Towards the targeted management of hereditary gingival fibromatosis.

Although hereditary gingival fibromatosis is a rare condition, it leaves unrestroable psychological, esthetical as well as functional problems to the affected patients. The purpose of the current research is to find a non-surgical pharmacological mechanism that could provide a control of the active phase of such disease helping individual to continue their lives.

The Autosomal Dominant Inheritance of Hereditary Gingival Fibromatosis: A Case Report.

Hereditary gingival fibromatosis (HGF) is a rare disorder characterized by progressive and varying degrees of gingival overgrowth. Oral manifestations vary from minimal to generalized enlargement, and HGF may occur as an isolated disorder or a feature of a syndrome. Unaesthetic appearance and functional impairment due to excessive gingival tissue dictate the need for surgical intervention. This article describes a family with three generations afflicted with a non-syndromic form of HGF with distinctive clinical, histological characteristics. Further, it highlights the need for genetic analysis and appropriate surgical technique to manage gingival enlargement and avoid recurrence.

Complete oral rehabilitation of a patient with hereditary gingival fibromatosis: a clinical report.

Hereditary gingival fibromatosis is a rare disease that has several debilitating oral and psychological manifestations, especially with young children and adolescents. The complexity of the treatment as well as the timing relative to the growth and maturation of the patient present a challenge. Few treatment options have been reported in the literature. This clinical report presents a multidisciplinary approach to treating a young patient with hereditary gingival fibromatosis by using implant-supported prostheses. The treatment steps and its outcome are discussed.

The orthodontic and surgical management of Zimmerman-Laband syndrome.

Zimmermann-Laband syndrome (ZLS) is a rare autosomal disorder characterized by gingival fibromatosis, abnormalities of the nose and/or ears, hypoplasia of the nails or terminal phalanges of the hands and feet, hyperextensibility of joints, hepatosplenomegaly, hirsutism, and intellectual disability. The characteristics of the syndrome are highly variable and complicated. This paper reports a case of ZLS and the clinical manifestations encountered in relation to the current available literature. The comprehensive management of the patient's dentition is described, focusing on the orthodontic and surgical considerations clinicians should be aware of when treating patients with the syndrome.

Hereditary gingival fibromatosis: a three-generation case and pathogenic mechanism research on progress of the disease.

BACKGROUND: Hereditary gingival fibromatosis (HGF) is a rare benign disorder characterized by progressive overgrowth of gingiva. Although the clinical and histopathologic characteristics of HGF are explicit, the pathogenic mechanism remains unclear. The goal of this article is to describe a three-generation HGF case and discuss the diagnosis, treatment, and inheritance of the disease. The known cellular and molecular features of HGF are also emphasized. METHODS: Family and medical histories of the patients were recorded, and a series of preliminary examinations, including clinical, histologic, radiographic, and gene examination, were performed to make a diagnosis and learn about the genetic characteristics. An all-quadrant flap surgery was performed to remove excess gingiva, and orthodontic treatment was undertaken to help tooth eruption. Recent advances were reviewed for further knowledge of genetic, cellular, and molecular features of HGF. RESULTS: The patient's manifestations and examinations showed a typical HGF characteristic. There was no recurrence after surgery, and the premolars and molars erupted to bite plane. Genetic studies have found several gene mutations involved in HGF. Only the son-of-sevenless-1 gene is identified. Multiple molecular factors, such as transforming growth factor-ß and matrix metalloproteinases, participate in HGF, regulating the extracellular matrix. CONCLUSIONS: Surgical intervention is the usual treatment of HGF, but patients still have to deal with the risk of recurrence. Once the correlations between gene mutations, molecular changes, histology, and clinical situation are clear, they can be applied to clinical application, providing novel methods for disease prognosis and diagnosis and targets for disease prevention and treatment.

Fibromatosis gingival: diagnóstico y tratamiento: reporte de un caso / Gingival fibromatosis: diagnosis and treatment: case report

La fibromatosis gingival es una deformidad desarrollada de baja prevalencia. Las características clínicas de la FG incluyen tejidos gingivales hiperplásicos de consistencia firme y nodular, presentándose en forma generalizada o localizada. Las características histológicas de la FG incluyen principalmente tejido conectivo con un denso infiltrado rico en fibras de colágeno y un epitelio denso con papilas epiteliales largas. Se presenta un reporte de caso de un paciente masculino de 12 años referido para su tratamiento al servicio de Periodoncia e Implantes dentales de la Clínica Estomatológica Central de la Universidad Peruana Cayetano Heredia.

Gingival fibromatosis is a developed deformity with low prevalence. The clinical features of FG include hyperplastic gingival tissues usually firm and nodular, occurring in a generalized or localized form. The histologic features of FG mainly include connective tissue with a dense infiltrate rich in collagen fibers and dense epithelium with epithelial papillae long. This is a case report of a male patient of 12 years referred for treatment to a Periodontics and Implants Service of the Central Dental Clinic of the Cayetano Heredia Peruvian University.

