Quantifying Sagittal Lip Changes in Patients with Bilateral Cleft Lip Post Abbe Flap Reconstruction.

OBJECTIVES: To objectively quantify results of sagittal lip changes following Abbe flap reconstruction in patients with bilateral cleft lip. DESIGN: Retrospective, observational study. SETTING: Single institution, 8-year retrospective review. PATIENTS/PARTICIPANTS: In total, 17 patients with bilateral cleft lip that underwent Abbe flap reconstruction were included in this study. INTERVENTION: Patients had lateral photographs taken prior to Abbe flap reconstruction and at least 8 months post-Abbe flap. MAIN OUTCOME MEASUREMENTS: Vegter's index, Sushner's S2 line and Burstone's B line reference lines were used for evaluation of sagittal lip changes. Wilcoxon signed rank tests were used in analysis. RESULTS: The mean pre-Abbe flap upper to lower lip ratio, defined as Vegter's Lip Index, was 0.906 compared to a mean of 0.946 following Abbe flap reconstruction. The mean upper to lower lip ratio for Sushner's S2 and Burstone's B line exhibited an increase in upper lip sagittal projection from -0.164 to 1.459 and 0.259 to 0.957, respectively (P < 0.001). CONCLUSIONS: This study quantifies sagittal changes to upper and lower lip position after Abbe flap reconstruction. These findings may aid in operative planning and patient/caregiver expectations during counseling.

Same-day Discharge for Cleft Palate Repair: A Single-Surgeon Retrospective Analysis.

OBJECTIVE: To evaluate the safety of same-day discharge for patients undergoing primary cleft palate repair. DESIGN: Single-surgeon retrospective review. SETTING: Tertiary care institution. PATIENTS/PARTICIPANTS: 40 consecutive patients that underwent primary cleft palate repair by a single surgeon from September 2018 to June 2023. INTERVENTIONS: Same-day discharge versus overnight admission after primary palatoplasty. MAIN OUTCOME MEASURES: 30-day readmission, reoperation, wound and all-cause complication rate and 1-year fistula incidence. RESULTS: Of 40 total cases, 20 patients were discharged on the same calendar day and 20 patients were admitted for overnight stay following primary cleft palate repair. In the same-day discharge group, readmission incidence was 10%(n = 2), wound complication incidence was 5%(n = 1), and postoperative complication incidence was 15%(n = 3). In comparison, patients admitted overnight had a readmission incidence of 5%(n = 1, P = 1.00), wound complication incidence of 10%(n = 2, P = 1.00), and postoperative complications of 20%(n = 4, P = 1.00) No patients had 30-day reoperations or fistulas at 1 year. A higher proportion of admitted patients held a preoperative diagnosis of unilateral cleft palate and alveolus (Veau 3) as compared to patients discharged on the same day (P = .019). During the postoperative hospital course, admitted patients received significantly more oxycodone at median of 2 doses (IQR 1.00-3.75) and acetaminophen at a median of 4 doses (IQR 3.00-5.00) than patients with same-day discharge with a median of 1 dose (IQR 0.00 -1.00, P < .001). CONCLUSIONS: In a low-risk patient population, same-day discharge following primary cleft palate repair may be safely undertaken and result in similar short-term outcomes and 1-year fistula incidence as patients admitted for overnight stay.

Transpalpebral/Blepharoplasty Incision and Supraorbital Craniotomy for the Treatment of Ethmoidal Dural Arteriovenous Fistulas: A Case Series.

