Faculty Bibliography

INTRODUCTION/BACKGROUND: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/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.

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.

Objective: Compare short term surgical outcomes and trends in cleft lip repair with or without gingivoperiosteoplasty (GPP). Design: Retrospective review of the ACS NSQIP-Pediatric database from 2014 to 2019. Patients: Patients between 2 and 18 months of age undergoing any initial cleft lip repair, with or without GPP, were selected via relevant CPT^® codes. Main Outcome Measures: Patient demographics, comorbidities, 30-day readmissions and post-operative complications are assessed. Results: From 2014 to 2019, a total of 6269 patients were identified, of which 6.67% underwent GPP (n = 418). Patients undergoing GPP were significantly older with an average age of 9 months compared to 5 months in the non-GPP group (P <.001). Co-morbidities were similar amongst both cohorts, although patients undergoing GPP were more likely to have a higher ASA class (P =.006), cardiac risk factors (P =.012) and syndromic diagnosis (P <.001). There were no differences in 30-day short term surgical outcomes. GPP was associated with increased operative time by ~25 minutes when compared to cleft lip repair alone (P <.001). Conclusion: GPP was not associated with increased 30-day postoperative complications, readmission, reoperation, or total length of hospital stay, and was associated with an increased operative time of 25 minutes. Children undergoing GPP were significantly older in age and were more likely to have a higher ASA class/cardiac risk factors.

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: < .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.

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/METHODS: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:  =  .808). CONCLUSIONS:Within the limitations of this study, these findings suggest that OGS after MDO in patients with CFM can produce stable results.

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.

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.

Background/Purpose: After LeFort I advancement surgery, soft tissue changes are unpredictable, especially in patients with orofacial clefts, as scar tissue from primary repair can alter soft tissue responses. Therefore, this study aimed to measure and evaluate soft tissue response following LeFort I advancement in skeletally matured patients with complete cleft lip and palate (CLP). Methods/Description: The cohort of 26 patients with non-syndromic CLP who underwent Le Fort I osteotomy between 2013 and 2019 and met the inclusion criteria. Patients were included if they had lateral cephalograms or CBCT at pre-operative (T1), immediately post-operative (T2), and one-year follow-up (T3). Patients who underwent nose/lip revision surgery before T3 were excluded. Four skeletal and dental hard-tissue (ANS, point A; A-point, upper incisor most labial; U1-most, upper incisor edge; U1-tip) and 5 softtissue (tip of nose or pronasale; Prn, subnasale; Sn, superior labial sulcus; SLS, upper lip anterior or labrale superius; LS, and stomion superius; SIMS) landmarks were digitized and measured. For the outcome analyses, 5 ratios of soft- to hard-tissue changes (Prn/ANS, Sn/A-point, SLS/A-point, LS/U1-most, and SIMS/ U1-tip) were calculated for each group, and associations between hard-and-soft tissue counterparts were assessed using Pearson correlation coefficient (r).
Result(s): Sixteen patients had UCLP, and 10 patients had BCLP. At one-year follow-up (T1-T3), the mean advancement in UCLP and BCLP groups at ANS were 4.4+/-3 and 4.7+/-3.9 mm, from point A were 6.6+/-2.5, 8.8+/- 2.6 mm, respectively. The mean horizontal changes of the corresponding soft tissue anatomy, Prn, were 2.7 +/-1.7, 4.6+/-3.5 mm, from Sn, were 3.9+/-1.9, 6.2+/-2.4. mm, and from SLS were 5.2+/-2.5, 7.4+/-2.8 mm. The mean advancement in at upper incisor most labial were 7.2+/-2.7 and 8.4+/-2.4 mm, and from the upper incisal edge were 7.5+/-2.9 and 8.4+/-2.7. mm. The mean horizontal changes of the soft tissue counterpart, LS, were 5.6+/-2.9, 7.9+/- 3.7 mm, and SIMS were 6.0+/-3.2, 7.3+/- 2.7 mm. All skeletal, dental, and soft tissue advancements from T1-T3 were significant (P< 0.01) except for Sn and LS in both groups and SIMS in UCLP group. For ratio and correlation analyses in UCLP and BCLP groups, Prn/AND were 0.48 (r=0.40) and (r=0.00), Sn/A-point were 0.58 (r=0.79) and 0.70 (r=0.77), SLS/A-point were 0.79 (r=0.82) and 0.85 (r=0.80), LS/U1-most were 0.74 (r=0.92) and 0.96 (r=0.74), and SIMS/U1-tip were 0.78 (r=0.75) and 0.82(r=0.67), respectively. All associations except for Prn/ANS were statistically significant (P< 0.01).
Conclusion(s): This study demonstrated a linear relationship between soft- and hard-tissue changes in the maxillary landmarks following LeFort I advancement in patients with complete cleft lip and palate (UCLP and BCLP)

Background/Purpose: The relative value unit (RVU) is a metric established by Medicare to quantify physician time and intensity required to furnish a surgical service, and is broadly used for the purposes of billing and physician compensation. Despite widespread use since the 1990s, the accuracy of RVU assignments has not been scientifically evaluated for cleft and craniofacial surgery. We hypothesize that unbalanced RVU allocation creates inappropriate disparities in value amongst procedures performed by cleft and craniofacial surgeons. Methods/Description: The American College of Surgeons Pediatric National Surgical Quality Improvement Program (NSQIP) database was queried to identify all cleft and craniofacial surgery cases performed by plastic surgeons from 2012-2019 based on Current Procedural Terminology (CPT) code. Microsurgical cases and CPT codes with a case count of fewer than 10 were excluded. Total RVUs per case were calculated based on the sum of work RVUs for the principal procedure, and any other procedure that was performed during the case. Efficiency was defined as total RVUs divided by total operative time (i.e. RVUs/hour), based on previously published methodology. Mean efficiency per CPT code was ranked and compared by quartile using Student's t-tests.
Result(s): The sample consisted of 69 CPT codes with a total of 50,450 cases. The most common procedure was cleft palate repair of the soft and/or hard palate (CPT 42200). The mean efficiency for the top quartile of CPT codes was 15.65+/-4.22 (range 12.05-26.56) RVUs/hour, compared to 7.39+/-0.98 (range 5.57-8.69) RVUs/hour for the bottom quartile (p<0.001). The mean operative time for the top quartile of CPT codes was 167.14+/-90.29 minutes, compared to 107.79 +/-55.17 minutes for the lowest quartile (p=0.029). In the top quartile, the majority of CPT codes were craniofacial procedures including frontofacial procedures (23.53%) and craniectomies for craniosynostosis or bony lesions (35.29%). The lowest quartile was comprised mainly of CPT codes for cleft procedures including surgeries for velopharyngeal insufficiency (17.65%), cleft palate repair (23.53%), and cleft septoplasty (5.88%). It was 2.5 times more efficient for a cleft and craniofacial surgeon to perform a local skin flap (15.18 RVUs/ hour, CPT 14040) than a secondary palatal lengthening for cleft palate (6.09 RVUs/hour, CPT 42200).
Conclusion(s): The current RVU allocation to cleft and craniofacial procedures creates arbitrary disparities in physician efficiency, with cleft procedures disproportionately negatively affected despite being among the most common procedures. RVU assignments should be reevaluated to avoid disincentivizing cleft surgical care