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1.
J Craniofac Surg ; 2020 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-32176017

RESUMO

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.

2.
J Craniofac Surg ; 2020 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-32176020

RESUMO

BACKGROUND: Among congenital ear deformities, helical rim deformities are most common. Non-surgical ear molding has emerged as an effective option to treat helical rim deformities and could reduce the need for surgery later in a child's life. Despite this, there has never been a study examining how the general public rates corrections after ear molding. METHODS: A retrospective chart review was conducted of all consecutive patients with helical rim deformities treated with InfantEar TM Molding System. Amazon Mechanical Turk (MTurk) was used to survey blinded respondents using photographs of patients' ears to determine the degree of normalcy on a scale of 1 (not normal) to 10 (completely normal) before and after molding. Ratings of ears were compared using a paired t test and percent improvement from baseline. RESULTS: A total of 59 ears met criteria for evaluation by 497 MTurk respondents. Average age of patients at the time of treatment was 34.2 ±â€Š16.8 days and mean therapy duration was 31.3 ±â€Š13.1 days. A paired t test analysis found that MTurk respondents identified significant improvement in 91.5% of ears (n = 54) (P < 0.05). The percent improvement in observed helical rim change was on average 82.7 ±â€Š61.1%. CONCLUSION: Ear molding is an effective and safe way to address helical rim deformities early in the neonatal period. The general public is able to recognize ears with rim deformities as abnormal, and appreciate the difference in architecture after molding. Crowdsourcing technology offers an opportunity to measure laypeople's perceptions regarding outcomes after ear molding.

3.
Plast Reconstr Surg ; 145(2): 382e-390e, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31985648

RESUMO

BACKGROUND: The purpose of this study was to determine what craniometric changes occur to both orbits of unicoronal craniosynostosis patients undergoing fronto-orbital advancement and remodeling, and which of these changes are associated with new onset of postoperative strabismus. METHODS: A retrospective analysis was performed of the preoperative and postoperative orbits of 24 unicoronal craniosynostosis patients and the orbits of 24 control subjects, totaling 144 orbits. Eight parameters were evaluated using multivariate logistic regression analysis. One of the parameters was modified orbital index, an indicator of severity of harlequin deformity. RESULTS: Significant differences in orbital dimensions and angles were present bilaterally in unicoronal craniosynostosis orbits when compared to controls. Fronto-orbital advancement and remodeling increased the ipsilateral unicoronal craniosynostosis orbital volume from 13,184 ± 2003 mm to 16,220 ± 2323 mm (p < 0.001). Ipsilateral horizontal cone angles were increased from 48 ± 5 degrees to 54 ± 7 degrees (p = 0.004). Ipsilateral vertical cone angles were decreased from 73 ± 8 degrees to 66 ± 10 degrees (p = 0.003). Ipsilateral modified orbital index improved from 0.83 ± 0.06 to 0.88 ± 0.06 (p = 0.003). Three of the 19 unicoronal craniosynostosis patients developed transient postoperative strabismus. Logistic regression analysis displayed a strong significant association between new-onset strabismus and a change in modified orbital index with a coefficient of 30.84 ± 14.51 (p < 0.05). CONCLUSIONS: The orbital dysmorphology in unicoronal craniosynostosis is bilateral in nature, and it is not wholly treated with conventional fronto-orbital advancement and remodeling. The severity of ipsilateral orbital dysmorphology is correlated with the incidence of postoperative strabismus following conventional fronto-orbital advancement and remodeling. Future research is needed to develop strategies to mitigate the risk of development of strabismus in this group of patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.


Assuntos
Craniossinostoses/complicações , Osso Frontal/cirurgia , Órbita/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Estrabismo/etiologia , Estrabismo/cirurgia , Estudos de Casos e Controles , Feminino , Humanos , Lactente , Modelos Logísticos , Masculino , Órbita/patologia , Estudos Retrospectivos , Estrabismo/patologia
4.
J Craniofac Surg ; 31(1): 166-168, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31794445

