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Q: Pallid breath holding and general anaesthetic ( Answered 5 out of 5 stars,   1 Comment )
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Subject: Pallid breath holding and general anaesthetic
Category: Health > Conditions and Diseases
Asked by: drmom-ga
List Price: $200.00
Posted: 19 Jun 2006 11:38 PDT
Expires: 19 Jul 2006 11:38 PDT
Question ID: 739398
Our daughter (currently 2.5 yrs) has been diagnosed with pallid breath
holding (PBH). She is scheduled for surgery in one month and we have
concerns about the anaesthetic and her condition.
We know that PBH is associated with triggering of the vagus nerve due
to fright or pain. She had her first "attack" at 9 mths; the last was
about 3 months ago. She has had about 10 attacks. The attacks occur as
follows: she is startled or has sudden pain. She opens her mouth as if
to scream, nothing comes out, she very quickly (seconds) goes deathly
pale and falls to the ground, not breathing. I have checked for a
pulse and not found one. She remains like this, perhaps with a limb or
two jerking, for up to 2 minutes and then recovers spontaneously. She
has also had 2 full grand-mal type seizures which may or may not be
associated with the pallid breath holding. The pediatrician isn't sure
if the grand mal events are associated with her breath holding, but as
the seizures aren't frequent, the breath holding attacks apparently
benign and the EEG normal, he is not testing further. She is otherwise
healthy, except for sleep apnea which is prompting the tonsil and
adnoid surgery behind this question. I have read up on breath holding
and it seems quite similar to a condition called reflex anoxic
seizures --RAS. I have also been told that children with RAS should be
given special care during general anaesthesia due to triggering of the
vagus nerve during intubation. What I would like to know is whether
RAS is the same thing as PBH and whether a child with PBH should
receive special consideration during intubation as well. Ideally, I
would like to provide the anaethetist with medical references if such
an approach is needed.
Answer  
Subject: Re: Pallid breath holding and general anaesthetic
Answered By: boquinha-ga on 20 Jun 2006 19:47 PDT
Rated:5 out of 5 stars
 
Hello drmom-ga!

Being a parent myself I can certainly understand your desire to have
as much information as possible. It sounds as though you have been
dealing with some scary things and I hope that the information I have
found will help to allay some of your fears. Of course, this answer is
in no way a substitute for the opinion of a qualified medical
professional, and if you have any other questions or concerns I urge
you to discuss them with a health professional that you trust. It
sounds as though this is your intention. Here is what I have found.

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BREATH HOLDING IN GENERAL

It seems like you are fairly up to date on pallid breath holding. I am
including a number of quotes and links on PBH for completion?s sake,
and also to compare features of PBH to reflex anoxic seizures. Breath
holding is considered one of the ?common, benign behaviors of
childhood.? It goes without saying that it has to be one of the
scariest as well. Common breath holding spells are usually a form of
temper tantrum where the child is attempting to exert her will and
secure her own wishes. During this breath holding the child will
usually turn blue or purple and then pass out.

?Thankfully, breath-holding spells resolve spontaneously soon after
the child passes out, and unless the fall hurts the child, she will be
fine afterwards. The spell usually resolves within 30 to 60 seconds,
with the child catching her breath and starting to cry or scream.
Sometimes children will have real seizures as part of breath-holding
spells, but these brief seizures are not harmful, and there is no
increased risk of the child's developing a seizure disorder.
Breath-holding spells occur in about 5% of children.

There is another, far less common, type of breath-holding spell, where
the child turns deathly pale instead of blue or purple. These pallid
spells are involuntary and unpredictable. They are brought on by a
sudden startle, such as falling and striking the head. The child stops
breathing, goes limp, passes out, and rapidly drains of color. Pallid
breath-holding spells also resolve spontaneously.?
http://www.drgreene.com/21_557.html


There is more information on breath holding from the University of
Chicago Medical School website. It reiterates that traditional breath
holding is a result of anger or frustration, and that the child will
turn blue and pass out. It resolves within one minute. PBH is usually
provoked by fear or pain, and the child becomes pale. There is a
period where the heart may not be beating due to the vagus nerve being
affected. Seizure activity may be seen, but there is no evidence of
abnormal brain activity on electroecephalogram (EEG). Like common
breath holding the entire episode typically resolves within one
minute. Here is a link to the complete outline.
http://pedclerk.bsd.uchicago.edu/breathHolding.html


The Merck website has additional information on PBH and breath holding in general.

?The pallid form typically follows a painful experience, such as
falling and banging the head or being suddenly startled. The brain
sends out a signal (via the vagus nerve) that severely slows the heart
rate, producing loss of consciousness. Thus, in this form, the loss of
consciousness and stoppage of breathing (which are both temporary)
result from a nerve response to being startled that leads to slowing
of the heart.

