Faculty Bibliography

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

Background/Purpose: The subspecialty of craniofacial surgery emphasizes skeletal exposure, preservation of critical structures, and provision of a superior cosmetic result. In recent decades, an emphasis on minimally invasive neurosurgical exposure has paved the way for increased collaboration between neurosurgeons and craniofacial surgeons. In conjunction with neurosurgery, we have applied craniofacial principles to address the major pitfalls of the transpalpebral craniotomy. Methods/Descriptions: Records of all patients who underwent transpalpebral craniotomy were reviewed. Patient age and gender, surgical indication, intraoperative details, hospital course, and postoperative complications were recorded. Results: Four patients underwent cerebrovascular aneurysm repair using the transpalpebral craniotomy approach from 2013-2015. The mean patient age was 66 years (range 57-73) and included 3 men and 1 woman. Indications included anterior communicating aneurysms in 3 patients and 1 anterior cranial fossa arteriovenous fistula (Table I). The frontal sinus was encountered in the craniotomy in 3 of 4 cases. In each case, it was partially cranialized and isolated with a medially based pericranial flap. No patients had a hematoma, infection, persistent cerebrospinal fluid leak, facial nerve injury or permanent sensory loss. One patient had an intraoperative aneurysm rupture, which was repaired at the time of surgery. Conclusions: Only recently described, transpalpebral craniotomy is the latest technique in neurosurgical exposure. Through collaboration between craniofacial surgery and neurosurgery, we have been able to utilize craniofacial principles to refine the approach. For the appropriate indication, transpalpebral craniotomy provides excellent exposure for the neurosurgeon while maintaining a superior cosmetic result and preservation of all critical structures

BACKGROUND: Reconstruction of extensive facial and scalp burns can be increasingly challenging, especially in patients that have undergone multiple procedures with less than ideal outcomes resulting in restricting neck and oral contractures, eyelid dysfunction, and suboptimal aesthetic appearance. METHODS: To establish a reconstructive solution for this challenging deformity, a multidisciplinary team was assembled to develop the foundation to a facial vascularized composite allotransplantation program. The strategy of developing and executing a clinical transplant was derived on the basis of fostering a cohesive and supportive institutional clinical environment, implementing computer software and advanced technology, establishing a cadaveric transplant model, performing a research facial procurement, and selecting an optimal candidate with the aforementioned burn defect who was well informed and had the desire to undergo face transplantation. RESULTS: Approval from the institutional review board and organ procurement organization enabled our face transplant team to successfully perform a total face, eyelids, ears, scalp, and skeletal subunit transplant in a 41-year-old man with a full face and total scalp burn. CONCLUSIONS: The culmination of knowledge attained from previous experiences continues to influence the progression of facial vascularized composite allotransplantation. This surgical endeavor methodically and effectively synchronized the fundamental principles of aesthetic, craniofacial, and microvascular surgery to restore appearance and function to a patient suffering from failed conventional surgery for full face and total scalp burns. This procedure represents the most extensive soft-tissue clinical face transplant performed to date. CLINICAL QUESTION/LEVEL OF EVIDEMCE: Therapeutic, V.

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.

The subspecialty of craniofacial surgery emphasizes skeletal exposure, preservation of critical structures, and provision of a superior cosmetic result. In recent decades, an emphasis on minimally invasive neurosurgical exposure has paved the way for increased collaboration between neurosurgeons and craniofacial surgeons.The 1990s saw the growing popularity of an eyebrow incision for orbital roof craniotomies in neurosurgery to address lesions in the anterior skull base. Disadvantages of this approach included conspicuous scarring above the brow skin, risk of injury to the frontal branch of the facial nerve, and numbness from supraorbital or supratrochlear nerve transection.A transpalpebral approach was first described in 2008 in the neurosurgical literature. An approach familiar to the craniofacial surgeon, transpalpebral exposure is used for zygomaticomaxillary complex fractures as well as aesthetic brow and periorbital surgery.In conjunction with neurosurgery, the authors have applied craniofacial principles to address the major pitfalls of the transpalpebral craniotomy. The authors present their patient series experience. Hopefully, in the future, other institutions will have increased collaboration between craniofacial surgeons and neurosurgeons.

