those without (0.62; P = .572). Metopic: Mean IFA with (117.9) and without (125.2) a speech-language concern were equivalent ( P = .326), as were mean IFA with (120.2) and without (123.2) a psychological concern ( P = .711). Unicoronal: Mean ACFR with (0.65) and without (0.69) a psychological concern ( P = .423) were equivalent. However, mean ACFR with (0.74) and without (0.63) a speech-language concern were not ( P = .022*). Bivariate rank correlation showed significant association between morphologic severity and speech-language score only for unicoronal synostosis (ρ = .722; P = .008*). Conclusion: A significant portion of patients with SSC had speech-language or psychological concerns. We found no correlation between morphologic severity and incidence of speech-language or psychological concerns for patients with sagittal or metopic synostosis. Morphological severity did correlate with speech concerns in patients with unicoronal synostosis.

Neurological Functional Connectivity in Unilateral Coronal Synostosis: A Side-Based Comparison.

Unicoronal synostosis (UCS) has been associated with reading, language, and social dysfunction. Limited brain function connectivity studies exist for UCS with none devoted to comparing outcomes by side of synostosis (left versus right-sided UCS). Twelve patients with surgically treated UCS, 7 right-sided and 5 left-sided, were age matched to healthy controls. Resting state functional MRI was acquired in a 3T Siemens TIM Trio scanner (Erlangen, Germany). Data was collected with intrinsic connectivity distribution and seed-connectivity analysis using BioImage Suite (Yale School of Medicine). Region of interest analysis was performed based on Brodmann areas related to emotional, executive, language, motor, and visuo-spatial function. Significance was set at P < 0.05. Compared to controls, all UCS patients demonstrated decreased connectivity in areas of the parietal and temporal cortices responsible for visuo-motor coordination and language function. Right UCS patients demonstrated decreased intrinsic connectivity in regions related to complex motor movement and proprioception relative to control subjects. Left UCS patients demonstrated decreased seed connectivity between regions of the parietal lobe and occipital lobe related to motor coordination, visual function, and language compared to right UCS patients. Unicoronal synostosis had decreased functional connectivity in regions associated with memory, visual information processing, and motor function. Moreover, left-sided UCS had decreased connectivity in circuits for motor coordination and language when compared to right-sided UCS. This study provides data suggestive of long-term sequelae of UCS that varies by sidedness, which may be responsible for neurocognitive impairments found in previous cognitive analyses.

Secondary vault reconstruction after open or minimal invasive correction for unisutural, multisutural or syndromic craniosynostosis: A cohort study on the impact of diagnosis and type of initial surgical technique

The aim of this study is to identify if certain types of craniosynostosis and/or surgical procedures are more likely to require a secondary intracranial procedure because of insufficient correction of skull shape or raised ICP after initial surgery. All consecutive cases with craniosynostosis that were operated at our center between January 2010 and January 2019 were included and the number of secondary operations of the vault were determined, as well as diagnosis, the indication and type and timing of initial surgery. Monobloc and facial bipartition procedures were excluded. Over a nine year period, 790 vault corrections were performed in 780 patients of which 38 procedures were reoperations in 35 patient because of raised intracranial pressure or an insufficient esthetic result of the skull shape. Particularly patients with a multisutural or syndromic craniosynostosis are represented in this group, as well as three surgical procedures: 1. biparietal outfracturing for sagittal synostosis; 2. endoscopic stripcraniectomy with helmet therapy for unicoronal, multisutural or syndromic craniosynostosis; 3. conventional occipital expansion for syndromic craniosynostosis. The risk of a second intracranial correction for insufficient outcome of skull shape or for raised ICP is related to type of synostosis and type of initial surgical technique. Particularly multisutural and syndromic craniosynostosis are more likely to require repeat surgery for these indications. Concerning initial technique, biparietal outfracturing does not correct sagittal synostosis sufficiently, stripcraniectomy with helmet therapy appears to undercorrect unicoronal, multisutural and syndromic synostosis, and conventional occipital expansion for multisutural and syndromic synostosis has poorer outcome than occipital expansion with distraction.

