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OBJECTIVE The diagnosis of single-suture craniosynostosis can be made by physical examination, but the use of confirmatory imaging is common practice. The authors sought to investigate preoperative imaging use and to describe intracranial findings in children with single-suture synostosis from a large, prospective multicenter cohort. METHODS In this study from the Synostosis Research Group, the study population included children with clinically diagnosed single-suture synostosis between March 1, 2017, and October 31, 2020, at 5 institutions. The primary analysis correlated the clinical diagnosis and imaging diagnosis; secondary outcomes included intracranial findings by pathological suture type. RESULTS A total of 403 children (67% male) were identified with single-suture synostosis. Sagittal (n = 267), metopic (n = 77), coronal (n = 52), and lambdoid (n = 7) synostoses were reported; the most common presentation was abnormal head shape (97%), followed by a palpable or visible ridge (37%). Preoperative cranial imaging was performed in 90% of children; findings on 97% of these imaging studies matched the initial clinical diagnosis. Thirty-one additional fused sutures were identified in 18 children (5%) that differed from the clinical diagnosis. The most commonly used imaging modality by far was CT (n = 360), followed by radiography (n = 9) and MRI (n = 7). Most preoperative imaging was ordered as part of a protocolized pathway (67%); some images were obtained as a result of a nondiagnostic clinical examination (5.2%). Of the 360 patients who had CT imaging, 150 underwent total cranial vault surgery and 210 underwent strip craniectomy. The imaging findings influenced the surgical treatment 0.95% of the time. Among the 24% of children with additional (nonsynostosis) abnormal findings on CT, only 3.5% required further monitoring. CONCLUSIONS The authors found that a clinical diagnosis of single-suture craniosynostosis and the findings on CT were the same with rare exceptions. CT imaging very rarely altered the surgical treatment of children with single-suture synostosis.

Candidates for endoscopic treatment for craniosynostosis must be less than 6 months old. Given the narrow window of eligibility, there is potential for barriers to access to impact the type of surgery a patient receives. We hypothesized that COVID may worsen these potential disparities. Charts were reviewed for children with single suture craniosynostosis from January 2014 to March 2020 (pre-COVID cohort) and March 2020 to September 2021 (COVID cohort) from 2 academic centers. 110 children were in the pre-COVID group; 56 were treated open and 54 endoscopically. Of the 44 patients in the COVID cohort, 20 were treated endoscopically and 24 were treated open. There was no significant difference in age of presentation or age of surgery across cohorts when considering type of surgery performed. Rate of endoscopy in the pre-COVID versus COVID cohorts was not significantly different. In both the pre-COVID and COVID cohorts there were significant differences between age of presentation, age of surgery, and type of surgery received based on race—black and Hispanic patients presented later, had surgery later, and underwent open surgery more frequently than their white/Asian counterparts ( P = .0095, P = .0067). The pandemic did not alter age of presentation/age of surgery or rates of patients receiving endoscopic surgery in patients with single suture craniosynostosis. There was no relationship between insurance status and type of surgery received during the pandemic, however Hispanic and black patients presented later and underwent surgery at an older age compared to white/Asian patients as they did pre-pandemic.

Objective: Multidisciplinary clinics are becoming widely utilized. Given the number of patients with craniofacial syndromes evaluated at our institution, and the burden of assessment by multiple subspecialists, we created an American Cleft Palate-Craniofacial Association–certified Craniofacial Multidisciplinary Clinic (CMC) composed of a nurse practitioner, neurosurgeon, plastic surgeon, otolaryngologist, oromaxillofacial surgeon, geneticist, pulmonologist, occupational therapist, dentist, and child life specialist to improve patient experience, lessen the burden of assessment, decrease time to surgery, and improve patients’ understanding of the diagnosis and treatment plan specifically for patients with complex craniofacial syndromes. We reviewed the impact of this clinic after 1 year of implementation. Design: Retrospective review was performed to identify patients with craniofacial syndromic diagnoses seen by the neurosurgery department before and after implementation of the CMC from February 2017 to present. Setting: The CMC is an outpatient clinic based in a tertiary care academic institution. Patients: Chart review was performed to identify demographic, diagnostic, clinical, and treatment data. We assessed clinic experience, and the impact on quality of clinical and surgical care was assessed via survey. We compared this cohort to patients with similar craniofacial syndromes treated prior to the CMC. Thirty patients seen at the CMC were identified, and data from a comparable cohort of 30 patients seen prior to the clinic’s inception was reviewed. Results: Our CMC survey response rate was 67% (n = 20/30) for the CMC patients. Second opinions sought by parents prior to CMC was higher (mean = 0.85, range: 0-3) than for patients seen at the CMC (mean = 0.16, range: 0-1). Mean time to surgery before the CMC was 10.1 months (range: 1-15) compared to 4 months (range: 3-5) after implementation. Parents agreed that they felt well-informed about their diagnosis (n = 18/20, 90%), and that the presence of a plastic surgeon (19/20, 95%) and a nurse practitioner (17/20, 85%) were valuable in coordination of their care. Following surgery, 76% (n = 13/17) of patients who received surgery were happy with the outcome, 76% (n = 13/17) were happy with the appearance of the scar, and 95% (n = 19/20) would recommend the CMC to others. Conclusion: Multidisciplinary evaluation of patients with complex craniofacial conditions provides comprehensive, efficient, and effective care, as well as improved parent satisfaction and knowledge base.

