Anesthesia

Introduction

What is Anesthesia? Anesthesia is when a doctor puts you to sleep during a medical procedure. There are several different kinds of anesthesia: sedation, local and regional (just a part of you can not feel anything), and general (being asleep).

Why this page? People with Kniest, SED, and SMD can have potentially serious complications with anesthesia. Most doctors are not aware of these risks. Use this web site to tell this this important information.

Without certain precautions, anesthesia can be dangerous for people with Kniest, SED, and SMD. In general, the anesthesiologist needs to be careful not to hyperextend the neck as much as she or he might with a non-dwarf person. Dosage should be regulated by weight rather than age. The surgeon should read articles about Kniest, SED, and SMD and Anesthesiology before surgery.

Bring a copy of this page to your pre-operation appointment. Make sure everyone involved in the surgery has read it:

your surgeon
the anesthesiologist (person who puts you to sleep during the operation)
nurses
your primary care physician (main doctor)

Please read the KSG disclaimer .

Articles from Skeletal Dysplasia Specific (Dwarfism-related) Sites

The following an article is reproduced on the KSG web site with permission from Dr. Judith Hall. The listing of these complications is not intended to frighten any individual who requires surgery, but rather to prepare your doctor and anesthesiologist for any possible complication so that the complication can be avoided or treated appropriately.

Special Problems Of Anesthesia For Little People

By Dr. Judith G. Hall
Prof. – Medical Genetics
University of British Columbia
LPBCA Inc. Medical ResourceThere are many different types of disproportionate short stature and each has a specific set of complications which may be associated with that type. People with Kniest, SED, and SMD should be be especially cautions of slippage of the vertebrae in the neck. Therefore, it is important to have a flexion and an extension lateral X-ray of the neck to make sure that the vertebrae do not move on each other prior to being made unconsciousness with anesthesia.There are some generalizations that can be made about all types of short stature if surgery is required:

Remind your physician and anesthesiologist that the dose of both anesthesia and other medications should be related to weight; that Little People do not take the average adult size of medication, but may require much less.
Little People often have small tracheas or breathing tubes and when intubating, i.e. putting a tube down to breathe for an individual, it may take a smaller tube or a pediatric size tube may be required.
Many individuals with different types chondrodysplasias do not have normal bone structure in the neck. Because of this the nerves to the neck can sometimes be squashed if special care is not given to supporting the neck during surgery and when a patient is anesthetized. Thus it is extremely important that the surgeon and anesthesiologist be aware of that and possibility and support the neck and head while the individual is unconscious.
The joints in condrodystrophies may not have full range of motion, and will not completely straighten out. Thus when an individual is anesthetized it is important not to put extra stress on those joints or attempt to straighten them completely.
Many types of condrodystrophies are associated with clefts of the palate or submucous clefts of the palate. It is important to be aware of these clefts since they may lead to aspiration or incomplete closure on insertion of breathing tubes.
Many specific types chondrodysplasias have particular complications to which they may be prone. In achondroplasia for an instance the spinal canal is small, and there is some greater risk of squashing the nerves in the spinal cord.
Specifically spinal anesthesia should not be used in achondroplasia or in most cases of people with Kniest, SED, or SMD. This may possibly lead to complications if a pregnant woman with achondroplasia or Kniest, SED, or SMD has a caesarian section since the usual anesthesia for caesarian section is a spinal anesthesia. However, it is important that instead, general anesthesia be used in this situation.
In all condrodystrophies which affect the spine ( spondyloepiphseal dysplasia, spondylometaphyseal dysplasia, mucopolysarcoidosis ) there may be absence of some of the structures of the neck and backbone which can lead to the vertebrae rubbing on each other. Specifically there can be lack of a structure called the odontoid in the high neck region which can lead to squashing of nerves in that area.
In osteogenesis imperfecta it is easy for bones to break, therefore during surgery or anesthesia it is important that individual with osteogenesis imperfecta may be a little more prone to an unusual reaction with anesthesia where the temperature goes very high, causing fever.

The listing of these complications is not intended to frighten any individual who requires surgery, but rather to prepare his/her doctor and anesthesiologist for any possible complication so that the complication can be avoided or treated appropriately.

This article above was reprinted with permission from Judith Hall, MD. The original article is at http://www.lpo.on.ca/ANESTHESIA.HTM . This article was published by the Little People of Ontario organization.

Journal Articles

In general, the anesthesiologist needs to be careful not to hyperextend the neck as much as she or he might with a non-dwarf person. Dosage should be regulated by weight rather than age.

Cleft palate repair in spondyloepiphyseal dysplasia congenita: minimizing the risk of cervical cord compression by Tofield CE, Mackinnon CA published in Cleft Palate Craniofac J. 2003 Nov;40(6):629-31. This is a case study based on an operation on one person.
This article discusses anethesiology and scoliosis (curved back) and other problems common in people with Kniest, SED, and SMD: Anesthesia for scoliosis: dwarfism and congenitally absent odontoid process published in American Association of Nurse Anesthetists 1995 Aug;63(4):332-7.
Science magazine article about dwarfism, scoliosis and anesthesia: Anesthesia for scoliosis: dwarfism and congenitally absent odontoid process by Roberts W, Henson LC published in 1995 in American Association of Nurse Anesthetists (Aug;63(4):332-7).
Dwarfs: pathophysiology and anesthetic implications by Berkowitz ID, Raja SN, Bender KS, Kopits SE published in Anesthesiology in 1990
This article is available on-line at LPA Medical Resource Center. You need Adobe PDF to read this article. Go to http://www.adobe.com/ to download Adobe Reader for free.
Spondyloepiphyseal dysplasia congenita syndrome: anesthetic implications by Mogera C, Muralidhar V published in 1996 in in Anesthesia and analgesia (Aug;83(2):433-4 ).
This is an older article. It does not have recommendations, but it raises the important issue that every dwarf should be carefully evaluated before having anesthesia. Anaesthesia for dwarfs and other patients of pathological small stature by Walts LF, Finerman G, Wyatt GM published in 1977 in the Canadian Anaesthetists’ Society journal (Nov;22(6):703-9.).
This 1998 case study of a person with SED is an important reminder. People with SED and Kniest must partner with their doctors to make sure they understand preparation needed for undergoing anesthesia: Massive Pyramidal Tract Signs after Endotracheal Intubation: A Case Report of Spondyloepiphyseal Dysplasia Congenita by published in Athestesiology journal by Redl, Gerhard MD(Volume 89(5) November 1998 pp 1262-1264 ).