Manejo anestésico de un niño con déficit de acetil-coA deshidrogenasa de cadena corta / Anesthetic management of a child with deficit of chain short acetyl-coA dehydrogenase

Los errores innatos del metabolismo de la betaoxidación de los ácidos grasos (déficit de acil-CoA deshidrogenasas) constituyen un nuevo grupo de enfermedades. El déficit de SCAD (Acil-CoA deshidrogenasa de ácidos grasos de cadena corta) es un fallo congénito de herencia autosómica recesiva de la oxidación mitocondrial de los ácidos grasos de cadena corta. Su incidencia probablemente esté subestimada, debiéndose tener un alto índice de sospecha diagnóstica para su detección. Esta enfermedad plantea importantes implicaciones anestésicas sobre todo en el uso de fármacos como el propofol o los bloqueantes neuromusculares por lo que es preciso conocer su fisiopatología (AU)

An inborn error of metabolism of beta-oxidation of fatty acids (acyl-coA deshydrogenasa deficiencies) is a new group of diseases. Short chain acylcoA deshydrogenasa deficiency (SCAD) is a congenital failure of autosomal recessive inheritance of mitochondrial oxidation of short chain fatty acids. The incidence is probably underestimated, whichever a high index of clinical suspicion is necessary for detection. This disease poses significant anesthetic implications especially in the use of drugs such propofol or neuomuscular blockers so it is necessary to understand its pathophisiology (AU)

[Use of a metal guide in the working channel of a fiberoptic scope to insert a tracheal tube in an infant with Treacher Collins syndrome and choanal atresia]. / Uso de guía metálica para intubación traqueal mediante fibroscopia en un neonato con síndrome de Treacher Collins y atresia de coanas.

Neonates with Treacher Collins syndrome can present difficult airways. Ventilation through a face mask and laryngoscopy for tracheal intubation may prove impossible due to the craniofacial malformations that are characteristic of this syndrome. Furthermore, patients with this syndrome are at high risk of airway obstruction, meaning that awake fiberoptic endoscopy provides the best option for tracheal intubation. This technique is especially difficult in children, however, and material required for performing it in neonates is not always available. We report the case of a 5-day-old infant boy with Treacher Collins syndrome and bilateral choanal atresia in whom we used a flexible metal guide inserted into the working channel of a fiberoptic scope. The tracheal tube could then be inserted.

Uso de guía metálica para intubación traqueal mediante fibroscopia en un neonato con síndrome de Treacher Collins y atresia de coanas / Use of a metal guide in the working channel of a fiberoptic scope to insert a tracheal tube in an infant with Treacher Collins syndrome and choanal atresia

Los neonatos afectos de síndrome de Treacher Collinspueden presentar dificultades en el manejo de la víaaérea. Las malformaciones a nivel del macizo craneofacial,características de este síndrome, implican que laventilación con mascarilla facial y la laringoscopia paraintubación orotraqueal puedan resultar imposibles. Además,el mayor riesgo de obstrucción de la vía aérea quepresentan estos pacientes hace que sea la fibroscopia,con el paciente despierto, el método más adecuado parala intubación orotraqueal. Esta técnica es especialmentedifícil en la población pediátrica y no siempre se disponede material específico para neonatos. Presentamos elcaso de un niño de 5 días de vida con síndrome de TreacherCollins y atresia coanal bilateral en el que se utilizóuna guía metálica flexible, introducida a través del canalde trabajo del fibroscopio para seguidamente insertar eltubo orotraqueal a través de dicha guía(AU)

Neonates with Treacher Collins syndrome can presentdifficult airways. Ventilation through a face mask andlaryngoscopy for tracheal intubation may proveimpossible due to the craniofacial malformations thatare characteristic of this syndrome. Furthermore,patients with this syndrome are at high risk of airwayobstruction, meaning that awake fiberoptic endoscopyprovides the best option for tracheal intubation. Thistechnique is especially difficult in children, however, andmaterial required for performing it in neonates is notalways available. We report the case of a 5-day-oldinfant boy with Treacher Collins syndrome and bilateralchoanal atresia in whom we used a flexible metal guideinserted into the working channel of a fiberoptic scope.The tracheal tube could then be inserted(AU)

[Recombinant activated factor VII in the postanesthetic recovery unit: review of 16 cases]. / Utilización del rFVIIa en una unidad de reanimación. Revisión de 16 casos.