Patients with syndromic craniosynostosis (CS) can present with both intracranial and extracranial manifestations. Extracranial features include proptosis, exorbitism, and midface hypoplasia. Intracranial manifestations can include elevated intracranial pressure (ICP), brainstem compression, foramen magnum stenosis or jugular foramen hypoplasia with resultant venous hypertension and anomalous drainage. While fronto-orbital advancement, cranial vault remodeling, and posterior fossa decompression are standard surgical approaches to normalizing orbito-cranial volume and morphology, associated hydrocephalus, anomalous venous drainage, and tonsillar herniation often affect the timing, safety, and selection of corrective interventions. The surgical decision-making to circumvent venous emissaries, effectively time treatment of hydrocephalus, and address posterior versus anterior pathology primarily has not been widely described in the literature, and is important in the development of guidelines in these complex cases. In this report, we describe the surgical management of a patient with Jackson-Weiss syndrome presenting with delayed, but rapidly progressive bilateral lambdoid CS, severe proptosis, midface hypoplasia, elevated ICP, hydrocephalus, tonsillar ectopia, and severe venous hypertension with anomalous drainage. We review the literature related to management of complex synostosis and present our surgical decision-making in the setting of complex syndromic synostosis to aid in the formation of guidelines toward approaching these cases.

Craniosynostosis is a group of disorders of premature calvarial sutural fusion. An incomplete understanding of the calvarial stem cells (CSCs) that produce fusion-driving osteoblasts has limited the development of non-surgical therapeutic approaches for craniosynostosis. Here we show that both physiologic calvarial mineralization and pathologic calvarial fusion in craniosynostosis reflect the interaction of two separate stem cell lineages; a recently reported CathepsinK (CTSK) lineage CSC (CTSK+ CSC)1 and a separate Discoidin domain-containing receptor 2 (DDR2) lineage stem cell (DDR2+ CSC) identified in this study. Deletion of Twist1, a gene associated with human craniosynostosis2,3, solely in CTSK+ CSCs is sufficient to drive craniosynostosis, however the sites destined to fuse surprisingly display a marked depletion of CTSK+ CSCs and a corresponding expansion of DDR2+ CSCs. This DDR2+ CSC expansion is a direct maladaptive response to CTSK+ CSC depletion, as partial suture fusion occurred after genetic ablation of CTSK+ CSCs. This DDR2+ CSC is a specific fraction of DDR2+ lineage cells that displayed full stemness features, establishing the presence of two distinct stem cell lineages in the sutures, with each population contributing to physiologic calvarial mineralization. DDR2+ CSCs mediate a distinct form of endochondral ossification where an initial cartilage template is formed but the recruitment of hematopoietic marrow is absent. Direct implantation of DDR2+ CSCs into suture sites was sufficient to induce fusion, and this phenotype was prevented by co-transplantation of CTSK+ CSCs. Lastly, the human counterparts of DDR2+ CSCs and CTSK+ CSCs are present in calvarial surgical specimens and display conserved functional properties in xenograft assays. The interaction between these two stem cell populations provides a new biologic interface to modulate calvarial mineralization and suture patency.

Endoscopic suturectomy is a minimally invasive surgical treatment for single-suture craniosynostosis in children between 1 and 4 months of age. This study sought to characterize the role played by diagnostic imaging in facilitating early surgical management with endoscopic suturectomy. The authors also characterized the overall diagnostic utility of imaging in patients assessed for abnormal head shape at their institution, regardless of surgical status. A retrospective cohort of children diagnosed with singlesuture synostosis undergoing either primary endoscopic suturectomy or open calvarial reconstruction at the authors' institution from 1998 to 2018 was first reviewed. Of 132 surgical patients, 53 underwent endoscopic suturectomy and 79 underwent open repair. There was no difference in the proportion of endoscopic and open surgery patients imaged preoperatively before (24.5% versus 35.4%; P = 0.24) or after (28.3% versus 25.3%; P = 0.84) craniofacial assessment. Stratifying by historical epoch (1998-2010 versus 2011-2018), there was also no difference found between preoperative imaging rates (63.6% versus 56.4%; P = 0.35). In another cohort of 175 patients assessed for abnormal head shape, 26.9% were imaged to rule out craniosynostosis. Positive diagnostic imaging rates were recorded for suspected unicoronal (100%), metopic (87.5%), lambdoidal (75.0%), sagittal (63.5%), multisuture (50%), and bicoronal (0%) synostosis. The authors conclude that the use of diagnostic imaging at their institution has not increased despite higher utilization of endoscopic suturectomy and need for expedient identification of surgical candidates.However, their results suggest that imaging may play a greater diagnostic role for suspected bicoronal, sagittal, and multi-sutural synostosis among sutural subtypes of synostosis.

Little is currently known about the mechanisms by which pathogenic variants of FGFR2 produce changes in the FGFR protein and influence the clinical presentation of affected individuals. We report on a patient with a de novo pathogenic variant of FGFR2 and a phenotype consistent with Jackson-Weiss syndrome who presented with delayed, rapidly progressive multisutural craniosynostosis and associated medical complications. Using 3-dimensional modeling of the FGFR protein, we provide evidence that this variant resulted in abnormal dimerization and constitutive activation of FGFR, leading to the Jackson-Weiss phenotype. Knowledge regarding the correlation between genotype and phenotype of persons with FGFR2-related craniosynostosis has the potential to allow for anticipation of medical complications, institution of early treatment, and improved clinical outcomes.

