Friday, October 7, 2016

Intracranial Hypertension and Chiari Malformation

 / By Dr. Margaret Aranda / 

This is a medical discussion with graphic pictures. It is not suitable for minors. Parents, please use discretion.

Have you ever wondered why a baby's head is so, so soft? It really is. This is because all the bones on the brain are separated at birth. Only later do they close (See Image 1).

Invisible illnesses: Intracranial Hypertension and Chiari Malformation

Image 1 Cranial Suture Lines.  They close Gradually with time, leaving us with a completely closed skull. At birth, this is quite the protective mechanism to house a growing brain with as much protection possible to safeguard internal injury. This is why a doctor always greets a baby by placing her hand on the baby's forehead, on the upper hairline to the skull. She is actually checking for a rise in intracranial pressure, ICP.   Image Courtesy .

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 It is a great thing is that if there are brain abnormalities at birth, the baby's head can swell. It may not look pretty, but it is definitely a good thing that the 'sutures' between the bones are so soft and pliable (See Image 2). When the 'water' (ie, cerebrospinal fluid) in the brain can not properly drain, it builds up and in a baby's head to cause hydrocephalus
This comments found for survival.

Invisible illnesses: Intracranial Hypertension and Chiari Malformation
Image 2. Increased Intracranial Pressure and hydrocephalus in a Baby: UNSEALED Growth Plates.  This is the normal mechanism that Ensures protection of the internal brain. Here the brain is swollen to 3x its normal size and the cerebrospinal fluid actually has "somewhere to go." Little 18-month old baby  Room to Begum  stimulated international awareness of a condition known as  hydrocephalusRoom peculiar to fans everywhere, as she defied the odds of survival. She is of the world's most famous babies with extreme  Increased Intracranial Pressure . Abdul and Fatima Begum parents were unable to pay for healthcare in India's Room. Multiple surgeries were needed to decrease the head by pulling the suture lines you back together. This image is from about one year ago. I am unable to discover recent publicity.  Image Courtesy 

Once the bone plates are sutured together in continuous bony formation, where is the only place to go for the brain?  The brain can only be pulled down into the spinal cord.

Chiari Malformation  is an anatomical deformation of the back of the brain, a separate entity called the cerebellum (vs. the 'regular' brain, the cerebral cortex that comments found us to process information, have imagination, feelings, and be who we are, consciously ). 

When either the Primary or Secondary Chiari malformation Occurs, the base of the brain is bulging down into the spinal cord (See Image 2) through a hole in the bottom skull called the foramen magnum.

Invisible illnesses: Intracranial Hypertension and Chiari Malformation
Image 2  Chiari Malformation Type II It is definitely not normal for the brain to herniate down into the spinal cord. Parents are cautioned to listen to their children when they complain of  SYMPTOMS that could be due to Chiari Malformation.  Image Courtesy

Whenever I saw the parents getting a "Chiari Syndrome" diagnosis for their 5-year old child, I thought:
"These parents really listen to their child."
                           ~ Dr. Margaret Aranda

History:  Chiari Malformation is named after  Austrian Pathologist Hans Chiari (1851-1916) . He was the first person to Widely distribute the information on this brain abnormality and assign initial CLASSIFICATIONS C lassificatio of  Types I - III; These were published in the year 1891 (See the original paper  here ). Born in Vienna, Austria, Dr. Chiari died On May 6, 1916 in Stasbourg, France. He is still beloved today, September 4th with Chiari celebrated as the Day. 
Chiara's father was JBVL Hans Chiari, MD., A famed gynecologist who described prolactinoma. JBVL Chiari, MD was Chair of Departments of Gynecology in Prague and Vienna, and wrote a book on the history of pathology. 
It is finely pointed out that Hans Chiari gave credit (unlike so many others) to  colleagues  who Formulated the concept of 'naming' and describing this cerebellar abnormality before him.  I. Solt  tells the story in 2010 in a Journal Editorial, which is far better than anyone can do, at this point ~
The title of this piece is:

"Chiari malformation eponym- time for historical justice"

"....... In 1883, the British anatomist John Cleland (1835-1925) was the first to publish a brief description of an infant with spina bifida and hydrocephalus 1. In 1891 and 1895, Chiari published feelings extensive and thorough landmark descriptions 2 3 , which contained comprehensive neuropathological evaluations of the malformation. in 1894, Julius Arnold (1835-1915) published a case report of an infant with a sacral teratoma, but without hydrocephalus, and described "a ribbon of tissue that protruded through the foramen magnum ' 4 . Arnold did not include in feeling report a pathological evaluation or further information, and did not cite the previous works of Cleland and Chiari, while Chiari had cited Cleland 1  in feeling first publication 2 , and went on to cite Cleland both 1  and Arnold 4  of feeling second publication 3 . Both Arnold Chiari and the reports published them in German and it is highly unlikely that Arnold was unaware of Chiara's first publication .... "
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I'll just add that John Cleland also had a penchant for poetry. 
Chiara's 1891 report drew much more attention for its highly acclaimed nature, as Cleland's descriptions were deemed to be rather incomplete. Finally, let's not forget Jean Cruveilhier (1) was a pathology illustrator, who drew images of several key brain and spinal cord pathologies without putting them into a formal classification; he did this 50 years before Chiara's original paper was published. A review and history of Chiari was published in the  British Medical Journal, 2000 , partially entitled, "Cleland Cruveilhier Chiari 'Syndrome to show the many and previous contributors to describing this syndrome.
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The cerebellum is the brain's primary organ responsible for your body's balance. Vision is also processed in the cerebellum. There is no known cause of Primary Chiari. However, "Acquired" or "Secondary" Statistically Chiari is more likely to occar if:
(1) Embryonic during fetal development was abnormal replication during cell division of the spinal cord (ie, a neural tube defect); or
(2) The bony-structured opening that leads to the base of the skull is abnormally small or otherwise shaped so that the brain just Becomes 'too crowded' in its own skull. 
The foramen magnum is the only place in the brain that is a last resort to absorb small changes in Increased intracranial pressure (ie, the pressure inside the skull, ICP).  The patient typically presents with thes the complaints, seen in Image 3:
Invisible illnesses: Intracranial Hypertension and Chiari Malformation

