Faculty Bibliography

Background/Purpose: Children with craniofacial dysostosis syndromes including Apert, Pfeiffer and Crouzon, may present with midface abnormalities but without major (calvarial) suture synostosis and head shape anomalies. This presentation is known as progressive postnatal craniosynostosis. Minor sutures/synchondroses are continuations of major calvarial sutures toward and within the skull base. Although skull base changes are associated with midface abnormalities, their role in major suture synostosis and calvarial shape anomalies are uncertain. We hypothesized that minor suture synostosis is present in infants with syndromic, progressive postnatal craniosynostosis, and underlie major suture synostosis. Methods/Description: We performed a multi-institutional review (CHOP and NYU) of infants (<1 year) with syndromic craniosynos-tosis and available CT scans. Major (metopic, sagittal, coronal, lambdoid) and minor suture/synchondrosis fusion was determined by two craniofacial surgeons and one radiologist using Mimics or Radiant software. Interrater-reliability scores were excellent between institutions (94.1%, kappa-0.821). Statistical assessments were performed using SPSS. Results: Seventy-three patients with 84 scans were included, with diagnoses of Crouzon, Pfeiffer, Apert, Antley-Bixler, Muenke, and Saethre-Chotzen syndromes. 13 scans lacked major suture synostosis; 10 of these had minor suture fusion present, and the remaining 3 had neither major nor minor suture synostosis. A diagnosis with an FGFR2 mutation was strongly associated with a lack of major suture fusion (OR 19.0, p=0.044). Examination of individual sutures revealed that minor suture fusion occurred significantly more often in the posterior branch of the coronal arch (OR 3.33, p<0.001), squamosal arch (OR 7.32, p<0.001) and posterior intraoccipital synchondroses (OR 15.84, p<0.001), among FGFR2 vs other patients. A strong temporal correlation between age at CT and suture fusion was identified for the metopic suture and 58% of minor sutures, but not in other major sutures. An analysis of those (n=9) with multiple scans revealed a pattern of minor suture fusion followed by increased minor and major suture synostosis. Four of these had no major suture synostosis initially, but progressed to increased minor suture fusion with or without major suture involvement. Over 84% of FGFR2-group patients had minor suture fusion, however 6 patients were identified with isolated major suture synostosis. This suggests that although minor suture fusion is common in these patients, it is not required for major suture synostosis. Conclusions: Perinatal, progressive, skull base suture/synchondrosis fusion occurs in most patients with FGFR2-related craniofacial dysostosis. Syndromic patients with patent calvarial sutures and findings consistent with increased intracranial pressure should be investigated for minor suture involvement. These data have important implications for the pathophysiology of skull growth and development in this select group of patients

BACKGROUND: Cleft and craniofacial centers require significant investment by medical institutions, yet variables contributing to their academic productivity remain unknown. This study characterizes the elements associated with high academic productivity in these centers. METHODS: The authors analyzed cleft and craniofacial centers accredited by the American Cleft Palate-Craniofacial Association. Variables such as university affiliation; resident training; number of plastic surgery, oral-maxillofacial, and dental faculty; and investment in a craniofacial surgery, craniofacial orthodontics fellowship program, or both, were obtained. Craniofacial and cleft-related research published between July of 2005 and June of 2015 was identified. A stepwise multivariable linear regression analysis was performed to measure outcomes of total publications, summative impact factor, basic science publications, total journals, and National Institutes of Health funding. RESULTS: One hundred sixty centers were identified, comprising 920 active faculty, 34 craniofacial surgery fellowships, and eight craniofacial orthodontic fellowships; 2356 articles were published in 191 journals. Variables most positively associated with a high number of publications were craniofacial surgery and craniofacial orthodontics fellowships (beta = 0.608), craniofacial surgery fellowships (beta = 0.231), number of plastic surgery faculty (beta = 0.213), and university affiliation (beta = 0.165). Variables most positively associated with high a number of journals were craniofacial surgery and craniofacial orthodontics fellowships (beta = 0.550), university affiliation (beta = 0.251), number of plastic surgery faculty (beta = 0.230), and craniofacial surgery fellowship (beta = 0.218). Variables most positively associated with a high summative impact factor were craniofacial surgery and craniofacial orthodontics fellowships (beta = 0.648), craniofacial surgery fellowship (beta = 0.208), number of plastic surgery faculty (beta = 0.207), and university affiliation (beta = 0.116). Variables most positively associated with basic science publications were craniofacial surgery and craniofacial orthodontics fellowships (beta = 0.676) and craniofacial surgery fellowship (beta = 0.208). The only variable associated with National Institutes of Health funding was craniofacial surgery and craniofacial orthodontics fellowship (beta = 0.332). CONCLUSION: Participation in both craniofacial surgery and orthodontics fellowships demonstrates the strongest association with academic success; craniofacial surgery fellowship, university affiliation, and number of surgeons are also predictive.

