Anaesthetic management in Moya-moya disease

Transcription

1 Anaesthesia, Volume 40, pages CASE REPORT Anaesthetic management in Moya-moya disease R. M. BINGHAM AND D. J. WILKINSON Summary A case of Moya-moya disease requiring anaesthesia for both investigation and attempted surgical correction is presented. The influence of the anaesthetic technique on the abnormal cerebral vasculature, with particular reference to induced hypocapnia, is discussed. Guidelines for a safe method of anaesthesia in this group of patients are suggested. Key words Anaesthesia; general, cerebral revascularisation. Moya-moya disease is a rare abnormality of the cerebral circulation with obscure aetiology. It is characterised by severe stenosis or occlusion of both internal carotid arteries, minimal filling of the anterior and middle cerebral arteries and the presence of a fine network of vessels around the basal ganglia. This basal telangiectasia is probably the result of a relatively gradual occlusion of the internal carotid artery,' and gives rise to the name moya-moya, which translates from the Japanese as 'something hazy, like a puff of cigarette smoke, drifting in the air'. The diagnosis is made principally from cerebral angiography (Fig. I), although computerised tomography (CT) scanning and electroencephalography (EEG) may provide further confirmati~n.~-~ The angiographic findings were first described in Many further publications and reports foll~wed,~-~ but the term 'cerebrovascular Moya-moya disease', which was subsequently adopted, did not appear until 1969.'O The majority of reported cases have been Japanese; it is more common in females and there is some evidence of a familial predisposition.' Classically, two distinct age groups are affected: children between 5 and 7 years and young adults in their mid-thirties. The clinical presentation is variable, but is essentially that of cerebrovascular insufficiency, resulting in focal motor and sensory disturbances. In the children, paroxysmal hemiplegia is a common presenting symptom, whilst in adults a sudden loss of consciousness associated with subarachnoid haemorrhage is a more common finding. The first attempts at treatment were medical, seeking to increase blood flow pharmacologically, either by vasodilatation or reduction of blood viscosity. However, surgical intervention is increasingly popular, and a variety of operations have been described, including cervical carotid sympathectomy and superior cervical ganglionectomy,' * intracranial transplantation of omenor temporalis muscle14 with an intact vascular supply, direct superficial temporal artery-middle cerebral anastomosis' and, more recently, encephalo-duro-arterio synangiosis. ' R.M. Bingham, MBBS. FFARCS, Senior Registrar, D.J. Wilkinson, MBBS, FFARCS, Consultant, Department of Anaesthesia, St Bartholomew's Hospital, London EClA 7BE /85/ The Association of Anaesthetists of Gt Britain and Ireland 1198

2 Moya-moya disease Fig. 1. Lateral, right common carotid arteriogram on a patient with Moya-moya disease. The pretenninal segment of the internal carotid artery is occluded, with Moya-moya collaterals from the posterior communicating cerebral perforators to the middle and anterior cerebral communicators. This latter operation involves the laying of an intact scalp artery onto the surface of the arachnoid to allow it to develop its own revascularisation process. The authors recently anaesthetised a 21- month-old boy with this syndrome on three occasions for investigative and surgical procedures, and suggest a method of anaesthetic practice which provides minimal disturbance to a critically compromised cerebral circulation. Case history A 21-month-old boy (D.M.) (weighing 10 kg), was transferred to our hospital for elective bilateral encephalo-duro-arterio synangiosis. Gestation and delivery had been normal, but he was admitted to his local hospital aged 3 weeks for failure to thrive, and at 6 weeks with laryngeal stridor. This laryngeal problem recurred intermittently over the next few months, and resulted in several admissions to hospital but was always responsive to conservative treatment. At 7 months he developed a pneumococcal septicaemia from which he made a good recovery. He was reaching his developmental milestones almost normally at this stage. However, at 19 months he developed signs of cerebral irritation and suffered intermittent fitting in the left arm and face, followed by a left hemiplegia. He responded well to conservative management with acyclovir and dexamethasone and, after 10 days, his conscious level was returning to his normal, although a left hemiplegia was still present. He then developed signs of a right hemiplegia and marked inspiratory stridor, which necessitated tracheal intubation. His lungs were electively ventilated for 3 days at this time, with a Paco, between 3.08 and 4.5 kpa. During this period he had focal and generalised seizures, which were controlled with diazepam and carbamazepine, and spastic quadraplegia with decerebrate and decorticate posturing. On weaning from the ventilator, he became much more alert and regained some spontaneous movement in his limbs. Repeat CT scanning demonstrated increasing cerebral atrophy and possible infarction, whilst angiography demonstrated classical Moyamoya disease. It was decided to transfer him for elective surgery in an attempt to improve cerebral blood flow, which was demonstrated to be ml/ 100g/minute(less than halftheexpected value). On admission he was agitated and ill, with a pyrexia of 40.2"C, stridor and a marked extensor posture. He was hyper-reflexic and hypertonic. He was being treated with carbamazepine,

