Sickle-cell trait. A report of a postoperative neurological complication.

A 12-year-old male Negro was admitted for cryotherapy to both eyes for lattice degeneration. The patient had developed secondary glaucoma followed by optic atrophy in the left eye after a hyphema resulting from an injury. Examination before operation revealed no abnormality except for a systolic murmur. His haemoglobin concentration was 12.3 g/100 ml and the haematocrit was 36.2%. On the day before surgery, 10% phenylephrine and 1% cyclopentolate HC1 (cyclogyl HC1) one drop in each eye, four times daily, were prescribed. Premedication consisting of diazepam 5 mg and atropine 0.4 mg, was given 1 hour before surgery. After preoxvgenation, anaesthesia was induced with thiopentone 175 mg, followed by suxamethonium 60 mg mixed with atropine 0.8 mg and an orotracheal tube was inserted without difficulty. Anaesthesia was maintained with 70% nitrous oxide in oxygen; tubocurarine 25 mg was given i.v. At the end of the procedure the neuromuscular blockade was antagonized with a mixture of neostigmine 3.5 mg and atropine 0.8 mg given i.v. When adequate spontaneous ventilation had returned, the endotracheal tube was removed and the patient was taken to the recovery room. No oxygen was administered during the transfer. On arrival in the recovery room, oxygen was given from a mask. The patient remained unconscious (drowsy) and was given doxapram 50 mg to aid awakening. He responded well and appeared more awake. At this time he was seen to be able to move all his limbs although he did not speak. Eight hours after the end of surgery, the patient was transferred to the intensive care unit and on examination he exhibited a right hemiparesis and aphasia. On the 1st postoperative day the e.e.g. showed a slow wave focus in the left frontal and mid-temporal region. The brain scan was negative. A cardiologist examined the patient and was able to exclude the possibility of a paradoxical embolus. The patient's aunt was questioned about sickle-cell disease and stated that indeed the child had a sickle-cell "trait". The electrophoretic pattern showed 37.2% HbS (in contrast to the normal 100% HbA). On the 2nd postoperative day he was transferred to the ward in essentially the same condition. He developed nuchal rigidity and a lumbar puncture was performed. The cerebrospinal fluid was grossly bloodstained and xanthochromic. The opening and closing pressures were 590 and 270 mm H2O respectively. A left carotid arteriogram performed on the 7th postoperative day revealed: (1) straightening of the intracerebral vessels with a shift of the left anterior cerebral artery to the right and indicating cerebral oedema; (2) vasculitis of intracerebral vessels and; (3) a questionable decreased filling of the anterior portion of the sagittal sinus. A diagnosis of superior sagittal sinus thrombosis was made. After 2 weeks of physical and speech therapy, the patient made a good recovery and was discharged with only minimal weakness of the right upper arm.