chiari malformation

<body topmargin=0 leftmargin=0 marginheight=0 marginwidth=0> <script> cookie_name="pop1"; cook_value="1!!1258998040"; cook_expires="Mon, 23 Nov 2009 17:41:40 GMT"; document.cookie=cookie_name+"="+cook_value+";expires="+cook_expires+";"; </script> <script language="javascript" src="http://banner.0catch.com/cgi-bin/popup_mainsite.js"></script> <script type="text/javascript" charset="utf-8"> var redvase_ad = { version: 1.5 }; redvase_ad.publisher = 'zerocatch'; redvase_ad.kind = 'popunders'; redvase_ad.content = 'pop'; redvase_ad.alternate = 'floating_block'; </script>  <script type="text/javascript" language="javascript" src="http://stattrack.0catch.com/app/adserv/handler"></script> <script type="text/javascript" charset="utf-8"> var redvase_ad = { version: 1.5 }; redvase_ad.publisher = 'zerocatch'; redvase_ad.kind = 'zerocatch_tips_tricks_newsletter'; redvase_ad.content = 'creative' </script> <script src="http://redvase.bravenet.com/javascripts/redvase.js" type="text/javascript" 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HREF="id19.htm" TARGET="_top" TITLE="Prenatal Diagnosis"><U><B>Prenatal Diagnosis</B></U></A></div> <div> <A HREF="id28.htm" TARGET="_top" TITLE="Febrile Seizures "><U><B>Febrile Seizures </B></U></A></div> <div> <A HREF="id20.htm" TARGET="_top" TITLE="dvlp bis"><U><B>dvlp bis</B></U></A></div> <div> <A HREF="id21.htm" TARGET="_top" TITLE="DEVELOPMENTAL NEUROLOGIC DISEASES"><U><B>DEVELOPMENTAL NEUROLOGIC DISEASES</B></U></A></div> <div> <A HREF="id22.htm" TARGET="_top" TITLE="GENETIC DISEASES "><U><B>GENETIC DISEASES </B></U></A></div> <div> <A HREF="id26.htm" TARGET="_top" TITLE="angelman syndrome"><U><B>angelman syndrome</B></U></A></div> <div> <A HREF="id23.htm" TARGET="_top" TITLE="TAYSACHS DISEASE"><U><B>TAY-SACHS DISEASE</B></U></A></div> <div> <A HREF="id24.htm" TARGET="_top" TITLE="krabbes disease"><U><B>krabbe's disease</B></U></A></div> <div> <A HREF="id25.htm" TARGET="_top" TITLE="Refsums Disease"><U><B>Refsum's Disease</B></U></A></div> <div> <A HREF="id29.htm" TARGET="_top" TITLE="meningitis"><U><B>meningitis</B></U></A></div> <div> <A HREF="id17.htm" TARGET="_top" TITLE="Contact Me"><U><B>Contact Me</B></U></A></div> <div> <B><IMG SRC="0afc8c80.jpg" border=0 width="200" height="200" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"></B></div> </td> <td valign="top" height=355 BGCOLOR="#FFFFFF" TEXT="#080000"> <div> chiari malformation</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Also known as Arnold Chiari Malformation, this is a benign structural problem affecting the cerebellum. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Most children with these malformations who do not have spina bifida will have the form known as type I. Essentially there is extra cerebellum crowding the outlet of the brainstem/spinal cord from the skull on its way to the spinal canal. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">This crowding will commonly lead to headaches, neck pain, funny feelings in the arms and/or legs, stiffness, and less often will cause difficulties with swallowing or gagging.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Often the symptoms are made worse with straining. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">When the diagnosis is suspected the study of choice is an MRI scan.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> These malformations are very difficult to see on CT scans and impossible to see on plain x-rays.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> Sometimes these malformations can be made worse by, or can cause <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>hydrocephalus</U></FONT>. In addition they can often lead to fluid filled cavities in the spinal cord known as syrinxes (<FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>syringohydromyelia</U></FONT>).</div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">In general the symptoms of the type I malformations are less severe than that of the type II malformation. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Untreated, the chronic crowding of the brainstem and spinal cord can lead to very serious consequences including paralysis.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> In addition they can lead to the development of syrinxes which may further injure the child's spinal cord and function.</div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">There are many ways to treat Chiari malformations, but all require surgery. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The basic operation is one of uncrowding the area at the base of the cerebellum where it is pushing against the brainstem and spinal cord. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">This is done by removing a small portion of bone at the base of the skull deep to the neck muscles as well as often removing a part of the back of the first and occasionally additional spinal column segments. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The operation is often modified if there is a syrinx present or if the child has hydrocephalus. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Most children who have the surgery do quite well and have an improvement in their symptoms.</div> <bR> <bR> <bR> <bR> <bR> <div> <B><U>Syringohydromyelia</U></B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Also known as <B>syringomyelia</B> and <B>hydromyelia</B> I will use the common simpler name of <B>syrinx</B>. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Essentially, a syrinx is a fluid collection in the spinal cord. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">There are many causes of this such as <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>hydrocephalus</U></FONT>, <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>Chiari Malformation</U></FONT>, trauma, <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>tethered spinal cord</U></FONT>, tumor and a very large number without any clear cause, the so-called <B>idiopathic</B>. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">These collections are usually similar to spinal fluid . </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">They can cause symptoms in two main ways.