Faculty Bibliography

PURPOSE: Currently, surgeons approach autogenous microtia repair by creating a two-dimensional (2D) tracing of the unaffected ear to approximate a three-dimensional (3D) construct, a difficult process. To address these shortcomings, this study introduces the fabrication of patient-specific, sterilizable 3D printed auricular model for autogenous auricular reconstruction. METHODS: A high-resolution 3D digital photograph was captured of the patient's unaffected ear and surrounding anatomic structures. The photographs were exported and uploaded into Amira, for transformation into a digital (.stl) model, which was imported into Blender, an open source software platform for digital modification of data. The unaffected auricle as digitally isolated and inverted to render a model for the contralateral side. The depths of the scapha, triangular fossa, and cymba were deepened to accentuate their contours. Extra relief was added to the helical root to further distinguish this structure. The ear was then digitally deconstructed and separated into its individual auricular components for reconstruction. The completed ear and its individual components were 3D printed using polylactic acid filament and sterilized following manufacturer specifications. RESULTS: The sterilized models were brought to the operating room to be utilized by the surgeon. The models allowed for more accurate anatomic measurements compared to 2D tracings, which reduced the degree of estimation required by surgeons. Approximately 20 g of the PLA filament were utilized for the construction of these models, yielding a total material cost of approximately $1. CONCLUSION: Using the methodology detailed in this report, as well as departmentally available resources (3D digital photography and 3D printing), a sterilizable, patient-specific, and inexpensive 3D auricular model was fabricated to be used intraoperatively. This technique of printing customized-to-patient models for surgeons to use as 'guides' shows great promise.

Background/Purpose: The aim of this study is to perform a cephalometric evaluation of the craniofacial skeleton of patients with TCS. Methods/Description: Retrospective single institution review of all patients (N= 104) with TCS and a preoperative cephalogram was conducted (30 patients). Patients were divided into three groups based on their ages: infancy (mean 0.62 yr; range:0.01-2.2 yrs) adolescence (mean 7.91 yr, range:5.18-11.26 yrs) and post adolescent-young adulthood (mean 17.04 yr; range:15.49-21.36 yrs). Right and left sides were evaluated separately if asymmetry was noted to be present (44 sides). The cephalometric variables were compared to Bolton and Moyers norms and also to each other using ANOVA and student's t-test. Results: All maxillary and mandibular measurements were significantly different from normative values with the exception of SNA and upper gonial angle (Na-Go-Me). SNB, SNPg angles were severely decreased and Pg (Pg-NB) was significantly retruded (p<0.001). Gonial angle (Ar-Go-Me) was significantly wider than normal as lower gonial angle (Ar-Go-Na) and antegonial angles were significantly increased (p<0.001) in all three age groups. There was no difference among the groups in terms of increased antegonial angles. All vertical plane angles (SN-MP, SN-GoGN, FH-MP, SN-PP, PP-MP) were increased significantly as well (p<0.001). Correspondingly, the ratio between lower anterior face height and total face height was significantly higher, while posterior face height to anterior face height was significantly decreased (p<0.001). More than half of the patients (N= 17/30) possessed a parasagittal symphyseal notch at the anterior surface of the chin. The depth and width of this notch were increased from infancy to adolescence (p<0.01). Accordingly, symphysis inclination (SN-Symp.) increased significantly over time (p<0.01). The maxillary posterior region showed decreased height (p<0.01). Our findings suggest that the maxillo-mandibular deformity demonstrates what we have termed a 'parasagittal orbito-maxillo-zygomatic cleft' which is aligned along the path of maximum mandibular atresia (diminished or missing coronoid, condylar processes and rami. Conclusions: When comparing cephalometric values in patients with TCS to Bolton and Moyers, all structures showed varying degrees of deformation or dislocation with the exception of maxillary sagittal position. These changes were most prevalent in the posterior maxillae, mandible, symphysis and antegonial area of the mandible. Certain skeletal changes did not show variance from infancy to adulthood, such as maxilla-mandibular angle and Wits value, however changes of the symphysis region became more severe over time. Further, soft tissue facial convexity increased severely in all growth periods

