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PURPOSE: The pediatric craniofacial trauma literature is limited to single institutions or short study periods. Herein, this study analyzes a national database over 10 years to delineate the epidemiology of pediatric craniofacial fractures and to identify risk factors for acute-level hospital course in the largest series to date. METHODS: Utilizing the National Trauma Data Bank, pediatric craniofacial fractures admitted between 2010 and 2019 were identified. Descriptive analyses and multivariable regression were performed to identify risk factors for acute-level hospital course. RESULTS: A total of 155,136 pediatric craniofacial fracture cases were reviewed, including cranial vault (49.0%), nasal (22.4%), midface (21.0%), mandibular (20.2%), and orbital floor fractures (13.7%). Midface and orbital floor fractures occurred commonly as multicraniofacial fractures. Cranial vault fractures were the most common among all age groups, but frequency declined with age. In contrast, facial fractures increased with age. Despite the inherent complexity of multicraniofacial trauma, isolated fractures remained a concern for acute-level hospital course.Cranial vault and midface fractures had an increased risk of intracranial injury and intensive care unit admission (P<0.001). Mandibular and midface fractures had an increased risk for cervical spine fracture and tracheostomy (P<0.001). Patient and injury-specific risk factors among the fractures with the strongest association for each outcome-cranial vault and mandible-were identified. CONCLUSIONS: The inherent limitations of prior studies-geographical biases, small cohorts, and short-term study periods-were addressed. Describing the independent contribution of each craniofacial fracture to the risk of acute-level hospital course outcomes can be employed to better optimize risk stratification, counseling, and management.
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Fracturas Craneales , Humanos , Factores de Riesgo , Niño , Masculino , Femenino , Fracturas Craneales/epidemiología , Preescolar , Adolescente , Lactante , Bases de Datos Factuales , Estudios Retrospectivos , Estados Unidos/epidemiología , Huesos Faciales/lesiones , Hospitalización/estadística & datos numéricos , Traqueostomía/estadística & datos numéricosRESUMEN
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.
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OBJECTIVES: To objectively quantify results of sagittal lip changes following Abbe flap reconstruction in patients with bilateral cleft lip. DESIGN: Retrospective, observational study. SETTING: Single institution, 8-year retrospective review. PATIENTS/PARTICIPANTS: In total, 17 patients with bilateral cleft lip that underwent Abbe flap reconstruction were included in this study. INTERVENTION: Patients had lateral photographs taken prior to Abbe flap reconstruction and at least 8 months post-Abbe flap. MAIN OUTCOME MEASUREMENTS: 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.
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OBJECTIVE: To evaluate the safety of same-day discharge for patients undergoing primary cleft palate repair. DESIGN: Single-surgeon retrospective review. SETTING: Tertiary care institution. PATIENTS/PARTICIPANTS: 40 consecutive patients that underwent primary cleft palate repair by a single surgeon from September 2018 to June 2023. INTERVENTIONS: Same-day discharge versus overnight admission after primary palatoplasty. MAIN OUTCOME MEASURES: 30-day readmission, reoperation, wound and all-cause complication rate and 1-year fistula incidence. RESULTS: Of 40 total cases, 20 patients were discharged on the same calendar day and 20 patients were admitted for overnight stay following primary cleft palate repair. In the same-day discharge group, readmission incidence was 10%(nâ =â 2), wound complication incidence was 5%(nâ =â 1), and postoperative complication incidence was 15%(nâ =â 3). In comparison, patients admitted overnight had a readmission incidence of 5%(nâ =â 1, Pâ =â 1.00), wound complication incidence of 10%(nâ =â 2, Pâ =â 1.00), and postoperative complications of 20%(nâ =â 4, Pâ =â 1.00) No patients had 30-day reoperations or fistulas at 1 year. A higher proportion of admitted patients held a preoperative diagnosis of unilateral cleft palate and alveolus (Veau 3) as compared to patients discharged on the same day (Pâ =â .019). During the postoperative hospital course, admitted patients received significantly more oxycodone at median of 2 doses (IQR 1.00-3.75) and acetaminophen at a median of 4 doses (IQR 3.00-5.00) than patients with same-day discharge with a median of 1 dose (IQR 0.00 -1.00, P < .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.
