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Objective: To evaluate the impact of the COVID-19 pandemic on the psychosocial functioning of individuals with cleft lip and/or palate (CL/P). Methods: Patients with CL/P ≥ 6 years old were prospectively recruited from the Cleft and Craniofacial Clinic of a tertiary children's hospital. From July-October 2021, eligible patients (or their parent/guardian) were sent a survey regarding their psychosocial functioning before and after the start of the pandemic. Main Outcome Measure: The difference between prepandemic and intrapandemic patient-reported outcome scores. Results: Thirty-six patients (20 female, age: 15.9 ± 9.8 years) responded. Most had cleft lip and palate (77.8%), responded online (69.4%), interacted remotely via both voice- and video-conferencing (62.9%), and wore masks routinely (77.1%). Similar numbers of patients responded independently (27.8%), responded with the help of a parent/guardian (36.1%), or had a parent/guardian respond on their behalf (36.1%). General social-emotional well-being (p = 0.004, r rb = 0.659) and satisfaction with facial appearance (p = 0.044, r rb = 0.610) significantly improved after the start of the pandemic. Compared to their general intrapandemic social-emotional well-being scores, patients reported higher scores while wearing a mask (r rb = 0.827) and lower scores while interacting remotely (r rb = 0.605), although all were still significantly improved compared to their prepandemic scores (p ≤ 0.010). Patients also reported significant improvement in social functioning while wearing a mask (p = 0.036, r rb = 0.519), whereas they did not when considering their general intrapandemic feelings/experiences (p = 0.269, r rb = 0.211). Conclusion: Patients with CL/P demonstrated significant improvement in overall social-emotional well-being, satisfaction with facial appearance, and social functioning after the start of the COVID-19 pandemic, particularly when wearing a mask.
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LEARNING OBJECTIVES: After studying this article, the participant should be able to: (1) Describe the pathology of velopharyngeal dysfunction (VPD) as it relates to patients with a cleft palate. (2) Use the perceptual speech assessment and objective diagnostic tools to determine the presence or absence of VPD. (3) Describe the surgical options available for the treatment of patients with VPD. (4) Develop an evidence-based, customized treatment plan for VPD founded on objective considerations. SUMMARY: To treat patients with cleft palate effectively, the surgeon must understand the diagnosis and surgical management of cleft-associated velopharyngeal dysfunction. The authors review diagnostic modalities including perceptual speech assessment, video nasendoscopy, fluoroscopy, magnetic resonance imaging, and nasometry. Surgical treatments including palatal lengthening with buccal myomucosal flaps, conversion Furlow palatoplasty, sphincter pharyngoplasty, and pharyngeal flap are discussed. Selection of an optimal surgical treatment is addressed.
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Fisura del Paladar , Insuficiencia Velofaríngea , Humanos , Insuficiencia Velofaríngea/diagnóstico , Insuficiencia Velofaríngea/cirugía , Insuficiencia Velofaríngea/etiología , Insuficiencia Velofaríngea/fisiopatología , Fisura del Paladar/cirugía , Fisura del Paladar/complicaciones , Procedimientos de Cirugía Plástica/métodos , Colgajos Quirúrgicos/trasplanteRESUMEN
Unilateral lambdoid craniosynostosis is differentiated from deformational plagiocephaly primarily by assessing the cranium from posterior and bird's-eye views. Findings include posterior displacement of the ipsilateral ear, ipsilateral occipitomastoid bossing, ipsilateral occipitoparietal flattening, contralateral parietal bossing, and contralateral frontal bossing. Diagnosis based off facial morphology may be an easier approach because the face is less obstructed by hair and head-coverings, and can easily be assessed when supine. However, frontofacial characteristics of unilateral lambdoid craniosynostosis are not well described. Methods: A retrospective cohort review of patients with isolated, unilateral lambdoid craniosynostosis from the Children's Hospital of Pittsburgh and the Children's Hospital of Philadelphia was performed. Preoperative frontal and profile photographs were reviewed for salient characteristics. Results: Nineteen patients met inclusion criteria. Eleven patients had left lambdoid craniosynostosis, and eight had right lambdoid craniosynostosis. All patients were nonsyndromic. Patients demonstrated contralateral parietal bossing and greater visibility of the ipsilateral ear. Contralateral frontal bossing was mild. The orbits were tall and turricephaly was present in varying severity. Facial scoliosis as a C-shaped deformity was present in varying severity. The nasal root and chin pointed to the contralateral side. Conclusions: The combination of greater visibility of the ipsilateral ear, contralateral parietal bossing, and C-shaped convex ipsilateral facial scoliosis are hallmark frontofacial features of unilateral lambdoid craniosynostosis. Although the ipsilateral ear is more posterior, the greater visibility may be attributed to lateral displacement from the mastoid bulge. Evaluation of long-term postoperative results is needed to assess if this pathognomonic facial morphology is corrected following posterior vault reconstruction.
