Extradural hematoma surgery in a child with Hutchinson–Gilford progeria syndrome: Perioperative concerns
A 7-year-old male child, known case of HGPS since 1 year of age, developed a right parietal extradural hematoma following a minor fall while playing. He presented with headache and nausea, was conscious but confused, and had no sensory-motor deficit. An emergency craniotomy and hematoma evacuation were planned. Pre-operative evaluation revealed a history of slow growth, progressively appearing features of ageing, normal intellectual development, and recurrent chest infections. The patient had typical manifestations of HGPS-like, a small thin built (height ~95 cm, weight ~11 kg), pinched nose with small nares, depressed nasal bridge, dry stretched-out skin, prominent veins, alopecia, sunken eyes without eyebrows and eyelashes, small pigeon-shaped chest, overall stiff joints, few missing teeth, a 2.5 cm inter-incisor gap, and normal mentation []. The presence of a disproportionately large head, mandibular hypoplasia, micrognathia, and class-3 Mallampati airway score was suggestive of a difficult airway in him. There was no evidence of cardiac, respiratory, or other systemic illness, and all relevant investigations including echocardiography were normal. Before the start of anesthesia, a well-equipped intubation cart and facilities for emergency tracheostomy were readied in anticipation of a difficult tracheal intubation; pediatric fibreoptic bronchoscope was not available to us. Direct laryngoscopy under deep inhalational anesthesia in the spontaneously breathing patient revealed a high, anterior larynx with non-visible glottic opening. After three attempts that utilized various intubation-facilitating maneuvres, it was possible to secure the airway with a small-sized (4.5 mm), uncuffed endotracheal tube with the help of an intubating stylet. The patient's remaining intra-operative course was uneventful; gentle patient positioning and handling during surgery and maintenance of hemodynamic stability were ensured. The child had an unremarkable post-operative period and subsequent hospital stay.