OBJECTIVEThe authors created a collaborative network, the Synostosis Research Group (SynRG), to facilitate multicenter clinical research on craniosynostosis. To identify common and differing practice patterns within the network, they assessed the SynRG surgeons’ management preferences for sagittal synostosis. These results will be incorporated into planning cooperative studies.METHODSThe SynRG consists of 12 surgeons at 5 clinical sites. An email survey was distributed to SynRG surgeons in late 2016, and responses were collected through early 2017. Responses were collated and analyzed descriptively.RESULTSAll of the surgeons—7 plastic/craniofacial surgeons and 5 neurosurgeons—completed the survey. They varied in both experience (1–24 years) and sagittal synostosis case volume in the preceding year (5–45 cases). Three sites routinely perform preoperative CT scans. The preferred surgical technique for children younger than 3 months is strip craniectomy (10/12 surgeons), whereas children older than 6 months are all treated with open cranial vault surgery. Pre-incision cefazolin, preoperative complete blood count panels, and an arterial line were used by most surgeons, but tranexamic acid was used routinely at 3 sites and never at the other 2 sites. Among surgeons performing endoscopic strip craniectomy surgery (SCS), most create a 5-cm-wide craniectomy, whereas 2 surgeons create a 2-cm strip. Four surgeons routinely send endoscopic SCS patients to the intensive care unit after surgery. Two of the 5 sites routinely obtain a CT scan within the 1st year after surgery.CONCLUSIONSThe SynRG surgeons vary substantially in the use of imaging, the choice of surgical procedure and technique, and follow-up. A collaborative network will provide the opportunity to study different practice patterns, reduce variation, and contribute multicenter data on the management of children with craniosynostosis.

Objective The goal of endoscopic treatment for craniosynostosis is to remove the fused suture and achieve calvarial remodeling with external orthosis. To reduce the need for secondary surgery and to minimize blood loss, instruments that maximize bone removal while minimizing blood loss and risk of dural injury are evolving. The authors therefore assess the safety and efficacy of the Sonopet Ultrasonic Bone Aspirator (UBA) (Stryker, Kalamazoo, MI) for endoscopic suturectomy compared to traditional instrumentation at our institution. Methods Retrospective chart review of consecutive endoscopic suturectomies performed from 2011 to 2019 at Weill Cornell Medical Center was conducted, including demographics, cephalic index, surgical indications, operative time, cosmetic and functional results, complications, estimated blood loss (EBL), re-operation rate, length of stay, and length of helmet therapy. These variables were then compared between the Sonopet and non-Sonopet cohorts. Results Of the 60 patients who underwent endoscopic suturectomy, 16 cases (26.7%) utilized the Sonopet. Mean operative time was 2.8 ± 0.4 hours in the Sonopet group, compared to 3.2 ± 1.2 hours (P = 0.05) without the Sonopet. EBL was 17.8 ± 23.9 cc versus 34.7 ± 75.5 cc (P = 0.20) with versus without the Sonopet respectively. Length of stay and duration of helmet therapy were similar in both groups, ranging from 1 to 3 days (P = 0.68) and 7.25 to 12 months (P = 0.30) respectively. There were no reoperations in the Sonopet group with a mean follow up of 9.18 months. There were 3 reoperations in the non-Sonopet group with a mean follow up of 11.3 months. Among the cases utilizing the Sonopet, 13 (81%) were metopic and three (19%) were coronal synostoses. Of the non-Sonopet cases, 27 (61%) were sagittal, 8 (18%) were metopic, 7 (16%) were coronal, and 2 (5%) were lambdoid synostoses. Conclusions The use of the Sonopet resulted in a mean decrease in operative time at our institution (P = 0.18). Lower EBL and reoperation rates with comparable LOS and helmet therapy duration were also seen. This modality should be considered a safe and effective adjunct in appropriate endoscopic craniosynostosis cases.

OBJECTIVE Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research. METHODS All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables. RESULTS Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group. CONCLUSIONS The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.

Introduction Endoscope-assisted craniectomy and spring-assisted cranioplasty with post-surgical helmet molding are minimally invasive alternatives to the traditional craniosynostosis treatment of open cranial vault remodeling. Families are often faced with deciding between techniques. This study aimed to understand providers' practice patterns in consulting families about surgical options. Methods An online survey was developed and distributed to 31 providers. The response rate was 84% (26/31). Results Twenty-six (100%) respondents offer a minimally invasive surgical option for sagittal craniosynostosis, 21 (81%) for coronal, 20 (77%) for metopic, 18 (69%) for lambdoid, and 12 (46%) for multi-suture. Social issues considered in determining whether to offer a minimally invasive option include anticipated likelihood of compliance (23 = 88%), distance traveled for care (16 = 62%) and financial considerations (6 = 23%). Common tools to explain options include verbal discussion (25 = 96%), 3D reconstructed CT scans (17 = 65%), handouts (13 = 50%), 3D models (12 = 46%), hand drawings (11 = 42%) and slides (10 = 38%). Some respondents strongly (7 = 27%) or somewhat (3 = 12%) encourage a minimally invasive option over open repair. Others indicate they remain neutral (7 = 27%) or tailor their approach to meet perceived needs (8 = 31%). One (4%) somewhat encourages open repair. Despite this variation, all completely (17 = 65%), strongly (5 = 19%) or somewhat agree (4 = 15%) they use shared decision making in presenting surgical options. Conclusion This survey highlights the range of practice patterns in presenting surgical options to families and reveals possible discrepancies in the extent providers believe they use shared decision making and the extent it is actually used.

Patients with cleft lip and/or palate have higher rates of failure to thrive (FTT), decreased growth, and more often experience feeding difficulties as compared with the general pediatric population (J Child Health Care. 2014;18:72-83). Although insufficient nursing, excessive air intake, and incorrect feeding methods have been established in the literature, the role of gastroesophageal reflux disease (GERD) as a contributing factor in cleft patients has not been thoroughly examined. Presently, there is a paucity of literature analyzing the incidence and effect of GERD on this unique population. Furthermore, no studies have evaluated the effect of GERD therapy on improvement of weight gain and FTT in cleft patients. The purpose of this retrospective review was to identify the incidence of GERD in the orofacial cleft population and to see if appropriate treatment was effective in improving weight gain. Fifty patients with cleft lip, cleft lip and palate, and isolated cleft palate were identified from a single surgeon's experience at a large academic medical center from 2015 to 2019. The data show that a significantly higher percentage of patients with cleft lip/and or palate have clinical evidence of GERD, which required treatment as compared with published reports of less than 1% in the noncleft population. The data also suggest that the patients diagnosed with GERD who received pharmacologic treatment showed improved weight gain as compared with those who did not. Given our findings, the diagnosis of GERD should be considered in orofacial cleft patients exhibiting signs of feeding difficulty or those with FTT. The early diagnosis and treatment of GERD in patients with orofacial clefts may improve weight gain.