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INTRODUCTION: 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: 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: 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.
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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.
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Fisura del Paladar , Fístula , Procedimientos de Cirugía Plástica , Humanos , Lactante , Fisura del Paladar/cirugía , Fisura del Paladar/complicaciones , Colgajos Quirúrgicos , Paladar Blando/cirugía , Fístula/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Pharmacologic agents are often used in the antepartum period, however, studies on their effect on fetal development are limited. Thus, this study aims to examine the effect of commonly prescribed antepartum medications on the development of orofacial clefting. METHODS: Utilizing EPIC Cosmos deidentified data from approximately 180 US institutions was queried. Patients born between January 1, 2013, to January 1, 2023, were included. Eight OC cohorts were identified. Gestational medication use was identified by medications prescribed, provider-administered, or reported use by mothers. Medications used in at least 1 in 10,000 pregnancies were included in this analysis. RESULTS: A total of 12â 098 newborns with available maternal pharmacologic data were born with any type of orofacial clefting. Prevalence for all oral clefts, any cleft palate, and any cleft lip were 20.56, 18.10, and 10.60 per 10â 000 individuals, respectively. Notable significant exposures include most anticonvulsants, such as lamotrigine (OR1.33, CI 1.10-1.62), and topiramate (OR1.35, CI 1.13-1.62), as well as nearly all SSRIs/SNRIs, including fluoxetine (OR1.34, CI 1.19-1.51), sertraline (OR1.25, CI 1.16-1.34), and citalopram (OR1.28, CI 1.11-1.47). Corticosteroids were also correlated including dexamethasone (OR1.19, CI 1.12-1.27), and betamethasone (OR1.64, CI 1.55-1.73), as were antibiotics, including amoxicillin (OR1.22, CI 1.14-1.30), doxycycline (OR1.29, CI 1.10-1.52), and nitrofuran derivatives (OR1.10, CI 1.03-1.17). CONCLUSION: New associations between commonly prescribed antepartum medications and orofacial clefting were found. These findings should be confirmed as causality is not assessed in this report. Practitioners should be aware of the potential increased risk associated with these medications.
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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: Of 230 eligible patients, 61 patients (27%) did not undergo or complete NAM (no-NAM). In this group, 37 (60.7%) received no presurgical intervention, 12 (19.7%) received presurgical nostril retainers, 3 (4.9%) received lip taping, 1 (1.6%) received a combination of taping/nostril retainers, and 8 (13.1%) discontinued NAM. The most common reasons for not receiving NAM were sufficiently aligned cleft alveolus (21.3%), medical complexity (16.4%), late presentation (16.4%), and alveolar notching (18%). Compared to the NAM group, the no-NAM group had significantly lower rates of prenatal cleft diagnosis/consult, and significantly higher proportion of non-married and non-English speaking caregivers. Multivariable analysis controlling for insurance type, primary language, prenatal consult, marital status, and age at first appointment found that age at first appointment is the only statistically significant predictor of NAM utilization (P < .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.
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Labio Leporino , Fisura del Paladar , Humanos , Lactante , Labio Leporino/cirugía , Nariz/cirugía , Fisura del Paladar/cirugía , Modelado Nasoalveolar , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: The effectiveness of virtual-reality (VR) simulation-based training in cleft surgery has not been tested. The purpose of this study was to evaluate learners' acceptance of VR simulation in airway management of a pediatric patient post-cleft palate repair. METHODS: This VR simulation was developed through collaboration between BioDigital and Smile Train. 26 medical students from a single institution completed 10â min of standardized VR training and 5â min of standardized discussion about airway management post-cleft palate repair. They spent 4-8â min in the VR simulation with guidance from a cleft surgery expert. Participants completed pre- and post-surveys evaluating confidence in using VR as an educational tool, understanding of airway management, and opinions on VR in surgical education. Satisfaction was evaluated using a modified Student Evaluation of Educational Quality questionnaire and scored on a 5-point Likert scale. Wilcoxon signed-rank tests were performed to evaluate responses. RESULTS: There was a significant increase in respondents' confidence using VR as an educational tool and understanding of airway management post-cleft palate repair after the simulation (P < .001). Respondents' opinions on incorporating VR in surgical education started high and did not change significantly post-simulation. Participants were satisfied with VR-based simulation and reported it was stimulating (4.31 ± 0.88), increased interest (3.77 ± 1.21), enhanced learning (4.12 ± 1.05), was clear (4.15 ± 0.97), was effective in teaching (4.08 ± 0.81), and would recommend the simulation (4.2 ± 1.04). CONCLUSION: VR-based simulation can significantly increase learners' confidence and skills in airway management post-cleft palate repair. Learners find VR to be effective and recommend its incorporation in surgical education.