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ABSTRACT: The authors observe significant bone gaps upon distractor removal in posterior vault distraction osteogenesis (PVDO). The purpose of this study was to quantify bone gaps upon distractor removal, determine whether they close over time, determine if they predispose to relapse, and investigate whether age affects rate and degree of re-ossification. The authors performed a retrospective review of PVDO patients and included those with computed tomography (CT) scans at 2 timepoints: 1 at completion of consolidation and another at least 4 months later. Using Mimics software, bone gaps were traced to calculate total surface area. A paired t test and linear regression were used to compare size of bone gaps, presence of relapse, and rates of re-ossification. Sixty-nine patients were identified, with 7 meeting inclusion criteria. Three were under 1 year. Consolidation began 28.3 ± 6.0 days after surgery and continued for 64.9 ± 14.5 days. Length of time between CT scans was 7.5 ± 2.7 months. A significant decrease in bone gaps occurred between scans (33.4 ± 14.6 cm2 versus 19.2 ± 17.2 cm2, P = 0.005). After consolidation, ossification occurred at a rate of 2.4 cm2/month (P = 0.046). The rate of bony regeneration in patients under and over 1 year was 4.3 cm3/month (P = 0.025) and 1.5 cm3/month (P = 0.552), respectively. Despite differential bony regeneration rates, no patient demonstrated relapse. From this study, the authors conclude the following. Calvarial bone gaps are present after PVDO consolidation. These gaps undergo re-ossification at a rate that appears to be faster in infants. Overall, they decrease in size over time. The presence of bone gaps does not correlate with relapse of cranial expansion.
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Craneosinostosis , Osteogénesis por Distracción , Humanos , Lactante , Osteogénesis , Osteogénesis por Distracción/métodos , Recurrencia , Estudios Retrospectivos , CráneoRESUMEN
INTRODUCTION: The 2021 interview cycle for craniofacial fellowship applicants was the first to be held virtually due to the coronavirus disease 2019 pandemic. Here, we detail the craniofacial fellowship applicant perceptions and experience on the virtual interview process. MATERIALS AND METHODS: An institutional review board-approved 35-question survey study on the perception of the virtual interview process among craniofacial fellowship applicants was conducted. Surveys were distributed to individuals who had applied through the match, overseen by the American Society of Craniofacial Surgeons (ASCFS). RESULTS: Ten surveys were fully completed with a corresponding response rate of 48%. The average number of interviews completed was 12.7±7.7 and 50% of applicants interviewed at >1 program in a single day. Overall, 90% of respondents preferred in-person interviews before the interview season, however, only 10% preferred the in-person format afterwards. Preference for a virtual-only format increased from 10% to 70%. Applicants cited cost (100%), ease of scheduling (90%), and ability to participate in more interviews (70%) as the primary strengths of the virtual platform; none reported difficulties with self-advocacy. After the interview cycle, 90% stated they would recommend virtual interviews. CONCLUSIONS: The greatest strengths of virtual interviews were the ability to participate in more interviews, the ease of scheduling, and the cost benefits. Most applicants reported the same or increased ability for self-advocacy with virtual interviews. Following the index interview cycle for 2021, the majority of fellowship applicants now appear to prefer a virtual-only or hybrid format and would recommend virtual interviews in the future.
