Youngest Composite Full-Face Transplant: A Model for Vascularized Composite Allograft in Younger Populations.

BACKGROUND: The field of face transplantation continues to evolve, with more complex defects being addressed, and, at the same time, increased outcome expectations. Given our unique long-term experience in this field, we consented one of the youngest patients to undergo a full-face transplant. METHODS: An 18-year-old woman presented with complete destruction of her central face and craniofacial structures. She had coexisting major injuries, including pituitary gland, visual axis, and motor control. After extensive rehabilitation and reconstruction techniques, the patient underwent face transplant on May 4, 2017, at the age of 21 years. RESULTS: The total operative time for the recipient was 26 hours. There were no major perioperative complications. Since transplant, the patient has undergone 3 revision surgeries. She is near completely independent from a daily life activity standpoint. She has had 1 episode of rejection above grade II that was successfully treated with a short-term increased in immunosuppression. CONCLUSIONS: Contrary to data in solid organ transplantation where youth is associated with increased risk of rejection, our current algorithm in immunosuppression, combined with this patient's compliance, has led to only 1 rejection episode beyond grade II. This successful transplant can serve as a model for future vascularized composite transplants in younger populations.

The Effect of Age on Fat Distribution in the Neck Using Volumetric Computed Tomography.

BACKGROUND: Neck fat distribution plays an important role in aging, yet how fat distribution changes with age is largely unknown. This study used volumetric computed tomography in live patients to characterize neck fat volume and distribution in young and elderly women. METHODS: A retrospective analysis was conducted of head and neck computed tomographic angiographs of 20 young (aged 20 to 35 years) and 20 old (aged 65 to 89 years) women. Fat volume in the supraplatysmal and subplatysmal planes was quantified. Distribution of fat volume was assessed by dividing each supraplatysmal and subplatysmal compartment into upper, middle, and lower thirds. RESULTS: Total supraplatysmal fat volume was greater than subplatysmal in all patients. Young patients had more total supraplatysmal fat than old patients (p < 0.0001). No difference was found between age groups in subplatysmal fat (p > 0.05). No difference was found between upper/middle/lower third supraplatysmal fat volumes in young patients. When comparing supraplatysmal thirds within the elderly population, the middle third fat volume (28.58 ± 20.01 cm3) was greater than both upper (18.93 ± 10.35 cm3) and lower thirds (15.46 ± 11.57 cm3) (p < 0.01). CONCLUSIONS: This study suggests that total supraplatysmal fat volume decreases with age. Older patients had more fat volume in the upper and middle thirds compared with the lower third of the supraplatysmal fat compartment, whereas young patients had more evenly distributed fat. These results suggest that fat deposition and redistribution in the neck occur with age and may be a contributing factor to the obtuse cervicomandibular angle of the elderly.

Current Trends in Management of Nonsyndromic Unilateral Coronal Craniosynostosis: A Cross-sectional Survey.

BACKGROUND: Although the natural history of nonsyndromic unilateral coronal craniosynostosis has been extensively described, optimal management remains controversial due to lack of Level 1 evidence. This study aims to assess the current state of practice among craniofacial surgeons. METHODS: Ninety-four craniofacial surgeons were approached to complete a survey consisting of 15 questions. Data were collected assessing surgeons' primary surgical indication, timing of intervention, preoperative imaging, and choice of technique for patients presenting with nonsyndromic unilateral coronal craniosynostosis. Choice of technique and timing of intervention in case of recurrence were also investigated. RESULTS: After 5 mailings, the response rate was 61%. The combination of both appearance and raised intracranial pressure was the primary indication for treatment for 73.2% of surgeons. Preoperative CT scan of the skull was "always" performed by 70.1% of respondents. Open surgical management was most commonly performed at 8-10 months of age (38.6%). Bilateral frontal craniectomy with remodeling of the supraorbital bandeau and frontal bone was the most common choice of procedure (84.2%). In case of mild to moderate and moderate to severe recurrences at 1 year of age, 89.5% and 47.4% of surgeons opted for conservative management, respectively. Optimal timing for repeat cranioplasty was after 4 years of age (65.5%). Overall, 43.4% quoted lack of evidence as the greatest obstacle to clinical decision-making when dealing with unilateral synostosis. CONCLUSION: This survey exposes the lack of consensus and the disparity of opinion among craniofacial surgeons regarding the management of nonsyndromic coronal synostosis, particularly in the setting of recurrence.

Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome.

BACKGROUND: We report new data for a rare face transplant performed 3 years ago. Granulomatosis with polyangiitis (GPA) (Wegener) is a severe autoimmune necrotizing vasculitis and parenchymal inflammatory disease that can affect any organ including those of the craniofacial region. Skin involvement manifests as malignant pyoderma. This account (1) highlights the technical details of face transplantation for this unique indication, (2) reports the 3-year posttransplant outcome, and (3) describes relevant immunological aspects. METHODS: A Le Fort III near-total face and near-total scalp transplant was performed after extensive trauma and subsequent bone and soft tissue infection in a patient with GPA. Incisions were planned along facial aesthetic subunits. The vascular pedicle comprised the facial and superficial temporal arteries bilaterally. The functioning left eye was preserved and fitted into the donor tissues. RESULTS: The procedure took 21 hours, and transfusion was limited to 4 units of packed red cells. Early medical and surgical complications were successfully treated. At 3 years, acceptable aesthetic outcome was achieved with adequate color match and scalp hair growth. The patient has recovered light touch, temperature, and 2-point discrimination and has evidence of symmetric cheek elevation albeit with limited eyelid and frontalis function. GPA relapse did not occur. Four acute rejections were fully reversed. CONCLUSIONS: This case represents a new underlying disease (trauma + GPA) leading to face transplantation and a unique clinical scenario where allografting was indicated for potentially life-threatening and sight-preserving reasons and not for mere functional and aesthetic concerns. Despite complexity, 3-year clinical outcome is encouraging, and the patient is no longer at risk for dural exposure, meningitis, and related morbidity.

Analysis of Craniofacial Remodeling in the Aging Midface Using Reconstructed Three-Dimensional Models in Paired Individuals.

BACKGROUND: Aging leads to a panoply of changes of facial morphology. The present study was conducted to analyze modifications of the facial skeleton with aging, using high-resolution imaging and comparing the same individuals at two time points. METHODS: The electronic medical record system was reviewed since its inception in 2001 for patients for whom two computed tomographic scans of the midface were obtained at least 9 years apart. The computed tomographic scans were converted into three-dimensional craniofacial models for each patient, using the initial and the follow-up computed tomographic scan data. The models were used to highlight areas of bone growth and bone resorption using a color scale and to perform a cephalometric analysis. RESULTS: Seven patients with a mean age of 61 years and computed tomographic scans on average 10.3 years apart were included. Bone resorption was consistently present (100 percent) at the pyriform aperture and the anterior wall of the maxilla. Resorption was noted at the superocentral (71 percent), inferolateral (57 percent), and superomedial (57 percent) aspects of the orbital rim. Resorption occurred earlier at the inferolateral orbital rim followed by the superomedial orbital rim in later decades of life. Paired-analysis of change in the orbital rim height and width demonstrated a mean decrease over time but was not significant. CONCLUSION: Bone remodeling in the same individual, over a period of 10 years, was characterized by resorption at the pyriform aperture; anterior wall of the maxilla; and superocentral, superomedial, and inferolateral aspects of the orbital rims.

American Society of Maxillofacial Surgeons 2006 to 2016: Another Decade of Excellence in Education and Research.

