A Novel Macroglossia Severity Index for Beckwith-Wiedemann Syndrome.

OBJECTIVE/ SUMMARY BACKGROUND DATA: We propose the first classification scheme for macroglossia in patients with Beckwith-Wiedemann Syndrome (BWS), the BWS Index of macroGlossia (BIG). METHODS: Patients with molecularly confirmed BWS seen from 2004-2023 were included to develop this system. Relationships among BIG scores, tongue reduction surgery, BWS clinical score, percent mosaicism, and polysomnography findings were examined. RESULTS: Patients were classified from BIG0 to BIG3. BIG0 includes those without macroglossia; BIG1 includes those with macroglossia not protruding beyond the teeth/alveolus; BIG2 includes those with tongue protrusion past the teeth/alveolus to the lips but that can be contained within the mouth; and BIG3 includes those with tongues that protrude beyond the teeth/alveolus and lips but that cannot be closed within the mouth. Of the 459 patients with molecularly confirmed BWS, 266 (58.0%) patients were scored. One hundred and eleven (41.7%) were BIG0, 44 (16.5%) were BIG1, 90 (33.8%) were BIG2, and 21 (7.9%) were BIG3. As scores increased, patients had an increased incidence of tongue reduction surgery (BIG0: 0% versus BIG1: 20.5% versus BIG2: 51.1% versus BIG3: 100%; r=0.66, P <0.01). Higher BIG scores were associated with elevated BWS clinical scores (r=0.68, P <0.01) and increased tissue mosaicism (r=0.50, P <0.01) as well as trends towards worse obstructive apnea-hypopnea indices (r=0.29, P =0.02) and lower SpO 2 nadirs (r=-0.29, P =0.02). CONCLUSION: In this large series of patients with Beckwith-Wiedemann Syndrome, increased BIG score correlates with undergoing tongue reduction surgery and increased phenotypic severity. Adoption of the BIG scoring system may facilitate communication and risk stratification across institutions.

Mystery of the Muenke midface: spheno-occipital synchondrosis fusion and craniofacial skeletal patterns.

PURPOSE: The spheno-occipital synchondrosis (SOS) is an important site of endochondral ossification in the cranial base that closes prematurely in Apert, Crouzon, and Pfeiffer syndromes, which contributes to varying degrees of midface hypoplasia. The facial dysmorphology of Muenke syndrome, in contrast, is less severe with low rates of midface hypoplasia. We thus evaluated the timing of SOS fusion and cephalometric landmarks in patients with Muenke syndrome compared to normal controls. METHODS: Patients with Muenke syndrome who had at least one fine-cut head computed tomography scan performed from 2000 to 2020 were retrospectively reviewed. A case-control study was performed of patient scans and age- and sex-matched control scans. SOS fusion status was evaluated as open, partially closed, or closed. RESULTS: We included 28 patients and compared 77 patient scans with 77 control scans. Kaplan-Meier analysis demonstrated an insignificantly earlier timeline of SOS fusion in Muenke syndrome (p = 0.300). Mean sella-orbitale (SO) distance was shorter (44.0 ± 6.6 vs. 47.7 ± 6.7 mm, p < 0.001) and mean sella-nasion-Frankfort horizontal (SN-FH) angle was greater (12.1° ± 3.8° vs. 10.1° ± 3.2°, p < 0.001) in the Muenke group, whereas mean sella-nasion-A point (SNA) angle was similar and normal (81.1° ± 5.7° vs. 81.4° ± 4.7°, p = 0.762). CONCLUSION: Muenke syndrome is characterized by mild and often absent midfacial hypoplasia, with the exception of slight retropositioning of the infraorbital rim. Interestingly, SOS fusion patterns in these patients are not significantly different from age- and sex-matched controls despite an increased odds of fusion. It is possible that differences in timing of SOS fusion may manifest phenotypically at the infraorbital rim rather than at the maxilla.

Early posterior vault distraction osteogenesis changes the syndromic craniosynostosis treatment paradigm: long-term outcomes of a 23-year cohort study.