BACKGROUND AND OBJECTIVES: Inherent complex angioarchitecture associated with ethmoidal dural arteriovenous fistulas (dAVFs) can make endovascular treatment methods challenging. Many surgical approaches are accompanied by unfavorable cosmetic results such as facial scarring. Blepharoplasty incision of the eyelid offers a minimal, well-hidden scar compared with other incision sites while offering the surgeon optimal visualization of pathogenic structures. This case series aims to report an initial assessment of the safety and efficacy of supraorbital craniotomy by blepharoplasty transpalpebral (eyelid) incision for surgical disconnection of ethmoidal dAVFs. METHODS: Retrospective chart review was conducted for all patients who underwent blepharoplasty incision and craniotomy for disconnection of ethmoidal dAVFs at our institution between October 2011 and February 2023. Patient charts and follow-up imaging were reviewed to report clinical and angiographic outcomes as well as periprocedural and follow-up complications. RESULTS: Complete obliteration and disconnection of ethmoidal dAVF was achieved in all 6 (100%) patients as confirmed by intraoperative angiogram with no resulting morbidity or mortality. Periprocedural complications included one case of transient nasal cerebrospinal fluid leak that was self-limiting and resolved before discharge without intervention. CONCLUSION: Surgical treatment for ethmoidal dAVFs, specifically by transpalpebral incision and supraorbital craniotomy, is a safe and effective treatment option and affords the surgeon greater access to the floor of the anterior fossa when necessary. In addition, blepharoplasty incision addressed patient concerns for facial scarring compared with other incision sites by creating a more well-hidden, minimal scar in the natural folds of the eyelid for patients with an eyelid crease.

Abbe Flap Division Before Two Weeks' Time: Harmful or Advantageous?

INTRODUCTION: The Abbe flap is a standard intervention to treat upper lip deformities in patients with bilateral cleft lip. This two-stage procedure requires a 2 to 3-week period in which the superior and inferior lips remain connected. This study evaluates the safety of Abbe flap division and inset prior to 14 days' time. MATERIALS AND METHODS: A single institution, 8-year review of all patients with a bilateral cleft lip who underwent Abbe flap reconstruction was performed. Patients were classified into two groups: those whom division was performed 14 days or later and those with division earlier than 14 days. RESULTS: A total of 26 patients were identified. Patients who underwent Abbe flap division in less than 14 days (n = 10) demonstrated an average time to division of 9.7 days (range 7-13 days) with no evidence of flap loss, wound breakdown or infection. Patients who underwent Abbe flap division within 14 days or more (n = 16) demonstrated an average time to division of 15 days with four minor complications and no flap loss. CONCLUSION: Dividing the Abbe flap after the first postoperative week appears to be safe and without additional risk to flap loss or wound breakdown. A shorter time to Abbe flap division may decrease the burden of care on patients and their caregivers.

The Burden of Care of Nasoalveolar Molding: An Institutional Experience.

Nasoalveolar molding (NAM) is an early presurgical intervention to facilitate primary cleft lip repair by reducing cleft severity and improving labial and nasal form. However, it continues to be associated with the burden of care that influences access and completion of therapy. The authors, therefore, aim to determine the burden of care of NAM therapy for families seeking treatment at a high-volume urban cleft center. A retrospective study of all patients undergoing primary cleft repair between 2012 and 2020 was performed. Patients were grouped based on whether or not NAM therapy was offered. Variables including physical, psychosocial, and financial factors were assessed. Two hundred and thirty patients underwent primary cleft repair between 2012 and 2020. Of these, 176 patients were indicated for NAM, with 4% discontinuing, and 54 patients did not undergo NAM. The 169 patients who completed NAM had a mean duration of treatment of 13.6±8.8 wks consisting of 15±6 scheduled NAM adjustment visits and 1±1 unscheduled visit made urgently to assess caregiver concerns. The mean travel distance was 28.6±37.1 miles. Eighty-four percent of caregivers were married, and 16% did not have English as a primary language. Though 57% had private insurance, 43% of patients received charity support for their treatment. NAM is a finite presurgical intervention that requires caregivers to participate in patient care for approximately three months of their early life. The decision to pursue NAM should be considered alongside the burden of care for caregivers to complete treatment.

Defining the Treatment Gap in Nasoalveolar Molding: Factors Affecting the Utilization of NAM in an Urban Cleft Center.

