To summarize the ophthalmic manifestations of unilateral coronal synostosis patients.
We performed a literature search in the electronic database of PubMed, CENTRAL, Cochrane, and Ovid Medline guided by Preferred Reporting Items for Systematic Reviews and Meta-Analysis Statement for studies evaluating ophthalmic manifestations of unilateral coronal synostosis.
Unilateral coronal synostosis, also called unicoronal synostosis, may be mistaken for deformational plagiocephaly, an asymmetric skull flattening common in newborns. Characteristic facial features, however, distinguish the two. Ophthalmic manifestations of unilateral coronal synostosis include a \"harlequin deformity\", anisometropic astigmatism, strabismus, amblyopia, and significant orbital asymmetry. The astigmatism is greater on the side opposite the fused coronal suture. Optic neuropathy is uncommon unless unilateral coronal synostosis accompanies more complex multi-suture craniosynostosis. In many cases, surgical intervention is recommended; without intervention, skull asymmetry and ophthalmic disorders tend to worsen with time. Unilateral coronal synostosis can be managed by early endoscopic stripping of the fused suture and helmeting through a year of age or by fronto-orbital-advancement at approximately 1 year of age. Several studies have demonstrated that anisometropic astigmatism, amblyopia, and severity of strabismus are significantly lower after earlier intervention with endoscopic strip craniectomy and helmeting compared to treatment by fronto-orbital-advancement. It remains unknown whether the earlier timing or the nature of the procedure is responsible for the improved outcomes. As endoscopic strip craniectomy can only be performed in the first few months of life, early recognition of the facial, orbital, eyelid, and ophthalmic characteristics by consultant ophthalmologists enables expeditious referral and optimized ophthalmic outcomes.
Timely identification of craniofacial and ophthalmic manifestations of infants with unilateral coronal synostosis is important. Early recognition and prompt endoscopic treatment appears to optimize ocular outcomes.

This study aimed to compare the bony relapse ratios of standard and accelerated distraction protocols (ADP) in one-piece fronto-orbital distraction osteogenesis (DO). Patients with unilateral or bilateral coronal synostoses who underwent one-piece fronto-orbital DO were included. The accelerated cranial distraction protocol included a 3-day latency period and a distraction rate of 1-2 mm/day, followed by a 4-week consolidation period. Intracranial volume was measured using computed tomography (CT) before the surgery, at the end of the consolidation period, and 1 year after the removal of distractors. The intracranial volume changes and relapse ratios were calculated. This study included 32 patients; of these, 16 were included in each of the ADP and standard protocol (SP) groups. The mean ages were 1.4 years and 1.6 years in the ADP and SP groups, respectively (p = 0.895). In the ADP and SP groups, the expanded volumes were 270.9 ± 90.3 cm3 and 284.6 ± 149.7 cm3 (p = 0.91) and the growth-corrected expanded volumes were 162.1 ± 67.5 cm3 and 177.1 ± 105.2 cm3, respectively (p = 0.867). The relapse and growth-corrected relapse ratios showed no significant differences between the two groups, suggesting similar stability between the two protocols. The relapse ratios were 7.1 ± 4.8% and 7.3 ± 5.0% (p = 0.91) and the growth-corrected relapse ratios were -3.0 ± 3.3% and -2.4 ± 2.7%, respectively (p = 0.498). Within the limitations of the study, it seems that the ADP can shorten the distraction period without compromising stability. This may contribute to resolving the disadvantages and highlighting the advantages of DO.

Fronto-orbital advancement remains a powerful technique for the correction of anterior cranial vault differences related to metopic (trigonocephaly) or unilateral coronal (anterior plagiocephaly) craniosynostoses. Traditional fronto-orbital advancement requires access to the forehead and superior 2/3 of the orbit via a coronal incision. The frontal bone and orbital segment (bandeau) are then separated from the skull and reshaped. In patients with metopic craniosynostosis, the bandeau and frontal bone will need to be advanced and widened. In patients with unilateral coronal craniosynostosis, the bandeau will need to be \"untwisted\" to address the supraorbital retrusion on the affected side, the affected orbit will need to be shortened and widened, and the frontal bone flap will need to be proportionately advanced on the affected side. Overcorrection of the affected dimension should be undertaken to account for growth and relapse.