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

Background/Purpose: The reconstruction of the wide Tessier #4 cleft is classically limited by persistent lower lid ectropion/medical canthal disruption or the incorporation of unaesthetically located scars which violate the subunit border principle of facial reconstruction. We present a novel repair technique which: can be applied at infancy; does not require tissue expansion; restores stable lower eyelid and medial canthal position; and respects the subunit border principle of facial repair. Methods/Description: A neonate with a complete, wide, Tessier #4 facial cleft presents with an over 2/3rd lower eyelid loss. Presurgical tape therapy was applied to lengthen the lateral tissues transversely and vertically. A Tenzel flap extended to a Schrudde cervicofacial flap was planned to radically mobilize the lower eyelid to the medial canthus in a tension-free manner. A robust vascular supply was maintained to this large flap using a deep plane dissection. Results: Surgical repair was performed at 3 months of age. No tissue expansion was used. A Tenzel pattern flap was mobilized in the subcutaneous plane. This flap was raised in continuity with a Schrudde cervicofacial flap raised in the deep plane. Facial nerves were directly visualized and preserved during the operation. A conjunctival flap was raised from the floor of the orbit was used to reconstruct the posterior lamella of the lower eyelid. The Tenzel/Schrudde flap was rotated, without tension over the defect and to the nose/cheek junction. At the time of inset, there was redundant flap skin superiorly at the level of the lower eyelid and medially at the area of the medial canthus. This redundancy was incorporated into the reconstruction to prevent ectropion and medial canthus disruption. Suspensory sutures were applied to the infraorbital rim and pyriform aperture to prevent sagging of the flap. A Millard repair was used to reconstruct the lip at the level of the philtrum. The flap demonstrated 100% take despite radical mobilization. The final scar followed the philtral line, the nasal/cheek junction, the subcilliary line and the anterior auricular/retro auricular border. Lower eyelid and medial canthal position was stable after 6 months. Facial nerve function was preserved with this approach. Conclusions: A Tenzel/Schrudde deep-plane cervicofacial flap can be safely applied to infants with a wide Tessier #4 facial cleft. No tissue expansion is needed. This is the first repair technique which places final scars perfectly along the subunit borders of the face while preserving lower eyelid and medial canthal position, even in the patient with significant lower eyelid loss

Background/Purpose: Autogenous ear reconstruction remains one of the most technically challenging procedures in plastic surgery. Current methods to autogenous ear construct design entail tracing the contralateral (unaffected) ear, if available, and using this 2 dimensional outline as a surgical model. This study explores the feasibility of creating in-house patient specific intraoperative 3D models of autogenous ear reconstruction. Methods/Description: A 3 dimensional photograph of the unaffected ear (3DMD, Atlanta, GA) of a patient with unilateral microtia was uploaded into Amira (FEI Company, Hillsboro, Oregon, USA) and transformed to a (.stl) digital model. After rendering the (.stl) model of the ear, it was imported into Blendere (Amsterdam, The Netherlands) where it was inverted along the vertical axis to create a working template of the contralateral ear. The depths of the scapha, triangular fossa and cymba were all deepened to accentuate these contours. Additional relief was added to the helical root to further define this structure. The final template was digitally separated to create the requisite auricular components for the Nagata technique reconstruction: helix; antihelical fold with the superior and inferior crus; base frame. The patient had an intact tragus. The helix was digitally straightened to optimize its use as a model. The complete auricular model and the separated auricular components were all individually 3D printed (Builder Premium 3D Printer, Noordwijkerhout, The Netherlands) using a polylactic acid filament and sterilized following the manufacturer's specifications (1218C for 1 hour and 30 minute dry cycle). Results: The total time of digital preparation was 5 hours. Total time of 3D printing was 5.5 hours. Total cost of manufacturing was $0.78. On the day of surgery these sterilized, patient-specific 3 dimensional models were brought to the operating room and placed on the back table with the ear sculpting tools and carving block. The sterilized models were placed on the cartilage grafts and the forms and relief of the cartilage construct was easily appreciated and incorporated into the cartilage shape. Compared to the classic auricular tracings also present during this surgery these 3D printed models contained more detailed anatomic information which eliminated much of the guesswork from auricular reconstruction and resulted in a more efficient and precise operation. Conclusions: Leveraging hardware, expertise and software platforms already existing within an academic medical center, affordable, sterilizable, patient-specific 3D auricular models can be manufactured and used during autogenous ear construction

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.