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1.
J Plast Reconstr Aesthet Surg ; 73(8): 1499-1505, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32546424

RESUMEN

BACKGROUND: Complex spine surgery in patients with major comorbidities leads to increased need for midline back wound reconstruction by plastic surgeons. Literature suggests that back wound reconstruction concurrent with high-risk immediate/index spine surgery may lead to fewer complication. This study aimed to validate this claim in a large cohort treated at a tertiary center. We hypothesize that immediate reconstruction may lead to fewer adverse events in comparison to delayed reconstruction. METHODS: This was a retrospective single-center review of 659 patients who underwent spinal surgery with/without reconstruction by plastic surgeons between November 2011 and December 2015. Three main cohorts were evaluated: patients who underwent spinal surgery with no reconstruction, patients with delayed reconstruction after spine surgery, and patients with immediate back wound reconstruction with index spine surgery. Demographic, clinical, and outcomes data were collected from electronic medical records. Primary endpoints were the incidence of any complications such as dehiscence, infection, seroma/hematoma, and exposed hardware. The secondary endpoint was return to the operating room and most recent follow-up. RESULTS: Forty-three patients underwent index reconstruction (follow-up 25.3 ± 12.7 months), 33 were delayed (follow-up 23.7 ± 12.5 months), and 583 had no reconstruction (follow-up 22.1 ± 15.2 months). Patients who underwent index reconstruction had more spinal levels involved than delayed reconstruction (7.8 ± 0.75 vs 5.6 ± 0.68; p = 0.03). The overall complications rate was 7.7%, most commonly wound dehiscence (2.7%), infections (0.9%), exposed hardware (2.0%), cerebrospinal fluid leaks (0.6%), and return to OR (3.8%). Patients who underwent index spinal wound reconstruction had a significantly lower complication rate (4.65%) than secondary spinal surgery patients (27.3%; p = 0.048). CONCLUSIONS: The data confirmed significantly decreased complication rates for index back wound reconstructions for high-risk patients compared to delayed spine wound reconstruction. Increased rates of wound dehiscence, exposed hardware, and revisions occurred with delayed reconstruction. Early employment of tension free, robust vascular flap closure may attribute to a decreased complication profile.

2.
Plast Reconstr Surg ; 145(5): 1241-1248, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32332546

RESUMEN

BACKGROUND: Reports of neurodevelopmental delays in adolescents with metopic craniosynostosis have ranged from 15 to 61 percent. Previously, event-related potentials have correlated preoperative radiographic severity with language deficiencies in infancy. This study sought to characterize neurocognitive testing at cranial maturity and correlate outcomes to preoperative radiographic severity. METHODS: Patients diagnosed with metopic craniosynostosis who underwent surgical correction in infancy completed a neurodevelopmental battery evaluating age-normalized intelligence quotient, academic achievement, and visuomotor integration. Data were stratified by preoperative endocranial bifrontal angle (moderate, >124 degrees; severe, <124 degrees). Multiple variable regression was used to control measured intelligence and achievement for age at surgery, age at testing, parental education, and income. Significance was set at p < 0.05. RESULTS: Twenty patients completed neurodevelopmental testing. Mean intelligence quotient was 111.7 ± 13 and academic achievement was similar to national averages (word reading, 53.4 percent; reading comprehension, 53.4 percent; reading composite, 53.5 percent; spelling, 44 percent; and math, 52.9 percent). Radiographic measurements revealed 36 percent of patients with moderate phenotype and 64 percent with severe. Patients with severe phenotypes had lower intelligence quotient measures and scored more poorly in every academic measure tested. Word reading (113 versus 95; p = 0.035) and reading composite (109 versus 98; p = 0.014) reached significance. CONCLUSIONS: Overall, cranial mature patients with metopic craniosynostosis had above average intelligence quotient and academic achievement near the national mean. Long-term neurocognitive function was correlated to preoperative radiographic severity in metopic craniosynostosis, with more severe cases performing worse. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.


