Intubação de via aérea difícil em paciente com síndrome de Treacher Collins / Difficult airway intubation in a patient with Treacher Collins syndrome - case report

A síndrome de Treacher Collins é uma patologia rara, com gene causador mapeado no braço longo do cromossomo cinco (5q31. 3-q33.3). Conhecida como disostose craniofacial, apresenta-se com hipoplasia malar, hipoplasia mandibular e malformações do pavilhão auricular. Tal condição representa previsão de dificuldade para o ato anestésico de intubação, necessitando de avaliação pré-operatória minuciosa e cuidado intensivo no perioperatório. A anestesia geral costuma ser realizada por indução de anestésicos inalatórios, uma vez que crianças submetidas a procedimentos cirúrgicos são não cooperativas, além de haver dificuldade de se obter acesso venoso. Assim, objetiva-se relatar caso de via aérea de intubação difícil em paciente com síndrome de Treacher Collins, correlacionando às manifestações clínicas, ao diagnóstico e ao tratamento cirúrgico, e revisando a literatura sobre o tema. Relatamos um caso cuja singularidade reside no manejo anestésico diferente dos executados em outros centros médicos, ao abordar pacientes com previsão de via aérea difícil. Ao invés de se utilizar máscara laríngea ou intubação com laringoscópio óptico, procedeu-se a: indução inalatória, sedação sem abolir respiração espontânea, visualização das estruturas para introdução do tubo endotraqueal (Cormack 3), acesso venoso, intubação orotraqueal e, posteriormente, indução anestésica e bloqueio neuromuscular. Julgamos importante divulgar tal relato para expor alternativas na indisponibilidade de certos dispositivos, como o fibroscópio. A técnica de intubação sem máscara laríngea ou fibroscópio em pacientes com síndrome craniofacial pode ocorrer sem intercorrências com a estratégia de não abolir a respiração do paciente, porém com leve sedação, devido à não cooperação e à dificuldade de se obter acesso venoso em crianças. (AU)

Treacher Collins syndrome is a rare disease with the culprit gene mapped on the distal long arm of chromosome five (5q31. 3-q33.3). It is known as craniofacial dysostosis, and presents with malar hypoplasia, mandibular hypoplasia, and pinnae malformations. Such condition represents expected difficult airway intubation during anesthesia, requiring detailed preoperative evaluation, and intensive perioperative care. General anesthesia is usually performed through inhaling anesthetics because children undergoing surgical procedures are not cooperative, and their venous access is difficult. Thus, the aim of the study is to report a case of difficult airway intubation in a patient diagnosed with Treacher Collins syndrome, correlating clinical manifestations, diagnosis e surgical treatment, and reviewing the literature on the subject. We report a case that is unique because the anesthetic management is different from what has been done in other medical centers, since it manages patients with expected difficult airway. Instead of using a laryngeal mask airway (LMA) device or a flexible optical intubation (FOI), an inhaling induction was performed, with preserved spontaneous breathing sedation, and visualization of the structures to receive the endotracheal tube (Cormarck 3), venous access, orotracheal intubation and then, anesthetic induction and neuromuscular block. We consider it important to share this report to give alternatives when some devices, such as the fiberscope, are not available. The intubation technique without laryngeal mask airway device or fiberscope in patients with craniofacial syndrome may take place with no complications, when the patient's spontaneous breathing is not aborted, but with light sedation, because of children's noncooperation, and difficulty venous access. (AU)

Modified bisecting angle technique in eliminating zygomatic superimposition over apices of maxillary molars.

Background: Image superimposition is a part and parcel of two‑dimensional radiography. However, some overlappings are a result of radiographic technique itself. The technique induced superimposition of zygomatic buttress on apices of maxillary molars is common in bisecting angle technique (BAT), which affects the endodontic performance. Certain alterations were carried out in BAT to remove these undiagnostic shadows. The aim of this study was to compare two techniques of periapical radiography namely, BAT and modified BAT (MBAT) in preventing zygomatic superimposition over apices of maxillary molars. Materials and Methods: A total of 62 patients requiring endodontic treatment for at least one maxillary molar were recruited in this cross‑sectional study. One tooth from every patient was subjected for two periapical radiographs, one with each BAT and MBAT respectively giving a total of 124 radiographs. Each radiograph was recorded as acceptable or unacceptable, based on zygomatic superimposition and different technical aspects. Chi‑square test was used for data analysis. The kappa statistic was used to test intra‑observer reliability. Results: With MBAT, the acceptability was 82.3% (n = 51) compared with 43.5% (n = 27) when BAT was employed. The statistically significant difference was found between these two techniques (P = 0.000). The level of agreement between two oral radiologists in their interpretation was high (kappa index = 0.897). Conclusion: Modified BAT was more accurate for periapical radiography of maxillary molars in preventing zygomatic superimposition and related technical errors than BAT.

