Temporomandibular Joint Ankylosis in an Infant: A Rare Cause of Difficult Airway

1.Pre-assessment data of the patient A 2-year-old boy, weighing 15 kg was admitted with a history of limited mouth opening(inter-incisor distance of 6 mm), hypoplastic and retrognathic mandible (bird face deformity) and facial asymmetry from left temporomandibular joint ankylosis (TMJA). He was born at term, after an uneventful pregnancy, and there was no report of trauma during caesarean section. No other possible aetiologies were identified. He was scheduled for mandibular osteotomy. Preoperative ENT examination revealed adenotonsillar hypertrophy. 2. Anaesthetic Plan A fiberoptic nasal intubation was performed under deep inhalation anaesthesia with sevoflurane, with the patient breathing spontaneously. Midazolam (0.05 mg.kg-1) and alfentanil (0.03 mg.kg-1) were given and anaesthesia was maintained with O2/air and sevoflurane. No neuromuscular blocking agent was administered since the surgical team needed facial nerve monitoring. 3. Description of incident During surgery an accidental extubation occurred and an attempt was made to reintubate the trachea by direct laryngoscopy. Although the osteotomy was nearly completed, the vocal cords could not be visualized (Cormack-Lehane grade IV laryngoscopic view). 4. Solving the problem Re-intubation was finally accomplished with the flexible fiberscope and the procedure was concluded without any more incidents. Extubation was performed 24 hours postoperatively with the patient fully awake. After surgery mouth opening improved to inter-incisor gap of 15 mm. 5. Lessons learned and take home message Two airways issues present in this case can lead to difficultventilation and intubation: TMJA and adenotonsillar hypertrophy. These difficulties were anticipated and managed accordingly. The accidental extubation brought to our attention the fact that, even after surgical correction, this airway remains challenging. Even with intensive jaw stretchingexercises there is a high incidence of re-ankylosis, especially in younger patients. One should bear that in mind when anaesthetizing patients with TMJA.

Tranexamic Acid for Major Spinal Surgery in Children. A Retrospective Study

Introduction: Paediatric patients who undergo posterior spinal fusion surgery to correct scoliosis often require multiple blood transfusions. Tranexamic acid is a synthetic antifibrinolytic drug that reduces transfusion requirements in scoliosis surgery (1),(2),(3). Methods: To evaluate the efficacy of prophylactic tranexamic acid (TA) (initial dose of 10mg/kg and infusion of 1mg.kg(-1).h(-1)) in reducing perioperative blood transfusion requirements, we reviewed patients files and compared the amount of blood lost and blood transfused in the perioperative period of 12 patients (54.5%) that received TA and 10 patients (45.5%) who did not received TA. T-Student test was applied. Results: The average difference of blood losses (2,67 +/- 6,06ml) and blood transfused (212,9 +/- 101,1ml) between the two groups was not statistically significant (p>0.05). No thrombotic complications were detected in either group. Discussion: Results of the current study showed that prophylactic low dose of TA did not have a significant effect in the management of intraoperative blood loss and transfusion requirements in children undergoing scoliosis surgery. It is important to emphasize that our study is retrospective and that the size of the sample is small. Further studies are needed to evaluate the efficacy and safety of TA on paediatric scoliosis surgery.

Lupus Myelopathy in a Child

A 5-year-old female developed, after a 7-month period of fever, anorexia, weight loss, and a transitory cutaneous erythematous eruption, a severe acute transverse myelopathy, with a partial recovery of motor and sensory function. She had positive antinuclear and antidouble-stranded DNA antibodies but no antiphospholipid antibodies. Six months later she had massive proteinuria and restarted treatment with steroids and cyclophosphamide. Our patient is one of the youngest reported with lupus myelopathy. We discuss the clinical presentation, the magnetic resonance imaging findings, and other relevant laboratory studies of this rare but serious complication of systemic lupus erythematosus.

Cerebral Sinovenous Thrombosis in Children: Clinical Presentation and Extension, Localization and Recanalization of Thrombosis

Many important questions regarding pathophysiology and treatment of cerebral sinovenous thrombosis need clarification and may depend on further knowledge on the etiology, site, extension and recanalization of the thrombosis. We studied these variables in a cohort of children and adolescents from seven Portuguese Centers. We conclude from our results that the deep venous system and the superior longitudinal sinus are less frequently affected with thrombosis but have a greater potential for serious neurologic disease and for major sequelae. Non-recanalization, at least in the long term, is not an adverse prognostic factor. Extensive propagation of the thrombus from the initial site of origin seems to be common. The early identification of risk factors and their treatment coupled with an aggressive attitude towards diagnosis and treatment for thrombosis involving the deep venous system would be warranted.

