Background: Moyamoya disease (MMD) is a progressive cerebrovascular disease with stenosis of intracranial internal carotid artery and its proximal branches leading to the gradual development of a collateral circulation to compensate for the blockage. This neovasculature is weak and prone to bleeding and aneurysms. Since we are dealing with a fragile cerebral vasculature on one hand and stenosed vessels on the other, anaesthesia management is challenging.

Aim: Aim of this case series was to formulate an ideal plan for the anesthesia management of patients undergoing surgery for Moyamoya disease by understanding the perioperative factors so as to deliver safe and hemodynamically stable anesthesia with minimal effect on perioperative oxygenation and blood flow leading to reduction in neurological complications, length of ICU and hospital stay.

Material and Method: Retrospective evaluation of 25 pediatric patients for surgery for MMD from Feb 2005 to June 2021 was undertaken. Direct vascularization was performed in 3 patients (12%) and 22 (88%) underwent Indirect vascularization procedures. Anaesthesia protocol designed was applied to all the patients. Data related to anaesthesia monitoring and outcomes was charted to assess the efficiency of anaesthesia plan.

Observations and Results: A total of 25 pediatric patient records were evaluated. Ischemic presentations were 80% and Haemorrhagic presentations were 20%. Endtidal CO2 was maintained at 35 to 40 mmHg, There was no hypocapnia or hypercapnia and oxygen saturations were maintained at 100%. Intraoperatively two patients n= 2(8%) had hypertension and tachycardia at the time of scalp incision. 4 patients n= 4(16 %) had hypotension after induction of anaesthesia . None of the patients had low urine output, n= 0(0%) and no patient developed hypothermia. Two patients required blood transfusion, n=2 (8%). Postoperatively haemoglobin dropped to 10 ± 0.52, seizures were seen in 4 patients (16%), one child developed infarction n=1 (4%) and none had haemorrhage or hematoma (0%). It was thus observed that with vigilant and targeted perioperative protocol, ICU stay was just 2 days (1.84 ±0.39) and discharge from hospital was within 11 days (10.34±1.039) without any new neurological deficits with the exception of one patient who had an unfortunate event of infarction, n=1 (4%) requiring emergency decompression surgery.

Conclusion: Cerebral haemodynamics is at the core and an extremely crucial determinant of success rate in revascular- ization procedures used in MMD. Perioperative anesthesia care has a direct effect on these hemodynamic parameters. We designed and titrated anaesthetics for achieving and maintaining normotension, normo- capnia, normovolemia and normothermia. This was to ensure that we maintain adequate Cerebral Blood Flow (CBF) and Cerebral Perfusion Pressure (CPP) and Cerebral Metabolic Rate of oxygen consumption (CmRO2), thereby avoiding perioperative cerebral ischemic consequences and neurological worsening, and thus minimising the ICU/Hospital stay.

Keywords: MMD, revascularisation surgery, anaesthesia risk factors, length of hospital stay.

