SUMMARY

This study highlights the possible evaulation errors of

the pupil diameter in anaesthesia and resuscitation.
A case study of Adies syndrome is observed, which
demonstrates how an error could occur due to the presence of a
dilated pupil with reflexes to a lack of light.
We underline that, in the anaesthetized patients, the inter-
vention of different medicines could jeopardize the evaluation
of the pupil diameter.
Finally, we affirm the importance of a pre-operative eva-
luation , which will diagnose any possible syndromes that can
disguise the clinical signs of the anaesthesia.
Key words: pupil diameter, Adies syndrome, errors in evalua-
ting, anaesthesiologic plaining, neurological monitoring
RIASSUNTO
Lo studio si propone di mettere in evidenza possibili
errori di valutazione del diametro pupillare in anestesia e ria-
nimazione.
Viene riportato un caso, occorso alla nostra osservazio-
ne, di sindrome di Adie, in cui lerrore potrebbe generare dalla
presenza di pupilla dilatata con riflesso alla luce assente.
Si sottolinea che, nei pazienti anestetizzati, si aggiunge
lazione di diversi farmaci che potrebbero compromettere la
valutazione del diametro pupillare e si afferma, infine, limpor-
tanza della valutazione pre-operatoria ai fini di diagnosticare
per tempo eventuali sindromi che possano mascherare i segni
clinici dellanestesia.
Parole chiave: diametro pupillare, sindrome di Adie, errori di
valutazione, piano di anestesia, monitoraggio neurologico
Acta Chirurgica Mediterranea, 2009, 25: 5
POSSIBLE ERRORS IN EVALUATING PUPIL DIAMETER IN ANAESTHESIA AND RESUSCITA-
TION: ADIES SYNDROME
RITAAZZOLINA - MARIA DI DIO - PIERFILIPPO DI MARCO - MARIA LUCIA DELLARTE - SILVESTRO MESSINA* - GIOVANNI LUCA
DI BARTOLO - GIOVANNI ARRABITO - AGOSTINO MESSINA
University of Catania - Biochemistry, Medical Chemistry and Molecular Biology Department - Anaesthesia and resuscitation Section
(Head Prof. R. Azzolina) *Vittorio Emanuele Ferrarotto-Santo Bambino- University Hospital Trust Catania - Hospital Reception
[Possibili errori di valutazione del diametro pupillare in anestesia e rianimazione: sindrome di Adie]
Introduction
The clinical evaluation of pupil diameter and
reactivity together with the assessment of pulse and
blood pressure are clinical parameters for monito-
ring the depth of anaesthesia, and are essential for
the intra-operative neurological monitoring of the
anaesthetized patient. In fact, such clinical and
instrumental parameters are used to assess both the
anaesthetic extent and the anaesthesiological plan
during surgical manoeuvres, while in resuscitation
they are useful just to evaluate critically ill patients.
Under normal circumstances, pupil diameter is the
same in both eyes (isocoria) and it measures 3.5
mm. The term miosis implies a pupil diameter
smaller than 2 mm; the condition in which it is
greater than 5 mm is called mydriasis.
During general anaesthesia, the possible side
effects of the different drugs used must be taken
into account such as some anaesthetics and opioids,
which cause miosis; while, adrenergic stimulants
and atropine are responsible for mydriasis.
Assessment errors can occur when detecting
the presence of diseases by which the patients them-
selves, sometimes, do not know they are affected
such as Adie's syndrome (or tonic pupil syndrome).
Pathophysiology and diagnosis
Under physiological conditions pupil diameter
tends to be lower in children, in elderly people and in
subjects with dark irises. It is influenced by the stimu-
lation of the parasympathetic system, which causes
miosis through innervations of the constrictive muscle
of the pupil; and by the orthosympathetic system,
which triggers a mechanism of mydriasis through
innervations of the dilator muscle of the pupil.
The parasympathetic way originates in the
nucleus of Edinger Westphal in the dorsal midbrain
and reaches the ciliary ganglion after having passed
through the trunk with motor fibers from the third cra-
nial nerve. The post-ganglion fibers innervate pupil
constrictive muscle otherwise known as iris sphincter
(mios) and ciliary muscle (accommodation).
The sympathetic way is formed by three neu-
rons: the first is the diencefalyc-bulbar, the second
is located in the ciliary-spinal centre and the third is
in the upper cervical ganglion; the efferenzes
(amyelinic) of the latter innervate the dilator muscle
of the pupil (mydriasis), the smooth muscle of the
upper eyelid and the facial vasomotor apparatus.
The continual variations of pupil diameter
depend on the changes of illumination (a light stimu-
lus is responsible for miosis while darkness results in
mydriasis), fixation distance (near vision determines
miosis while long vision causes mydriasis), neuro-
sensorial stimulus, an assumption of drugs and
various diseases such as Adies Syndrome.