BACKGROUND: Early surgical management of syndromic craniosynostosis varies widely between centers, with patients typically undergoing multiple intracranial procedures through childhood. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures. METHODS: We retrospectively analyzed the longitudinal operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up. Outcomes of patients who underwent early PVDO and conventional vault reconstruction were compared. RESULTS: Fifty-five patients met the inclusion criteria (30 PVDO and 25 conventional). Age at initial vault surgery was similar between the PVDO and conventional cohorts (7.6 vs. 8.8 months), as were baseline craniometrics (p > 0.05). Multiple fronto-orbital advancement (FOA) procedures were performed in only 1/30 (3%) PVDO-treated patient versus 12/25 (48%) conventionally-treated patients (p < 0.001). Twelve (40%) PVDO-treated patients did not undergo FOA at all. Among patients with Apert and Crouzon syndromes, fewer PVDO-treated patients required FOA prior to midface surgery (33% vs. 92%, p = 0.004) or repeat FOA (6% vs. 50%, p = 0.005) compared to conventionally-treated patients. Among patients with Muenke and Saethre-Chotzen syndromes, a similar proportion of patients required FOA in the PVDO and conventional cohorts (91% vs. 100%, p = 0.353), though no PVDO-treated patients required repeat FOA (0% vs. 44%, p = 0.018). CONCLUSIONS: Early PVDO is associated with a reduction in the high burden of both FOA and, critically, revisionary frontal procedures which are commonly seen in conventionally-treated patients with syndromic craniosynostosis.

Long-term sequelae of normocephalic pansynostosis: a rare but insidious entity.

OBJECTIVE: Patients with normocephalic pansynostosis, who have a grossly normal head shape, are often overlooked early in life and present late with elevated intracranial pressure (ICP) requiring timely cranial vault expansion. This study evaluates the long-term clinical outcomes of patients with normocephalic pansynostosis. METHODS: We retrospectively reviewed patients with a clinical and radiographic diagnosis of primary pansynostosis who underwent vault reconstruction between 2000 and 2023. Clinical and treatment course after craniofacial interventions was followed in patients with normocephaly to assess outcomes. RESULTS: Thirty-five patients with primary pansynostosis were identified, of which eight (23.5%) had normocephaly and underwent initial vault expansion at a mean age of 5.0 ± 2.4 years. All eight patients (50.0% male) presented with symptoms of elevated ICP including headaches (50.0%), nausea and vomiting (50.0%), and developmental delay (62.5%) and/or signs of elevated ICP including papilledema (75.0%) and radiologic thumbprinting on head computed tomography scan (87.5%). Three of the four normocephalic patients who had over 7 years of postoperative follow-up developed subjective headaches, vision changes, or learning and behavioral issues in the long-term despite successful vault reconstruction. CONCLUSIONS: Our longitudinal experience with this rare but insidious entity demonstrates the importance of timely intervention and frequent postoperative monitoring, which are critical to limiting long-term neurological sequelae. Multidisciplinary care by craniofacial surgery, neurosurgery, ophthalmology, and neuropsychology with follow-up into adolescence are recommended to assess for possible recurrence of elevated ICP secondary to cranio-cerebral disproportion.

Vaulting further: cranial vault expansion for craniocerebral disproportion without primary craniosynostosis.

PURPOSE: Treatment of subjects with refractory idiopathic intracranial hypertension (IIH) or shunted hydrocephalus with chronic shunt complications is challenging. What is the role for cranial vault expansion, particularly utilizing posterior vault distraction osteogenesis (PVDO), in these cases? This study assesses medium-term efficacy of cranial vault expansion in this unique patient population. METHODS: A retrospective review was conducted of patients who underwent cranial vault expansion from 2008 to 2023 at the Children's Hospital of Philadelphia. Subjects who did not have a diagnosis of primary craniosynostosis were included in the study. Demographic information, medical history, and perioperative details were collected from medical records. Primary outcomes were the rate of CSF diversion procedures and resolution of presenting signs and symptoms. Secondary outcomes were perioperative and 90-day complications and reoperation requirement. RESULTS: Among 13 included subjects, nine (69.2%) patients had a primary diagnosis of shunted hydrocephalus and 4 (30.8%) patients had IIH. Twelve (92.3%) subjects underwent posterior vault distraction osteogenesis (PVDO) and one (7.7%) underwent posterior vault remodeling (PVR). All 4 patients with IIH demonstrated symptomatic improvement following PVDO, including resolution of headaches, vomiting, and/or papilledema. Among 9 patients with shunted hydrocephalus, CSF diversion requirement decreased from 2.7 ± 1.6 procedures per year preoperatively to 1.2 ± 1.8 per year following cranial vault expansion (p = 0.030). The mean postoperative follow-up was 4.1 ± 2.1 years and four (30.8%) patients experienced complications within 90 days of surgery, including infection (n = 2), CSF leak (n = 1), and elevated ICP requiring lumbar puncture (n = 1). Four (30.8%) patients underwent repeat cranial vault expansion for recurrence of ICP-related symptoms. At most recent follow-up, 7 of 9 patients with shunted hydrocephalus demonstrated symptomatic improvement. CONCLUSION: Cranial vault expansion reduced intracranial hypertension-related symptomology as well as the rate of CSF diversion-related procedures in patients with refractory IIH and shunted hydrocephalus without craniosynostosis, and should be considered in those who have significant shunt morbidity.