RESUMO

INTRODUCTION: Neck pain is common in the post-operative period after craniofacial procedures. If patients present with neck pain and torticollis, it could be a manifestation of atlantoaxial rotatory subluxlation (AARS), which describes a rare condition in which there is lateral displacement of C1 relative to C2. When this occurs in the post-operative patient, it is termed Grisel syndrome. In this case series, we report on 3 patients diagnosed with Grisel syndrome after a craniofacial procedure. METHODS: A retrospective chart review of a single craniofacial surgeon at a pediatric hospital was conducted over the last 3 decades. Demographics, procedures performed, and management strategies of AARS were included for review. RESULTS: Three patients were identified who were diagnosed with Grisel syndrome after a craniofacial procedure and required inpatient cervical traction. All patients presented with torticollis within 1 week of their operation. Conservative management was ineffective, and all 3 patients were admitted for inpatient cervical traction, for an average of 13 days followed by an average of 47 days of outpatient therapy. No patients showed any signs of recurrence after removal of outpatient traction device. CONCLUSION: Grisel syndrome is a rare, but serious complication of craniofacial procedures. Physicians caring for these patients must have a high degree of suspicion if a patient with a recent craniofacial procedure presents with torticollis. Delaying the initiation of therapy has been shown to lead to higher rates of recurrence and increases the likelihood that patients will require surgical intervention.

5.
J Craniofac Surg ; 31(1): 85-90, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31609948

RESUMO

BACKGROUND: Lengthening temporalis myoplasty (LTM) and cross-face nerve graft with free gracilis muscle transfer (CFNG-FGMT) are the 2 most common procedures used to restore dynamic facial animation and improve facial symmetry. There has not been direct comparison or consensus. Here, the authors compare our experience with respect to muscle activity, symmetry, and excursion. METHODS: A retrospective review was performed of patients with facial palsy who had CFNG-FGMT or LTM from 2008 to 2016 at a single institution. Postoperative surface EMG was recorded at maximum open smile. Normal and paralyzed sides of the face were analyzed with Facial Assessment by Computer Evaluation software. Commissure excursion and symmetry was assessed. RESULTS: Six patients with LTM and 10 with CFNG-FGMT met inclusion criteria. Muscle activity was 1st identified in LTM patients after 3 months (47.42 mV, P < 0.001) and CFNG-FGMT patients after 3 months (28.30 mV, P < 0.001) compared to immediate postoperative period. Relative to preoperative excursion, there was significant increase of 3.33 mm in commissure excursion seen at the 0 to 3 month period for LTM patients (P = 0.04). Commisure excursion for CFNG-FGMT was seen later, in the 3- to 6-month postoperative period (4.01, P = 0.024). During smile, CFNG-FGMT patients had better symmetry than unilateral LTM patients. In bilateral LTM patients, there was no significant change in symmetry. CONCLUSION: Dynamic facial animation improved in both surgical groups. The LTM demonstrates a faster rate of muscle recruitment compared to CFNG-FGMT. After 3 months, both LTM and CFNG-FGMT groups had comparable excursions. A decision-making algorithm is presented.

6.
Childs Nerv Syst ; 36(3): 535-544, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31848721

RESUMO

BACKGROUND: Better understanding the incidence and patterns of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to alter neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retina can non-invasively diagnose elevated ICP, and has demonstrated high sensitivity and specificity among patients with craniosynostosis. This study sought to characterize patterns of elevated ICP among patients with craniosynostosis. METHODS: Quantitative retinal parameters were prospectively assessed in both eyes of patients with craniosynostosis using spectral-domain OCT. Based on retinal OCT thresholds associated with elevated ICP (> 15 mmHg), subjects were assigned an OCT diagnosis of elevated or non-elevated ICP which was analyzed relative to clinical characteristics and craniosynostosis patterns. RESULTS: Eighty subjects (aged 0.2-18 years) with craniosynostosis were enrolled; among these, 67 (84%) were nonsyndromic. OCT evaluation was performed at initial vault expansion in 56 (70%) patients. Among this subset, 27 (48%) patients had peri-papillary changes suggestive of elevated ICP, reflecting a 44% incidence in nonsyndromic and 83% in syndromic patients. The median age at initial vault expansion was higher among those with elevated ICP (11.1 months) than those without (7.8 months; p = 0.04.) Multi-suture synostosis was associated with changes consistent with elevated ICP in 9 (75%) patients compared with 18 (41%) with single suture synostosis (p = 0.05). CONCLUSIONS: OCT of the retina produces a potentially sensitive indicator of ICP in craniosynostosis patients. Elevated ICP may be associated with number of involved sutures and older patient presentation, and refining appropriate "cutoffs" will be important as the technology becomes more widespread.