BACKGROUND: Tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and subperiosteal release of the floor of the mouth (SPRFM) are the most commonly performed surgical procedures to treat severe airway obstruction in infants born with Pierre Robin sequence (PRS). OBJECTIVES: To determine the rate of failure of each type of procedure, in terms of mortality and the need for tracheostomy, and to determine what proportion of infants have significant airway obstruction postoperatively as determined by polysomnography (PSG) and compare the data by procedure type. METHOD: A comprehensive literature search (1981 through June 2015) was done of the National Library of Medicine database using PubMed. Extracted data included diagnosis, type of surgery, and outcome including mortality, need for postoperative tracheostomy and details of PSG. Persistence of significant airway obstruction was defined as an apnea-hypopnea index >15 events/h on PSG. RESULTS: Both mortality rate and need for tracheostomy were low for all procedures. Many studies lacked sufficient detail to identify significant airway obstruction postoperatively. In studies with sufficient data, MDO was associated with the lowest percentage of significant airway obstruction postprocedure (3.6%) compared to 50% for infants who underwent TLA. Insufficient PSG data was available for patients who were treated with SPRFM. CONCLUSIONS: There is a paucity of objective PSG data to definitively assess postoperative airway outcomes for PRS. MDO appears to be the most effective technique based on the available PSG data. Standardized use of PSG may lead to better identification and treatment of patients at risk for suboptimal airway outcomes postoperatively.

OBJECTIVE: To report the associations of oro-nasal fistulae on the patient-centered outcomes oral health-related quality of life and self-reported speech outcomes in school aged-children. DESIGN: Prospective, nonrandomized multicenter design. SETTING: Six ACPA-accredited cleft centers. PARTICIPANTS: Patients with cleft palate at the age of mixed dentition. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Prevalence of fistula and location of fistula (Pittsburgh Classification System). Patients were placed into one of three groups based on the following criteria: alveolar cleft present, no previous repair (Group 1); alveolar cleft present, previously repaired (Group 2); no congenital alveolar cleft (Group 3). Presence of fistula and subgroup classification were correlated to oral health-related quality of life (Child Oral Health Impact Profile [COHIP]) and perceived speech outcomes. RESULTS: The fistula rate was 5.52% (62 of 1198 patients). There was a significant difference in fistula rate between the three groups: Group 1 (11.15%), Group 2 (4.44%), Group 3 (1.90%). Patients with fistula had significantly lower COHIP scores (F1,1188 = 4.79; P = .03) and worse self-reported speech scores (F1,1197 = 4.27; P = .04). Group 1 patients with fistula had the lowest COHIP scores (F5,1188 = 4.78, P =.02) and the lowest speech scores (F5,1188 = 3.41, P = .003). CONCLUSIONS: Presence of palatal fistulas was associated with lower oral health-related quality of life and perceived speech among youth with cleft. The poorest outcomes were reported among those with the highest fistula rates, including an unrepaired alveolar cleft.

BACKGROUND: We present an outcomes analysis of the surgical treatment of Robin sequence including all infants and comorbid conditions treated by tongue-lip adhesion (TLA) or mandibular distraction osteogenesis (MDO). METHODS: A 19-year single-institution, multisurgeon retrospective review of all syndromic and nonsyndromic neonates with Robin sequence treated with TLA (1994-2004) or MDO (2004-2013) was performed. Comorbid conditions were recorded in all patients. Outcomes include incidence of tracheostomy, improvement in obstructive breathing, and surgical complications. Need for repeat distraction and conversion from TLA to MDO were included as secondary end-points. Polysomnography data were recorded preoperatively at 1 month and 1 year as a measure of airway improvement. RESULTS: Seventy-four MDO patients and 15 TLA patients during the study period met inclusion criteria. There was no significant difference in mean age at intervention (32.1 +/- 29.0 vs 35.5 +/- 32.1 days), birth weight (2.9 +/- 0.7 vs 3.2 +/- 0.6 kg), prematurity (23.0 vs 35.7%), or intrauterine growth restriction (31.1 vs 15.4%). Central nervous system anomalies (24.3% vs 0.0%; P < 0.04) and gastrostomy tubes (66.2% vs 33.3%; P < 0.03) were present more frequently in MDO patients versus TLA patients. Rates of other organ system anomalies were similar between the groups. The success rate was significantly higher in the MDO group (90.5% vs 60.0%; P < 0.008). Postoperative tracheostomies occurred in 8.1% of the MDO group and 33.3% of the TLA group (P < 0.02). Preoperative apnea-hyponea index was similar between the two groups (38.3 vs 38.1). The apnea-hyponea index was significantly improved in the MDO group at 1 month (4.0 vs 21.7; P < 0.002) and 1 year (5.7 vs 20.5; P < 0.005). Surgical complications were statistically less in the MDO group (20.3 vs 53.3%; P < 0.02). CONCLUSIONS: In the heterogeneous population of Robin sequence, MDO demonstrates superior outcomes measures at 1 month and 1 year compared to TLA. Fewer complications occurred in the MDO group compared to the TLA.

