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Variables interacting to predict outcomes following spring-mediated cranioplasty (SMC) for non-syndromic craniosynostosis, including spring parameters and calvarial thickness, are poorly understood. This study assessed interactions between spring parameters and calvarial thickness to predict changes in cephalic index (CI) following SMC.Patients undergoing SMC for non-syndromic sagittal craniosynostosis at our institution between 2014 and 2021 were included. Calvarial thickness was determined from patient preoperative CTs using Materalise Mimics at 27 points in relation to the sagittal suture. Linear mixed effects models were used to determine interactions between anterior, middle, and posterior calvarial thickness with spring force and length.Sixty-nine patients undergoing surgery at mean age 3.7 months were included in this study. Stronger posterior spring force interacted with thinner posterior calvarial thickness to predict greater changes in CI at 3 months postoperatively (p = 0.022). When evaluating spring force and calvarial thickness set distances from the sagittal suture, stronger posterior spring force interacted with thinner posterior calvarial thickness 5 mm (p = 0.043) and 10 mm (p = 0.036) from the sagittal suture to predict changes in CI. Interactions between spring parameters and calvarial thickness in the anterior and middle positions did not significantly predict changes in CI.Stronger posterior spring force interacted with thinner posterior calvaria to predict greater changes in CI 3 months following SMC for non-syndromic sagittal craniosynostosis. These results suggest dynamic interactions between several variables may impact outcomes following SMC.

This study assesses the diagnostic reliability of a novel photogrammetric measurement to distinguish sagittal craniosynostosis (SS) from control and false positive cases (SNS).Head CTs from 2014-2020 were reviewed for patients with sagittal synostosis (SS, n = 177), presumed sagittal synostosis with normal imaging (SNS, n = 30), and controls (n = 100). Using preoperative clinical photographs and CTs, a measurement reflecting the anterior-posterior location of the vertex was measured using an angle drawn between the cranial vertex, nasion, and opisthocranion (VNO) in profile view, with the head in a neutral position.Mean age at pre-operative head CT was 9.5 months for the SS cohort, 4.2 months for the SNS cohort, and 8.9 months for controls (p = .327). Mean age at pre-operative clinical photograph was 9.5 months for the SS cohort and 4.2 months for the SNS cohort (p = .149). Pearson correlations revealed no significant association between age and VNO angle. The average VNO angle measured on clinical photographs was 54.7° ± 3.8° for the SS group, 43.1° ± 2.2° for the SNS group, and 41.1° ± 3.7° for controls (p .001). Receiver operating characteristic (ROC) analysis yielded a cut-off of ≥ 50° to identify SS. Diagnostic sensitivity and specificity were 96.6% and 99.2%, respectively. Three-rater analysis yielded an average ICC of 0.742 (p = .004).Measurement of the VNO angle is a reliable screening tool to diagnose sagittal craniosynostosis, with an angle of 50° or more suggesting suture synostosis. This method relies on the relationship between the anterior displacement of the vertex and occipital bulleting to approach the diagnostic accuracy of CT imaging.

Cranial vault remodeling (CVR) in patients with craniosynostosis serves to correct abnormal skull morphology and increase intracranial volume to prevent or treat pathologic increases in intracranial pressure (Taylor and Bartlett, Plast Reconstr Surg 140: 82e-93e, 2017). Distraction osteogenesis is a well-established technique for bony repositioning and growth stimulation in the facial and long bones, in which the gradual separation of bony segments at an osteotomy site results in generation of new bone and subsequent bone lengthening (Greene, 2018). While initially described in the orthopedic literature, the relevance and applicability of distraction osteogenesis to craniofacial surgery has been well-studied and is now well-established (Steinbacher et al., Plast Reconstr Surg 127: 792-801, 2011). Posterior cranial vault distraction osteogenesis (PVDO) was introduced as a treatment option for cranial vault expansion in patients with craniosynostosis in 2009 by White et al., based upon the premise that posterior vault distraction could provide greater intracranial volume expansion than fronto-orbital advancement and remodeling (FOAR), but that acute posterior cranial vault expansions were limited by the soft tissue envelope of the infant scalp and prone to relapse related to the supine positioning typical of infants (White et al., Childs Nerv Syst 25: 231-236, 2009). Since this introduction, significant evidence has accrued regarding the safety of, and outcomes after, PVDO. PVDO is now known to provide larger increases in intracranial volume in comparison to anterior cranial vault remodeling procedures (Derderian et al., Plast Reconstr Surg 135:1665-1672, 2015) and to provide morphologic improvements in both the posterior and anterior cranial vaults (Goldstein et al., Plast Reconstr Surg 131:1367-1375, 2013). Perioperative major morbidity is comparable to conventional vault remodeling (Taylor et al., Plast Reconstr Surg 129:674e-680e, 2012) and the procedure has been safely applied to patients of various ages with syndromic and non-syndromic craniosynostosis (Zhang et al., J Craniofac Surg 29:566-571, 2018; Li et al., J Craniofac Surg 27:1165-1169, 2016). Many high-volume craniofacial centers now consider PVDO the preferred first operation in infants with syndromic craniosynostosis, and indications for this procedure continue to expand as evidence accrues regarding its utility and safety (Steinbacher et al., Plast Reconstr Surg 127: 792-801, 2011; Swanson et al., Plast Reconstr Surg 137:829e-841e, 2016).

