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Xeroderma Pigmentosum: What Anaesthetist Should Know?

机译:Xeroderma pigmentosum:麻醉师应该知道什么?

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A six year old male child, weighing 18 kg was referred to Department of Anasethesia, Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitalfor excision of ulceroproliferative lesion of upper lip. Lesion was 2.5×2 cm in size arising from angle of mouth for last one month (Fig.1). Patient is a known case of Xeroderma Pigmentosum (XP) since one year of age. He had extensive hyperpigmented macular as well as raised skin lesions in sun exposed areas along with diminution of vision in both eyes secondary to corneal opacity and keratomalacia. Physical examination of child was unremarkable. Preoperative blood investigations were within normal limits. Standard preoperative monitors were attached. Intravenous access was secured gently. Patients head, neck, trunk and extremities were covered with linen before turning the operation theatre lights on. Lesion was covered with sterile gauze prior to face mask ventilation to avoid trauma. General anaesthesia was induced with intravenous midazolam 0.5 mg, fentanyl 40 mcg, propofol 40 mg followed by I-gel no. 2 insertion (Fig. 2). Use of muscle relaxant was avoided. Anaesthesia was maintained with O and 2 propofol infusion along with assisted ventilation via circle absorber. Lubricating eye drops were instilled before patching the eyes. Local infiltration of surgical site was done with 0.25% bupivacaine 3 ml. Intraoperative course was uneventful. iGEL was removed after effortless spontaneous breathing. Postoperative analgesia maintained with appropriate dose of diclofenac and tramadol. Paracetamol was avoided as analgesic. Histopathology of lesion confirms basal cell carcinoma of lips.
机译:一名六岁的男孩,重18公斤被提交给Anasethesia,Byramjee Jeejeebhoy政府医学院和Sassoon Germany Healturance切除溃疡性病变的上唇。损伤为2.5×2厘米的尺寸,持续一个月的嘴角引起(图1)。患者是一种已知的Xeroderma Pigmentosum(XP)的情况。他具有广泛的过度沉着的黄斑,以及在阳光暴露区域中提高皮肤病变以及在角膜不透明度和角膜瘤的两只眼中的视觉减少。儿童的体检是不起眼的。术前血液调查在正常限制范围内。标准术前监测器已连接。静脉内接入轻轻固定。在转动操作剧院灯之前,患者头部,颈部,躯干和四肢覆盖。在面部掩模通风之前,病变被无菌纱布覆盖,以避免创伤。用静脉注射咪达唑仑0.5mg,芬太尼40mcg,丙糖醇40mg诱导全身麻醉,然后是I-凝胶NO。 2插入(图2)。避免使用肌肉松弛剂。通过圆形吸收剂与O和2个异丙酚输注保持麻醉和2个异丙酚输注。在修补眼睛之前滴注润滑滴眼液。手术部位的局部浸润是用0.25%Bupivacaine 3mL进行的。术中的术语是不行的。奥格尔在轻松的自发呼吸后被除去。术后镇痛与适当剂量的双氯芬酸和曲马多保持。帕拉基酰胺被避免为镇痛药。病变的组织病理学证实了嘴唇的基础细胞癌。

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