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Transpalpebral/Blepharoplasty Incision and Supraorbital Craniotomy for the Treatment of Ethmoidal Dural Arteriovenous Fistulas: A Case Series
Hagstrom, Rory; Nossek, Erez; Rutledge, Caleb W; Ponchione, Elizabeth; Suryadevara, Carter; Kremer, Caroline; Alcon, Andre; Sharashidze, Vera; Shapiro, Maksim; Raz, Eytan; Nelson, Peter K; Staffenberg, David A; Riina, Howard A
BACKGROUND AND OBJECTIVES/OBJECTIVE: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/CONCLUSIONS: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.
PMID: 38376155
ISSN: 2332-4260
CID: 5634152
Predictors of Long-Term Protraction Headgear Success in Patients With Cleft Lip and Palate: A 5-Year Follow-Up
Schechter, Jill; Alcon, Andre; Verzella, Alexandra; Diaz, Allison; Staffenberg, David; Flores, Roberto; Shetye, Pradip R
Patients with cleft lip and palate (CLP) characteristically present with maxillary hypoplasia and class III malocclusion. Protraction headgear (PHG) is a commonly used treatment for this type of malocclusion, with the goal of reducing future surgical needs. The purpose of this study was to evaluate the long-term effects of PHG treatment and determine the pretreatment predictors of long-term PHG success in patients with CLP. Twenty-nine patients with CLP who had undergone PHG treatment from 2012 to 2017 at a single institution were retrospectively analyzed. Patients were included if they had a lateral cephalogram or CBCT before, immediately after, and at least 5 years after their PHG treatment. Patients were divided into surgery and nonsurgery groups based on their 5-year follow-up clinical presentation. Student t tests, Wilcoxon signed-rank test, and a multivariate logistic regression model were used to compare pretreatment and post-treatment changes in both groups. Immediately post-treatment, the maxilla advanced 2.6 mm, the maxillary dentition advanced 4.7 mm, and the mandible rotated downward 5.0 mm and backward 2.6 mm. At long-term follow up the maxilla advanced 0.0 mm while the mandible advanced an additional 7.5 mm. Thus, PHG treatment is effective at improving class III malocclusion in the short term, but may not prevent future surgical need for patients with more severe skeletal discrepancies. Variables predictive of future surgical need include ANB, anterior facial height, overjet, and starting age of treatment, with ANB as the most reliable early predictor.
PMID: 39190786
ISSN: 1536-3732
CID: 5729672
Comparison of the Effects of Postoperative Arm Restraints and Mittens on Cleft Lip Scar Quality after Primary Repair
Verzella, Alexandra N; Laspro, Matteo; Diaz, Allison; Cassidy, Michael F; Park, Jenn; Schechter, Jill; Alcon, Andre; Shetye, Pradip R; Staffenberg, David A; Flores, Roberto L
PMCID:11242569
PMID: 38999195
ISSN: 2077-0383
CID: 5732512
Quantifying Sagittal Lip Changes in Patients with Bilateral Cleft Lip Post Abbe Flap Reconstruction
Muller, John N; Diaz, Allison; Verzella, Alexandra; Staffenberg, David A; Flores, Roberto L
OBJECTIVES/OBJECTIVE:To objectively quantify results of sagittal lip changes following Abbe flap reconstruction in patients with bilateral cleft lip. DESIGN/METHODS:Retrospective, observational study. SETTING/METHODS:Single institution, 8-year retrospective review. PATIENTS/PARTICIPANTS/METHODS:In total, 17 patients with bilateral cleft lip that underwent Abbe flap reconstruction were included in this study. INTERVENTION/METHODS:Patients had lateral photographs taken prior to Abbe flap reconstruction and at least 8 months post-Abbe flap. MAIN OUTCOME MEASUREMENTS/METHODS: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.
PMID: 38835231
ISSN: 1545-1569
CID: 5665272
Same-day Discharge for Cleft Palate Repair: A Single-Surgeon Retrospective Analysis
Diaz, Allison L; Groysman, Leya; Camison, Liliana; Flores, Roberto L; Staffenberg, David A
OBJECTIVE:To evaluate the safety of same-day discharge for patients undergoing primary cleft palate repair. DESIGN/METHODS:Single-surgeon retrospective review. SETTING/METHODS:Tertiary care institution. PATIENTS/PARTICIPANTS/METHODS:40 consecutive patients that underwent primary cleft palate repair by a single surgeon from September 2018 to June 2023. INTERVENTIONS/METHODS:Same-day discharge versus overnight admission after primary palatoplasty. MAIN OUTCOME MEASURES/METHODS:30-day readmission, reoperation, wound and all-cause complication rate and 1-year fistula incidence. RESULTS: < .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.