7.
Plast Reconstr Surg ; 2019 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-31842122

RESUMO

BACKGROUND: The purpose of this study is to determine what craniometric changes occur to both orbits of unicoronal craniosynostosis (UCS) undergoing frontal orbital advancement and remodeling (FOAR) and which of these changes are associated with new onset of postoperative strabismus. METHODS: A retrospective analysis of pre and postoperative 24 UCS orbits and 24 control orbits were analyzed, totaling 144 orbits. Eight parameters were evaluated utilizing multivariate logistic regression analysis. One of the parameters is modified orbital index (MOI), an indicator of severity of Harlequin deformity. RESULTS: Significant differences in orbital dimensions and angles were present bilaterally in UCS orbits when compared to controls. FOAR increased the ipsilateral UCS orbital volume from 13,184.± 2,003 mm to 16,220 ± 2,323mm (p<0.001). Ipsilateral horizontal cone angles were increased from 48 ± 5° to 54 ± 7° (p=0.004). Ipsilateral vertical cone angles were decreased from 73 ± 8° to 66 ± 10° (p=0.003). Ipsilateral MOI improved from 0.83 ± 0.06 to 0.88 ± 0.06 (p=0.003). Three of the nineteen UCS patients developed transient postoperative strabismus. Logistic regression analysis displayed a strong significant association between new-onset strabismus and a change in MOI with a coefficient of 30.84 ±14.51 (p<0.05). CONCLUSIONS: The orbital dysmorphology in UCS is bilateral in nature, and it is not wholly treated with conventional FOAR. Severity of ipsilateral orbital dysmorphology is correlated with incidence of postoperative strabismus following conventional FOAR. Future research is needed to develop strategies to mitigate risk of development of strabismus in this group of patients.

8.
Plast Reconstr Surg ; 144(4): 932-940, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31568307

RESUMO

BACKGROUND: Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations, with few data on the relative benefit of various surgical interventions. The authors compare the incidence and resolution of Chiari malformations and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) and patients undergoing conventional cranial vault remodeling. METHODS: Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 to 2016 at a single academic hospital, with adequate radiographic assessments, were reviewed. Demographics, interventions, the presence of a Chiari malformation on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher's exact tests were used as appropriate. RESULTS: Forty-nine patients underwent PVDO, and 23 patients underwent cranial vault remodeling during the study period. Median age at surgery (p = 0.880), sex (p = 0.123), and types of syndrome (p = 0.583) were well matched. Patients who underwent PVDO had a decreased incidence of developing Chiari malformations postoperatively compared with the cranial vault remodeling cohort (2.0 percent versus 17.4 percent; p = 0.033). Not surprisingly, no significant difference was found between the groups with regard to the incidence of postoperative hydrocephalus requiring shunt placement (PVDO, 4.1 percent; cranial vault remodeling, 4.3 percent; p = 0.999). CONCLUSIONS: As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a Chiari malformation; however, prospective evaluation is needed to determine causality. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.


Assuntos
Malformação de Arnold-Chiari/etiologia , Craniossinostoses/complicações , Craniossinostoses/cirurgia , Hidrocefalia/etiologia , Osteogênese por Distração/métodos , Crânio/cirurgia , Feminino , Humanos , Lactente , Masculino , Indução de Remissão , Estudos Retrospectivos , Síndrome
9.
Plast Reconstr Surg ; 2019 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-31274746

RESUMO

PURPOSE: Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations (CM), with little data on relative benefit of various surgical interventions. The authors compare the incidence and resolution of CM and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) versus conventional cranial vault remodeling (CVR). METHODS: Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 - 2016 at a single academic hospital with adequate radiographic assessments were reviewed. Demographics, interventions, the presence of a CM on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher's exact tests were used as appropriate. RESULTS: 49 patients underwent PVDO, and 23 patients underwent CVR during the study period. Median age at surgery (p=0.880), sex (p=0.123), and types of syndrome (p=0.583) were well-matched. Patients who underwent PVDO had a decreased incidence of developing CM post-operatively compared to the CVR cohort (2.0% vs 17.4%, p=0.033). Not surprisingly, no significant difference was found between the groups with regards to the incidence of post-operative hydrocephalus requiring shunt placement (PVDO: 4.1% vs CVR: 4.3%, p=0.999). CONCLUSION: As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a CM, though prospective evaluation is needed to determine causality.

