[Anesthesia in Sebastian syndrome: a new hereditary macrothrombocytopenia]. / Anestesia en el síndrome de Sebastian: una nueva macrotrombocitopenia hereditaria.

The macrothrombocytopenias make up a heterogeneous group of disease involving thrombocytopenia and giant platelets; other clinical or laboratory findings, such as hereditary nephritis, sensorineural hearing loss, leukocyte inclusions, and cataracts, may also be present. The tendency to bleeding is highly variable and is due to decreased expression of the GP1b-V-IX complex on the surface of platelets, leading to altered platelet-vessel wall and platelet-platelet interactions. The 5 autosomal dominant giant-platelet disorders that are associated with macrothrombocytopenia are May-Hegglin anormaly, Epstein, Fechtner, and Sebastian syndromes, and Alport-like syndrome with macrothrombocytopenia. The mutation responsible is in gene 9 (MYH9) coding for the nonmuscle myosin heavy chain IIA that has been identified in the long arm of chromosome 22 (22q12.3-q13.2). The most recently described macrothrombocytopenia is Sebastian syndrome, consisting of thrombocytopenia with giant platelets and leukocyte inclusions. We report the case of a woman with Sebastian syndrome scheduled for abdominoperineal resection for rectal carcinoma. Preoperative studies revealed isolated thrombocytopenia (35,000 platelets/microL) and a mean platelet volumen of 13 fL. Preoperative prophylactic platelet transfusion was carried out with no adverse events. After a postoperative transfusion of packed red cells, needed because of abundant bleeding, clinical course continued to be satisfactory. The anesthetic implications of this syndrome are not well known because few cases have been reported in the literature, and none was found that describes anesthetic management. Nevertheless, thrombocytopenia and the tendency to bleeding present challenges to the anesthesiologist.

Anestesia en el síndrome de Sebastian: una nueva macrotrombocitopenia hereditaria / Anesthesia in Sebastian syndrome, a new hereditary macrothrombocytopenia

Las macrotrombocitopenias son un grupo heterogéneo de enfermedades con trombocitopenia y plaquetas gigantes que pueden incluir otros hallazgos como nefritis hereditaria, sordera neurosensorial, inclusiones leucocitarias y cataratas. Su tendencia al sangrado es muy variable, por una disminución de los niveles de la expresión del complejo GPIb-V-IX en la superficie de las plaquetas que implica una alteración de la adhesión y de la agregación plaquetaria. Los cinco desórdenes plaquetarios, autosómicos dominantes que asocian macrotrombocitopenia son los síndromes de May-Hegglin, Epstein, Alport-like con macrotrombocitopenia, Fechtner y Sebastian. La mutación genética causante se localiza en el gen 9 (MYH9) para la cadena pesada de la miosina no muscular IIA, NMMHC-IIA, que se ha identificado en el brazo largo del cromosoma 22 (22q12.3-q13.2). La macrotrombocitopenia más recientemente descrita es el síndrome de Sebastian, que consiste en una trombocitopenia con plaquetas gigantes e inclusiones leucocitarias. Describimos el caso de una paciente con síndrome de Sebastian programada para amputación abdominoperineal por neoplasia de recto. En el preoperatorio se objetivó una trombocitopenia aislada (35.000 plaquetas/µl) con volumen plaquetario medio de 13 fl. Se realizó transfusión profiláctica de plaquetas antes de la intervención que transcurrió sin incidencias. En el postoperatorio requirió la transfusión de concentrados de hematíes por sangrado abundante y la evolución fue favorable. Las implicaciones anestésicas son poco conocidas, por el escaso número de casos descritos y no se ha encontrado bibliografía sobre su manejo anestésico, pero la trombocitopenia y la tendencia al sangrado son un reto para el anestesiólogo (AU)

[Constraints on the use of a nerve stimulator in peripheral conduction blocks when there is disturbance in the neuromuscular junction]. / Limitación del uso del neuroestimulador en la realización de bloqueos de conducción periféricos en alteraciones de la unidad neuromuscular.

