NIVALDO ALONSO
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Cirurgia, Faculdade de Medicina - Docente
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/04 - Laboratório de Microcirurgia, Hospital das Clínicas, Faculdade de Medicina - Líder
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/04 - Laboratório de Microcirurgia, Hospital das Clínicas, Faculdade de Medicina - Líder
9 resultados
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Agora exibindo 1 - 9 de 9
- Abordagem cirúrgica no tratamento da displasia fibrosa craniofacialexperiência de 14 anos(2014) ALONSO, NIVALDO; MATUSHITA, HAMILTON; ALESSI, MARIANA SISTOABSTRACT Introduction: Fibrous dysplasia is benign tumor of the craniofacial skeleton that primarily affects young patients. It is characterized by the progressive growth of benign fibrous tumors with resulting functional and aesthetic deformities. This study assesses the clinical and prognostic features in patients with fibrous dysplasia who underwent surgical treatment at our institution. Methods: Retrospective analysis of 19 patients with craniofacial fibrous dysplasia, treated between January 1997 and December 2011 with bone remodeling and surgical resection. We also review the literature regarding fibrous dysplasia. Results: Patients ranged between 8-65 years old, with a mean age of 21.75 years. Ten patients (52.7%) were women. The polyostotic form was predominant and present in15 cases (78.9%). The sphenoid, ethmoid, and frontal bones were most commonly involved in the polyostotic form and the mandibular and zygomatic bones were most commonly involved in the monostotic form. The main complaint was asymmetry of the face. One patient developed decreased visual acuity. Treatment was based on surgical resection and graft reconstruction in the localized form of the disease, and bone abrasion and remodeling in the polyostotic form. Intracranial access was necessary in only one case (5.2%) where the optic nerve was compressed. Repeat surgical treatment due to recurrent tumor growth was necessary in three patients. The only complication occurred in a patient who developed lagophthalmos and epicanthus postoperatively after undergoing surgery using infraorbital access. No other complications occurred during shortand long-term follow-up. Functional preservation and facial contour recovery outcomes were satisfactory. Conclusion: Our experience, along with that of other investigators, demonstrates that surgery is effective in treating selected cases of craniofacial fibrous dysplasia.
- FGFR2 Mutation Confers a Less Drastic Gain of Function in Mesenchymal Stem Cells Than in Fibroblasts(2012) YEH, Erika; ATIQUE, Rodrigo; ISHIY, Felipe A. A.; FANGANIELLO, Roberto Dalto; ALONSO, Nivaldo; MATUSHITA, Hamilton; ROCHA, Katia Maria da; PASSOS-BUENO, Maria RitaGain-of-function mutations in FGFR2 cause Apert syndrome (AS), a disease characterized by craniosynostosis and limb bone defects both due to abnormalities in bone differentiation and remodeling. Although the periosteum is an important cell source for bone remodeling, its role in craniosynostosis remains poorly characterized. We hypothesized that periosteal mesenchymal stem cells (MSCs) and fibroblasts from AS patients have abnormal cell phenotypes that contribute to the recurrent fusion of the coronal sutures. MSCs and fibroblasts were obtained from the periostea of 3 AS patients (S252W) and 3 control individuals (WT). We evaluated the proliferation, migration, and osteogenic differentiation of these cells. Interestingly, S252W mutation had opposite effects on different cell types: S252W MSCs proliferated less than WT MSCs, while S252W fibroblasts proliferated more than WT fibroblasts. Under restrictive media conditions, only S252W fibroblasts showed enhanced migration. The presence of S252W mutation increased in vitro and in vivo osteogenic differentiation in both studied cell types, though the difference compared to WT cells was more pronounced in S252W fibroblasts. This osteogenic differentiation was reversed through inhibition of JNK. We demonstrated that S252W fibroblasts can induce osteogenic differentiation in periosteal MSCs but not in MSCs from another tissue. MSCs and fibroblasts responded differently to the pathogenic effects of the FGFR2(S252W) mutation. We propose that cells from the periosteum have a more important role in the premature fusion of cranial sutures than previously thought and that molecules in JNK pathway are strong candidates for the treatment of AS patients.