10.
Am J Med Genet A ; 179(8): 1442-1450, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31111620

RESUMO

Muenke syndrome (MIM #602849), the most common syndromic craniosynostosis, results from the recurrent pathogenic p.P250R variant in FGFR3. Affected patients exhibit wide phenotypic variability. Common features include coronal craniosynostosis, hearing loss, carpal and tarsal anomalies, and developmental/behavioral issues. Our study examined the phenotypic findings, medical management, and surgical outcomes in a cohort of 26 probands with Muenke syndrome identified at the Children's Hospital of Philadelphia. All probands had craniosynostosis; 69.7% had bicoronal synostosis only, or bicoronal and additional suture synostosis. Three male patients had autism spectrum disorder. Recurrent ear infections were the most common comorbidity, and myringotomy tube placement the most common extracranial surgical procedure. Most patients (76%) required only one fronto-orbital advancement. de novo mutations were confirmed in 33% of the families in which proband and both parents were genetically tested, while in the remaining 66% one of the parents was a mutation carrier. In affected parents, 40% had craniosynostosis, including 71% of mothers and 13% of fathers. We additionally analyzed the medical resource utilization of probands with Muenke syndrome. To our knowledge, these data represent the first comprehensive examination of long-term management in a large cohort of patients with Muenke syndrome. Our study adds valuable information regarding neuropsychiatric and medical comorbidities, and highlights findings in affected relatives.

11.
Plast Reconstr Surg ; 143(6): 1725-1736, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31136491

RESUMO

BACKGROUND: The frequency of respiratory events in the perioperative period, and optimal duration of intubation during early mandibular distraction osteogenesis activation, are poorly understood. This study assesses potential risk factors associated with perioperative respiratory events, particularly the need for reintubation, following mandibular distraction osteogenesis surgery. METHODS: A retrospective review was conducted for infants (younger than 1 year) undergoing mandibular distraction osteogenesis for tongue-based airway obstruction between November of 2010 and December of 2017. Univariate and multivariate analyses of sentinel events and outcomes were performed. RESULTS: Ninety infants (median age, 35 days) were included (50 percent were syndromic). Twenty-seven subjects (30 percent) experienced a respiratory event requiring intervention before discharge, including 14 subjects who failed initial extubation. Subjects extubated earlier than postoperative day 5 failed extubation more frequently (33%) compared to those extubated later (9%; p = 0.005). Respiratory events occurred more frequently when extubation was attempted at distraction lengths of 5 mm or less (42 percent) compared to greater than 5 mm (21 percent; p = 0.032). Logistic regression modeling showed that syndromic status (OR, 14.8) and secondary airway anomaly (OR, 6.1) were significant predictors for respiratory events, whereas greater length of distraction at the time of extubation was protective (OR, 0.8; p < 0.05). CONCLUSIONS: Postoperative intubation of at least 5 days with associated mean distraction of 5 mm appears to be associated with successful extubation trial following mandibular distraction osteogenesis surgery. Patients with congenital syndromes and secondary airway anomalies are more likely to experience perioperative respiratory events. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.


Assuntos
Micrognatismo/cirurgia , Osteogênese por Distração/efeitos adversos , Assistência Perioperatória/métodos , Síndrome de Pierre Robin/cirurgia , Insuficiência Respiratória/fisiopatologia , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/fisiopatologia , Estudos de Coortes , Feminino , Seguimentos , Humanos , Incidência , Lactente , Intubação Intratraqueal/métodos , Modelos Logísticos , Masculino , Avanço Mandibular/efeitos adversos , Avanço Mandibular/métodos , Micrognatismo/diagnóstico por imagem , Análise Multivariada , Osteogênese por Distração/métodos , Período Perioperatório , Síndrome de Pierre Robin/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/terapia , Insuficiência Respiratória/epidemiologia , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Estudos Retrospectivos , Medição de Risco , Resultado do Tratamento
12.
J Craniofac Surg ; 30(2): 503-507, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31137452

