A 15 year-old girl presented with acute psychosis, seizures and right shoulder dislocation with an overlying abscess. The symptomatology was diagnosed to be due to Wilson's disease. The anaesthetic management and postoperative analgesia was a challenging task in presence of hepatic and acute neurological dysfunction as well as difficulty in pain assessment. There are only a few reported cases of anaesthetic management in patients with Wilson's disease with severe neuropsychiatric symptoms. This case portrays the safe use of both general and regional anaesthesia in such a patient.
Keywords:Wilson's disease; neuro-psychiatric symptoms; anaesthesia; interscalene block Case Report A 15yr old, 30kg girl presented with progressive slurring of speech, gait disturbance, insomnia and altered behaviour over a period of 20 days. She was admitted to a psychiatric hospital and started on risperidone and carbamazepine. A week later, she developed tightness of all four limbs and focal seizures. Quetiapine and thioridazine were added for better symptom control. She was then transferred to Kasturba Hospital, Manipal. There was no history of trauma, chronic fever or drug abuse. Perinatal period and development were within normal limits. There were no psychiatric/ genetic disorders or untimely deaths in her family. On admission, child had resting tremors, choreoathetoid movements, hypertonia of all four limbs and recurrent right sided focal seizures. Later, child was noted to have fluctuating consciousness, inattention and inappropriate laughter and crying. Examination revealed a swollen and dislocated right shoulder. A provisional diagnosis of encephalopathy was made and further evaluation was performed. Biochemical tests revealed increased serum copper (209mcg/dL) and urine copper levels (373mcg/24h) and mildly elevated liver enzymes in serum (Aspartate Transaminase-75 IU/L, Alanine Transaminase-109 IU/L, Alkaline phosphatase-87 U/L). Computed tomographic scan of brain and cerebro-spinal fluid studies were normal. Toxicology and metabolic screening tests ruled out toxins and inborn errors of metabolism. Steroids and penicillamine were started with a presumptive diagnosis of Wilson's disease.Trihexiphenidyl, quetiapine and levetiracetam were used to control her symptoms. A week after therapy, the swelling over the right shoulder was diagnosed to be a developing abscess. Orthopaedic surgeon scheduled her for closed/open reduction of the dislocation with drainage of abscess. Written informed consent was obtained from the parents for surgery and anaesthesia, after explaining the risk of perioperative adverse events.Our initial plan was to provide an interscalene block. However, due to the fluctuating consciousness and recurrent choreo-athetoid movements, we proceeded with general anaesthesia. Electrocardiogram, non-invasive blood pressure and pulse oximetry was established and a 20G venous cannula secured and anaesthesia induced with fentanyl 60mcg and propofol 40mg. Atracurium 15mg was used to facilitate tracheal intubation with 6....
Heat and moisture exchange filters (HMEFs) have been a controversial yet widely used adjunct to breathing circuit. Here we discuss a case scenario where clogging of HMEF with moisture presented as elevated peak airway pressures. Apart from stressing the importance of monitoring airway pressure, we discuss about the judicious use of HMEFs, problems associated with their use and measures to overcome the same. Being a ubiquitous adjunct, a thorough knowledge of its functioning and constant vigilance during its use is of paramount importance
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