We describe a series of 16 cases in which recombinant activated factor VII (rFVIIa) was used in our postanesthetic recovery unit. The mean age of the patients was 53.5 years (range, 30-84 years). Eleven were men and 5 women. The mean dose of rFVIIa used was 75 microg x kg(-1) (range, 60-90 microg x kg(-1)) and 25% of the patients needed a second dose. All the patients had postoperative bleeding, 62.5% after general surgery, 25% after a liver transplant, and 12.5% after a lung transplant. rFVIIa therapy was effective in 66% of the patients and no adverse thrombotic events related to treatment were observed. rFVIIa can be an efficacious therapeutic option for bleeding and coagulation disorders that are refractory to conventional replacement therapy. Approval to use rFVIIa in this setting and the establishment of indications should be based on further research.

Utilización del rFVIIa en una Unidad de Reanimación. Revisión de 16 casos / Recombinant activated factor VII in the postanesthetic recovery unit: review of 16 cases

Describimos una serie de 16 pacientes en los que seutilizó el factor VII recombinante activado (rFVIIa) ennuestro Servicio de Reanimación.La media de edad fue de 53,5 años (30-84), 11 varonesy 5 mujeres. La dosis media de rFVIIa utilizada fue de75 µg kg-1 (60-90 µg kg-1) precisando repetir la dosis en el25% de los casos. Todos los pacientes padecían complicacioneshemorrágicas postoperatorias, el 62,5% trascirugía general, el 25% tras trasplante hepático y el12,5% tras trasplante pulmonar.En el 66% de los enfermos, el factor fue efectivo y nohubo episodios trombóticos clínicos secundarios al tratamiento.El rFVIIa puede ser un arma terapéutica efectiva enhemorragias con coagulopatía refractarias al tratamientosustitutivo habitual. La aprobación para su uso eneste contexto así como la determinación de sus indicacionesprecisa de más estudios de investigación

We describe a series of 16 cases in which recombinantactivated factor VII (rFVIIa) was used in our postanestheticrecovery unit.The mean age of the patients was 53.5 years (range, 30-84 years). Eleven were men and 5 women. The mean doseof rFVIIa used was 75 µg·kg-1 (range, 60-90 µg·kg-1) and25% of the patients needed a second dose. All the patientshad postoperative bleeding, 62.5% after general surgery,25% after a liver transplant, and 12.5% after a lung transplant.rFVIIa therapy was effective in 66% of the patients andno adverse thrombotic events related to treatment wereobserved.rFVIIa can be an efficacious therapeutic option for bleedingand coagulation disorders that are refractory to conventionalreplacement therapy. Approval to use rFVIIa inthis setting and the establishment of indications should bebased on further research

[Anaphylactic reaction versus carcinoid crisis: the role of octreotide as a vasoconstrictor]. / Reaccin anafiláctica o crisis carcinoide? papel del octreótido como vasopresor.

A 35-year-old woman was scheduled for laparoscopic removal of an ovarian tumor. Intravenous metamizol was administered and when pneumoperitoneum was performed she developed severe hypotension and bronchospasm. Anaphylactic reaction was suspected, all drugs were suspended, and 2 doses of intravenous adrenalin (0.2 and 1 mg) were injected followed by continuous perfusion. Because response was slow and given the unknown origin of the tumor, the possibility of a carcinoid crisis was considered. When an intravenous bolus dose of octreotide was administered, pressure recovered, patient was extubated and could be transferred asymptomatic to the recovery ward. Tests later ruled out carcinoid syndrome, whereas tryptase levels in blood extracted during surgery and allergy tests confirmed an anaphylactic reaction to metamizol. Carcinoid crisis can be difficult to distinguish from anaphylactic reaction because the clinical pictures are similar. Anesthetic management of carcinoid crisis has been facilitated by administration of octreotide. Less is known about the use of octreotide to treat hypotension in patients with autonomic neuropathy based mainly on the drug's ability to produce splanchnic vasoconstriction. This is probably the reason why octreotide resolved our patient's shock in a context of systemic vasodilation caused by the anaphylactic reaction.