Asunto(s)
Éxito Académico , Craneosinostosis/diagnóstico , Discapacidades del Desarrollo/diagnóstico , Procedimientos Quirúrgicos Reconstructivos , Cráneo/diagnóstico por imagen , Adolescente , Niño , Craneosinostosis/complicaciones , Craneosinostosis/cirugía , Discapacidades del Desarrollo/etiología , Discapacidades del Desarrollo/prevención & control , Femenino , Humanos , Lactante , Pruebas de Inteligencia , Masculino , Periodo Preoperatorio , Radiografía , Índice de Severidad de la Enfermedad , Resultado del Tratamiento
3.
J Craniofac Surg ; 31(4): 1000-1005, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32176017

RESUMEN

BACKGROUND: Despite surgical correction of unilateral craniosynostosis (ULC), complex cranial base angulation can result in partial reversion to preoperative deformity with growth and time. Using 3-dimensional imaging, dysmorphic facial features of ULC in school-age patients were quantified and related to how they contribute to overall facial asymmetry and patient-reported outcomes. METHODS: Children who underwent surgical correction of ULC were recruited from Yale University and Children's Hospital of Philadelphia. The 3D photographs were analyzed utilizing a Procrustes analysis of shape. Pearson's correlation was used to determine dysmorphic features' impact on overall asymmetry. Patients were stratified into "moderate" and "severe" asymmetry. Finally, asymmetry was correlated to patient-reported outcome scores. Statistical analysis was performed with SPSS-25 with P < 0.05 as statistically significant. RESULTS: Twenty-one patients were included with average age at analysis of 12.3 years. Fifty-seven percent of patients had right-sided fusion. The overall Procrustes analysis indicated a root mean square difference of 2.21 mm. Pearson's correlation indicated that the facial middle 3rd (P ≤ 0.001), orbital dystopia (P < 0.001), chin point deviation (P = 0.011), and nasal root angulation (P = 0.019) contributed most to overall asymmetry. Patients in the severe asymmetry cohort had greater facial middle-third asymmetry (P < 0.001) and orbital dystopia (P < 0.001). Asymmetry did not correlate with patient-reported outcomes. CONCLUSION: Patients with ULC have persistent facial asymmetry at school-age with the greatest levels of asymmetry in the facial middle-third, orbit, and nasal root. Beyond the cranial dysmorphology, initial skull base angulation in unilateral coronal craniosynostosis manifests in long-term mid and lower-third facial asymmetry.

4.
Plast Reconstr Surg ; 2020 Feb 06.
Artículo en Inglés | MEDLINE | ID: mdl-32039968

RESUMEN

BACKGROUND: Reports of neurodevelopmental delays in adolescents with metopic craniosynostosis (MC) have ranged from 15-61%. Previously, event-related potentials have correlated pre-operative radiographic severity with language deficiencies in infancy. This study sought to characterize neurocognitive testing at cranial maturity and correlate outcomes to pre-operative radiographic severity. MATERIALS AND METHODS: Patients diagnosed with MC who underwent surgical correction in infancy completed a neurodevelopmental battery evaluating age-normalized intelligence quotient, academic achievement, and visual-motor integration. Data was stratified by pre-operative endocranial bifrontal angle (>124°: "Moderate"; <124°: "Severe"). Multiple variable regression was used to control measured intelligence and achievement for age at surgery, age at testing, parental education, and income. Significance was set at p < 0.05. RESULTS: Twenty patients completed neurodevelopmental testing. Mean intelligence quotient was 111.7 ± 13 and academic achievement was similar to national averages (53.4%, word reading; 53.4%, reading comprehension; 53.5%, reading composite; 44%, spelling; and 52.9% math). Radiographic measurements revealed 36% of patients with moderate phenotype and 64% were severe. Patients with severe phenotypes had lower I.Q. measures and scored more poorly in every academic measure tested. Word Reading (113 vs. 95; p=0.035) and reading composite (109 vs. 98; p=0.014) reached significance. CONCLUSION: Overall, cranial mature patients with MC had above average IQ and academic achievement near the national mean. Long-term neurocognitive function was correlated to pre-operative radiographic severity in MC with more severe cases performing worse.

5.
J Craniofac Surg ; 31(3): 678-684, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32068731

RESUMEN

BACKGROUND: Patients with Crouzon syndrome develop various types of anatomic deformities due to different forms of craniosynostosis, yet they have similar craniofacial characteristics. However, exact homology is not evident. Different pathology then may be best treated by different forms of surgical technique. Therefore, precise classification of Crouzon syndrome, based on individual patterns of cranial suture involvement is needed. METHODS: Ninety-five computed tomography (CT) scans (Crouzon, n = 33; control, n = 62) were included in this study. All the CT scans are divided into 4 types based on premature closure of sutures: class I = coronal and lambdoidal synostosis; class II = sagittal synostosis; class III = pansynostosis; and class IV = "Others." The CT scan anatomy was measured by Materialise software. RESULTS: The class III, pansynostosis, is the most prevalent (63.6%). The classes I, III, and IV of Crouzon have significantly shortened entire anteroposterior cranial base length, with the shortest base length in class III. The external cranial measurements in class I show primarily a decreased posterior facial skeleton, while the class III presented with holistic facial skeleton reduction. Class II has the least severe craniofacial malformations, while class III had the most severe. CONCLUSION: The morphology of patients with Crouzon syndrome is not identical in both cranial base and facial characteristics, especially when they associated with different subtypes of cranial suture synostosis. The classification of Crouzon syndrome proposed in this study, summarizes the differences among each subgroup of craniosynostosis suture involvement, which, theoretically, may ultimately influence both the timing and type of surgical intervention.

