Pediatric Desmoid Tumor of the Head and Neck: A Systematic Review and Modified Framework for Management by Age Group.

Background: Unlike in adults, pediatric head and neck desmoid tumors (DTs) have greater capacity to interfere with normal anatomical development. Therefore, adequate interventions and management must be defined. We aimed to provide the most comprehensive systematic review on pediatric head and neck DTs to date, including assessment of lesion location predominance, intervention, and management, and examination of any associations between age and outcomes like surgical margin status, recurrence, and complications. Methods: A systematic literature review was conducted between January 1990 and December 2023 using PubMed, Scopus, and MEDLINE databases following the Preferred Reporting Items for Systematic Review and Meta-Analyses 2020 guidelines. We aimed to elucidate intervention and management strategies by studying various outcomes in 0-11 and 12-21 year olds. Results: The literature search yielded 44 studies, totaling 121 patients. Most head and neck DTs localized to the mandible, cranium, and neck; occurred early (P = 4.18 years); and underwent local resection with positive margins. Older and younger patients shared no difference in complication or recurrence rates. Conclusions: We found recurrence is likely to occur with positive margins. Because standard treatment of DTs is surgical resection with negative margins, if technically feasible with reasonable associated morbidity, we suggest additional resection of the tumor to achieve negative margins. We also recommend more robust follow-up data collection, not only due to desmoid's high recurrence and data paucity in patients older than 12 years, but also, to better establish best management practices.

Total Resection of Pediatric Desmoid Tumor of the Left Neck with Utilization of 3D Virtual Surgical Planning.

A 13-year-old girl with a painful left neck mass was referred to our institution due to suspicions of malignancy. The patient reported pain that accompanied her frequent neck spasms. Computed tomography revealed a large, soft-tissue mass in the left neck, deep to the sternocleidomastoid. The lesion anteriorly displaced the internal carotid artery and both displaced and crushed the internal left jugular vein. Uniquely, a three-dimensional virtual reality model combining magnetic resonance imaging and computed tomography data was used to determine the lesion's resectability and visualize which structures would be encountered or require protection while ensuring total resection. During operation, we confirmed that the mass also laterally displaced the brachial plexus, cranial nerves X and XI, and spinal nerves C3-C5 (including the phrenic) of the cervical plexus. Postsurgical pathological analysis confirmed a diagnosis of desmoid tumor, also known as aggressive fibromatosis, whereas DNA sequencing revealed a CTNNB1 mutation, a somatic genetic marker found in approximately 90% of desmoid tumor cases. When possible, the most widely used method for the treatment of desmoid tumors has been gross resection. Chemotherapy, radiotherapy, and local excision are also used in the treatment of fibromatoses when complete resection is judged infeasible. In this case, a complete surgical resection with tumor-free surgical margins was performed. A standard cervical approach with a modified posterolateral incision site was implemented to avoid a conspicuous anterior neck scar. No flap, nerve repair, or reconstruction was warranted. At 1 year of postsurgical follow-up, the patient showed minimal scarring and no signs of recurrence.

Systematic review on the application of 3D-bioprinting technology in orthoregeneration: current achievements and open challenges.

BACKGROUND: Joint degeneration and large or complex bone defects are a significant source of morbidity and diminished quality of life worldwide. There is an unmet need for a functional implant with near-native biomechanical properties. The potential for their generation using 3D bioprinting (3DBP)-based tissue engineering methods was assessed. We systematically reviewed the current state of 3DBP in orthoregeneration. METHODS: This review was performed using PubMed and Web of Science. Primary research articles reporting 3DBP of cartilage, bone, vasculature, and their osteochondral and vascular bone composites were considered. Full text English articles were analyzed. RESULTS: Over 1300 studies were retrieved, after removing duplicates, 1046 studies remained. After inclusion and exclusion criteria were applied, 114 articles were analyzed fully. Bioink material types and combinations were tallied. Cell types and testing methods were also analyzed. Nearly all papers determined the effect of 3DBP on cell survival. Bioink material physical characterization using gelation and rheology, and construct biomechanics were performed. In vitro testing methods assessed biochemistry, markers of extracellular matrix production and/or cell differentiation into respective lineages. In vivo proof-of-concept studies included full-thickness bone and joint defects as well as subcutaneous implantation in rodents followed by histological and µCT analyses to demonstrate implant growth and integration into surrounding native tissues. CONCLUSIONS: Despite its relative infancy, 3DBP is making an impact in joint and bone engineering. Several groups have demonstrated preclinical efficacy of mechanically robust constructs which integrate into articular joint defects in small animals. However, notable obstacles remain. Notably, researchers encountered pitfalls in scaling up constructs and establishing implant function and viability in long term animal models. Further, to translate from the laboratory to the clinic, standardized quality control metrics such as construct stiffness and graft integration metrics should be established with investigator consensus. While there is much work to be done, 3DBP implants have great potential to treat degenerative joint diseases and provide benefit to patients globally.