BACKGROUND: To report refractive outcomes, describe types of strabismus and evaluate the outcomes of surgical intervention for unilateral coronal synostosis (UCS) in paediatric patients.
METHODS: This study retrospectively included 30 UCS cases. Patients aged from 3 months to 6 years (median: 1.8 years) were enrolled from January 2018 to December 2019 at Shanghai Children\'s Hospital. Sixteen patients had all types of strabismus; 15 of these patients underwent surgery.
RESULTS: Refractive errors of 30 cases were included. In 60% of patients, astigmatism of 1.00D or more existed in not less than one eye at last record. Twenty (66.7%) patients had the larger amount of astigmatism in the contralateral eye. Fifteen patients received strabismus surgery, of whom 6 patients with monocular elevation deficiency (MED) underwent the standard Knapp procedure, with or without a horizontal deviation procedure. Fifteen cases were horizontally aligned within 5 prism dioptres (Δ). Six patients with MED (100%) had attained ≥25% elevation improvement after surgery, and the vertical deviation decreased from 25.83 Δ ± 4.92 Δ (range, 20 Δ-30 Δ) to 0.83 Δ ± 4.92 Δ after surgery (range, 0 Δ-10 Δ), for an improvement of 26.67 Δ ± 4.08 Δ (t = 16 P < 0.05). In 1 patient with esotropia, the horizontal deviation decreased from + 80 Δ to + 5 Δ after surgery. One patient was diagnosed with trichiasis and one with contralateral lacrimal duct obstruction.
CONCLUSIONS: Contralateral MED was also the main type of strabismus in UCS. Superior oblique muscle palsy was still the most common, as previously reported. There is a risk of developing a higher astigmatism and anisometropia in the contralateral eye to synostosis. Other ophthalmic disorders should be treated in a timely manner.
BACKGROUND: The study was approved by the Institutional Review Board of Shanghai Children\'s Hospital (approval No. 2020R023-E01) and adhered to the tenets of the Declaration of Helsinki. Ethics approval was procured on March 30, 2020. This was a retrospective study. Written informed consent was sought from the patients\' parents or legal guardians. Clinical Trials Registry number: ChiCTR2000034910 . Registration URL: http://www.chictr.org.cn/showproj.aspx?proj=56726 .

V pattern strabismus is the most common ocular motor disorder reported in patients with craniosynostosis. Strabismus management may prove challenging, and few studies provide perspective on surgical approach. The purpose of this review is to discuss evaluation and surgical options for treating V pattern strabismus in patients with craniosynostosis. We provide a step-by-step approach to facilitate surgical planning.

BACKGROUND: Unilateral coronal synostosis (UCS) is a complex craniosynostosis, combining malformations of the upper (frontal bone, orbito-naso-frontal bandeau (ONFB) ) and the middle thirds of the face. In our centre, the surgical correction consists in the repositioning of the ONFB in front of the zygomaticofrontal suture on the affected side. Defects in the corrections have been observed post-operatively for some patients with a persistent asymmetry in the side walls of the orbits. The purposes of our study were to perform an analysis of the frontal process of zygoma (FPZ) in children affected by UCS using preoperative CT-scans and to consider the modification of surgical techniques in order to achieve a better ONFB repositioning and thus, better symmetry.
METHODS: The preoperative CT-scans of 13 children with UCS who underwent corrective surgery in our department from 2005 to 2016, were analyzed. After the selection of 6 morphological points in 2 and 3 dimensions using planning software, the sutures constituting the coronal arch and the sagittal distances between the coronal plane and the zygomaticofrontal sutures were analyzed. We compared the measurements on the pathological side to the healthy side, the non-affected side being the reference side. The patients included were those for whom the lack of symmetry between the healthy side and the affected side was more than 2 mm. Statistical analyses were carried out using a Student t-test.
RESULTS: Of the 13 children, 10 (1 day to 42 months old) met the inclusion criteria. The mean sagittal distance between the coronal plane and the healthy zygomaticofrontal suture was 33.8 mm ± 5.43 mm [range: 25.9: 40.9] [median: 34.3]. On the pathological side, the distance was 28.75 mm ± 4.76 mm [range: 20.8: 36.3] [median: 29.55], (p = 0.04).
CONCLUSIONS: There is a significant asymmetry between the FPZ on the healthy and the pathological sides in children affected by UCS. This asymmetry is variable, therefore justifying a personalized surgical correction whichtakes into account not only the shape of the ONFB but also the degree of asymmetry of the FPZ. A prospective study with immediate preoperative CBCT image acquisition and long-term clinical and radiological follow-up, will be our next step.