The Use of Periorbital Steroids to Reduce Postoperative Swelling in Fronto-orbital Advancement: An Analysis of Outcomes

PURPOSE: Marked facial swelling is a known consequence following fronto-orbital advancement (FOA), which can result in prolonged eye closure, patient discomfort, and postoperative hospitalization. There are limited reports on the efficacy and safety of periorbital steroids to help reduce facial swelling in craniofacial surgery, which has become standard practice at some centers. The purpose of this study is to compare outcomes with and without the use of periorbital steroids in patients undergoing FOA. METHODS: A retrospective chart review of patients who underwent FOA at Seattle Children’s Hospital between January 2012 and December 2019 was completed. All procedures were performed by 2 senior surgeons (R.A.H. and C.B.B.) in conjunction with a pediatric neurosurgeon. All patients received pre-, intra-, and postoperative care via a standardized clinical care pathway. In the periorbital steroid cohort, triamcinolone was administered as an injection into the subcutaneous tissues or soaked in gelfoam and placed in the frontal/periorbital region before closure of the scalp. Statistical significance between outcomes measures was determined using a 2-tailed unpaired Student’s t test or chi-square test as appropriate. RESULTS: A total of 167 patients were included in this study (80 control, 87 periorbital steroid). The majority of these patients underwent FOA for isolated metopic synostosis (52.1%) followed by multisuture synostosis (23.9%) and isolated unicoronal synostosis (18%). 15.6% of patients had craniosynostosis as part of a diagnosed craniofacial syndrome. The average postoperative length of stay following FOA was 4.3 ± 2.0 days. Criteria for discharge including adequate PO intake, appropriate pain control, removal of the surgical drain, and improvement in facial swelling with opening of at least 1 eye. The use of periorbital steroids resulted in a statistically significant decrease in the hospital length of stay (LOS) compared with controls for isolated metopic (12.5 hours; P = 0.031) and unicoronal (12 hours; P = 0.015) craniosynostosis; there was no statistically significant difference in LOS for multisuture craniosynostosis (5.2 hours; P = 0.329). There was a significantly higher rate of surgical site infection in patients who received periorbital steroids compared to controls (10.2% versus 2.5%; P = 0.041). All of these complications represented scalp wound infections requiring operative intervention. Ninety-one percent of patients required hospital readmission with an average LOS of 17.6 days and 36% required subsequent revision cranioplasty. There was no association between specific suture involvement, craniofacial syndrome, or age at FOA with infectious complications. CONCLUSIONS: The use of periorbital steroids has been reported in the literature to reduce facial swelling and shorten convalescence following FOA. This study demonstrates that there is a statistically significant decrease in hospital LOS with the use of periorbital steroids in isolated suture craniosynostosis. However, it is associated with a significantly higher rate of infectious complications requiring operative intervention, extended hospital readmissions, prolonged antibiotic therapy, and secondary reconstruction.

Endoscopic Versus Open Cranial Reconstruction Surgery for Anterior Craniosynostosis: Experience from South-East Asia

Introduction and objective: Craniosynostosis is a pathological condition resulting from premature closure of cranial sutures. Open cranial vault reconstruction has been the standard of care for isolated non-syndromic craniosynostosis. The current centre introduced the endoscopic craniosynostosis surgery in 2015. The aim of the study is to compare safety outcomes of endoscopic versus open surgery in craniosynostosis within the context of a developing healthcare system, with a focus on anterior synostosis. Methods: This was a retrospective study of all patients undergoing surgery for anterior craniosynostosis from 2012 to 2018 at a single institution. Data on age at surgery, demographics, sutures, surgery duration and complications, including follow up information was obtained. We excluded syndromic patients and patients with pan-synostosis. Statistical analysis was undertaken with P value set at &lt;.05. Results: There were totally 40 patients who underwent craniosynostosis surgery. Seventeen patients underwent endoscopic correction, and 23 open procedures. Metopic and coronal suture patients were equally distributed, with an equal sex distribution. The mean age at surgery in the endoscopic group was 3.7 months (3-5) and higher at 11.5 months (8-18) in the open group ( P &lt; .05). The duration of surgery, blood loss and overall length of hospital stay were significantly reduced in the endoscopic group ( P &lt; .05). In the endoscopic cohort, patients with metopic synostosis obtained complete correction, while patients with unicoronal synostosis had minor residual frontal asymmetry. There were no immediate post-operative complications in the endoscopic group, but the open surgery cohort had minor dural tears in 6 patients. Conclusion: The endoscopic technique has a better safety profile than open surgery for craniosynostosis and results in a complete correction in patients with metopic and bicoronal craniosynostosis.

