Prosthetic carotid bypass graft for in-stent restenosis performed for post-endarterectomy recurrent stenosis: technical details

Aim. Carotid artery stenting (CAS) is the treatment of choice for recurrent stenosis after carotid endarterectomy (CEA). However a significative incidence of in-stent restenosis could be occurred. Despite classical CEA leads to good results, in selective cases bypass graft may be the best treatment of in-stent restenosis. Case reports. We describe two cases of carotid bypass graft performed to treat a recurrent in-stent stenosis after CAS for post-CEA restenosis. No death and cardiac complication occurred and no cranial nerves impairment was detected. Conclusion. Prosthetic bypass graft is safe and effective in treatment of in-stent recurrent restenosis after CEA restenosis.

Morfometria craniana do boto (Phocoena phocoena) na costa portuguesa

Eventos climáticos extremos poderão ter conduzido à instabilidade de habitats e em última instância à sua fragmentação, causando repercussões ainda visíveis nos predadores de topo, tais como o boto (Phocoena phocoena). Consequentemente, os indivíduos que não se adaptaram, tiveram de procurar novos habitats com as respetivas condições ideais, nomeadamente os indivíduos da população de botos do Atlântico Este (norte e sul da Baía de Biscaia). O presente estudo focou-se em indivíduos da costa portuguesa (representando uma amostra da população residente a sul da Baía de Biscaia) e, recorrendo a informação proveniente de estudos previamente realizados, procurou salientar diferenças entre os botos que habitam ao longo do Atlântico Este. Foi aplicado um método de limpeza e branqueamento em crânios de boto, para posterior medição. Foram medidos 65 crânios de botos (programa Image J®), providenciados pela rede de arrojamentos de animais marinhos e obtidos no âmbito do projeto LIFE+ MarPro. As comparações das medidas dos crânios entre géneros foram realizadas a partir de modelos alométricos (programa GraphPad Prism ®) e as comparações entre géneros de cada estágio de desenvolvimento foram efetuadas com recurso a uma análise de PERMANOVA unifatorial (usando distâncias euclidianas), através do programa PAST® v. 2.12. Concluiu-se que na população em estudo, tanto as fêmeas como os machos apresentaram um modelo de alometria negativa. Reportou-se que as fêmeas apresentam comprimentos corporais superiores aos machos, assim como comprimentos e larguras cranianas. Comparativamente aos indivíduos da população a norte da Baía de Biscaia, a amostra de estudo da população do sul da Baía de Biscaia (representada pelos indivíduos da costa portuguesa) apresentou maiores comprimentos corporais e cranianos e maior largura craniana. Estes resultados corroboram observações prévias sobre a população de botos do Atlântico, que detetaram já diferenças genéticas e partilha limitada de genes, diferenças nos hábitos alimentares e habitats distintos nos botos de norte e sul da Baía de Biscaia.

Intellectual disability, coarse face, relative macrocephaly, and cerebellar hypotrophy in two sisters

We report on two Portuguese sisters with a very similar phenotype characterized by severe intellectual disability, absent speech, relative macrocephaly, coarse face, cerebellar hypotrophy, and severe ataxia. Additional common features include increased thickness of the cranial vault, delayed dental eruption, talipes equino-varus, clinodactyly, and camptodactyly of the fifth finger. The older sister has retinal dystrophy and the younger sister has short stature. Their parents are consanguineous. We suggest this condition constitutes a previously unreported autosomal recessive entity.