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COVID-19 , Internado y Residencia , Cirujanos , Humanos , Becas , COVID-19/epidemiología , Pandemias , Encuestas y CuestionariosRESUMEN
In just over 100 years, surgical education in the United States has evolved from a disorganized practice to a refined system esteemed worldwide as one of the premier models for the training of physicians and surgeons. But in the changing environment of health care, new challenges have arisen that could warrant a reform. To design our future, we must understand our past. The present work is not intended to be a comprehensive account of the history of American surgery. Instead, it tells the abridged history of surgical education in our country: the evolution from apprenticeships to residencies; the birth of hospital-based teaching; the impact of key historical events on training; the marks left by some preeminent characters; the conception of regulatory entities that steer our education; and, finally, how our process of training surgeons might need to be refined for the continued progress of our profession. Told in chronological order in a manner that will be memorable to readers, this story weaves together the key events that explain how our current surgical training models came to be. We conclude with a timely invitation to draw from these past lessons to redesign the future of graduate medical education, making a case for the transition to time-variable, competency-based medical education for surgical residency programs in America.
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BACKGROUND: Congenital midline nasal masses are rare anomalies and are typically benign nasal dermoid sinus cysts (NDSCs). Rhabdomyosarcomas (RMSs) are even less common, and only a fraction affect sites like the external nose, nasal cavity, nasopharynx, and paranasal sinuses. We review the clinical presentation and treatment of nasal, nasopharyngeal, and paranasal RMSs and report the first documented midline presentation. METHODS: We queried PubMed for articles with titles containing the terms rhabdomyosarcoma or sarcoma botryoides and nose, nasal, paranasal, sinonasal, nasopharynx, or nasopharyngeal. We then searched the references of each included article using the same parameters and continued this process iteratively until no new articles were found. RESULTS: The paranasal sinuses were the most commonly affected site, followed by the nasopharynx, nasal cavity, and external nose. Two patients presented with involvement of the external nose, but each presented with involvement of the right ala rather than a midline mass. The rates of intracranial extension and/or skull base involvement were comparable to those of NDSCs. The alveolar subtype was most common, followed by the embryonal subtype. CONCLUSIONS: Most midline nasal masses are benign; however, we report the first documented presentation of an RMS as a midline nasal mass. Accordingly, RMS should be included in the differential diagnosis of midline nasal masses in the pediatric population. Surgery for midline nasal masses is sometimes delayed due to the risks of interfering with developing structures and early anesthesia. However, early surgical treatment should be considered given this new differential and its predilection for early metastasis.
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BACKGROUND: Management of pediatric facial defects can be challenging, as reattachment of large composite grafts is usually unsuccessful. Hyperbaric oxygen therapy (HBO) has been researched to augment composite graft survival, but clinical use for this application remains anecdotal. The authors present their successful experience managing select cases with large composite grafts and HBO as an adjunct. METHODS: A retrospective chart review identified children presenting with facial defects and managed operatively with large composite grafts (≥1.5â¯×â¯1.5 cm) and HBO therapy. Records were reviewed for defect characteristics, management details, and outcomes at last follow-up. RESULTS: Nine children (avg. 8.4 years, range 1.6-15.1) presented with ear or nose defects secondary to dog bites (n=7), falls (n=1), or congenital causes (n=1). Three experienced ear amputations, and six suffered nasal avulsions of varying degrees. All avulsed ears were reattached. Three cases of nose avulsions were reattached; the other three underwent secondary reconstruction with composite ear grafts. HBO was initiated immediately and continued for 8-10 days. All grafts survived at least 80% with no postoperative complications. At last follow-up (avg. 30.1 months; 0.8-63.9), all patients demonstrated good cosmetic results with minimal residual deformity. CONCLUSION: When reconstruction of pediatric facial defects warrants a large chondrocutaneous graft, immediate postoperative HBO therapy can increase survival. Particularly when reattaching amputated segments, if successful, this approach offers an anatomically ideal result without donor site morbidity. If unsuccessful, it does not "burn bridges" and decreases the extent of secondary reconstruction. The authors present their HBO protocol along with a review of available literature.
