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.

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.

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.

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.

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.

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.

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.

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.

Pediatric vascularized composite allotransplantation.

BACKGROUND: Vascularized composite allotransplantation (VCA) has experienced a growing acceptance, which has led to a debate centered on extending the indications of the procedure to include pediatric patients. The aim of this article was to discuss such indications based on the evidence in pediatric solid organ transplantation, reconstructive surgery in children, and VCA in adult patients. METHODS: Papers published on the outcomes of pediatric solid organ transplantation, growth after replantation of extremities, vascularized autologous tissue transfer, craniofacial surgery, orthognathic procedures, facial fractures, and outcomes after repair of peripheral nerves in children were reviewed. RESULTS: Although the outcomes of solid organ transplantation in children have improved, the transplanted organs continue to have a limited lifespan. Long-term immunosuppressive therapy exposes the patients to an increased lifetime risk of infections, diabetes, hypertension, dyslipidemia, cardiovascular disease, and malignancy. Growth impairment and learning disabilities are other relevant drawbacks, which affect the pediatric recipients. Nonadherence to medication is a common cause of graft dysfunction and loss among the adolescent transplant recipients. Rejection episodes, hospitalizations, and medication adverse effects contribute negatively to the quality of life of the patients. Although normal growth after limb transplantation could be expected, pediatric facial transplant recipients may present with arrest of growth of transplanted midfacial skeleton. CONCLUSIONS: Considering the non-life-threatening nature of the conditions that lead to eligibility for VCA, it is suggested that it is premature to extend the indications of VCA to include pediatric patients under the currently available immunosuppressive protocols.

The temporoparietal adipofascial flap for the correction of recurrent idiopathic enophthalmos.

Enophthalmos, the posterior displacement of a normal-size ocular globe relative to the orbital cavity, is usually repaired using autogenous grafts or alloplastic materials. We present the case of a 40-year-old man with bilateral idiopathic enophthalmos whose symptoms recurred 8 years after initial successful surgical repair. We describe the successful and safe use of a bilateral temporoparietal adipofascial flap in the treatment of recurrent idiopathic enophthalmos.

Epitheloid hemangioma treated with naproxen sodium.

UNLABELLED: Epitheloid hemangioma (EH) is a vascular tumor characterized by an epithelioid endothelial cell. Predominantly affecting the head and neck, fewer than 30 cases involving the scrotum have been published. As this represents an extremely rare entity, a multitude of anecdotal treatment modalities have been utilized including systemic/intralesional steroid therapy, radiotherapy, and chemical therapy. However, surgical excision remains the most widely accepted treatment option.We present a case of EH of the scrotum in a 14-year-old male patient that regressed after treatment with naproxen sodium. To the best of our knowledge, this represents the first reported case of scrotal EH regression following treatment with naproxen sodium. LEVEL OF EVIDENCE: V.

Squamosal suture craniosynostosis in Muenke syndrome.

Muenke syndrome caused by point mutation (C749G) in the FGFR3 gene affects 1 in 30,000 newborns and accounts for 25% to 30% of genetic causes of craniosynostosis. Anomalies in patients with Muenke syndrome include craniosynostosis, hypertelorism, sensorineural hearing loss, and developmental delay, among others. Most craniosynostoses in patients with Muenke syndrome involve bicoronal suture fusion. This article reports, for the first time, the existence of squamosal craniosynostosis in patients with Muenke syndrome.

Management of sagittal synostosis: a solution to equipoise.

