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Burns are a common cause of pediatric injury and represent significant morbidity and mortality in this age group. In children, most burns are thermal, resulting from exposure to a hot surface, liquid, or fire. Cyanoacrylate is a liquid compound commonly found in households, mainly in “superglue” and nail glue. This compound solidifies through an exothermic reaction that is magnified when in contact with certain fibers that act as catalysts, such as cotton. In these circumstances, intense heat is produced in the contact area, potentially causing severe thermal burns. Despite its widespread availability, there is a paucity of safety information about cyanoacrylate and its role as a cause for burns. In the literature, only 18 cases of cyanoacrylate burns are reported in children. We present two cases of pediatric burns with cyanoacrylate and a review of the literature.

Introdução: A fenda lábio-palatina é a malformação congénita craniofacial mais frequente. Na presença de defeito ósseo, a técnica de enxerto ósseo alveolar secundário é o método de correção mais consensual entre os autores. Neste estudo avalia-se o resultado da aplicação desta técnica num hospital terciário.Material e Métodos: Análise dos enxertos ósseos alveolares secundários realizados entre 2007 e 2014, sendo incluídos os casos em que a crista ilíaca foi a região dadora e em que a informação clínica e imagiológica estava completa. A eficácia da intervenção foi avaliada radiologicamente com recurso à escala de Bergland (tipo I-IV), e correlacionada com variáveis associadas à patologia e/ou correção cirúrgica. Resultados: Dos 32 enxertos ósseos alveolares secundários realizados, 29 cumpriam os critérios de inclusão: 13 casos (44,8%) correspondiam a fendas pré-forâmen unilaterais completas; quatro (13,8%) a fendas pré-forâmen bilaterais completas; oito (27,6%) a fendas transforâmen unilaterais e quatro (13,8%) a fendas transforâmen bilaterais. Pela escala de Bergland (aplicada com um seguimento médio de 8 ± 5 meses), seis eram do tipo I, 15 do tipo II, cinco de tipo III e três do tipo IV. Não foi encontrada associação entre a eficácia da intervenção cirúrgica e o tipo de fenda lábio-palatina, presença do incisivo e fase de erupção do canino. Cinco doentes foram submetidos a novo enxerto ósseo alveolar (três tipo II e dois tipo III na avaliação inicial). Discussão: Na presente série, o enxerto ósseo alveolar foi eficaz na maioria dos doentes (72%, tipo I e II), independentemente do tipo de fenda lábio-palatina. A proporção de falências (10,3%) e a necessidade ulterior de reintervenção (17%) foram relativamente altas justificando o seguimento a longo-prazo e a continuação deste estudo. Conclusão: Importa realçar o envolvimento multidisciplinar para identificação atempada do momento ideal para intervenção e otimi-zação dos resultados.

OBJECTIVE The GBI (Glasgow Benefit Inventory) and the GCBI (Glasgow Children's Benefit Inventory) are used to evaluate patient-reported Health-Related Quality of Life (HRQoL) following otoplasty for prominent ears. The aim of this study was to validate them through patients' and caregivers' perspectives and in Portuguese language versions. STUDY DESIGN After translation to Portuguese, the GBI and the GCBI were applied to patients (n = 255) who underwent otoplasty throughout a five-year span and to their caregivers, respectively. A second set of questionnaires (retest) was applied to assess reliability. Caregivers were subsequently contacted to evaluate aesthetic satisfaction, recommendation to others, postoperative complications and main motivation for surgery. SETTING Paediatric Surgery Department of a tertiary hospital in Porto, Portugal. PARTICIPANTS 134 patients (aged 10.9 ± 3.6 years, 61.2% males) and their caregivers (52.5% responders). MAIN OUTCOME MEASURES Impact on HRQoL using the Portuguese GBI and GCBI (global score, -100 to 100). Validity assessed by principal component factor analysis, Cronbach's α and Intraclass Correlation Coefficient (ICC). RESULTS The GBI was 30.1 ± 25.7 and the GCBI was 22.6 ± 23.0. For the GBI, three subscales were identified, explaining 63.3% of variance. For the GCBI, two subscales were identified, accounting for 66.8% of variance. Both the GBI and the GCBI showed a high internal consistency (α = 0.92 and 0.94, respectively). 79% were satisfied and 92% recommended the surgery. Statistically significant higher scores were found in satisfied patients, in those who underwent surgery due to teasing, and in females. CONCLUSIONS The Portuguese GBI and GCBI proved to be valid and reliable to evaluate HRQoL following otoplasty in Portuguese-speaking children. The outcome was very favourable; almost all patients would recommend the surgery.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, neonatal developmental lung disorder, which usually presents as persistent pulmonary hypertension unresponsive to treatment. The authors report the case of a neonate with persistent pulmonary hypertension, associated with duodenal stenosis secondary to annular pancreas and intestinal malrotation. Support treatment, inhaled nitric oxide, oral sildenafil and nebulized iloprost were used with no clinical improvement. The neonate presented an overwhelming course, with hypoxemia refractory to treatment. At autopsy lung histology showed the characteristic features of ACD/MPV. DNA sequence analysis revealed a heterozygous nonsense mutation c.539C>A;p.S180X, in the first exon of FOXF1. FOXF1 has been identified as one of the genes responsible for ACD/MPV associated with multiple congenital malformations. This clinical case is the first report of a heterozygous nonsense mutation c.539C>A;p.S180X in the first exon of FOXF1, in a patient with ACD/MPV associated with annular pancreas and intestinal malrotation.

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Abdominoscrotal hydrocele (ASH) is a rare condition in childhood. It presents an intraabdominal portion with homogeneous content that is usually excised through a groin approach. A pale child with a recurrent scrotal hydrocele and a huge solid-cystic retroperitoneal mass is reported. Because the diagnostic workup has not ruled out malignancy, a laparotomy was performed; however, ultimate diagnosis was hemorrhagic ASH. The clinical features and surgical management of ASH are addressed. This entity should be considered in front of tense or recurrent hydroceles or coexistent abdominal mass even though with heterogeneous content.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, neonatal developmental lung disorder, which usually presents as persistent pulmonary hypertension unresponsive to treatment. The authors report the case of a neonate with persistent pulmonary hypertension, associated with duodenal stenosis secondary to annular pancreas and intestinal malrotation. Support treatment, inhaled nitric oxide, oral sildenafil and nebulized iloprost were used with no clinical improvement. The neonate presented an overwhelming course, with hypoxemia refractory to treatment. At autopsy lung histology showed the characteristic features of ACD/MPV. DNA sequence analysis revealed a heterozygous nonsense mutation c.539CA;p.S180X, in the first exon of FOXF1. FOXF1 has been identified as one of the genes responsible for ACD/MPV associated with multiple congenital malformations. This clinical case is the first report of a heterozygous nonsense mutation c.539CA;p.S180X in the first exon of FOXF1, in a patient with ACD/MPV associated with annular pancreas and intestinal malrotation.