Mönckeberg's sclerosis – is the artery the only target of calcification?

Abstract Background Since its first description, Mönckeberg's sclerosis has only been related to arterial media calcification, being listed among the primary diseases of the vessels. Case presentation We report here a clinically and histologically confirmed case of Mönckeberg's sclerosis in which the patient presented with massive areas of soft tissue calcifications in the pharynx and larynx. Polysomnographic parameters showed severe obstructive apnea refractory to nasal continuous positive airway pressure. Clinical and laboratory findings excluded concomitant endocrine or rheumatological diseases. Conclusion Our data provide a new insight about Mönckeberg's sclerosis, i.e., the fact that the etiopathogenic process involved in the phenomenon of calcification may not be restricted only to the arteries, but may occur in the entire organism. Further studies of the etiopathogenesis of this disease are needed.

Intranasal tooth and associated rhinolith in a patient with cleft lip and palate

We report the case of a 9-year-old girl who presented with a complaint of a malodorous bloody discharge from the left naris. The patient had previously undergone a complete repair of left-sided cleft lip and palate. Clinical examination revealed hyperplasia of the nasal mucosa on the left side. X-ray examination of the nasal cavity demonstrated a radiopaque structure that resembled a tooth and a radiopaque mass similar to an odontoma that was adherent to the root of the suspected tooth. With the patient under general anesthesia, the structure was removed. On gross inspection, the structure was identified as a tooth with a rhinolith attached to the surface of its root. Microscopic examination revealed normal dentin and pulp tissue. A nonspecific inflammatory infiltrate was observed around the rhinolith, and areas of regular and irregular mineralization were seen. Some mineralized areas exhibited melanin-like brownish pigmentation. Areas of mucus with deposits of mineral salts were also observed. Rare cases of an intranasal tooth associated with a rhinolith have been described in the literature. We believe that this case represents only the second published report of an intranasal tooth associated with a rhinolith in a patient with cleft lip and palate

Morphological characterization of the nasopalatine region in human fetuses and its association to pathologies

The nasopalatine region is composed of structures such as the vomeronasal organ and nasopalatine duct. The nasopalatine duct may provide the communication of the mouth to the nasal cavity in human fetuses and can be obliterated in an adult human. Knowledge on the development of the nasopalatine region and nasopalatine duct in humans is necessary for understanding the morphology and etiopathogenesis of lesions that occur in this region. Objective: The aim of the present study was to describe the morphological aspects of the nasopalatine region in human fetuses and correlate these aspects with the development of pathologies in this region. Material and Methods: Five human fetuses with no facial or palatine abnormalities were used for the acquisition of specimens from the nasopalatine region. After demineralization, the specimens were histologically processed. Histological cuts were stained with methylene blue to orient the cutting plane and hematoxylin-eosin for the descriptive histological analysis. Results: The age of the fetuses was 8.00, 8.25, 9.00 and 9.25 weeks, and it was not possible to determine the age in the last one. The incisive canal was observed in all specimens as an opening delimited laterally by the periosteum and connecting oral and nasal cavity. The nasopalatine duct is an epithelial structure with the greatest morphological variation, with either unilateral or bilateral occurrence and total patent, partial patent and islet forms. The vomeronasal organ is a bilateral epithelized structure located alongside the nasal septum above the incisive canal in all the fetuses. Conclusions: The incisive canal, nasopalatine duct and vomeronasal organ are distinct anatomic structures. The development of nasopalatine duct cysts may occur in all forms of the nasopalatine duct.

Neurovascular anatomical variations in the anterior palate observed on CBCT images

