Background: Arteriovenous malformations (AVMs) are rare in kids, estimated to represent 3% of all AVMs. They tend to rupture more frequently than in adults and, usually, are brought to attention after rupture, as the most common non-traumatic intracerebral hemorrhage. AVMs could also present with recurrent seizures or headaches. Their optimal management remains controversial.
Case report: We present the case of a 4.5 y old boy, presented in our department because of growth deficit. The history of the child revealed he was a healthy newborn, with normal parameters at birth, has grown up normally, until the age of 18 months, when he has experienced an episode of seizure. He was hospitalized in a pediatric department and the complete evaluation revealed mild hypoglycemia as the cause of seizure. The evaluation included serum IGF1, GH, cortisol, insulinemia, oral glucose test (OGTT), revealing normal levels. Thus, hypoglycemia was thought to have occurred in the absence of a proper meal schedule. He was recommended an appropriate for age meal plan and followed up for the next 6 months, as later, the parents did not show-up for the scheduled check-ups. At the age of 4,5 y the parents addressed the child to our department because of a slow growth, poor weight gain. Evaluation showed height deficit SDSheight=−3, weight deficit (Weight<3rd percentile), no particular clinical features. Investigations revealed retarded bone age, GH deficit (hypoglycemia, low IGF1, low stimulated GH) confirming GHD. MRI exam of the head revealed complex arteriovenous malformation involving the left carotid artery and the Willis polygon. The optic chiasma was dislocated anteriorly and the pituitary gland and stalk difficult to identify, possibly also dislocated by the AVM located in and above the sella turcica. The child was scheduled for surgical intervention, results are to be communicated subsequently.
Discussions: GHD is frequently encountered in any process that compresses the sella and pituitary gland. The particular evolution of this case lies in the lack of clinical signs suggestive either for compression by the AVM (headache, vomiting) or for GHD/other pituitary deficits, except for an isolated hypoglycemia associated with seizures. Therefore, the diagnosis was delayed and confirmed just when the growth deficit was evident.
Conclusion: Hypoglycemia requires complete evaluation for pituitary deficits, including GH stimulation tests and imaging of the pituitary and sella, in order to exclude hypopituitarism that may be due to a process exerting compression in this area.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology