Faculty Bibliography

BACKGROUND:The authors present outcomes analysis of the nasoalveolar molding treatment protocol in patients with a cleft followed from birth to facial maturity. METHODS:A single-institution retrospective review was conducted of cleft patients who underwent nasoalveolar molding between 1990 and 2000. Collected data included surgical and orthodontic outcomes and incidence of gingivoperiosteoplasty, alveolar bone grafting, surgery for velopharyngeal insufficiency, palatal fistula repair, orthognathic surgery, nose and/or lip revision, and facial growth. RESULTS:One hundred seven patients met inclusion criteria (69 with unilateral and 38 with bilateral cleft lip and palate). Eighty-five percent (91 of 107) underwent gingivoperiosteoplasty (unilateral: 78 percent, 54 of 69; bilateral: 97 percent, 37 of 38). Of those patients, 57 percent (52 of 91) did not require alveolar bone grafting (unilateral: 59 percent, 32 of 54; bilateral: 54 percent, 20 of 37). Twelve percent (13 of 107) of all study patients underwent revision surgery to the lip and/or nose before facial maturity (unilateral: 9 percent, six of 69; bilateral: 18 percent, seven of 38). Nineteen percent (20 of 107) did not require a revision surgery, alveolar bone grafting, or orthognathic surgery (unilateral: 20 percent, 14 of 69; bilateral: 16 percent, six of 38). Cephalometric analysis was performed on all patients with unilateral cleft lip and palate. No significant statistical difference was found in maxillary position or facial proportion. Average age at last follow-up was 20 years (range, 15 years 4 months to 26 years 10 months). CONCLUSIONS:Nasoalveolar molding demonstrates a low rate of soft-tissue revision and alveolar bone grafting, and a low number of total operations per patient from birth to facial maturity. Facial growth analysis at facial maturity in patients who underwent gingivoperiosteoplasty and nasoalveolar molding suggests that this proposal may not hinder midface growth. CLINICAL QUESTION/LEVEL OF EVIDENCE/METHODS:Therapeutic, IV.

ABSTRACT/UNASSIGNED:Velopharyngeal insufficiency (VPI) after cleft palate repair remains an intriguing problem for the cleft surgeon. While other options for the treatment of VPI, in many ways the sphincter pharyngoplasty has become a reliable and satisfying operation. When the applied to the properly selected patient, it rearranges the palatopharyngeus muscles to provide dynamic closure of the newly created central velopharngeal port. The dynamic action is particularly satisfying to the surgeon. The surgery evolved in part because of the dedication and creativity of Dr. Ian Jackson who's description is closest to the design used today. In his memory we felt it fitting to review Dr. Jackson's involvement with the surgery over the decades as well as include our own thoughts on the advantages of the procedure.

OBJECTIVE/UNASSIGNED: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/UNASSIGNED:Retrospective study. METHOD/UNASSIGNED:: ≤-10 mm. PARTICIPANTS/UNASSIGNED:Fifty-one patients with nonsyndromic CLP with hypoplastic maxilla who met inclusion criteria. INTERVENTION/UNASSIGNED:LeFort I advancement. MAIN OUTCOME MEASURE/UNASSIGNED:Skeletal and dental stability post-LeFort I surgery at a 1-year follow-up. RESULTS/UNASSIGNED: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/UNASSIGNED: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.

The aim of this report is to describe the combination of Crouzon syndrome and acanthosis nigricans with fibrous dysplasia of the maxilla. The diagnosis of fibrous dysplasia was confirmed clinically and pathologically during Le Fort III osteotomy and midface advancement with distraction osteogenesis. Crouzon syndrome with acanthosis nigricans is a known syndrome with an incidence of 1:1,000,000. This is the first report in the literature of Crouzon syndrome and acanthosis nigricans combined with fibrous dysplasia. As all 3 pathologies are related to fibroblasts, they may be different manifestations of malfunction of a single molecular pathway. The detection of fibrous dysplasia in a patient with Crouzon syndrome and acanthosis nigricans is important because it may complicate midface osteotomies and fixation of the hardware on the bones during craniofacial surgery.