Tumores do espaço parafaríngeo: experiência de 10 anos do IPO-LFG

Introdução: As neoplasias do espaço parafaríngeo são raras, representando apenas 0,5% dos tumores da cabeça e pescoço. A maioria são benignas, mas uma ampla variedade de patologias benignas e malignas podem ser encontradas neste espaço, o que cria desafios complexos de diagnóstico e tratamento. Objetivo: Descrever e analisar uma série de casos de neoplasias primárias do espaço parafaríngeo tratadas no Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG). Material e métodos: Estudo retrospetivo, com recolha e análise dos dados dos processos clínicos de tumores primários do espaço parafaríngeo, que foram diagnosticados ou referenciados ao IPOLFG entre 1 de Janeiro de 2003 e 31 de Dezembro de 2013. Resultados: Foram incluídos 38 doentes. A idade mediana foi de 52 anos (Âmbito Interquartil: 40-63 anos). Dez (26,3%) doentes eram assintomáticos. O sintoma mais comum à apresentação foi a sensação de corpo estranho orofaríngeo (23,7%) e o achado mais frequente foi um abaulamento orofaríngeo (78,4%). Todos os doentes fizeram exames de imagem pré-operatórios: 94,7% tomografia computorizada e 68,4% ressonância magnética. A citologia aspirativa foi realizada em 39,5%. 31 tumores eram benignos (81,6%), sendo os mais frequentes os adenomas pleomórficos (58,1%). 7 eram malignos (18,4%), com os carcinomas exadenomas pleomórficos (28,6%) e os linfomas (28,6%) sendo os mais comuns. 36 doentes (94,7%) foram submetidos a tratamento cirúrgico primário; os outros 2 doentes (5,3%) receberam tratamento não cirúrgico, com quimioterapia e quimioradioterapia, respectivamente. A abordagem cervical foi a mais utilizada (80%). A mandibulotomia foi necessária em apenas 5,7%. A complicação mais frequente foi a neuropatia de pares cranianos de novo, identificada em 22,2%. Destes, 75% foram sequela da resseção de tumores neurogénicos. Todas as neuropatias que resultaram da resseção de tumores não neurogénicos foram transitórias. O follow-up mediano foi de 6,5 anos. A taxa de recorrência foi de 13,5%. Conclusões: Os tumores do espaço parafaríngeo requerem um elevado índice de suspeição para serem diagnosticados num estadio precoce. A resseção cirúrgica completa é o principal tratamento. A abordagem cirúrgica deve ser selecionada caso a caso, mas a cervical fornece um excelente acesso à maioria dos tumores deste espaço

Two Cases of Arnold-Chiari Malformation with Respiratory Failure

Arnold–Chiari malformation is defined as downward displacement of the brainstem and cerebellum through the foramen magnum. It has different clinical presentations and four subtypes. It is known that downward migration of posterior fossa components through the foramen magnum and associated lower cranial nerve palsy and brainstem compression can cause respiratory failure. Acute respiratory failure could mark the onset of the disease. Posterior fossa decompression performed to treat primary disease can improve the central sleep abnormalities. As respiratory failure is rarely seen, this paper presents two cases of Arnold–Chiari malformation with respiratory failure.

OnabotulinumtoxinA for hemicrania continua: open label experience in 9 patients

BACKGROUND: Hemicrania continua is a strictly unilateral, continuous headache, typically mild to moderate in severity, with severe exacerbations commonly accompanied by cranial autonomic features and migrainous symptoms. It is exquisitely responsive to Indomethacin. However, some patients cannot tolerate treatment, often due to gastrointestinal side effects. Therapeutic alternatives are limited and controlled evidence lacking. METHODS: We present our experience of nine patients treated with OnabotulinumtoxinA for hemicrania continua. All patients were injected using the PREEMPT (Phase 3 REsearch Evaluating Migraine Prophylaxis Therapy) protocol for migraine. RESULTS: Five of nine patients demonstrated a 50% or more reduction in moderate to severe headache days with OnabotulinumtoxinA with a median reduction in moderate to severe headache days of 80%. Patient estimate of response was 80% or more in five subjects. The median and mean duration of response in the five responders was 11 and 12 weeks (range 6-20 weeks). Improvements were also seen in headache-associated disability CONCLUSIONS: OnabotulinumtoxinA adds a potential option to the limited therapeutic alternatives available in hemicrania continua.