RESUMO

BACKGROUND: Posterior cranial vault distraction osteogenesis (PVDO) has gained popularity as the initial intervention in patients with syndromic craniosynostosis. Patients may require secondary frontal orbital advancement (FOA) following PVDO, but little is known about the perioperative risks associated with this staged management. The purpose of this study is to compare the perioperative morbidity profile of secondary FOA (study) to that of primary FOA (control). METHODS: A retrospective review was conducted for patients with syndromic or complex craniosynostosis undergoing FOA between 2004 and 2017. Univariate and multivariate analysis of demographic and perioperative data were performed. RESULTS: Forty-three subjects met inclusion criteria, 17 in the study cohort and 26 in the control cohort. The 2 cohorts were similar with regards to diagnosis and suture involvement, as well as weight-adjusted estimated blood loss, blood transfusion volume, and length of hospital stay (P > 0.050). Secondary FOA procedures required longer operating time (231 ±â€Š58 versus 264 ±â€Š62 min, P = 0.031) and anesthesia time (341 ±â€Š60 versus 403 ±â€Š56 min, P = 0.002). The secondary FOA cohort had a significantly greater proportion of procedures with difficult wound closure (19% versus 59%, P = 0.008). Two subjects in the study cohort developed a wound dehiscence, compared with 1 subject in the control cohort (P = 0.552). Frontal orbital advancement as a secondary procedure after PVDO was a predictor variable in multivariate analysis for wound difficulties (odds ratio 8.6, P = 0.038). CONCLUSION: Syndromic and complex craniosynostosis may safely be managed with initial PVDO followed by FOA, with some increased wound closure difficulty.


Assuntos
Craniossinostoses/cirurgia , Osso Frontal/cirurgia , Órbita/cirurgia , Osteogênese por Distração/métodos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Análise Multivariada , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Síndrome , Resultado do Tratamento
13.
J Craniofac Surg ; 30(6): 1692-1695, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31033762

RESUMO

BACKGROUND: Posterior vault distraction osteogenesis (PVDO) has been demonstrated to effectively increase intracranial volume, treat increased intracranial pressure, and improve head shape in syndromic patients. The purpose of this study is to compare changes along the posterior cranial base before and after distraction. METHODS: A retrospective review was completed of subjects who underwent PVDO with computed tomography scans at 2 time-points: within 3 months preoperatively and 1 to 6 months postoperatively. Using Mimics software, craniometric landmarks were identified and surface area of the foramen magnum was calculated. A comparison of pre- to postoperative measurements was completed using Wilcoxon matched-paired signed rank tests and linear regression. RESULTS: A total of 65 PVDO subjects were identified, 12 subjects met inclusion criteria. Mean operative age was 3.0 ±â€Š4.0 years. The cranial vault was distracted on average 25.0 ±â€Š6.0 mm, with those < 12 months of age distracted 29.5 ±â€Š4.9 mm and >12 months of age distracted 22.0 ±â€Š4.9 mm (P = 0.0543). There was a significant increase in pre- to postoperative foramen magnum surface area (52.1 ±â€Š63.2 mm, P = 0.002), length (0.9 ±â€Š1.4 mm, P = 0.050), and width (0.6 ±â€Š1.0 mm, P = 0.050). Similarly, linear distances between nasion and posterior cranial base landmarks such as foramen magnum (3.4 ±â€Š4.2 mm, P = 0.010), and occipital protuberance (9.1 ±â€Š9.6 mm, P = 0.003) were increased. Subjects under 12 months had a greater percentage increases in posterior vault length than those over 12 months. CONCLUSION: Posterior vault distraction osteogenesis is associated with an increase in size of the foramen magnum, and lengthening of the posterior cranial base, both of which may be beneficial in patients with turribrachycephaly.


