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.

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.

Lesões Oculares por Processionária

Introdução: A Processionária (thaumetopoea pityocampa Schiff), vulgarmente conhecida como “lagarta do pinheiro” é um inseto dos pinheiros e cedros, endémico em meios rurais mas também em meios urbanos em Portugal. A toxicidade ocular, rara nas últimas décadas pelo desenvolvimento de métodos de erradicação eficazes, é provocada pelos seus pelos e prevê-se mais frequente com o recrudescimento deste inseto. Revemos a epidemiologia da Processionária e as suas lesões oculares a partir de 3 casos clínicos. Material e métodos: Caso 1: Doente de 64 anos recorre ao Serviço de Urgência (SU) com olho direito vermelho e sensação de corpo estranho após prática de jardinagem. A observação revela VODc: 0.5, erosão epitelial, presença de 1 filamento no estroma corneano profundo, flare (++) e Tyndall (+++). Caso 2: Doente de 28 anos, recorre ao SU por dor intensa no olho direito acompanhada de hiperémia após contacto com lagarta. Apresenta VODc: 0.6 e Tyndall (+++) com presença de múltiplos filamentos (mais de 20) a diferentes profundidades da córnea. Caso 3: Doente de 26 anos, recorre ao SU por sensação de corpo estranho e lacrimejo constante no olho direito, após ter estado a realizar exercícios militares num parque urbano. Apresenta VODc: 0.3, múltiplas erosões epiteliais punctiformes na metade nasal da córnea que recobriam filamentos de cor laranja e Tyndall (+). Foi instituída terapêutica com corticoide tópico e vigilância sintomática a cada um dos casos. Resultados: A patologia ocular por Processionária decorre da toxicidade dos seus pelos, cuja migração ocorre preponderantemente no sentido intraocular. Inclui por isso lesões precoces (conjuntivite, queratite e uveíte) e tardias (catarata, pars planite, vitrite e retinite). Os casos apresentados possuíam lesões iniciais, tendo recuperado totalmente do quadro inflamatório após 6 meses mas mantendo os pelos inativos no estroma corneano. A gravidade destes casos prende- -se com a possibilidade de migração intraocular, que pode ocorrer anos após o episódio inicial, obrigando a uma vigilância ao longo da vida. Conclusões: O recrudescimento da Processionária tanto em meios rurais como urbanos em Portugal justifica o conhecimento das lesões oculares que pode causar e do seu tratamento.

The Hybrid Approach for Palliation of Hypoplastic Left Heart Syndrome: Intermediate Results of a Single-Center Experience

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. METHODS: We reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. RESULTS: Nine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. CONCLUSIONS: In our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.