Embolisation of Vertebral Artery Damaged Following Cervical Disc Removal

Introduction: The incidence of vertebral artery (VA) injury during cervical spine surgery is rare. Even though tamponade is effective in many cases, early consultation of an endovascular team is recommended if bleeding cannot be controlled. We report a case of emergent endovascular embolisation of left VA due to iatrogenic injury during anterior cervical disc removal and fusion. Case: A 47-year-old woman was admitted to our emergency department with serious arterial bleeding from the neck only hours after undergoing anterior cervical disc removal and fusion surgery. She was intubated and mechanically ventilated, however hemorrhage could not be successfully controlled by packing with surgical hemostatic agents. Cranial computed tomography, computed tomography of the cervical spine and CT angiography confirmed the suspected diagnosis of injury to the VA. Emergent endovascular embolisation successfully stopped the bleeding. Occlusion of the vessel was achieved by vascular plugging. The patient was discharged from our hospital 14 days after the intervention, receiving a revision surgery of the cervical spine on the day of embolisation. At the date of discharge she presented without any focal neurological deficit. Conclusion: Pre-operative radiographic imaging of the cervical spine should be used routinely to identify anatomic abnormalities of the vertebral arteries. Endovascular embolisation appears to be effective in treating acute iatrogenic dissection of the vertebral arteries.

Giant Cell Arteritis Presenting with Bilateral Subdural Haematomas of Arterial Origin

Giant cell arteritis or temporal arteritis is an inflammatory condition affecting medium to large sized vessels, particularly the cranial arteries. A 76-year-old man with no significant past medical history presented to the emergency department with a 3-week history of diffuse headaches associated with fever, loss of appetite, weight loss and general malaise. A CT scan of the brain showed bilateral shallow chronic low density subdural haematomas. A complete laboratory panel was unremarkable except for a raised erythrocyte sedimentation rate and elevated C-reactive protein. A transthoracic echocardiogram and CT scan of the body were unremarkable. MRI of the brain confirmed bilateral old subdural collections and showed marked vessel wall enhancement in the frontal branches of the left superficial temporal artery. A left temporal artery biopsy confirmed giant cell temporal arteritis. We speculate that a vasculitic process in the small subdural arteries may have contributed to our patient’s spontaneous subdural haematomas.

Testicular arteries systematization based on different levels of scrotal configuration in caprines

O objetivo deste trabalho foi analisar a distribuição dos vasos arteriais nos testículos em caprinos com diferentes graus de divisão escrotal. A configuração escrotal foi classificada da seguinte forma: Grupo I: constituído por caprinos com escroto único, Grupo II: com escroto separado até a metade do testículo e Grupo III: com separação escrotal estendendo-se além da metade do testículos. As artérias foram injetadas e coradas com solução de acetado de vinil, sendo os orgãos (30 pares) submetidos à corrossão para obtenção dos moldes vasculares. As artérias testiculares emergem da aorta abdominal, com trajeto retilíneo, atravessam o canal inguinal, apresentam-se espiraladas e envolvidas parcialmente pelo plexo pampiniforme. Próximo à extremidade caudada do testículo, dividem-se mais freqüentemente nos ramos cranial e caudal, os quais emitem vasos colaterais, de onde emergem ramos penetrantes. Os testículos dos animais com nível intermediário de divisão escrotal (Grupo II) apresentam menor quantidade destes ramos, sendo os quadrantes mais povoados o ventrolateral e o dorsolateral. Conclui-se que a origem, o trajeto e a distribuição das artérias testiculares não apresentam variações relacionadas ao grau de divisão escrotal em caprinos.

