Introduction

Charcot Marie Tooth disease (CMT) is a kind of polyneuropathy which is genetically determined with autosomal dominant transition. It generally starts in patients aged 20-30 years. Starting with the forearms and distal foot muscles. All of the body muscles can olculate. It diagnose with abnormalities in nevre prompt speed and sural nevre biopsy. Symptoms are low foot and difficulties in walk, neuromotor debility and sensorial ataxia. Respiration muscles and vertebral anatomy can be add too.(1-5).

Selected neuromuscular blocker (NMB) is the most important problem when there is a need for anesthesia application in CMT cases. In CMT cases we need to argument about agent selection and cure which we will use.

In this case report we present our neuromuscular block and anesthesia experiments which we supply ıt with the atracurium on our fact who has an operation because of the CMT

Case

A 54 year old female patient referring us with the abdomen tumescence and pain. We determined that she has CMT disease, type 2 diabetes mellitus and hypertension for two years . Also we determined that she had cesarean section ( 3 times), tubovarian abscess drainage, total hysterectomy for myoma uteri and incisional hernia. It stated that she has tumescenec and pain because of the incisional hernia of phennenstiel incision and planned a surgery for that.

When we made detailed pulmonary examinations before the surgery, we state light medium restriction in lungs on the respiration function tests and on the arterial blood gases. While neurologic examinations , we determined more distinct Chorcoat neuropaty sequel on the hands and foots.

During the surgery anesthesia induction is supplied with remifentanil being 2mg/kg propofol and bolus 1mgr/kg per 30 second. Patients are evaluated for the depth of anesthesia during the whole surgery and intubation time.

Decrease of anesthesia depth is examinated with increase of the avarage arterial pressure from 15 mm/Hg ,over 90 /min heard beating rate, made a motion on the patient, eye motions, increase on the pupilla caliber , perspiration and tears. Remifentanil infüsion is increase till the dose which is disapper in these findings , for purpose of depth on anesthesia

0,5 mg /kg atracurium is given , as NMB. %0,5-1,5 vol sevoflurane is used together with oxygen and air mixture as inhalation agents. Applicated frequency 10 /min . tidal volume 8 ml/kg mechanical ventilation during the surgery . Anesthesia was maintanenced with 3-5 L/min supplied with the oxygen and air mixture, atracurium 0,1 mg/kg is iteracted if there is a neccesity. When remifentanil infüsion is terminated in last 5 min. , there is no neccesity for naloksan application for anyone. At the end of surgery , 0,01 mg/kg atropine , 0,02 mğ / kg neostigmine is given to patients for the neuromuscular blocker antagonization .

Ä°ntubation is perfectly applicated within 90 second. Surgery was about 4 minute and completed troubeless. After the surgery all of the laboratory findings and electrolyte of patients was normal. The patient was discharged from the hospital with full recovery on 6th day after the surgery.

Discussion

CMT is a type of hereditary neuropaty which is cause neuromotor and sensorial abnormalities because of the genetic lesion. CMT patients can be figured in awide spectrum which is cause step by step neuromotor and sensorial symptomps. It’s traditionally has two type including type1 and 2 . It has some subgroups like genotype and fenotype. It’s prevalance is determinated 1-4 as 10000 around the whole world (1).

CMT’s anesthesic experiments are limited with few case reports . If there is no malign hyperthermia in our case.

CMT patients generally has restrictively type respiration abnormality in lungs . There is restrictively type abnormality in our case too. This case can include neuromotor and sense defection . We had neuromotor and sense defection in our case.

CMT patients declare that they had extreme sensitivity to tyopental which is an anestehesic cure. There is no information aboutthe other hypnotic agents and using CMT in literature.We don’t use tyopental as induction agent in our case instead we prefer our routine propofol and we are not determining any unwished affect.

NMB agent selecting is the most important problem in CMT cases . Despite there is succesful applications in neuromuscler blocker applications , if there is a denervation suspicion on muscles , we need to applicate süksinilcolin because of the increasing hyperkalemia burned risk. Süksinilcolin is the most important anesthesic cure which is trigger to malign hypertermia . So , we don’t use süksinilcolin and we determined that potassium level is normal in the electrolyte follow after the surgery. In CMT we don’t determined any differences from normal facts as response to NMB non depolarization. We use medial effect atracurium which has organ independent elimination as NMB.

NMB effect should analyse with neuromuscular monitorizatıon , intubation ,antagonization and it should be done with the guiding of neuromusculer monitor.In this case , during the surgery our neuromuscular monitor is crashed and the patient is only evaluated with clinical findings . Despite this general anesthesia is applicated 4 times with any problem we can’t say that there is no risk in this case .Before some of the hypertermia cases , troubless anesthesia applications is possible. CMT cases should prepared to surgery like malign hypertermia and should applicate anesthesia for this purpose.

There is a necessity for technical anesthesia application in CMT disease. In all of the declared CMT cases, elasticy on complied process is possible.

Ä°nhalation anesthesia is one of the mutual attribute on patients. As you know inhalation anesthesias make potentialization and extense to the effects of the cures which we use. So , inhalation anesthesias can responsible for elongated process of post anasthesia care unit.

For the prevention of elongated effects of neuromuscular blocker , the inhalation anestesia is given at the most lower concentration on complied process.Applied intravenöz agent remifentanil which has shorten affect process, independent elimination process, and easy control of anesthesia depth. Doses are arranged regarding clinical situation of patients.

There is no any unwished situations on patients before and after the surgery by these general principles and mindful approaches. And finally patient is discharged from hospital with health.

Correspondence to

Ziya SALIHOGLU, 7-8 Kisim Ata sitesi, Deniz 9 Blok Daire no: 67, 34750 Ataköy-Istanbul/TURKEY Tel: 0 212 4143000-22574, Faks: 0 212 529 56 00 E-mail: zsalihoglu@yahoo.com