- Frontal-orbital advancement for the management of anterior plagiocephaly(2012) MATUSHITA, Hamilton; ALONSO, Nivaldo; CARDEAL, Daniel Dante; ANDRADE, Fernanda deThe main purposes of this manuscript are to provide an overview of various modalities of surgical correction of anterior plagiocephaly and to emphasize their differences with the classic open frontal-orbital advancement. Advancement of technology provides development of many other ways to achieve the same results. The authors describe the classic open frontal-orbital advancement and compare with other proposed techniques for correction of frontal plagiocephaly. The main limitation of the use of new forms of treatment of the anterior plagiocephaly is the age of the patient. There is still no consensus on criteria for quantitative evaluation of surgical results, and new forms of treatment do not present results with long follow-up. Frontal-orbital advancement is the preferred procedure to correct unicoronal synostosis due to its universal indication regardless of the age and degree of deformation of the anterior plagiocephaly.
- Frontofacial Monobloc Advancement With Internal Distraction: Surgical Technique and Osteotomy Guide(2022) FERREIRA JUNIOR, Tancredo Alcantara; FONTOURA, Renato Rinco; NASCIMENTO, Leyzeane Marques do; ALCANTARA, Mariana Torres; CAPUCHINHO-JUNIOR, Geraldo Andrade; ALONSO, Nivaldo; MATUSHITA, Hamilton; COSTA, Bruno Silva; LIMA, Franklin Bernardes Faraj deBACKGROUND:Craniosynostosis are cranial deformities resulting from the early closure of 1 or more sutures. Concomitant facial changes are complex and usually result from the involvement of multiple sutures, which may lead to restriction of cranial growth and brain expansion, ocular compression, and breathing difficulties. Surgical techniques to correct syndromic craniosynostosis have improved over time, considerably reducing the rate of complications of this procedure.OBJECTIVE:To describe in detail (step-by-step) and with pertinent anatomic considerations the technique of monobloc frontofacial advancement using internal distractors.METHODS:We describe the monobloc frontofacial advancement technique with the use of internal distractors, which we use in patients with primary syndromic craniosynostosis (Apert, Crouzon, and Pfeiffer) who have major facial hypoplasia and secondary respiratory repercussions. To illustrate this technique, the procedure was performed in 2 cranial models: an adult artificial acrylic skull of normal morphology for better evidence of anatomic repairs and a 3-dimensional printed infant skull from a tomography file obtained from a child diagnosed with Apert syndrome.RESULTS:The benefits of osteogenic distraction and better surgical timing for each procedure are presented. We presented the changes and details of osteotomies performed during the procedure, as well as anatomic details and care regarding the pterygomaxillary dysjunction.CONCLUSION:Monobloc frontofacial distraction is a procedure with widely demonstrated aesthetic and functional results, and this detailed step-by-step description may improve familiarity with the anatomic landmarks of the procedure and provide a better dynamic understanding of the distraction process.
bookPart Cranioestenose(2015) MATUSHITA, Hamilton; ALONSO, Nivaldo; CARDEAL, Daniel Dante; ANDRADE, Fernanda- Novel Molecular Pathways Elicited by Mutant FGFR2 May Account for Brain Abnormalities in Apert Syndrome(2013) YEH, Erika; FANGANIELLO, Roberto D.; SUNAGA, Daniele Y.; ZHOU, Xueyan; HOLMES, Gregory; ROCHA, Katia M.; ALONSO, Nivaldo; MATUSHITA, Hamilton; WANG, Yingli; JABS, Ethylin W.; PASSOS-BUENO, Maria RitaApert syndrome (AS), the most severe form craniosynostosis, is characterized by premature fusion of coronal sutures. Approximately 70% of AS patients carry S252W gain-of-function mutation in FGFR2. Besides the cranial phenotype, brain dysmorphologies are present and are not seen in other FGFR2-asociated craniosynostosis, such as Crouzon syndrome (CS). Here, we hypothesized that S252W mutation leads not only to overstimulation of FGFR2 downstream pathway, but likewise induces novel pathological signaling. First, we profiled global gene expression of wild-type and S252W periosteal fibroblasts stimulated with FGF2 to activate FGFR2. The great majority (92%) of the differentially expressed genes (DEGs) were divergent between each group of cell populations and they were regulated by different transcription factors. We than compared gene expression profiles between AS and CS cell populations and did not observe correlations. Therefore, we show for the first time that S252W mutation in FGFR2 causes a unique cell response to FGF2 stimulation. Since our gene expression results suggested that novel signaling elicited by mutant FGFR2 might be associated with central nervous system (CNS) development and maintenance, we next investigated if DEGs found in AS cells were also altered in the CNS of an AS mouse model. Strikingly, we validated Strc (stereocilin) in newborn Fgfr2(S252W/+) mouse brain. Moreover, immunostaining experiments suggest a role for endothelial cells and cerebral vasculature in the establishment of characteristic CNS dysmorphologies in AS that has not been proposed by previous literature. Our approach thus led to the identification of new target genes directly or indirectly associated with FGFR2 which are contributing to the pathophysiology of AS.
