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.

Psychosocial Status and Self-Perception in Patients with Cleft Lip and/or Palate.

OBJECTIVE: To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs). DESIGN: Cross-sectional retrospective chart review. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period. MAIN OUTCOME MEASURES: CLEFT-Q. RESULTS: Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales. CONCLUSIONS: Patient perception of cleft outcomes is linked to psychosocial factors.

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.

Autologous Cranioplasty Using a Dental SafeScraper Device.

SUMMARY: Conventional methods to reconstruct cortical bone defects introduced by pediatric cranial vault remodeling (CVR) procedures have shortcomings. Use of bone burr shavings as graft material leads to variable ossification, and harvesting split-thickness cortical grafts is time-intensive and often not possible in thin infant calvaria. Since 2013, the authors' team has used the SafeScraper, originally developed as a dental instrument, to harvest cortical and cancellous bone grafts during CVR. The authors assessed the effectiveness of this technique by analyzing postoperative ossification using computed tomography scans of 52 patients, comparing cohorts treated with the SafeScraper versus those who received conventional methods of cranioplasty during fronto-orbital advancement. The SafeScraper cohort had a greater reduction in total surface area of all defects (-83.1% ± 14.9 versus -68.9% ± 29.8; P = 0.034), demonstrating a greater and more consistent degree of cranial defect ossification compared with conventional methods of cranioplasty, suggesting potential adaptability of this tool. This is the first study that describes the technique and efficacy of the SafeScraper in reducing cranial defects in CVR. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Management of Human Bites in a Rural Kenyan Setting: A Plastic Surgeon's 8-Year Experience.

INTRODUCTION: The treatment of human bites is a common issue facing healthcare practitioners in the developing countries of Sub-Saharan Africa where it has been noted as a growing public health concern. Generally, the desired outcomes from surgical management are healing, function, and aesthetics. We share our 8-year experience at Kapsowar Hospital in Kenya with the presentation, management, and outcome of human bites. We are uniquely situated to do so given the prevalence of human biting in our community and the full-time presence of a plastic surgeon at our institution. To the best of our knowledge, this study is the first to present human bite surgical management by a plastic surgeon certified by the American Board of Surgery working full-time on the African continent. METHODS: A retrospective chart review was conducted on all patients who presented to the operating theater at Kapsowar Hospital with a human bite between 2013-2021. After identification of eligible patients, charts were reviewed assessing parameters including age, gender, timing from injury to presentation at hospital, localization of bite, severity of facial wounds using Lackmann's classification, surgical procedure(s), postoperative complications, surgical revisions, antibiotics administered, and circumstance of incident. Data were analyzed using SPSS 29.0.0.0. RESULTS: Forty-four patients were identified. Seventeen (38.6%) were female and 27 (61.4%) were male. Thirty-one patients had facial bites and 13 patients had bites to other parts of the body. Of facial bites, 70.3% resulted in amputation of the affected part while 29.7% were lacerations. Using Lackmann's classification for facial bite severity, IIIA bites (complete avulsion) were most common for both males (9) and females (7). Females comprised 68.4% of total lip bite victims. Bites to the extremity were associated with late presentation and infection. We report a salvage rate of 85.7% for infected digits. CONCLUSIONS: Compared with most other studies on human bites, our patient population presents with a higher rate of avulsive lip injuries, most commonly among women. Our postoperative infection rate is lower than many others reported in the literature. Satisfactory cosmetic outcome with low risk of infection can be achieved by following well-established principles of wound healing and tension-free closure.

Clinical practice guidelines for management of head and neck squamous cell carcinoma of unknown primary: an AGREE II appraisal.