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Cara/anomalías , Cara/cirugía , Traumatismos Faciales/cirugía , Supervivencia de Injerto , Oxigenoterapia Hiperbárica , Colgajos Quirúrgicos/irrigación sanguínea , Adolescente , Preescolar , Estética , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
Patients presenting with a unique unilateral cleft phenotype may be at risk of nasal airway obstruction which can be exacerbated by presurgical infant orthopedic (PSIO) appliance therapy and lip taping. Four patients presented to the UPMC Children's Hospital of Pittsburgh Cleft-Craniofacial Center with a cleft phenotype characterized by: An anteriorly projected greater alveolar segment and medial collapse of the lesser segment posteriorly, leading to cleft alar base displacement posteromedial to the anteriorly projected greater segment. Resultant bilateral nasal airway obstruction: cleft ala drape over the leading edge of the greater segment's alveolus (cleft side obstruction) and caudal septum displacement secondary to attachments to the orbicularis oris from the noncleft side (noncleft side obstruction). The patient described presented at 3 months old from an outside institution, where PSIO therapy was undertaken. A second opinion was sought due to concern of significant difficulty in breathing and feeding with the PSIO oral plate. Lip-nose adhesion (LNA) was elected and airway obstruction was immediately relieved after this intervention. Lip-nose adhesion releases the tethered cleft side alar base from the pyriform rim of the posteromedially collapsed lesser segment and unites the superior lip and nostril sill-relieving the cleft side nostril obstruction. During the LNA, the caudal septum is surgically released from the anterior nasal spine and is uprighted, relieving the obstructed noncleft nostril. In this cleft anatomy, the treatment alternatives of modification to the PSIO appliance or LNA should be carefully considered in consultation with the surgeon, PSIO provider, and the infant's caretakers.
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Obstrucción de las Vías Aéreas , Labio Leporino , Fisura del Paladar , Niño , Humanos , Lactante , Nariz , FenotipoRESUMEN
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 3.0â±â4.0 years. The cranial vault was distracted on average 25.0â±â6.0âmm, with those < 12 months of age distracted 29.5â±â4.9âmm and >12 months of age distracted 22.0â±â4.9âmm (Pâ=â0.0543). There was a significant increase in pre- to postoperative foramen magnum surface area (52.1â±â63.2âmm, Pâ=â0.002), length (0.9â±â1.4âmm, Pâ=â0.050), and width (0.6â±â1.0âmm, Pâ=â0.050). Similarly, linear distances between nasion and posterior cranial base landmarks such as foramen magnum (3.4â±â4.2âmm, Pâ=â0.010), and occipital protuberance (9.1â±â9.6âmm, Pâ=â0.003) 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.
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Cefalometría , Base del Cráneo/diagnóstico por imagen , Algoritmos , Niño , Preescolar , Foramen Magno , Humanos , Lactante , Recién Nacido , Hueso Occipital , Osteogénesis por Distracción , Periodo Posoperatorio , Estudios Retrospectivos , Base del Cráneo/cirugía , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVES: The objectives of this study were (1) to evaluate dog bite-related injuries and associated medical documentation and (2) to compare these results with a study of dog bites from the same institution 10 years prior. METHODS: Data were retrospectively collected from a pediatric emergency department from July 2007 to July 2011 for patients treated for dog bites. These data were then compared with data from the same institution from 10 years prior. RESULTS: A total of 1017 bite injuries were treated (average, 254.25 bites/year), which represents a 25% increase compared with 10 years prior. Comparing the 1997 and 2007 to 2011 cohorts, patient demographics, bite rate among children less than 5 years old, rate of dog breed documentation, and setting of injury were similar. Dog breed was reported in 47% (95% confidence interval [CI], 40.2-53.9) and 41% (95% CI, 38.0-44.0) of cases, respectively, in the 2 cohorts. Bites to the craniofacial region were most common (face only reported for 1997: 43.2%; 95% CI, 36.4-50 versus 2007-2011: 66.1%; 95% CI, 63.2-69.0). In both cohorts, the child's home was the most frequent setting, accounting for 43% of bites (1997: 95% CI, 30.2-55.9 and 2007-2011: 95% CI, 39.3-46.7). CONCLUSIONS: Pediatric dog bites continue to occur frequently, and the associated factors did not change over the 10-year period: young age of child, bites to the craniofacial region, and dogs familiar to the child. Although accurate medical documentation of dog bites is a prerequisite to develop effective prevention strategies, current medical documentation of dog bites may be misguided.
