Awake airway endoscopy in mucopolysaccharidosis: a case report.

Mucopolysaccharidosis (MPS) are a group of rare genetic inherited diseases with a progressive course due to the accumulation of glycosaminoglycans resulting in anatomic abnormalities and organ dysfunction, including the respiratory, cardiovascular, skeletal, and neurological systems that can increase the risk of anesthesia complications. Clinical manifestations are variable, multisystemic, and include severe morphological changes. The anesthetic management of these patients is complex, particularly airway management, which can be planned to include a fiberoptic airway investigation prior to surgery. We present two cases of patients with MPS type VI and VII who underwent fiberoptic airway mapping under conscious sedation, with no complications. Since MPS is a rare but challenging disease concerning the airway management, we propose a safe and effective anesthetic technique that could be used for fiberoptic bronchoscopy and allow fiberoptic-assisted tracheal intubation at the time of surgery.

Awake airway endoscopy in mucopolysaccharidosis: a case report

Abstract Mucopolysaccharidosis (MPS) are a group of rare genetic inherited diseases with a progressive course due to the accumulation of glycosaminoglycans resulting in anatomic abnormalities and organ dysfunction, including the respiratory, cardiovascular, skeletal, and neurological systems that can increase the risk of anesthesia complications. Clinical manifestations are variable, multisystemic, and include severe morphological changes. The anesthetic management of these patients is complex, particularly airway management, which can be planned to include a fiberoptic airway investigation prior to surgery. We present two cases of patients with MPS type VI and VII who underwent fiberoptic airway mapping under conscious sedation, with no complications. Since MPS is a rare but challenging disease concerning the airway management, we propose a safe and effective anesthetic technique that could be used for fiberoptic bronchoscopy and allow fiberoptic-assisted tracheal intubation at the time of surgery.

Breast myofibroblastoma in a woman: a case report

Breast myofibroblastoma is a rare benign neoplasm of mesenchymal origin with fibroblastic and myofibroblastic characterizations. Myofibroblastoma has a higher incidence in men between the ages of 50 and 70 years and is less common in women. It is described as a solitary, unilateral, painless and mobile tumor, with a firm consistency and slow growth. Microscopically, it is a non-encapsulated tumor, with lobular growth, consisting of spindle cells organized in short, intersecting fascicles and interrupted by bundles of hyalinized collagen. On ultrasound, it can manifest as a hypoechoic solid mass, well circumscribed, homogeneous and similar to fibroadenoma; whereas, on mammography, a single, well-defined, rounded or discretely lobulated lesion can be observed, without calcifications. We report here the case of a 58-year-old patient with no previous breast complaints, who presented with changes in ultrasound and mammography examinations performed for breast cancer screening. The examinations revealed a suspicious lump in the left breast, classified as BIRADS 4C. Core biopsy described a low-grade spindle cell neoplasm, showing no signs of invasion, with immunohistochemistry results suggesting myofibroblastoma. As treatment, a sectorectomy was performed, and the reevaluated material confirmed the diagnosis of myofibroblastoma due to the positive expression of the markers calponin, CD34, BCL2 and CD99. (AU)

Use of recombinant human deoxyribonuclease in pediatric intensive care unit - a single-center experience.

OBJECTIVE: Dornase alfa (rhDNase) reduces the viscosity of purulent sputum in the lungs. The use in patients with cystic fibrosis (CF) is proven. However, the evidence of its applicability to other conditions is limited. This study aims to present the authors' experience with the use of rhDNase in non-CF patients admitted to the Pediatric Intensive Care Unit (PICU). At the study center, rhDNase was used during flexible bronchoscopies in 24 cases, of which 20 (83%) had atelectasis and seven (29%) were admitted to PICU. Four patients (57%) were on invasive mechanical ventilation (MV). CASE DESCRIPTION: Two cases of daily rhDNase administration at PICU are presented: patient A was an 8-year-old boy admitted with septic shock and acute respiratory distress syndrome (ARDS). The patient required mechanical ventilation with aggressive settings and experienced several clinical complications. On D50, he started rhDNase treatment with an improvement in FiO2, PaCO2 and PaO2/FiO2 ratio according to radiologic findings. He was extubated on D23 of treatment.Patient B was a 17-month-old girl admitted with a convulsive status epilepticus who experienced respiratory complications (infectious and barotrauma) with ARDS, requiring aggressive ventilation. She initiated rhDNase treatment on D60. During the treatment an improvement in FiO2, PaO2/FiO2 ratio and a tendency of PaCO2 decrease were found. She had radiological improvement. No complications were described. COMMENTS: RhDNase may be a helpful and safe tool to use in PICU prolonged intubated patients with ventilator-induced lung injury. Further studies are needed to assess and propose valid indications.

[The multidisciplinary challenge of anesthesia for ex utero intrapartum treatment: a case report]. / O desafio multidisciplinar da anestesia para procedimento intraparto extra­uterino: relato de caso.

The Ex- Utero Intrapartum Treatment (EXIT) is a surgical procedure performed in cases of expected postpartum fetal airway obstruction, allowing the establishment of patent airway while maintaining placental circulation. Anesthesia for EXIT procedure has several specific features such as adequate uterine relaxation, maintenance of maternal blood pressure fetal anesthesia and fetal airway establishment. The anesthesiologist should be aware of these particularities in order to contribute to a favorable outcome. This is a case report of an EXIT procedure performed on a fetus with a cervical lymphangioma with prenatal evidence of partial obstruction of the trachea and risk of post-delivery airway compromise.

The multidisciplinary challenge of anesthesia for ex utero intrapartum treatment: a case report / O desafio multidisciplinar da anestesia para procedimento intraparto extra-uterino: relato de caso

Abstract The Ex Utero Intrapartum Treatment (EXIT) is a surgical procedure performed in cases of expected postpartum fetal airway obstruction, allowing the establishment of patent airway while maintaining placental circulation. Anesthesia for EXIT procedure has several specific features such as adequate uterine relaxation, maintenance of maternal blood pressure fetal anesthesia and fetal airway establishment. The anesthesiologist should be aware of these particularities in order to contribute to a favorable outcome. This is a case report of an EXIT procedure performed on a fetus with a cervical lymphangioma with prenatal evidence of partial obstruction of the trachea and risk of post-delivery airway compromise.

Resumo O procedimento Intraparto Extra-Uterino (EXIT) é procedimento cirúrgico realizado em casos de previsão de obstrução de via aérea fetal no pós-parto, que permite estabelecer via aérea patente enquanto a circulação placentária é mantida. A anestesia para o procedimento EXIT apresenta várias características específicas, tais como relaxamento uterino adequado, manutenção da pressão arterial materna, anestesia fetal e estabelecimento da via aérea fetal. O anestesiologista deve estar ciente dessas especificidades para contribuir para desfecho favorável. Trata-se de relato de caso de procedimento EXIT realizado em feto com linfangioma cervical e evidência pré-natal de obstrução parcial de traqueia e risco de comprometimento de via aérea pós-parto.