Abstract:Background: The link between the effects of recombinant human growth hormone (rhGH) therapy in patient with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is controversial.
Summary: We report the case of a patient with an unusual association of growth hormone deficiency (GHD) due to ectopic posterior pituitary and Chiari malformation type I (CM-1). Our patient developed headache and worsening of CM-1 after initiation of rhGH therapy. Following an atlo-occipital decompression surgery, th… Show more
Objectives
Patients with Chiari malformation (CM) are prone to a variety of neurological sequelae, including benign intracranial hypertension (BIH). In these patients, BIH is attributed to impaired cerebrospinal fluid (CSF) flow due to anatomical abnormalities of the posterior fossa. Occasionally, patients with CM may require growth hormone therapy (GHT), which can increase the production of CSF. It is thought that patients with CM who undergo GHT are at high risk of BIH-associated symptoms (BIHAS). We describe the incidence of neurological symptoms in 34 patients with CM before and during GHT.
Methods
The database of a pediatric endocrinology center was queried for patients with CM who received GHT from 2010–22. Records were reviewed for adverse events. Demographic and radiological data were collected and analyzed. Patients with neoplastic disease, active inflammation, or acute trauma were excluded. CM diagnoses were independently assigned by a neuroradiology department. Patients were grouped based on the presence and nature of symptoms before and during GHT. Relationships between starting dose/BMI and occurrence of BIHAS/all GHT-associated symptoms were evaluated.
Results
GHT was not associated with new-onset or worsening of preexisting BIHAS in 33 out of 34 patients with CM. Five complex patients continued to have preexisting BIHAS, which did not worsen. Of the four patients who developed new-onset BIHAS during GHT, three patients’ symptoms were attributed to other medical conditions. No patient permanently discontinued GHT due to BIHAS.
Conclusions
Growth hormone therapy is likely a safe treatment in patients with Chiari malformation and is unlikely to cause BIHAS.
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