A multi-stem cell basis for craniosynostosis and calvarial mineralization.

Craniosynostosis is a group of disorders of premature calvarial suture fusion. The identity of the calvarial stem cells (CSCs) that produce fusion-driving osteoblasts in craniosynostosis remains poorly understood. Here we show that both physiologic calvarial mineralization and pathologic calvarial fusion in craniosynostosis reflect the interaction of two separate stem cell lineages; a previously identified cathepsin K (CTSK) lineage CSC1 (CTSK+ CSC) and a separate discoidin domain-containing receptor 2 (DDR2) lineage stem cell (DDR2+ CSC) that we identified in this study. Deletion of Twist1, a gene associated with craniosynostosis in humans2,3, solely in CTSK+ CSCs is sufficient to drive craniosynostosis in mice, but the sites that are destined to fuse exhibit an unexpected depletion of CTSK+ CSCs and a corresponding expansion of DDR2+ CSCs, with DDR2+ CSC expansion being a direct maladaptive response to CTSK+ CSC depletion. DDR2+ CSCs display full stemness features, and our results establish the presence of two distinct stem cell lineages in the sutures, with both populations contributing to physiologic calvarial mineralization. DDR2+ CSCs mediate a distinct form of endochondral ossification without the typical haematopoietic marrow formation. Implantation of DDR2+ CSCs into suture sites is sufficient to induce fusion, and this phenotype was prevented by co-transplantation of CTSK+ CSCs. Finally, the human counterparts of DDR2+ CSCs and CTSK+ CSCs display conserved functional properties in xenograft assays. The interaction between these two stem cell populations provides a new biologic interface for the modulation of calvarial mineralization and suture patency.

The Lost and Variable Cause: A Systematic Review of Shunt-Related Craniosynostosis Occurrence.

OBJECTIVE: Shunt-related craniosynostosis (SRC) is the premature fusion of cranial sutures possibly due to a loss of tension across dura and suture lines after placement of a shunt for hydrocephalus. As modifications in approaches toward shunting represent a modifiable risk factor, prior literature has investigated the determinants and outcomes. However, the data remain highly variable and are limited by single-institution studies. METHODS: A systematic search of PubMed, Embase, and Web of Science from inception to February 2022 was conducted. Studies were screened by 2 reviewers for eligibility based on predefined inclusion/exclusion criteria. RESULTS: In the 9 included articles, the average follow-up time for the entire cohort ranged from 1.5 to 4.2 years. The pooled incidence of SRC across all 9 studies was 6.5% (140/2142), with an individual range of 0.53% (1/188) to 48.8% (61/125). The average time from shunt placement to SRC diagnosis ranged from 0.25 years to 4.6 years. 61% (65/110) of cases included only one suture, 88% (25/28) of these involved the sagittal suture, and those cases with multiple fusions also had 98% involvement of the sagittal suture (45/46). Overall, 94% (1783/1888) of patients had a fixed shunt placed. CONCLUSIONS: Shunt-related craniosynostosis is likely an underreported complication in the treatment of hydrocephalus. Older age at shunt placement, increased number of shunt revision procedures, and lower valve pressure settings may be risk factors for SRC development. Results also indicate that craniosynostosis can develop months to years after shunting. Future quality studies with standardization of data reporting processes are warranted to investigate this clinical problem.

Management of Total Frontal Bone Loss After Surgery for Craniosynostosis: The Modified Visor Bone Flap With Brain Cage.

Complications after craniosynostosis surgery occur in 11% to 36% of cases and may be precipitated by poor soft tissue coverage and concomitant exposure of non-sterile regions; sequelae may result in infection, osteomyelitis, and bone loss requiring complex reconstruction. In the pediatric population, autologous cranioplasty remains the gold standard due to growth potential and a more favorable complication profile than synthetic cranioplasty. Virtual surgery planning (VSP) and computer-assisted design (CAD)/computer-assisted manufacturing (CAM) technology can be utilized to create innovative, patient-specific autologous solutions, similar to the approach with synthetic cranioplasty. A novel surgical approach using VSP was used for an 18-month-old female with near total bifrontal bone loss. Surface area measurements were used to determine the amount of bone available to replace the infected frontal bone. VSP was utilized to determine the most efficient construct configuration possible to achieve maximal coverage via calculation of cranial bone surface area measurements. Surgical reconstruction of the defect was planned as a Modified Visor Bone Flap with Posterior Brain Cage. A construct was fashioned from available cranial bone struts to obtain widespread coverage. 3D Recon images from before and after surgery demonstrate almost complete re-ossification of the cranial vault with significant resulting clinical improvement. Reconstruction of total frontal bone loss is possible by utilizing this technique. VSP can improve the safety and efficiency of complex autologous cranial bone reconstructions. We propose a treatment algorithm to address the problem of near total frontal bone loss in young children for whom alloplastic implants are not suitable.

