Craniosynostosis of the Metopic Suture in a Patient With CADASIL/Lehman Syndrome.

ABSTRACT: A 3-month-old patient presented for evaluation by plastic surgery with marked trigonocephaly and was subsequently diagnosed with metopic craniosynostosis. During presurgical evaluation, the patient was found to have two variants of the NOTCH3 gene, resulting in the diagnosis of lateral meningocele (Lehman) syndrome. Due to the increased possibility of stroke associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, the patient underwent only anterior calvarial vault remodeling without fronto-orbital advancement for correction of her craniosynostosis. This unique constellation of symptoms, and its impact on operative management, has not been previously described in the literature.

Description of Mexican Cleft Surgeons' Experience With Foreign Surgical Volunteer Missions in Mexico.

BACKGROUND AND PURPOSE: Mexican cleft surgeons provide multidisciplinary comprehensive cleft lip and palate care to children in Mexico. Many Mexican cleft surgeons have extensive experience with foreign, visiting surgeons. The purpose of this study was to characterize Mexican cleft surgeons' domestic and volunteer practice and to learn more about Mexican cleft surgeons' experience with visiting surgeons. METHODS: A cross-sectional validated e-mail survey tool was sent to Mexican cleft surgeons through 2 Mexican plastic surgery societies and the Asociación Mexicana de Labio y Paladar Hendido y Anomalías Craneofaciales, the national cleft palate society that includes plastic and maxillofacial surgeons who specialize in cleft surgery. We utilized validated survey methodology, including neutral fact-based questions and repeated e-mails to survey nonresponders to maximize validity of statistical data; response rate was 30.6% (n = 81). RESULTS: Mexican cleft surgeons performed, on average, 37.7 primary palate repairs per year with an overall complication rate of 2.5%; 34.6% (n = 28) of respondents had direct experience with patients operated on by visiting surgeons; 53.6% of these respondents performed corrective surgery because of complications from visiting surgeons. Respondents rated 48% of the functional outcomes of visiting surgeons as "acceptable," whereas 43% rated aesthetic outcomes of visiting surgeons as "poor"; 73.3% of respondents were never paid for the corrective surgeries they performed. Thirty-three percent of Mexican cleft surgeons believe that there is a role for educational collaboration with visiting surgeons. CONCLUSIONS: Mexican cleft surgeons have a high volume of primary cleft palate repairs in their domestic practice with good outcomes. Visiting surgeons may play an important role in Mexican cleft care through educational collaborations that complement the strengths of Mexican cleft surgeons.

Number of Surgical Procedures for Patients With Cleft lip and Palate From Birth to 21 Years Old at a Single Children's Hospital.

BACKGROUND/PURPOSE: Pediatric patients with cleft lip and palate undergo numerous surgeries throughout their childhood and early adulthood to correct the aesthetic and functional stigmata of their diagnoses. This study identifies patient and clinical factors that contribute to the number of surgeries and anesthesia events children undergo for their cleft repair. METHODS/DESCRIPTION: Retrospective chart review was performed using the genetic and dysmorphology database at Rady Children's Hospital San Diego. All patients with cleft lip or cleft palate diagnosis who underwent surgery at Rady Children's Hospital San Diego between 1988 and 2014 were included. A sample size of 71 patients was analyzed. Poisson regression was used to determine if there is a relationship between each variable of interest and the number of procedures. RESULTS: For our sample of 71 patients, the average number of surgical procedures was 8.6 (SD, 4.4). The average number of anesthesia events was 6.7 (SD, 3.3). Across and within diagnosis, race and sex were not statistically significant factors in patients' number of surgeries. Patients with bilateral cleft lip and palate (BCLP) and unilateral cleft lip and palate had, on average, 10 and 9.4 procedures, respectively. This is in contrast to patients with unilateral cleft lip and isolated cleft palate who had, on average, 5.3 and 5.9 procedures, respectively. This difference was significant (P value = 0.01). Patients were also compared based on continuity of care. Patients who had their cleft surgeries by multiple plastic surgeons affiliated with our children's hospital had significantly more surgeries (P = 0.01). A surgical outlier subset of 17 patients (24%) was identified who had more than 10 surgeries. These patients also had, on average, 11.3 (unilateral cleft lip and palate) and 11.8 (bilateral cleft lip and palate) anesthesia events; this is in contrast to the nonoutliers who had, on average, 4.1 to 8 anesthesia events. CONCLUSIONS: This retrospective review identifies patient and clinical factors that contributed to the number of surgeries and anesthesia events that children with a cleft underwent from birth to adulthood at a single children's hospital. We found that "heralding" events, such as palatal fistula and multiple nasal or lip revisions, can put children at risk for an increasing--and perhaps deleterious--number of surgery and anesthesia events.

Comparing Gender Congruency in Nonsurgical versus Postsurgical Top Surgery Patients: A Prospective Survey Study.

