A 52-year old, White man (height of 1.72 m, bodyweight of 78 kg) showed signs of limping in the right leg beginning in January 1998. No neurologic disorders had been previously diagnosed in the family. The patient had experienced a neck and occiput injury (struck with a stone) about 20 years earlier, but this was resolved without complications. Information on smoking was unavailable, and the patient occasionally consumed small amounts of alcohol.
Initially, the Neurology Department suspected a motor neuron disease and, due to initial uncertainties with the diagnosis, the patient was consulted at a national reference centre for motor neuron disease, where the diagnosis of ALS was ultimately confirmed 15 months after disease onset. Riluzole (Rilutek, Aventis Pharma, France) was introduced as the sole medication.
Twelve months after the disease onset the patient was forced to begin using a wheel chair, and, after another eight months, hypoventilation developed. The patient became dyspneic and was directed to the Infectious Diseases Department Intensive Care Unit to consider the option of chronic mechanical ventilation support. Following admission to the hospital, the patient was extensively informed about the benefits and disadvantages of chronic mechanical ventilation support, and a written consent was obtained. Tracheal intubation was performed, followed by a tracheostomy and introduction of chronic mechanical ventilation support with a Nellcor Puritan-Bennet 740 ventilator. The progression of the disease could be clearly observed over the course of the next six years. The upper and lower extremities became totally paralyzed, and the bulbar nerves became afflicted – the patient lost the ability to move, swallow, and whisper. With time, communication with the patient became possible only with the use of an alphabet table or a text editor supported by a photomechanically controlled keyboard linked to a computer. The first signs of cataracts appeared in the summer of 2005. Deterioration of eyelid winking occurred simultaneously. The rapid maturation of the cataract resulted in a nearly complete loss of communication with the patient – he was unable to use either the electronic writing device or the alphabet table. A decision regarding surgical treatment of the cataract was made by a council consisting of the engaged physicians and the consulting ophthalmologists.
The first phacoemulsification of the right eye took place on November 17, 2005. Preparation for this operation included the insertion of a central venous catheter via the left subclavian vein, and insertion of a urinary catheter. Before entering the operation room (OR), the patient received premedication consisting of pethidine (1 mg·kg-1) and midazolam (0.1 mg·kg-1) applied intramuscularly. ECG monitoring, non-invasive arterial blood pressure, pulsoximetry, and capnometry were carried out in the OR. General anaesthesia was introduced by a bolus injection of propofol (1 mg·kg-1), and was supported by volatile anaesthesia consisting of 65 vol% N2O, 0.7 vol% sevoflurane, and oxygen administered by a volume controlled anaesthesia ventilator through the tracheostomy tube, using a circle circuit with a CO2 absorber. Furthermore, the operating surgeon anaesthetized the eye by topical administration of proxymetacaine 0.5% eye drops. The ophthalmologic procedure lasted 25 minutes, without any complications. Following the operation, delivery of the volatile anaesthetics was ceased and the patient was transported back to the recovery room. He became responsive to alerts 20 minutes after discontinuation of the volatile anaesthetics. The total anaesthesia handling in the OR lasted 40 min, and the stay in the recovery room lasted 3 hours.
The outcome from the first operation was complicated by development of an endophthalmitis with choroids inflammation, due to a methicillin-resistant Staphylococcus epidermidis strain. Although the infection was successfully treated with topical and systemic antibiotics, corticosteroids, and non-steroid anti-inflammatory drugs, residual postinflammatory membranes impaired the vision and necessitated further surgical treatment. The second operation, consisting of the phacoemulsification of the left eye and a vitrectomy due to the endophthalmitis of the right eye, took place on December 20, 2005. The preparation for the operation was similar to the first one. Again, general anaesthesia was introduced by an intravenous bolus of propofol and sustained by volatile anaesthesia. For the most painful stages of the operation (vitrectomy), an additional bolus of propofol and fentanyl was administered. The ophthalmologic procedures lasted 100 min., the anaesthesiologic – 130 min. The patient stayed again for two hours in the recovery room before he was transported back to his room.
The treatment and rehabilitation after the second operation took about three months, but resulted in substantial improvement in vision and the maintenance of satisfactory communication with the patient. Two years after the ophthalmologic interventions the patient remained in a relatively stable condition, with communication still intact.