BACKGROUND: Many cleft centers incorporate NasoAlveolar Molding (NAM) into their presurgical treatment protocols. However, there are limited data on eligible patients who do not receive or complete NAM. This study characterizes the demographics associated with non-utilization or completion of NAM. METHODS: A single-institution retrospective review was performed of all patients with cleft lip and alveolus undergoing primary unilateral and bilateral cleft lip repair from 2012-2020. Patients were grouped based on utilization or non-utilization of NAM. Demographic and treatment data were collected, including documented reasons for not pursuing or completing NAM. RESULTS: Of 230 eligible patients, 61 patients (27%) did not undergo or complete NAM (no-NAM). In this group, 37 (60.7%) received no presurgical intervention, 12 (19.7%) received presurgical nostril retainers, 3 (4.9%) received lip taping, 1 (1.6%) received a combination of taping/nostril retainers, and 8 (13.1%) discontinued NAM. The most common reasons for not receiving NAM were sufficiently aligned cleft alveolus (21.3%), medical complexity (16.4%), late presentation (16.4%), and alveolar notching (18%). Compared to the NAM group, the no-NAM group had significantly lower rates of prenatal cleft diagnosis/consult, and significantly higher proportion of non-married and non-English speaking caregivers. Multivariable analysis controlling for insurance type, primary language, prenatal consult, marital status, and age at first appointment found that age at first appointment is the only statistically significant predictor of NAM utilization (P < .001). CONCLUSIONS: Common reasons for non-utilization of NAM include well-aligned cleft alveolus, medical complexity, and late presentation. Early presentation is an important modifiable factor affecting rates of NAM utilization.

LeFort I Horizontal Osteotomy: Defining the Feasibility of the "High Osteotomy".

OBJECTIVE: To define "high osteotomy" and determine the feasibility of performing this procedure. DESIGN: Single institution, retrospective review. SETTING: Academic tertiary referral hospital. PATIENTS, PARTICIPANTS: 34 skeletally mature, nonsyndromic patients with unilateral CLP who underwent Le Fort I osteotomy between 2013 and 2020. Patients with cone-beam computed tomography (CBCT) scans completed both pre- (T1) and post-operatively (T2) were included. Patients with bilateral clefts and rhinoplasty prior to post-operative imaging were excluded. INTERVENTIONS: Single jaw one-piece Le Fort I advancement surgery. MAIN OUTCOME MEASURES: Measurements of the superior ala and inferior turbinates were taken from the post-operative CBCT. RESULTS: The sample included 26 males and 8 females, 12 right- and 22 left-sided clefts. The inferior turbinates are above the superior alar crease at a rate of 73.53% and 76.48% on the cleft and non-cleft sides, respectively. One (2.9%) osteotomy cut was above the level of the cleft superior alar crease, and no cuts were above the level of the non-cleft superior ala. On average, the superior ala was 2.63 mm below the inferior turbinates. The average vertical distances from the superior alar crease and the inferior turbinates to the base of the non-cleft side pyriform aperture were 12.17 mm (95% CI 4.00-20.34) and 14.80 mm (95% CI 4.61-24.98), respectively. To complete a "high osteotomy," with 95% confidence, the cut should be 20.36 mm from the base of the pyriform aperture. CONCLUSIONS: A "high" osteotomy is not consistently possible due to the relationship between the superior alar crease and the inferior turbinate.

Characterizing Cleft Rhinoplasty Across Skeletal Maturity: A Systematic Review of Terminology and Surgical Techniques.

OBJECTIVE: The purpose of this study is to assess cleft rhinoplasty terminology across phases of growth.Design/Setting: A systematic review was performed on cleft rhinoplasty publications over 20 years.Interventions: Studies were categorized by age at surgical intervention: infant (<1 year); immature (1 to 14 years); mature (>15 years).Main Outcome Measures: Collected data included terminology used and surgical techniques. RESULTS: The 288 studies included demonstrated a wide range of terminology. In the infant group, 51/54 studies used the term "primary." In the immature group, 7/18 studies used the term "primary," 3/18 used "secondary." In the mature group, 2/33 studies used the term "primary," 16/33 used "secondary," 2/33 used "definitive," 5/33 used terms such as "mature," "adult," and "late," and 8/33 did not use terminology.Surgical technique assessment demonstrated: cleft rhinoplasty at infancy used nostril rim or no nasal incision, immature rhinoplasty used closed and open rhinoplasty incisions; and mature rhinoplasty used a majority of open rhinoplasty. Infant and immature cleft rhinoplasty incorporated septal harvest or spur removal in <10% of cases, whereas these procedures were common in mature rhinoplasty. No studies in infants or immature patients used osteotomies or septal grafts, common techniques in mature rhinoplasty. CONCLUSIONS: Current terminology for cleft rhinoplasty is varied and inconsistently applied across stages of facial development. However, cleft rhinoplasty performed at infancy, childhood, and facial maturity are surgically distinct procedures. The authors recommend the terminology "infant," "immature," and "mature" cleft rhinoplasty to accurately describe this procedure within the context of skeletal growth.