INTRODUCTION: The relationship between nonsyndromic craniosynostosis and neurodevelopment remains controversial. Beyond standardized testing, little data exist about parental perceptions of their child's development. METHODS: Parents of children who underwent cranial vault remodeling for nonsyndromic craniosynostosis from 2011 to 2015 were asked to complete an anonymous survey. RESULTS: Twenty-two parents (31%) completed the survey. Patients included 52.4% males and 47.6% females with a mean age at surgery of 9.2 months and mean follow-up time of 19.8 months. Craniosynostosis types were primarily metopic (45%) and coronal (35%).The mean score for overall satisfaction with head shape with a maximum score of 10 was 9.2 (range 5-10). When answering whether their child is different for the better other than appearance with a 5 rating as "strongly agree," the mean score was 3.8 (range 1-5). When asked whether their child's motor, speech and behavior, or attention were different following surgery, the majority responded "No" (74%, 83%, and 67%, respectively).All parents would have their child undergo surgery again or recommend surgery to another family. There were no statistically significant differences when stratifying by age at surgery or length of follow-up. When stratifying by sex, parents of males gave higher ratings for whether their children were better other than appearance compared with females, 4.8 versus 3.1 (P = 0.031). DISCUSSION: Parents rated their child's neurodevelopment as largely age-appropriate following cranial vault remodeling. These findings are concordant with the majority of formal, standardized testing for this age group. Assessing parental perceptions once children enter school age may reveal increased developmental delays.

BACKGROUND: The application of aesthetic, craniofacial, and microsurgical principles in the execution of face transplantation may improve outcomes. Optimal soft-tissue face transplantation can be achieved by incorporating subunit facial skeletal replacement and subsequent tissue resuspension. The purpose of this study was to establish a reconstructive solution for a full face and scalp burn and to evaluate outcome precision and consistency. METHODS: Seven mock face transplants (14 cadavers) were completed in the span of 1 year. Components of the vascularized composite allograft included the eyelids, nose, lips, facial muscles, oral mucosa, total scalp, and ears; and skeletal subunits of the zygoma, nasal bone, and genial segment. Virtual surgical planning was used for osteotomy selection, and to evaluate postoperative precision of hard- and soft-tissue elements. RESULTS: Each transplant experience decreased each subsequent transplant surgical time. Prefabricated cutting guides facilitated a faster dissection of both donor and recipient tissue, requiring minimal alteration to the allograft for proper fixation of bony segments during inset. Regardless of donor-to-recipient size discrepancy, ample soft tissue was available to achieve tension-free allograft inset. Differences between virtual transplant simulation and posttransplant measurements were minimal or insignificant, supporting replicable and precise outcomes. CONCLUSIONS: This facial transplant model was designed to optimize reconstruction of extensive soft-tissue defects of the craniofacial region representative of electrical, thermal, and chemical burns, by incorporating skeletal subunits within the allograft. The implementation of aesthetic, craniofacial, and microsurgical principles and computer-assisted technology improves surgical precision, decreases operative time, and may optimize function.

BACKGROUND: Cadaveric face transplant models are routinely used for technical allograft design, perfusion assessment, and transplant simulation but are associated with substantial limitations. The purpose of this study was to describe the experience of implementing a translational donor research facial procurement and solid organ allograft recovery model. METHODS: Institutional review board approval was obtained, and a 49-year-old, brain-dead donor was identified for facial vascularized composite allograft research procurement. The family generously consented to donation of solid organs and the total face, eyelids, ears, scalp, and skeletal subunit allograft. RESULTS: The successful sequence of computed tomographic scanning, fabrication and postprocessing of patient-specific cutting guides, tracheostomy placement, preoperative fluorescent angiography, silicone mask facial impression, donor facial allograft recovery, postprocurement fluorescent angiography, and successful recovery of kidneys and liver occurred without any donor instability. Preservation of the bilateral external carotid arteries, facial arteries, occipital arteries, and bilateral thyrolinguofacial and internal jugular veins provided reliable and robust perfusion to the entirety of the allograft. Total time of facial procurement was 10 hours 57 minutes. CONCLUSIONS: Essential to clinical face transplant outcomes is the preparedness of the institution, multidisciplinary face transplant team, organ procurement organization, and solid organ transplant colleagues. A translational facial research procurement and solid organ recovery model serves as an educational experience to modify processes and address procedural, anatomical, and logistical concerns for institutions developing a clinical face transplantation program. This methodical approach best simulates the stressors and challenges that can be expected during clinical face transplantation. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.