The child stops breathing, rapidly loses consciousness, and becomes
pale and limp. A seizure may occur. The heart typically beats very
slowly during an attack. After the attack, the heart speeds up again,
breathing restarts, and consciousness returns without any treatment.
Because this type is rare, if the attacks occur often, further
diagnostic evaluation and treatment may be needed.?
http://www.merck.com/mmhe/sec23/ch269/ch269g.html


Here is a short Q&A between a parent and a physician in the U.K.
regarding breath holding.
http://www.netdoctor.co.uk/ate/childrenshealth/202500.html


The Epilepsy Therapy Development Project has an excellent website with
information on seizure disorders. They also have a page on conditions
often confused with epilepsy, like PBH.
http://www.epilepsy.com/info/family_kids_conditions.html

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REFLEX ANOXIC SEIZURE

In my research, most of the information regarding RAS came from U.K.
sites. There is also a pretty big organization there committed to
education and research regarding RAS. It is called STARS (Syncope
Trust and Reflex Anoxic Seizures). RAS, like PBH, is usually brought
on by some sort of frightening or painful event, and is a result of
stimulation of the vagus nerve.

This first quote comes from a site for parents of children with
disabilities. It describes the sequence of events in an RAS episode
and suggests that terms such as PBH may in fact be describing the same
thing.

?Reflex Anoxic Seizure is the term used to describe a particular type
of fit which is neither epileptic nor due to breath-holding. It is a
particular type of severe syncope or ?faint? which is the result of a
temporary cutting off of the supply of oxygenated blood to the brain.

Reflex Anoxic Seizures can occur in children as young as just a few
days old. It is most common in toddlers, who usually grow out of it,
but can reoccur in adolescents and adults. Any unexpected stimuli,
such as pain, fear, fright or even a pleasant surprise, causes the
heart to stop, the eyes to roll, marked pallor of the skin, and
clenching of the jaw. The body also stiffens and there may be jerking
movements of the arms and legs. After a few seconds to half a minute
the heart starts and the body relaxes. The child may remain
unconscious for anything from a few minutes to well over an hour as
children often fall into a deep sleep afterwards. The frequency of
attacks varies from child to child and Reflex Anoxic Seizure can be
caused by even a mild pain if unexpected.

Reflex Anoxic Seizure is often misdiagnosed as breath holding, temper
tantrums or as epilepsy. The associated marked pallor of the skin has
led to these attacks being called Pallid Infantile Syncope or White
breath-holding but some people now prefer the term Reflex Asystolic
Syncope.?
http://www.cafamily.org.uk/Direct/r15.html


The STARS website also has a brief description of RAS. 

?REFLEX ANOXIC SEIZURES occur mainly in young children but can occur
at any age. Any unexpected stimuli, such as pain, shock, fright causes
the heart and the breathing to stop, the eyes to roll up into the
head, the complexion to become deathly white/grey, often blue around
the mouth and under the eyes, the jaw to clench and the body to
stiffen, sometimes the arms and legs jerk. After 30 seconds or so, the
body relaxes, the heart and breathing resume and the person is
unconscious. One or two minutes later the person may regain
consciousness but can remain unconscious for well over an hour. Upon
recovery the person maybe very emotional and then fall into a deep
sleep for two to three hours and looks extremely pale. RAS attacks may
occur several times a day / week / month. The attacks appear to come
in batches.?
http://www.stars.org.uk/whatras.html


?Contemporary Pediatrics? is a medical publication with information on
current pediatric practices. It describes PBH and also suggest that
the term may be interchanged with RAS.

?Pallid, or type 2, spells are more often provoked by a sudden,
unexpected event that frightens the child, such as a bump to the head
or an immunization. The child cries only a little, becomes pale and
limp, and may posture or show convulsive movements before regaining
consciousness. These spells are sometimes called ?white
breath-holding,? ?reflex anoxic seizures,? or ?infantile syncope.??
http://www.contemporarypediatrics.com/contpeds/article/articleDetail.jsp?id=139704


On health24.com there is an article written by a child health expert
who also says that the terms PBH and RAS are actually describing the
same thing.

?This term is a misnomer [PBH], because breath is not held during the
spell as in the breath-holding attacks just mentioned. The modern term
is ?reflex anoxic seizures.? These are invariably induced by sudden
pain or a fright.

The child gives a cry, and then abruptly loses consciousness. The face
is ashen. Colour improves after ten to 15 seconds and consciousness
returns. These spells are due to a pain-induced surge of vagal tone
which slows the heart to a standstill for a few beats?
http://www.health24.com/medical/Head2Toe/777-778-781,25461.asp


British pediatric cardiologist Dr K A McLeod also states that PBH and
RAS are  interchangeable terms.