3 1200 R.M. Bingham and D.J. Wilkinson baclofen (which acts at a spinal level to reduce muscle tone) and cotrimoxazole. He had a severe gastrointestinal disturbance, with vomiting and diarrhoea. He remained neurologically unchanged over the next few days, while his temperature and gastrointestinal problems were treated. His EEG at this time showed a deterioration from previous recordings and it was decided to repeat CT scanning. General anaesthesia was required to prevent the child moving during the investigation. The scan showed increased cerebral loss. Following further supportive treatment he underwent right encephalo-duro-arterio synangiosis under general anaesthesia. The left side was operated on one week later. Anaesthetic techniques CTscan. Ketamine had been used previously, as the sole anaesthetic agent for the CT scan performed elsewhere, with a satisfactory result. On this occasion, despite a dose of 2 mg/kg intravenously, the child continued to make involuntary movements, which prevented accurate scanning. The anaesthetic was therefore continued with nitrous oxide, oxygen and halothane inhalation, followed by 2 mg/kg of suxamethonium intravenously. An oral uncuffed 3.5 mm tracheal tube was passed and the child s lungs were ventilated manually using a Jackson Rees modified Ayre s T-piece until spontaneous ventilation recommenced. Anaesthesia was then uneventful, with the child continuing to breathe oxygen, nitrous oxide and halothane for the duration ofthe investigation. Postoperatively, thechild was agitated and stridulous. There were no changes in his neurological signs however, and the symptoms had settled within 24 hours. The child remained stable over the next week, receiving further symptomatic treatment for the muscle spasms, diarrhoea and dehydration. He was transfused to bring the haemoglobin within normal limits. Right encephalo-duro-arterio synangiosis. This was performed ten days after the CT scan. The child received an atropine premedication (20 pg/ kg). Anaesthesia was induced with oxygen, nitrous oxide and halothane; suxamethonium 1 mg/kg facilitated the placement of a 3.5 uncuffed Oxford tracheal tube, which was checked for the presence of a leak. Once spontaneous ventilation had resumed, fentanyl was used in incremental doses (total I.5 pg/kg) to supplement the nitrous oxide, oxygen and halothane, delivered with a Jackson Rees modified Ayre s T-piece system. During the procedure, in addition to heart and breath sound monitoring with a precordial stethoscope, blood pressure (Critikon Dinamap), pulse, ECG (Roche), temperature (Roche) and end-tidal carbon dioxide (IL 200) were monitored continuously. The child was placed on a warming mattress and heat loss minimised by using foil and wool. Anaesthesia was uneventful; the child made a normal, early postoperative recovery and was returned to the ward. He was agitated, had marked stridor and produced copious sputum. These symptoms responded well to simple analgesia, physiotherapy and the use of a mist tent to increase humidity. Left encephalo-duro-arterio synangiosis. This operation took place one week after the first. The anaesthetic technique was identical, except for a smaller fentanyl supplement of only 0.5 pg/kg intra-operatively and the use of invasive arterial monitoring. On this occasion, earlier use of humidified oxygen therapy and physiotherapy enabled a smoother postoperative course and the child was discharge from hospital 6 days later. He was generally much improved and was more responsive to his surroundings and his family. There was also an improvement in his motor tone, although he still had signs of spastic quadriparesis. Discussion There appears to be only one reference to anaesthesia in Moya-moya disease, in the form of a short letter from Sumikawa and Nagai. They noted that in two patients, severe long lasting neurological sequelae followed neuroleptanaesthesia with controlled ventilation and hypocapnia. No complications occurred on five occasions, following the same operation, when normal or raised CO, levels were maintained during nitrous oxide, oxygen and halothane anaesthesia. The authors postulate that cerebral ischaemia. secondary to vasoconstriction induced by hypocapnia, is a likely cause of the neurological deterioration. They advocate the maintenance of normal or raised C02 levels to avoid such problems. The case we report involves a very much younger patient and lends support to this view. The child s cerebral blood flow was clearly