Clinical Outcomes of Bilateral Cleft Lip and Palate Repair with Nasoalveolar Molding and Gingivoperiosteoplasty to Facial Maturity.

BACKGROUND: The long-term effects of nasoalveolar molding (NAM) on patients with bilateral cleft lip and palate (BCLP) are unknown. The authors report clinical outcomes of facially mature patients with complete BCLP who underwent NAM and gingivoperiosteoplasty (GPP). METHODS: A single-institution retrospective study of nonsyndromic patients with complete BCLP who underwent NAM between 1991 and 2000 was performed. All study patients were followed to skeletal maturity, at which time a lateral cephalogram was obtained. The total number of cleft operations and cephalometric measures was compared with a previously published external cohort of patients with complete and incomplete BCLP in which a minority (16.7%) underwent presurgical orthopedics before cleft lip repair without GPP. RESULTS: Twenty-four patients with BCLP comprised the study cohort. All patients underwent GPP, 13 (54.2%) underwent alveolar bone graft, and nine (37.5%) required speech surgery. The median number of operations per patient was five (interquartile range, two), compared with eight (interquartile range, three) in the external cohort ( P < 0.001). Average age at the time of lateral cephalogram was 18.64 years (1.92). There was no significant difference between our cohort and the external cohort with respect to sella-nasion-point A angle (SNA) [73 degrees (6 degrees) versus 75 degrees (11 degrees); P = 0.186] or sella-nasion-point B angle (SNA) [78 degrees (6 degrees) versus 74 degrees (9 degrees); P = 0.574]. Median ANB (SNA - SNB) was -3 degrees (5 degrees) compared with -1 degree (7 degrees; P = 0.024). Twenty patients (83.3%) underwent orthognathic surgery. CONCLUSION: Patients with BCLP who underwent NAM and GPP had significantly fewer total cleft operations and mixed midface growth outcomes at facial maturity compared with patients who did not undergo this treatment protocol. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Transpalpebral Eyelid Approach for Supraorbital Frontal Craniotomy and Access to the Anterior Cranial Fossa.

SUMMARY: The transpalpebral eyelid approach is an innovative alternative to traditional incisions for exposure of the anterior cranial fossa for neurosurgery. However, there is a paucity of data on this surgical technique in the plastic surgery literature for accessing the anterior cranial fossa. A retrospective review was performed of patients who underwent supraorbital frontal craniotomy using an anterior skull base approach with transpalpebral exposure over 8 years by a single plastic surgeon. Surgical techniques, medical comorbidities, intraoperative complications, and long-term complications were assessed. Twenty patients (mean age 52 ± 12 years, 55% male, 45% female) underwent supraorbital frontal craniotomy using an anterior skull base approach with upper transpalpebral exposure. Operative indications included the following: 75% had anterior communicating aneurysms, with a mean aneurysm size of 5.36 ± 1.91 mm; 10% had meningiomas; 10% had dural fistulas; and 5% had an orbital hemangioma. A total of 60% of patients had a smoking history. No intraoperative complications were encountered, and no cases required conversion to a traditional open approach. Mean length of hospital stay was 3.2 ± 1.5 days. Postoperative imaging revealed no residual or recurrent pathology. Mean follow-up time was 62.2 ± 30.6 months. No long-term neurologic or ophthalmologic complications or infections occurred. No forehead paresthesias, brow ptosis, or brow paralysis were noted. The transpalpebral technique is a safe, minimally invasive method to approach lesions of the anterior cranial fossa. Successful application may require appropriate management of the frontal sinus and supraorbital nerve. This approach does not limit neurosurgical access or results and led to no neurosurgical complications. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Reconstructive Approaches Following Sphenoorbital Meningioma Resection.