?In toddlers, reflex syncope tends to manifest as so called ?reflex
anoxic seizures? or ?pallid breath holding spells? with reflex
asystole followed by an anoxic seizure in response to a noxious
stimulus.?
http://adc.bmjjournals.com/cgi/content/full/88/4/350


The University of Indiana has a case description of a little girl with
RAS on its website. It describes in detail what episodes of RAS look
like as well as treatment and coping strategies. It describes briefly
how the vagus nerve is involved in RAS.
http://www.indiana.edu/~pietsch/ras.html


Here is a Q&A between a parent and a physician in the U.K. regarding RAS.
http://www.netdoctor.co.uk/ate/childrenshealth/203326.html


In an article entitled ?Pallid breath-holding spells: Evaluation of
the autonomic nervous system,? it mentions the vagus nerve as the
cause of PBH. It also describes how most of the participants in the
study with PBH experienced seizures as well.

?Excessive vagal tone has been implicated as the cause of pallid
breath-holding spells (PBHS) in children.?
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=2293948&dopt=Abstract

- - - - - - - - - - - - - - - - - - - -

ANESTHESIA CONCERNS

Given that PBH and RAS seem to be such similar entities, and recently
have been labeled as the same condition, it is reasonable to alert
your anesthesiologist or anesthetist to your daughter?s condition.
This way he or she can be at least aware of potential complications
during surgery.

The STARS website has the most succinct information regarding
precautions that should be taken during surgery in patients with RAS.

?The vagus nerve can be stimulated to produce an RAS attack by
pressing the eyeballs [the ocular compression test] and, in
anaesthesia when the tube is pushed down the throat. If the person is
anaesthetised lying flat, after being given a pre-operation dose of
atropine, there will be no further complications.

Complications arise where, particularly in dental anaesthesia, the RAS
sufferer is anaesthetised sitting up. The intubation causes the RAS
attack, and because the body is upright the blood falls to the legs.
When the fail-safe mechanism restarts the heart the lack of blood in
the heart could cause a cardiac arrest. Complications, including brain
damage could then ensue.

The advice is always tell the anaesthetist that the child or adult
has, or did suffer from RAS, and insist that all anaesthesia is
administered while the patient is lying flat.?
http://www.stars.org.uk/faq.html
STARS also has a pamphlet available to order entitled ?Information for
Dentists & Anaesthetists.? You can find it midway down the page in the
middle. This may be something your anesthesiologist would like to see.
http://www.stars.org.uk/doctors/medpubs.htm


Pubmed.com is a database maintained by the National Library of
Medicine and National Institute of Health. Here you can search through
about 16 million medical articles dating back to the 1950s. A search
for ?reflex anoxic seizures anesthesia? produced only 3 hits. None of
them have abstracts available online, but all have full text versions
available online if you pay for access. All three of these articles
would be good resources for your anesthesiologist. It is possible that
he or she has seen them already, or has access to either the online or
print version.

The first article is entitled ?Anesthesia for a Child with Reflex
Anoxic Seizures? and is from the August 2005 issue of ?Anesthesia and
Analgesia.?
http://www.anesthesia-analgesia.org/cgi/pmidlookup?view=long&pmid=15976265

The other two articles are both in the medical journal ?Paediatric
Anesthesia.? The first is entitled ?Anesthetic considerations for a
child with reflex anoxic seizures? and is in the July 2003 issue.
http://www.blackwell-synergy.com/doi/abs/10.1046/j.1460-9592.2003.01088.x

The next is entitled ?Reflex anoxic seizures and anaesthesia? and is
in the September 1999 issue.
http://www.blackwell-synergy.com/doi/abs/10.1046/j.1460-9592.1999.00416.x

- - - - - - - - - - - - - - - - - - - -

I hope that this information is useful to you. I sincerely wish you
and your daughter the best and I hope that your physicians are open to
the information you present to them. Again, this answer is not
intended to substitute for the opinion of a qualified medical
professional and I know that you intend to discuss all of your
daughter?s options with her doctors. If you have any further need of
clarification, please let me know how I can help.

Sincerely,
Boquinha-ga


Search strategy:

Online search
Pubmed.com search


Search terms:

pallid breath holding
reflex anoxic seizures
anesthesia reflex anoxic seizures
anesthesia pallid breath holding
drmom-ga rated this answer:5 out of 5 stars
Very comprehensive review. Thank you for the timely response.

Comments  
Subject: Re: Pallid breath holding and general anaesthetic
From: boquinha-ga on 26 Jun 2006 09:12 PDT
 
Thank you very much for your kind words and 5 stars! I wish you and
your family much success and health.

Sincerely,
Boquinha-ga

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