IMPORTANCE: Squamous cell carcinoma without a known primary is an uncommon form of head and neck cancer that requires multidisciplinary collaboration for effective management. OBJECTIVE: To evaluate the quality of clinical practice guidelines (CPG) using the Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument. DESIGN: A systematic literature search was performed to identify CPGs pertaining to the diagnosis and treatment of head and neck squamous cell carcinoma of unknown primary (HNSCCUP). Data were abstracted from guidelines meeting inclusion criteria and appraised by four independent reviewers in the six domains of quality defined by the AGREE II. SETTING: Online database. PARTICIPANTS: None. EXPOSURE: None. MAIN OUTCOME(S) AND MEASURE(S): Quality domain scores and intraclass correlation coefficients (ICC) were calculated across domains to qualify inter-rater reliability. RESULTS: Seven guidelines met inclusion criteria. Two guidelines achieved a score of > 60% in five or more AGREE II quality domains to gain designation as 'high'-quality content. One "average-quality" guideline authored by the ENT UK Head and Neck Society Council achieved a score of > 60% in three quality domains. The remaining four CPGs demonstrated low-quality content, with deficits most pronounced in domains 3 and 5, suggesting a lack of rigorously developed and clinically applicable information. CONCLUSIONS AND RELEVANCE: As the diagnosis and treatment of head and neck cancer continues to evolve, identification of high-quality guidelines will become increasingly important. The authors recommend consulting HNSCCUP guidelines from the National Institute for Health and Care Excellence (NICE) or the American Society of Clinical Oncology (ASCO). TRIAL REGISTRATION: None.

Spinal pseudomeningocele closure: capsulofascial interposition technique.

INTRODUCTION: The development of a spinal pseudomeningocele is a complication of dural repair or reconstruction that carries significant morbidity for pediatric patients. In addition to cerebrospinal fluid (CSF) hypotension and positional headaches, CSF leaking into the extradural space increases the risk for incisional breakdown, meningitis, and cosmetic deformity. Spinal pseudomeningocele management is challenging, and reported techniques range from local wound revisions/exploration to shunt placement for permanent CSF diversion. METHODS: At our institution, we have recently implemented a multidisciplinary approach of capsulofascial interposition for the surgical repair of the symptomatic spinal pseudomeningocele with a combined neurosurgery-plastic surgery team. To our knowledge, this technique has not previously been characterized, and we describe the technical aspects of this surgery here. RESULTS: Among 10 patients treated with this technique, none required reoperation or developed infections following pseudomeningocele closure; 2 patients received postoperative transfusions; and 2 patients underwent CSF diversion procedures. CONCLUSION: We propose that this capsulofascial interposition technique should be employed in the surgical treatment of symptomatic spinal pseudomeningoceles.

Allegations of Failure to Obtain Informed Consent in Otolaryngology: Evidenced-Based Recommendations for Sinus Surgeons.

BACKGROUND: Informed consent requires preoperative discussion of surgical risks, complications, and alternative treatment options. Allegations of incomplete informed consent are common in the field of otolaryngology. OBJECTIVES: Analyze outcomes and case variables in cases of alleged informed consent failure involving otolaryngologists. METHODS: A legal research database containing state and federal case records from across the United States was retrospectively reviewed for malpractice claims involving informed consent and otolaryngology. RESULTS: Among the 128 informed consent cases identified, 72.6% resulted in favorable verdicts for otolaryngologists. Functional endoscopic sinus surgery (FESS) was the most common source of informed consent litigation in the field of otolaryngology, with an incidence four-fold higher than the next most litigated procedure of uvulopalatopharyngoplasty (21.9% vs 5.4%). The top four factors cited in FESS-related cases were CSF leak (10), inadequate discussion of alternative therapies (4), diplopia (3), and meningitis (3). Cases resulting in a transient injury were significantly less likely to result in a payment from a plaintiff verdict or settlement (9.1%) as compared to payment-rates among cases involving permanent complications (34.6%) (p = 0.005). CONCLUSIONS: Failure to obtain informed consent is an important factor in medical malpractice litigation. This report identifies specific, actionable recommendations aimed at protecting sinus surgeons from liability and ensuring that patients are better informed.