6.
Plast Reconstr Surg ; 145(1): 117e-125e, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31592950

RESUMEN

BACKGROUND: De novo or rare transmitted mutations in the SMAD6 gene affect 7 percent of midline nonsyndromic synostosis patients. This study aimed to determine the neurocognitive sequelae of SMAD6 synostosis. METHODS: Nonsyndromic synostosis patients 6 years or older with SMAD6 mutations and non-SMAD6 nonsyndromic synostosis controls were recruited. All patients completed a double-blinded neurodevelopmental battery (i.e., Wechsler Fundamentals, Wechsler Abbreviated Scale of Intelligence, Beery-Buktenica Developmental test), and parents/guardians completed behavioral surveys (Behavior Rating Inventory of Executive Function and Behavior Rating System for Children). RESULTS: Twenty-eight patients participated: 10 known SMAD6 patients (average age, 10 years; 1 female; eight metopic and two sagittal; nine treated with cranial vault remodeling and one treated with strip craniectomy) and 18 non-SMAD6 controls (age, 9.5 years; three female; 12 metopic and six sagittal; 17 treated with cranial vault remodeling and one treated with strip craniectomy). There were no differences between any demographics. Testing age, surgical age, parental education, and household income correlated with cognition (p < 0.05). After controlling for these factors, SMAD6 patients performed worse on numerical operations (p = 0.046), performance intelligence quotient (p = 0.018), full-scale intelligence quotient (p = 0.010), and motor coordination (p = 0.043) compared to age/race/gender/synostosis/operation-matched controls. On behavioral surveys, SMAD6 patients scored worse on 14 assessments, including aggression, communication, and behavior. CONCLUSIONS: This prospective double-blinded study revealed that neuropsychiatric development of nonsyndromic synostosis may be under genetic control. SMAD6 mutations led to poorer mathematics, performance intelligence quotient, full-scale intelligence quotient, and motor coordination, even after controlling for exogenous factors. Genetic testing may be critical for advocating early adjunctive neurodevelopmental therapy. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

7.
Plast Reconstr Surg ; 145(1): 80-83, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31881606

RESUMEN

Facial aging patients frequently seek improved neck and jawline definition. Microgenia, or lack of chin support, is often overlooked or underdiagnosed in these patients. The authors propose a simultaneous genioplasty, in addition to platysmaplasty and face/neck lift, to address both soft-tissue and bony components, and enhance the cervicomental area. The platysmaplasty is performed in a novel fashion using the intraoral genioplasty incision. The operative sequence includes intraoral incision, platysmal exposure, fat excision, platysmal tightening, genioplasty osteotomy/fixation, and external skin redraping (face/neck lift). Case examples are shown to illustrate appropriate candidate selection and results. Parameters for success include increased chin-throat distance and definition, improved cervicomental angle, and soft-tissue rejuvenation. The novel approach the authors describe enables discrete access to the platysmal and submental region and provides bony definition to help optimize the soft-tissue drape and definition. CLINICAL QUESTION/LEVEL OF EVIDENCE:: Therapeutic, V.


Asunto(s)
Mentoplastia/métodos , Boca/cirugía , Ritidoplastia/métodos , Anciano , Femenino , Humanos , Persona de Mediana Edad , Cuello/cirugía , Resultado del Tratamiento
8.
Plast Reconstr Surg ; 144(6): 1371-1383, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-31764655

