Phenytoin induced life threatening macroglossia in a child
An 8-year-old boy, previously diagnosed as cerebral palsy with developmental delay, microcephaly and mental retardation, presented with sudden onset of huge swelling of the tongue for last 24 hours. Two weeks back, he developed generalized tonic clonic convulsion with status epilepticus for the first time. He was then admitted to a nursing home and managed with intravenous diazepam followed by loading dose of phenytoin as per standard protocol. He was discharged with instruction to get intravenous phenytoin (300 mg/day) once daily at home for the next 15 days. There was no history of headache or vomiting. There was no history of respiratory distress, swelling of face, itching, and loose stool. No history of atopy or similar type of episodes seen in other family members.\nOn examination, the child was conscious but irritable. There was swelling of whole dorsum of tongue, which was protruding outside the mouth with oozing of blood over both the edges of tongue following superadded infection []. The oral cavity was so blocked by edematous tongue that the child could not speak or swallow anything. Face, lips, and gums were normal. His body weight was 24.5 kg, height was 123 cm and head circumference was 45.5 cm. His pulse rate was 144/min, regular, normal volume, respiratory rate 32/min, blood pressure 120/70 mm of Hg, temperature 99.8°F. The child had mild pallor without any pedal edema or cyanosis. Abdomen was soft without any visceromegaly. There was mild respiratory distress due to oro-pharyngeal obstruction. Auscultation of his chest including cardiovascular system was otherwise normal. There was no ataxia, nystagmus, or altered sensorium. However, he had neurologic manifestation of spastic diplegic cerebral palsy with associated complication.\nInvestigation revealed hemoglobin of 12.8 g/dL. Total leukocyte count of 16900/cmm, polymorphs 86%, lymphocytes 12%, platelet counts 2.2 lakhs/cmm, ESR 22/1st hour. Biochemistry showed serum sodium 138 meq/L, potassium 5.2 meq/L, calcium 8.6 mg/dL. Renal function and liver function tests were normal. Chest X-ray and sonography of abdomen did not reveal any abnormality.\nSerum C1 esterase inhibitor was 0.62 mg/L (normal: 0.21-0.39 mg/L), which excludes hereditary angioedema. Serum phenytoin level (37.20 μg/ml, toxic range > 20 μg/ml) was elevated.\nThe child responded to supportive management along with parenteral empirical antibiotics and with intravenous steroids (hydrocortisone), antihistamines, and epinephrine within 2 days. Macroglossia due to edema was reduced and the tongue could be repositioned within the oral cavity []. He was discharged with valproate sodium and other advice like physiatric management, etc.