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.

Trends in Blood Mosaicism and Clinical Phenotype Score in Patients with Beckwith-Wiedemann Syndrome Evaluated for Tongue Reduction Surgery.

INTRODUCTION: Most patients with Beckwith-Wiedemann syndrome (BWS) have macroglossia with some requiring tongue reduction surgery (TRS). This study reports correlations between levels of affected cells (mosaicism) and BWS clinical score in patients evaluated for TRS. We also show correlations of clinical score and mosaicism with obstructive sleep apnea (OSA) severity. METHODS: Blood mosaicism levels and BWS clinical score were recorded in patients with macroglossia referred to plastic surgery for evaluation. Associations among blood mosaicism, BWS clinical score, TRS, and OSA were assessed with appropriate statistics. RESULTS: Of the 225 patients included, BWS blood testing was available in 128 (56.9%). Mosaicism levels were higher in those who underwent TRS compared to those who did not (85.9 85.9 (56.5-95.9)% vs. 29.7 (2.8-73.1)%, p<0.001). BWS clinical score was also higher in those requiring TRS (9.0 (8.0-11.0) versus 7.0 (6.0-9.0), p<0.001). There was a positive correlation between clinical score and obstructive apnea-hypopnea index (r=0.320, p=0.011). Receiver operating characteristic curve analysis showed a clinical score ≥11 had 100% specificity and 36.4% sensitivity for detecting patients requiring TRS. Blood mosaicism ≥80% had 63.6% sensitivity and 83.6% specificity for predicting surgery. A combined criteria of BWS clinical score ≥11 or mosaicism ≥80% had 72.7% sensitivity and 83.6% specificity for predicting TRS. CONCLUSION: Blood mosaicism levels and higher BWS clinical scores appear associated with a greater frequency of having surgery in patients with macroglossia referred for surgical evaluation. Elevations in BWS clinical scoring are associated with increased OSA severity while increased blood mosaicism is not.

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.

Mandibular condyle volumes are associated with facial asymmetry in patients with cleft lip and palate: A retrospective cohort study.

This study compares condylar volumetric asymmetry and facial asymmetry in patients with cleft lip and/or palate (CLP) and controls. The mandibular condyle is important to facial growth, but its role in facial asymmetry for those with CLP has not been described. Condylar volumes and mandibular asymmetry were retrospectively calculated using Mimics Version 23.0 (Materialise, Leuven, Belgium) from patients with CLP undergoing computed tomography (CT) imaging and a cohort of controls. A total of 101 participants, 60 with CLP and 41 controls, had mean condylar volumetric asymmetry of 16.4 ± 17.4 % (CLP) and 6.0 ± 4.0 % (controls) (p = 0.0002). Patients with CLP who had clinically significant chin deviation (>4 mm) had more asymmetric condyles than those without significant chin deviation (p = 0.003). The chin deviated toward the smaller condyle in patients with facial asymmetry more often than in patients without facial asymmetry (81 % vs. 62 %, p = 0.033). While controls had some degree of condylar asymmetry, it tended to be milder and not associated with facial asymmetry. There is a greater degree of condylar volumetric asymmetry in patients with CLP compared to individuals in the general population. Clinically significant facial asymmetry in CLP is associated with a higher degree of condylar asymmetry, with the facial midline deviating toward the smaller condyle.

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.

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.

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.

Robotic mitral valve re-repair with removal of transcatheter mitral valve repair clips.

We demonstrate a totally endoscopic and percutaneous approach to robotic mitral valve re-repair after the failure of transcatheter edge-to-edge repair.

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.

Endoaortic balloon occlusion versus transthoracic cross-clamp for totally endoscopic robotic mitral valve surgery: a retrospective cohort study.

Endoaortic balloon occlusion (EABO) and transthoracic cross-clamping have been shown to have comparable safety profiles for aortic occlusion in minimally invasive mitral valve surgery (MIMVS). However, few studies have focused exclusively on the totally endoscopic robotic approach. We sought to compare outcomes for patients undergoing totally endoscopic robotic mitral valve surgery with aortic occlusion via EABO and transthoracic clamping after a period where EABO was unavailable required us to use the transthoracic clamp. Retrospective review identified 113 patients who underwent robotic mitral valve surgery at our facility between 2019 and 2021 with EABO (n = 71) or transthoracic clamping (n = 42). Relevant data were extracted and compared. Preoperative characteristics were similar other than a higher rate of coronary artery disease [EABO: 69.0% (49/71) vs clamp: 45.2% (19/42), p = .02] and chronic lung disease [EABO: 38.0% (27/71) vs clamp: 9.5% (4/42), p < .01] in the EABO group. Median percutaneous cardiopulmonary bypass time, operative time, and cross-clamp time were comparable. Similar rates of postoperative bleeding complications were observed, and no aortic complications were observed. One patient in each group underwent conversion to an open approach. 30-day mortality and readmission rates were comparable. EABO and transthoracic clamp were associated with similar bleeding and aortic outcomes, and mortality and readmission rates were comparable at thirty days postoperatively. Our findings support the comparable safety of the two techniques, which is well documented in studies encompassing all MIMVS techniques, within the specific context of the totally endoscopic robotic approach.