RESUMEN

BACKGROUND: Nonsyndromic craniosynostosis may manifest with complex behavioral, attentional, and emotional sequelae. The authors characterized higher level brain connectivity in adolescent nonsyndromic craniosynostosis patients in response to emotional frustration. METHODS: Surgically corrected patients older than 9 years with nonsyndromic craniosynostosis were age/sex/handedness matched to controls. Patients participated in a "go/no-go" task, structured as win/lose/recovery paradigms. BioImage Suite was used to analyze whole-brain intrinsic connectivity between tasks with cluster-corrected group-level t maps. A value of p < 0.05 was significant. RESULTS: Seven unilateral coronal (average age, 12.2 years), six metopic (average age, 11.5 years), and controls were included. Unilateral coronal had worse emotional regulation scores on the Behavior Rating Inventory of Executive Function survey (p = 0.065) and performed poorly on the go/no-go task (p < 0.001). Metopic had four regions of interest, with the majority having decreased activity compared with controls, and few differences between tasks. Unilateral coronal patients had 11 regions of interest; the majority decreased during the win and lose conditions, but all increased during the recovery condition. Metopic patients had decreased blood oxygenation level- dependent signal in the posterior cingulate (p = 0.017) and middle temporal gyrus (p = 0.042). Unilateral coronal had decreased signal in the posterior cingulate (p = 0.023), middle temporal gyrus (p = 0.027), and thalamus (p = 0.033), but increased signal in the cuneus (p = 0.009) and cerebellum (p = 0.009). Right unilateral coronal, but not metopic/controls, had increased right brain activity in the caudate (p = 0.030), thalamus (p = 0.011), temporal lobe (p = 0.012), and cerebellum (p = 0.029). CONCLUSIONS: Unilateral coronal patients may have emotional dysregulation in response to frustration, whereas metopic patients may have attenuated emotional reactions. Evidence of right unilateral coronal brain laterality suggests that the area of suture fusion may contribute to the mechanism of dysfunction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.


Asunto(s)
Craneosinostosis/psicología , Regulación Emocional/fisiología , Frustación , Estudios de Casos y Controles , Niño , Craneosinostosis/sangre , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Oxígeno/sangre , Estudios Prospectivos , Pruebas Psicológicas
9.
Proc Natl Acad Sci U S A ; 116(30): 15116-15121, 2019 07 23.
Artículo en Inglés | MEDLINE | ID: mdl-31292255

RESUMEN

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.


Asunto(s)
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 Exoma Completo
10.
Plast Reconstr Surg Glob Open ; 7(4): e2222, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-31321193

RESUMEN

Harvest of the deep inferior epigastric vessels for microsurgical breast reconstruction can be complicated by an intricate and lengthy subfascial dissection. Although multiple preoperative imaging modalities exist to help visualize the vascular anatomy and assist in perforator selection, few can help clearly define the intramuscular course of these vessels. The authors introduce their early experience with 3D-printed anatomical modeling (to-scale) of the infraumbilical course of the deep inferior epigastric subfascial vascular tree to better assist in executing the intramuscular dissection.

11.
Plast Reconstr Surg ; 144(1): 89e-97e, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31246828

RESUMEN

BACKGROUND: Virtual surgical planning has facilitated preoperative planning, splint accuracy, and intraoperative efficiency in orthognathic surgery. The translation of the virtual surgical plan to the actual result has not been adequately examined. The authors examined the conformity of the virtual surgical plan to the postoperative result. They hypothesize that the greatest conformity exists in the anteroposterior dimensions. METHODS: The authors examined patients who underwent Le Fort I maxillary advancement, bilateral sagittal split osteotomy, and genioplasty. The preoperative virtual surgical planning file and postoperative cone beam computed tomographic scan were registered in Mimics using unchanged landmarks. The conformity to the virtual surgical plan was quantified using linear and angular measurements between bone surface landmarks. Results were compared using t tests, with p < 0.05 considered statistically significant RESULTS:: One hundred patients who underwent Le Fort I maxillary advancement, bilateral sagittal split osteotomy, and genioplasty were included. Three-dimensional analysis showed significant differences between the plan and outcome for the following landmarks: A point (y, p = 0.04; z, p = 0.04), B point (y, p = 0.02; z, p = 0.02), pogonion (y, p = 0.04), menton (x, p = 0.02; y, p = 0.01; z, p = 0.03), and anterior nasal spine (x, p = 0.04; y, p = 0.04; z, p = 0.01). Angular measurements sella-nasion-A point, sella-nasion-B point, and A point-nasion-B point were not statistically different. CONCLUSIONS: There is a high degree of conformity comparing the orthognathic virtual surgical plan to the actual postoperative result. However, some incongruency is seen vertically (maxilla) and sagittally (mandible, chin). Departures of the actual position compared with the plan could be the result of condylar position changes, osteotomy locations, aesthetic intraoperative decisions, and/or play in the system.