- FGFR2 Mutation Confers a Less Drastic Gain of Function in Mesenchymal Stem Cells than in Fibroblasts (vol 8, pg 685, 2012)(2013) YEH, Erika; ATIQUE, Rodrigo; ISHIY, Felipe A. A.; FANGANIELLO, Roberto Dalto; ALONSO, Nivaldo; MATUSHITA, Hamilton; ROCHA, Katia Maria da; PASSOS-BUENO, Maria Rita
- Idade e indicações de osteotomias para avanço frontofacial em pacientes com craniossinostoses sindrômicas(2012) ALONSO, Nivaldo; MATUSHITA, Hamilton; GOLDENBERG, Dov Charles; BASTOS, Endrigo OliveiraBACKGROUND: Craniofacial surgery has overcome many challenges since its initiation into clinical practice. Several technical issues have been addressed and the basic infrastructure of the specialty has now been developed. At present, 25 years after the first publications on frontofacial advancement, questions still remain as to the appropriate age for surgery and the appropriate type of surgery that should be performed. The aim of this study was to evaluate patients surgically treated for syndromic craniosynostosis over the last 10 years at our institution. METHODS: All syndromic patients who underwent monobloc frontofacial advancement or only isolated facial advancement from 2001 to 2011were selected. Out of 70 patients in total, 56 underwent monobloc frontofacial advancement and 14 underwent facial advancement after fronto-orbital remodeling. All data concerning these patients were correlated with patient age and final result. Moreover, age at surgery, complications, and final results were correlated with the main preexisting problems. RESULTS: Final results for syndromic patients varied, depending on the syndrome and the age at which the procedure was performed. Monobloc frontofacial advancements had a low index of immediate postoperative complications, but there was a clear need for further procedures at the time of final facial growth. The index of positive outcome was higher in patients who underwent surgery at an older age. CONCLUSIONS: In cases of severe craniosynostosis with functional problems, monobloc frontofacial advancement is still the best therapeutic option.
- Major clinical features of synostotic occipital plagiocephaly: mechanisms of cranial deformations(2014) MATUSHITA, Hamilton; ALONSO, Nivaldo; CARDEAL, Daniel Dante; ANDRADE, Fernanda Goncalves deThe clinical diagnosis of most common single-suture craniosynostosis is easily set, based on the stereotype of deformities and knowledge of the mechanisms of cranial deformations. However, synostosis of unilateral lambdoid suture, probably due to its lower incidence and similarity with other non-synostotic deformities affecting the posterior portion of the skull, makes its clinical diagnosis more difficult and imprecise. The aim of this study is to evaluate the most easily and accurate clinical characteristics to be recognized in the synostotic occipital plagiocephaly. This study consisted of clinical evaluation of eight patients with synostotic occipital plagiocephaly, whose diagnosis was further corroborated by computed tomography. We identified the following: unilateral occipital flattening in eight out of eight patients (100 %), bulging of ipsilateral mastoid process in eight out of eight (100 %), ""edge effect"" of ipsilateral lambdoid suture in eight out of eight (100 %), inferior deviation of the ear in eight out of eight (100 %), ""Dumbo"" ears in eight out of eight (100 %), horizontal slant of the bimastoid line in seven out of eight (87.5 %), tilt of the head viewed from behind in seven out of eight (87.5 %), trapezoidal contour of the skull in top view in six out of eight (75 %), contralateral parietal bossing in six out of eight (75 %), and bossing of the contralateral forehead three out of eight (37.5 %). The most important clinical features specific to the clinical diagnosis of synostotic occipital plagiocephaly, not present in the positional posterior plagiocephaly, were bulging of the ipsilateral mastoid process, edge effect of the synostotic lambdoid suture, tilt of the head, and slant of the bimastoid line viewed from behind, inferior deviation of the ear, and contralateral parietal bossing.