Objective: This study aimed to assess the presence of additional foramina and canals in the anterior palate region, through cone beam computed tomography (CBCT) images, describing their location, direction, and diameter. Materials & Methods: CBCT exams of 178 subjects displaying the anterior maxilla were included and the following parameters were registered: gender; age group; presence of additional foramina in the anterior palate (AFP) with at least 1 mm in diameter; location and diameter of AFP; and direction of bony canals associated with AFP. Results: Twenty-eight patients (15.7%) presented AFP and in total 34 additional foramina were registered. No statistical differences between patients with or without AFP were found for gender or age. The average diameter of AFP was 1.4 mm (range from 1 to 1.9 mm). Their location was variable, with most of the cases occurring in the alveolar process near the incisors or canines (n = 27). In 18 cases, AFP was associated with bony canals with upward or oblique direction toward the anterior nasal cavity floor. In 14 cases, the canal presented as a direct extension of the canalis sinuosus, in an upward direction laterally to the nasal cavity aperture. In two cases, the canal was observed adjacent to the incisive and joined the nasopalatine canal superiorly. Discussion: CBCT images have a crucial role in the recognition of anatomical variations by allowing detailed tridimensional evaluations. Additional foramina and canals in the anterior region of the upper jaw are relatively frequent. Practitioners should be aware and trained to identify these variations. Conclusions: Over 15% of the population studied had additional foramina in the anterior palate, between 1 mm and 1.9 mm wide, with variable locations. In most cases the canals associated with these foramina either presented as a direct extension of the canalis sinuosus, or coursed towards the nasal cavity floor.

Nevo de células fusiformes de Reed na conjuntiva

Os autores relatam um caso de nevo de Reed, lesão que apresenta aspecto histológico de malignidade, mas tem evolução benigna. Paciente de 48 anos, masculino, cor parda, apresentava pterígio nasal no olho direito, associado a uma lesão pequena e pouco pigmentada localizada na cabeça do mesmo. Realizou-se exérese de ambos, sem intercorrências, sem sinais de recidiva. O exame histopatológico revelou lesão com bordas definidas, restrita ao epitélio, constituída por células fusiformes perpendiculares à superfície, com pigmentação melânica esparsa. O diagnóstico inicial foi nevo de Spitz, mas, posteriormente, chegou-se à conclusão que se tratava do nevo de células fusiformes de Reed. O presente relato é o segundo na literatura mundial e o primeiro no Brasil.

Efetividade da fonoterapia e proposta de intervenção breve em respiradores orais

OBJETIVOS: verificar o efeito da intervenção fonoaudiológica em um grupo de respiradores orais e propor terapia fonoaudiológica mínima no tratamento da respiração oral. MÉTODO: estudo prospectivo longitudinal, casuística de 40 sujeitos respiradores orais tratados no Hospital das Clínicas - setor de Reabilitação Orofacial do Ambulatório de Respirador Oral da Disciplina de Otorrinolaringologia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas - UNICAMP. Realizada documentação fotográfica, avaliação clínica, aplicação de protocolo para categorizar modo respiratório, postura (lábios e bochechas); força e praxias (lábios, bochechas e língua) nas semanas 0, 12 e 24. A proposta terapêutica constou de treino e conscientização da respiração nasal; manobras para aquecimento e vascularização da musculatura orofacial; aplicação de pontos e zonas motoras na face; manobras passivas; uso do impulso distal; exercícios miofuncionais e registro da percepção dos pacientes sobre suas condições olfativas e obstruções nasais.Teste estatístico: não paramétrico de Igualdade de Duas Proporções, p < 0,05. RESULTADOS: houve adequação da função respiração nasal. Aumento da força de lábios, língua e bochechas. Melhora nas praxias: bico direita, bico esquerda, estalo e vibração de lábios. Vibração e estalo de língua. Inflar simultaneamente as bochechas, inflar bochecha direita e esquerda. O tempo com maior ganho terapêutico foi de 12 semanas. A partir deste dado foi esquematizado protocolo com 12 sessões estruturadas abordando estratégias utilizadas nesta pesquisa. CONCLUSÃO: a pesquisa demonstrou que o uso da reabilitação miofuncional para pacientes respiradores orais foi eficiente com maior evolução terapêutica observada na semana 12.

Comparison of different protocols for the extraction of microbial DNA from reef corals

This study aimed to test different protocols for the extraction of microbial DNA from the coral Mussismilia harttii. Four different commercial kits were tested, three of them based on methods for DNA extraction from soil (FastDNA SPIN Kit for soil, MP Bio, PowerSoil DNA Isolation Kit, MoBio, and ZR Soil Microbe DNA Kit, Zymo Research) and one kit for DNA extraction from plants (UltraClean Plant DNA Isolation Kit, MoBio). Five polyps of the same colony of M. harttii were macerated and aliquots were submitted to DNA extraction by the different kits. After extraction, the DNA was quantified and PCR-DGGE was used to study the molecular fingerprint of Bacteria and Eukarya. Among the four kits tested, the ZR Soil Microbe DNA Kit was the most efficient with respect to the amount of DNA extracted, yielding about three times more DNA than the other kits. Also, we observed a higher number and intensities of DGGE bands for both Bacteria and Eukarya with the same kit. Considering these results, we suggested that the ZR Soil Microbe DNA Kit is the best adapted for the study of the microbial communities of corals.