Objective Venous-arterial extracorporeal membrane oxygenation (VA-ECMO) is a well-established therapy for refractory cardiopulmonary failure. Femoral cannulation offers a quick and effective means of providing circulatory support but is not without complication. Inflammation or lymphatic disruption at the site of cannulation can cause the formation of lymphoceles, leading to the patient's discomfort and possibly necessitating intervention. The purpose of this study was to evaluate the incidence of in-hospital lymphocele formation in VA-ECMO patients and to identify predictors for their development. Methods We conducted a single-center retrospective review of 192 patients who underwent femoral VA-ECMO insertion and subsequent decannulation from March 2007 to August 2016 for cardiogenic shock. Baseline demographics, risk factors, and cannulation strategies were examined. Groin lymphocele formation was assessed as the primary outcome. Results Median age was 58 years (interquartile range, 48-67 years) with a median duration of support of 4 days (interquartile range, 2-6 days). Lymphocele formation was identified in 31 patients (16%). Patients who developed lymphoceles were more likely to have post-heart transplantation primary graft dysfunction (PGD) as an indication for ECMO support compared with those who did not (54.2% vs 8%; P P = .010). Compared with those PGD patients who did not develop lymphoceles, PGD lymphocele patients had higher rates of diabetes mellitus preoperatively (62% vs 8%; P = .006). Thirteen (42%) patients required surgical incision and drainage, and 4 of these patients (31%) required repeated surgical intervention. Conclusions Lymphocele formation is relatively common after femoral VA-ECMO. There was a significantly higher incidence of lymphocele formation in diabetic patients requiring support for PGD after heart transplantation.

Recent studies indicate that recombinant human bone morphogenetic protein-2 (rhBMP-2) in a demineralized bone matrix scaffold is a comparable alternative to iliac bone autograft in the setting of secondary alveolar cleft repair. Postreconstruction occlusal radiographs demonstrate improved bone stock when rhBMP-2/demineralized bone matrix (DBM) scaffold is used but lack the capacity to evaluate bone growth in three dimensions. This study uses cone beam computed tomography to provide the first clinical evaluation of volumetric and density comparisons between these two treatment modalities.A prospective study was conducted with 31 patients and 36 repairs of the alveolar cleft over a 2-year period. Twenty-one repairs used rhBMP-2/DBM scaffold and 14 repairs used iliac bone grafting. Postoperatively, occlusal radiographs were obtained at 3 months to evaluate bone fill; cone beam computed tomographic images were obtained at 6 to 9 months to compare volumetric and density data.At 3 months, postoperative occlusal radiographs demonstrated that 67 percent of patients receiving rhBMP-2/DBM scaffold had complete bone fill of the alveolus, versus 56 percent of patients in the autologous group. In contrast, cone beam computed tomographic data showed 31.6 percent (95 percent CI, 24.2 to 38.5 percent) fill in the rhBMP-2 group compared with 32.5 percent (95 percent CI, 22.1 to 42.9 percent) in the autologous population. Density analysis demonstrated identical average values between the groups (1.38 g/cc).These data demonstrate comparable bone regrowth and density values following secondary alveolar cleft repair using rhBMP-2/DBM scaffold versus autologous iliac bone graft. Cone beam computed tomography provides a more nuanced understanding of true bone regeneration within the alveolar cleft that may contribute to the information provided by occlusal radiographs alone.Therapeutic, II.

Smooth, round, silicone implants predominate device-based breast reconstruction in the USA; despite their prevalence, complications can include bottoming out, superior contour deformity, rippling, and/or lateral malposition. This complication profile increases the need for revision surgery and subsequent patient dissatisfaction. With the resurgence of shaped, textured, silicone implants in the USA, we report the senior author’s success with these devices and outline a strategy to optimize outcomes in breast reconstruction surgery. A retrospective chart review was conducted on a prospectively collected IRB-approved database of nipple-sparing mastectomies (NSMs) with immediate breast reconstruction with smooth, round, silicone implants (Group A) in 2011 in comparison to textured, shaped, silicone implants (Group B) in 2012. Changes in operative technique were highlighted and extrapolated. Outcomes were reviewed. In Group A, 128 NSMs were performed in 76 patients. In Group B, 109 NSMs were performed in 59 patients. Thirteen percent of patients in Group A had direct to implant reconstruction as compared with 21% in Group B. Patients with textured, shaped implants were more likely to have acellular dermal matrix (61 vs 34%, p

BACKGROUND There is no universally accepted classification system for unilateral cleft lip that objectively quantifies the spectrum of disease, making it difficult to evaluate postoperative outcomes in the context of preoperative severity. METHODS Anthropometric measurements and photographs were prospectively collected from unilateral cleft lip patients in Morocco, Bolivia, Vietnam, and Madagascar. Columellar angle, cleft width, nostril widths, vertical lip heights, and horizontal vermillion lengths were measured preoperatively and postoperatively. "Unacceptable" postoperative outcomes were defined as those with a cleft-side/non-cleft-side vertical lip height discrepancy greater than 3 mm, based on previous sociologic and cleft outcome studies. RESULTS Of the 147 patients studied, 22 had unacceptable outcomes. Univariate logistic and multivariate logistic stepwise models showed that among preoperative characteristics, cleft width ratio (preoperative cleft width divided by commissure width) was the most significant predictor for unacceptable outcomes, controlling for surgeon experience. Cleft width ratio was normally distributed. Two severity categories were created based on iterative data and regression analysis: "severe" (cleft width ratio >0.5) and "not-severe" (cleft width ratio