Sphenoorbital meningiomas are a challenge to access and reconstruct. Although there is much neurosurgical literature on resection of such tumors, there is little discussion on the best methods for the reconstruction of consequent defects, which are often extensive due to large areas of hyperostosis requiring resection. We performed a retrospective analysis of patients who underwent resection and reconstruction of a sphenoorbital meningioma by the senior authors (C.S. and D.A.S.) between 2010 and 2020. Surgical access in all cases included an orbitozygomatic osteotomy. The study cohort consisted of 23 patients (20 female, 3 male) with an average age of 50 (range: 37-72) years at the time of surgery. Most patients had progressive proptosis before the ablative operation. Orbital reconstruction was with a combined titanium-Medpor implant in 18 patients, split calvarial bone graft in 3 patients, and a Medpor implant in 2 patients. Calvarial reconstruction was performed with titanium mesh in 21 patients, split calvarial bone graft and titanium mesh in 1 patient, and craniotomy bone and titanium plate in 1 patient. Reoperation was required in 7 patients due to hypoglobus or enophthalmos (N=2), orbital implant malposition (N=1), abscess (N=1), pain (N=1), intracranial fat graft modification (N=1), and soft tissue deformities (N=2). Our experience demonstrates that sphenoorbital meningiomas can require broad areas of resection of the skull base and calvarium and necessitate comprehensive reconstruction of the anterior cranial fossa, orbital walls, and cranium. Collaboration between craniofacial surgeons and neurosurgeons can achieve optimal results.

Free Fibula Flap for the Treatment of Agnathia in a 10-Year-Old With Severe Agnathia-Otocephaly Complex.

Agnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia and is a rare and often fatal diagnosis. Herein, the technical considerations and details of mandibular reconstruction using virtual surgical planning (VSP) and a vascularized free fibula flap for further mandibular reconstruction in a 10-year-old boy are presented. The patient's preoperative examination was consistent with agnathia (absence of mandibular symphysis, bilateral mandibular bodies, condyles, coronoids, rami, and temporomandibular joint), severe microstomia, and a Tessier # 30 cleft (maintained to allow oral access until later in treatment). Virtual surgical planning was utilized to plan a 3-segment fibula for the reconstruction of the mandibular symphysis and bilateral body segments, and bilateral costochondral grafts were planned for the rami. To the authors' knowledge, this represents the first application of virtual surgical planning for mandibular reconstruction with a vascularized free fibula flap in a pediatric patient with severe agnathia-otocephaly complex.

"Septoplasty" Performed at Primary Cleft Rhinoplasty: A Systematic Review of Techniques and Call for Accurate Terminology.

OBJECTIVE: Primary cleft nasal repair can include septal reconstruction. We hypothesize that primary cleft septoplasty and adult septoplasty have fundamental differences that render these procedures as distinct surgical entities. DESIGN: Systematic review of the PubMed, Cochrane, and Embase databases performed on pediatric cleft and general adult septoplasty techniques through December 2021. (PROSPERO ID CRD42022295763). MAIN OUTCOME MEASURES: Collected data included information on septal dissection, septal detachment, and management of the bony and cartilaginous septum. RESULTS: Twenty-eight pediatric cleft septoplasty and 229 adult septoplasty studies were included. Dissection in primary cleft septoplasty was limited to the anterocaudal septum, while secondary cleft septoplasty and adult septoplasty techniques entailed wide exposures of the cartilaginous septum with or without exposure of the perpendicular plate of the ethmoid. In primary cleft septoplasty, detachment of the septum was mostly limited to the nasal spine and anterior base of the cartilaginous septum, while secondary cleft septoplasty and adult septoplasty included detachment from the vomer, and ethmoid. In the few reports of cartilage excision during primary cleft septoplasty, removal was limited to the anterior inferior border of the septum, while secondary cleft septoplasty and adult septoplasty included excision of the cartilaginous and bony septum. CONCLUSION: Primary cleft septoplasty is distinct from septoplasty performed on facially mature patients. More specifically, septal dissection and detachment are limited to the anterior caudal area during primary lip repair, with rare removal of cartilage or bone. Given these differences, the authors suggest the term "septal reset" to describe septoplasty performed during primary cleft nasal repair.