Facial Asymmetry in Nonsyndromic and Muenke Syndrome–Associated Unicoronal Synostosis: A 3-Dimensional Study Based on Facial Surfaces Extracted From CT Scans

To quantify soft tissue facial asymmetry (FA) in children with nonsyndromic and Muenke syndrome-associated unicoronal synostosis (NS-UCS and MS-UCS), hypothesizing that MS-UCS presents with significantly larger FA than NS-UCS. Retrospective cohort study. Twenty-one children (mean age: 0.6 years; range: 0.1-1.4 years) were included in the study (NS-UCS = 14; MS-UCS = 7). From presurgical computed tomography scans, facial surfaces were constructed for analysis. A landmark guided atlas was deformed to match each patient's surface, obtaining spatially detailed left-right point correspondence. Facial asymmetry was calculated in each surface point across the face, as the length (mm) of an asymmetry vector, with its Cartesian components providing 3 directions. Mean FA was calculated for the full face, and the forehead, eye, nose, cheek, mouth, and chin regions. For the full face, a significant difference of 2.4 mm (P = .001) was calculated between the 2 groups, predominately in the transverse direction (1.5 mm; P < .001). The forehead and chin regions presented with the largest significant difference, 3.5 mm (P = .002) and 3.2 mm (P < .001), respectively; followed by the eye (2.4 mm; P = .004), cheek (2.2 mm; P = .004), nose (1.7 mm; P = .001), and mouth (1.4 mm; P = .009) regions. The transverse direction presented with the largest significant difference in the forehead, chin, mouth, and nose regions, the sagittal direction in the cheek region, and the vertical direction in the eye region. Muenke syndrome-associated unicoronal synostosis presented with significantly larger FA in all regions compared to NS-UCS. The largest significant differences were found in the forehead and chin regions, predominantly in the transverse direction.

3D printing guided surgery in the treatment of unicoronal craniosynostosis orbital dysmorphology.

The purpose of our study was to improve the minor asymmetries of fronto-orbital advancement (FOA) by introducing a simple model to guide the FOA in unicoronal synostosis which may help saving time and cost. A retrospective analysis of 16 consecutive patients with unicoronal synostosis corrected by FOA guided by a guide model. Patients with syndromic craniosynostosis or associated craniofacial anomalies were excluded from the analysis. In all cases, 3D mirror image models were used for guiding unilateral fronto-orbital advancement. Demographic, perioperative, and follow-up data were collected for comparison. Cranial and orbital volumes were documented preoperatively and postoperatively and compared with the non-synostotic side. The postsurgical appearance of the face was documented photographically and then evaluated and scored using the Whitaker scoring system. The study included nine males and seven females. The mean age of the patients at the time of the operation was 20.4 months. The mean follow-up duration was 36 months. Mean operative time was 170 min, mean anesthetic time was 230 min, mean blood loss was 50-80 ml, and the average hospital stay was 4.4 days. No relapse that required surgical correction was reported. There were improvements in the orbital indices and volume to be near equal to the normal side. Excellent to good results were obtained in all patients according to the Whitaker classification system. Residual deformity after FOA mandates another tool to optimize the results. Our study introduced a simple, easy, and applicable method to guide the FOA with lesser asymmetries.