Clinical Research Fellowship Fosters Mentorship, Teamwork, and Productivity: Our 11-Year Experience With a Craniofacial Research Fellowship.

INTRODUCTION: This study describes the development and explores the academic impact of a cleft and craniofacial research fellowship. MATERIALS AND METHODS: Research and career outcomes from 3 cleft and craniofacial surgeons, 14 clinical fellows, and 25 research fellows between 2010 and 2023 were examined. Academic productivity was measured by the number of peer-reviewed publications indexed in PubMed and podium presentations at national/international meetings. Residency match statistics were recorded for eligible research fellows. RESULTS: Over this 14-year period (11 with research fellows), the team produced 500 publications in 96 peer-reviewed journals, with 153 (31%) in Plastic and Reconstructive Surgery, 117 (23%) in the Journal of Craniofacial Surgery, and 32 (6%) in The Cleft Palate-Craniofacial Journal. Yearly publications increased from 15.3±7.6 per year (before fellowship) to 23.0±5.3 (with 1 fellow) to 38.3±12.9 (2 fellows) to 81.0±5.7 (3 fellows; P<0.001). There was a strong annual linear growth in publications since the beginning the research fellowship position (r=0.88, P<0.001). All (100%) clinical research fellows developed strong relationships with senior surgeons, and all who applied to plastic surgery residency matched a significantly higher success rate than the national average (P<0.05). CONCLUSION: Implementing a structured cleft and craniofacial clinical research fellowship was associated with a broad impact across all cleft and craniofacial team members, as reflected by increased academic output and high match rates among fellows. The fellowship also strengthens the talent pipeline into plastic surgery by fostering meaningful mentor/mentee relationships and provides a model that can be adopted in both surgical and nonsurgical fields.

Nasal Airway Volumes are More Asymmetric in Skeletally Mature Patients With Cleft lip and Palate Than Controls on 3-Dimensional Analysis.

BACKGROUND: This study assesses nasal airway volumes in skeletally mature patients with CLP and healthy controls and examines the relationship among nasal volumes, cleft laterality, and facial asymmetry. METHODS: Computed tomography images from patients with CLP and controls were analyzed using Mimics Version 23.0 (Materialise, Leuven, Belgium). Relationships among nasal airway volume, cleft laterality, and facial asymmetry were compared. RESULTS: The 89 patients in this study included 66 (74%) CLP and 23 (17%) controls. Nasal airway volumes in CLP were more asymmetric than controls (26.8±17.5% vs. 17.2±14.4%; P=0.015). In UCLP, the smaller nasal airway was on the cleft side 81% of the time (P<0.001). Maximum airway stenosis was on the cleft side 79% of the time (P<0.001), and maximum stenosis was on the same side as the smaller airway 89% of the time (P<0.001). There was a mild linear relationship between nasal airway asymmetry and maximum stenosis (r=0.247, P=0.023). On 3-dimensional image reconstruction, the septum often bowed convexly into the cleft-sided nasal airway with a caudal deviation towards the noncleft side. Nasal airway asymmetry was not associated with facial midline asymmetry (P>0.05). CONCLUSION: The nasal airway is more asymmetric in patients with cleft lip and palate compared with the general population, with the area of maximum stenosis usually occurring on the cleft-sided airway. In patients with unilateral cleft lip and palate, the septum often bows into the cleft side, reducing the size of that nasal airway. Nasal airway asymmetry did not correlate with facial asymmetry.