Closing the Gap: A Systematic Review and Meta-Analysis of Enhanced Recovery After Surgery Protocols in Primary Cleft Palate Repair.

OBJECTIVE: Assess the evidence for Enhanced Recovery After Surgery (ERAS) protocols in the cleft palate population. DESIGN: A systematic review of MEDLINE, Embase, Cochrane, and CINAHL databases for articles detailing the use of ERAS protocols in patients undergoing primary palatoplasty. SETTING: New York-Presbyterian Hospital. PATIENTS/PARTICIPANTS: Patients with cleft palate undergoing primary palatoplasty. INTERVENTIONS: Meta-analysis of reported patient outcomes in ERAS and control cohorts. MAIN OUTCOME MEASURE(S): Methodological quality of included studies, opioid use, postoperative length of stay (LOS), rate of return to emergency department (ED)/readmission, and postoperative complications. RESULTS: Following screening, 6 original articles were included; all were of Modified Downs & Black (MD&B) good or fair quality. A total of 354 and 366 were in ERAS and control cohorts, respectively. Meta-analysis of comparable ERAS studies showed a difference in LOS of 0.78 days for ERAS cohorts when compared to controls (P < .05). Additionally, ERAS patients utilized significantly less postoperative opioids than control patients (P < .05). Meta-analysis of the rate of readmission/return to ED shows no difference between ERAS and control groups (P = .59). However, the lack of standardized reporting across studies limited the power of meta-analyses. CONCLUSIONS: ERAS protocols for cleft palate repair offer many advantages for patients, including a significant decrease in the LOS and postoperative opioid use without elevating readmission and return to ED rates. However, this analysis was limited by the paucity of literature on the topic. Better standardization of data reporting in ERAS protocols is needed to facilitate pooled meta-analysis to analyze their effectiveness.

"Healthcare Disparities in the Care of Children with Craniosynostosis in the United States: A Systematic Review".

BACKGROUND: Healthcare inequity is a pressing concern in pediatric populations with craniofacial conditions. Little is known about the barriers to care affecting children with craniosynostosis. This systematic review investigates disparities impacting care for children with craniosynostosis in the U.S. METHODS: A comprehensive literature search was performed in the following databases from inception to December 2022: Ovid MEDLINE, Ovid EMBASE, and The Cochrane Library. Studies were screened for eligibility by two authors. All original articles that focused on disparities in access, treatment, or outcomes of craniosynostosis surgery were included. Studies describing disparities in other countries, those not written English, and review articles were excluded (Figure 1). RESULTS: An initial database search revealed 607 citations of which 21 met inclusion criteria (Figure 1). All included studies were retrospective reviews of databases or cohorts of patients. The results of our study demonstrate that barriers to access in treatment for craniosynostosis disproportionally affect minority children, children of non-English speaking parents and those of lower socioeconomic status or with Medicaid. Black and Hispanic children, non-English speaking patients, and children without insurance or with Medicaid were more likely to present later for evaluation, ultimately undergoing surgery at an older age. These patients were also more likely to experience complications and require blood transfusions compared to their more privileged, white peers. CONCLUSIONS: There is a discrepancy in treatment received by minority patients, patients with Medicaid, and those who are non-English speaking. Further research is needed to describe the specific barriers that prevent equitable care for these patients.

Pediatric Pain and Pain Management After Ambulatory Plastic Surgery: Trends in the Age of Opioid Awareness.