Over the past 10 years, the American Society of Maxillofacial Surgeons (ASMS) has continued to advance to meet its mission of being the premier organization to represent maxillofacial and pediatric plastic surgery in the United States. These advances are focused on education of its members, to include the American Society of Maxillofacial Surgeons basic course, the preconference symposium, the annual meeting, two basic maxillofacial courses per year, advanced maxillofacial courses, a boot camp for craniofacial fellows, a cleft course, quarterly webinars, sponsored fellowships, a visiting professorship, and the ASMS journal. In addition, the ASMS has continued to advance as the premier national organization representing maxillofacial and pediatric plastic surgery in the United States, thereby positioning the organization as a primary advocate for these surgical specialties. Outreach of the ASMS has grown over the past decade and now includes representatives to the American Society of Plastic Surgeons/Plastic Surgery Foundation, the American Board of Plastic Surgeons, the American Medical Association, and most recently a seat as a governor with the American College of Surgeons. The ASMS has also initiated an annual Summer Leadership Seminar to explore topics of relevance in a changing health care environment. The present report outlines the major initiatives of the ASMS over the past 10 years.

Management options of non-syndromic sagittal craniosynostosis.

There have been various effective surgical procedures for the treatment of non-syndromic sagittal craniosynostosis, but no definitive guidelines for management have been established. We conducted a study to elucidate the current state of practice and establish a warranted standard of care. An Internet-based study was sent to 180 pediatric neurosurgeons across the country and 102 craniofacial plastic surgeons in fourteen different countries, to collect data for primary indication for surgical management, preference for timing and choice of surgery, and pre-, peri-, and post-operative management options. The overall response rate from both groups was 32% (n=90/284). Skull deformity was the primary indication for surgical treatment in patients without signs of hydrocephalus for both neurosurgeons and craniofacial surgeons (80% and 63%, respectively). Open surgical management was most commonly performed at six months of age by neurosurgeons (46%) and also by craniofacial surgeons (35%). Open surgical approach was favored for patients younger than four months of age by neurosurgeons (50%), but endoscopic approach was favored by craniofacial surgeons (35%). When performing an open surgical intervention, most neurosurgeons preferred pi or reversed pi procedure (27%), whereas total cranial vault remodeling was the most commonly performed procedure by craniofacial surgeons (37%). The data demonstrated a discrepancy in the treatment options for non-syndromic sagittal craniosynostosis. By conducting/comparing a wide survey to collect consolidative data from both groups of pediatric neurosurgeons and craniofacial plastic surgeons, we can attempt to facilitate the establishment of standard of care.

Relevant Surgical Anatomy of Pterygomaxillary Dysjunction in Le Fort III Osteotomy.

BACKGROUND: Le Fort III osteotomy represents the foundation of surgical correction for midface hypoplasia. One serious complication of Le Fort III osteotomy is severing the internal maxillary artery or its branches during osteotome advancement for pterygomaxillary dysjunction. This study sought to characterize the relevant surgical anatomy of the infratemporal fossa and of the internal maxillary artery as it enters the pterygomaxillary fissure. METHODS: Bilateral midface dissections were performed on 15 fresh, normocephalic adult cadavers (30 hemifaces). Four superficial anatomical measurements were performed on the surface of the face, followed by 10 deep measurements of the internal maxillary artery and its branches relative to the infratemporal fossa and its surrounding bony landmarks. RESULTS: The distance from the anterosuperior aspect of the zygomatic arch to the sphenopalatine artery entering the pterygomaxillary fissure was 38.9 ± 3.2 mm. The distance from the alveolar process of the maxillary bone to the sphenopalatine artery entry into the pterygomaxillary fissure was 30.3 ± 6.4 mm. The zygomaticofrontal suture was 43.4 ± 8.5 mm from the sphenopalatine artery entry into the pterygomaxillary fissure, 58.8 ± 8.0 mm from the pterygomaxillary junction, and 74.9 ± 6.5 mm from the maxillary alveolar process. The distance from the sphenopalatine artery to the posterior superior alveolar artery was 14.4 ± 4.1 mm. Elevation of the internal maxillary artery from the lateral pterygoid plate was 5.8 ± 2.5 mm. CONCLUSION: This study characterizes the surgical anatomy of the infratemporal fossa in the context of Le Fort III osteotomies and their associated pterygomaxillary dysjunctions.