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Mordeduras y Picaduras/epidemiología , Mordeduras y Picaduras/prevención & control , Perros , Servicio de Urgencia en Hospital/estadística & datos numéricos , Adolescente , Distribución por Edad , Animales , Mordeduras y Picaduras/terapia , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Mascotas , Estudios Retrospectivos , Factores de Riesgo , Distribución por SexoRESUMEN
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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BACKGROUND: Sphenoid wing dysplasia in patients with neurofibromatosis type 1 may result in challenging and significant changes, including ultimately vision loss. The authors describe the radiographic patterns of sphenoid dysmorphology with time and age, and the impact of surgical intervention on preservation of vision. METHODS: A retrospective study was performed at a single pediatric hospital, identifying subjects with neurofibromatosis type 1. Records were reviewed in their entirety for each subject, with attention to ophthalmologic evaluation. Radiographic images were digitally analyzed and scored for sphenoid transformations on a grade of 0 to 3. RESULTS: Fifty-two subjects were identified. On initial imaging, 42.31 percent had a normal sphenoid (grade 0), 32.69 percent had an abnormal contour (grade 1), 11.54 percent had thinning (grade 2), and 13.46 percent had a gross defect (grade 3). Among the 45 subjects with serial imaging, 55.56 percent demonstrated progression of the deformity of at least one grade. Deformity progression correlated with length of imaging interval. Enucleation was noted to occur more often in subjects with a gross sphenoid defect (p < 0.0001). Of subjects identified as having a gross sphenoid defect, 26.7 percent were managed using orbitosphenoid reconstruction with titanium mesh and cranial bone graft, and 33.3 percent were managed with soft-tissue debulking and canthopexy only. Vision was more likely to be preserved with early orbitosphenoid reconstruction (p < 0.05). CONCLUSIONS: Sphenoid dysplasia is a progressive disease. Loss of vision is associated with a gross defect, and appears to be better preserved with early orbitosphenoid reconstruction with titanium mesh and cranial bone graft.
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Enfermedades del Desarrollo Óseo/cirugía , Trasplante Óseo/métodos , Neurofibromatosis 1/cirugía , Procedimientos de Cirugía Plástica/métodos , Cráneo/trasplante , Hueso Esfenoides/cirugía , Enfermedades del Desarrollo Óseo/diagnóstico por imagen , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Hueso Esfenoides/diagnóstico por imagen , Mallas Quirúrgicas , Trastornos de la Visión/prevención & controlRESUMEN
LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Identify skeletal differences that are treated with orthognathic surgery; describe the goals of orthognathic surgery; and understand modern virtual surgical planning of orthognathic movement of the mandible, maxilla, and chin. 2. Appreciate the surgical principles of maxilla- versus mandible-first surgery, and orthognathic surgery before orthodontic correction; and understand when an osseous genioplasty may be beneficial, and the potency of this bony movement. 3. Appreciate the utility of fat grafting as an adjunct to orthognathic bony movements, and demonstrate understanding of the utility of orthognathic surgery in the treatment of obstructive sleep apnea. 4. Be aware of associated complications and be able to critically assess outcomes following orthognathic surgery. SUMMARY: This CME article outlines the goals of orthognathic surgery, highlighting advances in the field and current controversies. The principles of the sequencing of osteotomies are discussed and literature is reviewed that may assist in decision-making as to maxilla-first versus mandible-first surgery. The emergence of "surgery first," in which surgery precedes orthodontics, is discussed and important parameters for patient candidacy for such a procedure are provided. The emerging standard of virtual surgical planning is described, and a video is provided that walks the reader through a planning session. Soft-tissue considerations are highlighted, especially in the context of osseous genioplasty and fat grafting to the face. The utility of orthognathic surgery in the treatment of obstructive sleep apnea is discussed. The reader is provided with the most current data on complications following orthognathic surgery and advice on avoiding such pitfalls. Finally, outcome assessment focusing on the most current trend of patient-reported satisfaction and the psychological impact of orthognathic surgery are discussed.