OBJECTIVE The sagittal suture is usually considered an external anatomical landmark, indicating the location of the superior sagittal sinus (SSS) for surgical approaches. Children with unilateral coronal synostosis (UCS) often present with an important deviation of the sagittal suture. Because these patients usually undergo frontal reconstruction or even endoscope-assisted minimally invasive procedures, it is imperative to know the location of the SSS. The aim of this investigation was to study the anatomical relationship between the SSS and the sagittal suture in children with anterior plagiocephaly. METHODS The authors retrospectively studied the relationship between the sagittal sinus and the sagittal suture at 5 points: nasion, midpoint nasion-bregma, bregma, midpoint bregma-lambda, and lambda. The study analyzed CT scans of 50 children with UCS admitted to the craniofacial unit of Necker Enfants Malades Hospital between March 2006 and March 2013 and compared them with 50 control children with no evidence of craniosynostosis, bone disease, or genetic syndromes. The authors also analyzed the presence of extracerebral fluid collection and ventricular asymmetry in children with UCS. RESULTS Fifty-six percent of patients had anterior right UCS and 44% had left-sided UCS. Type I UCS was seen in 1 patient, Type IIA in 20 patients, Type IIB in 20 patients, and Type III in 9 patients. The authors found that the nasion is usually deviated to the ipsilateral side of the synostosis, the bregma contralaterally, and the lambda ipsilaterally. The gap distances between the reference point and the SSS were 0-7.3 mm (mean 1.4 mm) at the nasion; 0-16.7 mm (mean 3.8 mm) at the midpoint nasion-bregma; 0-12 mm (mean 5.8 mm) at the bregma; 0-9.5 mm (mean 3 mm) at the midpoint bregma-lambda; and 0-11.6 mm (mean 5.5 mm) at the lambda. Conversely, a discrepancy of more than 1 mm between the SSS and the position of the suture was found only in 7 control cases (14%). Of patients with UCS, 38% presented with an extracerebral fluid collection contralateral to the fused coronal suture. Fifty-two percent had a ventricular asymmetry, which was characterized by reduced ventricular volume ipsilateral to the synostosis in all but 1 patient. CONCLUSIONS In this study, the SSS was usually deviated contralaterally to the closed coronal suture. It tended to be in the midline of the cranial vault and could be projected virtually along an imaginary line passing through the midline of the cranial base. The authors recommend a distance of 37 mm from the sagittal suture as a safety margin during surgery.

Apert syndrome is a congenital craniosynostosis syndrome comprising of bilateral coronal synostosis , symmetric syndactyly of hands and feet and midface hypoplasia. We present an atypical phenotype of this syndrome with right sided unilateral coronal synostosis. However, type I apert hand and other clinical and radiological features suggestthe diagnosis. Genetic analysis revealed an absence of the specific missense mutations in the FGFR 2 gene that is found in patients with this syndrome. We conclude that this patient represented a rare atypical variant of Apert syndrome. Further analysis is required to map the associated genotype.

We report on a case where craniosynostosis surgery for a left-sided coronal synostosis was performed successfully on an 11-month old infant with a hypoplastic left ventricle with a dysplastic mitral valve, double outlet right ventricle, transposition of the great arteries, atrial septal defect, multiple ventricular septal defects, and surgically applied pulmonary banding. Craniosynostosis surgery is considered high-risk surgery, because of possible sudden and extensive blood loss, and is usually performed in cardiopulmonary healthy children. Children with congenital heart disease undergoing noncardiac surgery have an increased risk of perioperative morbidity and cardiac arrest. Patients with hypoplastic left heart syndrome are a high-risk population when undergoing noncardiac surgery, in all stages of palliation. This infant would be undergoing a partial cavo-pulmonary connection (PCPC) within a few months. With a PCPC, drainage of cranial vessels is dependent on passive flow via the superior caval vein directly into the pulmonary artery. Consequently, this could lead to an increased blood loss during craniosynostosis surgery. Therefore, it was decided to perform the craniosynostosis surgery first, before establishing a PCPC. When a child with CHD presents for high-risk noncardiac surgery, future cardiac procedures and physiology also have to be taken into account. A multidisciplinary approach, involving pediatric cardiologists and pediatric anesthesiologists, is essential in making this decision.

Plagiocephaly has traditionally been corrected by unilateral or bilateral frontal bone advancement or rotation using bone-molding forceps and distraction devices. Complete symmetrical correction of deformed frontal bones is considered almost impossible because the curvature of each frontal bone varies. We evaluated the feasibility of measuring the optimal curvature of frontal and occipital bones using a plaster skull model and applying these measurements to \"switch\" them for simultaneous correction of frontal and occipital plagiocephaly. A 2-year-old girl suffering from unifrontal flattening visited our clinic. Unilateral coronal synostosis was observed. The 3-D rapid prototype model and skull replica method using thin paper clay were used for preplanned virtual surgery. The triangular bone was harvested from the contralateral bulging side of the occipital bone (\"occipitofrontal switching\") for the best optimal curvature in the affected frontal bone. Another triangular bone was harvested from the ipsilateral flattened side of the frontal bone, and bones were switched with each other. Further bending of the frontal or occipital segment was not necessary for optimal curvature. Symmetrical correction was made by switching the triangular bone of the frontal area with that of the contralateral occipital area. Revision has not been necessary, and infection was not observed at 1-year follow-up. Our novel technique of preplanning surgery using a 3-D plaster model for simultaneous correction of frontal and occipital plagiocephaly is effective and time-saving.