Facial lesions caused by renal osteodystrophy in a patient with chronic renal insufficiency: a case report

PURPOSE: Chronic renal insufficiency (CRI) is the last stage of a chronic renal condition in which the kidney loses its filtration and endocrine functions. Chronic endocrine hypofunction causes generalized damage to the body known as Uremic Syndrome, which affects the central nervous system as well as the cardiovascular, hematologic, dermatologic, ophthalmic, endocrine, respiratory, gastrointestinal and skeletal systems. The present study reports the case of a female patient with CRI who presented facial osteodystrophy of the osteitis fibrosa type, and highlights the main features of this condition. CASE DESCRIPTION: A 24-year old, female, Caucasian patient presented chronic glomerulonephritis recurrence and lost the transplanted kidney five years before, undergoing arteriovenous fistula hemodialysis three times a week. She presented swelling of the left masseter area with a hard consistency on palpation, covered by intact skin, swelling at the bottom of the left atrium, with a hard consistency on palpation, a mucosa-like color and absence of inflammation signs, suggesting expansive bone lesions on the face. These features were compatible with hyperparathyroidism brown tumor and/or osteodystrophy. The CT scan showed expansive bone lesions of heterogeneous appearance on the left jaw, maxilla/nasal floor, and right frontotemporal suture areas. The clinical and histopathological characteristics of the lesion, in association with PHT hormone high serum levels led to renal osteodystrophy diagnosis. The patient was referred to the nephrology services. CONCLUSION: Osteodystrophic bone alterations have a high prevalence in renal disease patients, and the dentist must take these alterations into consideration in bone lesion diagnosis for this specific group of patients.

Resultados da ressecção de hemangiomas infantis nasais em fase proliferativa: abordagem segura para os tumores centrais da face

INTRODUÇÃO: O hemangioma infantil é o tumor benigno mais comum da infância, predominando na região cervicofacial. É caracterizado por apresentar 3 fases distintas, observando-se frequentemente regressão espontânea dessas lesões. No entanto, sequelas residuais ou deformidades das estruturas anatômicas em crescimento podem ocorrer. A abordagem cirúrgica precoce e definitiva é indicada, em decorrência da localização dos hemangiomas nasais e seu potencial desfigurante, visando à obtenção de bons resultados estéticos e preservação anatômica. O objetivo do presente estudo foi analisar os resultados da abordagem cirúrgica definitiva para hemangiomas proliferativos nasais, com base em uma avaliação objetiva. MÉTODO: No período de 1997 a 2009, 20 pacientes portadores de hemangiomas nasais em fase proliferativa foram submetidos a tratamento cirúrgico. As lesões foram avaliadas segundo local de acometimento e tratamento realizado. Foram analisados índices de complicações e necessidade de procedimentos adicionais. Os resultados estéticos foram avaliados por avaliadores independentes. RESULTADOS: As lesões estavam localizadas na ponta nasal em 50% dos pacientes; no dorso, em 20%; em todas as subunidades, em 15%; nas áreas paranasais, em 10%; e na unidade alar, em 5%. A ressecção foi total em 60% dos pacientes e subtotal em 40%. O período médio de acompanhamento foi de 42,6 meses. A média de procedimentos cirúrgicos por paciente foi de 1,3 + 0,7. Nenhuma complicação importante foi observada. Os resultados foram positivamente avaliados quanto a redução do volume da lesão e melhora do contorno facial, corroborando a conduta proposta. CONCLUSÕES: No manejo dos hemangiomas nasais, o tratamento cirúrgico definitivo pode ser considerado uma alternativa segura e eficaz, com baixas taxas de complicação.