Ethmoidal dural arteriovenous fistulas are vascular malformations with arterial supply from the anterior ethmoidal artery and ultimate drainage into the sagittal sinus.1-3 They are characterized by a high risk of hemorrhage. Microsurgical disconnection of the fistula represents a safe and robust treatment option. Endovascular treatment requires catheterization of the ophthalmic artery and carries a risk of visual deficits. The supraorbital craniotomy provides an excellent corridor to the anterior skull base and is well suited for the treatment of ethmoidal dural arteriovenous fistulas. The supraorbital craniotomy may be performed through a transpalpebral "eyelid" incision. The transpalpebral incision allows for a well-hidden scar and does not have any associated hair loss, as can be seen with the eyebrow incision. The patient consented to the procedure and being videotaped.

Background/UNASSIGNED:A major challenge in face transplantation (FT) is the limited donor allograft pool. This study aimed to investigate the feasibility of cross-sex FT (CSFT) for donor pool expansion by: (1) comparing craniomaxillofacial metrics following CSFT versus same-sex FT (SSFT); and (2) evaluating the public and medical professionals' perception of CSFT. Methods/UNASSIGNED:Seven cadaveric FTs were performed, resulting in both CSFT and SSFT. Precision of bony and soft tissue inset was evaluated by comparing pre- versus post-operative cephalometric and anthropometric measurements. Fidelity of the FT compared to the virtual plan was assessed by imaging overlay techniques. Surveys were administered to medical professionals, medical students, and general population to evaluate opinions regarding CSFT. Results/UNASSIGNED:< 0.001). On non-blinded and blinded assessments, 62.9% and 79% of responses rated the CSFT superior or equal to SSFT, respectively. Conclusions/UNASSIGNED:Our study demonstrates similar anthropometric and cephalometric outcomes for CSFT and SSFT. Participants were more reticent to undergo CSFT, with increased willingness if supported by research. CSFT may represent a viable option for expansion of the donor pool in future patients prepared to undergo transplantation.

BACKGROUND/UNASSIGNED:The aim of this study is to assess the effect of nasoalveolar molding (NAM) versus no-NAM on nasal morphology in patients with unilateral cleft lip and palate (UCLP) at the time of nasal maturity. METHODS/UNASSIGNED:A retrospective, single-institution review was conducted on all non-syndromic patients with UCLP. Inclusion criteria included age 14 years or above, unilateral cleft repair at the time of infancy, and adequate photography taken at nasal maturity and prior to rhinoplasty. Exclusion criteria included age less than 14 years, syndromic diagnosis, and rhinoplasty prior to nasal maturity. Ten parameters were measured twice from standardized clinical photographs using the Dolphin Imaging Software for establishment of intrarater reliability. Subjective analysis was achieved through completion of the Asher McDade grading scale by 3 expert cleft practitioners. RESULTS/UNASSIGNED:Nostril height, columellar angle, alar cant, vertical alar height, alar height angle, nasofacial angle, and nasolabial angle were found to be significantly less severe in patients who had undergone NAM in conjunction with surgical repair when compared with those who had undergone surgical repair alone. Asher McDade grading revealed significant improvement in nasal form, nasal symmetry/deviation, nasal profile, vermillion border, and overall score in patients who underwent NAM compared to no-NAM. CONCLUSION/UNASSIGNED:The use of presurgical NAM during infancy can improve nasal symmetry and nasal proportions at the time of nasal maturity.