Image 3. Symptoms of Increased Intracranial Pressure.  For Hasta with Chiari Malformation, headaches are so Commonly and painfully felt. This is why it is importante to listen to your child's complaints.  Image Courtesy I 

One can imagine several things that would make this situation is either better or worse. Factors that make Chiari SYMPTOMS worse Causes include anything that an increas in ICP. Just think of a 'mad as a hatter.' Anything that makes the face red also increases blood supply to the brain, worsening SYMPTOMS:

Makes Increased ICP Worse                            

  • Standing on your head
  • vomiting
  • Screaming
  • Holding your breath
  • High blood pressure
  • Tachycardia (fast heart rate)
  • High salt diet
  • Increased water / fluid intake
  • laying down
  • Laying down with your feet up

Eventually, the brain swelling Becomes incompatible with life. For lovers of increased & ICP Interventions are Necessary. Patients that live with Chiari Malformation frequently live with lifelong dread at all the swings of increased & ICP is going on in their head:

Increased ICP Makes Better:

  • Sitting upright with head
  • Avoid vomiting
  • Lowering your blood pressure
  • Slowing your heart rate (but be careful)
  • Breathing fast
  • quiet
  • Having the surgeon to drain the fluid

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What is the worst-case scenario, and the favorite question from the American National Board of Medical Examinations (NMBA)? 

"Which of the following is found in a patient with intracranial hypertension lovers?"
1. systolic hypotension
2. Decreased carbon dioxide levels on arterial blood gas analysis
3. Increased Heart Rate
4. Decreased Respiratory Rate

Answer in "Type K" format: 
A. 1 and 3 are correct
B. 2 and 4 are correct
C. Only 4 is correct
D. 1, 2, and 3 are correct
4 E. All are correct
Go ahead and Answer before you read on.

The reasoning behind the answer to this question is the famous  Cushing's triad  that every medical student, resident and Fellow for American National Board Exams should know :

Invisible illnesses: Intracranial Hypertension and Chiari Malformation
Image 3 Cushing's triad.  All medical students, interns, and residents need to know that if you have a patient with liver failure and on the Liver Transplantation List, you better be watching for the classic signs that your patient is about to herniate into the spinal cord below the foramen magnum.  Cushing's triad is the classic formula that every doctor should know. The answer to the above question is "D." The choices given in 1 - 5, are deliberately set out to draw the fine shades of decision-making required of a physician. So when your doctor 'thinks,' I should really be 'thinking hard.'
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Leaving you on a positive note is importante to me. So I'll do my best to show you a medical 'miracle' at the end of each Article. Here, let us look at the beloved Baby Rooke today: after one year of surgeries to decrease every head circumference, she smiled for the first time (See Image 4):

Invisible illnesses: Intracranial Hypertension and Chiari MalformationImage 4. One Year Later.  Room to smiling in 2014. A survivor of 'water on the brain, "hydrocephalus and the Resulting Intracranial Hypertension. Reportedly, the local community cast pressure on the parents, Particularly the mother, saying she should give the baby up for adoption each in a religious home or place of compassion. The mother refused, Desiring the closeness of wanting her baby to die on every lap. Not anywhere else.

Epilogue: To be provided with updates, I registered as a medical professional. That allowed me to access it in the July 2014 update to the article on Room,  here . Additional information on the Baby Room is not available on my research effort. I think it is reasonable to assume that she has passed away. Another possibility is that the family was under too much pressure and stress, and went out of the mainstream public. The Baby Room is still, and always will be, a medical miracle. She defied the doctor's assessments time and time again. She smiled. She laughed. She knew every mother. It is a testimony of the strong love between a mother and a daughter, no matter which way you tell this story. That is the second miracle to behold.
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So let us continue to the increased awareness. 
Additional information on Intracranial Hypertension : en Español  here  and hereİngilizce  and  Chinese too.  
And spread the word on  Chiari Malformationen Español and İngilizce.  
Here is  good information  for patient advocates, caregivers and medical professionals.
Thank you for reading my writings .

(1)   Cruveilhier JL'Anatomie pathologiqu du corps humain, descriptions avec figures have coloriées lithographiées; Alterations morbides diverses et dont le corps humain susceptible . 2 Vols. Paris: Bailliere, 1829-42. 




International Chiari  Association  

Twitter  @ıntlchiariassn 

II. hydrocephalus


Hydrocephalus Association  / Twitter @hydroassoc

Sea Download App for hydrocephalus
~ Hydrocephalus Association  on Twitter.
~ Hydrocephalus Association  on Facebook.

III. Intracranial Hypertension


Intracranial Hypertension Research Foundation  / @ıhresearchfound

~ Intracranial Hypertension Research Foundation En Español
MEDICAL  DISCLAIMER:  This article is for information Purposes only and is not intended as medical advice, treatment or cure. Always consult a qualified professional when seeking medical care for your medical complaints. In the USA, call 911. In Spain, Portugal and the UK, call 112. 

Thank you for Increasing awareness of Intracranial Hypertension and Chiari Malformation.

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