Watershed Areas in Face Transplantation.

BACKGROUND: The maxillary artery has traditionally been considered the main blood supply of the facial skeleton. However, the deep and concealed location makes the harvest of facial allografts based on this artery challenging, giving preference to the facial artery. There is growing evidence that the junction between the hard and soft palate may represent a watershed area in facial artery-based allografts. The aim of this study was to review the occurrence of partial allograft necrosis and modify the available craniofacial techniques, allowing for a reliable harvest of maxillary artery-based facial allografts. METHODS: PubMed/MEDLINE databases were searched for articles presenting allograft perfusion details and the occurrence of partial flap necrosis. Next, 25 fresh cadaver heads were used: eight allografts were harvested by means of a traditional Le Fort III approach, in six the maxillary artery was injected with latex, in three cadaver heads lead oxide gel was injected in the maxillary artery, and eight full facial allografts were harvested through a modified approach. RESULTS: Seven patients developed palatal fistulas or palatal necrosis (41 percent) when allograft was perfused through the facial artery. The traditional Le Fort III approach demonstrated consistent injury to maxillary artery/branches. The modified approach allowed for preservation of the maxillary artery under direct vision. CONCLUSIONS: Current facial transplantation outcomes indicate that facial artery-based allografts containing Le Fort III bony components can experience compromised palate perfusion. The described modified Le Fort III approach allowed safe dissection of the maxillary artery, preserving the arterial blood supply to the facial skeleton. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Skeletal Changes of an Osteomyocutaneous Facial Allograft Five Years Following Transplantation.

BACKGROUND: More than 30 face transplantations have been performed worldwide, most including part of the facial skeletal framework. In this study, the modifications of the skeletal component of a facial allograft were evaluated. METHODS: Standard head computed tomography (CT) scans, CT angiogram, and bone mineral densitometry were evaluated. Cephalometric analysis was performed. The pre and postoperative CT images were overlapped and the skeletal changes were expressed in a numeric and color-coded scale. The values of the serum calcium, phosphate, vitamin D, alkaline phosphatase, thyroid and parathyroid hormones, TSH, FHS, LH, estradiol, total protein and albumin, serum creatinine, and creatinine clearance were reviewed. RESULTS: At 5 years follow-up the patient was 51 years old, asymptomatic and presented good stability of the Le Fort III component of the allograft. Computed tomography images revealed fibrous union of all fixation sites. There was minimal bone resorption at the osteotomy sites, left infraorbital rim and left maxillary buttress, and anterior maxilla (-0.28 mm). Computed tomography angiogram showed segmental absence at the origin of the left external carotid artery, good opacification of the rest of the external carotid arteries and its branches. Bone mineral densitometry evidenced osteopenia of the spine. The patient presented mild hypoalbuminemia (3.4 g/dL) and perimenopausal hormonal levels. CONCLUSIONS: The skeletal component of the facial allograft was stable over time. Minimal bone resorption was discovered at the level of the left infraorbital rim and anterior maxilla. Transplantation of bone within the facial allograft is a viable reconstructive option.

Squamous Suture Synostosis: A Review With Emphasis on Cranial Morphology and Involvement of Other Cranial Sutures.

Squamosal suture synostosis has received little attention, potentially due to its rare nature. The authors present here a clinical report of isolated unilateral squamosal suture synostosis and a literature review, which produced 6 articles describing 33 patients of squamosal synostosis.Of the reported patients, 15 were associated with a craniofacial syndrome, 10 were nonsyndromic, and 8 were not specified. The cranial morphology varied greatly and only 1 patient was consistent with the morphology predicted by Virchow law-decreased vertical growth with compensatory ipsilateral longitudinal growth (manifesting as occipital and possibly frontal zygomatic bulging). Additional suture synostoses were observed in 36.3% of nonsyndromic and 80% of syndromic patients, suggesting that either squamosal synostosis may have an effect on other sutures, or more likely, only the most severe patients are recognized and reported.Surgical and nonsurgical interventions have found limited utility due to the subtle nature of the cranial defects and a lack of increased intracranial pressure, with a conservative follow-up course being the preferred treatment.