Análise Descritiva de Parturientes e Recém-Nascidos na Maternidade: Hospital Dona Estefânia entre 2005 e 2008

Entre 2005 e 2008, registaram-se 8413 nascimentos na maternidade do Hospital Dona Estefânia (HDE), aproximadamente 8% do total nacional no mesmo período. A mortalidade fetal (0,20%) atingiu os objectivos do Plano Nacional de Saúde (PNS) para 2010 e está aos melhores niveis da União Europeia. Contudo, a percentagem média de nascimentos prétermo (8,1%) e de cesarianas (31,9%) situam-se ainda acima das metas estabelecidas pelo PNS de, respectivamente, 4,9% e 24,8%. O odds ratio de ocorrência de um índice Apgar baixo aos 5 minutos por cada 100 g de peso a menos à nascença foi de 1,35 e por cada semana a menos de gestação foi de 1,33. As parturientes tiveram em média 30,4 anos de idade, sendo 3,8% adolescentes. Cerca de 22% eram estrangeiras, valor superior à média nacional de 9%. Registou-se variabilidade entre as principais nacionalidades quanto a percentagem de partos pré-termo e de cesarianas, sendo menor nas mães chinesas. Encontrou-se significado estatístico na relação do peso à nascença com a idade de gestação,tipo de gravidez (simples/gemelar), sexo, paridade e idade da mãe. Fixando as covariáveis,uma semana de gestação a mais correspondeu, em média, a mais 176 g; um recém-nascido(RN) gémeo teve, em média, menos 381 g que um não-gémeo e um RN do sexo feminino pesou, em média, menos 48 g que um masculino. Apresentam-se tabelas de percentis de peso por sexo e idade gestacional (36-41 semanas) para os RN do HDE.

A Memória e o Impacto do Trauma: Numa Perspectiva Desenvolvimental

Pretende-se realizar uma revisão sobre as estruturas neuroanatómicas e os processos de neurotransmissão e de modulação neuronal envolvidos na memória. A memória é vista como um processo activo, baseado na reunião de variados aspectos dos padrões de activação neuronal, num processo dependente da experiência. Descreve-se a importância das regiões límbicas e sua inter-relação no processamento da memória, desde a codificação até à consolidação que implica uma reestruturação neuronal. Procede-se à caracterização dos tipos de memória (implícita e explícita) e suas características,a relação com as diferentes fases do desenvolvimento, tocando alguns aspectos mais descritivos de diferentes componentes da memória. Numa perspectiva contextual são também focadas as áreas da amnésia infantil, a lembrança, o esquecimento e o trauma. O esquecimento é parte essencial da memória explícita. Vários estudos apontam para um efeito de curva em U invertida, em relação com o impacto emocional. Eventos com intensidade moderada a elevada parecem ser etiquetados como importantes (envolvimento de estruturas do sistema límbico como a amígdala e o córtex orbito-frontal) e são mais facilmente recordadas no futuro. Se os eventos são muitos intensos o processamento no hipocampo da codificação memoria explícita e subsequentemente a recordação são inibidos,há também um bloqueio da consolidação cortical da experiência, porém mantém-se a memória implícita, que pode levar a vivência de imagens intrusivas não elaboradas. No que concerne ao trauma, levanta-se a questão da precisão da memória e do seu impacto, tanto ao nível das implicações neurofisiológicas como das consequências psicopatológicas. As alterações ao nível das neurotransmissões induzidas pelo stress vão condicionar alterações na mielinização, sinaptogénese e neurogénese, que podem levar a alterações a longo prazo em várias regiões do cérebro entre as quais a amígdala. Nestas condições, o processo explícito e a aprendizagem podem ser cronicamente impedidas, para além do risco importante para o desenvolvimento de psicopatologia, nomeadamente: Perturbação da Personalidade, Perturbações de Humor (depressão); Perturbações de Ansiedade; Perturbações Dissociativas e maior risco de Psicose.

