Manejo anestésico en paciente pediátrico con vía aérea difícil afectado de epidermólisis ampollosa distrófica / Anaesthetic management in a paediatric patient with a difficult airway due to epidermolysis bullosa dystrophica

La epidermólisis ampollosa distrófica (EAD) es una patología hereditaria infrecuente, caracterizada por la formación de ampollas ante mínimos traumatismos, que tienden a formar cicatrices distróficas y llevan a secuelas limitantes y amenazantes para la vida. Es frecuente que los pacientes afectados de EAD precisen diferentes intervenciones quirúrgicas a lo largo de su vida. Los principales objetivos del manejo anestésico son la prevención del traumatismo/infección sobre la piel o las mucosas y el establecimiento de una vía aérea segura sin producir ampollas. El posicionamiento del paciente y el material utilizado para la monitorización de signos vitales y para la administración de agentes anestésicos pueden producir nuevas lesiones. Debe lubricarse todo el material y evitar adhesivos y movimientos de fricción sobre la piel. Estos pacientes suelen presentar múltiples comorbilidades y accesos vasculares y vía aérea difíciles. La formación de nuevas ampollas durante la instrumentación de vía aérea puede llevar a una obstrucción aguda de la misma. Presentamos el caso de un paciente con EAD y vía aérea difícil sometido a corrección de sindactilia y exodoncia múltiple (AU)

Dystrophic epidermolysis bullosa (DEB) is a rare inherited disorder characterized by blistering after minimal trauma. These blisters tend to form dystrophic scars, leading to limiting and life-threatening sequelae. The anaesthetic management of patients with DEB is a challenge, even for the most experienced anaesthesiologists, but basic principles can help us prepare the plan of care. The main goals are to prevent trauma/infection of skin/mucous, and to establish a secure airway without causing bullae. Patient positioning and the instruments used to monitor vital signs and administering anaesthetic agents can cause new lesions. It is advisable to lubricate the instruments and to avoid adhesive material and shearing forces on the skin. Besides the implications of the comorbidities, there is a potential difficult intubation and difficult vascular access. Acute airway obstruction can occur due to airway instrumentation. We report the case of a patient diagnosed with EBD difficult airway and undergoing correction of syndactylyl and dental extractions (AU)

[Anaesthetic management in a paediatric patient with a difficult airway due to epidermolysis bullosa dystrophica]. / Manejo anestésico en paciente pediátrico con vía aérea difícil afectado de epidermólisis ampollosa distrófica.

Dystrophic epidermolysis bullosa (DEB) is a rare inherited disorder characterized by blistering after minimal trauma. These blisters tend to form dystrophic scars, leading to limiting and life-threatening sequelae. The anaesthetic management of patients with DEB is a challenge, even for the most experienced anaesthesiologists, but basic principles can help us prepare the plan of care. The main goals are to prevent trauma/infection of skin/mucous, and to establish a secure airway without causing bullae. Patient positioning and the instruments used to monitor vital signs and administering anaesthetic agents can cause new lesions. It is advisable to lubricate the instruments and to avoid adhesive material and shearing forces on the skin. Besides the implications of the comorbidities, there is a potential difficult intubation and difficult vascular access. Acute airway obstruction can occur due to airway instrumentation. We report the case of a patient diagnosed with EBD difficult airway and undergoing correction of syndactylyl and dental extractions.

[WITHDRAWN: Fetus with large cervical mass underwent Extrauterine Intrapartum Treatment.] / WITHDRAWN: Feto con gran masa cervical sometido a Tratamiento Intraparto Extrauterino.

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[Giant omphalocele correction in a patient with pentalogy of Cantrell]. / Corrección de onfalocele gigante en un paciente con pentalogía de Cantrell.

