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

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.

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.

BACKGROUND: Given the great variability in perioperative management of craniosynostosis, a large-scale national survey of current practice patterns was conducted. METHODS: Using scaphocephaly as a test diagnosis, 115 craniofacial surgeons at all levels of career experience across the United States were invited to participate in an anonymous survey. RESULTS: Fifty-three surgeons (46%) completed the survey. All respondents complete repair before 1 year of age with a majority operating between 4 and 8 months. Surgeons with greater than 10 years of experience were significantly more likely to perform open repair at extremes of age (<4 months and 8-12 months) (P = 0.03) and reported shorter operative times (P = 0.01) compared with their less experienced colleagues. More than two-thirds of surgeons (68.8%) obtain preoperative imaging for every case; 83% of these prefer computed tomography scans. More than one-fourth of respondents (28%) routinely prescribe an extended course (>24 hours) of antibiotics. Overall transfusion rates remain high, with nearly 2 (65.2%) in 3 transfusing in 76% to 100% of operations. The overwhelming majority of respondents (93.6%) routinely send patients to an intensive care unit postoperatively. CONCLUSIONS: We present the largest US survey of craniosynostosis surgical practice patterns to date. General consensus exists regarding safety and emergency preparedness standards. In addition, we identified several patterns that deviate from published evidence-based guidelines. Specifically, these practices relate to the routine use of high-dose radiation imaging, long-term antibiotics, blood transfusions, and intensive postoperative surveillance. For the first time, stratifying by surgeon experience revealed significant differences in clinical practice.