[Comparative study of zygomaticmaxillary buttresses osteotomies with or without midpalatal osteotomy in surgically assisted rapid maxillary expansion by using palatal distractor]

Assessment the efficiency of separating zygomaticmaxillary buttresses, with or without midpalatal suture osteotomy, on shape and size expansion of the maxilla using palatal distractor. Fourteen adult patients [ages between 18-25 years and no inborn deformities or congenital syndromes] with skeletal maxillary narrowness being clinically and radially assessed. The studied sample is separated to two groups; Group I: seven patients with bilateral zygomaticmaxillary buttress osteotomy, Group II: the previous procedure was repeated on another 7 patients but with midpalatal osteotomy. The expansion in group II has V shape vertically and horizontally, but that was not clear in group I [p=0.03]. Also the maxillary width immediately and 6 months after the expansion in group II increased statistically when compared with group I [p=0.00], and the percentage of skeletal maxillary expansion after 6 months in group I was 26.17%, but it was 61.88% in group II. We conclude that midpalatal osteotomy affects the shape of expansion and increases the percentage of skeletal maxillary expansion in adults

Morphology of autogenous bone graft and castor oil polyurethane in the infraorbital rim of rabbits: a comparative study / Estudo morfológico comparativo do enxerto ósseo autógeno e polímero de mamona em rebordo infra-orbitário de coelhos

PURPOSE: Morphological study comparing castor oil polyurethane and autogenous bone graft to repair bone defect in zygomatic bone of rabbits. METHODS: Twenty-four adult, male New Zealand rabbits were randomly distributed between two groups of twelve. Bone defects of 5mm in diameter were cut through the zygomatic bone and filled with polyurethane discs in the experimental group or autogenous bone harvested from the tibia in the control group. Animals were sacrificed after 30, 60 or 90 days, and the zygomatic bones were macro- and microscopically analyzed. Student's, Fisher's, chi-squared and McNemar's tests were used for statistical analysis. RESULTS: Both the castor oil polyurethane and the autograft adapted well to the defect, with no need for fixation. Fibrous connective tissue encapsulated the polyurethane, but no inflammation or giant cell reaction was observed. Acidophilic and basophilic areas were observed inside the micropores of the polyurethane, suggesting cell nuclei. After 90 days, bone repair with a lamellar pattern of organization was observed in the control group. CONCLUSION: The castor oil polyurethane was biocompatible and did not cause inflammation. It may be considered an alternative to fill bone defects.

OBJETIVO: Estudo morfológico comparativo do implante de poliuretana de mamona e enxerto ósseo autógeno em defeito ósseo padrão em osso zigomático de coelhos. MÉTODOS: Vinte e quatro coelhos Nova Zelândia, machos, adultos, foram distribuídos aleatoriamente em dois grupos de 12. Defeitos de 5mm de diâmetro, perenes, foram confeccionados em osso zigomático e preenchidos com discos pré-fabricados de poliuretana no grupo experimento ou osso autógeno extraído da tíbia no grupo controle. Os animais foram sacrificados após 30, 60 e 90 dias e as peças anatômicas foram avaliadas macro e microscopicamente. Foram utilizados os testes de Student, Fisher,qui-quadrado e McNemar para a análise estatística dos resultados. RESULTADOS: A poliuretana e o osso autógeno se adaptaram ao defeito sem necessidade de fixação. Houve formação de tecido conjuntivo fibroso envolvendo a poliuretana, sem reação inflamatória ou presença de células gigantes. Verificaram-se áreas acidófilas e basófilas nos poros do material implantado, sugestivas de núcleos celulares. No grupo controle, observou-se aos 90 dias o reparo ósseo de padrão lamelar clássico. CONCLUSÃO: A poliuretana de mamona foi biocompatível e não causou reação inflamatória deletéria. Pode ser uma alternativa para o preenchimento de defeitos ósseos.

Nager's acrofacial dysostosis with hypertrophic cardiomyopathy

Nager syndrome is a rare condition associated with craniofacial malformations such as, micrognathia, zygomatic hypoplasia, external ear malformations, and preaxial limb deformities. This report features a case of Nager syndrome occurring in a one-year-old boy showing microretrognathia, thumb hypoplasia, brachydactyly, hexadactyly, and hypertrophic cardiomyopathy, characteristics not usually encountered in published cases

Síndrome de Treacher Collins: características clínicas e relato de caso / Treacher Collins syndrome: clinical aspects and a case report

A síndrome de Treacher Collins (STC) é também conhecida como Disostose Mandibulofacial. É uma síndrome crânio-facial que apresenta alterações bilaterais e simétricas de estruturas originadas do primeiro e segundo arcos branquiais e placódios nasais. Sua prevalência ao nascimento é de 1 para 25.000 a 1 para 50.000. A origem é genética e a transmissão é autossômica dominante com expressividade variável. No presente trabalho, os autores apresentam o relato de um paciente portador da síndrome de Treacher Collins que compareceu ao Grupo de Estudo e Atendimento a Pacientes Especiais - GEAPE, do Departamento de Ortodontia e Odontopediatria da Faculdade de Odontologia da Universidade de São Paulo para tratamento odontológico.

Hemifacial microsomia. A case report and review.

Hemifacial microsomia (HFM) is a variable asymmetric craniofacial malformation resulting in hypoplasia of the components of the first and second branchial arches. Because of the extremely variable expressively of the HFM, treatment measures also vary considerably from the use of activator to total reconstruction of TM joint and management of secondary deformities of maxilla, nose, orbit and zygomatic bone. We present a case of HFM type I of mild deformity treated with onlay bone grafting.

Multiple intracranial aneurysms associated with branchio-oto-dysplasia

Branchio-oto-dysplasia is characterized by abnormalities of embryonic branchial arch system and deafness inherited as autosomal dominant with variable gene expression. We present a rare case of multiple intracranial aneurysms associated with branchio-oto-dysplasia. A 40-yr-old man with severe headache presented as spontaneous subarachnoid hemorrhage on brain computed tomographic scan. The patient also manifested clinical features of branchio-oto-dysplasia and right hemifacial hypoplasia. Carotid angiogram confirmed an aneurysm in the anterior communicating artery. Intraoperative findings demonstrated multiple aneurysms in the anterior communicating artery and in the left posterior communicating artery, which were clipped successfully. Postoperative course was uneventful. This condition has not been reported previously. We also reviewed literatures to discuss whether the intracranial aneurysm was as a coincidental finding or a part of this malformation.