Validating the Supraorbital Notch-Nasion-Supraorbital Notch Angle as a Marker for Metopic Craniosynostosis Severity.

OBJECTIVE: Metopic craniosynostosis (MCS) can be difficult to differentiate from metopic ridge (MR) or normal frontal morphology. This study assess whether the supraorbital notch-nasion-supraorbital notch (SNS) angle can help identify MCS. METHODS: Records of 212 patients with preoperative three-dimensional computed tomography scans were examined. The SNS angles, surgeon craniofacial dysmorphology rankings, and CranioRate metopic severity scores (MSSs) were compared with the Spearman rank correlation coefficient. Receiver operating characteristic (ROC) curves with Youden J-statistic and cross-validation of regression models assessed the ability of these measures to predict surgery. RESULTS: A total of 212 patients were included, consisting of 78 MCS, 37 MR, and 97 controls. Both the mean SNS angle (MCS: 111.7 ± 10.7 degrees, MR: 126.0 ± 8.2 degrees, controls: 130.7 ± 8.8 degrees P < 0.001) and MSS (MCS: 5.9 ± 2.0, MR: 1.4 ± 1.9, controls: 0.2 ± 1.9, P < 0.001) were different among the cohorts. The mean SNS angle (111.5 ± 10.7 versus 129.1 ± 8.8, P < 0.001) was lower in those who had surgery and CranioRate score (5.9 ± 2.1 versus 0.8 ± 2.2, P < 0.001) was higher in those who underwent surgery. SNS angles were positively correlated with surgeon craniofacial dysmorphology rankings ( r = 0.41, P < 0.05) and CranioRate MSS ( r = 0.54, P < 0.05). The ROC curve requiring high sensitivity revealed an SNS angle of 124.8 degrees predicted surgery with a sensitivity of 88.7% and a specificity of 71.3%. A ROC curve using the CranioRate MCC values ≥3.19 predicted surgery with 88.7% sensitivity and 94.7% specificity. CONCLUSION: Orbital dysmorphology in patients with MCS is well captured by the supraorbital-nasion angle. Both the SNS angle and CranioRate MSS scores accurately predict surgical intervention.

Columbia-Suicide Severity Rating Scale (C-SSRS) Reveals High Rates of Suicidality in 602 Patients With Cleft and Craniofacial Conditions.

INTRODUCTION: Little is known about rates of suicidal ideation and behavior among youth with cleft lip and/or palate (CLP) and other craniofacial conditions. METHODS: Records of patients ages 6 and older who were administered the Columbia-Suicide Severity Rating Scale (C-SSRS) Lifetime Version during routine multidisciplinary cleft or craniofacial team visits between 2019 and 2023 were examined. Demographics information, C-SSRS data, and diagnoses were assessed with statistics including t tests, the Fisher exact test, and odds ratios. RESULTS: A total of 1140 C-SSRS questionnaires across 602 (433 CLP and 169 craniofacial) patients with an average age of 11.2±3.7 years were included. Eighty-four (13.6%) patients endorsed lifetime suicidal ideation, 9 (1.5%) had at least one instance of suicidal behavior, 30 (5.0%) endorsed nonsuicidal self-injury, and 2 (0.3%) engaged in self-injurious behavior. Compared with CLP, those with other craniofacial conditions had similar odds of endorsing suicidal ideation and behavior (P≥0.05). Compared with those with isolated cleft palates, CLP had greater odds of endorsing suicidal ideation and behavior, though those differences were not significant (P≥0.05). Incidence of suicidality was unchanged before, during, and after the COVID-19 pandemic (P≥0.05). Dividing patients by sex or insurance type revealed no difference in suicidality (P≥0.05). CONCLUSION: Patients with CLP and craniofacial conditions have a high incidence of suicidal ideation and behavior, though levels are similar between these groups. Suicidality in these patients was not negatively impacted by the COVID-19 pandemic. Early identification of safety risks and psychosocial challenges through regular screening can facilitate connection with appropriate clinical interventions.

Crowdsourcing the Impact of Illustration in Cleft Surgery Education.