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Procedimientos Quirúrgicos Ortognáticos/tendencias , Tejido Adiposo/trasplante , Mentoplastia/métodos , Mentoplastia/tendencias , Humanos , Consentimiento Informado , Mandíbula/cirugía , Maxilar/cirugía , Procedimientos Quirúrgicos Ortognáticos/métodos , Planificación de Atención al Paciente , Complicaciones Posoperatorias/etiología , Cuidados Preoperatorios/métodos , Apnea Obstructiva del Sueño/cirugía , Resultado del TratamientoRESUMEN
BACKGROUND: Cranial base sutures are important drivers of both facial and cranial growth. The purpose of this study was to compare the incidence and location of cranial base suture fusion among three groups: nonaffected controls, patients with nonsyndromic craniosynostosis, and patients with syndromic craniosynostosis. METHODS: Patients and computed tomographic scans were accrued from the authors' prospective craniofacial database. Computed tomographic scans were graded on the frequency of cranial vault and cranial base suture/synchondrosis fusion (0, open; 1, partially/completely fused) by an attending craniofacial surgeon and neuroradiologist. Statistical comparisons were conducted on location and rates of fusion, age, and diagnosis. RESULTS: One hundred forty patients met inclusion criteria: 55 syndromic, 64 nonsyndromic, and 21 controls. Average age at computed tomography of syndromic patients (3.6 ± 3.1 months) was younger than that of nonsyndromic patients (5.4 ± 3.1 months; p = 0.001) and control subjects (5.1 ± 3.2 months; p = 0.058). Syndromic craniosynostotic patients had over three times as many cranial base minor sutures fused (2.2 ± 2.5) as nonsyndromic craniosynostosis patients (0.7 ± 1.2; p < 0.001) and controls (0.4 ± 0.8; p = 0.002), whose rates of fusion were statistically equivalent (p = 0.342). Syndromic craniosynostosis patients had a greater frequency of cranial base suture fusion in the coronal branches, squamosal arch, and posterior intraoccipital synchondrosis (p < 0.05). CONCLUSIONS: Patients with syndromic craniosynostosis have higher rates of cranial base suture fusion in infancy, especially in the coronal arches, and this may have significant implications for both cranial and facial growth. In contrast, patients with nonsyndromic craniosynostosis have similar rates and sites of cranial base suture fusion as controls. Interestingly, there is a low, "normal," rate of cranial base suture/synchondrosis closure in infancy, the implications of which are unknown. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.
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Suturas Craneales/patología , Craneosinostosis/patología , Estudios de Casos y Controles , Suturas Craneales/diagnóstico por imagen , Craneosinostosis/diagnóstico por imagen , Femenino , Humanos , Lactante , Modelos Logísticos , Masculino , Oportunidad Relativa , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Although studies have analyzed quality of life in children with nonsyndromic craniosynostosis, to date nobody has investigated long-term quality of life in adults with nonsyndromic craniosynostosis. The purpose of this study was to compare quality of life in adult nonsyndromic craniosynostosis patients with a cohort of unaffected controls. METHODS: The authors queried their institution's prospectively maintained craniofacial registry for nonsyndromic craniosynostosis patients aged 18 years and older, and administered the validated World Health Organization Quality of Life Biomedical Research and Education Foundation questionnaire. Responses were compared, using a two-sample t test, to an age-matched U.S. normative database provided by the World Health Organization. RESULTS: One hundred fifty-one adults met inclusion criteria: 52 were successfully contacted and 32 completed the questionnaire. Average age of respondents was 23.0 ± 6.1 years (range, 18.1 to 42.1 years). Nonsyndromic craniosynostosis patients had a superior quality of life compared with comparative norms in all domains: physical health (17.8 ± 2.7 versus 15.5 ± 3.2; p < 0.001), psychological (16.3 ± 2.9 versus 13.8 ± 3.2; p < 0.001), social (16.9 ± 2.6 versus 13.2 ± 3.6; p < 0.001), and environmental (17.2 ± 2.5 versus 11.7 ± 2.7; p < 0.001). Comparison between single-suture subtypes revealed no difference in any quality-of-life domain (p > 0.05). CONCLUSIONS: Adult patients previously treated for nonsyndromic craniosynostosis perceive their quality of life to be high, superior to that of a normative U.S. SAMPLE: Future work will seek to analyze additional patients and better understand the reasons behind these findings.