Ablefaro macrostomia - síndrome e audição: relato de dois casos familiares

Introdução: Síndrome de Ablefaro MAcrostomia (AMS) é uma condição rara que compreende pálpebras ausentes ou curto, orelhas anormais, macrostomia, genitália anômalo, pele redundante e cabelos ausente. Brancati et al (2004) relataram uma ocorrência estimada de perda auditiva em 70% desta população. Estudos específicos sobre a audição em AMS não estavam presentes nos jornais que compilados. Relato dos casos: Paciente 1 é o primeiro filho de um ano de idade, a mãe 23 anos e pai de 25 anos de idade, não consangüíneos. Suas características clínicas são pouco cabelo no couro cabeludo, orelhas em forma de taça, raiz nasal larga, narinas antevertidas, macrostomia, os dedos com membranas, pele redundante e hipoplasia mamilos e lábios. Ela não tem atraso no desenvolvimento neuropsicomotor e a fala é normal. A audição foi avaliada aos 15 anos com uma perda auditiva em 6 kHz. Paciente 2 é o terceiro filho do mesmo casal. Ela tem falta grave das pálpebras, uma ponte nasal baixa com narinas hipoplásica e anteversão, macrostomia, orelhas anormalmente modelados, a ausência de mamilos, um de 6 cm onfalocele, ânus anteriormente localizado, hipoplasia dos grandes lábios, unhas hipoplasia, atenuação distal de falanges e pele redundante. Ela está se desenvolvendo com atraso no desenvolvimento neuropsicomotor, fala normal e perda auditiva condutiva leve bilateral. A avaliação audiológica incluiu quatro procedimentos: história clínica audiológica, inspeção otológica, imitanciometria, audiometria tonal e discurso. Conclusões: Os pacientes estudados com AMS apresentaram perda auditiva leve e esta perda de audição pode ser considerado como uma parte do fenótipo AMS sendo compatível os achados com a literatura.

Fisioterapia respiratória em pacientes com fissuras labiopalatinas : rotinas do Hospital de Reabilitação de Anomalias Craniofaciais

As malformações craniofaciais apresentam-se como alterações frequentes sendo as fissuras labiopalatinas mais comumente encontradas. Essa condição leva à ocorrência de infecções de vias aéreas superiores e broncopneumonias pois a comunicação entre a cavidade oral e nasal modifica toda depuração de vias aéreas. O Setor de Fisioterapia do Hospital de Reabilitação de Anomalias Craniofaciais (HRAC/USP) atua em conjunto com a equipe multidisciplinar tanto no ambulatório quanto nas unidades de internação, onde realiza terapia respiratória para melhora do quadro clínico e condição cirúrgica desses pacientes. Diversas são as técnicas de fisioterapia respiratória utilizadas, sendo necessária uma modificação ou adaptação para melhor realização no paciente fissurado. Objetivo: Descrever e divulgar as rotinas de atendimento fisioterapêutico respiratório do Setor de Fisioterapia do HRAC/USP. Método: Baseado em um estudo descritivo da experiência clínica. Resultados: Realiza-se primeiramente uma anamnese, após verifica-se exames laboratoriais e de imagem seguindo para uma avaliação fisioterapêutica respiratória e assim são traçadas as condutas necessárias com o paciente. Na Unidade Ambulatorial foi observado que os pacientes ganham condição cirúrgica após realização de terapia respiratória pré operatória. Na Unidade de Internação a Fisioterapia auxilia na diminuição do tempo de hospitalização, orientando acompanhantes à continuidade do tratamento em casa. Conclusão: Acredita-se que o presente estudo possa contribuir para a divulgação da atuação do fisioterapeuta na assistência de pacientes com fissuras labiopalatinas.