Report of a case of Zimmermann-Laband syndrome with new manifestations.

Zimmermann-Laband syndrome is a rare disorder characterized by gingival fibromatosis, abnormalities of the nose and/or ears, absence and/or hyperplasia of the nails or terminal phalanges of the hands and feet, hyperextensibility of joints, hepatosplenomegaly, mild hirsutism and mental retardation. The syndromic characteristics of Zimmermann-Laband syndrome are highly variable and complicated. This paper described a patient with Zimmermann-Laband syndrome with new manifestations and discusses the possible underlying genetic mechanisms.

Idiopathic gingival fibromatosis: description of two cases.

Gingival fibromatosis is an enlargement localized or generalized of the gingival tissue characterized by an expansion and accumulation of the connective tissue, predominantly type I collagen, with occasional presence of increased number of cells, supposed fibroblastic proliferation. Gingival fibromatosis can be induced as a side effect of systemic drugs, such as phenytoin, cyclosporin, and nifedipine, or due to hereditary factors. However, in some cases, the gingival overgrowth is idiopathic. This paper reports two cases of idiopathic gingival fibromatosis and discusses the diagnosis, histopathological features, treatment and immunohistochemical evaluation of myofibroblasts of this condition. The tissues removed were fixed in formalin, and sections used for hematoxylin and eosin and Masson tricromic stain. To determine the presence of myofibroblasts, we performed immunohistochemistry against a-SMA protein. Histological examination revealed epithelial hyperplasia with long rete pegs and increase in the dense fibrous connective tissue. The Masson tricromic stain revealed wide bundles of collagen strongly stained. It was showed negative labeling to a-SMA. These results strongly suggest that myofibroblasts are not involved in gingival overgrowth in the cases of IGF reported. Future studies will be necessary to determine the pathogenesis of idiopathic gingival fibromatosis.

Fibromatosis gingival y amelogenésis imperfecta. Evaluación y tratamiento / Gingival fibromatosis and amelogenesis imperfecta. Assessment and treatment

El presente artículo reporta el caso clínico y tratamiento de un paciente de 23 años de edad de sexo femenino, que asiste a la consulta por presentar aumento de volumen en la mucosa oral correspondiente a la encía del maxilar superior, lo que le impide cerrar la boca y juntar los labios, así como en la región de la tuberosidad del maxilar superior el agrandamiento gingival no le permite pasar los alimentos con facilidad, en el examen clínico se observó dientes con alteraciones compatibles con amelogenésis imperfecta. La paciente fue tratada bajo anestesia general en el quirófano, realizando tratamiento quirúrgico de gingivoplastía, osteotomía, exodóncias múltiples y prótesis fija antero superior.

This article reports the case of a patient 23 years old female, who attends consulted for increased volume in the oral mucosa corresponding to maxillary gingival, which prevents it from closing the mouth and lips together and in the region of the maxillary tuberosity gingival enlargement does not allow food to pass easily. The patient was treated under general anesthesia in the operating room, performance surgical treatment of gingivoplasty, osteotomy, multiple extractions and upper anterior fixed prosthesis.

Idiopathic gingival fibromatosis associated with generalized aggressive periodontitis: a case report.

Idiopathic gingival fibromatosis, a benign, slow-growing proliferation of the gingival tissues, is genetically heterogeneous. This condition is usually part of a syndrome or, rarely, an isolated disorder. Aggressive periodontitis, another genetically transmitted disorder of the periodontium, typically results in severe, rapid destruction of the toothsupporting apparatus. The increased susceptibility of the host population with aggressive periodontitis may be caused by the combined effects of multiple genes and their interaction with various environmental factors. Functional abnormalities of neutrophils have also been implicated in the etiopathogenesis of aggressive periodontitis. We present a rare case of a nonsyndromic idiopathic gingival fibromatosis associated with generalized aggressive periodontitis. We established the patient"s diagnosis through clinical and radiologic assessment, histopathologic findings and immunologic analysis of neutrophil function with a nitro-blue-tetrazolium reduction test. We describe an interdisciplinary approach to the treatment of the patient.