Implementation of an Ambulatory Cleft Lip Repair Protocol: Surgical Outcomes.

OBJECTIVES: Cleft lip repair has traditionally been performed as an inpatient procedure. There has been an interest toward outpatient cleft lip repair to reduce healthcare costs and avoid unnecessary hospital stay. We report surgical outcomes following implementation of an ambulatory cleft lip repair protocol and hypothesize that an ambulatory repair results in comparable safety outcomes to inpatient repair. DESIGN/SETTING: This is a single-institution, retrospective study. PATIENTS/PARTICIPANTS: Patients undergoing primary unilateral (UCL) and bilateral (BCL) cleft lip repair from 2012 to 2021 with a minimum 30-day follow-up. A total of 226 patients with UCL and 58 patients with BCL were included. INTERVENTION: Ambulatory surgery protocol in 2016. OUTCOME MEASURES: Variables include demographics and surgical data including 30-day readmission, 30-day reoperation, and postoperative complications. RESULTS: There were no differences in rates of 30-day readmission, reoperation, wound complications, or postoperative complications between the pre- and post-protocol groups. Following ambulatory protocol implementation, 80% of the UCL group and 56% of the BCL group received ambulatory surgery. Average length of stay dropped from 24 h pre-protocol to 8 h post-protocol. The 20% of the UCL group and 44% of the BCL group chosen for overnight stay had a significantly higher proportion of congenital abnormalities and higher American Society of Anesthesiology (ASA) class. Reasons for overnight stay included cardiac/airway monitoring, prematurity, and monitoring of comorbidities. There were no differences in surgical outcomes between the ambulatory and overnight stay groups. CONCLUSIONS: An ambulatory cleft lip repair protocol can significantly reduce average length of stay without adversely affecting surgical outcomes.

Simultaneous Le Fort III and Le Fort I Osteotomy: Surgical Outcomes and Clinical Parameters.

INTRODUCTION: Simultaneous Le Fort III/I (LF III/I) osteotomies are often performed when a differential advancement of the upper and lower midface is needed. This study aims to evaluate midface position preoperative and 1 week postoperative in patients with severe midface hypoplasia. In addition, this study aims to compare the planned surgical movements to the actual postoperative movements. MATERIALS AND METHODS: A retrospective review was conducted using cephalometry for patients treated with a simultaneous LF III/I osteotomy at a single institution. Osteotomies were performed during 1980-2018 on skeletally mature patients with a craniofacial syndrome, with clinical and radiographic follow-up available. RESULTS: Twelve patients met the inclusion criteria with a mean age of 20.2±6.4 years. Treatment resulted in statistically significant anterior movements related to Orbitale, anterior nasal spine, A Point, and the upper incisor tip, and inferior movements related to anterior nasal spine, A Point, upper and lower incisor tips, B point, and pogonion. Stability after 1 year showed only statistically significant changes at ANB. The predictable error for planned movements versus actual movements was greater in the vertical plane than the horizontal plane. CONCLUSIONS: A simultaneous LF III/I osteotomy significantly improved the midface position and occlusal relationship in syndromic patients with midface hypoplasia in a predictable manner. Further multicenter studies with larger sample sizes are needed to validate the conclusions.

De novo mutations in the BMP signaling pathway in lambdoid craniosynostosis.

