The Use of Resorbable Fixation for Posterior Cranial Vault Distraction Osteogenesis.

SUMMARY: Posterior vault distraction osteogenesis (PVDO) traditionally relies on the use of cranial distractors affixed with titanium screws to the posterior cranial vault transport segment to allow for expansion of intracranial volume. We describe a novel technique using the Synthes RAPIDSORB IPS resorbable fixation system for stable fixation of the distraction hardware for PVDO. In a retrospective review of our experience with the IPS system, there was no instance of hardware or fixation failure. This off-label use of the IPS system results in a significant reduction in operative time for hardware removal while allowing for a more limited dissection, which can help reduce devascularization of the bony regenerate, bony relapse, and other complications.

Intracranial Pressure Patterns in Children with Sagittal Craniosynostosis.

BACKGROUND: Elevated intracranial pressure (ICP) in sagittal craniosynostosis has a wide spectrum of reported incidence, and patterns are not well understood across infancy and childhood. Characterizing the natural history of ICP in this population may clarify risks for neurocognitive delay and inform treatment decisions. METHODS: Infants and children with sagittal craniosynostosis and unaffected control subjects were prospectively evaluated with spectral-domain optical coherence tomography (OCT) from 2014-2021. Elevated ICP was determined based on previously validated algorithms utilizing retinal OCT parameters. RESULTS: Seventy-two patients with isolated sagittal craniosynostosis, and 25 control subjects were evaluated. Overall, 31.9% (n=23) of patients with sagittal craniosynostosis had evidence of ICP ≥15 mmHg, and 27.8% (n=20) of patients had ICP ≥20 mmHg.Children with sagittal craniosynostosis younger than 6 months of age were more likely to have normal intracranial pressure (88.6% <15 mmHg, 91.4% <20 mmHg) than those between 6-12 months of age (54.5%, p=.013; 54.5%, p=.005) and than those older than 12 months of age (46.2%, p<.001; 53.8%, p=.001). Intracranial pressure was directly correlated with severity of scaphocephaly (p=.009). No unaffected control subjects at any age exhibited retinal thickening suggestive of elevated ICP. CONCLUSIONS: Elevated ICP is rare in isolated sagittal craniosynostosis below 6 months of age, but becomes significantly more common after 6 months of age, and may correlate with severity of scaphocephaly.

Birk-Barel Intellectual Disability Dimorphism and KCNK9 Imprinting Syndrome: Craniofacial Surgery Considerations for an Exceedingly Rare Syndrome.

Birk-Barel intellectual disability dimorphism syndrome, also referred to as KCNK9 imprinting syndrome, is an exceedingly rare condition described in under 20 cases that presents with intellectual disability, hypotonia, scoliosis, dysphonia, dysphagia, and craniofacial dysmorphic features. The condition follows an autosomal dominant pattern of inheritance in the maternally expressed KCNK9 gene on chromosome 8. Due to the complexity of presentation, patients with Birk-Barel syndrome are optimally managed by a multidisciplinary team including a craniofacial surgeon. Previously described craniofacial dysmorphic features include micrognathia, cleft palate, dolichocephaly, broad nasal tip, and broad philtrum, among others. Here the authors describe a genetically confirmed case that has been managed in our institution's multidisciplinary cleft and craniofacial clinic. The authors aim to discuss Birk-Barel syndrome for a surgical and craniofacial audience with considerations for operative management in the context of a multidisciplinary team.

The Impact of Senior Author Profile on Publication Level of Evidence in Plastic and Reconstructive Surgery.

Plastic and Reconstructive Surgery (PRS) incorporated the level of evidence (LOE) pyramid in 2011 to highlight evidence-based medicine in plastic surgery. This study aimed to assess the relationship between the profile of senior authors publishing in PRS and the LOE of publications. Methods: All accepted publications by American senior authors to PRS between January 2018 and March 2020 were classified by LOE. Demographic, educational, and career data on all senior authors were collected using publicly available online resources. Results: A total of 1030 articles were screened' of which 266 (25.8%) were eligible for LOE classification with the following distribution: I, 0.8%; II, 14.3%; III, 45.1%; IV, 33.1%; and V, 7.1%. Senior author demographic factors, including gender and race/ethnicity, did not significantly impact LOE (P > 0.05 for all). An advanced degree was associated with more publications per year, and only a PhD (P = 0.022) and board certification (P = 0.012) were associated with a higher LOE of publications (P = 0.022). Physicians working in an academic setting were significantly more likely to publish level III evidence than their private/community-based counterparts (P = 0.006). Breast papers constituted the highest proportion of level III evidence (41.4%; P < 0.001). Conclusions: Most publications in PRS contain level III-IV evidence. Senior author gender, race, and ethnicity did not impact LOE of publications. Plastic surgery board certification, possession of a PhD, and academic practice setting were associated with higher LOE of publications.

Degree of Sagittal Suture Fusion, Cephalic Index, and Head Shape in Nonsyndromic Sagittal Craniosynostosis.

BACKGROUND: Sagittal craniosynostosis may present with complete or partial fusion of the sagittal suture, but relationships between degree of sagittal suture fusion and head shape are currently poorly described. The aim of this study was to characterize sagittal suture fusion patterns and determine associations with head shape in a cohort of patients with nonsyndromic sagittal craniosynostosis. METHODS: Patients with nonsyndromic sagittal craniosynostosis at a tertiary care center with available computed tomography imaging were included in this study. The anterior and posterior distances of sagittal suture patency were measured along 3-dimensional parietal bones. Degree of sagittal suture fusion was compared to head shape characteristics, including cephalic index (CI), frontal bossing, and occipital bulleting. RESULTS: Ninety patients (69 male) were included in this retrospective study. The sagittal suture was on average 85.6±20.1% fused, and 45 (50.0%) patients demonstrated complete fusion of the sagittal suture. CI was associated with increased degree of fusion for the anterior one-half (ρ=0.26, P =0.033) and anterior one-third (ρ=0.30, P =0.012) of the sagittal suture. Complete fusion of the anterior one-third of the sagittal suture predicted higher CI (ß=13.86, SE=6.99, z =-0.25, P =0.047). Total degree of sagittal suture fusion was not predictive of CI or head shape in any analysis. CONCLUSIONS: Decreased fusion of the anterior one-third of the sagittal suture, but not total suture, may paradoxically predict increased severity of scaphocephaly as quantified by CI in nonsyndromic sagittal craniosynostosis.

Cadaveric Bone Allografting for Severe Mandibular Hypoplasia With Alleviation of Upper Airway Obstruction in Goldenhar Syndrome.

ABSTRACT: Severe congenital mandibular hypoplasia may cause significant upper airway obstruction, often necessitating immediate tracheostomy. Recent surgical advances have demonstrated early intervention with mandibular distraction osteogenesis over tracheostomy may yield desirable aesthetic and functional outcomes. Here we present a case of bilateral mandibular hypoplasia, severe on the right and mild on the left, secondary to Goldenhar syndrome yielding significant tongue-based upper airway obstruction that was surgically corrected with mandibular bone allograft reconstruction of the right condyle, ramus, and posterior half of the mandibular body. Postoperative polysomnography revealed significantly improved obstructive apnea and computed tomography demonstrated adequate placement of the bone allograft to overcorrect the affected hemi-mandible. To the authors' knowledge, this is the first report of immediate mandibular allografting alleviating tongue-based upper airway obstruction secondary to mandibular hypoplasia in an infant. Mandibular bone allografting may be a favorable alternative to immediate tracheostomy with delayed surgical intervention, though long-term follow up is needed to assess graft durability and maintenance of airway patency.