Faculty Bibliography

BACKGROUND:This study characterizes the potential loss of velar length in patients with a wide cleft and rescue of this loss of domain by local flap reconstruction, providing anatomic evidence in support of primary lengthening of the soft palate during palatoplasty. METHODS:A retrospective review was conducted of all patients with a cleft palate at least 10mm in width, who underwent primary palatoplasty with a buccal flap prior to 18 months of age over a 2-year period. All patients underwent primary palatoplasty with horizontal transection of the nasal mucosa, which was performed after nasal mucosa repair, but prior to muscular reconstruction. The resulting palatal lengthening was measured and the mucosal defect was reconstructed with a buccal flap. RESULTS:Of the 22 patients included, 3 (13.6%) had a history of Pierre Robin sequence, and 5 (22.7%) had an associated syndrome. No patients had a Veau I cleft, 7 (31.8%) had a Veau II, 12 (54.5%) had a Veau III, and 3 had (13.6%) a Veau IV cleft. All patients had a right buccal flap during primary palatoplasty. The mean cleft width at the posterior nasal spine was 10.6  ±  2.82mm, and mean lengthening of the velum after horizontal transection of the nasal mucosa closure was 10.5  ±  2.23mm. There were 2 (9.1%) fistulas, 1 (4.5%) wound dehiscence, 1 (4.5%) 30-day readmission, and no bleeding complications. CONCLUSIONS:Patients with a wide cleft palate have a potential loss of 1cm velar length. The buccal flap can rescue the loss of domain in palatal length, and potentially improve palatal excursion.

BACKGROUND:Vascularized composite allotransplantation (VCA) has redefined the frontiers of plastic and reconstructive surgery. At the cutting edge of this evolving paradigm, we present the first successful combined full face and bilateral hand transplant (FT-BHT). METHODS:A 21-year-old man with sequelae of an 80% total body surface area burn injury sustained following a motor vehicle accident presented for evaluation. 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 FT-BHT. 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 (eyelids, ears, nose, lips, and skeletal subunits) and bilateral hand transplantation (forearm level) was performed over 23 hours on August 12-13th, 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 eight months, the patient is approaching functional independence and remains free of acute rejection. He has significantly improved range of motion, motor power, and sensation of the face and hand allografts. CONCLUSION/CONCLUSIONS:Combined FT-BHT is feasible. This is the most comprehensive VCA procedure successfully performed to date, marking a new milestone in plastic and reconstructive surgery for patients with otherwise irremediable injuries.

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/METHODS: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/METHODS: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/CONCLUSIONS: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.

The current standard of care for an alveolar cleft defect is an autogenous bone graft, typically from the iliac crest. Given the limitations of alveolar bone graft surgery, such as limited supply, donor site morbidity, graft failure, and need for secondary surgery, there has been growing interest in regenerative medicine strategies to supplement and replace traditional alveolar bone grafts. Though there have been preliminary clinical studies investigating bone tissue engineering methods in human subjects, lack of consistent results as well as limitations in study design make it difficult to determine the efficacy of these interventions. As the field of bone tissue engineering is rapidly advancing, reconstructive surgeons should be aware of the preclinical studies informing these regenerative strategies. We review preclinical studies investigating bone tissue engineering strategies in large animal maxillary or mandibular defects and provide an overview of scaffolds, stem cells, and osteogenic agents applicable to tissue engineering of the alveolar cleft. An electronic search conducted in the PubMed database up to December 2021 resulted in 35 studies for inclusion in our review. Most studies showed increased bone growth with a tissue engineering construct compared to negative control. However, heterogeneity in the length of follow up, method of bone growth analysis, and inconsistent use of positive control groups make comparisons across studies difficult. Future studies should incorporate a pediatric study model specific to alveolar cleft with long-term follow up to fully characterize volumetric defect filling, cellular ingrowth, bone strength, tooth movement, and implant support.

This case presents a facially mature patient with Beckwith-Wiedemann Syndrome (BWS) who presented with severe class III malocclusion. Computed tomography imaging revealed an anterior crossbite of 19 mm and a narrow pharyngeal airway at the level of the tongue base precluding mandibular setback surgery. The patient was indicated for a LeFort III combined with a LeFort I advancement, each of 10 mm, for a 20 mm combined advancement. Stable, functional occlusion was achieved without airway compromise. This novel use of the combined LeFort III/I can restore stable class I occlusion in patients with BWS at risk for tongue base airway compromise.