BACKGROUND: Overprescribing by physicians has been shown to be a major contributor to the opioid epidemic. Although pediatric ambulatory plastic surgery patients are commonly prescribed opioids for postoperative pain control, there is a lack of evidence for their necessity. This study aimed to investigate the role of prescribed narcotics in the ambulatory pediatric plastic surgery setting. METHODS: All assenting patients/guardians, ages 0 to 17 years, who underwent an ambulatory plastic surgery procedure by 1 attending surgeon from March 2018 to March 2019, were asked to participate in the study. A questionnaire was distributed at the first postoperative visit to interrogate postoperative pain, management, and narcotic use. RESULTS: A total of 95 patients/guardians completed the questionnaire. Seventy-eight percent (74) of patients picked up the narcotic medication, with 33% (31) taking at least 1 dose of narcotics, and only 9% (9) taking 4 or more doses. Patients overall found no difference in efficacy of the narcotics versus nonprescription analgesics (3.93/5 and 4.31/5, P = 0.11). Age was a significant predictor, with older patients requiring more narcotics (odds ratio, 1.12; 95% confidence interval, 1.02-1.24; P = 0.019). The type of surgery a patient underwent was not a significant predictor of the amount of narcotic used. Few patients knew how to properly dispose of the excess narcotics, with almost 50% still having it stored in their homes. CONCLUSIONS: This study demonstrates that the majority of pediatric ambulatory plastic surgery patients do not require narcotic pain medications and experience adequate pain relief with over-the-counter analgesics. Importantly, education on proper disposal of narcotic medications may be a simple, yet effective target to decrease opioid availability for abuse.

Impact of health disparities on treatment for single-suture craniosynostosis in an era of multimodal care.

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.

Analysis of the Sonopet Ultrasonic Bone Aspirator to Traditional Instrumentation for Endoscopic Suturectomy for 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 ±â€Š0.4 hours in the Sonopet group, compared to 3.2 ±â€Š1.2 hours (P = 0.05) without the Sonopet. EBL was 17.8 ±â€Š23.9 cc versus 34.7 ±â€Š75.5 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.

Incidence of Gastroesophageal Reflux Disease in Children With Cleft Lip and Palate and an Evaluation of Its Impact on Weight Gain.

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.

Proteus Syndrome With a Cranial Intraosseous Lipoma.

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.

Transition from Round to Shaped Implants in Immediate Breast Reconstruction: Our Preferred Approach and Clinical Outcomes.

BACKGROUND: 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. METHODS: 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. RESULTS: 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 < 0.0001) than those with smooth, round implants. Patients who had smooth, round implants were more likely to have postoperative nipple malposition (18 vs 0%, p < 0.0001,) and rippling (29 vs 0%, p < 0.0001.) Patients with textured, shaped implants had fewer operative revision reconstructions as compared with those with smooth, round implants (36.71 vs 12.8%, p < 0.0001) Based on these results, our technique has evolved and has eight key technical modifications. CONCLUSION: With a few adaptations in surgical technique, the transition to textured, shaped, silicone devices for breast reconstruction can be seamless with superior breast contour and reduced complications/revision rates. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Intracranial hypertension after surgical correction for craniosynostosis: a systematic review.

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.

A Comparative Retrospective Analysis of Complications After Oncoplastic Breast Reduction and Breast Reduction for Benign Macromastia: Are These Procedures Equally Safe?

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.

The Role of Virtual Surgical Planning in Surgery for Complex Craniosynostosis.

Background: Virtual surgical planning (VSP) decreases reliance on intraoperative subjective assessment of aesthetic and functional outcomes in craniofacial surgery. Here, we describe our experience of using VSP for complex craniosynostosis surgery to inform preoperative decision making and optimize postoperative outcomes. Methods: Chart review was performed for children treated with craniosynostosis at our institution from 2015 to 2021. Eight VSP maneuvers were defined and assigned to each patient when applicable: (1) complex cranioplasty: combined autologous and synthetic; (2) autologous cranioplasty; (3) synthetic cranioplasty; (4) vector analysis and distractor placement; (5) complex osteotomies; (6) multilayered intraoperative plans; (7) volume analysis; and (8) communication with parents. Outcomes between VSP and non-VSP cohorts were compared. Results: Of 166 total cases, 32 were considered complex, defined by multisutural craniosynostosis, syndromic craniosynostosis, or revision status. Of these complex cases, 20 underwent VSP and 12 did not. There was no difference in mean operative time between the VSP and non-VSP groups (541 versus 532 min, P = 0.82) or in unexpected return to operating room (10.5% versus 8.3%, P = 0.84). VSP was most often used to communicate the surgical plan with parents (90%) and plan complex osteotomies (85%). Conclusions: In this cohort, VSP was most often used to communicate the surgical plan with families and plan complex osteotomies. Our results indicate that VSP may improve intraoperative efficiency and safety for complex craniosynostosis surgery. This tool can be considered a useful adjunct to plan and guide intraoperative decisions in complex cases, reducing variability and guiding parental expectations.