Pentalogy of Cantrell is a rare congenital malformation characterised by a large defect in the lower region of the sternum, diaphragm, and lower abdominal region. It is also characterised by the migration of organs, and its prognosis depends on the presence of cardiac malformations. We present the case of an 18 year-old male subjected to a scheduled giant omphalocele correction. Invasive monitoring, including cardiac output, was used to avoid episodes of instability due to the increase in abdominal pressure and the increase in venous return (as had happened on two previous operations). The prognosis depends on multiple factors, with good haemodynamic and respiratory control being the most important. Mortality is high despite the advances in monitoring.

Corrección de onfalocele gigante en un paciente con pentalogía de Cantrell / Giant omphalocele correction in a patient with pentalogy of Cantrell

La pentalogía de Cantrell es una rara malformación congénita caracterizada por un amplio defecto de la región inferior del esternón, el diafragma y la región anterior abdominal. Se caracteriza por la migración de órganos y su pronóstico depende de la presencia de malformaciones cardiacas. Presentamos el caso de un varón de 18 años sometido a corrección programada de un onfalocele gigante. Se empleó monitorización invasiva y del gasto cardiaco para evitar (como había sucedido en dos intervenciones previas) episodios de inestabilidad por aumento de la presión intraabdominal y disminución del retorno venoso. El pronóstico depende de múltiples factores; lo más importante es el buen control hemodinámico y respiratorio. A pesar de la monitorización avanzada, la mortalidad es muy alta(AU)

Pentalogy of Cantrell is a rare congenital malformation characterised by a large defect in the lower region of the sternum, diaphragm, and lower abdominal region. It is also characterised by the migration of organs, and its prognosis depends on the presence of cardiac malformations. We present the case of an 18 year-old male subjected to a scheduled giant omphalocele correction. Invasive monitoring, including cardiac output, was used to avoid episodes of instability due to the increase in abdominal pressure and the increase in venous return (as had happened on two previous operations). The prognosis depends on multiple factors, with good haemodynamic and respiratory control being the most important. Mortality is high despite the advances in monitoring(AU)

Tratamiento perioperatorio de una niña con origen anómalo de la arteria coronaria izquierda desde la arteria pulmonar (Síndrome de Alcapa) / No disponible

No disponible

Masa cervical gigante en recién nacida pretérmino / Giant cervical mass in a premature neonate

No disponible

No disponible

[Bilateral ossification of the earlobes. Addisonian crisis in the postoperative period]. / Osificación bilateral de los pabellones auriculares. Crisis addisoniana en el postoperatorio.

Case report. A 53 year-old male patient was scheduled for surgical resection of biliary lithiasis and correction of anal fistula. He had previous history of repetitive biliary colics and appendicectomy at the age of 34 with undetermined anesthetic complications. Under epidural anesthesia with 270 mg of mepivacaine at the L1-L2 level cholecystectomy and fistulectomy was performed with any immediate surgical or postoperative complication. Eighteen hours after surgery the patient presented clinical signs of shock with systolic arterial pressure between 40 and 50 mmHg. This clinical situation associated with the finding of hard ear auricles with radiologic signs of calcification allowed to suspect the presence of an addisonian crisis. Clinical recovery was obtained with corticosteroid therapy: a bolus of 500 mg of methylprednisolone followed by 40 mg every 8 hours. The postoperative period was therefore uneventful. Examination of suprarenal function demonstrated the presence of Addison disease. Calcification of ear auricles is an uncommon clinical sign which is associated to local traumatisms, gout, and several endocrine diseases. The presence of ear auricle calcification in postoperative patients with shock should lead to consider the possibility of addisonian crisis.

[Myopericarditis in the 3d pregnancy trimester. Apropos of a case]. / Myopéricardite au troisième trimestre de la grossesse. A propos d'un cas.

The authors describe a case of myocardiopathy, during the third trimester of pregnancy, in a patient without any significant previous cardiac history nor associated obstetrical pathology. The clinical course was uneventful without sequelae. The clinical importance of peri-partum myocardiopathy, especially regarding myocardial sequelae, imposes a differential diagnosis and an analysis of the current state of this pathological process.