A child with xeroderma pigmentosum for excision of basal cell carcinoma
A female child of 9 years old, diagnosed to have xeroderma pigmentosum 6 years back, presented with wound over the tip of the nose and watering of the eyes on exposure to sunlight. On examination, the child was conscious, cooperative, poorly built, and nourished, presented with generalized bilateral distributed freckles, and hypo pigmented nodules seen over face, trunk, and extremities. Ulcerated nodule of 2 × 2 cm2 with crusting seen over the tip of the nose. Hypo-pigmented macules were present over lips. Conjunctiva was congested, and the cornea was hazy. Immature cataract and entropion were present in left eye []. Investigations were within normal limits. Chest X-ray was normal.\nGeneral anaesthesia was planned for surgery. After taking consent IV line was cannulated on hand. Preoxygenation was done by holding the mask at a distance in the face. The child was pre-medicated with atropine 0.02 mg/kg, fentanyl 0.5 ug/kg, and midazolam 0.5 mg/kg intravenously. Once the child was induced with propofol 2 mg/kg, then trachea was intubated with 5.5 no cuffed oral endotracheal tube after suxamethonium 2 mg/kg IV in a single attempt. Anaesthesia was maintained with O2, N2O, and inj atracurium 0.3 mg/kg by controlled ventilation using Bains circuit. Local infiltration of surgical site was done with inj bupivacaine 0.5% 3 ml. Intra operative monitoring was done with SPO2, NIBP, and ECG. Intra operative course was uneventful. Neuromuscular block was reversed with reversal agents and trachea extubated at the end of surgery.