Neurologic Characterization of Craniosynostosis: Can Direct Brain Recordings Predict Language Development?

PURPOSE: Nonsyndromic craniosynostosis (NSC) is associated with language deficits. Conventional tests, such as the Bayley Scales of Infant Development (BSID), may not reflect accurate long-term cognition. Alternatively, mismatch negativity (MMN) waves recorded via electroencephalogram (EEG) measure neural responses to speech and may objectively predict language development. This study aimed to (1) correlate infant MMN to future language achievement and (2) compare MMN among subtypes of NSC. METHODS: Pre and postoperatively (mean operative age 9.5 months), NSC participants received the BSID and EEG phoneme-discrimination paradigm(80 dB,250 Hz). The MMN was the largest negative amplitude in the difference wave 80 to 300 ms after stimuli. To measure cognitive outcome, patients completed a neurodevelopmental battery (Wechsler-Abbreviated Scale of Intelligence and Wechsler-Fundamentals) at >6 years of age. RESULTS: Eleven NSC patients with EEG testing in infancy were neurocognitively tested (average age 8.0 years; 27% female; 55% sagittal, 27% metopic, 9% unicoronal, 9% sagittal/metopic). The left frontal cluster MMN strongly correlated with word-reading (r = 0.713, P = 0.031), reading-comprehension (r = 0.745, P = 0.021), and language-composites (r = 0.0771, P = 0.015). Conversely, BSID scores did not yield significant predictive value (r < 0.5, P > 0.05). Follow-up event related potentials (ERP) comparison included 39 normal control, 18 sagittal, 17 metopic, 6 unilateral-coronal infants. Preoperatively, sagittal (P = 0.003) and metopic (P = 0.003) patients had attenuated left frontal MMN compared to controls. Postoperatively, the sagittal cohort was normalized to controls while metopic patients retained attenuations (P = 0.041). CONCLUSION: ERP assessment in NSC had significantly better predictive value for future neurocognition than the BSID. Preoperatively, sagittal and metopic patients had attenuated neural response to language; postoperatively, sagittal patients had improved responses in comparison to metopic patients. Use of ERP assessment may help tailor treatment for language deficits earlier in development.

Immediate plastic surgery closure at index spinal surgery minimizes complications compared to delayed reconstruction: A retrospective cohort review.

