ESPE Abstracts

Introduction: Turner’s syndrome (TS) is caused by an abnormality of one of the X chromosomes. Short stature or slow growth is one of the first manifestations of TS and it is recommended that GH therapy should be initiated as soon as it becomes apparent that affected girls are not growing normally to optimise final adult height. Idiopathic intracranial hypertension (IIH) is a well-known side effect of GH therapy, and it has also been reported in girls with TS with or without GH therapy.

Case: A patient with TS aged 3.55 years, height 83 cm (−4.04 SDS) started treatment with GH (Norditropin SimpleXx) 0.5 mg subcutaneously (43 μg/kg per day). Within 2 weeks of the start of treatment she presented to the Emergency Department with headache and vomiting. Ophthalmological review revealed that she had bilateral papilloedema. A lumbar puncture (LP) was performed under general anaesthesia. The opening pressure was 24.5 cm H₂O. GH was stopped, however, she continued to have intermittent headaches but with no vomiting. Neurological examination remained normal. After 3 months; she presented with worsening headache and vomiting. At this time the opening pressure on LP was 33 cm H₂O. She started treatment with acetazolamide with good effect: Opening pressure after 1 week treatment, 24 cm H₂O.

Conclusion: We concluded this patient may have had pre-existing, asymptomatic IIH that became symptomatic when she started GH therapy as IIH worsening despite stopping the GH therapy. We therefore recommend that all patients with TS should have detailed ophthalmological examination to rule out papilloedema before starting GH therapy.