Faculty Bibliography

OBJECTIVE:To define "high osteotomy" and determine the feasibility of performing this procedure. DESIGN/METHODS:Single institution, retrospective review. SETTING/METHODS:Academic tertiary referral hospital. PATIENTS, PARTICIPANTS/METHODS: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/METHODS:Single jaw one-piece Le Fort I advancement surgery. MAIN OUTCOME MEASURES/METHODS: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.

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.

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.

The transpalpebral "eyelid" approach is an innovative alternative to the traditional incisions for exposure of the anterior cranial fossa for neurosurgery. Yet, there is a paucity of data on such a 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 eight years by a single plastic surgeon (D.A.S.). Surgical techniques, medical co-morbidities, intra-operative 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: 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. Notably, 60% had a smoking history. No intra-operative complications were encountered, and no cases required conversion to a traditional open approach. Mean length of hospital stay was 3.2±1.5 days. Post-operative imaging revealed no residual or recurrent pathology. Mean follow up time was 62.2±30.6 months. No long-term neurological or ophthalmologic complications or infections occurred. No forehead paresthesias, and no brow ptosis or brow paralysis were noted. The transpalpebral technique is an excellent, minimally invasive alternative to approach lesions of the anterior cranial fossa. Successful application may require appropriate management of the frontal sinus and supraorbital nerve. As described, this approach does not limit neurosurgical access or results, and led to no neurosurgical complications.

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.

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.

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.

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.