BACKGROUND: In the craniofacial surgery literature, there is a wide disparity of opinions regarding the appropriate treatment of nonsyndromic sagittal synostosis. With the lack of level 1 evidence to support a particular regimen, our study aims to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. METHODS: An internet-based survey was sent to 102 craniofacial surgeons in 14 countries on 4 continents. Data were collected regarding the following parameters: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated sagittal synostosis with normative intracranial pressure values. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. RESULTS: After 2 mailings, the response rate was 58% (59/102). For 63% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management of sagittal craniosynostosis was most commonly performed at 6 months (35%) of age. Total cranial vault remodeling was the most commonly performed procedure (37%). Thirty-five percent of craniofacial surgeons chose an endoscopic surgical approach for patients presenting at younger than 4 months. Only 10% of craniofacial surgeons selected spring-assisted strip craniectomy. Seventy-one percent of polled surgeons performed computed tomographic scans of the skull in all cases, irrespective of presentation. CONCLUSION: Our survey demonstrates that there exists a wide disparity of opinion regarding diagnosis and treatment of nonsyndromic sagittal synostosis. When current practice is compared to findings in the literature, significant discrepancies exist.

Priorities for the education of members of the American Society of Maxillofacial Surgery.

We present the complete results of our 2013 survey of the members of the American Society of Maxillofacial Surgery (ASMS). We surveyed all 799 members of the Society from around the world regarding educational themes and modalities they wish to add to future ASMS educational programs. We also asked our members about surgical modalities and care paths in which they have the most expertise. The objective of the survey was to provide data that can be used to improve the education and training of the ASMS members. The results suggest that some widely taught topics perhaps should be taught less, whereas coverage of other topics should be increased.

The expansile properties of kryptonite relating to cranioplasty.

BACKGROUND AND PURPOSE: Since 2009, a synthetic material known as kryptonite has become increasingly utilized during cranioplasty to repair bony defects. It provides bone-like strength and adhesive properties that make it a suitable replacement for bone. However, applications have been observed in the immediate postoperative period that demonstrates an increase in its original volume, giving rise to irregularities in the cranial surface. METHODS: Ten kryptonite samples were reconstituted and allowed to polymerize according to the manufacturer's directions. The kryptonite samples were molded into a cylindrical shape, and they were immersed in 10 graduated cylinders filled with normal saline. Measurements of the rise in saline relative to baseline were taken at 0, 10, 20, 30, 40, 50, and 60 minutes, and then hourly through 5 hours, with the final measurement recorded at 24 hours. RESULTS: The mean expansion of kryptonite was approximately 49% with an SD of 22%. The bulk of the expansion occurred within the first 2 hours, after which the rate tended to plateau for the remaining 22 hours. CONCLUSIONS: Kryptonite has been touted as an excellent alternative for repairing contour abnormalities manifested in cranioplasty. Given the unpredictability of its expansile properties, the surgeon must take this variability into careful consideration when planning the desired surgical outcome. The results of the current study were communicated with the manufacture. Immediately thereafter, the manufacturer withdrew the product from the US market and is no longer Food and Drug Administration approved for cranioplasty.

Opinion leaders and evidence-based medicine in craniofacial surgery.

BACKGROUND: In health care, it is widely known that evidence-based medicine (EBM) has a significant impact on clinical practice, and opinion leaders can enhance the clinician's application of EBM in various disciplines. In this article, we examine the existence and impact of opinion leaders in craniofacial surgery as well as barriers to evidence-based practice. METHODS: We compiled the answers of an Internet questionnaire, which was sent to 102 craniofacial surgeons. RESULTS: Our results demonstrate that opinion leaders most definitely can be identified in craniofacial surgery. They are tightly connected to their field's social network and promote EBM. In this survey, 44% of craniofacial surgeons reported that their greatest obstacle to clinical decision making in the management of nonsyndromic synostosis was lack of surgical consensus. In addition, craniofacial surgeons stated that EBM and opinion leaders are the most influential factors that caused them to change their management of craniosynostosis. CONCLUSIONS: We expect that the use of opinion leaders can further enhance the uptake of EBM in craniofacial surgery.

Scalp metastasis from esophageal adenocarcinoma: comparative histopathology dictates surgical approach.

