Journal of Vasculitis

Primary angiitis of the CNS (PACNS) is a distinct form of cerebral vasculitis, the former being confined to the central nervous system (CNS) with no systemic involvement. Diagnosing PACNS is challenging due to its varied presentation and rarity. We describe a case highly suggestive of PACNS which despite extensive investigation could not be confirmed even on histology but responded to immunosuppression. We present a 69-year old man who presented to the emergency department (ED) with acute confusion. Initial magnetic resonance (MR) imaging of the head had shown ischaemic infarcts and haemorrhages. Cerebrospinal fluid (CSF) analysis and serological work-up had ruled out a systemic vasculitis and an infectious disease to account for the presentation. Computerised tomography (CT) angiography nor digital subtraction angiography had reported any evidence of vasculopathy. The brain biopsy had failed to show features of vasculitis. Although the diagnosis of PACNS was not definitive, immunotherapy was commenced. A significant cognitive improvement was noted and correlated by resolution of the initial imaging changes.

Background: The PDA defines the pathological persistence after the birth of a fetal physiological communication between the aorta and the pulmonary artery frequently encountered in preterm infants and whose clinical and hemodynamic consequences depend on the importance of the shunt directly bound to the diameter of the canal. Percutaneous closure is the most frequent management modality with excellent immediate and long-term results (two modes of closure: using coil or Occluder). The surgery that remains reserved for complex anatomies or associated with other surgical congenital anomalies.

Case presentation: We detail in this document the two methods of percutaneous closure step by step illustrated by pediatric cases. The first case concerns a 7 years old girl of 17Kg weight with history of heart murmur that presented in the TTE a PDA estimated at 1mm with LV dilation. The second case concerns a 12 years old girl of 30 kg weight with also history of heart murmur that presented on TTE a PDA of 4.5mm with LV dilation.

Therapeutic intervention: In the first case, we perform a closure with coil 5/5 by a unique femoral arterial approach as a standardized attitude in our center avoiding an additional venous access. For the second case, we opted for a closure with prosthesis N° 6/8 by a double femoral approach (arterial and venous access).

Outcomes: The follow-up was favorable for both patients, with total sealing of the defect immediately after the procedures that persist on the 6 months control.

Conclusions: The closure of PDA in children is a challenging procedure whose safety requires a good pre- and perprocedural evaluation allowing the right choice of the method and size of the closing device. The respect of the different closure stages and the critical perprocedural ultrasound and angiographic control reduce the rate of complications making this technique accessible and safe. In our series of 108 PDA closures by Coil in children the unique femoral arterial approach is the standardized attitude in the first line in all patients avoiding an additional venous access, which allows the Coil release in the basic technique while the arterial access allows opacification and measurement of the channel. The unique arterial approach has reduced the risk of local complications at the puncture site and the duration of procedure without difference in closure efficiency and embolization risk. In our series of 92 PDA closures by Occluder in children the double femoral approach is the standardized attitude for all patients, the venous access allows the device release while the arterial access allows opacification/ measurement of the channel and control device deployment.