Faculty Bibliography

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: There is controversy regarding the treatment of young patients with unilateral craniofacial microsomia and moderate dysmorphism. The relative indication for mandibular distraction in such patients poses several questions: Is it deleterious in the context of craniofacial growth and appearance? This study was designed to address these questions. METHODS: A retrospective review of patients undergoing mandibular distraction by a single surgeon between 1989 and 2010 was conducted. Patients with "moderate" unilateral craniofacial microsomia (as defined by Pruzansky type I or IIa mandibles) and follow-up until craniofacial skeletal maturity were included for analysis. Patients were divided into two cohorts: satisfactory and unsatisfactory results based on photographic aesthetic evaluation by independent blinded observers at the initial presentation and at the age of skeletal maturity. Clinical variables were analyzed to detect predictors for satisfactory distraction. RESULTS: Nineteen patients were included for analysis. The average age at distraction was 68.2 months and the average age at follow-up was 19.55 years. Thirteen patients (68.4 percent) had Pruzansky type IIA and six patients (31.6 percent) had Pruzansky type I mandibles. Twelve patients (63.2 percent) had satisfactory outcomes, whereas seven patients (36.8 percent) had unsatisfactory outcomes. Comparing the two cohorts, patients with satisfactory outcomes had distraction at an earlier age (56.4 months versus 89.8 months; p = 0.07) and a greater percentage overcorrection from craniofacial midline (41.7 percent versus 1.8 percent; p = 0.003). CONCLUSION: Mandibular distraction is successful in patients with mild to moderate dysmorphism, provided that there is a comprehensive clinical program emphasizing adequate mandibular bone stock, proper vector selection, planned overcorrection, and comprehensive orthodontic management. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

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.

Background/Purpose: NasoAlveolar molding (NAM) is employed to reduce the severity of nasolabial deformity in the weeks prior to the primary surgical repair of patients with UCLP and BCLP. There is considerable interest among clinicians as to the impact that NAM may have on mid face growth. The purpose of this study was to determine the effect of NAM on growth of the mid face in patients with nonsyndromic UCLP at approximately 9 years of age. Methods/Descriptions: This retrospective cohort study includes 61 consecutive non syndromic Caucasian patients with UCLP, ages 6-11 years, treated with NAM. 28 cephalometric hard and soft tissue landmarks were identified and measured by two examiners. For comparison, cephalometric measurements were obtained from the Eurocleft centers (n=56) that did not utilize pre-surgical infant orthopedics (Non-PSIO). Meta-analysis was used to derive an average expected result from these trials. Student's t-tests were used to compare means from NAM-prepared patients with meta-analytic averages derived from the Eurocleft centers that did not utilize presurgical infant orthopedics (n=56). Results: On average, no significant differences were found between the NAM-prepared group and the Eurocleft centers that did not utilize NAM on the following hard and soft tissue cephalometric relationships: SNA, ANB, A'N'B' (soft tissue), nasolabial angles (CT-Sn-LS), and ANS-Me/N-Me% measurements. Conclusions: On average, no significant differences were found between the NAM prepared group and the Eurocleft centers that did not utilize presurgical infant orthopedics in the SNA, ANB, A'N'B', nasolabial angles (CT-Sn-LS), and ANS-Me/N-Me% measurements. An in depth comparison of all corresponding variables for the NAM and Non-PSIO groups will be reported. In conclusion, NAMhas no apparent long-term (age 9 years) negative or positive effect on skeletal or soft tissue facial growth in this comparison to the outcomes of Eurocleft non-PSIO treatment centers for children with non-syndromic UCLP