PMID: 38715425
ISSN: 1545-1569
CID: 5658382
The Burden of Care of Nasoalveolar Molding: An Institutional Experience
Alfonso, Allyson R; Park, Jenn J; Kalra, Aneesh; DeMitchell-Rodriguez, Evellyn M; Kussie, Hudson C; Shen, Chen; Staffenberg, David A; Flores, Roberto L; Shetye, Pradip R
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.
PMID: 38231199
ISSN: 1536-3732
CID: 5737472
Abbe Flap Division Before Two Weeks' Time: Harmful or Advantageous?
Muller, John N; Diaz, Allison; Flores, Roberto L; Staffenberg, David A
INTRODUCTION/BACKGROUND: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/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/CONCLUSIONS: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.
PMID: 38166385
ISSN: 1545-1569
CID: 5736992
Regulatory elements in SEM1-DLX5-DLX6 (7q21.3) locus contribute to genetic control of coronal nonsyndromic craniosynostosis and bone density-related traits
Nicoletti, Paola; Zafer, Samreen; Matok, Lital; Irron, Inbar; Patrick, Meidva; Haklai, Rotem; Evangelista, John Erol; Marino, Giacomo B; Ma'ayan, Avi; Sewda, Anshuman; Holmes, Greg; Britton, Sierra R; Lee, Won Jun; Wu, Meng; Ru, Ying; Arnaud, Eric; Botto, Lorenzo; Brody, Lawrence C; Byren, Jo C; Caggana, Michele; Carmichael, Suzan L; Cilliers, Deirdre; Conway, Kristin; Crawford, Karen; Cuellar, Araceli; Di Rocco, Federico; Engel, Michael; Fearon, Jeffrey; Feldkamp, Marcia L; Finnell, Richard; Fisher, Sarah; Freudlsperger, Christian; Garcia-Fructuoso, Gemma; Hagge, Rhinda; Heuzé, Yann; Harshbarger, Raymond J; Hobbs, Charlotte; Howley, Meredith; Jenkins, Mary M; Johnson, David; Justice, Cristina M; Kane, Alex; Kay, Denise; Gosain, Arun Kumar; Langlois, Peter; Legal-Mallet, Laurence; Lin, Angela E; Mills, James L; Morton, Jenny E V; Noons, Peter; Olshan, Andrew; Persing, John; Phipps, Julie M; Redett, Richard; Reefhuis, Jennita; Rizk, Elias; Samson, Thomas D; Shaw, Gary M; Sicko, Robert; Smith, Nataliya; Staffenberg, David; Stoler, Joan; Sweeney, Elizabeth; Taub, Peter J; Timberlake, Andrew T; Topczewska, Jolanta; Wall, Steven A; Wilson, Alexander F; Wilson, Louise C; Boyadjiev, Simeon A; Wilkie, Andrew O M; Richtsmeier, Joan T; Jabs, Ethylin Wang; Romitti, Paul A; Karasik, David; Birnbaum, Ramon Y; Peter, Inga
PURPOSE/UNASSIGNED:The etiopathogenesis of coronal nonsyndromic craniosynostosis (cNCS), a congenital condition defined by premature fusion of 1 or both coronal sutures, remains largely unknown. METHODS/UNASSIGNED:We conducted the largest genome-wide association study of cNCS followed by replication, fine mapping, and functional validation of the most significant region using zebrafish animal model. RESULTS/UNASSIGNED:intron and enriched in 4 rare risk variants. In zebrafish, the activity of the transfected human eDlx36 enhancer was observed in the frontonasal prominence and calvaria during skull development and was reduced when the 4 rare risk variants were introduced into the sequence. CONCLUSION/UNASSIGNED:Our findings support a polygenic nature of cNCS risk and functional role of craniofacial enhancers in cNCS susceptibility with potential broader implications for bone health.
PMCID:11434253
PMID: 39345948
ISSN: 2949-7744
CID: 5803132
Clinical Outcomes of Bilateral Cleft Lip and Palate Repair with Nasoalveolar Molding and Gingivoperiosteoplasty to Facial Maturity
Rochlin, Danielle H; Park, Jenn; Parsaei, Yassmin; Kalra, Aneesh; Staffenberg, David A; Cutting, Court B; Grayson, Barry H; Shetye, Pradip R; Flores, Roberto L
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.
PMID: 36943703
ISSN: 1529-4242
CID: 5590622
LeFort I Horizontal Osteotomy: Defining the Feasibility of the "High Osteotomy"
Verzella, Alexandra N; Alcon, Andre; Schechter, Jill; Shetye, Pradip R; Staffenberg, David A; Flores, Roberto L
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.
PMID: 37885216
ISSN: 1545-1569
CID: 5614352