Multiple trichoblastomas in a dog

Background – Hair follicle tumours generally present as benign, solitary masses and have a good prognosis following surgical resection. Hypothesis/Objectives – This report describes a case of multiple trichoblastomas in a dog. Animal – A 2-year-old crossbred dog presented with multiple soft cutaneous periocular, perilabial, submandibular and nasal nodules, between 2 and 9 cm in diameter, located on the right side of the face. New nodules were observed on the same side of the face at a second consultation 3 weeks later. Methods – Surgical resection of all nodules was performed in two procedures. Three nodules were initially resected and submitted for histolopathology and immunohistochemistry. The diagnosis was trichoblastoma for all three. At the time of the second consultation, new and remaining nodules were biopsied and the diagnosis of trichoblastoma confirmed. The dog was treated with doxorubicin and piroxicam for 30 days prior to the second surgical procedure in an attempt to reduce new tumour growth and the size of present tumours. All nodules were resected and the defects closed using rotation flaps. Results – No recurrence of the neoplasm was noted within 10 months after surgery. Conclusions and clinical importance – Trichoblastomas are generally benign but can present as multiple neoplasms that may require surgical resection and may respond to chemotherapy. To the authors’ knowledge, this is the first report of multiple trichoblastomas in a dog.

Revisione critica dei risultati e nuovi algoritmi decisionali sulla chirurgia dell'OSAS

Obstructive sleep apnoea/hypopnoea syndrome (OSAHS) is the periodic reduction or cessation of airflow during sleep. The syndrome is associated whit loud snoring, disrupted sleep and observed apnoeas. Surgery aims to alleviate symptoms of daytime sleepiness, improve quality of life and reduce the signs of sleep apnoea recordered by polysomnography. Surgical intervention for snoring and OSAHS includes several procedures, each designed to increase the patency of the upper airway. Procedures addressing nasal obstruction include septoplasty, turbinectomy, and radiofrequency ablation (RF) of the turbinates. Surgical procedures to reduce soft palate redundancy include uvulopalatopharyngoplasty with or without tonsillectomy, uvulopalatal flap, laser-assisted uvulopalatoplasty, and RF of the soft palate. More significant, however, particularly in cases of severe OSA, is hypopharyngeal or retrolingual obstruction related to an enlarged tongue, or more commonly due to maxillomandibular deficiency. Surgeries in these cases are aimed at reducing the bulk of the tongue base or providing more space for the tongue in the oropharynx so as to limit posterior collapse during sleep. These procedures include tongue-base suspension, genioglossal advancement, hyoid suspension, lingualplasty, and maxillomandibular advancement. We reviewed 269 patients undergoing to osas surgery at the ENT Department of Forlì Hospital in the last decade. Surgery was considered a success if the postoperative apnea/hypopnea index (AHI) was less than 20/h. According to the results, we have developed surgical decisional algorithms with the aims to optimize the success of these procedures by identifying proper candidates for surgery and the most appropriate surgical techniques. Although not without risks and not as predictable as positive airway pressure therapy, surgery remains an important treatment option for patients with obstructive sleep apnea (OSA), particularly for those who have failed or cannot tolerate positive airway pressure therapy. Successful surgery depends on proper patient selection, proper procedure selection, and experience of the surgeon. The intended purpose of medical algorithms is to improve and standardize decisions made in the delivery of medical care, assist in standardizing selection and application of treatment regimens, to reduce potential introduction of errors. Nasal Continuous Positive Airway Pressure (nCPAP) is the recommended therapy for patients with moderate to severe OSAS. Unfortunately this treatment is not accepted by some patient, appears to be poorly tolerated in a not neglible number of subjects, and the compliance may be critical, especially in the long term if correctly evaluated with interview as well with CPAP smart cards analysis. Among the alternative options in Literature, surgery is a long time honoured solution. However until now no clear scientific evidence exists that surgery can be considered a really effective option in OSAHS management. We have design a randomized prospective study comparing MMA and a ventilatory device (Autotitrating Positive Airways Pressure – APAP) in order to understand the real effectiveness of surgery in the management of moderate to severe OSAS. Fifty consecutive previously full informed patients suffering from severe OSAHS were enrolled and randomised into a conservative (APAP) or surgical (MMA) arm. Demographic, biometric, PSG and ESS profiles of the two group were statistically not significantly different. One year after surgery or continuous APAP treatment both groups showed a remarkable improvement of mean AHI and ESS; the degree of improvement was not statistically different. Provided the relatively small sample of studied subjects and the relatively short time of follow up, MMA proved to be in our adult and severe OSAHS patients group a valuable alternative therapeutical tool with a success rate not inferior to APAP.