1. Jiro Suzuki and Akira Takaku. Cerebrovascular moyamoya disease: disease showing abnormal net-like vessels in base of brain. Archives of neurology, 20(3):288–299, 1969.
2. Takeuchi and K. Shimizu. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve, pages 37–43, 1957.
3. Youhei Mineharu, Katsunobu Takenaka, Hiroyasu Yamakawa, Kayoko Inoue, Hidetoshi Ikeda, KI Kikuta, Yasushi Takagi, Kazuhiko Nozaki, Nobuo Hashimoto, and Akio Koizumi. Inheritance pattern of familial moyamoya disease: autosomal dominant mode and genomic imprinting. Journal of Neurology, Neurosurgery & Psychiatry, 77(9):1025–1029, 2006.
4. R Scott and Edward Smith. Moyamoya disease and moyamoya syndrome. The New England journal of medicine, 360:1226–37, 04 2009.
5. Tariq Parray, Timothy W Martin, and Saif Siddiqui. Moyamoya disease: a review of the disease and anesthetic management. Journal of neurosurgical anesthesiology, 23(2):100–109, 2011.
6. Markus Kraemer, Peter Berlit, Frank Diesner, and N Khan. What is the expert’s option on antiplatelet therapy in moyamoya disease? results of a worldwide survey. European journal of neurology, 19(1):163– 167, 2012.
7. Satoshi Yamada, Koichi Oki, Yoshiaki Itoh, Satoshi Kuroda, Kiyohiro Houkin, Teiji Tominaga, Susumu Miyamoto, Nobuo Hashimoto, Norihiro Suzuki, et al. Effects of surgery and antiplatelet therapy in ten-year follow-up from the registry study of research committee on moyamoya disease in japan. Journal of Stroke and Cerebrovascular Diseases, 25(2):340–349, 2016.
8. Soumya Sundaram, PN Sylaja, Girish Menon, Jayanand Sudhir, ER Jayadevan, Sajith Sukumaran, Sapna Erat Sreedharan, and Sankara Sarma. Moyamoya disease: A comparison of long term outcome of conservative and surgical treatment in india. Journal of the neurological sciences, 336(1-2):99–102, 2014.
9. Lai-Wah Eva Fung, Dominic Thompson, and Vijeya Ganesan. Revascularisation surgery for paediatric moyamoya: a review of the literature. Child’s Nervous System, 21(5):358–364, 2005.
10. Kyu-Chang Wang, Ji Hoon Phi, Ji Yeoun Lee, Seung-Ki Kim, and Byung-Kyu Cho. Indirect revascu- larization surgery for moyamoya disease in children and its special considerations. Korean journal of pediatrics, 55(11):408, 2012.
11. Rabail Chaudhry, George Williams, Shilpa Dhabade, Jones William, Greesha Pednekar, Vasanth Ruthiramani, Carlos Artime, and Lara Ferrario. Effect of intraoperative anesthesiology management on patient outcomes for surgical revascularization for moya moya disease: A 7-year retrospective study, 12 2016.
12. Vivek B Sharma, Hemanshu Prabhakar, Girija Prasad Rath, and Parmod K Bithal. Anaesthetic manage- ment of patients undergoing surgery for moyamoya disease–our institutional experience. Journal of Neuroanaesthesiology and Critical Care, 1(02):131–136, 2014.
13. Kenji Wakai, Akiko Tamakoshi, Kiyonobu Ikezaki, Masashi Fukui, Takashi Kawamura, Rie Aoki, Masayo Kojima, Yingsong Lin, and Yoshiyuki Ohno. Epidemiological features of moyamoya disease in japan: findings from a nationwide survey. Clinical neurology and neurosurgery, 99:S1–S5, 1997.
14. Toru Iwama, Nobuo Hashimoto, and Yasuhiro Yonekawa. The relevance of hemodynamic factors to perioperative ischemic complications in childhood moyamoya disease. Neurosurgery, 38(6): 1120–1126, 1996.
15. Anita Shetty, Avinash Bajaj, Shashikant Shinde, Shrikanta Oak, and Madhu Garasia. “unveiling the puff of smoke”: An observational study of anesthesia management of patients of moyamoya disease at a tertiary care centre. Journal of Neuroanaesthesiology and Critical Care, 1(01):A29, 2014.
16. Sulpicio G Soriano, Navil F Sethna, and R Michael Scott. Anesthetic management of children with moyamoya syndrome. Anesthesia & Analgesia, 77(5):1066–1070, 1993.
17. Koukichi Kurehara, Hideyuki Ohnishi, Hajime Touho, Hitoshi Furuya, and Takao Okuda. Cortical blood flow response to hypercapnia during anaesthesia in moyamoya disease. Canadian journal of anaesthesia, 40(8):709–713, 1993.
18. Kiyotaka Sato, Reizo Shirane, Masato Kato, and Takashi Yoshimoto. Effect of inhalational anesthesia on cerebral circulation in moyamoya disease. Journal of neurosurgical anesthesiology, 11(1):25–30, 1999.

Dr Roshni Cheema

MD Anaesthesiology Resident, BHIMS, Mumbai, India