4 Moya-moya disease 120 I severely compromised, at less than half the predicted value. Nevertheless, no neurological deterioration followed three halothane anaesthetics, during which spontaneous ventilation was maintained (with an end-tidal C02 between 5.4% and 7.1% during the two operations in which it was measured). However, neurological deterioration, in the form of focal and general seizures, had been immediately apparent during the period of controlled ventilation, prior to his admission to this hospital, during which Pam, was continuously low (between 3.08 kpa and 4.0 kpa). These seizures resolved and his level of consciousness improved during and after weaning from controlled ventilation. The relationship between Paco, and CBF in normal circumstances is well known, but to our knowledge this relationship has not been studied in people with Moya-moya disease. Theoretically, cerebral vasoconstriction induced by hypocapnia may either increase blood flow to compromised areas by constricting normal vessels, or, conversely, reduce it by directly constricting the Moya-moya vessels. Our experience, and that of Sumikawa and Nagai, provides indirect evidence of the latter effect. Further support for this is provided by descriptions of persistent delta wave activity on the EEG during and after voluntary hyperventilation in patients with this syndrome. * * Thus, if hypocapnia should be profound or long lasting, both of which are likely during anaesthesia for long neurosurgical procedures, then ischaemic neuronal damage may result. The effects of inhalational agents on CBF in Moya-moya disease are equally unpredictable. Again, blood flow to compromised areas may either increase or decrease, depending on the relative degrees of vasodilatation in the normal and abnormal vessels. However, since the use of halothane was common to all the uneventful anaesthetics, an alternative, but less likely, explanation for the absence of neurological deterioration in these patients, is that halothane may produce cerebral vasodilatation and may afford protection against possible ischaemic insults in association with surgery and/or anaesthesia. Further study into the effects of anaesthesia on cerebral blood flow in Moya-moya disease would be valuable; however, on the existing evidence, we would recommend anaesthesia with nitrous oxide, oxygen and halothane, maintaining normal or slightly raised CO, levels as the method of choice in patients with this condition. Acknowledgments The authors would like to thank Professor J.P.S. Lumley for permission to publish details of this case. References I. LEVIN S. Moya-moya disease. Developmental Medicine Child Neurology 1982; 24: 85Ck3. 2. TAKAHASHI M, MIYAUCHI T, KODAWA M. Compute tomography of moya-moya disease; demonstration of occluded arteries and collateral vessels as important diagnostic signs. Radiology 1980; KODAMA N, AOKI Y, HIRACA H. WADA T, SUZUKI J. Electroencephalographic findings in children with Moya-moya disease. Archives of Neurology 1979; 36: PICARD L. LEVWUE M, CROUZET G, SIMON J, ANDRE JM. The moya-moya syndrome. Journal de Neuroradiologie 1914; 1: TAKEUCHI K. Occlusive diseases of the carotid artery: especially on their surgical treatment. Recent Advances in Research of Nerv. Supt. Shinkei Shimpo 1961; 5: WEIDNER W, HANAFFE W, MARKHAM CH. Intracranial collateral circulation via leptomeningeal and rete mirabile anastomoses. Neurology 1965; 15: LEEDS NE, ABBOT KH. Collateral circulation in cerebrovascular disease in childhood via rete mirabile and perforating branches of anterior choroidal and posterior cerebral arteries. Radiology 1965; KUDO T. Spontaneous occlusion of the circle of Willis A disease apparently confined to the Japanese. Neurology 1968; 18: NISHOMOTO A, TAKEUCHI S. Abnormal cerebrovascular network related to the internal carotid arteries. Journal of Neurosurgery 1968; SUZUKI J, TAKAKU A. Cerebrovascular moyamoya disease, showing abnormal net-like vessels in base of brain. Archives of Neurology 1969; It. KITAHARA T, AIRCA N, YAMAURA, MAKINO H, MAKl Y. Familial occurrence of moya-moya disease: report of three Japanese families. Journal of Neurology, Neurosurgery, Psychiatry 1979; 42: SUZUKI J, TAKAKU A, KODAMA N, SATO S. An attempt to treat cerebrovascular moya-moya disease in children. Childs Brain 1975; 1: YONEKAWA Y, TASARGIL MG. Brain vascularization by transplanted omentum: a possible treatment of cerebral ischaemia. Neurosurgery 1977; 1: KARASAWA J, KIKUCHI H, FURUSE S, SAKAKI T, YOSHIDA Y, ONISHI H, TAKE W. A surgical treatment of moya-moya disease encephalo-

5 1202 R.M. Bingham and D.J. Wilkinson myosynangiosis. Neurologia Medico Chirurgica preliminary report. Surgical Neurology 1981; 15 (Tokyo) 1977; 17: IS. KARASAWA J, KIKUCHI H, FURUSE S, KAWAMIJRA 17. SUMIKAWA K, NAGAI H. Moya-moya disease and J, SAKAKI T. Treatment of moya-moya disease with anesthesia. Anesthesiology 1983; 58: STA-MCA anastomosis. Journal of Neurosurgery 18. SUNDER TR, ERWIN CW, Dueois PJ. Hyperventila- 1978; tion induced abnormalities in the electroencephalo- 16. MAISUSHIMA Y, FUKAI N, TANAKA K, TS~RUOKA gram of children with moya-moya disease. Electro- S, INABA Y, AOYAGI M, OHNO K. A new surgical encephalography and Clinical Neurophysiology treatment of moya-moya disease in children: a 1980; 49 41k20.