Complications in craniosynostosis surgery in patients with rickets: illustrative case and systematic review of literature.

BACKGROUND: Craniosynostosis (CSS) is the premature fusion of calvarial sutures associated with identified genetic mutations or secondary to alterations in intracranial pressure, brain, or bone growth patterns. Of the metabolic etiologies implicated in CSS, X-linked hypophosphatemic rickets (XLHR) is the most common, with dysfunctional bone mineralization leading to progressive hyperostosis and delayed synostosis. There is a paucity of literature discussing the unique surgical considerations for XLHR-related CSS. OBSERVATIONS: A 26-month-old male with XLHR-related sagittal CSS underwent cranial vault remodeling (CVR). Surgery was complicated by the presence of diploic hypertrophy with significant intraoperative estimated blood loss (EBL). EBL greatly exceeded reference ranges for CVR in all-cause CSS. As a result, the surgical goals were modified and the complete planned procedure aborted. Subsequent review of preoperative imaging revealed multiple fine vascular lacunae within the bone. A systematic literature review was conducted to identify reported complications relating to surgical intervention for rickets-associated CSS. LESSONS: Future considerations for patients with XLHR-related CSS should emphasize awareness of metabolic risk factors with associated complications, and the need for selection of approach and operative management techniques to avoid EBL. Further research is required to elucidate underlying mechanisms and determine whether the encountered phenomenon is characteristic across this patient population and potentially minimized by preoperative medical therapy.

The Smile Index: Part 2. A Simple, Prognostic Severity Scale for Unilateral Cleft Lip.

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 <0.5). Severe patients had a higher likelihood of unacceptable outcomes versus not-severe patients (OR, 2.9; 95 percent CI, 1.1 to 7.7; p = 0.029; 27 percent versus 11 percent). The probability of having unacceptable outcomes for severe individuals was higher versus not-severe individuals (positive predictive value, 73 percent versus 89 percent). CONCLUSIONS: Preoperative cleft width ratio greater than 0.5 is associated with having an unacceptable surgical outcome. The authors propose a simple, objective, and clinically reproducible scale to unify the language of unilateral cleft lip severity, as a step toward improving algorithms of care, directing surgical technique, guiding patient/family discussions, and optimizing patient outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Anesthesia Exposure in Children: Practitioners Respond to the 2016 FDA Drug Safety Communication.

In December 2016, the US Food and Drug Administration (FDA) issued a drug safety warning stating that 11 commonly used anesthetic and sedative medications had potential neurotoxic effects when used in children under the age of 3 years and in pregnant women during the third trimester. A panel presentation at the sixth biennial Pediatric Anesthesia Neurodevelopmental Assessment (PANDA) symposium addressed the FDA announcement in a session entitled "Anesthesia Exposure in Children During Surgical and Non-Surgical Procedures: How Do We Respond to the 2016 FDA Drug Safety Communication?" Panelists included representatives from pediatric anesthesiology, obstetrics, pediatric surgery, and several pediatric surgical subspecialties. Each panelist was asked to address the following questions: How has the FDA labelling change affected your clinical practice including patient discussions, timing, and frequency of procedures? Has your professional society provided any guidelines for this discussion? Has there been any discussion of this topic at your national meetings? The panelists provided important perspectives specific to each specialty, which generated a lively discussion and a detailed response from the Deputy Director of the Division of Anesthesia and Addiction of the FDA describing the FDA procedures that led to this drug safety warning.

Risk Factors for Preoperative Developmental Delay in Patients with Nonsyndromic Sagittal Craniosynostosis.

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.