Assuntos
Cefalometria , Base do Crânio/diagnóstico por imagem , Algoritmos , Criança , Pré-Escolar , Forame Magno , Humanos , Lactente , Recém-Nascido , Osso Occipital , Osteogênese por Distração , Período Pós-Operatório , Estudos Retrospectivos , Base do Crânio/cirurgia , Tomografia Computadorizada por Raios X
14.
Clin Pediatr (Phila) ; 58(7): 798-805, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31007048

RESUMO

The incidence of congenital ear deformities has been documented to be as high as 58% of all newborns. Unfortunately, the majority of these deformations do not spontaneously self-correct, leaving the child with a permanent deformity that can only be corrected through surgery. With the evolving simplicity of ear molding that can be done in the office, this can obviate the need for unnecessary surgery that comes with its own set of complications and a higher cost. Early detection and early treatment is key for successful ear molding. The purpose of this article is to review the types of deformational ear deformities and the most up-to-date literature on ear molding, increase awareness to this topic, and ultimately increase patient and parental satisfaction with their overall care.

15.
Magn Reson Med ; 81(5): 3007-3016, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30565286

RESUMO

PURPOSE: To develop a dual-radiofrequency (RF), dual-echo, 3D ultrashort echo-time (UTE) pulse sequence and bone-selective image reconstruction for rapid high-resolution craniofacial MRI. METHODS: The proposed pulse sequence builds on recently introduced dual-RF UTE imaging. While yielding enhanced bone specificity by exploiting high sensitivity of short T2 signals to variable RF pulse widths, the parent technique exacts a 2-fold scan time penalty relative to standard dual-echo UTE. In the proposed method, the parent sequence's dual-RF scheme was incorporated into dual-echo acquisitions while radial view angles are varied every pulse-to-pulse repetition period. The resulting 4 echoes (2 for each RF) were combined by view-sharing to construct 2 sets of k-space data sets, corresponding to short and long TEs, respectively, leading to a 2-fold increase in imaging efficiency. Furthermore, by exploiting the sparsity of bone signals in echo-difference images, acceleration was achieved by solving a bone-sparsity constrained image reconstruction problem. In vivo studies were performed to evaluate the effectiveness of the proposed acceleration approaches in comparison to the parent method. RESULTS: The proposed technique achieves 1.1-mm isotropic skull imaging in 3 minutes without visual loss of image quality, compared to the parent technique (scan time = 12 minutes). Bone-specific images and corresponding 3D renderings of the skull were found to depict the expected craniofacial anatomy over the entire head. CONCLUSION: The proposed method is able to achieve high-resolution volumetric craniofacial images in a clinically practical imaging time, and thus may prove useful as a potential alternative to computed tomography.

16.
J Craniofac Surg ; 30(1): 105-109, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30376505

RESUMO

BACKGROUND: Hospital resource overutilization can significantly disrupt patient treatment such as cancelling surgical patients due to a lack of intensive care unit (ICU) space. The authors describe a clinical pathway (CP) designed to reduce ICU length of stay (LOS) for nonsyndromic single-suture craniosynostosis (nsSSC) patients undergoing cranial vault reconstruction (CVR) in order to minimize surgical disruptions and improve patient outcomes. METHODS: A multidisciplinary team implemented a perioperative CP including scheduled laboratory testing to decrease ICU LOS. Hospital and ICU LOS, interventions, and perioperative morbidity-infection rate, cerebrospinal fluid (CSF) leaks, and unplanned return to the operating room (OR)-were compared using Mann-Whitney U, Fisher exact, and t tests. RESULTS: Fifty-one ICU admissions were managed with the standardized CP and compared to 49 admissions in the 12 months prior to pathway implementation. There was a significant reduction in ICU LOS (control: mean 1.84 ±â€Š0.93, median 1.89 ±â€Š0.94; CP: mean 1.15 ±â€Š0.34, median 1.03 ±â€Š0.34 days; P < 0.001 for both). There were similar rates of hypotension requiring intervention (CP: 2, control: 1; P = 0.999), postoperative transfusion (CP: 3, control: 0; P = 0.243), and artificial ventilation (CP: 1, control: 0; P = 0.999). Perioperative morbidity such as infection (CP: 1, control: 0; P = 0.999), return to the OR (CP: 1, control: 0; P = 0.999), and CSF leak (no leaks; P = 0.999) was also similar. CONCLUSION: Implementation of a standardized perioperative CP for nsSSC patients resulted in a significantly shorter ICU LOS without a measured change in perioperative morbidity. Pathways such as the one described that improve patient throughput and decrease resource utilization benefit craniofacial teams in conducting an efficient service while providing high-quality care.