Background: Gender dysphoria can result in reduced quality of life. Treatments include hormone replacement therapy (HRT) and gender-affirming surgery. Our study compared congruency, satisfaction, and discrimination in patients who underwent top surgery and HRT versus HRT alone. We hypothesized improved outcomes in top surgery patients but that lack of access is a common barrier. Methods: Transgender and nonbinary subjects who underwent at least 6 months of HRT were recruited and answered questions on gender congruency, discrimination, and barriers to care. Surgical patients were asked about postoperative satisfaction using the BREAST-Q. A Mann-Whitney test compared survey responses between study arms. Results: One hundred twelve eligible subjects completed the survey. Surgical subjects answered significantly more positively (P < 0.001) on all questions regarding gender congruency. The greatest difference was observed in how subjects' physical bodies represented their gender identity, where the surgery group rated higher on the five-point Likert scale by 2.0 points (P < 0.001). Surgical patients also reported less violence, verbal abuse, and discrimination (P < 0.003). Within the hormone arm, 87.1% stated desire for surgery and 62.5% declared barriers to surgery, with cost and insurance coverage representing the most common barriers. Finally, surgical subjects reported high satisfaction on the BREAST-Q, scoring more than 3.0 in all categories of breast augmentation and more than 2.6 for breast reduction on a four-point Likert scale. Conclusions: Top surgery, in addition to HRT, significantly improves gender congruency and decreases discrimination and abuse, compared with HRT alone. Unfortunately, barriers including cost and lack of insurance continue to be obstacles for care.

Ethical Dilemmas in Global Plastic Surgery: Divergent Perspectives of Local and Visiting Surgeons.

BACKGROUND: As more of the world's resources are directed toward improving patient access to safe surgical and anesthesia care, there is a growing concern that volunteer surgeons' "desire to help" has numerous unintended consequences. The purpose of this study was to ask in-country, local surgeons and visiting volunteer plastic surgeons about the frequency of ethical dilemmas for different types of global surgery collaborations and to assess their perception of compliance with the concepts of autonomy, beneficence, nonmaleficence, and justice. METHODS: A cross-sectional email survey tool was sent to a representative sample of domestic American Society of Plastic Surgeons members, all international members of the American Society of Plastic Surgeons, and international partners of the Volunteers in Plastic Surgery committee of the Plastic Surgery Foundation. The survey response rate was 7.7 percent, with 356 respondents from 65 countries. RESULTS: This survey data showed a statistically significant divergence of perspectives between local and visiting surgeons in regard to scope of practice, quality of patient care, impact on local health care systems, perception of the quality of care by local providers, the integrity of the informed consent process, and photography in global plastic surgery collaborations. Though the short-term mission trip model was particularly polarizing when comparing the responses of local and visiting surgeons, educational and research exchanges may also significantly interrupt local health care and incite ethical lapses. CONCLUSION: This survey provides quantitative insight into the impact of current global plastic surgery collaborations and highlights ethical areas of disagreement between local and visiting surgeons.

Philanthropy in Plastic Surgery: Best Practices and Measuring Impact.

BACKGROUND: Philanthropy in plastic surgery supports research, clinical care, academic infrastructure, and education in the United States and internationally. Plastic surgeons have opportunities to innovatively address unmet needs in their local and global communities by forming philanthropic nonprofit organizations. METHODS: The authors queried three national philanthropic databases (Charity Navigator, Guidestar, and ProPublica) for Internal Revenue Service 990 form tax return information related to philanthropic plastic surgery organizations. The authors analyzed the financial information publicly available about current plastic surgery philanthropic organizations and their funding sources. RESULTS: Seventy-three federally tax-exempt groups identified plastic surgery as their primary area of work to the Internal Revenue Service in 2019, and 52 of those organizations said they engaged in clinical and/or educational philanthropy.3 In 2017, a total of $158.5 million was donated to these groups in cash and noncash donations including equipment and time. Analyses of 6 years (2013 to 2018) of tax records from groups that provide plastic surgery clinical, research, or educational philanthropy reveal that government grants provide very little funding for this work. Eighty-six percent of money raised for these groups in 2017 was collected by means of direct donations. Money obtained from fundraising events contributed 2 percent to their total monies raised in 2017 on average and 8 percent came from noncash gifts. CONCLUSIONS: A broad body of academic research is reviewed here that guides best practices and measuring a group's impact and outcomes. The details of a philanthropic group's organization and finances directly influence the impact of their work and, as such, are worthy of our sustained attention.

Rethinking Farkas: Updating Cephalic Index Norms in a Large, Diverse Population.