Cognitive performance of preschool children with different types of non-syndromic craniosynostosis

AbstractBackground:Craniosynostosis is defined as a premature fusion of one or more cranial sutures. Several studies have revealed cognitive deficits in some children who had undergone surgery to treat craniosynostosis. However, no general distinction has been drawn in the cognitive abilities between the various types of craniosynostosis. The purpose of the present study was to analyze if there is a difference in cognitive and motor function among the different types of non-syndromic craniosynostosis in preschool children.Methods:Twenty-seven children with different types of non-syndromic craniosynostosis were assessed using the Wechsler Preschool and Primary Scale of Intelligence – Third Edition, as well as the Quantitative, Memory and Motor scales of McCarthy Scales of Children’s Abilities (MSCA). The children were aged between 3 and 5 years and 11 months. The various types of craniosynostosis were compared.Results:The unicoronal synostosis group performed significantly worse than the multisuture synostosis group on the MSCA Motor scale. No differences in cognitive functions were found between the various types of craniosynostosis.Conclusions:Children with unicoronal synostosis may experience impaired motor skills and screening of their motor ability is recommended.

A protocol of situation-dependent transfusion, erythropoietin and tranexamic acid reduces transfusion in fronto-orbital advancement for metopic and coronal craniosynostosis.

Assess the effect of a protocol of preoperative erythropoietin (EPO) and ferrous sulfate in addition to perioperative tranexamic acid (TXA) on blood transfusions in patients with coronal or metopic craniosynostosis undergoing cranial vault remodeling (CVR) with fronto-orbital advancement (FOA). Retrospective review of all coronal and metopic craniosynostosis patients undergoing CVR and FOA from March 2010 to June 2019 was performed. Before 2014 ("Control group"), all patients received blood transfusion at the start of surgery. In 2014, a protocol of preoperative EPO and ferrous sulfate with perioperative TXA and non-automatic transfusion was instituted ("Study group"). Patient demographics and anthropometrics, perioperative hemoglobin (Hb) levels, and transfusion details were collected and compared. Thirty-six patients met inclusion criteria. Twenty-one patients were in the control group, and 15 in the Study group. Nineteen patients had metopic synostosis, 11 had unicoronal synostosis, and 6 had bicoronal synostosis. There were no significant differences between groups in demographics, operative time, intraoperative crystalloid volume, craniofacial syndromes, or sutures affected. The Study group had higher preoperative Hb (13.9 ± 1.0 vs. 12.6 ± 0.8g/dL, p < 0.001), lower intraoperative Hb nadir (7.4 ± 1.8 vs. 9.2 ± 1.2g/dL) lower intraoperative transfusion rate (66.7% vs. 100%, p = 0.008), lower postoperative transfusion rate (0% vs 28.6%, p = 0.03), and exposure to fewer unique units of packed red blood cells (0.7 ± 0.6 vs. 1.5 ± 0.9units). Our protocol resulted in decreased transfusion needs. These results add valuable information to the growing body of work on transfusion reduction in craniosynostosis surgery.

Abstract 26: Neurologic Functional Connectivity In Unicoronal Craniosynostosis: A Side-based Comparison