Cutaneous metastasis of esophageal cancer, in particular esophageal adenocarcinoma, is rare and metastasis to the scalp is extremely rare. We describe such a case that was originally diagnosed as an adnexal carcinoma. A 77-year-old male with a history of esophageal adenocarcinoma status after esophagectomy at our institution 4.5 years prior, presented to our plastic surgery clinic with a 2-month history of 2 temporoparietal scalp lesions. He was referred to our clinic by a community dermatologist who had performed a shave biopsy of the lesions. The clinical diagnosis was adnexal cyst. The history of esophageal carcinoma was not provided to the pathologist. The dermatopathology report came back as malignant adnexal neoplasm and considerations included apocrine carcinoma. We reexamined the pathologist's slides from the outside facility, comparing them to the histopathology from his esophagectomy. Histopathologic changes were identical. Thus, our surgical and postoperative approach changed significantly. Clinical suspicion should be high for cutaneous metastases in patients with a history of solid organ cancers. It is important for clinicians to illicit a history of malignancy. A biopsy should be performed on any suspicious lesions, and clinical data along with histopathology of the prior cancer resection(s) should be provided to the pathologist for comparison. Diagnosis of the suspicious lesion should be made before definitive excision, as this may change the approach, with the potential for postoperative chemotherapy and radiation. The definitive operative approach consists of surgical debulking with the evidence of negative margins. On the scalp, we feel that 5-mm margins are appropriate to obtain clear margins. One should appreciate the subdermal extent of metastases and adjust the margins accordingly. We recommend excising the galea with the skin as an en bloc resection. This will both assure clear deep margins of resection and assist in a tension-free closure of the scalp.

Fat embolism after liposuction in Klippel-Trenaunay syndrome.

Fat embolism syndrome (FES) is a rare but potentially fatal postoperative complication from liposuction. We present the case of a 24-year-old woman with Klippel-Trenaunay syndrome who developed FES as a complication of lower extremity liposuction. There may be an increased risk of FES in patients with vascular malformations undergoing liposuction.

Local opinion leaders: effects on professional practice and health care outcomes.

BACKGROUND: Clinical practice is not always evidence-based and, therefore, may not optimise patient outcomes. Opinion leaders disseminating and implementing 'best evidence' is one method that holds promise as a strategy to bridge evidence-practice gaps. OBJECTIVES: To assess the effectiveness of the use of local opinion leaders in improving professional practice and patient outcomes. SEARCH STRATEGY: We searched Cochrane EPOC Group Trials Register, the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, HMIC, Science Citation Index, Social Science Citation Index, ISI Conference Proceedings and World Cat Dissertations up to 5 May 2009. In addition, we searched reference lists of included articles. SELECTION CRITERIA: Studies eligible for inclusion were randomised controlled trials investigating the effectiveness of using opinion leaders to disseminate evidence-based practice and reporting objective measures of professional performance and/or health outcomes. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data from each study and assessed its risk of bias. For each trial, we calculated the median risk difference (RD) for compliance with desired practice, adjusting for baseline where data were available. We reported the median adjusted RD for each of the main comparisons. MAIN RESULTS: We included 18 studies involving more than 296 hospitals and 318 PCPs. Fifteen studies (18 comparisons) contributed to the calculations of the median adjusted RD for the main comparisons. The effects of interventions varied across the 63 outcomes from 15% decrease in compliance to 72% increase in compliance with desired practice. The median adjusted RD for the main comparisons were: i) Opinion leaders compared to no intervention, +0.09; ii) Opinion leaders alone compared to a single intervention, +0.14; iii) Opinion leaders with one or more additional intervention(s) compared to the one or more additional intervention(s), +0.10; iv) Opinion leaders as part of multiple interventions compared to no intervention, +0.10. Overall, across all 18 studies the median adjusted RD was +0.12 representing a 12% absolute increase in compliance in the intervention group. AUTHORS' CONCLUSIONS: Opinion leaders alone or in combination with other interventions may successfully promote evidence-based practice, but effectiveness varies both within and between studies. These results are based on heterogeneous studies differing in terms of type of intervention, setting, and outcomes measured. In most of the studies the role of the opinion leader was not clearly described, and it is therefore not possible to say what the best way is to optimise the effectiveness of opinion leaders.