Faculty Bibliography

The surgical treatment of nonsyndromic craniosynostosis is one of the most common procedures performed by craniofacial surgeons. However, for residents and fellows, the high degree of difficulty and complex anatomy may result in slow progress along a steep learning curve. This is particularly important in the context of contemporary academic practice, where work-hour limits and other factors restrict operative exposure and opportunities for trainees to learn. These issues have prompted the development of surgical simulation modalities that, while prevalent in other surgical specialties, have not been fully adopted in reconstructive surgery.Among these resources is the Craniofacial Interactive Virtual Assistant - Professional Edition (CIVA-Pro), a procedural cognition simulator that is free of charge. By integrating 3-dimensional virtual animation, voice over, and high-definition intraoperative video, CIVA-Pro describes cardinal craniofacial surgery procedures in an engaging platform. In this study, a detailed, step-by-step description of the fronto-orbital advancement surgical technique to complement the corresponding CIVA-Pro module was presented. This synergistic combination of multimedia educational resources provides a unique didactic option for current trainees to prepare for surgery.

BACKGROUND:Simulation is progressively being integrated into surgical training, yet its utility in plastic surgery has not been well described. We present a prospective, randomized, blinded trial comparing digital simulation to a surgical textbook for conceptualization of cleft-lip repair. METHODS:Thirty-five medical students were randomized to learning cleft repair using a simulator or textbook. Participants outlined markings for a standard cleft-lip repair before (pre-intervention) and after (post-intervention) 20 minutes of studying their respective resource. Two expert reviewers blindly graded markings according to a 10-point scale, on two separate occasions. Intra-rater and inter-rater reliability were calculated using intra-class correlation coefficients. Paired and independent t-tests were performed to compare scoring between study groups. A validated student satisfaction survey was administered to assess the two resources separately. RESULTS:Intra-rater grading reliability was excellent among both raters for pre-intervention and post-intervention grading (rater 1 ICC=0.94 and 0.95, respectively; rater 2 ICC=0.60 and 0.92, respectively; p<0.001). Mean pre-intervention performances for both groups were comparable (0.82±1.17 vs. 0.64±0.95; p=0.31). Significant improvement from pre-intervention to post-intervention performance was observed in the textbook (0.82±1.17 v. 3.50±1.62; p<0.001) and simulator (0.64±0.95 vs. 6.44±2.03; p<0.001) groups. However, the simulator group demonstrated a significantly greater improvement (5.81±2.01 vs. 2.68±1.49; p<0.001). Participants reported the simulator to be more effective (p<0.001) and a clearer tool (p<0.001), that allowed better learning (p<0.001) than textbooks. All participants would recommend the simulator to others. CONCLUSIONS:We present level I evidence supporting online digital simulation as a superior educational resource for novice learners, compared to traditional textbooks.

Amniotic band sequence is a complex congenital anomaly in which infants with typically no known genetic mutation have bands of maternal amniotic tissue wrapped around body parts, most commonly the limbs and digits. The authors report a novel variation on this presentation in 3 patients from 2 centers with complex craniofacial clefting and amniotic band sequence. They presented with hypertelorism, different forms of complex craniofacial clefting, and bands connecting ipsilateral hands to facial clefts, with digital-facial translocation in 2 cases. These findings support a model in which complex craniofacial clefts result in areas of exposed, sticky, and temporally and spatially coincident mesenchyme within the embryo that are susceptible to adherence of ipsilateral fetal hands. This strongly supports the intrinsic and adhesion theories of the etiology of amniotic band syndrome.

INTRODUCTION/BACKGROUND:Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. MATERIALS AND METHODS/METHODS:A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed. Twenty-four patients were identified after exclusion due to different diagnoses or insufficient medical records. Data on comorbidities, airway status, MDO operations, and complications were collected. Data were compared with published clinical outcomes in RS and data for 13 RS patients from our institution. RESULTS:Surgical success, defined as prevention of imminent tracheostomy or successful decannulation within 1 year after primary distraction, was observed in 21% of TC patients and 65% of RS patients (P = 0.01). Repeat distraction was necessary for 11 TC patients (46%) and 1 RS patient. Complications were divided into minor, moderate, and major based on need for invasive management. Overall, 67% of TC patients had complications, 20% of which were major. CONCLUSIONS:Clinical outcomes to airway function after MDO are significantly inferior in patients with TC compared with patients with RS. Repeat MDO and longer course to decannulation are more prevalent in patients affected by TC.