OBJECTIVECraniosynostosis correction, including cranial vault remodeling, fronto-orbital advancement (FOA), and endoscopic suturectomy, requires practical experience with complex anatomy and tools. The infrequent exposure to complex neurosurgical procedures such as these during residency limits extraoperative training. Lack of cadaveric teaching tools given the pediatric nature of synostosis compounds this challenge. The authors sought to create lifelike 3D printed models based on actual cases of craniosynostosis in infants and incorporate them into a practical course for endoscopic and open correction. The authors hypothesized that this training tool would increase extraoperative facility and familiarity with cranial vault reconstruction to better prepare surgeons for in vivo procedures.METHODSThe authors utilized representative craniosynostosis patient scans to create 3D printed models of the calvaria, soft tissues, and cranial contents. Two annual courses implementing these models were held, and surveys were completed by participants (n = 18, 5 attending physicians, 4 fellows, 9 residents) on the day of the course. These participants were surveyed during the course and 1 year later to assess the impact of this training tool. A comparable cohort of trainees who did not participate in the course (n = 11) was also surveyed at the time of the 1-year follow-up to assess their preparation and confidence with performing craniosynostosis surgeries.RESULTSAn iterative process using multiple materials and the various printing parameters was used to create representative models. Participants performed all major surgical steps, and we quantified the fidelity and utility of the model through surveys. All attendees reported that the model was a valuable training tool for open reconstruction (n = 18/18 [100%]) and endoscopic suturectomy (n = 17/18 [94%]). In the first year, 83% of course participants (n = 14/17) agreed or strongly agreed that the skin and bone materials were realistic and appropriately detailed; the second year, 100% (n = 16/16) agreed or strongly agreed that the skin material was realistic and appropriately detailed, and 88% (n = 14/16) agreed or strongly agreed that the bone material was realistic and appropriately detailed. All participants responded that they would use the models for their own personal training and the training of residents and fellows in their programs.CONCLUSIONSThe authors have developed realistic 3D printed models of craniosynostosis including soft tissues that allow for surgical practice simulation. The use of these models in surgical simulation provides a level of preparedness that exceeds what currently exists through traditional resident training experience. Employing practical modules using such models as part of a standardized resident curriculum is a logical evolution in neurosurgical education and training.

Intraosseous lipomas are almost exclusively seen in the long bones. Presence in the craniofacial skeleton is extremely rare. A 7-year-old male is presented with a marked craniofacial deformation from a bony tumor containing an intraosseous lipoma. This finding established a clinical diagnosis of Proteus syndrome. Given the size of the tumor, producing an extensive deformity, three-dimensional modeling was used to generate a three-dimensional printed implant. The process to achieve a successful outcome is herein described.

Background Some patients with isolated sagittal craniosynostosis have demonstrated mild neurodevelopmental delays. This study examined potential preoperative risk factors for developmental delay. Methods Patients completed preoperative Bayley Scales of Infant and Toddler Development, Third Edition, and medical records were reviewed. Multivariate analyses of covariance and correlations were calculated. Results Participants (n = 77) were predominantly male (77.9 percent) and were aged 2 to 12 months (mean, 5.1 ± 2.3 months). Patients were classified with no delays [n = 63 (82 percent)] or delays [n = 14 (18 percent)] in one or more developmental area(s). There were no group sociodemographic differences. Prenatally, patients with delays versus no delays had lower mean gestational age in weeks (36.9 ± 2.8 weeks versus 39.1 ± 1.7 weeks; p = 0.001) with higher rates of gestational diabetes (36 percent versus 5 percent; p = 0.006) and premature rupture of membranes (14 percent versus 2 percent; p = 0.026). At birth, patients with delays had lower mean birth weight (2982 ± 714 g versus 3374 ± 544 g; p = 0.053), higher rates of respiratory distress (29 percent versus 5 percent; p = 0.005), additional medical diagnoses (57 percent versus 13 percent; p = 0.001), and longer mean neonatal intensive care unit stays (1.4 ± 1.8 weeks versus 0.2 ± 0.9 week; p = 0.002). Variables differing by group had moderate correlations. Conclusions Patients with nonsyndromic sagittal craniosynostosis that had delays in development had lower gestational age and birth weight, with more prenatal and birth complications. These factors can help identify patients who might be at risk for delay and need close monitoring. Clinical question/level of evidence Risk, III.