Mutilations Due to Medical Disorders in Children

Soft-tissue and bone necrosis, although rare in childhood, occasionally occur in the course of infectious diseases, either viral or bacterial, and seem to be the result of hypoperfusion on a background of disseminated intravascular coagulation. Treatment consists in correction of septic shock and control of necrosis. Necrosis, once started, shows extraordinarily rapid evolution, leading to soft-tissue and bone destruction and resulting in anatomic, functional, psychological, and social handicaps. Ten mutilated children were treated from January 1986 to January 1999 in Hospital de Dona Estefaˆ nia, Lisbon, Portugal. One was recovering from hemolytic-uremic syndrome with a severe combined immunodeficiency, another malnourished, anemic child had malaria, and three had chicken pox (in one case complicated by meningococcal septicemia). There were three cases of meningococcal and two of pyocyanic septicemia (one in a burned child and one in a patient with infectious mononucleosis). The lower limbs (knee,leg, foot) were involved in five cases, the face (ear, nose, lip) in four, the perineum in three, the pelvis (inguinal region, iliac crest) in two, the axilla in one, and the upper limb (radius, hand) in two. Primary prevention is based on early recognition of risk factors and timely correction. Secondary prevention consists of immediate etiologic and thrombolytic treatment to restrict the area of necrosis. Tertiary prevention relies on adequate rehabilitation with physiotherapy and secondary operations to obtain the best possible functional and esthetic result.

Adolescents and School Asthma Knowledge and Attitudes

Introduction: bronchial asthma is a chronic disease that affects a high percentage of adolescents, with a significant restriction of daily activities, and is a cause of school absenteeism. The relationships between adolescents and asthma disease in school were assessed, with a view to improving knowledge about the asthmatic adolescent. Methods: a survey was conducted in the Lisbon metropolitan area, covering urban (Lisbon) and rural (Lourinh˜a ) zones and including 1879 students and 81 teachers from the 7th to 9th high school years. The study groups were asthmatic students, their peers, and teachers. A self-administered questionnaire was applied to collect information. The results were compared with a reference group of 91 asthmatic students attending our Department of Immunoallergy-Hospital Dona Estefânia. Cotinine urinary measurements were made in a sample of asthmatics and a control group. Results: the prevalence of current asthma among students was 10%. Estimates of asthma annual burden among 7th to 9th year students from Lisbon and Lourinh˜a high schools included 4,307 days missed from school, 4,148 medical consultations and a minimum of 351 hospital emergency care and 80 hospital admissions. Exposure to passive smoking was not significantly different between asthmatic students and theirs peers. Cotinine urinary measurements did not discriminate between exposed and non-exposed individuals. Cigarette smoking was almost as common among adolescent asthmatics (5.4%) as it was in non-asthmatic subjects (6.7%). However, 55% of asthmatics mentioned active and passive smoking as an asthma exacerbating factor. Asthmatic students, theirs peers and teachers showed a deficient knowledge about asthma (mean group scores: 17.6; 14.2 and 17.7 of a possible 30), particularly in the areas related to asthma recognition and its management. Asthmatics attending our Allergy Department had the highest scores. All groups showed tolerance in the sense of a positive and understanding attitude toward a person with asthma. However, traditional beliefs about asthma disease (dependence, inferiority...) were confirmed. A positive correlation between knowledge levels and tolerance attitudes was found. Conclusion: in view of the dimension of the asthma problem in adolescence and its social and economic impact, it is justifiable to assess the need for the implementation of asthma education programs in schools in order to improve asthma management by the adolescents and their schools.

Ciclosporina: Riscos em Idade Pediátrica

Introdução: A eficácia e segurança da ciclosporina têm sido demonstradas em patologias inflamatórias dermatológicas, nomeadamente psoríase e eczema, em adultos e crianças. Na idade pediátrica o seu uso é no entanto ainda limitado. Apresentamos três casos clínicos em que a ciclosporina, foi interrompida por aparecimento de complicações. Este trabalho visa alertar para potenciais efeitos secundários da ciclosporina, a fim de evitar utilizações abusivas. Casos clínicos: Foram submetidas a terapêutica com ciclosporina oral duas crianças de quatro e 13 anos de idade com eczema e uma criança de dois anos, com psoríase eritrodérmica. No primeiro caso interrompeu-se terapêutica pelo aparecimento de impétigo ao sexto dia de ciclosporina. Iniciou corticóides e inibidores tópicos da calcineurina com boa resposta. No segundo caso, a ciclosporina foi interrompida pelo aparecimento de herpes facial exuberante e toxicidade hepática e renal no quarto dia de tratamento. No último caso, de psoríase generalizada e impétigo, medicado com flucloxacilina e gentamicina,a terapêutica foi interrompida ao sexto dia por angioedema e urticária generalizados por quadro de angioedema e urticária generalizados, interpretado como reacção de hipersensibilidade a beta-lactâmicos, não sendo contudo possível excluir papel da ciclosporina. Discussão: Os dados sobre a utilização da ciclosporina em crianças são ainda escassos. A utilização deve ser limitada a casos com indicações precisas, após considerar riscos e benefícios.