Lambdoid craniosynostosis (CS) is a congenital anomaly resulting from premature fusion of the cranial suture between the parietal and occipital bones. Predominantly sporadic, it is the rarest form of CS and its genetic etiology is largely unexplored. Exome sequencing of 25 kindreds, including 18 parent-offspring trios with sporadic lambdoid CS, revealed a marked excess of damaging (predominantly missense) de novo mutations that account for ~ 40% of sporadic cases. These mutations clustered in the BMP signaling cascade (P = 1.6 × 10-7), including mutations in genes encoding BMP receptors (ACVRL1 and ACVR2A), transcription factors (SOX11, FOXO1) and a transcriptional co-repressor (IFRD1), none of which have been implicated in other forms of CS. These missense mutations are at residues critical for substrate or target sequence recognition and many are inferred to cause genetic gain-of-function. Additionally, mutations in transcription factor NFIX were implicated in syndromic craniosynostosis affecting diverse sutures. Single cell RNA sequencing analysis of the mouse lambdoid suture identified enrichment of mutations in osteoblast precursors (P = 1.6 × 10-6), implicating perturbations in the balance between proliferation and differentiation of osteoprogenitor cells in lambdoid CS. The results contribute to the growing knowledge of the genetics of CS, have implications for genetic counseling, and further elucidate the molecular etiology of premature suture fusion.

Clinical Outcomes in Orthognathic Surgery for Craniofacial Microsomia Following Mandibular Distraction Using CBCT Analysis: A Retrospective Study.

OBJECTIVE: The aim of this study was to evaluate the outcomes of orthognathic surgery (OGS) in patients with craniofacial microsomia (CFM) who had previously undergone mandibular distraction osteogenesis (MDO). DESIGN: A retrospective cohort study was performed including all patients with CFM who were treated with OGS at a single institution between 1996 and 2019. The clinical records, operative reports, and cone beam computed tomography (CBCT) scans were reviewed. CBCT data before OGS (T1), immediately after OGS (T2), and at long-term follow-up (T3) were analyzed using Dolphin three-dimensional software to measure the occlusal cant and chin point deviation. RESULTS: The study included 12 patients with CFM who underwent OGS (6 underwent OGS without MDO and 6 underwent OGS after MDO). There was a statistically significant improvement in occlusal cant and chin point deviation in both groups postoperatively. Occlusal cant relapsed by a mean of 0.6° (standard deviation [SD] 1.1°) in the patients who had OGS alone compared with 0.7° (SD 1.2°) in the patients who had OGS after MDO (P = .745) between T2 and T3. There was no statistically significant difference in chin point relapse between patients who had OGS alone compared with those who had OGS after MDO (0.1 mm [SD 2.5mm] vs 0.7mm [SD 2.2mm]; P = .808). CONCLUSIONS: Within the limitations of this study, these findings suggest that OGS after MDO in patients with CFM can produce stable results.

The First Successful Combined Full Face and Bilateral Hand Transplant.

BACKGROUND: Vascularized composite allotransplantation has redefined the frontiers of plastic and reconstructive surgery. At the cutting edge of this evolving paradigm, the authors present the first successful combined full face and bilateral hand transplant. METHODS: A 21-year-old man presented for evaluation with sequelae of an 80 percent total body surface area burn injury sustained after a motor vehicle accident. The injury included full face and bilateral upper extremity composite tissue defects, resulting in reduced quality of life and loss of independence. Multidisciplinary evaluation confirmed eligibility for combined face and bilateral hand transplantation. The operative approach was validated through 11 cadaveric rehearsals utilizing computerized surgical planning. Institutional review board and organ procurement organization approvals were obtained. The recipient, his caregiver, and the donor family consented to the procedure. RESULTS: Combined full face (i.e., eyelids, ears, nose, lips, and skeletal subunits) and bilateral hand transplantation (i.e., forearm level) was performed over 23 hours on August 12 to 13, 2020. Triple induction and maintenance immunosuppressive therapy and infection prophylaxis were administered. Plasmapheresis was necessary postoperatively. Minor revisions were performed over seven subsequent operations, including five left upper extremity, seven right upper extremity, and seven facial secondary procedures. At 8 months, the patient was approaching functional independence and remained free of acute rejection. He had significantly improved range of motion, motor power, and sensation of the face and hand allografts. CONCLUSIONS: Combined face and bilateral hand transplantation is feasible. This was the most comprehensive vascularized composite allotransplantation procedure successfully performed to date, marking a new milestone in plastic and reconstructive surgery for patients with otherwise irremediable injuries.