Craniosynostosis is the premature fusion of the skull. There are two forms of treatment: open surgery and minimally invasive endoscope-assisted suturectomy. Candidates for endoscopic treatment are less than 6 months of age. The techniques are equally effective; however, endoscopic surgery is associated with less blood loss, minimal tissue disruption, shorter operative time, and shorter hospitalization. In this study, the authors aimed to evaluate the impact of race/ethnicity and insurance status on age of presentation/surgery in children with craniosynostosis to highlight potential disparities in healthcare access. Charts were reviewed for children with craniosynostosis at two tertiary care hospitals in New York City from January 1, 2014, to August 31, 2020. Clinical and demographic data were collected, including variables pertaining to family socioeconomic status, home address/zip code, insurance status (no insurance, Medicaid, or private), race/ethnicity, age and date of presentation for initial consultation, type of surgery performed, and details of hospitalization. Children with unknown race/ethnicity and those with syndromic craniosynostosis were excluded. The data were analyzed via t-tests and chi-square tests for statistical significance (p < 0.05). A total of 121 children were identified; 62 surgeries were performed open and 59 endoscopically. The mean age at initial presentation of the cohort was 6.68 months, and on the day of surgery it was 8.45 months. Age at presentation for the open surgery cohort compared with the endoscopic cohort achieved statistical significance at 11.33 months (SD 12.41) for the open cohort and 1.86 months (SD 1.1473) for the endoscopic cohort (p < 0.0001). Age on the day of surgery for the open cohort versus the endoscopic cohort demonstrated statistical significance at 14.19 months (SD 15.05) and 2.58 months (SD 1.030), respectively. A statistically significant difference between the two groups was noted with regard to insurance status (p = 0.0044); the open surgical group comprised more patients without insurance and with Medicaid compared with the endoscopic group. The racial composition of the two groups reached statistical significance when comparing proportions of White, Black, Hispanic, Asian, and other (p = 0.000815), with significantly more Black and Hispanic patients treated in the open surgical group. The results demonstrate a relationship between race and lack of insurance or Medicaid status, and type of surgery received; Black and Hispanic children and children with Medicaid were more likely to present later and undergo open surgery.

INTRODUCTION Oncoplastic breast reduction has been shown to be an effective approach to breast conservation surgery in women with macromastia. Clear surgical margins can be achieved while simultaneously improving symptomatic macromastia and enhancing aesthetic outcomes. Little has been written about postoperative complications after this procedure, beyond the risk of locoregional recurrence. This study aimed to compare the complication profile for oncoplastic breast reduction versus reduction for benign macromastia. METHODS A retrospective review of our experience with oncoplastic breast reduction was performed. This represented a consecutive series of 118 patients undergoing bilateral breast reduction during the 7-year study period from March 2005 to March 2012. There were 64 patients identified who underwent oncoplastic breast reduction. Patients were determined to be a good candidate for breast conservation therapy if it was felt that clear surgical margins could be obtained without mastectomy. Postoperative complications (within 6 weeks of surgery) were compared to a control group of 56 patients undergoing reduction for benign macromastia. The associations between complications and potential risk factors were analyzed using logistic regression. RESULTS Patients undergoing oncoplastic breast reduction and reduction for benign macromastia had some key differences. In general, macromastia patients were younger (mean age, 42.3 vs 57.5 years; P < 0.001) and had lower body mass index (mean, 26.1 vs 30.6 kg/m2; P < 0.001) compared to those patients having oncoplastic reduction. Within the oncoplastic reduction group, 14 (21.9%) patients had a total of 16 complications; among the benign macromastia group, 9 (16.1%) patients had a total of 10 complications (P = 0.420). On univariate analysis, oncoplastic reduction was not predictive of having a perioperative complication (odds ratio, 1.462; 95% confidence interval, 0.579-3.696; P = 0.422). Body mass index was found to be predictive of having a complication after reduction for either indication (odds ratio, 1.108; 95% confidence interval, 1.018-1.206; P = 0.017). Within the oncoplastic reduction cohort at an average follow-up of 34.6 months (range, 0.3-90.3 months), 5 (7.9%) patients developed locoregional recurrence and 2 patients developed distant metastasis. CONCLUSIONS Compared with reduction mammoplasty for benign macromastia, a widely accepted procedure, patients undergoing oncoplastic breast reduction were equally likely to have a postoperative complication. Elevated body mass index was shown to be a statistically significant predictor of having a complication after reduction for either indication. Overall complication rates were acceptably low for both procedures.

BACKGROUND Unilateral cleft lip has a spectrum of disease morphology, but severity classifications are difficult given the absence of accessible, objective assessment tools or reference data. The authors characterize the spectrum of cleft morphology before and after surgical repair for a large, multi-ethnic population using easily identifiable facial landmarks collected through a novel smart phone-based application. METHODS Anthropometric measurements and standardized photographs were prospectively collected in Morocco, Bolivia, Vietnam, and Madagascar during medical missions in 2015 using an application designed specifically for the study. After data collection, two experienced cleft surgeons and two laypersons subjectively ranked photographs based on the degree of deformity/aesthetics. RESULTS One hundred forty-seven patients were analyzed. Mean preoperative cleft width ratio was 0.4 ± 0.12. Nasolabial symmetry improved significantly from preoperatively to postoperatively for the following measurements: columellar angle (65 ± 17 degrees to 87 ± 8 degrees), nostril width ratio (1.7 ± 0.68 to 1.0 ± 0.22), philtral height ratio (0.8 ± 0.14 to 1.0 ± 0.14), and lip length ratio (0.9 ± 0.26 to 1.0 ± 0.11) (p < 0.001). Surgeon and layperson rankings showed high inter-rater reliability (r = 0.64, p < 0.001). Preoperatively, multivariate regression showed that cleft width ratio, nostril width ratio, and philtral height ratio were predictive of rank (p < 0.01). Postoperatively, philtral height ratio was most predictive of rank (p = 0.0097). Most cleft characteristics were not significantly different between countries. CONCLUSIONS The authors present simpler, more straightforward measures to quantify preoperative and postoperative morphology/aesthetics and introduce a novel technology to streamline and standardize measurements to make data collection more accessible.