BACKGROUND: Complex spine surgery in patients with major comorbidities leads to increased need for midline back wound reconstruction by plastic surgeons. Literature suggests that back wound reconstruction concurrent with high-risk immediate/index spine surgery may lead to fewer complication. This study aimed to validate this claim in a large cohort treated at a tertiary center. We hypothesize that immediate reconstruction may lead to fewer adverse events in comparison to delayed reconstruction. METHODS: This was a retrospective single-center review of 659 patients who underwent spinal surgery with/without reconstruction by plastic surgeons between November 2011 and December 2015. Three main cohorts were evaluated: patients who underwent spinal surgery with no reconstruction, patients with delayed reconstruction after spine surgery, and patients with immediate back wound reconstruction with index spine surgery. Demographic, clinical, and outcomes data were collected from electronic medical records. Primary endpoints were the incidence of any complications such as dehiscence, infection, seroma/hematoma, and exposed hardware. The secondary endpoint was return to the operating room and most recent follow-up. RESULTS: Forty-three patients underwent index reconstruction (follow-up 25.3 ± 12.7 months), 33 were delayed (follow-up 23.7 ± 12.5 months), and 583 had no reconstruction (follow-up 22.1 ± 15.2 months). Patients who underwent index reconstruction had more spinal levels involved than delayed reconstruction (7.8 ± 0.75 vs 5.6 ± 0.68; p = 0.03). The overall complications rate was 7.7%, most commonly wound dehiscence (2.7%), infections (0.9%), exposed hardware (2.0%), cerebrospinal fluid leaks (0.6%), and return to OR (3.8%). Patients who underwent index spinal wound reconstruction had a significantly lower complication rate (4.65%) than secondary spinal surgery patients (27.3%; p = 0.048). CONCLUSIONS: The data confirmed significantly decreased complication rates for index back wound reconstructions for high-risk patients compared to delayed spine wound reconstruction. Increased rates of wound dehiscence, exposed hardware, and revisions occurred with delayed reconstruction. Early employment of tension free, robust vascular flap closure may attribute to a decreased complication profile.

Classification of Subtypes of Crouzon Syndrome Based on the Type of Vault Suture Synostosis.

BACKGROUND: Patients with Crouzon syndrome develop various types of anatomic deformities due to different forms of craniosynostosis, yet they have similar craniofacial characteristics. However, exact homology is not evident. Different pathology then may be best treated by different forms of surgical technique. Therefore, precise classification of Crouzon syndrome, based on individual patterns of cranial suture involvement is needed. METHODS: Ninety-five computed tomography (CT) scans (Crouzon, n = 33; control, n = 62) were included in this study. All the CT scans are divided into 4 types based on premature closure of sutures: class I = coronal and lambdoidal synostosis; class II = sagittal synostosis; class III = pansynostosis; and class IV = "Others." The CT scan anatomy was measured by Materialise software. RESULTS: The class III, pansynostosis, is the most prevalent (63.6%). The classes I, III, and IV of Crouzon have significantly shortened entire anteroposterior cranial base length, with the shortest base length in class III. The external cranial measurements in class I show primarily a decreased posterior facial skeleton, while the class III presented with holistic facial skeleton reduction. Class II has the least severe craniofacial malformations, while class III had the most severe. CONCLUSION: The morphology of patients with Crouzon syndrome is not identical in both cranial base and facial characteristics, especially when they associated with different subtypes of cranial suture synostosis. The classification of Crouzon syndrome proposed in this study, summarizes the differences among each subgroup of craniosynostosis suture involvement, which, theoretically, may ultimately influence both the timing and type of surgical intervention.

Staged Abbe-Rhinoplasty Technique to Correct Bilateral Cleft Deformity.

The mature bilateral cleft nasolabial deformity exhibits prolabial and vermilion deficiency, a broad depressed nasal tip, and short columella. Many strategies attempt to correct these stigmata, with varying degrees of success. The purpose of this communication is to describe a novel sequenced approach, staging an Abbe flap and rhinoplasty, with morphometric analysis of results. CLINICAL QUESTION/LEVEL OF EVIDENCE:: Therapeutic, IV.

SMAD6 Genotype Predicts Neurodevelopment in Nonsyndromic Craniosynostosis.

