ESPE Abstracts (2021) 94 P1-96

ESPE2021 ePoster Category 1 Thyroid A (10 abstracts)

Thyroid storm and transient bulbar myopathy in a 22-month-old girl with Graves’ Disease

Jessica Sandy 1 , Rosemary Brungs 1 , Kristen Neville 2 & John Lawson 2

1Sydney Children’s Hospital Network, Sydney, Australia; 2Sydney Children’s Hospital, Sydney, Australia *J Sandy and R Brungs are co-first authors.

22-month girl presented with 3 days of cough and fevers, 2 weeks of rhinorrhoea and frequent “clearing of throat” and 6 months of diaphoresis and growth spurt. On examination, she was flushed and diaphoretic with a large goitre. She had a low grade fever (T=37.9) with signs of cardiac compromise (bounding pulse sinus tachycardia [HR=200bpm]. She was hypertensive [BP=145/90mmHg] and tachypnoeic [RR=36/min] with respiratory distress and right-sided crepitations. She had a raised JVP but no peripheral oedema or hepatomegaly. Length (98thpercentile) was well above expected (mid-parental height 10-25th percentile). She had a family history of hyperthyroidism and diabetes. Initial investigations: Rapid clinical deterioration (pyrexia, agitation, vomiting, and worsening cardiorespiratory status) occurred, despite commencing carbimazole 0.9mg/kg/day, propranolol 0.6mg/kg/day, and Lugol’s iodine, and resulted in an overnight ICU admission. It was then noticed she was unable to manage oral secretions and had profound proximal myopathy (positive Gowers’ sign). Bulbar myopathic dysphagia was confirmed on modified barium swallow, and was thought to be secondary to thyrotoxic myopathy after exclusion of a neurological event, Myasthenia Gravis or other myopathy and with subsequent resolution. Free T4 normalised after 2 weeks, free T3 by 3 weeks and she was treated with "block-and-replace" therapy from 7 weeks. This is the youngest reported case of thyroid storm and first of bulbar myopathy complicating paediatric thyrotoxicosis. Less than 35 cases of thyroid storm have been reported in children (youngest 2 years). Consequently, evidence guiding diagnosis and management is limited, and published diagnostic criteria for thyroid storm (Burch Wartofsky Point Scale and Japanese Thyroid Association) are for adults with limited utility in the paediatric population. Thyrotoxic myopathy presenting as dysphagia has been described in case reports and series in adults, but not in children. Management includes considering the differential Myasthenia Gravis, which can be associated with Grave’s disease, and treatment of thyrotoxicosis. Consistent with adult literature, where resolution of bulbar myopathy occurred with normalisation of thyroid function from 3 days to 14 weeks, in our case resolution of proximal myopathy took 6 weeks and dysphagia 3.5 months.

Test (reference range)Value
TSH (0.70-5.97mU/l)<0.005
fT4 (12.3-22.8pmol/l)>100
fT3 (3.69-8.46pmol/l)45.5
TSH receptor antibodies (<1.8U/L)>20
TPO antibodies (0-34IU/ml)343
Thyroid ultrasoundDiffuse enlargement, heterogeneous. No nodules.
Chest X-rayMild Cardiomegaly
ECGLeft ventricular hypertrophy, sinus tachycardia
EchocardiogramMild left ventricular hypertrophy

Volume 94

59th Annual ESPE (ESPE 2021 Online)

22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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