Background Alveolar cleft reconstruction using iliac crest bone graft is considered standard of care for children with complete cleft lip and palate at the time of mixed dentition. Harvesting bone may result in donor-site morbidity and additional operating time and length of hospitalization. Recombinant human bone morphogenetic protein (rhBMP)-2 with a demineralized bone matrix is an alternative bone source for alveolar cleft reconstruction. The authors investigated the outcomes of rhBMP-2/demineralized bone matrix versus iliac crest bone graft for alveolar cleft reconstruction by reviewing postoperative surgical complications and cleft closure. Methods A retrospective chart review was conducted for 258 rhBMP-2/demineralized bone matrix procedures (mean follow-up, 2.9 years) and 243 iliac crest bone graft procedures (mean follow-up, 4.1 years) on 414 patients over a 12-year period. The authors compared complications, canine eruption, and alveolar cleft closure between the two groups. Results In the rhBMP-2/demineralized bone matrix group, one patient required prolonged intubation because of intraoperative airway swelling not thought to be caused by rhBMP-2, 36 reported facial swelling and one required outpatient steroids as treatment, and 12 had dehiscence; however, half of these complications resolved without intervention. Twenty-three of the 228 rhBMP-2/demineralized bone matrix patients and 28 of the 242 iliac crest bone graft patients required repeated surgery for alveolar cleft repair. Findings for canine tooth eruption into the cleft site through the graft were similar between the groups. Conclusions The rhBMP-2/demineralized bone matrix appears to be an acceptable alternative for alveolar cleft repair. The authors found no increase in serious adverse events with the use of this material. Local complications, such as swelling and minor wound dehiscence, predominantly improved without intervention. Clinical question/level of evidence Therapeutic, III.

Objective To compare the incidence of surgical site infection (SSI) and wound complications between standard and plastic multilayered closure (PMC) in patients with pediatric nonidiopathic scoliosis undergoing primary or revision instrumentation or fusion. Study Design Retrospective cohort study. Summary of Background Data Although PMC has been used for adult spine patients as well as infected spinal wound closure, it has not been reported as routine closure in the pediatric nonidiopathic scoliosis population. Methods Patients with nonidiopathic scoliosis undergoing primary or revision instrumentation or fusion between 2013 and 2016 were enrolled. Clinical charts and operative reports were reviewed. Following September 2015, all patients with nonidiopathic scoliosis received PMC closure by plastic or orthopedic surgery. The SSI and wound complication incidence in patients undergoing PMC was compared to standard closure. The mean Risk Severity Score (RSS) for SSI, which uses patient characteristics to calculate the excepted SSI probability, was compared to the observed (actual) incidence. The CDC definition of SSI (infection ≤90 days of surgery) was used. Results 115 patients were identified, including 56 standard closure and 59 PMC (mean age of 10.6 years; 54.8% female; mean preoperative major curve = 57.5°). There was no significant difference in age, sex, race, preoperative major curve, BMI, hemoglobin, blood loss, mean RSS score, or etiology between both groups (p > .05). Following PMC implementation, the observed incidence of wound complications decreased significantly from 19.6% in standard closures to 5.1% in the PMC group (p = .02), and the SSI incidence decreased from 8.9% to 1.7% (p = .11). The overall change in the observed (actual) incidence versus expected (RSS score) incidence of SSI indicated a 7.1% reduction following PMC. Conclusion Utilizing PMC in patients undergoing spinal surgery for nonidiopathic scoliosis significantly decreases the incidence of wound complications. Surgeons should consider using PMC in patients with nonidiopathic scoliosis. Level of Evidence Level II.

Background: The optimal timing for cleft lip repair has yet to be established. Advances in neonatal anesthesia, along with a growing body of literature, suggesting benefits of earlier cleft lip and nasal repair, have set the stage for a reexamination of current practices. Methods: In this prospective study, cleft lip and nasal repair occurred on average at 34.8 days (13–69 days). Nasal correction was achieved primarily through molding the nasal cartilage without the placement of nasal sutures at the time of repair. A standardized anesthetic protocol aimed at limiting neurotoxicity was utilized in all cases. Anesthetic and postoperative complications were assessed. A 3-dimensional nasal analysis compared pre- and postoperative nasal symmetry for unilateral clefts. Surveys assessed familial response to repair. Results: Thirty-two patients were included (27 unilateral and 5 bilateral clefts). In this study, the overall complication rate was 3.1%. Anthropometric measurements taken from 3-dimensional-image models showed statistically significant improvement in ratios of nostril height (preoperative mean, 0.59; postoperative mean, 0.80), nasal base width (preoperative mean, 1.96; postoperative mean, 1.12), columella length (preoperative mean, 0.62; postoperative mean, 0.89; and columella angle (preoperative mean, 30.73; postoperative mean, 9.1). Survey data indicated that families uniformly preferred earlier repair. Conclusions: We present evidence that early cleft lip and nasal repair can be performed safely and is effective at improving nasal symmetry without the placement of any nasal sutures. Utilization of this protocol has the potential to be a paradigm shift in the treatment of cleft lip and nasal deformity.

The objective of this study was to evaluate the antimicrobial performance of a rifampin/minocycline-coated, non-cross-linked, acellular porcine dermal matrix (XenMatrix AB) compared to an uncoated, non-cross-linked, acellular porcine dermal matrix (Strattice) after implantation/inoculation with methicillin-resistant Staphylococcus aureus or Escherichia coli in a dorsal rabbit model.Forty male New Zealand White rabbits were bilaterally implanted with XenMatrix AB or Strattice grafts and inoculated with clinically isolated methicillin-resistant S. aureus (5 × 10 colony-forming units/ml) or E. coli (1 × 10 colony-forming units/ml). At 2 and 8 weeks, sites were analyzed for viable methicillin-resistant S. aureus/E. coli colony-forming units, abscess formation, and histologic response (n = 5 rabbits per group per bacterium per time point).XenMatrix AB completely inhibited bacterial colonization of the graft, inhibited abscess formation, reduced inflammation and encapsulation, and improved neovascularization compared with Strattice. XenMatrix AB implants exhibited significantly fewer colony-forming units compared with Strattice implants at 2 weeks (methicillin-resistant S. aureus) (p0.01) and at 2 and 8 weeks (E. coli) (p0.05). In addition, XenMatrix AB implants demonstrated a significantly lower abscess score at 2 weeks (methicillin-resistant S. aureus) and 8 weeks (E. coli) (p0.01 in both cases). For both types of bacteria and both time points evaluated, XenMatrix AB implants exhibited minimal inflammation and encapsulation and a lack of neutrophils. In contrast, Strattice implants displayed marked inflammatory and neutrophilic responses and moderate encapsulation.This study demonstrated the antimicrobial performance of a rifampin/minocycline-coated bioprosthetic (XenMatrix AB) in a rabbit inoculation model. XenMatrix AB completely inhibited bacterial colonization of the graft, with minimal host inflammation and encapsulation, and improved neovascularization compared with Strattice.