BACKGROUND: De novo or rare transmitted mutations in the SMAD6 gene affect 7 percent of midline nonsyndromic synostosis patients. This study aimed to determine the neurocognitive sequelae of SMAD6 synostosis. METHODS: Nonsyndromic synostosis patients 6 years or older with SMAD6 mutations and non-SMAD6 nonsyndromic synostosis controls were recruited. All patients completed a double-blinded neurodevelopmental battery (i.e., Wechsler Fundamentals, Wechsler Abbreviated Scale of Intelligence, Beery-Buktenica Developmental test), and parents/guardians completed behavioral surveys (Behavior Rating Inventory of Executive Function and Behavior Rating System for Children). RESULTS: Twenty-eight patients participated: 10 known SMAD6 patients (average age, 10 years; 1 female; eight metopic and two sagittal; nine treated with cranial vault remodeling and one treated with strip craniectomy) and 18 non-SMAD6 controls (age, 9.5 years; three female; 12 metopic and six sagittal; 17 treated with cranial vault remodeling and one treated with strip craniectomy). There were no differences between any demographics. Testing age, surgical age, parental education, and household income correlated with cognition (p < 0.05). After controlling for these factors, SMAD6 patients performed worse on numerical operations (p = 0.046), performance intelligence quotient (p = 0.018), full-scale intelligence quotient (p = 0.010), and motor coordination (p = 0.043) compared to age/race/gender/synostosis/operation-matched controls. On behavioral surveys, SMAD6 patients scored worse on 14 assessments, including aggression, communication, and behavior. CONCLUSIONS: This prospective double-blinded study revealed that neuropsychiatric development of nonsyndromic synostosis may be under genetic control. SMAD6 mutations led to poorer mathematics, performance intelligence quotient, full-scale intelligence quotient, and motor coordination, even after controlling for exogenous factors. Genetic testing may be critical for advocating early adjunctive neurodevelopmental therapy. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Control of Nasal Tip Position: Quantitative Assessment of Columellar Strut versus Caudal Septal Extension Graft.

BACKGROUND: Control and maintenance of nasal tip position are critical in rhinoplasty. Two frequent methods of exerting tip control are columellar strut and caudal septal extension graft. However, no quantitative data exist comparing the two methods over time. The purpose of this study was to analyze maintenance of tip projection and rotation following either columellar strut or septal extension graft. METHODS: A retrospective cohort study of patients undergoing rhinoplasty was reviewed. Three-dimensional photogrammetric evaluation of patients with either columellar strut or septal extension graft to increase tip projection was performed. Anthropometric points were analyzed in a blinded fashion. Outcome variables were tip projection, nasal length, the Goode ratio, and tip rotation. Results were stratified based on technique and compared statistically. RESULTS: One hundred six patients were included. Overall, 66 percent were female, with an average age of 34.5 years. A columellar strut was used in 42 percent of cases (n = 45), and a septal extension graft was used in 57 percent (n = 61). Analysis showed greater maintenance of tip rotation over time with the septal extension graft compared with the columellar strut [-1.01 percent change (p = not significant versus -5.08 percent change (p = 0.009)]. Tip projection, nasal length, and the Goode ratio decreased over time for both groups, but the differences were not statistically different. CONCLUSIONS: Nasal tip projection and rotation appear to decrease from the immediate postoperative position. In this study, both septal extension graft and columellar strut exhibit similar changes in tip projection with time; however, septal extension graft is better able to preserve tip rotation compared with the columellar strut. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Comparing Outcomes in Orthognathic Surgery Using Clear Aligners Versus Conventional Fixed Appliances.

PURPOSE: Orthognathic surgery for dentofacial deformities is typically preceded and followed by orthodontic treatment. Traditionally, orthodontic hardware is secured to the dentition to allow dental movement and stabilization. Clear-aligner therapy (eg, Invisalign) provides an aesthetic alternative, consisting of a series of transparent trays. Its use has not been described in complex triple-jaw orthognathic surgery. The purpose of this study is to evaluate perioperative outcomes and 3-dimensionally quantify postoperative edema in Invisalign patients undergoing triple-jaw orthognathic procedures, comparing this to patients treated with conventional fixed appliances. The surgical approach to patients with clear-aligners is also outlined. METHODS: The authors conducted a retrospective chart review and 3-dimensional morphometric study of Invisalign patients undergoing triple-jaw surgery (LeFort I osteotomy, bilateral sagittal split osteotomy, and genioplasty). An identical assessment of demographically matched patients treated with conventional fixed appliances was performed and compared with the Invisalign group. RESULTS: Thirty-three patients, with a mean age of 19.99 years, were included: 13 with Invisalign and 20 with conventional fixed appliances. No significant difference was observed in operating time, concurrent extraction of teeth, fat grafting, duration of hospital stay, diet advancement, and use of narcotic analgesics between the 2 groups. Nine patients had sufficient 3-dimensional images for volumetric analysis (4 with Invisalign and 5 with conventional fixed appliances). Postoperative edema was not significantly different (P = 0.712) when comparing conventional fixed appliances (44.29 ±â€Š23.16 cm) to Invisalign (37.36 ±â€Š31.19 cm). CONCLUSION: The present study demonstrates that complex multiple-jaw orthognathic procedures can be successfully performed in Invisalign patients. Perioperative and short-term clinical outcomes are not compromised.