Cold Urticaria and Infectious Mononucleosis in Children

Physical urticaria includes a heterogeneous group of disorders characterized by the development of urticarial lesions and/or angioedema after exposure to certain physical stimuli. The authors present the case of a child with severe acquired cold urticaria secondary to infectious mononucleosis. Avoidance of exposure to cold was recommended; prophylactic treatment with ketotifen and cetirizine was begun and a self-administered epinephrine kit was prescribed. The results of ice cube test and symptoms significantly improved. Physical urticaria, which involves complex pathogenesis, clinical course and therapy, may be potentially life threatening. Evaluation and diagnosis are especially important in children. To our knowledge this is the first description of persistent severe cold-induced urticaria associated with infectious mononucleosis in a child.

Apendicite Aguda - Clínica Versus Histologia

Introdução: A apendicite aguda continua a ser um dos principais diagnósticos nos Serviços de Cirurgia Pediátrica. A taxa de apendicectomia negativa (apendicectomia sem evidência histológica de patologia), frequentemente utilizada como índice de qualidade hospitalar, permanece alta, apesar dos esforços para a reduzir, especialmente em crianças com menos de 6 anos. Objectivos: os objectivos primários foram: o cálculo da taxa de apendicectomia negativa, da concordância entre diagnóstico clínico (pós-operatório) e diagnóstico histológico e a caracterização da discordância diagnóstica por tipo de apendicite (fleimonosa, gangrenada, perfurada). A caracterização dos grupos apendicectomia negativa (A) e apendicites perfuradas (B), bem como a relação entre estes dois grupos ao longo dos anos, constituíram objectivos secundários. Material e Método: Estudo retrospectivo dos dados clínicos de 1000 doentes consecutivamente operados com o diagnóstico clínico de apendicite aguda, no Hospital de Dona Estefânia, no período de 1 de Janeiro 2003 – 30 de Setembro 2007, procedendo-se à consulta da folha de requisição de exame histo-patológico enviada para o Serviço de Anatomia Patológica. Foram revistos os dados epidemiológicos, a qualidade da informação da referida folha de requisição e calculada a taxa de apendicectomia negativa nesta amostra. Resultados: O diagnóstico clínico pós-operatório foi concordante com o diagnóstico histológico em cerca de 60% casos, sendo subvalorizado ou sobrevalorizado nos restantes casos. A taxa de apendicectomia negativa observada foi de 5,5%, o que está abaixo dos valores apresentados na literatura. Conclusões: As apendicectomias negativas devem ser um “mal menor” em relação às apendicites perfuradas. A discordância clínico-histológica pode ter implicações médico-legais e tem seguramente implicações clínicas e económicas pelo que urge reavaliar o modelo de abordagem desta patologia tão frequente.

Risk Factors for Latex Sensitization in Children with Spina Bifida

Background: Children with spina bifida represent the major risk group for latex sensitization. Purpose: To determine the prevalence of latex sensitization in these children and to identify risk factors. Material and methods: We studied 57 patients with spina bifida. The mean age was 5.6 years and the male/female ratio was 0.8/1. In all patients a questionnaire, skin prick test (SPT) with latex (UCBStallergènes, Lofarma and ALK-Abelló), common aeroallergens and fruits (UCB-Stallergènes) and serum determination of total IgE (AlaSTAT) were performed. Results: The prevalence of latex sensitization was 30 %; only two sensitized children (12 %) had symptoms after exposure. Risk factors for latex sensitization were age 5 years (p = 0.008; OR = 6.0; 95% CI = 1.7-22.1), having at least four previous surgical interventions (p < 0.0001; OR = 18.5; 95% CI = 3.6-94.8), having undergone surgery in the first 3 months of life (p = 0.008; OR = 5.4; 95% CI = 0.7-29.2) and total serum IgE 44 IU/ml (p = 0.03; OR = 3.8; 95 %CI = 1.1-13.1). Multiple logistic regression analysis showed that only a history of four or more surgical interventions (p < 0.0001; OR = 26.3; 95 %CI = 2.9-234.2) and total serum IgE 44 IU/ml (p = 0.02; OR = 8.6; 95% CI = 1.4-53.4) were independently associated with latex sensitization. Sex, family and personal allergic history, hydrocephalus with ventriculoperitoneal shunt, cystourethrograms, intermittent bladder catheterization and atopy were not related to latex sensitization. Conclusions: In children with spina bifida, significant and independent risk factors identified for latex sensitization were multiple interventions and higher levels of total serum IgE. A prospective study will clarify the clinical evolution of assymptomatic children sensitized to latex.