Patients with nonsyndromic sagittal craniosynostosis (SC) were previously thought to have normal neurocognitive development; however, a pattern of mild delays has been described in these patients. We reviewed our patients with SC to identify potential perinatal risk factors that serve as indicators for subsequent developmental delay.Nonsyndromic patients with SC (n = 66) completed preoperative Bayley Scales of Infant and Toddler Development (III) with a single examiner between August 2009 and April 2015. Patients were classified as having no delays (n = 52; 79%) or having delays (n = 14; 21%) below the ninth percentile in one or more area(s) of development. Mean differences were compared using Multivariate Analyses of Variance.Participants were mostly male (79%) and aged 2 to 12 months at testing. There were no group differences in sociodemographic categories. Prenatally, patients in the group with delays vs the group with no delays had lower gestational age in weeks (36.9 vs 39.2, P

OBJECT The authors’ aim was perform a systematic review on the incidence of intracranial hypertension (IH) after surgery for craniosynostosis. METHODS A systematic literature review was conducted using PubMed to assess the rate of postoperative IH in studies published between 1985 and 2014. Inclusion criteria were 1) English-language literature; 2) human subjects; 3) pediatric cases; and 4) postoperative IH confirmed with invasive intracranial pressure monitoring. RESULTS Seven studies met inclusion criteria. IH was reported to be present in 5% of patients postoperatively with sagittal synostosis and 4% of patients with all forms of nonsyndromic craniosynostosis. Inadequate numbers were available to determine the incidence of postoperative IH for syndromic and individual nonsyndromic sutural synostosis based on the inclusion criteria. Surgical groups were subdivided into cranial remodeling procedures without orbital advancement and craniofacial procedures with orbital advancement. IH was reported to be present in 5% of patients with all forms of nonsyndromic sutural stenosis after cranial remodeling procedures and 1% after craniofacial advancement. CONCLUSIONS Postoperative development of elevated intracranial pressure has been described by multiple institutions, but the variation in how IH is determined and the multiple surgical procedures to correct craniosynostosis has limited the number of studies subject to a meta-analysis. Nonetheless, this entity deserves special attention, and further studies are required to determine the true incidence of postoperative IH, including the role of various surgical procedures on its incidence. The long-term consequences of chronic IH in this group of patients also need to be evaluated.

The stimulating article and proposed hypothesis by Costagliola et al. [1] represent another step forward in our attempts to understand and better classify the clinical entity known most commonly as tuberous breast deformity. Since the original description of this entity by Rees and Aston [2] in 1976, a diverse descriptive nomenclature has arisen within the literature [3]. Concomitant with this has been the proposal of numerous surgical approaches for correction of this deformity [3]. In this article, the authors present their evidence for an expansion of the Grolleau et al. [4] classification system initially described in 1999. In addition to Grolleau types 1, 2, and 3 breast, Costagliola et al. [1] newly describe the type 0 tuberous breast typified by a normal base volume but with herniation of breast tissue through the nipple-areolar complex (NAC). Although this is not the first time this minor form of tuberous breast has been suggested, in this article, the authors clearly expound on their hypothesis for the basic pathophysiology behind this entity [1, 5]. With the publication of this study, we currently have two major theories for the development of the tuberous breast deformity in the literature, namely, the constricted ring theory described by Mandrekas et al. [6] and the theory of weakened peri-NAC skin and fascia, as described in this article [1, 5]. Although these two theories may seem to be in apparent opposition, they both provide explanations for features of tuberous breast deformity commonly seen clinically. The constricted ring theory suggests that abnormal development of the superficial thoracic fascia, which invests the nascent mammary tissue, leads to restricted growth of the breast in one or more directions at the time of puberty. Histologic support for this theory, showing aberrant fibrosis in breast tissue taken from operative specimens, has been offered for only two patients [6, 7]. One obvious criticism of this theory is that this ‘‘constricted ring’’ is not always apparent clinically, and whether its absence represents a fundamental flaw in this theory or a milder form of the deformity is unclear. In contrast to this is the ‘‘weakened NAC skin and fascia’’ theory, supported by Costagliola and others [1, 5]. According to this theory, the causative aberrancy is a weakening within the NAC fascia, which leads to herniation of tissue through the NAC as the breast develops. Theoretically, this may be able to explain the relative loss of breast volume in the lower pole of the tuberous breast seen in more severe forms as well as the isolated milder form of the deformity (i.e., Costagliola type 0) described in this article. Again, limited histologic evidence has been presented in support of this theory [1, 5]. One apparent issue with the authors’ claim that NAC herniation is the sine qua non of the tuberous breast deformity is that it is not seen in all patients presenting with this entity. This has been previously shown by Meara and others [2, 3, 8]. For this reason, Meara et al. [8] excluded concerns with the NAC from their proposed classification system. The authors explain this T. A. Imahiyerobo J. A. Ascherman (&) Division of Plastic Surgery, Columbia University Medical Center, 161 Fort Washington Ave., Suite 509, New York, NY 10032, USA e-mail: jaa7@columbia.edu