The Role of Conservative Management in Pneumosinus Dilatans Frontalis With Minimal Bony Deformity.

The authors describe the conservative management of 2 rare patients of uncomplicated pneumosinus dilatans of the frontal sinus with minimal bossing of the forehead. Regular nasal hygiene in combination with topical corticosteroids was administered with following complete resolution symptoms.

Malignant Pyoderma Associated with Granulomatosis with Polyangiitis (Wegener Granulomatosis) as a Unique Indication for Facial Vascularized Composite Allotransplantation: Part I.

BACKGROUND: Granulomatosis with polyangiitis (Wegener granulomatosis) is a rare disease that commonly starts in the craniofacial region and can lead to considerable facial disfigurement. Granulomas and vasculitis, however, can involve many other tissues (especially pulmonary and renal). Dermatologic and subcutaneous components can lead to malignant pyoderma. METHODS: The authors describe a unique pathologic condition, where significant Le Fort type trauma was associated with subsequent development of granulomatosis with polyangiitis and malignant pyoderma. Successive operations to excise necrotic tissue and reconstruct the defects were followed by worsening inflammation and tissue erosions. Trauma and surgery in proximity to the eye and sinuses masked the initial clinical presentation and led to delay in diagnosis and disease progression. The resultant facial disfigurement and tissue loss were substantial. RESULTS: Despite multiple confounding factors, accurate diagnosis was eventually established. This was based on persistence of sinus inflammations in the absence of infective agents, proven sterility of lung lesions, and antineutrophil cytoplasmic antibody positivity with proteinase 3 specificity. Skin lesion biopsy specimens were identified as pyoderma gangrenosum and later as malignant pyoderma. Institution of immunosuppressive therapy allowed successful control of the disease and wound healing. The resulting craniofacial destruction, however, necessitated facial vascularized composite allotransplantation. CONCLUSION: Recognition of this rare pathologic association is essential, to prevent delays in diagnosis and treatment that can lead to major craniofacial tissue loss. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Microsurgical Anatomy of the Terminal Hypoglossal Nerve Relevant for Neurostimulation in Obstructive Sleep Apnea.

BACKGROUND: Neurostimulation of the hypoglossal nerve has shown promising results in the treatment of obstructive sleep apnea. This anatomic study describes the detailed topography of the hypoglossal nerve's motor points as a premise for super-selective neurostimulation in order to optimize results and minimize the risk of complications related to main nerve trunk manipulation. METHODS: Thirty cadaveric hypoglossal nerves were dissected and characterized by number of branches, arborization pattern, and terminal branch motor point location. For each motor point, the distance to cervical midline (x axis), distance to posterior aspect of the symphysis (y axis), and depth from the plane formed by the inferior border of symphysis and anterior border of hyoid (z axis) were recorded. RESULTS: The average number of distal branches for each hypoglossal nerve was found to be 9.95 ± 2.28. The average number of branches per muscle was found to be 3.3 ± 1.5 for the hyoglossus muscle, 1.8 ± 0.9 for the geniohyoid muscle, and 5.0 ± 1.6 for the genioglossus muscle. It was found that branches to the genioglossus and geniohyoid muscles were located closer to midline (relative lengths of 0.19 ± 0.07 and 0.19 ± 0.05, respectively) while hyoglossus branches were located more laterally (0.38 ± 0.10 relative length). On the y-axis, the branches to the genioglossus were the most anterior and therefore closest to the posterior symphysis of the mandible (relative length of 0.48 ± 0.11), followed by the geniohyoid (0.66 ± 0.09), and the hyoglossus (0.76 ± 0.16). The branches to the geniohyoid were the most superficial (relative length of 0.26 ± 0.06), followed by the genioglossus (0.36 ± 0.09), and finally, the hyoglossus branches (0.47 ± 0.11), which were located deeply. CONCLUSION: A topographical map of the hypoglossal nerve terminal motor points was successfully created and could provide a framework for the optimization of the neurostimulation techniques.