Background/Purpose: Multidisciplinary Cleft and Craniofacial Centers require significant investment and maintenance by medical schools and/or departments, and yet the variables contributing to their academic productivity remain unknown. This study characterizes the elements that result in high academic productivity in Cleft and Craniofacial Centers. Methods/Descriptions: All cleft and craniofacial centers accredited by American Cleft Palate-Craniofacial Association were included. Vari-ables such as university affiliation (UNI); resident training (RES); number of active surgical and orthodontic faculty (FAC); and investment in a craniofacial surgery (CF) or craniofacial orthodontics (CO) fellowship program, or both (CF+CO), were obtained for each center. All craniofacial and cleft-related research published between July 2005 and June 2015 was identified using the National Library of Medicine database; for each article, journal of publication and impact factor were also recorded. A stepwise multivariable linear regression analysis was performed on the listed variables to outcome measures of total publications, summative impact factor, and basic science publications. Results: A total of 160 centers were identified, comprising 690 active faculty, 29 craniofacial fellowships and 9 orthodontic fellowships; 2,093 articles were published in 199 journals within the study period. Variables most positively associated to a high number of publications were, in order: CF+CO (beta, CF+CO = 0.555, p < 0.001), CF (beta, CF= 0.248, p < 0.001), RES (beta, RES = 0.198, p = 0.003). Variables most positively associated to a high summative impact factor are, in order: CF+CO (beta, CF+CO = 0.551, p < 0.001), CF (beta, CF = 0.313, p < 0.001), FAC (beta, FAC = 0.183, p = 0.006). Variables most positively associated to basic science publications are, in order: CF+CO (beta, CF+CO=0.491, p < 0.001), CF (beta, CF=0.322, p < 0.001), and RES (beta, RES = 0.164, p = 0.032). Conclusions: Participation in both craniofacial surgery and orthodontic fellowships demonstrate the strongest association with academic success; craniofacial fellowship alone, residency programs and number of active faculty are also predictive. Cleft and Craniofacial Centers interested in academic performance should allocate funds and resources into these variables, particularly interdisciplinary partnerships between surgery and dentistry

Background/Purpose: Septal deviation is a common finding in skeletally mature patients with cleft lip and palate (CL/P), however the contribution of the cartilaginous and bony septum to airway obstruction is poorly defined. This study characterizes the septal and airway anatomy in skeletally mature patients with CL/P utilizing cone beam computed tomography (CBCT) and will help guide airway management of this patient population at the time of definitive rhinoplasty. Methods/Descriptions: This is a retrospective single institution review of all CL/P patients over the age of 15 who have undergone CBCT analysis. Septal deviation was measured in coronal sections of CBCT scans at the cartilaginous septum [anterior nasal spine (ANS)], and bony septum [posterior nasal spine (PNS) and midpoint between the ANS and PNS (MID)]. Airway obstruction was defined as the smallest linear distance between nasal septum and adjacent turbinate and was similarly measured at all three points. Superior (perpendicular plate of ethmoid) and inferior (vomer) bony septal displacement was measured as an angle from vertical at the coronal slice of maximal septal deviation. CL/P patients were compared to age-matched controls using Student's t-test. Stepwise multivariable linear regression was used to compare septal deviation to obstruction. Measurements were performed by two separate raters and interrater reliability was assessed using Pearson's r coeffecient. Statistical significance was held at p<0.05. Results: 24 CL/P patients and 16 age-matched controls were identified for the study. Interrater reliability for 210 independent measurements was r=0.94 (p<0.0001). Results are reported as CL/P versus control. Septal deviation was significantly increased at the ANS (2.1+/-2.2 mm vs 0.7+/-1.0 mm, p=0.03), MID (4.6+/-3.1 mm vs 2.2+/-1.2 mm, p=0.01), and PNS (2.9+/-1.8 mm vs 1.0+/-0.6 mm, p=0.0002). The airway was significantly obstructed at the ANS (1.8+/-0.8 mm vs 2.3+/-0.6 mm, p=0.03). Maximal septal deviation occurred at the bony septum in 39 of 40 patients. Both the perpendicular plate of the ethmoid (14+/-7.8degree vs 8.0+/-5.4degree, p=0.01) and vomer (25+/-15degree vs 9.0+/-7.9degree, p=0.0006) were significantly displaced from vertical. Midpoint bony septal deviation was a good predictor of anterior nasal airway obstruction (r=-0.525, p=0.008). Conclusions: Skeletally mature patients with a cleft demonstrate severe septal deviation which includes both cartilage and bone. Resection of the bony and cartilaginous septum should be considered at time of definitive rhinoplasty in CL/P patients

