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Background: Unlike in adults, pediatric head and neck desmoid tumors (DTs) have greater capacity to interfere with normal anatomical development. Therefore, adequate interventions and management must be defined. We aimed to provide the most comprehensive systematic review on pediatric head and neck DTs to date, including assessment of lesion location predominance, intervention, and management, and examination of any associations between age and outcomes like surgical margin status, recurrence, and complications. Methods: A systematic literature review was conducted between January 1990 and December 2023 using PubMed, Scopus, and MEDLINE databases following the Preferred Reporting Items for Systematic Review and Meta-Analyses 2020 guidelines. We aimed to elucidate intervention and management strategies by studying various outcomes in 0-11 and 12-21 year olds. Results: The literature search yielded 44 studies, totaling 121 patients. Most head and neck DTs localized to the mandible, cranium, and neck; occurred early (P = 4.18 years); and underwent local resection with positive margins. Older and younger patients shared no difference in complication or recurrence rates. Conclusions: We found recurrence is likely to occur with positive margins. Because standard treatment of DTs is surgical resection with negative margins, if technically feasible with reasonable associated morbidity, we suggest additional resection of the tumor to achieve negative margins. We also recommend more robust follow-up data collection, not only due to desmoid's high recurrence and data paucity in patients older than 12 years, but also, to better establish best management practices.
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A 13-year-old girl with a painful left neck mass was referred to our institution due to suspicions of malignancy. The patient reported pain that accompanied her frequent neck spasms. Computed tomography revealed a large, soft-tissue mass in the left neck, deep to the sternocleidomastoid. The lesion anteriorly displaced the internal carotid artery and both displaced and crushed the internal left jugular vein. Uniquely, a three-dimensional virtual reality model combining magnetic resonance imaging and computed tomography data was used to determine the lesion's resectability and visualize which structures would be encountered or require protection while ensuring total resection. During operation, we confirmed that the mass also laterally displaced the brachial plexus, cranial nerves X and XI, and spinal nerves C3-C5 (including the phrenic) of the cervical plexus. Postsurgical pathological analysis confirmed a diagnosis of desmoid tumor, also known as aggressive fibromatosis, whereas DNA sequencing revealed a CTNNB1 mutation, a somatic genetic marker found in approximately 90% of desmoid tumor cases. When possible, the most widely used method for the treatment of desmoid tumors has been gross resection. Chemotherapy, radiotherapy, and local excision are also used in the treatment of fibromatoses when complete resection is judged infeasible. In this case, a complete surgical resection with tumor-free surgical margins was performed. A standard cervical approach with a modified posterolateral incision site was implemented to avoid a conspicuous anterior neck scar. No flap, nerve repair, or reconstruction was warranted. At 1 year of postsurgical follow-up, the patient showed minimal scarring and no signs of recurrence.
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Neoplasias do Ânus/cirurgia , Nádegas/cirurgia , Doença de Paget Extramamária/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Protectomia/métodos , Retalhos Cirúrgicos , Idoso , Humanos , Masculino , Posicionamento do Paciente , Cuidados Pré-Operatórios , Protectomia/efeitos adversos , Procedimentos de Cirurgia Plástica/efeitos adversos , Procedimentos Cirúrgicos Robóticos/efeitos adversos , Procedimentos Cirúrgicos Robóticos/métodos , Retalhos Cirúrgicos/efeitos adversosRESUMO
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.
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Craniossinostoses/cirurgia , Craniotomia/métodos , Deficiências do Desenvolvimento/genética , Procedimentos de Cirurgia Plástica/métodos , Proteína Smad6/genética , Estudos de Casos e Controles , Criança , Comportamento Infantil , Desenvolvimento Infantil , Pré-Escolar , Craniossinostoses/complicações , Craniossinostoses/genética , Deficiências do Desenvolvimento/diagnóstico , Deficiências do Desenvolvimento/prevenção & controle , Método Duplo-Cego , Feminino , Seguimentos , Humanos , Lactente , Testes de Inteligência , Mutação com Perda de Função , Masculino , Estudos Prospectivos , Crânio/cirurgia , Resultado do TratamentoAssuntos
Aplicativos Móveis , Procedimentos de Cirurgia Plástica/instrumentação , Cirurgia Assistida por Computador/métodos , Cirurgia Plástica/instrumentação , Previsões , Humanos , Procedimentos de Cirurgia Plástica/métodos , Cirurgia Assistida por Computador/tendências , Cirurgia Plástica/métodosRESUMO
Burns constitute a significant portion of the worldwide disability adjusted life years by compromising form and function. Through the field's numerous reconstructive techniques, plastic surgery can treat many of these deficiencies stemming from burn injuries. We describe the steps necessary to establish international burn missions including realizing synergies among nonprofits and academic plastic surgery centers to restore form and function to burn patients.
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Queimaduras/cirurgia , Missões Médicas/organização & administração , Saúde Global , Humanos , Índia , Satisfação do Paciente , Desenvolvimento de ProgramasRESUMO
PURPOSE: Use of tissue-engineered vascular grafts (TEVGs) in the repair of congenital heart defects provides growth and remodeling potential. Little is known about the mechanisms involved in neovessel formation. We sought to define the role of seeded monocytes derived from bone marrow mononuclear cells (BM-MNCs) on neovessel formation. METHODS: Small diameter biodegradable tubular scaffolds were constructed. Scaffolds were seeded with the entire population of BM-MNC (n = 15), BM-MNC excluding monocytes (n = 15), or only monocytes (n = 15) and implanted as infrarenal inferior vena cava (IVC) interposition grafts into severe combined immunodeficiency/bg mice. Grafts were evaluated at 1 week, 10 weeks, or 6 months via ultrasonography and microcomputed tomography, as well as by histologic and immunohistochemical techniques. RESULTS: All grafts remained patent without stenosis or aneurysm formation. Neovessels contained a luminal endothelial lining surrounded by concentric smooth muscle cell layer and collagen similar to that seen in the native mouse IVC. Graft diameters differed significantly between those scaffolds seeded with only monocytes (1.022 +/- 0.155 mm) and those seeded without monocytes (0.771 +/- 0.121 mm; P = .021) at 6 months. CONCLUSIONS: Monocytes may play a role in maintaining graft patency. Incorporation of such findings into the development of second-generation TEVGs will promote graft patency and success.
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Prótese Vascular , Monócitos/citologia , Engenharia Tecidual , Alicerces Teciduais , Veia Cava Inferior/transplante , Animais , Proliferação de Células , Modelos Animais de Doenças , Feminino , Sobrevivência de Enxerto , Humanos , Imuno-Histoquímica , Camundongos , Camundongos SCID , Desenho de Prótese , Grau de Desobstrução Vascular/fisiologia , Veia Cava Inferior/citologiaRESUMO
Previous studies have demonstrated the benefits of cell seeding in the construction of tissue-engineered vascular grafts (TEVG). However, seeding methods are diverse and no method is clearly superior in either promoting seeding efficiency or improving long-term graft function. As we head into an era during which a variety of different TEVG are under investigation in clinical trials around the world, it is important to consider the regulatory issues surrounding the translation of these technologies. In this review, we summarize important advances in the field of vascular tissue engineering, with particular attention on cell-seeding techniques for TEVG development and special emphasis placed on regulatory issues concerning the clinical translation of these various methods.