Strabismus as a possible sign of subclinical muscular dystrophy predisposing to rhabdomyolysis and myoglobinuria: a study of an affected family

Abstract

Administration of succinylcholine to normal individuals results in alterations in muscle membrane integrity expressed as a slight increase in the concentrations of creatine phosphokinase (CK) in serum and appearance of small amounts of myoglobin in the urine, but without clinical symptoms. Subjects with strabismus due to congenital muscular dystrophy may develop more significant rhabdomyolysis expressed as muscle stiffness and weakness, massive myoglobinuria, marked elevation of serum CK and other enzymes, metabolic acidosis, tachycardia and moderate elevation of body temperature. In some cases grave malignant hyperthermia with significant hypoxia, metabolic acidosis, tachycardia and marked abnormalities in serum electrolyte concentrations may cause irreversible damage to the central nervous system and other vital organs and death.A case of difficult anaesthesia for a six year old boy belonging to family affected with muscular dystrophy is presented. More attention must be given to preoperative exarmnation (anamnesis, serum enzymes) of ophthalmological patients and more careful monitoring during anaesthesia and in the early postoperative period must be instituted to prevent and treat complications induced by suecinylcholine and volatile anaesthetic agents.KEY WORDS: COMVLICArtONS, muscular dystrophy, strabismus, rhabdomyolysis, myoglobinuria, malignant hyperthermia.A PREVIOUSLY HEALTHY six year old boy was presented for correction of divergent strabismus of the right eye. The child had been anaesthetized uneventfully six months earlier for the same operation on his left eye. He had no allergies and was considered a minor anaesthetic risk (group 1). On the morning of the operation his blood pressure, pulse, body temperature, haemoglobin and blood leueocytes were normal. Forty-five minutes before anaesthesia, the patient received meperidine 17.5 mg and atropine 0.25rag intramuscularly (weight 17.5 kg). The patient arrived in the operating room well sedated and cooperative. Ten minutes before the start of the anaesthetic methohexitone 120rag was given by rectum. Anaesthesia was started by a junior member of staff with nitrous oxide and oxygen, four litres per minute of each, and halothane 0.5-2.0 per cent given by mask from a Jaekson-Rees system. After the patient had reached the surgical level of anaesthesia, suecinyleholine chloride 25 mg was injected intravenously. The muscle fasciculation was unusually strong and tracheal intubation could not be

Sodium Nitroprusside, Pregnancy and Multiple Intracranial Aneurysms

“…Three of the six patients who developed renal failure had clues to muscle disease, namely preoperative elevation of creatinine kinase, 10 history of painful swelling of the thighs 6 and family history of Duchenne muscular dystrophy.9 Subclinical muscle disease possibly predisposing to rhabdomyolysis should be suspected in patients with squints or scoliosis. 21 The relationship of malignant hyperpyrexia to suxamethonium-induced rhabdomyolysis is unclear. It has been suggested that this condition is a 'forme fruste' of malignant hyperpyrexia, in which rhabdomyolysis also occurs.…”

Masseter Muscle Spasm in Children

“…Even leaving aside the controversial issue of the reported high percentage of biopsies positive for MH after MMS, the clinical literature contains many case reports of fulminant MH, sometimes leading to death, if inhalation anesthetic agents are continued after MMS (4)(5)(6)(7)(8). In at least four of the patients presented by Littleford et al, dantrolene was used and the anesthetic stopped.…”

Anaesthesia and Progressive Muscular Dystrophy

“…Suxamethonium is known to release potassium from cells, increase serum CPK concentrations (Tammisto and Airaksinen, 1966) and cause myoglobinuria (MacLaren, 1968) in normal patients, including children. Halothane potentiates the enzyme-releasing effects of suxamethonium, but may also produce this result to a lesser degree when administered as a sole agent to normal patients (Lewandowski, 1982). The potassium-and enzyme-releasing effects are potentiated in patients with Duchenne's PMD and in patients with subclinical myopathy, as demonstrated by increased resting CPK and lactic dehydrogenase (LD) concentrations in serum (Lewandowski, 1981).…”

“…Halothane potentiates the enzyme-releasing effects of suxamethonium, but may also produce this result to a lesser degree when administered as a sole agent to normal patients (Lewandowski, 1982). The potassium-and enzyme-releasing effects are potentiated in patients with Duchenne's PMD and in patients with subclinical myopathy, as demonstrated by increased resting CPK and lactic dehydrogenase (LD) concentrations in serum (Lewandowski, 1981). Lewandowski (1982) described a boy with normal resting serum CPK concentration and increased serum LD concentrations who developed rhabdomyolysis, myoglobinuria, acidosis, tachycardia and hyperpyrexia following halothane and suxamethonium.…”

“…The potassium-and enzyme-releasing effects are potentiated in patients with Duchenne's PMD and in patients with subclinical myopathy, as demonstrated by increased resting CPK and lactic dehydrogenase (LD) concentrations in serum (Lewandowski, 1981). Lewandowski (1982) described a boy with normal resting serum CPK concentration and increased serum LD concentrations who developed rhabdomyolysis, myoglobinuria, acidosis, tachycardia and hyperpyrexia following halothane and suxamethonium. Both the patient and a brother had strabismus and a cousin suffered from severe Duchenne's PMD.…”