Purpose: Unicoronal synostosis has been associated with impaired reading, language, and social function. Functional MRI (fMRI) can evaluate brain connectivity in targeted brain regions to compare imaging analysis to previously published clinical performance. fMRI was used in this study to compare brain function connectivity in unicoronal synostosis (UCS) and compare outcomes by cerebral dominance (left versus right-sided UCS). Methods: Twelve adolescents with surgically treated UCS, 7 right-sided and 5 left-sided, were individually matched to age, gender and handedness controls. Resting state fMRI was acquired in a 3T Siemens TIM Trio scanner (Erlangen, Germany). Data was collected with intrinsic connectivity distribution and seed-connectivity analysis with BioImage Suite (Yale School of Medicine). Region of interest (ROI) analysis was performed based on Brodmann’s areas (BA) related to emotional, executive, language, motor and visuo-spatial function, left BA5, 6, 7, 18, 19, 37, 39, and 44. BA5, 6, 7 are areas of the frontal and parietal cortex important for visuomotor coordination and complex movement. BA18, 19 are areas of the occipital lobe related to visual information processing. BA 37, 39, and 44 are areas of the fusiform, angular gyri and temporal lobe important for language function. P<0.05 was significant. Results: Compared to controls, all UCS patients demonstrated decreased connectivity in left BA7, bilateral BA39, and right BA41 (p<0.01)), which are areas of the parietal and temporal cortices responsible for vision, language function, and motor coordination. Right UCS patients demonstrated increased connectivity in right BA17, BA18, BA19, BA20, BA36 when compared to controls (p<0.05). In the ROI analysis, right UCS exhibited decreased connectivity between the anterior cingulate cortex and right BA18, 19, and 37 compared to controls (p<0.05). The increased connectivity in BA17-20 (visual processing regions) supports our previous neurocognitive study results of increased visual perception abilities in right UCS patients. The subsequent decreased connectivity between BA18-19 and the anterior cingulate cortex (a section of which is implicated in complex motor coordination) is supported by neurocognitive data that shows decreased visual-motor integration ability in right UCS. This decreased connectivity between the aforementioned areas and the anterior cingulate gyrus despite increased intrinsic connectivity may also imply discordance in connecting emotion and executive function to language and visual information in right UCS patients. Connectivity between the left parahippocampus and right BA36, 37, and 54 was increased (p<0.05). Compared to controls and right UCS, left UCS demonstrated decreased connectivity between left BA6 and left BA17 and 18 (p<0.05). Left UCS patients did not demonstrate significantly different intrinsic or seed-based connectivity to right UCS or controls otherwise. Conclusion: Unilateral coronal synostosis had decreased connectivity and greater potential for neurocognitive dysfunction in regions associated with memory, visual information processing, and motor function. Moreover, left-sided UCS had decreased connectivity in circuits crucial in complex motor movement when compared to right-sided UCS. This study provides data suggestive of long-term sequelae of UCS that varies by sidedness, which may underlie the different phenotypes of neurocognitive impairment found in previous cognitive analyses.

Skeletal Deformity in Patients With Unilateral Coronal Craniosynostosis: Perceptions of the General Public.

A two-alternative forced choice design was used to gather perceptual data regarding unicoronal synostosis (UCS). Cranial vault remodeling aims at improving the aesthetic appearance of infants with UCS by reshaping the forehead and reducing the potential for psychosocial discrimination. People's perception of craniofacial deformity plays a role in the stigma of deformity. The purpose of this study is to examine the relationship between objective skull deformity in UCS patients and laypersons' perception of skull normality. Forty layperson skull raters were recruited from the general public. Skull raters were asked to categorize 45 infant skull images as normal or abnormal. Twenty-one of the images were UCS skulls, and 24 were normal skulls. Skulls were displayed briefly on a computer to simulate a first impression scenario and generate a perceptual response. A χ 2 analysis and mixed-effects regression model were used to analyze the response data. Members of the general public were good at distinguishing between skull groups, χ 2 (1) = 281.97, P < .001. In addition, skull raters' responses were predicted by the severity of deformity in the UCS skulls (b = -0.10, z = -2.6, P = .010, CI: -0.18, -0.02). A skull with a deformity value of 2.8 mm (CI: 1.8, 4.1) was equally likely to be rated normal or abnormal. This is the first study to investigate the relationship between objective skull deformity in UCS and public perception. Laypersons were good at distinguishing the difference between normal and UCS skulls, and their perceptions of normality were predicted by the degree of skull deformity.

Reattachment of the Temporalis Muscle After Frontoorbital Advancement in Craniosynostosis Surgery.