Conformity of the Actual to the Planned Result in Orthognathic Surgery.

BACKGROUND: Virtual surgical planning has facilitated preoperative planning, splint accuracy, and intraoperative efficiency in orthognathic surgery. The translation of the virtual surgical plan to the actual result has not been adequately examined. The authors examined the conformity of the virtual surgical plan to the postoperative result. They hypothesize that the greatest conformity exists in the anteroposterior dimensions. METHODS: The authors examined patients who underwent Le Fort I maxillary advancement, bilateral sagittal split osteotomy, and genioplasty. The preoperative virtual surgical planning file and postoperative cone beam computed tomographic scan were registered in Mimics using unchanged landmarks. The conformity to the virtual surgical plan was quantified using linear and angular measurements between bone surface landmarks. Results were compared using t tests, with p < 0.05 considered statistically significant RESULTS:: One hundred patients who underwent Le Fort I maxillary advancement, bilateral sagittal split osteotomy, and genioplasty were included. Three-dimensional analysis showed significant differences between the plan and outcome for the following landmarks: A point (y, p = 0.04; z, p = 0.04), B point (y, p = 0.02; z, p = 0.02), pogonion (y, p = 0.04), menton (x, p = 0.02; y, p = 0.01; z, p = 0.03), and anterior nasal spine (x, p = 0.04; y, p = 0.04; z, p = 0.01). Angular measurements sella-nasion-A point, sella-nasion-B point, and A point-nasion-B point were not statistically different. CONCLUSIONS: There is a high degree of conformity comparing the orthognathic virtual surgical plan to the actual postoperative result. However, some incongruency is seen vertically (maxilla) and sagittally (mandible, chin). Departures of the actual position compared with the plan could be the result of condylar position changes, osteotomy locations, aesthetic intraoperative decisions, and/or play in the system.

Classification of Subtypes of Apert Syndrome, Based on the Type of Vault Suture Synostosis.

BACKGROUND: Apert syndrome patients are different in clinical pathology, including obstructive sleep apnea, cleft palate, and mental deficiency. These functional deficiencies may be due to anatomic deformities, which may be caused by different forms of associated suture fusion. Therefore, a classification system of Apert syndrome based on the type of craniosynostosis pattern might be helpful in determining treatment choices. METHODS: CT scans of 31 unoperated Apert syndrome and 51 controls were included and subgrouped as: class I. Bilateral coronal synostosis; class II. Pansynostosis; and class III. Perpendicular combination synostosis: a. unilateral coronal and metopic synostosis; b. sagittal with bilateral/unilateral lambdoid synostosis; and c. others. RESULTS: Class I is the most common (55%) subtype. The cranial base angulation of class I was normal; however, the cranial base angulation on the cranium side of the skull in class II increased 12.16 degrees (P = 0.006), whereas the facial side cranial base angle of class IIIa decreased 4.31 degrees (P = 0.035) over time. The external cranial base linear measurements of class I showed more evident reduction in anterior craniofacial structures than posterior, whereas other subtypes developed more severe shortening in the posterior aspects. CONCLUSIONS: Bicoronal synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined pansynostosis patients have flatter cranial base, whereas the combined unilateral coronal synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.

Association of Regional Cranial Base Deformity and Ultimate Structure in Crouzon Syndrome.