OBJECTIVE:To quantify 3-dimensional (3D) nasal changes in infants with unilateral cleft lip with or without cleft palate (UCL±P) treated by nasoalveolar molding (NAM) and cheilorhinoplasty and compare to noncleft controls. DESIGN/METHODS:Retrospective case series of infants treated with NAM and primary cheilorhinoplasty between September, 2012 and July, 2016. Infants were included if they had digital stereophotogrammetric records at initial presentation (T1), completion of NAM (T2), and following primary cheilorhinoplasty (T3). Images were oriented in 3dMD Vultus software, and 16 nasolabial points identified. PATIENTS/METHODS:Twenty consecutively treated infants with UCL±P. INTERVENTIONS/METHODS:Nasoalveolar molding and primary cheilorhinoplasty. MAIN OUTCOME MEASURES/METHODS:Anthropometric measures of nasal symmetry and morphology were compared in the treatment group between time points using paired Student t tests. Postsurgical nasal morphology was compared to noncleft controls. RESULTS:Nasal tip protrusion increased, and at T3 was 2.64 mm greater than noncleft controls. Nasal base width decreased on the cleft side by 4.01 mm after NAM and by 6.73 mm after cheilorhinoplasty. Columellar length of the noncleft to cleft side decreased from 2:1 to 1:1 following NAM. Significant improvements in subnasale, columella, and nasal tip deviations from midsagittal plane were observed. Treatment improved symmetry of the alar morphology angle and the nasal base-columella angle between cleft and noncleft sides. CONCLUSIONS:Three-dimensional analysis of UCL±P patients demonstrated significant improvements in nasal projection, columella length, nasal symmetry, and nasal width. Compared to noncleft controls, nasal form was generally corrected, with overcorrection of nasal tip projection, columella angle, and outer nasal widths.

BACKGROUND: Infants with craniofacial dysostosis syndromes may present with midface abnormalities but without major (calvarial) suture synostosis and head shape anomalies. Delayed presentation of their calvarial phenotype is known as progressive postnatal craniosynostosis. Minor sutures/synchondroses are continuations of major sutures toward and within the skull base. The authors hypothesized that minor suture synostosis is present in infants with syndromic, progressive postnatal craniosynostosis, and is associated with major suture synostosis. METHODS: The authors performed a two-institution review of infants (<1 year) with syndromic craniosynostosis and available computed tomographic scans. Major (i.e., metopic, sagittal, coronal, and lambdoid) and minor suture/synchondrosis fusion was determined by two craniofacial surgeons and one radiologist using Mimics or Radiant software. RESULTS: Seventy-three patients with 84 scans were included. Those with FGFR2 mutations were more likely to lack any major suture fusion (OR, 19.0; p = 0.044). Minor suture fusion occurred 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 versus other patients. Patients (n = 9) with multiple scans showed a pattern of minor suture fusion followed by increased minor and major suture synostosis. Over 84 percent of FGFR2 patients had minor suture fusion; however, six (13 percent) were identified with isolated major suture synostosis. CONCLUSIONS: Minor suture fusion occurs in most patients with FGFR2-related craniofacial dysostosis. Syndromic patients with patent calvarial sutures 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. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

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/Purpose: Amniotic band sequence (ABS) is a complex congenital anomaly in which infants with no known genetic mutation have bands of maternal amniotic tissue wrapped around body parts, most commonly the limbs and digits. Two disparate theories attempt to explain the etiology of ABS. The extrinsic theory posits that disruption of the amnion is the primary event. The intrinsic theory suggests that the bands are the result of a fetal anomaly during development. Neither theory is widely accepted with proponents of both citing evidence to support their arguments. ABS is frequently associated with complex craniofacial clefting. We report a novel variation on this presentation, which strongly supports the intrinsic theory. Methods/Description: Three patients from two centers with complex craniofacial clefting and ABS were identified. The nature of the overlap of craniofacial clefting with banding phenotypes was characterized for each patient, with photographs, comprehensive physical exams, and Genetics evaluations. Results: The three patients presented with hypertelorism, plagioceph-aly, and different forms of complex craniofacial clefting: Patient 1-bilateral Tessier 2 with left Tessier 12 clefts, a left extrophic lacrimal duct and bilateral blindness, Patient 2-left Tessier 2 and right Tessier 3 clefts, Patient 3-right Tessier 12 cleft. Patient 1 had amniotic bands connecting the left extrophic lacrimal duct, left brow and left hand, with resultant complex acrosyndactyly. Patient 2 had partial amputations of the left 3rd-5th digits, and autotransplantation of 2 digits, with one along the ipsilateral Tessier 3 cleft and one on the parietal scalp, 2 cm above the left ear. Patient 3 had amputations of the 1st-4th digits of the right hand, and autotransplantation of a portion of an unspecified finger remnant onto the right brow. Conclusions: All three patients presented with amniotic bands connecting complex craniofacial clefts with ipsilateral digits, or amputated finger remnants reimplanted within ipsilateral clefts. This finding supports a model in which complex craniofacial clefts result in areas of exposed mesenchyme within the embryo. These exposed sticky areas are susceptible to adherence of ipsilateral fetal hands. In support of this possibility, facial and early digital development are temporally coincident, and these structures are anatomically adjacent early during embryogenesis. Exposed craniofacial mesenchyme also provides a surface for amniotic attachment, resulting in bands that lead to ABS, digital amputation and autotransplantation. An alternative extrinsic interpretation of this finding in which the amnion primarily ruptures is not supported, as this would have to occur around 8 weeks of embryonic development to cause the observed phenotypes. This is well before the amnion is likely to rupture from extrinsic forces. Therefore, these findings strongly suggest that development of amniotic bands occurs secondary to intrinsic fetal anomalies