Evolução morfométrica dos anexos embrionários e fetais bovinos obtidos por monta natural, com 10 a 70 dias da gestação

O período inicial da gestação de bovinos é caracterizado por grandes perdas embrionárias. Considerando a importância deste fator no âmbito da reprodução animal foram estudados os anexos embrionários e fetais bovinos fecundados por monta natural de 15-70 dias de gestação, com o objetivo de estabelecer parâmetros morfométricos da placenta na fase inicial da gestação. Com uso de um paquímetro foram realizadas mensurações do comprimento (crânio caudal), largura (latero lateral) e altura (dorso ventral) das membranas corioalantóide e amniótica. O início da formação dos cotilédones foi observado e quantificado, assim como, o peso placentário. O peso médio do saco gestacional aumentou com o evoluir da idade gestacional, entretanto, o crescimento foi acelerado a partir de 20-30 dias de gestação. O comprimento crânio caudal e dorso ventral da membrana corioalantóide e do âmnio apresentaram crescimento lento e gradual com o evoluir dos períodos gestacionais analisados. Com 30-40 dias de gestação, os primeiro cotilédones já eram visualizados e contatos com facilidade na superfície coriônica. Os períodos de crescimento coincidiram com os maiores índices de perdas gestacionais em bovinos. Os parâmetros aqui analisados poderão servir para futuras investigações dos anexos embrionários de organismos manipulados em laboratório.

Incidence and Pattern of Pneumonia in Goats Slaughtered at the Kumasi Abattoir, Ghana

This study aimed at determining the incidence and pattern of pneumonia, in slaughtered goats in Kumasi abattoir, Ghana. One thousand three hundred and fifty lungs of goats; (1,012 Sahelian and 338 West Africa Dwarf goats (WAD) lungs) of different ages (less than a year to above 4 years) were used in this study. The frequency of occurrence of pneumonia, the degree of consolidation as a percentage of the total lung volume and histological assessment were determined by standard techniques. Fifty five (55) lungs (39 Sahelian, 16 WAD goats) were pneumonic (4.07% prevalence). The right lungs had a significant higher average lung consolidation percentage (19.11) while the right cranial lobes were more affected (9.37). WAD goats of 1-2 years are mostly affected with an average percentage consolidation of 11.73% while Sahelian goats above 4 years of age were the most affected with 32.59% consolidation. Does of both breeds were more while Sahelian goats had higher consolidation than other breeds. Histological examination revealed the presence of giant cell, fibrinous and suppurative bronchointerstitial pneumonia suggesting complicated viral pneumonia which was observed to be the most important caprine pneumonia in slaughtered goats in Ghana. Transportation and pregnancy stress were the major contributory factor to the pneumonia observed hence effective ante-mortem examinations will help to minimize the slaughter of pregnant does and transportation stress in Ghana.

Insights into Neural Crest Evolution

Neural crest cells are unique to vertebrates and essential to the development and evolution of the craniofacial skeleton. Using a combination of DiI cell lineage tracing, transcriptomics, and analysis of key transcription factors of the Sox Family, I examined neural crest development in the sea lamprey, Petromyzon marinus, as the most basal extant vertebrate from which it is possible to get embryos. The results have uncovered distinct cranial and trunk neural crest subpopulations along the anterior-posterior axis of the lamprey embryo, with a clear separation between the two. However, no evidence of the presence of an intermediate vagal neural crest population was uncovered. Comparing cranial neural crest genes between lamprey and chick, either by examining individual candidate genes or whole genome transcriptome analysis, reveals significant changes in the cranial neural crest gene regulatory network of lamprey compared with chick. In particular, the lamprey cranial neural crest is "missing" several gnathostome cranial crest genes. We speculate that these may underlie the evolutionary divergence of craniofacial development between jawed and jawless vertebrates. Despite the absence of vagal neural crest, DiI-labeling shows that trunk neural crest-derived cells, likely homologous to mammalian Schwann cell precursors, contribute to the lamprey enteric nervous system, potentially representing the most primitive form of neural crest cells contribution to the ENS. Finally, I characterized key members of the Sox Family (Sox B-F) due to their importance in neural crest specification in other species. In comparative studies of the SoxC genes (Sox4, Sox11, and Sox12) in both lamprey and Xenopus, I found similar expression patterns and a novel key role in early neural crest specification, suggesting a conserved role of the SoxC genes amongst vertebrates. Taken together, this work represents important progress in characterizing the early evolution of the neural crest in vertebrates and its role in the transition from jawless to jawed vertebrates.