A New Frontier: No Working Port for Robotic Mitral Valve Repair.

A 61-year-old male presented via referral for mitral regurgitation and was deemed an appropriate robotic surgery candidate for complex mitral valve repair with the maze procedure and patent foramen ovale and left atrial appendage closures, using all percutaneous cannulation. We report upon the first case in the literature that describes the use of only 4 robotic ports, with no working port used.

Endoaortic Balloon Occlusion in Totally Endoscopic and Percutaneous Robotic Cardiac Surgery.

Previous studies have shown that the endoaortic balloon occlusion (EABO) can provide satisfactory aortic cross-clamping with comparable surgical outcomes to thoracic aortic clamping in the setting of minimally invasive and robotic cardiac surgery. We described our approach to EABO use in totally endoscopic and percutaneous robotic mitral valve surgery. Preoperative computed tomography angiography is required to evaluate the quality and size of the ascending aorta, identify access sites for peripheral cannulation and endoaortic balloon insertion, and screen for other vascular anomalies. Continuous bilateral upper extremity arterial pressure and cranial near-infrared spectroscopy monitoring are essential to detect obstruction of the innominate artery due to distal balloon migration. Transesophageal echocardiography is needed for continuous monitoring of balloon positioning and antegrade cardioplegia delivery. Direct fluorescent visualization of the endoaortic balloon on the robotic camera allows for verification of balloon and efficient repositioning if needed. The surgeon should assess hemodynamic and imaging information simultaneously during the balloon inflation and delivery of antegrade cardioplegia. Aortic root pressure, systemic blood pressure, and balloon catheter tension affect the position of the inflated endoaortic balloon in the ascending aorta. The surgeon should eliminate all slack in the balloon catheter and lock it into position to prevent proximal balloon migration after the completion of antegrade cardioplegia. Using scrupulous preoperative imaging assessment and continuous intraoperative monitoring, the EABO can achieve adequate cardiac arrest in totally endoscopic robotic cardiac surgery, even in patients with previous sternotomy without compromise of surgical outcomes.

Comparison of Outcomes of Scapholunate Ligament Reconstruction Techniques.

Introduction Injury to the scapholunate interosseous ligament (SLIL) is a common cause of carpal instability, yet surgical management of chronic SLIL disruption remains challenging with no optimal technique identified. Purpose The purpose of this meta-analysis was to comparatively review the available evidence of clinical, radiographic, and patient-reported outcome measures among popular techniques of SLIL reconstruction (capsulodesis, tenodesis, and bone-tissue-bone graft) to better guide management of SLIL injuries. Methods A total of 1,172 patients from 42 included studies were assessed. Standardized data extraction and analysis were performed. The mean of postoperative outcome assessments with standard deviation was used to calculate pooled standardized mean difference with 95% confidence interval. Results Visual Analog Scale (VAS) score for postoperative pain was lowest in bone-tissue-bone patients at 0.9 ( p = 0.0360). Bone-tissue-bone patients had the highest percentage of "excellent" functional outcomes at 64.5% ( p < 0.0001). Disabilities of the Arm, Shoulder, and Hand (DASH)/QuickDASH score was best in bone-tissue-bone patients at 9.7 ( p < 0.0001). Patient-Rated Wrist Evaluation (PRWE) score was best in tenodesis patients at 37.8 ( p = 0.0255). There were no statistically significant differences in grip strength, range of motion, or radiographic outcomes among the techniques. Conclusion Existing data demonstrate some benefit of bone-tissue-bone reconstruction over capsulodesis and tenodesis in pain reduction and functional improvement of the injured wrist. No statistically significant differences among radiographic outcomes could be ascertained, possibly attributable to the heterogeneity of procedures. This review provides an updated reference and highlights the need for multicenter trials with longer term follow-up and more standardized outcome measures.

Parasport: Effects on Musculoskeletal Function and Injury Patterns.

➤: Sports participation can improve gait, muscle strength, and functional abilities in patients with a wide variety of disabilities. Para athletes are also at substantial risk for injury during sports participation. ➤: Ambulant athletes with cerebral palsy are at risk for soft-tissue injuries about the knee as well as foot and ankle injuries. Wheelchair athletes are at risk for osteoporotic fractures and shoulder girdle injuries. Limb-deficient athletes are prone to low back pain and overuse injuries of the contralateral extremity. ➤: Para athletes are vulnerable to abuse during sports participation, and physicians should promptly report any possible abuse or mistreatment. ➤: Orthopaedic surgeons should understand disability and sport-specific risk factors for injury in para athletes in order to initiate early management and injury prevention protocols.

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 .

Totally Endoscopic Robot-Assisted Aortic Valve Replacement and Complex Mitral Valve Repair: The Lateral Approach.

A 76-year-old male patient was referred to our institution with moderate-to-severe aortic and mitral insufficiency. The patient underwent totally endoscopic robot-assisted aortic valve replacement and mitral valve repair. In this article, we present our lateral approach to the robotic double valve surgery.

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 .