Oral manifestations of Chediak-Higashi syndrome: A systematic review.

Chediak-Higashi syndrome (CHS) is an autosomal recessive disorder characterized by leukocytes with giant secretory granules and a myriad of clinical features. However, it is unknown whether oral lesions are part of the syndrome or are refractory to systemic treatment. Herein, we integrated the available data published in the literature on the oral manifestations of individuals with CHS. Searches on PubMed, Web of Science, Embase, Scopus, and LILACS were conducted to identify studies published up to March/2022. The Joanna Briggs Institute tool was used for the critical appraisal of studies. Fourteen articles (21 cases) were detected. The mean age of individuals was 15.9±8.8 years. There was a slight predominance of males (52.4%). The major manifestation was periodontal disease (81%), although ulceration of the oral mucosa (14.3%), gingival/labial abscess (4.8%), and periodontal abscess (4.8%) were also reported. Oral rehabilitation including dental implants (9.5%) was performed after tooth losses due to the poor prognosis of periodontal therapy. CHS is usually diagnosed in an early stage due to its systemic manifestations such as classic oculocutaneous albinism, recurrent infections, and a propensity for bleeding. Oral health providers should be aware of the manifestations of individuals with CHS. Special care, including oral prophylaxis, is indispensable.

Solução de Carnoy no tratamento de ceratocisto / Carnoy's solution in treatment of keratocyst

Introdução: O objetivo do trabalho é relatar um caso de ceratocisto associado à impactação dentária, o qual foi tratado com descompressão, seguido de enucleação da lesão e utilização de solução de Carnoy. Relato de caso: Paciente do sexo masculino, 14 anos, encaminhado para avaliação de lesão encontrada após exame imaginológico de rotina. O mesmo demonstrou extensa lesão radiolúcida localizada na região de ângulo e ramo da mandíbula, com presença do elemento 48 intralesional próximo a basilar. Foi realizado biópsia incisional e instalação de dispositivo de descompressão no mesmo tempo cirúrgico, o qual o resultado histopatológico foi de ceratocisto. Após 6 meses com o dispositivo, observou-se diminuição da lesão e melhora no posicionamento do dente incluso. Frente a boa resposta à descompressão, decidiu-se pela enucleação total da lesão, exodontia dos dentes 47 e 48, curetagem rigorosa e tereapia adjuvante com aplicação da solução de Carnoy. O paciente evoluiu bem, neoformação óssea na área operada e encontra-se em acompanhamento há 6 meses, sem sinais de recidiva. Considerações finais: O uso da descompressão cirúrgica em lesões císticas mandibulares minimiza os danos as estruturas circunvizinhas, riscos de fratura patológica e lesão nervosa. Em função das altas taxas de recidiva, a terapia adjuvante após a enucleção é imprescindível para essa lesão, sendo a aplicação da solução de Carnoy uma das técnicas com melhores resultados. Dessa forma, para aumentar a taxa de sucesso e minimizar as sequelas, o planejamento cirúrgico dos ceratocistos mandibulares extensos deve ser feito de forma criteriosa e cuidadosa... (AU)

Introduction: The objective of this study is to report a case of keratocyst associated with dental impaction, which was treated with decompression, followed by enucleation lesion and Carnoy solution. Case report: Male patient, 14 years old, referred for evaluation of lesion found after routine imaging. He showed extensive radiolucent lesion located in the region of the angle and branch of the mandible, with the presence of the intralesional element 48 near the basilar. An incisional biopsy was performed and a decompression device was installed during surgical time and the histopathological result was keratocyst. After 6 months of observation a reduction of the lesion and improvement in the positioning of the tooth even were noticed. Given the good response to decompression, it was decided to complete the enucleation of the lesion, extraction of teeth 47 and 48, rigorous curettage and adjuvant therapy with Carnoy's solution. Followed up for 6 months, patient evolved well creating a new bone formation in the operated area with no signs of relapse. Final considerations: The use of surgical decompression in cystic mandibular lesions minimizes damage to surrounding structures, pathological fracture risks, and nerve damage. Because of the high rates of recurrence, keratocysts require adjuvant therapy after enucleation and Carnoy's solution is one of the best performing techniques. Thus, to increase success rate and minimize sequelae, the surgical planning of extensive mandibular keratocysts should be done carefully and judiciously... (AU)

Calcifying odontogenic cyst, dentinogenic ghost cell tumor, and ghost cell odontogenic carcinoma: A systematic review.