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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.
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Craneosinostosis , Ratones , Animales , Craneosinostosis/genética , Craneosinostosis/metabolismo , Mutación , Transducción de Señal/genética , Factores de Transcripción/genética , Diferenciación Celular , Factores de Transcripción NFI/genética , Factores de Transcripción NFI/metabolismoRESUMEN
INTRODUCTION: 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: 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.
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Huesos Faciales , Osteotomía Le Fort , Humanos , Adolescente , Adulto Joven , Adulto , Osteotomía Le Fort/métodos , Huesos Faciales/cirugía , Cara , Cefalometría , Estudios Retrospectivos , Resultado del Tratamiento , Maxilar/cirugíaRESUMEN
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 19â 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 10â mm, for a 20â 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.
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Síndrome de Beckwith-Wiedemann , Maloclusión de Angle Clase III , Maloclusión , Procedimientos Quirúrgicos Ortognáticos , Humanos , Síndrome de Beckwith-Wiedemann/diagnóstico por imagen , Osteotomía Le Fort/métodos , Maloclusión de Angle Clase III/terapia , Maloclusión de Angle Clase III/cirugía , Procedimientos Quirúrgicos Ortognáticos/métodos , Faringe , Mandíbula/cirugía , Maxilar/cirugía , Cefalometría/métodosRESUMEN
OBJECTIVE: To define "high osteotomy" and determine the feasibility of performing this procedure. DESIGN: Single institution, retrospective review. SETTING: Academic tertiary referral hospital. PATIENTS, PARTICIPANTS: 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: Single jaw one-piece Le Fort I advancement surgery. MAIN OUTCOME MEASURES: 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 2.63â 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 12.17â mm (95% CI 4.00-20.34) and 14.80â mm (95% CI 4.61-24.98), respectively. To complete a "high osteotomy," with 95% confidence, the cut should be 20.36â 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.
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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: 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: 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: 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.
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Rinoplastia , Adulto , Humanos , Niño , Rinoplastia/métodos , Tabique Nasal/cirugía , Resultado del Tratamiento , Cartílago , Vómer/cirugíaRESUMEN
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.
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Injerto de Hueso Alveolar , Fisura del Paladar , Animales , Niño , Humanos , Proceso Alveolar/cirugía , Trasplante Óseo/métodos , Fisura del Paladar/cirugía , Osteogénesis , Ingeniería de Tejidos/métodos , Guías de Práctica Clínica como AsuntoRESUMEN
OBJECTIVES: 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: This is a single-institution, retrospective study. PATIENTS/PARTICIPANTS: 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: Ambulatory surgery protocol in 2016. OUTCOME MEASURES: 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 24â h pre-protocol to 8â 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.
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Labio Leporino , Humanos , Labio Leporino/cirugía , Estudios Retrospectivos , Tiempo de Internación , Complicaciones Posoperatorias/epidemiología , Resultado del TratamientoRESUMEN
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.
