Journal of the Endocrine Society
Oxford University Press
SAT-088 The Incidence of Neurological Symptoms During Growth Hormone Therapy in Patients with Chiari Malformation
Volume: 4, Issue: Suppl 1
DOI 10.1210/jendso/bvaa046.086




Background: Patients (PTs) with Chiari malformation (CM) are prone to a wide variety of neurologic symptoms (SX), including headaches (HA), vision abnormalities, and nausea. These SX are attributed to impaired flow of CSF leading to benign intracranial hypertension (BIH). Occasionally, PTs with CM may require growth hormone therapy (GHT). This can potentially increase CSF accumulation and risk of BIH. The literature is limited to a small number of case reports on GHT in PTs with CM. Here, we describe the incidence of neurologic SX in 15 PTs.

Methods: Our database was queried for PTs with CM who were treated with GHT from 2010–18 and records were reviewed for adverse events. PTs with neoplastic disease, active inflammation, or acute trauma were excluded. CM was defined as cerebellar tonsils located below the foramen magnum on MRI.

Results: Mean and median ages of the 15 PTs (10 male, 5 female) who met inclusion criteria were 15.3 and 11.7 years, respectively. 14 were diagnosed as Type 1 and 1 was diagnosed as Type 2 CM. Tonsillar displacement ranged from 2-21mm, but was not specified in 5 PTs. Indications for GHT included isolated GH deficiency, panhypopituitarism, and chronic renal disease. Duration of GHT ranged from 0.2 to 12.25 years with a mean and median of 3.7 and 1.75 years, respectively. 7% (1 of 15) PTs experienced new-onset SX of BIH that could be attributed to GHT.

8 PTs (53.3%) did not experience any SX consistent with CM before, during, or after GHT. 3 PTs (20%) experienced neurologic SX prior to GHT. 1 PT reported diplopia and abnormal sleep patterns prior to GHT that resolved and did not recur during GHT. 1 PT prior to GHT manifested papilledema, 1 seizure, central sleep apnea, and occipital HA that resolved after posterior fossa decompression. Post-operatively and during GHT, this PT developed and continued to manifest nonpathologic pseudopapilledema. 1 PT continued to have pre-existing seizures and insomnia that did not worsen with GHT. 1 PT (7%) had congenital neurologic abnormalities in addition to CM. This PT had surgery to alleviate BIH caused by congenital hydrocephalus and SX permanently resolved. GHT has been continuous since birth with no new manifestations of CM reported post-operatively. 2 PTs (13%) developed new-onset neurologic SX while on GHT. 1 PT with diabetes experienced HA, 1 report of loss of consciousness, and 1 instance of apnea during periods of hyperglycemia. It was determined that these SX were unrelated to BIH and GHT was not interrupted. 1 PT experienced mild HA and 1 episode of occipital pounding with emesis during GHT. These SX resolved without intervention and GHT was continued without interruption. Despite the complexity of these cases, 0 PTs discontinued GHT.

Conclusion: Our study demonstrates that in a majority (93%) of cases, GHT does not cause onset or worsening of SX of BIH in PTs with complicated and uncomplicated CM. GHT should be regarded as a safe treatment in these PTs.

Alexandrov, Buysse, Patale, McGuirk, Gold, Krasnow, Haigney, and Noto: SAT-088 The Incidence of Neurological Symptoms During Growth Hormone Therapy in Patients with Chiari Malformation The Incidence of Neurological Symptoms During Growth Hormone Therapy in Patients with Chiari Malformation&author=Alice Alexandrov,Tavia Buysse,Tara Patale,Liam McGuirk,Steven Gold,Nicholas Andrew Krasnow,James Haigney,Richard A Noto,&keyword=&subject=Pediatric Endocrinology,Pediatric Growth and Adrenal Disorders,AcademicSubjects/MED00250,