OBJECTIVE: This study aimed to (1) assess layperson preferences for how surgical information is presented; (2) evaluate how the format of visual information relates to layperson comfort with undergoing surgery, perceptions of surgeon character traits, and beliefs about artistic skill impacting plastic surgery practice; and (3) identify sociodemographic characteristics associated with these outcomes. DESIGN: A survey was developed in which one of five standardized sets of information depicting a unilateral cleft lip repair was presented as (1) text alone, (2) quick sketches, (3) simple drawings, (4) detailed illustrations, or (5) photographs. SETTING: Online crowdsourcing platform. PARTICIPANTS: Raters aged 18 years and older from the United States. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): After viewing the surgical information, participants answered three sets of Likert scale questions. Ratings were averaged to produce three composite scores assessing (1) comfort with undergoing surgery (2) perceptions of surgeon character traits, and (3) beliefs about plastic surgery and artistry. RESULTS: Four hundred seventy-nine participants were included. Surgeon character traits score was highest among participants who viewed detailed illustrations at 4.46 ± 0.59, followed by photographs at 4.43 ± 0.54, text alone at 4.28 ± 0.59, simple drawings at 4.17 ± 0.67, and quick sketches at 4.17 ± 0.71 (p = 0.0014). Participants who viewed detailed illustrations rated surgical comfort score and plastic surgery and artistry score highest, although differences did not achieve statistical significance. CONCLUSIONS: Viewing detailed cleft lip repair illustrations was significantly associated with positive perceptions of surgeon character traits. Our data help to contextualize methods of communication and education valued by the public when seeking cleft care.

Tourniquet Use in Extremity-Based Microsurgery.

BACKGROUND: The use of tourniquets and their role in extremity-based microsurgery has not been thoroughly investigated. The purpose of this study was to investigate tourniquet use and its associated outcomes and complications. The authors hypothesize that tourniquets enhance visualization, bloodless approaches to vessel harvest, flap elevation, and anastomosis without added complications. METHODS: A retrospective chart review was completed for patients who had undergone extremity-based microsurgery with the use of a tourniquet between January 2018 and February 2022 at two large academic institutions. Demographic characteristics, initial reasons for surgery, complications, and outcomes were recorded. Patients were separated into groups based on tourniquet use during three operative segments: (1) flap elevation, (2) vessel harvest, and (3) microvascular anastomosis. An internal comparison of complication rate was performed between cases for which a tourniquet was used for one operative segment to all cases in which it was not used for the same operative segment. Univariate and multivariate statistical analyses were performed to identify statistically significant results. RESULTS: A total of 99 patients (106 surgeries) were included in this study across sites. The mean age was 41.2 years and 67.7% of the patients were male. The most common reason for microsurgical reconstruction was trauma (50.5%). The need for an additional unplanned surgery was the most common surgical complication (16%). A total of 70, 61, and 32% of procedures used a tourniquet for flap elevation, vessel harvest, and for anastomosis, respectively. Statistical analyses identified no difference in complication rates for procedures for which a tourniquet was or was not used for interventions. CONCLUSION: Based on these results, the authors state that tourniquets can be utilized for extremity-based microsurgery to enable bloodless dissection without the concern of increased complication rates.

Anatomic Relationship of Hand Intrinsic Tendons at the Metacarpal Head as It Relates to the Diagnosis of Saddle Syndrome: A Cadaveric Study.

PURPOSE: The purpose of this cadaveric study was to investigate the intrinsic anatomy surrounding the metacarpal head and the relationship between the interosseous-lumbrical junction (ILJ) and transverse metacarpal ligament (TML) as it pertains to saddle deformity-posttraumatic adhesions at the ILJ that cause impingement during intrinsic activation. METHODS: Ten fresh frozen cadaveric arms underwent dissections, identifying the intrinsic musculature within the second through fourth webspaces. The TML and ILJ, or "true tendon," were identified. A separate area of nontendinous fibrous tissue identified proximal to the ILJ was referred to as "pseudotendon." Measurements were made within each webspace to identify distances between these structures in full finger extension and intrinsic plus position to assess for changes during simulated motion. RESULTS: The true tendon to TML distance progressively decreased toward the ulnar digits. In the intrinsic plus position, the pseudotendon to TML distance was 0 mm at all webspaces for each specimen. When moving from neutral to intrinsic plus, the true tendon to TML distance decreased the most in the third and fourth webspaces compared with the second, consistent with the trend toward a smaller ILJ to TML gap in the ulnar digits. CONCLUSIONS: There is a fibrous pseudotendinous region proximal to the ILJ that abuts the TML in the intrinsic plus position, which may cause impingement when inflamed in the setting of saddle syndrome. Furthermore, a decreased ILJ to TML gap in the ulnar digits may be related to an increased predilection for saddle deformity in those areas. CLINICAL RELEVANCE: These results suggest that there is a fibrous region present proximal to the ILJ that may be implicated in the pathology of saddle deformity. Furthermore, decreased distances found between the ILJ and TML in vivo may be an explanation for increased occurrence of saddle syndrome in the third and fourth webspaces in clinical practice.