OBJECTIVE:Describe the first hybrid global simulation-based comprehensive cleft care workshop, evaluate impact on participants, and compare experiences based on in-person versus virtual attendance. DESIGN/METHODS:Cross-sectional survey-based evaluation. SETTING/METHODS:International comprehensive cleft care workshop. PARTICIPANTS/METHODS:Total of 489 participants. INTERVENTIONS/METHODS:Three-day simulation-based hybrid comprehensive cleft care workshop. MAIN OUTCOME MEASURES/METHODS:Participant demographic data, perceived barriers and interventions needed for global comprehensive cleft care delivery, participant workshop satisfaction, and perceived short-term impact on practice stratified by in-person versus virtual attendance. RESULTS: = .01). CONCLUSION/CONCLUSIONS:Hybrid simulation-based educational comprehensive cleft care workshops are overall well received by participants and have a positive perceived impact on their clinical practices. In-person attendance is associated with significantly higher satisfaction and perceived impact on practice. Considering that financial and health constraints may limit live meeting attendance, future efforts will focus on making in-person and virtual attendance more comparable.

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/METHODS:Risk, III.

BACKGROUND:Mandibular distraction osteogenesis is effective for the correction of severe tongue-based airway obstruction in infants with Pierre Robin sequence. Involved osteotomies may damage developing tooth buds and/or the inferior alveolar nerve. The authors evaluated the theoretical safety of various osteotomy techniques to better define infantile mandibular anatomy using computer-aided modeling. METHODS:Seven mandibular osteotomy techniques (oblique, inverted-L, multiangular, walking stick, high oblique, vertical/high inverted-L, and horizontal) were simulated using computed tomography studies from infants with Pierre Robin sequence and without other associated conditions. Software was used to manually segment the mandibular bone, inferior alveolar nerve, and tooth buds. RESULTS:Sixty-five computed tomography scans were included, yielding 130 hemimandibles. The horizontal osteotomy pattern had significantly lower theoretical risk of tooth bud (p < 0.001) and inferior alveolar nerve involvement (p < 0.001) than all other patterns. Osteotomies with high vertical components (i.e., vertical, walking stick, and multiangular) had lower theoretical tooth bud involvement than the more proximal oblique and inverted-L osteotomies (p < 0.001). Average lingula location was measured at a point 65 percent of the mandibular width from anterior mandibular border and 63 percent of the mandibular height from the inferior mandibular border. CONCLUSIONS:Surgical planning with computed tomography scans can help evaluate an infant's mandibular anatomy to select an osteotomy that reduces morbidity risks. Regardless of technique, tooth buds and the inferior alveolar nerve are often included in osteotomies. The lingula location in this study demonstrates a position more superior and posterior than that previously described. CLINICAL QUESTION/LEVEL OF EVIDENCE/METHODS:Therapeutic, V.

OBJECTIVES/OBJECTIVE: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/METHODS:This is a single-institution, retrospective study. PATIENTS/PARTICIPANTS/METHODS: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/METHODS:Ambulatory surgery protocol in 2016. OUTCOME MEASURES/METHODS: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.

Skeletal conditions represent a considerable challenge to health systems globally. Barriers to effective therapeutic development include a lack of accurate preclinical tissue and disease models. Most recently, work was attempted to present a novel whole organ approach to modeling human bone and cartilage tissues. These self-assembling skeletal organoids mimic the cellular milieu and extracellular organization present in native tissues. Bone organoids demonstrated osteogenesis and micro vessel formation, and cartilage organoids showed evidence of cartilage development and maturation. Skeletal organoids derived from both bone and cartilage tissues yielded spontaneous polarization of their cartilaginous and bone components. Using these hybrid skeletal organoids, we successfully generated "mini joint" cultures, which we used to model inflammatory disease and test Adenosine (A_2A ) receptor agonists as a therapeutic agent. The work and respective results indicated that skeletal organoids can be an effective biological model for tissue development and disease as well as to test therapeutic agents.