BACKGROUND: Considerable craniofacial features of Crouzon syndrome are attributed to the dysmorphology of the cranial base. As cephalometric studies have focused mainly on the facial deformity, rather than the cranial base, the underlying cause of deformity is not as well understood. Therefore, the authors compared the cranial base development of Crouzon syndrome to controls to trace the timing of deformity in the cranial base and face, to analyze their temporal correlation. METHODS: Ninety computed tomographic scans were included (Crouzon, n = 36; controls, n = 54) and divided into five age subgroups. Craniofacial cephalometric measurements were analyzed by Materialise software. RESULTS: The overall cranial base length in Crouzon syndrome compared with controls decreased 8 percent (p = 0.014) on average. The posterior cranial fossa shortening accounted for most of this reduction. The cranial base displaced with the distances from basion, sella, and ethmosphenoid to posterior nasal spine shortened by 21%, 18%, and 16%, respectively (all p < 0.01) during life. Although the cranial base angle on intracranial surface remains normal, the angles on facial surface narrowed were reduced. CONCLUSIONS: The cranial base deformity of Crouzon syndrome consists of the whole skull base and particularly anterior skull base shortening early, leading to a compensatory widened anterior skull base. However, when this widening did not compensate fully for the rapid enlargement of the brain, the posterior skull base displaced inferiorly and became kyphotic. The cranial base deformity develops sequentially anterior to posterior in a probable cascade of influence pattern. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Mandibular Spatial Reorientation and Morphological Alteration of Crouzon and Apert Syndrome.

BACKGROUND: From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. METHODS: One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analysis. RESULTS: In Crouzon syndrome, the angle between the cranial base and gnathion was increased prior to 6 months of age by 10.29 degrees (P < 0.001) and by adulthood to 11.95 degrees (P = 0.003) compared with normal. After 6 months of age, the distance between bilateral mandibular condylions (COR-COL) was narrower by 15% (P < 0.001) in Crouzon syndrome compared with control subjects. Before 6 months of age, Apert COR-COL decreased 16% (P < 0.001) compared with control subjects and 13% (P = 0.006) narrower than Crouzon. During 2 to 6 years of age, Apert mandibular ramus height caught up to, and became longer than, Crouzon by 12% (P = 0.011). The nasion-sella-articulare angle of the Apert skull was 5.04 degrees (P < 0.001) less than Crouzon overall. CONCLUSIONS: In Crouzon syndrome, the changes of the spatial relationship of the mandible to the cranial base develop earlier than the mandibular shape deformity, whereas in Apert syndrome, the spatial and morphological changes are synchronous. The morphological changes of the mandible are disproportional in 3 directions, initially significant shortening of the mandibular width and length, and, subsequently, reduced height. Crouzon has more shortening in mandibular height compared with Apert, reflecting the more shortened posterior cranial base length. The narrowed angle between the mandible and the posterior cranial base in Apert skulls is consistent with the more limited nasopharyngeal and oropharyngeal airway space.

Syndromic Craniosynostosis.

Management strategies for syndromic craniosynostosis patients require multidisciplinary subspecialty teams to provide optimal care for complex reconstructive approaches. The most common craniosynostosis syndromes include Apert (FGFR2), Crouzon (FGFR2), Muenke (FGFR3), Pfeiffer (FGFR1 and FGFR2), and Saethre-Chotzen (TWIST). Bicoronal craniosynostosis (turribrachycephaly) is most commonly associated with syndromic craniosynostosis. Disease presentation varies from mild sutural involvement to severe pansynostoses, with a spectrum of extracraniofacial dysmorphic manifestations. Understanding the multifaceted syndromic presentations while appreciating the panoply of variable presentations is central to delivering necessary individualized care. Cranial vault remodeling aims to relieve restriction of cranial development and elevated intracranial pressure and restore normal morphology.

Spatial and temporal changes of midface in Apert's syndrome.

