“Spectacular” unilateral proptosis treatment receives LINNC Paris Best Case Award

Rashmi Saraf

An interesting case” of unilateral proptosis in a 14-year-old girl, treated using a hybrid approach via direct external carotid artery (ECA) puncture, was presented during a roundtable session at this years LINNC Paris Course (30 May–1 June, Paris, France) and was subsequently recognised with the LINNC facultys Best Case Award.

Rashmi Saraf (King Edward Memorial [KEM] Hospital, Mumbai, India) delivered the case in question, detailing that a 14-year-old girl presented at her hospital with severe, unilateral proptosis and virtually no vision in one eye. She noted that an initial angiogram showed the girl had previously undergone an ECA and superior ophthalmic vein (SOV) ligation at another centre, and a digital subtraction angiography revealed two discreet intraosseous arteriovenous fistulas (AVFs) in the greater wing of sphenoid bone—both of which had eventual common drainage.

Saraf detailed that, owing to the previous access being closed, her team attempted to use the collaterals from the deep cervical artery but got “nowhere near” the fistulas, and could not even reach the occipital artery. After discussing alternatives with her hospital’s cardiovascular and thoracic surgeon, Saraf said the decision was taken to “re-explore the neck” with a direct ECA puncture distal to the ligation.

This procedure also proved “very difficult” for the surgeon, according to Saraf, as the ligation was so high up (almost at C2 level) and distal. A surgical neck resection was performed, with direct puncture being done in the ECA distal to ligation, and access was achieved. A balloon was kept in the right internal carotid artery (ICA) to protect the vessel. The liquid embolic used (Onyx, Medtronic) started penetrating “after some time” following injection into the distal internal maxillary artery (IMAX).

A final, post-embolisation angiogram revealed “complete obliteration” of the targeted fistula. Saraf asserted the importance of packing the intraosseous pouches, as the fistulas can reoccur otherwise. She also reported that the patient’s proptosis gradually decreased over the following six weeks and she is now doing “quite well”.

Saraf then detailed that further investigations after the procedure revealed a diagnosis of cerebrofacial arteriovenous metameric syndrome (CAMS)—at the age of four years—with a slowly proliferating intraosseous lesion in the right sphenoid bone that, over the course of a decade, progressed to intraosseous AVF draining into the SOV with venous congestion and carotid cavernous fistula (CCF)-like symptoms.

Discussing the management of this case, Saraf added that it was a “technical challenge” because prior arterial and venous surgical ligation had “taken away all of our endovascular accesses”. Saraf closed her presentation with multiple take-home messages—the first of these being that regular follow-up is necessary in childhood facial haemangioma cases with bony enhancement, and this is especially true in female patients, as hormonal changes during puberty may lead to lesions progressing into more obvious fistulas, even if they have previously remained stable for a long period.

In addition, the speaker told the LINNC audience that proper diagnoses and assessment of imaging can prevent catastrophic surgeries like the one this patient had to undergo. “There is always a way out,” she concluded. “When there is no endovascular access, or no ultrasound guidance, surgeons can be of great help—and direct surgical exposure may be considered if access is not available.”

Following this presentation, Vitor Pereira (St Michael’s Hospital, Toronto, Canada) highlighted the fact that future advances may also open up the possibility of treating cases such as this with medications. Adam Arthur (University of Tennessee, Knoxville, USA) noted these types of hybrid approaches can be “very useful” in difficult cases and said it was interesting to see a case in which “a surgeon caused your problem, but you then used a surgeon to solve that problem”.

LINNC course director Jacques Moret (Bicêtre University Hospital, Paris, France) claimed this was a “spectacular” case backed by good understanding, management and results. At the close of the session, Saraf was presented with the 2022 Best Case Award and was joined on stage by many of the LINNC faculty members who had voted for her to receive this prize.

Saraf on stage with faculty members at LINNC


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