Asunto(s)
Registro de la Relación Maxilomandibular/métodos , Procedimientos Quirúrgicos Ortognáticos/métodos , Planificación de Atención al Paciente , Adolescente , Adulto , Puntos Anatómicos de Referencia , Femenino , Humanos , Procesamiento de Imagen Asistido por Computador/métodos , Imagenología Tridimensional/métodos , Masculino , Mandíbula/cirugía , Maxilar/cirugía , Persona de Mediana Edad , Cirugía Asistida por Computador/métodos , Adulto Joven
12.
J Craniofac Surg ; 30(4): 1201-1205, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31166266

RESUMEN

BACKGROUND: High volume centers (HVC) is commonly associated with increased resources and improved patient outcomes. This study assesses efficacy and outcomes of high volume centers in cleft palate repair. METHODS: Cleft palate procedures were identified in the Kids' Inpatient Database from 2003-2009. Demographics, perioperative factors, co-morbidities, and complications in HVC (90th percentile, >48 cases/year) and non-high volume centers (NHVC) were compared across various cohorts of cleft repair. RESULTS: Four thousand five hundred sixty-three (61.7%) total cleft palate surgeries were performed in HVC and 3388 (38.3%) were performed in NHVC. The NHVC treated a higher percentage of Medicaid patients (P = 0.005) and patients from low-income quartiles (P = 0.018). HVC had larger bedsizes (P <0.001), were more often government/private owned (P <0.001), and were more often teaching hospitals (P <0.001) located predominantly in urban settings (P <0.001). The HVC treated patients at younger ages (P = 0.008) and performed more concurrent procedures (P = 0.047). The most common diagnosis at HVC was complete cleft palate with incomplete cleft lip, while the most common diagnosis at NHVC was incomplete cleft palate without lip. Overall, length of stay and specific complication rates were lower in HVC (P = 0.048, P = 0.042). Primaries at HVCs showed lower pneumonia (P = 0.009) and specific complication rates (P = 0.023). Revisions at HVC were associated with older patients, fewer cardiac complications (P = 0.040), less wound disruption (P = 0.050), but more hemorrhage (P = 0.040).


Asunto(s)
Fisura del Paladar/cirugía , Hospitales de Alto Volumen/estadística & datos numéricos , Labio Leporino/cirugía , Fisura del Paladar/economía , Bases de Datos Factuales , Femenino , Hospitales de Enseñanza/estadística & datos numéricos , Humanos , Renta , Seguro de Salud/estadística & datos numéricos , Tiempo de Internación/estadística & datos numéricos , Masculino , Medicaid/estadística & datos numéricos , Complicaciones Posoperatorias/epidemiología , Reoperación/estadística & datos numéricos , Estudios Retrospectivos , Estados Unidos/epidemiología , Servicios Urbanos de Salud/estadística & datos numéricos
13.
Plast Reconstr Surg ; 2019 May 08.
Artículo en Inglés | MEDLINE | ID: mdl-31107783

RESUMEN

PURPOSE: Nonsyndromic craniosynostosis (NSC) may manifest with complex behavioral, attentional, and emotional sequelae. We characterized higher-level brain connectivity in adolescent NSC patients in response to emotional frustration. METHODS: Surgically corrected patients age >9 with NSC were age/gender/handedness matched to controls. Patients participated in a GoNoGo task, structured as 'win/lose/recovery' paradigms; 'Win' allowed point increases, 'lose' led to net negative points, and 'recovery' allowed re-accumulation. BioImage Suite was used to analyze whole-brain intrinsic connectivity between tasks with cluster-corrected group-level T-maps. P<0.05 was significant. RESULTS: Seven unilateral-coronal (ULC; average age 12.2 years), six metopic (average age 11.5 years), and respective matched controls were included. ULC had worse emotional regulation scores on the Behavior Rating Inventory of Executive Function survey(p=0.065) and performed poorly on the GoNoGo task(p<0.001). Metopics had four regions of interest (ROI) with majority decreased activity compared to controls, and few differences between tasks. ULC had eleven ROIs, majority decreased during 'win' and 'lose', but all increased during 'recovery'. Metopic patients had decreased blood-oxygenation-level-dependent signal in the posterior cingulate(p=0.017) and middle temporal gyrus(MTG;p=0.042). ULC had decreased signal in the posterior cingulate(p=0.023), MTG(p=0.027), and thalamus(p=0.033), but increased signal in the cuneus(p=0.009) and cerebellum(p=0.009). R-ULC, but not metopic/controls, had increased right brain activity in the caudate(p=0.030), thalamus(p=0.011), temporal lobe(p=0.012), and cerebellum(p=0.029). CONCLUSIONS: ULC patients may have emotional dyregulation in response to frustration while metopic patients may have attenuated emotional reactions. Evidence of R-ULC brain laterality suggest that the area of suture fusion may contribute to the mechanism of dysfunction.