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Craniosynostosis (CS) is a frequent congenital anomaly featuring the premature fusion of 1 or more sutures of the cranial vault. Syndromic cases, featuring additional congenital anomalies, make up 15% of CS. While many genes underlying syndromic CS have been identified, the cause of many syndromic cases remains unknown. We performed exome sequencing of 12 syndromic CS cases and their parents, in whom previous genetic evaluations were unrevealing. Damaging de novo or transmitted loss of function (LOF) mutations were found in 8 genes that are highly intolerant to LOF mutation (P = 4.0 × 10-8); additionally, a rare damaging mutation in SOX11, which has a lower level of intolerance, was identified. Four probands had rare damaging mutations (2 de novo) in TFAP2B, a transcription factor that orchestrates neural crest cell migration and differentiation; this mutation burden is highly significant (P = 8.2 × 10-12). Three probands had rare damaging mutations in GLI2, SOX11, or GPC4, which function in the Hedgehog, BMP, and Wnt signaling pathways; other genes in these pathways have previously been implicated in syndromic CS. Similarly, damaging de novo mutations were identified in genes encoding the chromatin modifier KAT6A, and CTNNA1, encoding catenin α-1. These findings establish TFAP2B as a CS gene, have implications for assessing risk to subsequent children in these families, and provide evidence implicating other genes in syndromic CS. This high yield indicates the value of performing exome sequencing of syndromic CS patients when sequencing of known disease loci is unrevealing.
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Craneosinostosis/genética , Glipicanos/genética , Histona Acetiltransferasas/genética , Mutación , Proteínas Nucleares/genética , Factores de Transcripción SOXC/genética , Factor de Transcripción AP-2/genética , Proteína Gli2 con Dedos de Zinc/genética , alfa Catenina/genética , Adolescente , Niño , Preescolar , Craneosinostosis/diagnóstico , Craneosinostosis/patología , Exoma , Femenino , Expresión Génica , Humanos , Masculino , Linaje , Medición de Riesgo , Transducción de Señal , Cráneo/anomalías , Cráneo/crecimiento & desarrollo , Cráneo/metabolismo , Secuenciación del ExomaRESUMEN
OBJECTIVE: 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: Retrospective study. METHOD: Lateral cephalograms were digitized at preoperative (T1), immediately postoperative (T2), and 1-year follow-up (T3) and compared to untreated unaffected controls. Based on the severity of cleft maxillary hypoplasia, the sample was divided into 3 groups using Wits analysis: mild: ≤0 to ≥-5 mm; moderate: <-5 to >-10 mm; and severe: ≤-10 mm. PARTICIPANTS: Fifty-one patients with nonsyndromic CLP with hypoplastic maxilla who met inclusion criteria. INTERVENTION: LeFort I advancement. MAIN OUTCOME MEASURE: Skeletal and dental stability post-LeFort I surgery at a 1-year follow-up. RESULTS: 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: 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.
Asunto(s)
Labio Leporino , Fisura del Paladar , Osteogénesis por Distracción , Cefalometría , Labio Leporino/cirugía , Fisura del Paladar/cirugía , Humanos , Maxilar/cirugía , Osteotomía Le Fort , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: Utilize 3-dimensional (3D) photography to evaluate the nasolabial changes in infants with bilateral cleft lip and palate (BCLP) who underwent nasoalveolar molding (NAM) and primary reconstructive surgery. DESIGN: This is a retrospective serial longitudinal study of consecutively enrolled infants from September 2012 to July 2016 with BCLP who underwent NAM before primary lip and nose reconstructive surgery. It included infants who had digital 3dMD stereophotogrammetry records at initial presentation (T1), completion of NAM (T2), and 3 weeks following primary repair (T3). Twelve infants fulfilled the inclusion criteria. 3dMD Vultus software was used to orient images and plot 16 nasolabial points with x, y, z coordinates to obtain the linear and angular measurements. Nasal form changes were measured and analyzed between T1 (0.5 months old), T2 (5 months old), and T3 (6 months old). Intraclass correlation coefficient was performed for intrarater reliability. Averaged data from the 3D images was statistically analyzed from T1 to T2 and T2 to T3 with Wilcoxon tests. Unaffected infant norms from the Farkas publication were used as a control sample. RESULTS: After NAM therapy, statistically significant changes in the position of subnasale and labius superius improved nasolabial symmetry. Both retruded after NAM were displaced downward after NAM and surgical correction with respect to soft tissue nasion. The nasal tip's projection was maintained with NAM and surgical correction. The columella lengthened from 1.4 to 4.71 mm following NAM. CONCLUSIONS: There was a significant improvement in the nasolabial anatomy after NAM, and this was further enhanced after primary reconstructive surgery.