Background/Purpose: Treacher Collins (TC) and Robin Sequence (RS) are both associated with a retrognathic mandible, glossoptosis, and airway obstruction. TC is associated with aberrations of the lower jaw, upper jaw and nasal airway in a manner distinct from RS. Although in both TC and RS, airway obstructions in severe cases may benefit from mandibular distraction (MDO), the different pathophysiology suggests MDO to be less successful in TC compared to the current literature on RS. The purpose of this study is to report on the clinical outcomes of tracheostomy removal utilizing MDO in the TC patient population. Methods/Descriptions: A single center, twenty-year retrospective review (1991-2010) was conducted of all patients with TC treated with MDO. Recorded variables included: age of MDO, number of MDO procedures, presence of tracheostomy and complications. Literature review of clinical outcomes of MDO in the RS population demonstrates age of MDO of under one year, average on distraction per patient and avoidance of tracheostomy in over 90% of patients. Results: 24 patients with TC who underwent MDO were included in our analysis. The follow up time was 9.2 years (range1.7-17 years). The mean age of the first MDO was 4.97 years. The mean number of distractions was 1.42 with 46% of patients who had more than onedistraction attempted. The distraction devices used were external in 67% and internal in 33% of cases. 19 patients (79%) had a tracheostomy prior to MDO and only 9 (47%) patients were decannulated within one year of distraction. An additional 5 patients were decannulated several years later after further distraction and other airway procedures. Complications were divided into major (ankylosis, device failure) moderate and minor (pin infection, hypertrophic scar). Overall, 67% of TC patients had at least one complication with 41% having major complications. There was a 20% incidence of TMJ ankylosis. Conclusions: Compared to the RS population, TC patients undergo MDO at a later age, require more distraction and have less successful decannulation. Further surgery is required to effectively treat airway obstruction. The incidence of major complications

The management of patients with cleft lip and cleft palate requires prolonged orthodontic and surgical treatment and an interdisciplinary approach in providing them with optimal esthetics, function, and stability. This article describes an update on the current concepts and principles in the treatment of patients with cleft lip and palate. Sequencing and timing of orthodontic/ orthopedic and surgical treatment in infancy, early mixed dentition, early permanent dentition, and after the completion of facial growth will be discussed. (C) 2016 Elsevier Inc. All rights reserved.

Agnathia-otocephaly complex (AOC) is characterized by mandibular hypo- or aplasia, ear abnormalities, microstomia, and microglossia. Though rare and often fatal, this is the first report detailing various reconstructive strategies beyond infancy as well as longitudinal follow-up into adulthood.All patients with AOC treated at our institution over a 30 year period were reviewed. Four patients were identified, one with agnathia, one with micrognathia. Two males with nanognathia (defined as a symphyseal remnant without body nor ramus) were also included. The mean follow-up was 17 years. All four underwent perinatal tracheostomy and gastrostomy-tube placement. Commissuroplasties were typically performed before 3 years of age and repeated as necessary to allow for oral hygiene. Mandibular reconstruction was most successful with rib between ages 3 and 8, after which time, free fibula transfer was utilized. Due to some resoprtion or extrusion, all patients underwent repeated bone grafting procedures. Tissue expansion of the neck was used to restore the lower third of the face, but was most successful in the teenage years. At last follow-up of the eldest patients, one was in college while another was pursuing graduate education.AOC need not be a fatal nor untreatable condition; a reasonable quality of life can be achieved. Although the lower-facial contour may be improved, and a stoma created, the lack of musculature make deglutition virtually impossible with current therapies. Just as transplantation has emerged as a modality for facial restoration following severe trauma, so too may it be a future option for congenital deformities.