PURPOSE: Alveolar bone graft (ABG) has traditionally been performed with a postoperative inpatient stay secondary to donor site pain. Upon transitioning from an open iliac bone harvesting technique to an Acumed trephine, the authors observed that donor site pain was reduced eliminating an inpatient stay. This study examines the cost savings associated with outpatient ABG surgery. METHODS: A retrospective single-institution review was conducted on all patients who had an ABG performed from 2012 to 2015. Patients were categorized based upon hospital stay: inpatient, observation (23-hour), or outpatient. Cost data reported included: total direct cost, total variable direct cost, fixed direct cost, and the sum of total direct costs for both medical/surgical supplies and operating room costs. T tests were used to determine differences in various cost categories between groups of patients. RESULTS: Sixty-two procedures were performed: 7 procedures were inpatient, 16 observation, and 39 outpatient. The total direct costs averaged $4536 for inpatients, $3222 for the observation group, and $3340 for the outpatient group. Inpatient and outpatient costs were significantly different (P <0.01). Total variable direct costs (P <0.05) and fixed direct costs (P <0.01) were significantly lower in the outpatient/observation group. All costs for the observation group were significantly lower than inpatient costs, but were not significantly different than outpatient costs. There were no readmissions reported. CONCLUSIONS: Cost of an inpatient stay is significantly higher than outpatient or 23-hour observation for ABG procedures. The Acumed trephine technique allows for same-day discharge. In the face of declining reimbursement, safe and cost-efficient treatments are an appealing option.

Contemporary plastic surgery residents are increasingly challenged to learn a greater number of complex surgical techniques within a limited period. Surgical simulation and digital education resources have the potential to address some limitations of the traditional training model, and have been shown to accelerate knowledge and skills acquisition. Although animal, cadaver, and bench models are widely used for skills and procedure-specific training, digital simulation has not been fully embraced within plastic surgery. Digital educational resources may play a future role in a multistage strategy for skills and procedures training. The authors present two virtual surgical simulators addressing procedural cognition for cleft repair and craniofacial surgery. Furthermore, the authors describe how partnerships among surgical educators, industry, and philanthropy can be a successful strategy for the development and maintenance of digital simulators and educational resources relevant to plastic surgery training. It is our responsibility as surgical educators not only to create these resources, but to demonstrate their utility for enhanced trainee knowledge and technical skills development. Currently available digital resources should be evaluated in partnership with plastic surgery educational societies to guide trainees and practitioners toward effective digital content.

Importance: Septal deviation commonly occurs in patients with cleft lip and palate (CLP); however, the contribution of the cartilaginous and bony septum to airway obstruction in skeletally mature patients is poorly understood. Objectives: To describe the internal nasal airway anatomy of skeletally mature patients with CLP and to determine the contributors to airway obstruction. Design, Setting, and Participants: This single-center retrospective review included patients undergoing cone-beam computed tomography (CBCT) from November 1, 2011, to July 6, 2015, at the cleft lip and palate division of a major academic tertiary referral center. Patients met inclusion criteria for the study if they were at least 15 years old at the time of CBCT, and images were used only if they were obtained before Le Fort I osteotomy and/or formal septorhinoplasty. Twenty-four skeletally mature patients with CLP and 16 age-matched control individuals were identified for the study. Main Outcomes and Measures: Septal deviation and airway stenosis were measured in the following 3 coronal sections: at the cartilaginous septum (anterior nasal spine), bony septum (posterior nasal spine), and midpoint between the anterior and posterior nasal spine. The perpendicular plate of the ethmoid bone and vomer displacement were measured as angles from the vertical plane at the coronal section of maximal septal deviation. The site of maximal septal deviation was identified. Results: Among the 40 study participants, 26 were male. The mean (SD) age was 21 (5) and 23 (6) years for patients with CLP and controls, respectively. Septal deviation in patients with CLP was significantly worse than that of controls at the anterior nasal spine (2.1 [0.5] vs 0.8 [0.2] mm; P < .05) and posterior nasal spine (2.9 [0.5] vs 1.0 [0.3] mm; P < .01) and most severe at the midpoint (mean [SD], 4.4 [0.6] vs 2.1 [0.3] mm; P < .01). The point of maximal septal deviation occurred in the bony posterior half of the nasal airway in 27 of 40 patients (68%). The CLP bony angular deviation from the vertical plane was significant in the CLP group compared with the control group (perpendicular plate of the ethmoid bone, 14 degrees [2 degrees ] vs 8 degrees [1 degrees ]; vomer, 34 degrees [5 degrees ] vs 13 degrees [2 degrees ]; P < .05 for both), and vomer deviation was significantly associated with anterior nasal airway stenosis (r = -0.61; P < .01). Conclusions and Relevance: Skeletally mature patients with CLP have significant septal deviation involving bone and cartilage. Resection of the bony and cartilaginous septum should be considered at the time of definitive cleft rhinoplasty. Level of Evidence: NA.