RESUMEN
Macrocephaly is defined as an abnormal increase in head circumference greater than two standard deviations above the mean for a given age and sex. We present the case of a 16-month-old boy with congenital progressive macrocephaly, who was referred to our hospital for a ventriculoperitoneal shunt placement for external hydrocephalus diagnosed at 13 months of age. The patient had a febrile seizure 12 hours after the shunt was placed and the emergency CT exam revealed collapsed ventricles and a right frontal subdural collection, suggestive of an over-drainage and intracranial hypotension. A subsequent electroencephalogram (EEG) revealed some anomalies, but the patient was discharged two days later due to having no neurological symptoms after being placed on anticonvulsants. The patient returned to the hospital one week later due to recurrent seizures. Further clinical examination revealed prominent and tortuous veins of the skull, palpated in the left occipital region. A thrill and a left carotid murmur were heard during auscultation. A subsequent brain MRI with MR arteriography and venography was performed in search of an explanation for hydrocephaly. The sequences were suggestive of a dural arteriovenous fistula, which was confirmed and then treated using coils during an interventional angiography. A second procedure was performed two months later to complete the embolization, with subsequent imaging follow-ups showing the procedure to have been successful. The measurement of the cranial circumference, its regular evaluation, and its evolution allow a hierarchical diagnosis strategy by distinguishing primary and secondary macrocephaly, progressive or not. Dural arteriovenous fistulas (DAVF) are an under-appreciated cause of macrocephaly, with which they are associated in 35% of cases. Intracranial DAVFs are pathologic shunts between dural arteries and dural venous sinuses, meningeal veins, or cortical veins. Patients with DAVFs may be completely asymptomatic. Symptoms, when present, may range from neurological deficits, seizures, and hydrocephaly to fatal hemorrhage. The symptoms depend on the location and venous and drainage patterns of the DAVF. They can be difficult to identify on routine MRIs unless specifically searched for, especially in cases of technically suboptimal examinations. We aim to give a practical approach to identify the clinical clues that warrant further investigation. Several specific protocols exist regarding the management of macrocephaly and should be followed carefully once a diagnosis has been reached, but further studies are needed to integrate more clinical and neuroimaging findings to permit an early diagnosis.
RESUMEN
BACKGROUND AND PURPOSE: Cerebral vasospasm (CV) following aneurysmal subarachnoid hemorrhage (aSAH) may lead to morbidity and mortality. Endovascular mechanical angioplasty may be performed if symptomatic CV is refractory to noninvasive medical management. Off-label compliant remodelling balloons tend to conform to the course of the vessel, contrary to noncompliant or semi-compliant balloons. Our objective is to describe our initial experience with the semi-compliant Neurospeed balloon (approved for intracranial stenosis) in cerebral vasospasm treatment following aSAH. METHODS: All patients included in the prospective observational SAVEBRAIN PWI (NCT05276934 on clinicaltrial.gov) study who underwent cerebral angioplasty using the Neurospeed balloon for the treatment of medically refractory and symptomatic CV after aSAH were identified. Patient demographic information, procedural details and outcomes were obtained from electronic medical records. RESULTS: Between February 2022 and June 2023, 8 consecutive patients underwent CV treatment with the Neurospeed balloon. Angioplasty of 48 arterial segments (supraclinoid internal carotid artery, A1 and A2 segments of the anterior cerebral artery, M1 and M2 segments of the middle cerebral artery) was attempted and 44/48 (92%) were performed. The vessel diameter significantly improved following angioplasty (+81%), while brain hypoperfusion decreased (-81% of the mean TMax). There was no long-term clinical complication, 4% periprocedural complications occurred. CONCLUSION: The semi-compliant Neurospeed balloon is effective in the treatment of cerebral vasospasm following aSAH, bringing a new device into the armamentarium of the neurointerventionalist to perform intracranial angioplasty.