Orbital hypertelorism (OHT) represents a congenital condition defined by lateralization of the bony orbit, unlike soft tissue telecanthus in which there is an increase in intercanthal distance without true bony lateralization. Existing literature remains very limited in its postoperative assessment of bony versus soft tissue relapse, which may both clinically present as telecanthus. We performed a critical appraisal of the literature to determine the postoperative incidence of bony versus soft tissue relapse following OHT repair.The PubMed, MEDLINE, EMBASE, Scopus, Cochrane Central Register of Controlled Trials, and clinicaltrials.org were searched systematically for all English studies published in any time frame reporting relapse rates following primary OHT repair. The primary outcome was incidence of bony and soft tissue relapse defined as orbital lateralization and medial canthal drift, respectively. The secondary outcome measures include postoperative complications, predictors of postoperative complications, timing and type of surgery, and revision rates.Eleven articles were included. A total of 84 (35.3%) patients experienced bony relapse while 43 (27.2%) patients experienced soft tissue relapse. Age at time of intervention (p0.92), severity at presentation (p0.90), and surgical technique (p0.09) were not found be significantly associated with relapse rate. Methods for long-term follow-up were not standardized, and there was no consistent measure to objectively assess telecanthus.There is no general consensus on predictive factors of long-term relapse following OHT repair in the form of box osteotomy or facial bipartition. These findings call for cross-sectional outcome standardization to better understand long-term outcomes across institutional, provider, and patient differences.

The primary objective of this study is to investigate post-operative morbidity and shunt revision rates of patients with shunt-dependent hydrocephalus (SDH) undergoing posterior vault distraction osteogenesis (PVDO) compared to patients undergoing conventional posterior vault reconstruction (PVR). A retrospective case-controlled cohort analysis of all patients with SDH undergoing PVDO and PVR for syndromic or complex craniosynostosis was performed. Demographic information, perioperative variables, distraction protocols, and shunt-related complications—infection, surgical revision of shunt, increased length of stay (LOS), and readmission within 90 days of surgery—were compared using the appropriate statistical tests. Fourteen patients with ventricular shunts who underwent PVDO and eight patients with shunts who underwent PVR were identified. Shunt-related complication rates were significantly higher with PVDO (n = 5) compared to PVR (n = 0), p = 0.0093. Among the five patients who suffered complications, the most common were shunt infection (n = 4), shunt malfunction (n = 4), and wound infections (n = 3). All patients with complications required additional operations for shunt revision and/or replacement; four patients required multiple takebacks for such procedures, with an average of three additional procedures per patient. In complex or syndromic craniosynostosis patients who have previously undergone ventricular shunting, PVDO is associated with higher shunt-related complications and need for additional procedures when compared to traditional PVR. While the benefits of PVDO in the treatment of syndromic craniosynostosis are well documented, the risks of PVDO in the face of a VP shunt must be considered. Further investigation into patient-specific risk factors and risk reduction strategies is warranted.

Better understanding the incidence and patterns of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to alter neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retina can non-invasively diagnose elevated ICP, and has demonstrated high sensitivity and specificity among patients with craniosynostosis. This study sought to characterize patterns of elevated ICP among patients with craniosynostosis. Quantitative retinal parameters were prospectively assessed in both eyes of patients with craniosynostosis using spectral-domain OCT. Based on retinal OCT thresholds associated with elevated ICP (> 15 mmHg), subjects were assigned an OCT diagnosis of elevated or non-elevated ICP which was analyzed relative to clinical characteristics and craniosynostosis patterns. Eighty subjects (aged 0.2–18 years) with craniosynostosis were enrolled; among these, 67 (84%) were nonsyndromic. OCT evaluation was performed at initial vault expansion in 56 (70%) patients. Among this subset, 27 (48%) patients had peri-papillary changes suggestive of elevated ICP, reflecting a 44% incidence in nonsyndromic and 83% in syndromic patients. The median age at initial vault expansion was higher among those with elevated ICP (11.1 months) than those without (7.8 months; p = 0.04.) Multi-suture synostosis was associated with changes consistent with elevated ICP in 9 (75%) patients compared with 18 (41%) with single suture synostosis (p = 0.05). OCT of the retina produces a potentially sensitive indicator of ICP in craniosynostosis patients. Elevated ICP may be associated with number of involved sutures and older patient presentation, and refining appropriate “cutoffs” will be important as the technology becomes more widespread.

There is a paucity of literature on how limitations of distraction osteogenesis (DO) are perceived by physicians and parents of pediatric patients. Specifically understanding which features of DO are most concerning to these two groups may better inform parent education, as well as direct improvements in distraction protocols and devices. Parents/guardians of patients (between January 2016 and October 2017) being treated with craniofacial distraction were recruited to complete a survey regarding level of stress (1 = not stressful, 9 = maximally stressful) associated with eight features of DO. Craniofacial surgeons completed a survey asking them to report (1) their personal level of stress and (2) their perceptions of parental stress regarding these same eight features of DO. Thirty-five parents and 15 craniofacial surgeons completed the survey. The risk of the device getting infected was perceived as most stressful by parents (5.5 ± 2.3) followed by the device sticking through the skin (4.9 ± 2.6) and the second operation for removal (4.7 ± 2.3). These same three features also elicited the highest level of stress among surgeons. Surgeon-perceived parental stress regarding turning of the distractor (5.8 ± 1.5) was significantly higher than parent self-reported stress (4.2 ± 2.8, p = 0.042). Both parents and surgeons perceive risk of device-associated infection, the protrusion of the device through the skin, and the requirement of a second operation for removal as the most stressful drawbacks of distraction. Infection reduction protocols, less obtrusive devices, and devices that do not require removal are potential targets for stress reduction.