Novel Patient-Reported Outcome Measures for the Assessment of Patient Satisfaction and Health-Related Quality of Life Following Postmastectomy Breast Reconstruction.

BACKGROUND: Patient-reported outcome measures (PROMs) have become an integral part of the evaluation of reconstruction surgery outcomes. However, there are limitations in current PROMs when it comes to the assessment of well-being during inpatient stay, patient perception of health, relationship with partner, and vitality (i.e., mood and ability to work and pursue hobbies, carry out daily tasks, and sleep) following breast reconstructive surgery. The aim was to develop a novel set of measures to compare patient satisfaction and health-related quality of life following different types of postmastectomy breast reconstruction. METHODS: A novel questionnaire was created and refined through cognitive interviews with patients and expert feedback. A field test study was conducted, including patients who had undergone delayed postmastectomy breast reconstruction with implant, autologous tissue, or combination of implant and autologous tissue. Based on the results, confirmatory factor analysis and examination of reliability of the questionnaire were conducted. Results of patient responses were analyzed using Chi-square test, Kruskal-Wallis test, and Mann-Whitney U test. RESULTS: Confirmatory factor analysis showed good model fit, and Cronbach's alpha indicated high internal consistency of the questionnaire. Besides that, patients with combination reconstruction reported significantly lower vitality than patients with implant and autologous reconstruction (p = 0.048). CONCLUSIONS: This novel questionnaire expands the current knowledge base of postmastectomy breast reconstruction PROMs. Results of the field test study showed that combination reconstruction was associated with lower patient vitality than other reconstruction types. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Evaluation of the Number of Follow-up Surgical Procedures and Time Required for Delayed Breast Reconstruction by Clinical Risk Factors, Type of Oncological Therapy, and Reconstruction Approach.

BACKGROUND: Breast reconstructive surgery is often a multistage process. The aim was to understand which factors might increase the number of follow-up surgeries and the length of time required to complete the reconstruction process. METHODS: A cross-sectional analysis was performed. Clinical data of 110 patients who underwent delayed postmastectomy breast reconstruction with a 5-year examination period were reviewed retrospectively. Impact of clinical risk factors, oncological therapy, and reconstruction approach on the number of surgeries and the length of required time was analyzed. Mann-Whitney U test and Kruskal-Wallis rank sum test were used. RESULTS: In patients undergoing perioperative hormone therapy, an average of 2.9 surgeries and length of 20.2 months were required compared to 2.3 surgeries and 14.0 months in patients without hormone therapy (P = 0.003; P = 0.005). Previous abdominal surgery was associated with an increased number of breast reconstruction stages of 3.1 per patient (P = 0.056) and a longer reconstruction time of 23.0 months (P = 0.050). Patients undergoing nipple reconstruction or implant revision required an increased number of surgeries (P < 0.001; P = 0.012) and a longer reconstruction time (P = 0.002; P < 0.001). Contralateral breast surgery and flap revision were associated only with an increased number of surgeries (P < 0.001; P < 0.001). CONCLUSION: Perioperative hormone therapy was associated with a significantly higher number of surgeries and duration of time required to complete the reconstruction process. The highest increase in the number of surgeries was in patients with flap revision including anastomosis revision and necrectomy. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Biology of sinus floor augmentation with an autograft versus a bone graft substitute in a preclinical in vivo experimental model.