BACKGROUND: Cephalic index, the ratio of head width to length, is one normative indicator used by insurers to derive criteria for plagiocephaly helmet authorization. Current norms were established by a small sample of white children in the 1987 Farkas and Munro data set. This study establishes updated cephalic index values for infants and children in a large, diverse patient population. METHODS: Children aged 0 to 3 months, 3 to 6 months, 9 to 12 months, 2 to 3 years, and 12 to 14 years were recruited at their well-child appointment. Cephalic index was calculated for each age group and compared to previously established norms. RESULTS: Eight hundred seventy patients met inclusion criteria. The means for boys and girls between 0 and 6 months were 83.5 (n = 155, SD 6.01) and 83.5 (n = 191, SD 5.80), respectively. Established means for boys and girls between 0 and 6 months were 74.4 (n = 38, SD 5.2) and 74.3 (n = 49, SD 6.1), respectively. The difference between norms is highly statistically significant (p < 0.0001). For this age range, insurance criteria for a helmet is >83.7 for boys and >82.7 for girls. Using previous norms, 74 boys (44.6 percent) and 104 girls (54.5 percent) would meet criteria for a helmet under current guidelines. CONCLUSIONS: The mean cephalic index of children has changed. The reasons could include diversifying populations in the United States and the introduction of the Back to Sleep campaign. Over 50 percent of children may inappropriately meet criteria for a helmet based on prior norms. Updating norms could change the definition of plagiocephaly for a helmet orthosis.

Endoscopic surgery for nonsyndromic craniosynostosis: a 16-year single-center experience.

OBJECTIVE: In this paper the authors review their 16-year single-institution consecutive patient experience in the endoscopic treatment of nonsyndromic craniosynostosis with an emphasis on careful review of any associated treatment-related complications and methods of complication avoidance, including preoperative planning, intraoperative management, and postoperative care and follow-up. METHODS: A retrospective chart review was conducted on all patients undergoing endoscopic, minimally invasive surgery for nonsyndromic craniosynostosis at Rady Children's Hospital from 2000 to 2015. All patients were operated on by a single neurosurgeon in collaboration with two plastic and reconstructive surgeons as part of the institution's craniofacial team. RESULTS: Two hundred thirty-five patients underwent minimally invasive endoscopic surgery for nonsyndromic craniosynostosis from 2000 to 2015. The median age at surgery was 3.8 months. The median operative and anesthesia times were 55 and 105 minutes, respectively. The median estimated blood loss (EBL) was 25 ml (median percentage EBL 4.2%). There were no identified episodes of air embolism or operative deaths. One patient suffered an intraoperative sagittal sinus injury, 2 patients underwent intraoperative conversion of planned endoscopic to open procedures, 1 patient experienced a dural tear, and 1 patient had an immediate reexploration for a developing subgaleal hematoma. Two hundred twenty-five patients (96%) were admitted directly to the standard surgical ward where the median length of stay was 1 day. Eight patients were admitted to the intensive care unit (ICU) postoperatively, 7 of whom had preexisting medical conditions that the team had identified preoperatively as necessitating a planned ICU admission. The 30-day readmission rate was 1.7% (4 patients), only 1 of whom had a diagnosis (surgical site infection) related to their initial admission. Average length of follow-up was 2.8 years (range < 1 year to 13.4 years). Six children (< 3%) had subsequent open procedures for perceived suboptimal aesthetic results, 4 of whom (> 66%) had either coronal or metopic craniosynostosis. No patient in this series either presented with or subsequently developed signs or symptoms of intracranial hypertension. CONCLUSIONS: In this large single-center consecutive patient series in the endoscopic treatment of nonsyndromic craniosynostosis, significant complications were avoided, allowing for postoperative care for the vast majority of infants on a standard surgical ward. No deaths, catastrophic postoperative morbidity, or evidence of the development of symptomatic intracranial hypertension was observed.

Predictors, Quality Markers, and Economics of Volunteering Internationally: Results from a Comprehensive Survey of American Society of Plastic Surgeons Members.

BACKGROUND: Plastic surgeons have a long history of international volunteer work. To date, there have been no outcome-based studies among surgeons who volunteer internationally. The purpose of this study was to describe predictors of volunteering, clinical quality markers, and economics of international volunteering among American plastic surgeons. METHODS: A cross-sectional validated e-mail survey tool was sent to all board-certified plastic surgeons by the American Society of Plastic Surgeons. The survey response rate was 15 percent (745 total individuals), of which 283 respondents traveled within the past 5 years. Analysis was performed in R. Stepwise multivariate logistic regression was performed to determine the predictors of death/complication. RESULTS: Respondents reported high use of medical records, follow-up care, and host affiliation. Fewer than half of all respondents reported use of international safety surgery guidelines, and the majority of respondents reported volunteering abroad outside of their scope of practice. The majority of children younger than 5 years were not cared for by a pediatric anesthesiologist. The majority of participants reported personally spending more than $1000 on their last trip and performing surgery estimated to be worth on average $28,000 each. CONCLUSIONS: International surgical volunteer trips attempt to ease the global burden of surgical disease. The authors' study reports variation in quality of care provided on these trips. Most significantly, the majority of children younger than 5 years were not cared for by a pediatric anesthesiologist, and many plastic surgeons operated outside of their scope of practice.