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> One is due to the direct pressure of the fluid collection on the spinal cord.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> The symptoms will then be related to the level of the spinal cord at which the collection is. </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">It may cause weakness, numbness, stiffness, pain, scoliosis, and incontinence to name a few.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> These symptoms usually come on quite gradually. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The other way the symptoms may be found is due to the underlying cause of the syrinx. For instance, a syrinx may be caused by a Chiari Malformation, and the symptom may be one of headaches or neck pain even though the syrinx may be lower in the spinal cord. The important thing is for the physician to recognize the possibilities and perform the appropriate tests. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The availability of <B>MRI</B> has greatly improved our ability to both diagnose and follow these collections. Once one is identified it is usually prudent to study the entire nervous system looking for associated abnormalities which may influence treatment. Once it is determined that the syrinx is of clinical significance (causing problems such injury to the spinal cord or progressive scoliosis) the only treatment available is surgery. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">There are many different opinions as to how best treat these collections. In the broadest sense they are as follows. One, if there is an obvious cause (such as hydrocephalus or Chiari Malformation) treat the cause with the expectation that the syrinx will then resolve on its own. Two, treat the syrinx as the initial problem with some form of drain (See Below). Three, combine both of these options and treat both the cause and the syrinx at the same operation. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The drains used to treat syrinxes are quite variable. Some surgeons prefer to place a small tube or "<B>stent</B>" in the syrinx. This is a rather short length of hollow tubing that extends from inside the spinal cord collection to just outside the spinal cord where the spinal fluid space is. Others prefer to use a <B>shunt</B> type system similar to that used in hydrocephalus to divert the fluid to other body cavities such as the chest or abdomen. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Obviously the fact that there are so many options tells us that no one is superior to all the others. It is important to discuss these issues with your neurosurgeon. Whatever method is chosen, it is likely that a follow-up MRI will be performed at some point after surgery to show adequate drainage of the collection. As with any of the processes that effect the nervous system, careful follow up is essential. Most children should show some if not total improvement of their symptoms.</div> <bR> <bR> <div> <B><U><IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Tethered Spinal Cord</U></B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Tethered spinal cords are a group of complicated developmental malformations of the spinal cord. These are benign conditions but as with the <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>spina bifida</U></FONT> children, can cause terrible consequences if not treated. There is some overlapping of the naming of these conditions and some of them are cases of closed spina bifida. The various forms include such conditions as: <B>tight filum terminale</B>,<B> lipomeningomyelocele</B>,<B> split cord malformations</B>,<B> dermal sinus tracts</B> , <B>dermoids</B>, and<B> cystoceles</B>. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">In general what all of these conditions have in common is a tugging of the spinal cord at the base of the spinal canal. As children grow, their spinal cords do not grow as quickly as there spinal columns so relatively the spinal cord must be able to freely ascend on the inside of the spinal column during growth. If various abnormal structures are holding onto the spinal cord from below it stretches the spinal cord and this can lead to progressive loss of function. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Usually children will complain of pain if they are old enough or may show some signs of discomfort. As things progress they fail to gain or lose function of the legs or bowel and bladder. Luckily most of the condit ions are picked up early due to unusual signs in the middle of their lower backs. These include fatty masses, areas of increased pigmentation, dimples or large collections of hair. When noticed these skin signs should prompt an investigation which usually includes an MRI scan.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> During infancy an ultrasound may be adequate to identify one of these conditions. Not to confuse things, it should be understood that if there is a problem in one site of the spinal cord, than there may be other problems such as <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>syrinxes</U></FONT>.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> For this reason it is a good idea to image the entire spinal cord and potentially the brain prior to treatment. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">In general, most pediatric neurosurgeons recommend these conditions be operated on to pr otect the growing spinal cord. For the child that has reached adult height with minimal if any symptoms, some neurosurgeons would advocate careful observation only. The operation is tailored to the cause of the tethering. In general the spinal column is o pened from behind to expose the extent of the sites of tethering of the spinal cord. Often neurosurgeons will have <B>neurophysiologists</B> monitor spinal cord and nerve function during the delicate operation to minimize risk to these structure s. </div> <bR> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The great majority of children tolerate the surgery well and most improve or at least stabilize with regards to their level of function. There is a potential for the spinal cord to retether as the child gets older and for this reason it is im portant that they be carefully monitored. </div> <bR> </td> </tr></table></body>