14.
Plast Reconstr Surg Glob Open ; 7(3): e2158, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31044122

RESUMEN

Background: Apert syndrome patients are different in clinical pathology, including obstructive sleep apnea, cleft palate, and mental deficiency. These functional deficiencies may be due to anatomic deformities, which may be caused by different forms of associated suture fusion. Therefore, a classification system of Apert syndrome based on the type of craniosynostosis pattern might be helpful in determining treatment choices. Methods: CT scans of 31 unoperated Apert syndrome and 51 controls were included and subgrouped as: class I. Bilateral coronal synostosis; class II. Pansynostosis; and class III. Perpendicular combination synostosis: a. unilateral coronal and metopic synostosis; b. sagittal with bilateral/unilateral lambdoid synostosis; and c. others. Results: Class I is the most common (55%) subtype. The cranial base angulation of class I was normal; however, the cranial base angulation on the cranium side of the skull in class II increased 12.16 degrees (P = 0.006), whereas the facial side cranial base angle of class IIIa decreased 4.31 degrees (P = 0.035) over time. The external cranial base linear measurements of class I showed more evident reduction in anterior craniofacial structures than posterior, whereas other subtypes developed more severe shortening in the posterior aspects. Conclusions: Bicoronal synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined pansynostosis patients have flatter cranial base, whereas the combined unilateral coronal synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.

15.
Plast Reconstr Surg ; 143(6): 1233e-1243e, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-31136487

RESUMEN

BACKGROUND: Considerable craniofacial features of Crouzon syndrome are attributed to the dysmorphology of the cranial base. As cephalometric studies have focused mainly on the facial deformity, rather than the cranial base, the underlying cause of deformity is not as well understood. Therefore, the authors compared the cranial base development of Crouzon syndrome to controls to trace the timing of deformity in the cranial base and face, to analyze their temporal correlation. METHODS: Ninety computed tomographic scans were included (Crouzon, n = 36; controls, n = 54) and divided into five age subgroups. Craniofacial cephalometric measurements were analyzed by Materialise software. RESULTS: The overall cranial base length in Crouzon syndrome compared with controls decreased 8 percent (p = 0.014) on average. The posterior cranial fossa shortening accounted for most of this reduction. The cranial base displaced with the distances from basion, sella, and ethmosphenoid to posterior nasal spine shortened by 21%, 18%, and 16%, respectively (all p < 0.01) during life. Although the cranial base angle on intracranial surface remains normal, the angles on facial surface narrowed were reduced. CONCLUSIONS: The cranial base deformity of Crouzon syndrome consists of the whole skull base and particularly anterior skull base shortening early, leading to a compensatory widened anterior skull base. However, when this widening did not compensate fully for the rapid enlargement of the brain, the posterior skull base displaced inferiorly and became kyphotic. The cranial base deformity develops sequentially anterior to posterior in a probable cascade of influence pattern. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.


Asunto(s)
Disostosis Craneofacial/patología , Base del Cráneo/anomalías , Adolescente , Adulto , Factores de Edad , Estudios de Casos y Controles , Niño , Preescolar , Disostosis Craneofacial/diagnóstico por imagen , Cara/anomalías , Asimetría Facial/diagnóstico por imagen , Asimetría Facial/patología , Femenino , Humanos , Lactante , Recién Nacido , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Base del Cráneo/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adulto Joven
16.
Plast Reconstr Surg ; 143(6): 1738-1745, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-31136493

RESUMEN

BACKGROUND: Various factors can influence outcomes in cleft palate care. This study sought to determine the impact of race on admissions, hospital costs, and short-term complications in cleft palate repair. METHODS: Cleft palate operations were identified in the Kids' Inpatient Database data, from 2000 to 2009. Data were combed for demographics, perioperatives, complications, and hospital characteristics. Bivariate and multivariate analyses were performed between races in total, primary, and revision cohorts. RESULTS: There were 3464 white, 1428 Hispanic, 413 black, 398 Asian/Pacific-Islander, and 470 patients of other races captured. Black patients experienced more emergent admissions (p = 0.005) and increased length of stay (p = 0.029). Hospital charges were highest for black and Hispanic patients and lowest for white patients (p = 0.019). Black patients had more total complications than non-black patients (p = 0.039), including higher rates of postoperative fistula (p = 0.020) and nonspecific complications among revision repairs (p = 0.003). Asian/Pacific Islander in the primary cohort experienced higher rates of accidental puncture (p = 0.031) and fistula (p < 0.001). Other patients had the highest rates of wound disruption (p = 0.013). After controlling for race, diagnosis, Charlson Comorbidity Index score, region, elective/nonelective, payer, and income quartile, length of stay (p < 0.001) and age (p < 0.001) were associated with increases in both total complications and costs. CONCLUSIONS: Race may play a significant role in cleft palate repair, as white patients had fewer complications, shorter length of stay, and lower costs following repair. Delayed age at treatment may predispose patients to adverse sequelae in minority populations, in terms of influencing length of stay and costs. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.