Inflammatory orbital lesions include a broad list of diagnoses, many of them with overlapping clinical and radiographic features. They often present a diagnostic conundrum, even to the most experienced orbital specialist, thus placing considerable weight on surgical biopsy and histopathological analysis. However, histopathological diagnosis is also inherently challenging due to the rarity of these lesions and the overlaps in histologic appearance among distinct disease entities. We herein present the case of an adolescent male with a subacutely progressive orbital mass that generated a significant diagnostic dilemma. Early orbital biopsy was consistent with a benign fibro-inflammatory lesion, but corticosteroid therapy was ineffective in halting disease progression. After an initial substantial surgical debulking, histopathological analysis revealed several key features consistent with IgG4-related disease (IgG4-RD), a systemic fibro-inflammatory process typically accompanied by multifocal tumor-like lesions. Surprisingly, within months, there was clear evidence of clinical and radiographic disease progression despite second-line rituximab treatment, prompting a second surgical debulking. This final specimen displayed distinctive features of Rosai-Dorfman disease (RDD), a systemic inflammatory disease characterized by uncontrolled histiocytic proliferation. Interestingly, certain features of this re-excision specimen were still reminiscent of IgG4-RD, which not only reflects the difficulty in differentiating RDD from IgG4-RD in select cases, but also illustrates that these diagnoses may exist along a spectrum that likely reflects a common underlying pathogenetic mechanism. This case emphasizes the importance of surgical biopsy or resection and histopathological analysis in diagnosing-and, ultimately, treating-rare, systemic inflammatory diseases involving the orbit, and, furthermore, highlights the shared histopathological features between RDD and IgG4-RD.

OBJECTIVE/UNASSIGNED:This systematic review aims to evaluate nasoalveolar molding (NAM) in the context of burden of care defined as physical, psychosocial, or financial burden on caregivers. SEARCH METHODS/UNASSIGNED:Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, 5 databases were searched from inception through December 24, 2019, for keywords and subject headings pertaining to cleft lip and/or palate and NAM. ELIGIBILITY CRITERIA/UNASSIGNED:Clinical studies on NAM with reference to physical (access to care, number of visits, distance traveled), psychosocial (caregiver perceptions, family interactions, breast milk feeding), and financial (direct and indirect costs) burden were included. DATA COLLECTION AND ANALYSIS/UNASSIGNED:Study selection was performed by 2 independent reviewers. RESULTS/UNASSIGNED:The search identified 1107 articles and 114 articles remained for qualitative synthesis. Burden of care domains were discussed but not measured in 43% of articles and only 25% assessed burden of care through a primary outcome. Of these, 20 articles reported on physical, 8 articles on psychosocial, and 12 articles on financial burden. Quality of evidence is limited by study design and risk of bias. CONCLUSION/UNASSIGNED:Nasoalveolar molding has been indiscriminately associated with burden of care in the literature. Although NAM may not be the ideal treatment option for all patients and families, the physical considerations are limited when accounting for the observed psychosocial advantages. Financial burden appears to be offset, but further research is required. Teams should directly assess the impact of this early intervention on the well-being of caregivers and advance strategies that improve access to care.

PURPOSE/OBJECTIVE:Open cranial vault remodeling (CVR) with autologous split calvarial bone grafts redistributes and recontours an abnormal calvarium to create an expanded cranial vault in patients with craniosynostosis. We report a 12-year retrospective review of 162 nonsyndromic patients who underwent operative repair using our previously-described technique which portends excellent surgical outcomes and can be applied to patients of any age group and with any variety of suture fusion. METHODS:Data was gathered on patients who underwent CVR from 2005 to 2016. Surgical records for each patient were analyzed and included operative time, estimated blood loss, and intraoperative transfusion volumes. Intraoperative and postoperative complications, the need for revision surgery, postoperative length of stay, and follow-up records were also reviewed. Syndromic patients were excluded, as well as patients with incomplete data sets. Patients who underwent either anterior or posterior vault remodeling were compared. RESULTS:A total of 162 patients were included in this case series. Patients undergoing anterior CVR were significantly older than those undergoing posterior CVR (13.3 versus 11.0 months, P < 0.015) and also had significantly greater intraoperative red blood transfusion volumes (20.3 versus 15.3cc/kg, P < 0.0207) and longer operative time than posterior CVR patients (274.9 versus 216.7 minutes, P < 0.0001). No patients required reoperation for resorption or recurrence or persistent contour irregularities. There were no visual or neurological complications. Calvarial bone was successfully split in 100% of cases. CONCLUSIONS:This surgical approach to CVR results in good surgical outcomes with a low recurrence rate, while also maximizing operative efficiency, and minimizing total blood loss and transfusion volume. This technique can be applied to any affected suture in a patient with craniosynostosis and in patients of any age group.