Classification and Management of Metopic Craniosynostosis.

BACKGROUND: In the craniofacial surgery literature, there is a wide disparity of opinions regarding the management of nonsyndromic metopic synostosis. With the lack of level I evidence to support a particular regimen, we aimed to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. METHODS: A survey was sent to 102 craniofacial surgeons. The survey featured 2 parts: clinical scenarios and questions regarding the following: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated metopic synostosis. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. RESULTS: The total response rate was 72% (73/102) for the clinical scenarios and 63% (64/102) for the complete survey. There was a large discrepancy when classifying and managing mild metopic synostosis, with between 16% and 35% of surgeons electing to operate on a mild case. All surgeons agreed to operate on moderate and severe cases. For 95% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management was most commonly performed at 6 months (29%) of age. Open frontal orbital advancement was the most commonly performed procedure in mild (27%), moderate (77%), and severe (89%) cases. Endoscopic approaches were more likely to be used in milder cases by 19% of surgeons. CONCLUSION: Our survey demonstrates that there is a wide disparity of opinion among craniofacial surgeons regarding the diagnosis and management of mild nonsyndromic metopic synostosis. LEVEL OF EVIDENCE: Diagnostic, level 5.

The American Society of Maxillofacial Surgery Preceptorship Program: A Product of the 2013 American Society of Maxillofacial Surgery Executive Board Strategy Session and Survey.

One of the main goals of the American Society of Maxillofacial Surgery (ASMS) is to develop educational programs that increase expertise in maxillofacial surgery. We describe the outline of the new ASMS Preceptorship Program, a collective effort by ASMS members to increase access to all areas of maxillofacial surgery. Furthermore, we discuss the original survey pertinent to the development of this program, the results of the survey, and specifics regarding the structure of the program. We hope for the preceptorship program to be an excellent resource for members to mentor one another, develop intellectual and academic curiosity, provide avenues for collaboration, and further the ASMS's role in shaping maxillofacial surgery into the future.

Surgical anatomy relevant to the transpalpebral subperiosteal elevation of the midface.

BACKGROUND: The surgical anatomy of the midface relevant to its subperiosteal elevation and repositioning is assessed. OBJECTIVES: The aim of this study is to give more details on the anatomy relevant to the midface lift. METHODS: Twenty hemifacial dissections were performed. The location of the zygomatic branches of the facial nerve (ZBFN) and the terminal branches of the infraorbital nerve (ION) were dissected. The location of the masseteric and zygomatic ligaments and the elevation of midface following their release were documented. RESULTS: On average, there were 3 branches of the facial nerve innervating the zygomatic major muscle and 1.8 branches entering the muscle superior to the caudal origin of the zygomaticomaxillary (ZM) suture. The most superior ZBFN was found to lie at an average of 6.2 ± 1.6 mm cranial to ZM suture and 1.4 ± 0.4 mm superficial to the bone. The most inferior branch was at a mean of 4.8 ± 3.3 mm inferior to ZM suture. On average the ION had 5.2 branches that traveled for 10.2 mm above the periosteum before they passed into a superficial plane. Division of the zygomatico-masseteric retaining ligaments allowed for elevation of the midface by 4.8 ± 1.0 mm medially and 5.5 ± .9 mm laterally. CONCLUSIONS: Branches of the ZBFN and ION lie in close proximity to the subperiosteal plane in the midface. These branches are at risk for damage during release of the upper zygomatic ligaments and placement of the periosteal suspension sutures during midface elevation procedures.