Masaki Komiyama, M.D.
@kanachan2020Ex-neurointerventionalist. Love phylogeny, embryology, vascular anatomy, 🐶, 🌷; HHT and ped vascular anomalies. Tweets mine.
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Embryological origin of the dura mater is controversial, and inconsistent results were reported. My opinion is that tentorium cerebelli is the border between neural crest and mesodermal origins in consideration of the vertebrate evolution and cephalic neural crest contribution.
Developmental regulatory genes play an important role in the segmental structures of the brain. They are conservative among vertebrates. Interestingly, each histogenetic (phylogenetic) unit has its own arterial supply. Brain AVM is believed to be located within each unit.
In the last 3 decades, dramatic advancement has occurred in AVM research. In 1994, Endoglin was discover as the first germline mutation for AVM. In 2009, TIE2 was discovered as the first somatic mutation for VM. In 2018, KRAS was discovered as the first somatic mutation for bAVM.
Last day of the 17th WFITN Congress in New York, I have presented "Embryology, phylogeny and anatomy are essential knowledge for neurointervention". Part of my lecture in video.
WFITN 2024 17th Congress is over. It was a fruitful and exciting meeting. Today, I have presented "Embryology, phylogeny and anatomy are essential knowledge for neurointervention". Thank you, President Seon-Kyu Lee and stuffs.
My idea: ICA system is the arterial supply for the prosencephalon, and the median vein of Markowski is the venous counterpart of the ICA system. Both systems are evolutionally obtained by vertebrates with neural crest contribution.
DEF: Disease Education Forum in Paris was successfully ended. "Skull and related structures" are discussed from the various aspects scientifically. There were so many excellent lectures. I was honored to present on "phylogenetic evolution of skull vault and base" there.
JAWS: innovation of the vertebrates. Jawless fish to jawed fish; cartilaginous to membranous ossification (dermal bone); longer jaws for predation; articulated upper jaw to incorporated upper jaw to the skull base; articular bones to two ear ossicles (malleus and incus)!! WOW
DEF seminar defseminar.com/en/ will be held in Paris on Sep 16-17. Its topic is "The bony skull: vault, base, craniofacial area and related pathologies". I will present "Phylogenetic evolution of skull vault and base". It is my pleasure and honor to be the first speaker.
I am working in the same hospital for more than 30 years. This might be very rare and has pros and cons. Great merit is that I can follow the patient very long which allows me to know the true prognosis. This patient who I knew him before his birth is now more than 20 years old.
Propranolol, rapamycin, thalidomide Although propranolol is effective for infantile hemangioma and rapamycin for Kasabach-Merritt phenomenon, it is believed that these two are not so effective for AVMs. Case series showed that thalidomide is effective for some extensive AVMs.
Propranolol This drug is originally for the heart, but now effective for infantile hemangioma (IH) and familial cerebral cavernous malformation (CCM). IH can be managed without drug when it is simple. I believe propranolol is very useful when CCM is not the surgical candidate.
HHT is an autosomal dominant disease, thus children have 50% chances to have pathological mutations. When parents don't have the same mutation, it is de novo. Rarely, one of parents is mosaic without HHT symptoms. His/her children may have heterozygous mutation with HHT symptoms.
Screening of Pediatric HHT-related brain AVM Although 2nd International guideline recommends screening of pediatric brain AVM, there is controversy for it. European Drs are against it in general, but North-American ones are for it. I am on the latter side.
Venous malformation (VM) occasionally causes serious hematological problem. When interventional procedure is required in patients with VMs with local intravascular coagulopathy (low fibrinogen, high DD), LMW heparin management is required.
Anemia and Edema of the foot Chronic severe anemia "for any reasons" may cause foot and/or ankle edema. This edema is caused by sodium and water retention to the interstitial spaces. HHT may cause severe chronic anemia due to severe, repetitive nosebleeds and/or GI bleedings.
In glue embolization, catheter tip gluing to the wall of middle miningeal artery in a neonate with dural sinus malformation on the day of birth had occurred only once in my medical carrier. It was about 18 years ago. Nothing has happened to this patient, luckily enough.
To avoid distal migration of the glue In high-flow AVF, distal migration of the glue may cause serious problem. To avoid this and facilitate the glue to stick to the nearest vascular wall, tip of the microcatheter is intentionally shaped in curve.
High concentration glue in neuro-intervention It is not easy to use high concentration (> 75%) glue in high flow AVF for fear of catheter gluing to the feeder. My advice in this situation is that as long as glue is flowing forward from the catheter, catheter gluing never occurs.
Iatrogenic pulmonary embolism in the glue embolization for neonatal AV fistula It may happen that an inappropriate injection of glue may migrate to lungs. Despite temporary BP drop, my neonatal patient tolerated this. However, I know this may cause sudden death on the table.
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