Assuntos
Protocolos Clínicos , Craniossinostoses/cirurgia , Unidades de Terapia Intensiva/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Procedimentos Cirúrgicos Reconstrutivos , Adolescente , Adulto , Criança , Pré-Escolar , Procedimentos Clínicos , Feminino , Recursos em Saúde/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Assistência Perioperatória , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Adulto Jovem
17.
Plast Reconstr Surg ; 143(2): 521-530, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30531617

RESUMO

BACKGROUND: This study characterizes the perioperative morbidity of a large cohort of subjects with syndromic craniosynostosis who underwent transcranial or subcranial midface distraction. METHODS: Demographic and perioperative data were compared between those who underwent transcranial or subcranial midface distraction osteogenesis between July of 1999 and December of 2017. Univariate analysis was conducted using chi-square and Fisher's exact tests for categorical variables and the Mann-Whitney U test for continuous variables. Multivariate analysis was conducted using logistic regression modeling. Complications were graded using the Clavien-Dindo classification. RESULTS: Sixty-four subjects underwent a total of 71 midface distraction procedures. There was a total of 28 complications (39 percent). The trans cranial cohort had a significantly higher frequency of complications (58 percent) compared with the subcranial cohort (29 percent; p = 0.017), with a significantly greater proportion of infection-related complications in the transcranial cohort (80 percent versus 54 percent; p = 0.028). Transcranial complications included cranial contamination, whereas most subcranial cohort infections were superficial or limited facial abscesses. The only significant predictor variable for complications in a multivariate analysis was whether the osteotomy approach was transcranial as opposed to subcranial, with an odds ratio of 5.80 (p = 0.013). CONCLUSIONS: Complication rates in midface distraction remain high, with transcranial procedures having significantly higher complication rates, infection-related complications, and notably greater severity of complications. Although the goals of surgery often dictate choice of osteotomy, the risks associated with transcranial procedures must be thoroughly understood by surgeon and patient alike. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.


Assuntos
Abscesso/epidemiologia , Craniossinostoses/cirurgia , Face/patologia , Osteogênese por Distração/efeitos adversos , Infecção da Ferida Cirúrgica/epidemiologia , Abscesso/etiologia , Adolescente , Criança , Pré-Escolar , Face/cirurgia , Feminino , Humanos , Masculino , Período Perioperatório , Estudos Prospectivos , Sistema de Registros/estatística & dados numéricos , Estudos Retrospectivos , Crânio/cirurgia , Infecção da Ferida Cirúrgica/etiologia , Síndrome
18.
Cleft Palate Craniofac J ; 56(2): 177-186, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-29698113

RESUMO

OBJECTIVE: To characterize the epidemiology and risk factors for nasal obstruction among subjects with cleft lip and/or cleft palate (CL/P) utilizing the well-validated Nasal Obstruction Symptom Evaluation (NOSE) survey. DESIGN: Retrospective cross-sectional study. SETTING: Cleft Lip and Palate Program, Children's Hospital of Philadelphia. PATIENTS, SUBJECTS: One thousand twenty-eight surveys obtained from 456 subjects (mean age: 10.10 (4.48) years) with CL/P evaluated between January 2015 and August 2017 with at least 1 completed NOSE survey. INTERVENTIONS: Nasal Obstruction Symptom Evaluation surveys completed at each annual visit. MAIN OUTCOME MEASURES: Composite NOSE and individual symptom scores. RESULTS: Sixty-seven percent of subjects had nasal obstruction at some point during the study period, with 49% reporting nasal obstruction at latest follow-up. subjects aged 14 years and older reported the most severe symptoms ( P = .002). Subjects with cleft lip and alveolus (CL+A) and unilateral cleft lip and palate (CLP) reported more severe nasal blockage than other phenotypes ( P = .021). subjects with a history of either posterior pharyngeal flap (PPF) or sphincter pharyngoplasty (SP) had significantly higher NOSE scores than subjects with no history of speech surgery ( P = .006). There was no significant difference ( P > .050) in NOSE scores with regard to history of primary tip rhinoplasty, nasal stent use, or nasoalveolar molding. CONCLUSIONS: There are more severe nasal obstructive symptoms among subjects older than 14 years of age, with CL+A or unilateral CLP, and with a history of PPF or SP. Future studies utilizing the NOSE are needed to evaluate and address this prevalent morbidity in the CLP population.