Asunto(s)
Fisura del Paladar/etnología , Fisura del Paladar/cirugía , Disparidades en Atención de Salud/etnología , Costos de Hospital , Procedimientos Quirúrgicos Reconstructivos/métodos , Afroamericanos/estadística & datos numéricos , Preescolar , Fisura del Paladar/diagnóstico , Estudios de Cohortes , Bases de Datos Factuales , Procedimientos Quirúrgicos Electivos/métodos , Procedimientos Quirúrgicos Electivos/estadística & datos numéricos , Grupo de Ascendencia Continental Europea/estadística & datos numéricos , Femenino , Disparidades en Atención de Salud/estadística & datos numéricos , Hispanoamericanos/estadística & datos numéricos , Humanos , Lactante , Tiempo de Internación/economía , Masculino , Análisis Multivariante , Evaluación de Necesidades , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/fisiopatología , Racismo/etnología , Racismo/estadística & datos numéricos , Procedimientos Quirúrgicos Reconstructivos/efectos adversos , Procedimientos Quirúrgicos Reconstructivos/economía , Reoperación/métodos , Estudios Retrospectivos , Estados Unidos
17.
Ann Plast Surg ; 83(5): 568-582, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31008788

RESUMEN

BACKGROUND: From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. METHODS: One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analysis. RESULTS: In Crouzon syndrome, the angle between the cranial base and gnathion was increased prior to 6 months of age by 10.29 degrees (P < 0.001) and by adulthood to 11.95 degrees (P = 0.003) compared with normal. After 6 months of age, the distance between bilateral mandibular condylions (COR-COL) was narrower by 15% (P < 0.001) in Crouzon syndrome compared with control subjects. Before 6 months of age, Apert COR-COL decreased 16% (P < 0.001) compared with control subjects and 13% (P = 0.006) narrower than Crouzon. During 2 to 6 years of age, Apert mandibular ramus height caught up to, and became longer than, Crouzon by 12% (P = 0.011). The nasion-sella-articulare angle of the Apert skull was 5.04 degrees (P < 0.001) less than Crouzon overall. CONCLUSIONS: In Crouzon syndrome, the changes of the spatial relationship of the mandible to the cranial base develop earlier than the mandibular shape deformity, whereas in Apert syndrome, the spatial and morphological changes are synchronous. The morphological changes of the mandible are disproportional in 3 directions, initially significant shortening of the mandibular width and length, and, subsequently, reduced height. Crouzon has more shortening in mandibular height compared with Apert, reflecting the more shortened posterior cranial base length. The narrowed angle between the mandible and the posterior cranial base in Apert skulls is consistent with the more limited nasopharyngeal and oropharyngeal airway space.

18.
Plast Reconstr Surg ; 143(5): 1053e-1059e, 2019 05.
Artículo en Inglés | MEDLINE | ID: mdl-31033831

RESUMEN

BACKGROUND: Complete reoperation is defined as undergoing reoperative/repeated jaw osteotomies, in a patient who previously underwent orthognathic surgery. The purpose of this study is to (1) describe jaw positions at three time-points (before primary and before and after reoperative surgery), (2) investigate factors necessitating reoperation, and (3) outline the technical challenges. METHODS: Repeated orthognathic surgery cases >1-year out were included. Demographic, radiologic, and perioperative data were compiled. Repeated osteotomies (Le-Fort and/or bilateral split sagittal osteotomy, with or without genioplasty), were compared to their respective primary procedures. Statistical analysis was performed using t tests and z-scores. RESULTS: Fifteen patients were included (28.1 years; 71 percent female). Reoperative/repeated surgery was most often needed to address iatrogenic bony malposition and asymmetry. Relapse was a less common indication. Time between reoperative and primary surgery was 14 months. Sagittal discrepancies (p = 0.029) were the most frequent reason for primary orthognathic surgery (e.g., mandibular hypoplasia (p = 0.023). Reoperative/repeated orthognathic was performed for asymmetry (p = 0.014). Repeated procedures used more 3-dimensional planning (p < 0.001), required all three osteotomies (p = 0.034), had longer operative times (p = 0.078), and all required hardware removal (p < 0.001). Anatomical outcomes were good with 100% patient satisfaction at long-term follow-up. CONCLUSIONS: Reoperative/repeated orthognathic surgery is challenging and underreported in the literature. Whereas primary orthognathic typically addressed sagittal discrepancies, reoperative/repeated osteotomies were needed to correct iatrogenic bone malposition and asymmetries. Challenges include: re-planning, scar burden, need to remove integrated hardware, and repeated osteotomy/fixation. Despite these difficulties, outcomes and patient acceptance were good. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.