Attention Deficit Hyperactivity Disorder in a Patient With Congenital Mirror Movement Disorder and Colpocephaly

Introduction: Congenital mirror movement disorder designates involuntary movements on one side of the body that occur as mirror of the intentional movements on the contralateral side. Colpocephaly is described as persistence of fetal configuration of lateral ventricles. Case Presentation: A two-month old male infant was brought to the hospital due to bilateral identical movements of the hands. Except for bilateral involuntary synkinetic imitative movements in hands, neurological and physical examination was normal. Cranial MRI showed corpus callosum dysgenesis, hypogenesis and dilation of bilateral lateral ventricular posterior horns (colpocephaly). At the age of 7 years, he was started to use metylphenydate to mitigate attention deficit and hyperactivity disorder. The mirror movements were decreasing in amplitude by years and were not so serious to affect normal life activities. Conclusions: Mirror movements, diagnosed usually during childhood, may be congenital or secondary to neurological diseases. Although they generally do not affect normal life activities, in some cases severity of mirror movements causes a real debilitating disease. In our case the patient was diagnosed at the age of 2 months and on follow-up no debilitating problems were observed. This is the first case to describe the association of colpocephaly and mirror movements. The exact mechanism of this association is not known. Although it is known that mirror movements may be in relation with some pychiatric pathologies, this is the first report of attention deficit and hyperactivity disorder in conjunction with mirror movements and/or colpocephaly. Managing comorbidities, either physical or psyhchological, will help the patient to live in good health without trying to cope with other pathological diseases.

The Role of the Transcription Factor Ets1 in Melanocyte Development

Melanocytes, pigment-producing cells, derive from the neural crest (NC), a population of pluripotent cells that arise from the dorsal aspect of the neural tube during embryogenesis. Many genes required for melanocyte development were identified using mouse pigmentation mutants. The deletion of the transcription factor Ets1 in mice results in hypopigmentation; nevertheless, the function of Ets1 in melanocyte development is unknown. The goal of the present study was to establish the temporal requirement and role of Ets1 in murine melanocyte development. In the mouse, Ets1 is widely expressed in developing organs and tissues, including the NC. In the chick cranial NC, Ets1 is required for the expression of Sox10, a transcription factor critical for the development of melanocytes, enteric ganglia, and other NC derivatives. Using a combination of immunofluorescence and cell survival assays Ets1 was found to be required between embryonic days 10 and 11, when it regulates NC cell and melanocyte precursor (melanoblast) survival. Given the requirement of Ets1 for Sox10 expression in the chick cranial NC, a potential interaction between these genes was investigated. Using genetic crosses, a synergistic genetic interaction between Ets1 and Sox10 in melanocyte development was found. Since Sox10 is essential for enteric ganglia formation, the importance of Ets1 on gut innervation was also examined. In mice, Ets1 deletion led to decreased gut innervation, which was exacerbated by Sox10 heterozygosity. At the molecular level, Ets1 was found to activate a Sox10 enhancer critical for Sox10 expression in melanoblasts. Furthermore, mutating Ets1 at a site I characterized in the spontaneous variable spotting mouse pigmentation mutant, led to a 2-fold decrease in enhancer activation. Overexpression and knockdown of Ets1 did not affect Sox10 expression; nonetheless, Ets1 knockdown led to a 6-fold upregulation of the transcription factor Sox9, a gene required for melanocyte and chondrocyte development, but which impairs melanocyte development when its expression is prolonged. Together, these results suggest that Ets1 is required early during melanocyte development for NC cell and melanoblast survival, possibly acting upstream of Sox10. The transcription factor Ets1 may also act indirectly in melanocyte fate specification by repressing Sox9 expression, and consequently cartilage fate.