Assuntos
Fenda Labial , Fissura Palatina , Obstrução Nasal , Rinoplastia , Adolescente , Criança , Humanos , Estudos Retrospectivos , Avaliação de Sintomas , Resultado do Tratamento
19.
Plast Reconstr Surg ; 142(5): 1285-1293, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30511982

RESUMO

BACKGROUND: Controversy exists regarding the treatment of mandibular hypoplasia in craniofacial microsomia patients, notably the role of mandibular distraction osteogenesis. The authors compared the need for orthognathic surgery in skeletally mature craniofacial microsomia subjects who either did (study group) or did not (control group) undergo early mandibular distraction osteogenesis. METHODS: A retrospective review was conducted of all craniofacial microsomia patients evaluated between January of 1993 and March of 2017. This study included patients with a Kaban-Pruzansky grade I to III mandible, and who were at least 14 years old at the time of the latest follow-up. RESULTS: Thirty-eight subjects met inclusion criteria: 17 who underwent mandibular distraction osteogenesis and 21 who did not (mean age, 18.95 ± 2.82 years versus 17.95 ± 2.14 years, respectively; p = 0.246). The degree of mandibular deformity was matched (distraction, 29.4 percent Kaban-Pruzansky grade IIb and 5.9 percent grade III; no distraction, 23.8 percent grade IIb and 9.5 percent grade III; p = 0.788). No significant difference was noted between the distraction and no-distraction cohorts with regard to need for orthognathic surgery [distraction, n = 10 (58.8 percent); no distraction, n = 8 (38.1 percent); p = 0.203]. CONCLUSIONS: The results seem to suggest that there is no significant difference in orthognathic surgery rates at skeletal maturity between craniofacial microsomia subjects who underwent early mandibular distraction osteogenesis and those who did not. Subjects who undergo distraction may still ultimately require orthognathic surgery to correct facial asymmetry. Additional studies are required to determine the optimal timing and technique of distraction, the importance of orthodontic management during and after distraction, and the early psychosocial benefits of improved facial symmetry. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.


Assuntos
Síndrome de Goldenhar/cirurgia , Reconstrução Mandibular/métodos , Osteogênese por Distração/métodos , Adolescente , Transplante Ósseo/métodos , Criança , Feminino , Seguimentos , Humanos , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Reoperação , Costelas/transplante , Resultado do Tratamento
20.
Plast Reconstr Surg Glob Open ; 6(9): e1856, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30349770

RESUMO

Vertical mandibular distraction results in translation of both proximal and distal segments. The force exerted on the condylar segment not only places unwanted force on the joint but also rotates the coronoid process into the cranial base. To prevent these sequelae, we investigate the use of a "check plate" on the condylar segment in an attempt to decrease force at the Temporomandibular joint (TMJ) and prevent unwanted rotation of the coronoid. Patients with hemifacial microsomia, seen at our Children's Hospital from 2012 to 2016 having undergone unilateral vertical mandibular distraction with placement of check plate were compared with a sample of those similarly having undergone distraction without use of the plate. Preoperative and postoperative cephalometric measures and 3-dimensional computed tomography imaging were analyzed. Three subjects were identified in each group. Age and Pruzansky-Kaban classification did not differ between groups. Vertical distance from the coronoid process perpendicular to the Frankfort Horizontal did not differ between groups (P < 0.07); however, postoperative distance significantly differed with the coronoid process rotating upward into the cranial base in subjects without a check plate (P < 0.005). Preoperative angle of the coronoid process based on the Frankfort Horizontal did not differ (P < 0.06); however, postoperative angle significantly changed, confirming upward rotation into the cranial base (P < 0.01). Total regenerate did not differ (P < 0.08). Vertical mandibular distraction results in undesirable upward rotation of the proximal segment into the cranial base and superior displacement of regenerate. This can be prevented with the use of a check plate.

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