Asunto(s)
Mentoplastia/estadística & datos numéricos , Enfermedades Maxilomandibulares/cirugía , Osteotomía Le Fort/estadística & datos numéricos , Reoperación/estadística & datos numéricos , Adulto , Femenino , Estudios de Seguimiento , Mentoplastia/métodos , Humanos , Imagenología Tridimensional , Enfermedades Maxilomandibulares/diagnóstico por imagen , Masculino , Tempo Operativo , Osteotomía Le Fort/métodos , Planificación de Atención al Paciente , Recurrencia , Reoperación/métodos , Estudios Retrospectivos , Resultado del Tratamiento
19.
J Plast Surg Hand Surg ; 53(3): 130-137, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30782083

RESUMEN

The dysplastic maxilla and retracted zygoma characterize Apert's syndrome. The relationship between the cranial base and facial development is believed to be influential and substantial. The purpose of this study is to explore the temporal relationships of maldevelopment of these structures to identify potential influence patterns. Fifty-four CT scans (unoperated Apert's, n = 18; control, n = 36) were included and divided into three age subgroups (0-6 months, 6 months-2 years, and 2-6 years). All measurements were analyzed by Materialize software. Cephalometrics relating to midface and cranial base were collected. In anteroposterior direction, prior to 6 months, the zygoma was markedly retruded by 12% in Apert's, followed by persistent retrusive shape into adulthood, averaging 17% shorter compared to controls. The maxillary anteroposterior dimension was 22% shorter than normal before 6 months of age, thereafter, it maintained at least an 18% deficiency into adulthood. In the horizontal direction, the transverse width of the zygoma increased 39% between 6 months and 2 years of age, and it was 14% wider on average overall into adulthood. The maxilla had normal growth in transverse and vertical directions. The zygoma is the most severely deformed anatomic facial structure in early infancy, in both positional relation and geometric shape in Apert's syndrome. This may develop as a 'bridge', influencing the structure, transmitting malformation stresses, caused by premature fused coronal and peri-zygomatic sutures, into facial structures and the maxilla.


Asunto(s)
Acrocefalosindactilia/complicaciones , Maxilar , Cigoma , Adolescente , Estudios de Casos y Controles , Cefalometría , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Maxilar/anomalías , Maxilar/diagnóstico por imagen , Nariz/anomalías , Nariz/diagnóstico por imagen , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Adulto Joven , Cigoma/anomalías , Cigoma/diagnóstico por imagen
20.
J Oral Maxillofac Surg ; 77(4): 850.e1-850.e5, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30576673

RESUMEN

Wide alveolar clefts are challenging to treat and are often associated with a higher chance of failure requiring repeated bone grafts. Even if successful, an excessively large cleft leads to a wide edentulous space, and patients are faced with prolonged orthodontic treatment. We present a series of large alveolar clefts closed with the aid of segmental maxillary osteotomies. Cases of alveolar clefts treated by segmental maxillary osteotomy from 2014 to 2016 were reviewed. Preoperative and postoperative computed tomography scans were analyzed to assess the alveolar gap and bone filling. Demographic characteristics, diagnoses, and operative techniques were reviewed and described. A total of 10 alveolar fistulae were addressed, using 6 segmental osteotomies in 6 patients. Of the cases, 66% were bilateral. There were 4 female and 2 male patients with an average age of 12.6 years. The average preoperative cleft width was 14.2 mm on computed tomography scans. Postoperatively, all clefts were successfully closed and no adverse effects were observed. Segmental maxillary osteotomy with concurrent bone grafting and fistula closure is an effective approach to treat excessively wide alveolar clefts. This enables a prudent dental substitution or prosthodontic plan while minimizing prolonged orthodontia, in addition to achieving the traditional goals of alveolar bone grafting (stabilizing the maxillary arch, closing fistulae, and permitting canine eruption).

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