Fibromatose gengival hereditária: identificação e tratamento / Hereditary Gingival Fibromatosis: Identification and Treatment

A Fibromatose Gengival Hereditária (FGH) é descrita como uma condição bucal rara, clinicamente manifestada por um crescimento lento, progressivo, difuso e benigno dos tecidos gengivais. Alguns autores defendem a hipótese de o aumento gengival ser transmitido por um gene autossômico dominante, porém outros mostram que a FGH parece ser transmitida por um gene autossômico recessivo, em que pessoas não afetadas transmitemessa anormalidade a seus descendentes. O modo de transmissão hereditária parece ser autossômicodominante, relacionado ao cromossomo 2p21. A FGH pode se manifestar, de um modo geral, em duas situações diferentes: a primeira, quando o crescimento exagerado da gengiva constitui a única alteração patológica clinicamente observável, e a segunda, quando a fibromatose gengival é uma das alterações que se manifesta associada a outras patologias sistêmicas, como o retardo mental, a epilepsia, a hipertricose e a surdez progressiva. Através de uma revisão da literatura, este trabalho buscou elucidar todas as suas características, objetivando facilitar sua identificação, tratamento e controle

Hereditary gingival fibromatosis (HGF) is described as a rare oral condition, clinically manifested by a slow, progressive and benign enlargement of gingival tissues. Some authors support the hypothesis that HGF is transmitted by an autosomal dominant gene, while others think that HGF seems to be transmitted by an autosomal recessive gene, when non-affected subjects transmit this abnormality to their descendants. Thehereditary transmission mode seems to be an autosomal dominant inheritance related to the chromosome 2p21. In general, HGF can be observed in two different clinical situations: the first, when the exaggerated growth of the gingiva constitutes the only clinical pathological alteration observable and the second, when gingival fibromatosis associates with other systemic conditions, such as mental retardation, epilepsy, hypertrichosis and gradual deafness. The aim of this study was to review the literature on hereditary gingival fibromatosis and describe its characteristics to enable the diagnosis, treatment and control of this disease

Fibromatose gengival anatômica: caso clínico / Anatomical gingival fibromatosis: a clinical case

As fibromatoses gengivais são lesões proliferativas, não neoplásicas, raras na cavidade bucal, sendo caracterizadas por um aumento gengival lento e progressivo. Podem ser classificadas em hereditária, irritativa, medicamentosa e anatômica. Este artigo relata o caso clínico de um paciente de 56 anos que apresentou hiperplasia gengival da região de molares ao tuber da maxila estendendo-se para porção posterior do palato, bilateralmente, com maior envolvimento do lado direito. Essa lesão caracterizava-se por superfície lisa, consistente a manipulação, não pediculada e de coloração rosa-pálido. O tratamento consistiu de orientação da higienização bucal ao paciente, e caso a lesão afetasse a fonação e/ou a mastigação, poderia se considerar a possibilidade de correção cirúrgica.

Idiopathic gingival hyperplasia and orthodontic treatment: a case report.

There are many reasons for gingival hyperplasia. Mostly, proper oral hygiene is sufficient to achieve normal healthy gingiva. In some situations, however, gingival hyperplasia is drug-induced or can be a manifestation of a genetic disorder. In the latter, it may exist as an isolated abnormality or as part of a syndrome. If orthodontic treatment is needed in patients with gingival hyperplasia, both orthodontic and periodontal aspects need to be considered. Extreme hereditary gingival fibromatosis was periodontally treated, by removal of all gingival excess using flaps and gingivectomies. After a follow-up period, the orthodontic treatment started with fixed appliances. Monthly periodontal check-ups (scaling and polishing) were scheduled to control the gingival inflammation. After the orthodontic treatment, permanent retention was applied, once more followed by a complete gingivectomy in both maxilla and mandible. One of the most important keys to successful treatment of hyperplasia patients is the cooperation between the periodontist and the orthodontist.

Long-term management of an idiopathic gingival fibromatosis patient with the primary dentition.

Gingival fibromatosis is usually seen as an isolated finding or occasionally in association with other features as part of a syndrome. The combination of gingival enlargement, hypertrichosis, epilepsy and mental retardation is also a commonly reported syndrome that features gingival fibromatosis. The following report is about a mentally retarded patient who has shown no sign of hypertrichosis, but has been taking phenobarbital as a long-term therapy drug for anti-convulsion. Long-term management of this patient has been carried out from the age of one-and-a-half years to 14 years old. The patient's clinical features, treatment received, histopathologic presentation of gingival fibromatosis and proper management of the condition are discussed.

Periodontal and soft-tissue abnormalities.

Young children come to our offices with a variety of periodontal and soft-tissue abnormalities, which require accurate diagnosis, treatment or reassurance, and possible referral to our medical colleagues. Periodic review of soft-tissue lesions can help the dental team to recognize both common and rare abnormalities affecting young children. Recent years have brought new insights into the etiology and treatment of periodontal diseases of children, making it possible to prevent or treat many formerly untreatable conditions.

Fibromatosis gingival: presentación de un caso / Gingival fibromatosis: a case presentation

Se presenta el caso de un paciente infantil con fibromatosis gingival y su seguimiento por 7 años, describiéndose sus manifestaciones bucales, clínicas, radiográficas e histológicas, así como sus características generales. Se analiza la evolución del caso y el efecto de los factores que estimulan la hiperplasia gingival, señalándose los procedimientos empleados para evitar las retenciones dentarias y favorecer el periodonto y la oclusión