Il ruolo del collicolo superiore nell'orientamento spaziale

This thesis was aimed at verifying the role of the superior colliculus (SC) in human spatial orienting. To do so, subjects performed two experimental tasks that have been shown to involve SC’s activation in animals, that is a multisensory integration task (Experiment 1 and 2) and a visual target selection task (Experiment 3). To investigate this topic in humans, we took advantage of neurophysiological finding revealing that retinal S-cones do not send projections to the collicular and magnocellular pathway. In the Experiment 1, subjects performed a simple reaction-time task in which they were required to respond as quickly as possible to any sensory stimulus (visual, auditory or bimodal audio-visual). The visual stimulus could be an S-cone stimulus (invisible to the collicular and magnocellular pathway) or a long wavelength stimulus (visible to the SC). Results showed that when using S-cone stimuli, RTs distribution was simply explained by probability summation, indicating that the redundant auditory and visual channels are independent. Conversely, with red long-wavelength stimuli, visible to the SC, the RTs distribution was related to nonlinear neural summation, which constitutes evidence of integration of different sensory information. We also demonstrate that when AV stimuli were presented at fixation, so that the spatial orienting component of the task was reduced, neural summation was possible regardless of stimulus color. Together, these findings provide support for a pivotal role of the SC in mediating multisensory spatial integration in humans, when behavior involves spatial orienting responses. Since previous studies have shown an anatomical asymmetry of fibres projecting to the SC from the hemiretinas, the Experiment 2 was aimed at investigating temporo-nasal asymmetry in multisensory integration. To do so, subjects performed monocularly the same task shown in the Experiment 1. When spatially coincident audio-visual stimuli were visible to the SC (i.e. red stimuli), the RTE depended on a neural coactivation mechanism, suggesting an integration of multisensory information. When using stimuli invisible to the SC (i.e. purple stimuli), the RTE depended only on a simple statistical facilitation effect, in which the two sensory stimuli were processed by independent channels. Finally, we demonstrate that the multisensory integration effect was stronger for stimuli presented to the temporal hemifield than to the nasal hemifield. Taken together, these findings suggested that multisensory stimulation can be differentially effective depending on specific stimulus parameters. The Experiment 3 was aimed at verifying the role of the SC in target selection by using a color-oddity search task, comprising stimuli either visible or invisible to the collicular and magnocellular pathways. Subjects were required to make a saccade toward a target that could be presented alone or with three distractors of another color (either S-cone or long-wavelength). When using S-cone distractors, invisible to the SC, localization errors were similar to those observed in the distractor-free condition. Conversely, with long-wavelength distractors, visible to the SC, saccadic localization error and variability were significantly greater than in either the distractor-free condition or the S-cone distractors condition. Our results clearly indicate that the SC plays a direct role in visual target selection in humans. Overall, our results indicate that the SC plays an important role in mediating spatial orienting responses both when required covert (Experiments 1 and 2) and overt orienting (Experiment 3).

Apnea e reflusso gastroesofageo nel neonato pretermine

Objective: To document the existence of a relationship between apnea of prematurity (AOP) and gastroesophageal reflux (GER) in preterm infants. Setting: One Neonatal Intensive Care Unit Patients: Twenty-six preterm infants (gestational age<32 weeks) with recurrent apneas. Intervention: Simultaneous and synchronized recording of polysomnography and pH-impedance monitoring (pH-MII). Polysomnography detects and characterizes apneas, by recording of breathing movement, nasal airflow, electrocardiogram, pulse oximeter saturation. pH-MII is the state-of-theart methodology for GER detection in preterm newborns. Main outcome measures: Relationship between AOP and GER, which were considered temporally related if both started within 30 seconds of each other. Results: One-hundred-fifty-four apneas out of 1136 were temporally related to GER. The frequency of apnea during the one-minute time around the onset of GER was significantly higher than the one detected in the GER-free period (p=0.03). Furthermore, the frequency of apnea in the 30 seconds after GER (GER-triggered apneas) was greater than that detected in the 30 seconds before (p=0.01). A great inter-individual variability was documented in the proportion of GERtriggered apneas. A strong correlation between total number of apneas and the difference between apneas detected 30 seconds after and before GER was found (p=0.034). Conclusions: Our data show that a variable rate of apneas can be triggered by GER in very preterm infant. Further studies are needed to recognise clinical features which identify those patients who are more susceptible to GER-triggered apneas.