(1) Background: Non-syndromic unicoronal craniosynostosis (UCS) is associated with a high prevalence of ocular anomalies. Currently, the etiology of this association remains obscure, however, it is presumed to be primarily attributed to their orbital malformations and/or secondary to craniofacial surgery. We assessed pre-operative ophthalmological examinations of non-syndromic UCS patients and compared them with their postoperative outcomes and long-term follow-up. (2) Methods: A retrospective case series was conducted on medical records of patients with non-syndromic UCS at Sophia Children\'s Hospital, Rotterdam. Ophthalmologic examinations were collected at different time periods: T1 (first visit), T2 (<1 year after cranioplasty), and T3 (long-term follow-up at last visit). The McNemar\'s test was used for statistical analysis. (3) Results: A total of 101 patients were included, for whom examinations were available at T1 and T3. Patients had a mean age of 2.8 years (±2.7) and 9.5 (±4.9) at T1 and T3, respectively. At T1, 52 patients (51.5%) were diagnosed with strabismus, and 61 patients (60.4%) at T3. Vertical strabismus increased significantly from 23 patients (22.8%) at T1 to 36 patients (35.6%) at T3 (p = 0.011). Followed by astigmatism, which increased significantly from 38 (37.6%) at T1 to 59 (58.4%) patients at T3 (p = 0.001). T1 was available in 20 patients prior to fronto-orbital advancement (FOA), therefore, a sub-analysis was conducted on these patients, which was followed shortly after FOA at T2. Prior to FOA, strabismus was present in 11 patients (55.0%) and in 12 patients (60.0%) at T2. After FOA, strabismus worsened in two patients. (4) Conclusions: This study showed the high prevalence of ocular anomalies in patients with non-syndromic UCS before and after cranioplasty and at long-term follow-up. The findings of this study show that ophthalmic and orthoptic examinations are an important part of the optimal treatment of patients with non-syndromic UCS.

Fronto-orbital advancement and remodeling (FOAR) is among the most common surgical approaches for unicoronal craniosynostosis (UCS), although some data demonstrate failure to achieve long-term aesthetic normalcy, leading some to seek alternative treatment paradigms such as fronto-orbital distraction osteogenesis (FODO). This study compares long-term aesthetic outcomes of patients with UCS treated with FOAR and FODO.
Twenty patients (four males) with non-syndromic UCS presenting to our institution and undergoing distraction were compared to a matched cohort of 20 patients (six males) undergoing FOAR. Clinical photographs and ImageJ were used to quantify periorbital anatomy including palpebral fissures, pupil-to-brow distance (PTB), and margin-reflex distance (MRD1) in pixels. Whitaker classification was blindly assigned by craniofacial surgeons.
Photogrammetric analysis and Mann-Whitney U tests demonstrated significantly improved postoperative symmetry in distraction patients for palpebral width (p = 0.020), MRD1 (p = 0.045), and canthal tilt (p = 0.010). Average Whitaker classification scores between FOAR (1.94) and distraction (1.79) cohorts were similar (p = 0.374).
UCS patients demonstrated significant postoperative improvements in periorbital symmetry, with distraction patients demonstrating superior results in palpebral width and canthal tilt. FOAR and FODO patients achieved similar Whitaker classification scores. These cohorts will be followed until craniofacial maturity prior to making any definitive conclusions.

Unicoronal craniosynostosis correction with fronto-orbital advancement and cranial vault remodeling has traditionally been the gold standard. Distraction osteogenesis has the advantage of increased size of movement without constriction of the scalp and decreased morbidity. Although fronto-orbital advancement and cranial vault remodeling are usually performed at 6 months of age or later, distraction osteogenesis is performed at a younger age, between 3 and 6 months, to take advantage of the infant bony physiology. Herein, the authors demonstrate a case of distraction osteogenesis for unicoronal craniosynostosis in a 3-month-old female with significant improvement of her orbital, nasal, and frontal symmetry. The video can be found here: https://vimeo.com/519047922.

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.

BACKGROUND: Endoscopic strip craniectomy with helmeting (ESCH) has been shown to be a safe and efficacious alternative to fronto-orbital remodeling (FOR) for selected children with craniosynostosis. In addition to clinical factors, there may be economic benefits from the use of ESCH instead of FOR.
METHODS: A retrospective review of 23 patients with nonsyndromic unicoronal craniosynostosis (UCS) treated with FOR was carried out at Great Ormond Street Hospital (GOSH) for Children in London, UK. Secondary data were used for the ESCH cohort from a paper published by Jimenez and Barone (2013). Data were collected on surgical time, transfusion rates, length of hospital stay, adverse event rates, reintervention rates, and overall costs. Costs were categorized and then assigned to the appropriate data sets.
RESULTS: The mean age of patients undergoing FOR (vs. ESCH) was 17.4 mo (vs. 3.1 mo) with a mean surgical time of 234 min (vs. 55 min), mean transfusion volume of 221.6 mL (vs. 80.0 mL), mean transfusion rate of 14/23 (vs. 2/115), and a total immediate overnight stay of 3.13 days (vs. 97% next-day discharge). The FOR group had a higher adverse event rate (5/23 vs. 4/115, p=<0.005) and a higher number requiring extraocular muscle surgery (4/23 vs. 7/109, p=0.16). There was a substantial difference in overall costs between the two groups. Total variance cost for the FOR group was £7436.5 vs. £4951.35, representing a cost difference of £2485.15 over the 24-month study period.
CONCLUSIONS: ESCH, in comparison to FOR, appears as a more economical method in the management of USC patients, as well as having clinical benefits including reduced adverse event rate and improved ophthalmic outcomes.

Anterior plagiocephaly due to unicoronal craniosynostosis is commonly associated with an ipsilateral hypertropia and a contralateral head tilt. The ipsilateral superior oblique weakness has been attributed to the shortening of the orbital roof with retroplacement of the trochlea, and to heterotopic positioning of the vertical rectus muscles within the orbit. These anatomical aberrations may alter ocular rotations to mimic a true congenital superior oblique palsy. This paper describes two siblings with synostotic plagiocephaly and simulated superior oblique palsy with ocular torticollis successfully managed by strabismus surgery. Synostotic plagiocephaly should be included in the differential diagnosis of familial congenital superior oblique palsy.