The Incidence and Morphology of Deformational Plagiocephaly in Unicoronal Synostosis.

It has been our observation that unicoronal synostosis and deformational plagiocephaly (DP) are not mutually exclusive. The incidence and phenotypical manifestations of a patient presenting with both conditions have not been studied previously. The authors performed a retrospective review of patients presenting with unicoronal synostosis, examining their 3DCT scans for the presence of DP and assessing their endocranial and orbital morphology. The authors found that 38.4% of patients with unicoronal synostosis also have DP. Ipsilateral DP is less common than contralateral DP, but these patients tend to have a delayed presentation due to a reduction in forehead asymmetry corresponding to the parallelogram deformity. Regardless, DP has no impact on the endocranial nor orbital morphology in unicoronal synostosis.

Early Bone and Suture Reformations in Different Cranial Regions After Cranial Vault Remodeling for Sagittal Craniosynostosis.

Cranial vault remodeling (CVR) is a common procedure for correcting sagittal craniosynostosis. Some approaches leave significant craniectomy defects. The authors investigated the reosteogenesis in different cranial defect areas after CVR. A cross-sectional study was conducted in nonsyndromic sagittal craniosynostosis. Available early postoperative computed tomography (CT) scans were analyzed. The segmentation of three-dimensional reconstructed images was performed. Different cranial defect areas, including coronal, vertex, and occipital regions, were further investigated using an automated three-dimensional analysis software for reosteogenesis percentage. Forty-four CT scans were included. The average age at CVR was 8.8 months. The median time of postoperative CT scans was 6.1 weeks. The median bone reformation percentage of the entire cranial defect was 56.7%. Given the similar postoperative CT timing, the median bone reformation at the coronal, vertex, and occipital areas demonstrated 44.21%, 41.13%, and 77.75%, respectively (P < 0.001). In the simultaneously removed coronal and lambdoid sutures, there were 45% with coronal and lambdoid sutures reformation, followed by lambdoid suture reformation alone, no suture reformation and coronal reformation alone in 35%, 20%, and 0%, respectively (P = 0.013). There was no coronal reformation in the removed coronal suture group. However, 40% demonstrated lambdoid suture reformation after the isolated lambdoid suture removal. The occipital region has the highest reosteogenesis compared with the other cranial defects after CVR in nonsyndromic sagittal craniosynostosis. Within the removed previous patent sutures, the lambdoid suture reformation showed a higher rate than the coronal suture.

Long-Term Follow-Up of Apert Syndrome Following Mid-Face Advancement: More Than 3 Decades Later.

Apert syndrome is characterized by craniosynostosis, a hypoplastic mid-face, skeletal abnormalities, symmetric syndactyly of the hands and feet, and a degree of neurocognitive impairment. Long-term outcomes of patients who have undergone surgical correction of Apert syndrome are limited. The authors present the case of a 73-year-old female with Apert syndrome, with follow-up of more than 3 decades following a fronto-orbital advancement and Le Fort III advancement. Clinical and radiologic images demonstrate a degree of skeletal resorption and relapse that is likely unavoidable. This provides insight into the long-term skeletal stability and esthetic outcomes for patients with Apert syndrome.

VACTERL Association in Patients With Metopic Synostosis: Is There a Link?

VACTERL association is diagnosed based on the non-random co-occurrence of at least 3 out of 6 congenital malformations. The prevalence is thought to be less than 1 in 10,000 to 1 in 40,000. There is no known link between VACTERL association and metopic synostosis in the literature. There were 122 operated cases of metopic synostosis at our institution from 1999 to 2023, with a 2.3:1 male-to-female ratio. The authors describe the co-occurrence of VACTERL association and metopic synostosis in 3 female patients with no identifiable genetic variants. Given that VACTERL association is a diagnosis of exclusion, other rare syndromes were considered but ultimately excluded. This suggests that the co-occurrence of VACTERL association and metopic synostosis is a potentially rare finding, and underlying pathogenic variants are yet to be identified.

Characterization of a full-thickness decellularized and lyophilized human placental membrane for clinical applications.

Allografts derived from live-birth tissue obtained with donor consent have emerged as an important treatment option for wound and soft tissue repairs. Placental membrane derived from the amniotic sac consists of the amnion and chorion, the latter of which contains the trophoblast layer. For ease of cleaning and processing, these layers are often separated with or without re-lamination and the trophoblast layer is typically discarded, both of which can negatively affect the abundance of native biological factors and make the grafts difficult to handle. Thus, a full-thickness placental membrane that includes a fully-intact decellularized trophoblast layer was developed for homologous clinical use as a protective barrier and scaffold in soft tissue repairs. Here, we demonstrate that this full-thickness placental membrane is effectively decellularized while retaining native extracellular matrix (ECM) scaffold and biological factors, including the full trophoblast layer. Following processing, it is porous, biocompatible, supports cell proliferation in vitro, and retains its biomechanical strength and the ability to pass through a cannula without visible evidence of movement or damage. Finally, it was accepted as a natural scaffold in vivo with evidence of host-cell infiltration, angiogenesis, tissue remodelling, and structural layer retention for up to 10 weeks in a murine subcutaneous implant model.

Heterozygous FGFR3 c.138G>A Mutation Presenting With Achondroplasia and Hemifacial Microsomia.

