EDITOR:
A 29-yr-old, 100 kg female (body mass index 38) presented for day case bilateral hip injection of steroids. At a preoperative assessment clinic, she had been noted to have moderate learning difficulties (IQ 65), eat a special thickened diet due to ‘swallowing problems’ and have Worster-Drought syndrome (WDS) but the anaesthetic implications of her syndrome had not been appreciated. She had received general anaesthesia as an 11-yr-old, for surgical correction of bilateral slipped femoral epiphyses, which had led to bilateral hip osteoarthritis. Preoperative examination revealed an excess of submandibular soft tissue, Mallampati grade 3 mouth opening and a thyromental distance of 6 cm.
She was adamant in wanting general anaesthesia for the procedure, but given her risk of oesophageal reflux, this would have mandated intubation, and difficult airway equipment (specifically fibre-optic bronchoscopy) was not available at the Independent Sector Treatment Centre to which she had presented. Fortunately, she gave competent consent to sedation, once the nature, purpose, risks and consequences of sedation had been explained to her.
After placement of standard monitoring equipment, a 20-G cannula was inserted into the dorsum of her left hand; 5 L min−1 of supplemental oxygen was administered by face mask. End-tidal carbon dioxide monitoring was used to assess respiratory rate. In all, 50 μg fentanyl, followed by 50 mg boluses of propofol (200 mg in total) were administered to achieve sedation. The patient remained self-ventilating and rousable only to gentle physical stimuli. In all, 5 mL 0.5% bupivacaine mixed with 40 mg methylprednisolone were injected into each hip by a consultant orthopaedic surgeon (using a 24-G Whitacre spinal needle) prior to bilateral hip manipulation. Recovery was uneventful. She was discharged from hospital 3 h after the procedure.
WDS (congenital suprabulbar paresis) is a form of cerebral palsy that was first described in 1956 [Reference Worster-Drought1], occurring as a result of congenital (heterogeneous, predominantly X-linked), bilateral perisylvian cortical dysfunction [Reference Gordon2,Reference Jansen and Andermann3]. It is not an uncommon form of cerebral palsy, but it is under-diagnosed [Reference Clark, Carr, Reilly and Neville4]. WDS displays variable expression, but is characterized by suprabulbar paresis (100% prevalence, causing disordered oesophageal motility, speech difficulty, drooling, gastro-oesophageal reflux (41%) and aspiration), mild four limb pyramidal tetraplegia (91%), cognitive (81%) and behavioural (41%) impairments, and epilepsy (28%).
Surgery may be required in childhood to insert grommets or gastrostomy tubes, or to correct congenital defects such as cleft palate or contractures. A multidisciplinary approach to perioperative care is advocated. Preoperative communication may be difficult, although adult patients with WDS should not be assumed to be incompetent to give consent for treatment [Reference White and Baldwin5]. Preoperative oral clonidine (5 μg kg−1) may be beneficial as a sedative and antisialogogue, and antacid therapy should be considered. Without additional sedation, regional anaesthesia may be compromised by behavioural difficulties. General anaesthesia necessitates tracheal intubation, due to the high risk of gastro-oesophageal reflux; this procedure may be complicated by palatal abnormalities (4%), dental problems (20%), jaw contractures (9%) or micrognathia (1.5–15%). Extubation should be performed with the patient awake, and in the left lateral or semi-recumbent positions.
