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Background: Intracranial hypertension (IH) is a rare but serious complication of growth hormone (GH) therapy. Incidence is roughly 13 per 100,000 cases. Risk factors for GH-induced IH include obesity, chronic renal failure, and Turner Syndrome. Blindness and intractable headache are primary concerns. GH therapy discontinuation is commonly sufficient for resolution of IH, but some patients require additional therapies. Most cases resolve within 12 months of GH discontinuation with favorable outcomes. We describe an unusual case of a very prolonged course of IH after GH therapy.
Case Presentation: A 19-year-old non-obese female developed persistent intracranial hypertension at age eleven within two months of starting GH therapy (0.264 mg/kg/week) for GH deficiency. Initially, she presented with new-onset headaches, pulsatile tinnitus, visual obscurations, and bilateral grade 1 papilledema. Lumbar puncture (LP) revealed an opening pressure of 44 cm H2O, and neuroimaging revealed a congenital hypoplastic transverse sinus. Despite promptly discontinuing GH therapy, symptoms remained persistent eight years later (opening pressure: 29 cm H2O; elevated OCT RNFL measurements: OD 120 um, OS 121 um). Her course was resistant to multiple standard diuretics (acetazolamide, topiramate, furosemide) and she was treated with repeated LPs for symptom relief. Her headache phenotype evolved into chronic migraine (16 days/month, MIDAS=69, severe) with medication overuse headache.
Discussion: Through our literature review of 169 reported cases, we found that symptom onset of GH-induced IH was typically within three months. Growth hormone discontinuation alone was often sufficient for symptom resolution, but up to 43% of patients underwent additional treatments– most commonly with acetazolamide, but interventions also included serial LPs, and, rarely, ventriculoperitoneal shunts. The prognosis is favorable. In those with detailed data, an estimated 95% reported resolution by 6 months and 98% by 12 months. Two cases reported complete resolution at 2 years after discontinuation. At 8 years, our patient’s duration is unusually prolonged. Although CSF pressures have improved over time, this is the longest persistence of GH-induced IH to the best of our knowledge. IH is an uncommon event during GH treatment, but our patient's course demonstrates the potential for long-lasting and debilitating adverse effects. Routine ophthalmological monitoring and a high index of suspicion is essential for early recognition, enabling timely interventions to prevent vision loss and headache disability.