OBJECTIVES: Compared to autografts, bone graft substitutes are slower to consolidate. If we understood why, this might open strategies to accelerate new bone formation and thus shorten the time to implant placement. In this study, we aimed at comparing autologous bone graft with a bovine bone graft substitute in a preclinical sinus lift model. MATERIALS AND METHODS: The mouse posterior paranasal sinus served as a recipient site for grafting. Autograft from the oral cavity was compared against bone graft substitute using molecular, cellular, and histological analyses conducted on post-grafting days (PSD) 0, 9, 18, and 120. RESULTS: Either autografts or bone graft substitutes were positioned on the sinus floor and remained in situ throughout the study. At the time of grafting and until day 9, bone graft substitutes were devoid of cells and alkaline phosphatase (ALP) activity while autografts were comprised of viable cells and showed strong ALP (mineralization) activity. Consequently, new bone formed faster in autografts compared to bone graft substitutes (140.21 ± 41.21 µm vs. 41.70 ± 10.09 µm, respectively, PSD9, p = .0143). By PSD18, osteogenesis was evident in autografted and xenografted sites. Osteoclasts identified by tartrate resistant acid phosphatase attached to, but did not resorb the bone graft substitute matrix. Autograft matrix, however, underwent extensive resorption. Transgenic mice revealed that Wnt-responsive osteoprogenitor cells originated primarily from the internal periosteum of the maxillary bone, and not from the Schneiderian membrane. CONCLUSION: Autografts produce new bone sooner, but bovine bone graft substitutes eventually consolidate and then resist resorption. Enhancing osteoprogenitor cell recruitment to a bone graft substitute constitutes a viable strategy for accelerating bone formation in a sinus lift procedure.

Patient-Reported Outcomes of Three Different Types of Breast Reconstruction with Correlation to the Clinical Data 5 Years Postoperatively.

BACKGROUND: The aim of this study was to compare long-term clinical and patient-reported outcomes in terms of satisfaction and health-related quality of life (HRQoL) following postmastectomy breast reconstruction using BREAST-Q in patients undergoing implant-based reconstruction, abdominal-based autologous reconstruction, and combined reconstruction (with implant and LD flap or implant and TDAP flap). METHODS: A cross-sectional study was conducted. Patients had undergone delayed postmastectomy breast reconstruction and completed the BREAST-Q reconstruction module. The results were related to the clinical data obtained from the clinic's patient record system. A 5-year examination was included. Mean scores and standard deviations were calculated. Kruskal-Wallis test, Chi-square goodness of fit test and Chi-square test were used for the statistical analysis. RESULTS: Overall, 110 patients (n = 24 implant, n = 38 autologous, n = 48 combination) were included. Patients with autologous reconstruction reported greater postoperative satisfaction with breasts (p < 0.001), satisfaction with outcome (p < 0.001), psychosocial well-being (p = 0.001), and sexual well-being (p = 0.051). CONCLUSION: This study represents a comprehensive long-term examination of postmastectomy breast reconstruction. Autologous reconstruction patients had higher postoperative satisfaction and HRQoL than patients receiving other types of reconstruction despite having more intense oncological therapy and the highest mean number of follow-up surgical procedures. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Preliminary Outcomes of Scapholunate Ligament Augmentation with Internal Brace Technique.

Purpose: Injury to the scapholunate (SL) interosseous ligament (SLIL) is a common cause of carpal instability. Internal brace augmentation has been used in various ligament repair procedures; however, further investigation of its outcomes in hand surgery is needed. This study aimed to examine outcomes for patients who underwent SLIL repair with internal brace augmentation. Methods: Patients who underwent SLIL repair with the internal brace technique and had at least 1 year of follow-up were contacted. Available patients returned for an in-person evaluation with new radiographs and physical examination. If patients could not be contacted but had x-rays and physical examinations performed at greater than 1 year after surgery, these data were collected from their medical records. Participating patients completed the QuickDASH and Patient-Rated Wrist Evaluation surveys and rated their satisfaction with the surgery. Outcomes assessed included wrist range of motion, grip strength, scaphoid shift test, SL gap, SL angle, and radiographic evidence of radiocarpal arthritis. Results: We collected outcomes for 14 SLIL repairs among 13 patients (12 male). Mean length of the follow-up was 41 months (n = 14, 17-64). Mean QuickDASH and Patient-Rated Wrist Evaluation scores were 6.1 (0-43.2) and 9.6 (0-65), respectively. Radiographic measurements remained stable from immediate to latest follow-up, and no radiocarpal arthritic changes were noted. However, SL gap decreased from a mean of 5.33 mm (3.4-6.7) before surgery to 3.34 mm (2-4.6) at the latest follow-up, and SL angle decreased from a mean of 79.5° (67°-97°) before surgery to 67.3° (51°-85°) at the latest follow-up. All scaphoid shift tests were stable. Conclusions: Therefore, SL internal brace augmentation has favorable short-term results with improvements in pain, function, satisfaction, and carpal alignment at greater than 1 year postoperatively. This technique can be an effective option for the management of SL instability in the short term. Type of study/level of evidence: Therapeutic IV.