Genetic Influence on Neurodevelopment in Nonsyndromic Craniosynostosis.

BACKGROUND: Nonsyndromic craniosynostosis is one of the most common anomalies treated by craniofacial surgeons. Despite optimal surgical management, nearly half of affected children have subtle neurocognitive deficits. Whereas timing and type of surgical intervention have been studied, the possibility of genetic influence on neurodevelopment in nonsyndromic craniosynostosis patients remains unexplored. METHODS: The authors performed whole-exome sequencing for 404 case-parent trios with sporadic nonsyndromic craniosynostosis. Statistical analyses were performed to assess the burden of de novo mutations in cases compared to both expectation and 1789 healthy control trios. Individuals with and without each mutation class were analyzed, and the presence or absence of various types of neurodevelopmental delay were recorded alongside demographic information. RESULTS: The authors identified a highly significant burden of damaging de novo mutations in mutation-intolerant [probability of loss of function intolerance (pLI) >0.9] genes in nonsyndromic craniosynostosis probands (p = 5.9 × 10-6). Children with these mutations had a two-fold higher incidence of neurodevelopmental delay (p = 0.001) and a more than 20-fold greater incidence of intellectual disability (p = 7.2 × 10-7), and were 3.6-fold more likely to have delays that persisted past 5 years of age (p = 4.4 × 10-4) in comparison with children with nonsyndromic craniosynostosis without these mutations. Transmitted loss of function mutations in high-pLI genes also conferred a 1.9-fold greater risk of neurodevelopmental delay (p = 4.5 ×10-4). CONCLUSIONS: These findings implicate genetic lesions concurrently impacting neurodevelopment and cranial morphogenesis in the pathoetiology of nonsyndromic craniosynostosis and identify a strong genetic influence on neurodevelopmental outcomes in affected children. These findings may eventually prove useful in determining which children with nonsyndromic craniosynostosis are most likely to benefit from surgical intervention. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Skeletal and Dental Correction and Stability Following LeFort I Advancement in Patients With Cleft Lip and Palate With Mild, Moderate, and Severe Maxillary Hypoplasia.

OBJECTIVE: This study evaluates skeletal and dental outcomes of LeFort I advancement surgery in patients with cleft lip and palate (CLP) with varying degrees of maxillary skeletal hypoplasia. DESIGN: Retrospective study. METHOD: Lateral cephalograms were digitized at preoperative (T1), immediately postoperative (T2), and 1-year follow-up (T3) and compared to untreated unaffected controls. Based on the severity of cleft maxillary hypoplasia, the sample was divided into 3 groups using Wits analysis: mild: ≤0 to ≥-5 mm; moderate: <-5 to >-10 mm; and severe: ≤-10 mm. PARTICIPANTS: Fifty-one patients with nonsyndromic CLP with hypoplastic maxilla who met inclusion criteria. INTERVENTION: LeFort I advancement. MAIN OUTCOME MEASURE: Skeletal and dental stability post-LeFort I surgery at a 1-year follow-up. RESULTS: At T2, LeFort I surgery produced an average correction of maxillary hypoplasia by 6.4 ± 0.6, 8.1 ± 0.4, and 10.7 ± 0.8 mm in the mild, moderate, and severe groups, respectively. There was a mean relapse of 1 to 1.5 mm observed in all groups. At T3, no statistically significant differences were observed between the surgical groups and controls at angle Sella, Nasion, A point (SNA), A point, Nasion, B point (ANB), and overjet outcome measures. CONCLUSIONS: LeFort I advancement produces a stable correction in mild, moderate, and severe skeletal maxillary hypoplasia. Overcorrection is recommended in all patients with CLP to compensate for the expected postsurgical skeletal relapse.