Formas Imagiológicas Atípicas de “PRES” em Idade Pediátrica – Envolvimento Bulbo-Medular e Múltiplas Hemorragias – Nada Posterior e Pouco Reversível!

Introdução: Osíndrome de encefalopatia posterior reversível, ou PRES, é uma entidade clinico-radiológica associada a uma grande variedade de contextos clínicos, nomeadamente em idade pediátrica, com padrões imagiológicos mais típicos com envolvimento dos lobos parietais e occipitais mas também com outras formas imagiológicas, mais ou menos frequentes dependendo dos locais envolvidos (frontais e temporais, tronco cerebral, ganglios da base, cerebelo...). Objectivos: Apresentar formas de PRES em idade pediátrica com características imagiológicas infrequentes. Casos Clínicos: Apresentam-se os casos em duas jovens de sexo feminino, ambas com HTA grave e insuficiência renal, que se apresentaram com cefaleias intensas e uma delas com crises convulsivas, alteração do estado consciência e hemiparésia esquerda. Num dos casos a RM demonstrou um envolvimento extenso da medula e bulbo, apenas com uma pequena lesão parietal direita, com total reversão destas alterações em estudos de controle. No outro caso, a TC e a RM evidenciaram inúmeras lesões de edema com envolvimento multifocal de todos os lobos cerebrais e com múltiplas áreas hemorrágicas igualmente dispersas, desde micro-hemorragias a um hematoma mais volumoso, tendo-se constatado nos estudos de controle uma regressão parcial destas alterações. Conclusão: Apesar do envolvimento mais típico das regiões parietais e occipitais no PRES por lesões de edema vasogénico, padrões atípicos e infrequentes estão documentados e devem ser reconhecidos, incluindo-se nestes padrões o envolvimento predominantemente medular e a presença de múltiplas hemorragias.

Partial Rhombencephalosynapsis.

Clinical history - A 4-year-old boy, born prematurely at 29 weeks (twin pregnancy), with periventricular leukomalacia and epilepsy underwent brain MRI. Neurological examination showed severe developmental retardation with axial hypotonia, spastic tetraparesis and convergent strabismus. Imaging findings - Cranial MRI revealed typical aspects of partial rhombencephalosynapsis with vermian hypoplasia, midline fusion of the cerebellar hemispheres and transversely oriented folia and fissures. There was also mild dilatation and dysmorphism of the ventricular system, the septum pellucidum was absent, the hippocampi were malrotated and had vertical orientation and additional finding of associated periventricular cystic leukomalacia. Discussion - Rhombencephalosynapsis (RS) is a rare congenital defect of the cerebellum classically characterised by vermian agenesis or hypogenesis, fusion of the hemispheres, and closely apposed or fused dentate nuclei. It is now considered to result from an absence of division of the cerebellar hemispheres, following an insult between the 28th and 44th day of gestation (i.e., before the formation of the vermis). Other features have also been described such as fusion of the thalami and cerebral peduncles, malrotated hippocampi, corpus callosum agenesis, hypoplastic chiasm, absence of the septum pellucidum, ventriculomegaly, agenesis of the posterior lobe of the pituitary and cortical malformations. Musculoskeletal, cardiovascular, urinary tract, and respiratory abnormalities have been reported. Typical symptoms consist of swallowing difficulties, delayed motor acquisitions, muscular hypotonia, spastic quadriparesis, cerebellar signs including dysarthria, gait ataxia, abnormal eye movements, and seizures and hydrocephalus. The major MRI signs consist of fused cerebellar hemispheres, with absent or hypoplastic vermis, narrow diamond-shaped fourth ventricle and fused dentate nuclei. In a minority of cases, partial RS has been identified by MRI, demonstrating the presence of the nodulus and the anterior vermis and absence of part of the posterior vermis with only partial fusion of the hemispheres in the inferior part. Other cerebellar malformations involving vermian agenesis or hypoplasia include the Dandy–Walker continuum, Joubert syndrome, tectocerebellar dysraphy or pontocerebellar hypoplasias, and are now easily distinguished from RS by both brain MRI and morphology.