BACKGROUND: The aim of this study was to integrate the available data published on calcifying odontogenic cyst (COC), dentinogenic ghost cell tumor (DGCT), and ghost cell odontogenic carcinomas (GCOCs) into a comprehensive analysis of their clinicoradiological features, treatment, and recurrence. MATERIALS AND METHODS: An electronic search with no publication date restriction was undertaken in October 2017 in the following databases: PubMed, Medline Ovid, Web of Science, and Scopus. Eligibility criteria included publications containing enough clinical, radiological, and histopathological information to confirm a definite diagnosis of these lesions. Data were evaluated descriptively. RESULTS: The literature review indicated a total of 234 publications reporting 367 COCs, 55 DGCTs and 44 GCOCs. These lesions have a predilection for Asian males. COCs mainly affect the mandible and patients in the second decade of life, DGCTs mostly affect the mandible and patients in the fourth decade of life, and GCOCs mostly affect the maxilla and patients in the fifth decade of life. CONCLUSION: Conservative surgery was the most common therapy for COCs and DGCTs, while radical surgery was most common for GCOCs. This study provides important and interesting data that could help clinicians and surgeons as well as oral and maxillofacial pathologists with the diagnosis and management of these lesions.

Head and neck myiasis: a case series and review of the literature.

OBJECTIVE: This study describes 9 cases of myiasis affecting the head and neck region and discusses the demographic distribution, treatment, clinical characteristics, and sequelae of the disease in light of the literature. STUDY DESIGN: The study was performed in 2 steps. In the first part, 9 cases seen over a period of 10 years at the Emergency Department of Hospital da Restauração in Brazil were studied. In the second part, a literature search was performed in PubMed for articles on head and neck myiasis published from 1975 to March 2017. RESULTS: The case series mainly consisted of male patients in their 30s. The palate was the most commonly affected site. Myiasis was caused by Cochliomyia hominivorax in all patients, who were treated by mechanical removal of the larvae and debridement of necrotic tissue, followed by oral ivermectin. All patients had sequelae resulting from bone destruction. CONCLUSIONS: Head and neck myiasis generally affects individuals with poor hygiene habits, drug users, and individuals with neurologic and psychosocial disorders. The treatment of choice is mechanical removal of larvae and surgical debridement combined with oral ivermectin. This study provides information that could help clinicians in the diagnosis and management of this condition.

Vascular Malformation of the Face: a Rare Case with Follow-up of 18 Years.

OBJECTIVE: Vascular malformations are structural anomalies in the blood vessels. They are present at birth and persist throughout life. These malformations can be classified according to the type of vessel involved. A port-wine stain is a vascular malformation characterized by an increased number of ectatic vessels in the dermal vascular plexus, which can be found in any part of the body, including the head and neck region. Over time, most of these stains result in soft tissue hypertrophy and this growth can cause severe facial deformity. MATERIALS AND METHODS: This study describes a rare case of a giant proliferative vascular anomaly arising from a port-wine stain on the face. RESULTS: The report highlights the continuous and proliferative growth of the malformation observed after follow-up of 18 years, as well as the difficulty in establishing the diagnosis due to the complexity and dimension of the lesion and the conditions related to the patient's low socioeconomic status. CONCLUSIONS: We have described a rare and unusual case report of a giant proliferative vascular malformation arising from a Port-wine stain on the face.