The dysplastic maxilla and retracted zygoma characterize Apert's syndrome. The relationship between the cranial base and facial development is believed to be influential and substantial. The purpose of this study is to explore the temporal relationships of maldevelopment of these structures to identify potential influence patterns. Fifty-four CT scans (unoperated Apert's, n = 18; control, n = 36) were included and divided into three age subgroups (0-6 months, 6 months-2 years, and 2-6 years). All measurements were analyzed by Materialize software. Cephalometrics relating to midface and cranial base were collected. In anteroposterior direction, prior to 6 months, the zygoma was markedly retruded by 12% in Apert's, followed by persistent retrusive shape into adulthood, averaging 17% shorter compared to controls. The maxillary anteroposterior dimension was 22% shorter than normal before 6 months of age, thereafter, it maintained at least an 18% deficiency into adulthood. In the horizontal direction, the transverse width of the zygoma increased 39% between 6 months and 2 years of age, and it was 14% wider on average overall into adulthood. The maxilla had normal growth in transverse and vertical directions. The zygoma is the most severely deformed anatomic facial structure in early infancy, in both positional relation and geometric shape in Apert's syndrome. This may develop as a 'bridge', influencing the structure, transmitting malformation stresses, caused by premature fused coronal and peri-zygomatic sutures, into facial structures and the maxilla.

Effect of Fat Grafting on Postoperative Edema After Orthognathic Surgery.

BACKGROUND: Autologous fat grafting concurrent with orthognathic surgery is a powerful adjunctive aesthetic tool, and has been shown to have anti-inflammatory properties in prior studies. The purpose of this study is to evaluate the effect of fat grafting on postoperative edema following orthognathic surgery, and also consider the impact of age and BMI. METHODS: A retrospective cohort study was performed. Three-dimensional photos (Canfield, Fairfield, NJ) from a series of postprocedure time-points were analyzed using Geomagic Studio 2013 (3D Systems, Morrisville, NC). An unstructured covariance linear mixed model was created to analyze the effect and extent of the effect of age, BMI, and fat using SPSS Statistics 24.0 (IBM, Armonk, NY). RESULTS: One hundred thirty postoperative three-dimensional pictures were analyzed, from 31 patients. The linear mixed model demonstrates that the fat injected (P<0.001), age (P=0.001), and BMI (P<0.001) are significant factors in the postoperative volume. Age and BMI increase postoperative edema by 3.63 cm per year and 14.60 cm per kg/m, respectively. Fat injected reduces postoperative edema by 8.72 cm per 1 cc injected. CONCLUSION: Increasing age and BMI lead to greater postoperative edema (3.88 cm per year and 14.60 cm per kg/m, respectively). Fat grafting concurrent with orthognathic surgery reduces postoperative edema by 8.72 cm per 1 cc injected, and hastens return to steady state. This impact is more profound in patients with a greater age and BMI.

Temporal Evaluation of Craniofacial Relationships in Apert Syndrome.

Complicated craniofacial malformations interfacing with multiple intracellular regulatory mechanisms, lead to ambiguous growth patterns in Apert syndrome. This study aims to explore the chronology and pathogenesis of the development of craniofacial anatomic relationships and to verify the positional correlates between skull and facial structures in Apert syndrome. Fifty-four computed tomography scans (Apert, n = 18; control, n = 36) were included and divided into 3 age subgroups. Craniofacial 3-dimensional cephalometries were analyzed by Materialize software. The angle between sella-nasion plane and maxillary plane widens 7.74° (P = 0.003) prior to 6 months of age; thereafter, this widening increases by 10.36° (P < 0.001) in 6 months to 2 years of age, and remains increased by 8.9° (P = 0.046) throughout childhood. The angle between Frankfort horizontal plane and maxillary plane widens 5.17° (P = 0.022) before 6 months. Angles SNA, SNB, and ANB showed decreases, averaging 12.23° (P < 0.001), 5.19° (P = 0.004), and 6.72° (P = 0.001), respectively. The linear measurements showed synchronicity and continuing deformity into adulthood. Between 6 months to 2 years of age, the distance from sella to nasion (S-N), anterior nasal spine (S-ANS), and posterior nasal spine (S-PNS) decreased 8% (P = 0.006), 16% (P < 0.001), and 19% (P = 0.002), respectively, and remained shortened into adulthood. The angulation changes occur earlier in development than linear distance reduction in Apert syndrome patients compared with controls. Angular adjustments were not sufficient to maintain normal cranial base length. Facial deformity of Apert syndrome temporally begins with the midface, and affects orbit and mandible later in life.