An 81-year-old woman with right lower limb ischemia was scheduled for revascularization of a femoropopliteal bypass grafted 10 years earlier. A popliteal blockade, attempted as part of regional anesthesia with the aid of a nerve stimulator, was not achieved because the posterior tibial nerve could not be located. After surgery, the patient mentioned symptoms in the region of the right knee consistent with complex regional pain syndrome (Ducke's stage 3); the symptoms appeared after the first operation and would explain the absence of response to the nerve stimulator. Using a nerve stimulator to facilitate location of the various nerve trunks for anesthesia involves obtaining a motor response to electrical stimulation. The procedure is becoming more and more frequent because of its many advantages over other more traditional methods. However, it may be impossible to locate a nerve for a variety of reasons.

Limitación del uso del neuroestimulador en la realización de bloqueos de conducción periféricos en alteraciones de la unidad neuromuscular / Constraints on using a nerve stimulator for peripheral blocks when the neuromuscular bundle is altered

Una paciente de 81 años con isquemia de la extremidad inferior derecha fue programada para una revascularización de una derivación femoropoplítea realizada 10 años antes. Se intentó la realización de un bloqueo poplíteo, como componente de la anestesia regional, con la ayuda de un neuroestimulador pero no se pudo lograr localizar por este método el nervio tibial posterior. Posteriormente a la intervención, la paciente refirió un cuadro compatible con síndrome de dolor complejo regional en estadio 3 de Duke en la región de la rodilla derecha, que se inició posteriormente a la primera intervención, y que explicaba la ausencia de respuesta a la neuroestimulación.El uso del neuroestimulador para facilitar la localización de los diferentes troncos nerviosos en anestesia troncular se basa en la obtención de una respuesta motora tras su estimulación eléctrica. Este procedimiento es cada vez más usado por sus numerosas ventajas sobre otros métodos más tradicionales. Sin embargo, hay diferentes causas que pueden impedir la localización de un tronco nervioso mediante el uso de este aparato (AU)

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[The utility of digital infiltration of mepivacaine and ketorolac in postoperative analgesia of the unilateral hallux valgus]. / Utilidad de la infiltración digital con mepivacaína y ketorolaco en la analgesia postoperatoria del hallux valgus unilateral.

OBJECTIVES: To determine whether locally injected ketorolac provides analgesia additional to that of mepivacaine, and also to prevent, diminish or delay the peripheral hypersensitivity response of postoperative pain. PATIENTS AND METHODS: Prospective, randomized, double-blind study of 72 patients scheduled for surgery to correct unilateral hallux valgus. Group 1 (n = 24) received median infiltration at the first metatarsus of 5 ml of 2% mepivacaine and 1 ml (30 mg) of ketorolac. Group 2 (n = 21) received local infiltration of 5 ml of 2% mepivacaine and 1 ml of saline solution. Group 3, the control group (n = 27) received the same solution as did group 2, plus 30 mg of ketorolac intravenously. The postoperative analgesia prescribed was 10 mg of ketorolac orally every 8 hours. Pain was measured on a visual analog scale (VAS) 0, 1, 4, 8 and 24 hours after surgery. Time elapsed until the appearance of pain, number of ketorolac pills consumed and overall patient satisfaction were recorded. RESULTS: There were no differences in anthropometric characteristics. Time until pain appeared was significantly longer in group 1 than in groups 2 and 3 (14.66 +/- 7.19, 5.90 +/- 2.27 and 8.70 +/- 5.02 hours, respectively). The VAS scores were significantly lower in group 1 after the fourth postoperative hour. Analgesic consumption was significantly lower in group 1. CONCLUSIONS: Infiltration of 30 mg of ketorolac along with mepivacaine delays the appearance of postoperative pain and diminishes it in the first 24 hours after surgery to correct hallux valgus, in comparison with infiltration of mepivacaine alone plus intravenous ketorolac.