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Craneosinostosis/psicología , Calidad de Vida , Adolescente , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Sistema de Registros , Reoperación/psicología , Reoperación/estadística & datos numéricos , Encuestas y Cuestionarios , Adulto JovenRESUMEN
BACKGROUND: Posterior vault distraction osteogenesis (PVDO) is believed to improve frontal contour in infants with syndromic craniosynostosis and turribrachycephaly. This study provides an objective craniometric analysis to determine how PVDO affects anterior cranial morphology. METHODS: A retrospective chart review of patients who underwent PVDO was performed. Inclusion criteria included pre- and postoperative computed tomography (CT) scans within 3 months before surgery and another 1 to 6 months after device removal. Volumetric and craniometric data were derived using Mimics software and compared using paired t-test and Wilcoxon rank-sum test. RESULTS: About 65 patients underwent PVDO, and 13 patients met inclusion criteria. Mean age at intervention was 3.4â±â4.2 years. Total cranial volume increased 249â±â159 cm in all patients (Pâ=â0.0001) and 380â±â128âcm in patients younger than 1 year of age (nâ=â6, Pâ=0â.0008). Supraorbital retrusion decreased from 5.44â±â3.89 to 4.54â±â3.91âmm postoperatively (Pâ=â0.0004), decreasing significantly in patients without previous frontal surgery and not in those with previous frontal surgery (Pâ=â0.2115; comparison Pâ=â0.0047). Basofrontal angle decreased by 2.92â±â2.16 degrees (Pâ=â0.0004) with a greater decrease of 3.33±2.68 degrees in those younger than 12 months (Pâ=â0.0289) and 2.58±1.74 degrees in those older (Pâ=â0.0079). No change was found in anterior cranial height and anterofrontal angle (Pâ>â0.05). CONCLUSION: PVDO improves frontal contour by decreasing supraorbital retrusion and reducing frontal bossing in syndromic craniosynostosis patients with turribrachycephaly. When combined with its demonstrated efficacy for cranial expansion, these frontal changes likely reinforce PVDO's ability to influence the timing of, and to a degree, the need for frontal surgery in this group.
Asunto(s)
Craneosinostosis/cirugía , Osteogénesis por Distracción , Cráneo/patología , Cráneo/cirugía , Cefalometría , Niño , Preescolar , Craneosinostosis/diagnóstico por imagen , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Tamaño de los Órganos , Estudios Retrospectivos , Cráneo/anomalías , Cráneo/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
PURPOSE: The purpose of this study was to evaluate the indications, safety, and short-term outcomes of posterior vault distraction osteogenesis (PVDO) in patients with no identified acrocephalosyndactyly syndrome (study) and to compare those to a syndromic cohort (controls). METHODS: Demographic and perioperative data were recorded and compared across the study and control groups for those who underwent PVDO between January 2009 and December 2016. Univariate analysis was conducted using χ and Fisher exact tests for categorical variables, and Mann-Whitney U test for continuous variables. RESULTS: Sixty-three subjects were included: 19 in the nonsyndromic cohort, 44 in the syndromic cohort. The cohorts had similar proportion of subjects exhibiting pansynostosis (42.1% of nonsyndromic versus 36.4% of syndromic, Pâ=â0.667). The nonsyndromic cohort was significantly older (4.04â±â3.66 years versus 2.55â±â3.34 years, Pâ=â0.046) and had higher rate of signs of raised intracranial pressure (68.4% versus 25.0%, Pâ=â0.001) than the syndromic cohort. There was no significant difference in perioperative variables or rate of complications (Pâ>â0.05). The mean total advancement distance achieved was similar, 27â±â6âmm in the nonsyndromic versus 28â±â8âmm in the syndromic cohort (Pâ=â0.964). All nonsyndromic subjects with signs of raised intracranial pressure demonstrated improvement at an average follow-up of 22 months. CONCLUSION: As in the syndromic patient, PVDO is a safe and, in the short-term, effective modality for cranial vault expansion in the nonsyndromic patient. The benefits and favorable perioperative profile of PVDO may therefore be extended to patient populations other than those with syndromic craniosynostosis.