Craniosynostosis surgery may result in temporal hollowing occasionally. Overexpansion of the normal side and undergrowth of the affected side exacerbate the problem in unilateral cases (like unicoronal synostosis). Temporalis muscle lies in the temporal fossa, and it is usually severed or detached from its origin in order to reach the lateral aspect of the fronto-orbital bar. Reattachment of the temporalis muscle is challenging especially when the orbital bar is moved forward. In this technical note, exposure of the lateral cranial vault with zigzag incision of the temporalis muscle in 35 nonsyndromic anterior plagiocephaly patients has been described in detail, and the ease of reattachment is demonstrated afterward.

Cranioorbital Morphology Caused by Coronal Ring Suture Synostosis.

Minor cranial sutural synostosis is currently regarded as a rare diagnosis. As clinical awareness grows, a greater number of cases are being documented. This study aims to describe the variants of unicoronal synostosis with regard to major and minor sutural involvement and secondary effects on cranial and orbital morphology. The information is aimed to improve clinical diagnosis and management. A retrospective study was conducted collecting preoperative computed tomographic scans of patients diagnosed with unicoronal synostosis and listed for surgical interventions, identified from a craniofacial database. Within these patients, different synostotic variants were identified based on which suture was affected. Scans of normal pediatric skulls (trauma) were used for a control group. Computed tomographic scans were analyzed for sutural involvement, cranial base deflection, and ipsilateral and contralateral orbital height and width. One-way analysis of variance was used to detect differences between synostotic variants and controls. A total of 57 preoperative computed tomographic scans of patients with unicoronal synostosis were reviewed, in addition to 18 computed tomographic scans of normal skulls (control group). Four variants of unicoronal synostosis were identified: frontoparietal, frontosphenoidal, frontoparietal and frontosphenoidal, and frontosphenoidal and frontoparietal. The last two variants differ in their temporal involvement in the direction of sutural synostosis and ultimately cranial and orbital morphology. Three variants have been previously identified, but the fourth is presented for the first time. An understanding of the variants of unicoronal synostosis and their temporal relationships is integral for accurate clinical diagnosis and surgical correction. Recommendations for treatment are based on discrete changes in orbital morphology.

Comparison of Black Bone MRI and 3D-CT in the preoperative evaluation of patients with craniosynostosis

Black Bone (BB) magnetic resonance imaging (MRI) is a nonionizing imaging method and a recent alternative to computed tomography (CT) in the examination of cranial deformities. The purpose of this study was to compare BB-MRI and routine 3D-CT in the preoperative evaluation of patients with craniosynostosis. At our center, we have routinely performed preoperative CT of the skull and brain MRI for patients with clinical suspicion of craniosynostosis. We recently changed our MRI protocol into one that includes sequences for the evaluation of both brain anatomy and skull bone and sutures by BB-MRI. A semi-automatic skull segmentation algorithm was developed to facilitate visualization. Both BB-MRI and 3D-CT were performed on 9 patients with clinical craniosynostosis, and the images were evaluated by two craniofacial surgeons, one pediatric neurosurgeon, and two neuroradiologists. We obtained informative 3D images using BB-MRI. Six (6/9) patients had scaphocephaly, 1 (1/9) patient had unicoronal synostosis, and 2 (2/9) patients had lambdoid synostosis. The affected synostotic sutures could be identified both by BB-MRI and by 3D-CT in all patients. Intra-rater and inter-rater reliability for rating the calvarial sutures was high. However, the reliability for rating the intracranial impressions was low by both imaging methods. BB-MRI is an alternative to 3D-CT in the preoperative evaluation of patients with craniosynostosis. BB-MRI provides information not only on cranial sutures and intracranial impressions but also on the brain structure in one imaging session. This method can replace ionizing radiation-based methods in analyzing skull deformities.

Extraocular muscle positions in anterior plagiocephaly: V-pattern strabismus explained using geometric mophometrics

IntroductionOphthalmological involvement in anterior plagiocephaly (AP) due to unicoronal synostosis (UCS) raises management challenges. Two abnormalities of the extraocular muscles (EOM) are commonly reported in UCS without objective quantification: (1)...