The Kaleidoscope of Midface Management in Apert Syndrome: A 23-Year Single-Institution Experience.

BACKGROUND: This study assesses operative trends over time and outcomes of five osteotomy techniques used to treat the Apert midface. Using clinical and photogrammetric data, we present our institution's selection rationale for correcting specific dysmorphologies of the Apert midface based on the individual phenotype. METHODS: We retrospectively reviewed patients with Apert syndrome who underwent midface distraction from 2000 to 2023. Patients were temporally divided by the year 2012 to assess differences in surgical approach. Postoperative facial dimension changes, surgical and perioperative characteristics, and complications data were compared across techniques. RESULTS: Thirty-nine patients with Apert syndrome underwent 41 midface distraction procedures: 23 (56%) in the early cohort and 18 (44%) in the late cohort. The use of segmental osteotomies for frontofacial advancement increased from 0% before 2012 to 61% from 2012 onwards (p<0.001). Monobloc with bipartition was the only technique that decreased intercanthal distance (p=0.016), and Le Fort II with zygomatic repositioning achieved the greatest median change in bilateral canthal tilt of 8.7° (IQR 1.3°, 8.7°; p=0.068). Monobloc with Le Fort II achieved the greatest median change in facial convexity of -34.9° (IQR -43.3°, -29.2°; p=0.031). Severity of complications, stratified by Clavien-Dindo grade, was greater in transcranial than subcranial procedures but similar between segmental and non-segmental osteotomies (p=0.365). CONCLUSIONS: In studying the Apert midface and attempting to resolve its varying functional and aesthetic issues, we document an evolution towards multi-piece osteotomies over time. With an appreciation for differential midface hypoplasia, segmentation is associated with more effective normalization of the Apert face.

Delayed Postnatal Synostosis without Spheno-occipital Synchondrosis Fusion: A Curious Case of Apert Syndrome.

Apert syndrome classically presents with craniosynostosis at birth, most commonly of the bilateral coronal sutures, which may lead to cephalocranial disproportion and elevated intracranial pressure, the latter of which is associated with optic atrophy, visual loss, and developmental delays. A small number of patients with syndromic craniosynostosis demonstrate open sutures at birth; however, all previously reported patients of this subtype have been reported to develop premature suture fusion in the early postnatal period and/or require cranial vault expansion for increased intracranial pressure. Here, we report on a patient with Apert syndrome who did not have closed sutures at birth, and only began to demonstrate unilateral coronal suture fusion between ages 4 and 6 years, yet neither developed phenotypic signs of craniosynostosis nor evidence of intracranial hypertension. Moreover, despite demonstrating patency of the spheno-occipital synchondrosis, the patient developed progressive midface hypoplasia, requiring a subcranial Le Fort 3 advancement with external distraction at age 9. Now at skeletal maturity, this patient has a normal cranial shape and will likely never require cranial vault surgery for functional or aesthetic concerns. We are not aware of any prior reports of a patient with Apert syndrome who did not require intracranial surgery over long-term follow-up.

The Expanded Forehead Flap for Resurfacing of Multi-unit Congenital Nevi of the Face.

The forehead flap is a timeless and robust reconstructive option for complex facial defects. In accordance with aesthetic subunit principles, it has traditionally been used to resurface defects affecting a single cervicofacial region, most commonly the nose or periorbital unit. In this article, we present three cases of congenital nevi treated with expanded forehead flap reconstruction of the nasal, periorbital, and cheek units in early childhood. This series demonstrates an approach that, while violating facial units, limits total scar burden and optimizes aesthetic and functional results. With precise staging and execution, this reconstructive technique allows for a single flap to resurface multi-unit defects in the pediatric population with excellent long-term results.