We present a case of a patient with achondroplasia, hemifacial microsomia and an fibroblast growth factor receptor3 c.138G>A mutation. An association between the 2 conditions has not been previously described, but there is biological plausibility that the etiology of the 2 conditions is linked.

Secondary metopic craniosynostosis after posterior cranial decompression in cloverleaf skull deformity.

Cloverleaf skull deformity or Kleeblattschadel syndrome is a severe condition where multiple cranial sutures are absent and prematurely fused, leading to a trilobate head shape. The remaining open sutures or fontanelles compensate for rapid brain expansion, while the constricted fused calvarium restricts brain growth and results in increased intracranial pressure. Recent data show that early posterior cranial and foramen magnum decompression positively affects infants with cloverleaf skulls. However, long-term sequelae are still rarely discussed. We hereby report a child who developed secondary metopic craniosynostosis after posterior cranial decompression, which required a front-orbital advancement and cranial remodelling as a definitive procedure.

Diagnostic Accuracy of Point-of-Care Ultrasound Versus Radiographic Imaging for Pediatric Distal Forearm Fractures: A Randomized Controlled Trial.

STUDY OBJECTIVE: In patients aged 5 to 15 years with a clinically nondeformed distal forearm injury presenting to the emergency department (ED), we examined whether point-of-care ultrasound or radiographic imaging had better diagnostic accuracy, with the reference diagnosis determined by an expert panel review. METHODS: This multicenter, open-label, diagnostic randomized controlled trial was conducted in South East Queensland, Australia. Eligible patients were randomized to receive initial imaging through point-of-care ultrasound performed by an ED clinician or radiograph. Images were defined as "no," "buckle," or "other" fracture by the treating clinician. The primary outcome was the diagnostic accuracy of the treating clinician's interpretation compared against the reference standard diagnosis, which was determined retrospectively by an expert panel consisting of an emergency physician, pediatric radiologist, and pediatric orthopedic surgeon, who reviewed all imaging and follow-up. RESULTS: Two-hundred and seventy participants were enrolled, with 135 randomized to each initial imaging modality. There were 132 (97.8%) and 112 (83.0%) correctly diagnosed participants by ED clinicians in the point-of-care ultrasound and radiograph groups, respectively (absolute difference [AD]=14.8%; 95% confidence interval [CI] 8.0% to 21.6%; P<.001). Point-of-care ultrasound had better accuracy for participants with "buckle" fractures (AD=18.5%; 95% CI 7.1% to 29.8%) and "other" fractures (AD=17.1%; 95% CI 2.7% to 31.6%). No clinically important fractures were missed in either group. CONCLUSION: In children and adolescents presenting to the ED with a clinically nondeformed distal forearm injury, clinician-performed (acquired and interpreted) point-of-care ultrasound more accurately identified the correct diagnosis than clinician-interpreted radiographic imaging.

Re-imagining early cloverleaf skull deformity management from front to back approach-30 years on.

The cloverleaf skull deformity remains among the most complicated craniofacial conditions to successfully manage. Many cases achieve largely unsatisfactory outcomes due to the requirement for frequent reoperation on the cranial vault and failure to deal with all the elements of the craniofaciostenosis in a timely fashion. Early cranial vault surgery without addressing the cranial base deformity and its attendant cerebrospinal fluid flow changes is invariably challenging and disappointing. A recent focus on the expansion of the posterior cranial vault as a primary procedure with the greater volume change allows a delay in fronto-orbital advancement and reduced need for repeat surgery. We herein describe three cases of complex multisuture craniosynostosis with cloverleaf skull deformity who underwent neonatal posterior cranial vault decompression along with foramen magnum decompression. Our report examines the safety and rationale for this pre-emptive surgical approach to simultaneously deal with the cranial vault and craniocervical junction abnormalities and thus change the early trajectory of these complex cases.

Measuring the Change in Soft Palate Length and Shape Following Maxillary Advancement: A Cohort Study in Patients with Orofacial Clefts.

OBJECTIVE: To evaluate a method of measuring the change in palatal length and shape following maxillary advancement using synchronous lateral videofluoroscopy and voice recording in order to understand how movement of the maxilla may affect VPI risk in patients with cleft lip and/or palate (CL/P). DESIGN: Retrospective cohort study of children with cleft lip and/or palate. SETTING: Single center, tertiary children's hospital. PARTICIPANTS: Patients with cleft lip and/or palate who underwent maxillary advancement between 2016-21 inclusive. INTERVENTIONS: Maxillary advancement surgery, including those who underwent concurrent mandibular procedures. MAIN OUTCOME MEASURES: The length of the soft palate and the genu angle were measured throughout palatal dynamic range. Pre- and post-operative measurements were compared using a one sided T-test, with subgroup analysis for patients with clinical VPI. RESULTS: Ten patients were examined. The mean distance of maxillary advancement was 10.5 mm. The average increase in pre-genu soft palate length was 2.8 mm in the resting position and 2.9 mm in the closed position. The genu angle decreased in the closed position by 16.3 degrees. CONCLUSIONS: The soft palate showed limited ability to lengthen following maxillary advancement and this may explain the risk of VPI. There was partial compensation by the muscle sling of the palate as demonstrated by a more acute post-operative genu angle and this suggests one reason for the variability of VPI reported. Future research is required to investigate how length and shape changes measured using this method can predict VPI risk.