Adies Syndrome derives from the name of the
English neurologist G. Adie (1886-1935) and is cau-
sed by a significant slowing down of the pupillary
reflex: the pupil is usually dilated and the light reflex
is absent, but it may be evoked by exposing the
subject to very intense light for at least half an hour.
With near vision, the pupil shrinks slowly, and
gradually expands when the visual field is in the
distance. In most cases, a reduction in the accom-
modative extent is associated with near vision and a
slow release of accommodation is connected to
long vision.
By consulting the histopathological data for
Adies Syndrome it is evident that the degeneration
of the parasympathetic post-ganglional fibers inner-
vate both the pupil sphincter muscle and the ciliary
muscle, which is why the pupillary reflexes and
accommodation are impaired.
In the initial phase of the disease, the pupil
appears mydriatic and does not respond to the
instillation of pilocarpine or other parasympathetic-
mimetic drugs.
Subsequently, it develops fine "vermifugal"
contractions of the pupillar sphincter, as a result of
its re-innervation following the regeneration of
ciliary ganglion axons.
In time,80% of the subjects develop choliner-
gic hypersensitivity and the pupil, once mydriatic,
can become miotic.
Clinical case
On 29/01/2009, the patient G.C., a male aged
34, who had been suffering from Adies Syndrome,
came for the pre-anaesthetic evaluation at the out-
patients department of the University Hospital
Gaspare Rodolico in Catania, before undergoing
cholecystectomy surgery.
After carefully looking at his medical history,
the patient was reported to be suffering from Adies
syndrome and after assessing the pupil diameter it
became obvious that the pupil was irregular, mydria-
tic and slow to react and to accommodate to light.
All the data was written in an anaesthesiologic
folder and also reported in the notes to avoid possi-
ble errors in the diagnostic evaluation; pupil dila-
tion could be considered as a clinical sign of exces-
sive superficially and depth of the anaesthetic plan.
Discussion
Adies Syndrome is a disease of unknown
ethiology characterized, as stated above, by a
dilated and irregular pupil with little or no
reaction to light.
In some cases, pupillary disorders may be
associated with reduction, elimination or asymme-
try of tendon reflexes (especially the Achilles ten-
don and the rotulian ones) a condition known as
Adie-Holmes Syndrome.
Patients with Adies Syndrome can be erro-
neously interpreted by the Anaesthetist Resusci-
tator, as the disappearance of a bilateral persistent
pupil reactivity in the presence of mydriatic pupils
is a result of the excessive depth of the anaesthesio-
logic plan, an irreversible coma, or even the relief
of unilateral mydriasis with a reduced or absent
pupil reactivity which may simulate a transtentorial
hernia by compression of the third cranial nerve.
The tonic pupil is also present in the Fisher
variant of Guillain-Barrs Syndrome (paralysis of
the third cranial nerve associated with ataxia and
areflexia) and in Charcot-Marie-Tooth disease
(hereditary motor-sensory neuropathy).
In anaesthesia the effect that an anaesthetic
has on CNS (depth anaesthesia) must be evaluated
such as desflurane, for example, which causes a
transient bilateral mydriasis by stimulating a recep-
tor localized in the CNS on which the parasym-
pathetic response of the pupillar constrictive
muscle depends, a reflection that is attenuated by
drugs such as opioids, clonidine and nitrogen pro-
toxide.
Conclusions
Clearly the action of drugs may be misleading
in the clinical evaluation of the depth of anaesthesia
as each drug induces a different response for each
receptor stimulated reflection.
6 R. Azzolina - M. Di Dio et al.

In some clinical situations or in specific disea-
ses such as Adies syndrome it is important to
assess the change in pupil diameter in order to not
confuse it with a sign of the superficiality or depth
of the anaesthesia or as a sign of deterioration in
critically ill patients.
The pupil diameter is therefore important to be
able to assess the stadium and the anaesthetic plan,
which in patients with Adies syndrome may be
masked by the degeneration of postganglionic para-
sympathetic fibers that innervate the pupil sphincter
muscle and the ciliary muscle.
In this work we want to emphasize the impor-
tance of a preoperative assessment in surgical
patients to obtain an early diagnosis of syndromes
that can cause assessment errors in the course of
anaesthesia and/or in patients hospitalized at inten-
sive care centres.
Bibliografia
1. Duke Elder S.: System of Ophtalmology. St. Louis, CV
Mosby, 1991, Vol. II, P. 170.
2. Harriman DGF, Garland H.: The pathology of Adies
Syndrome. Brain 1998, 91: 401-418.
3. Harriman DGF: Pathologic aspects of Adies Syndrome.
ADV Ophthalmol 1970, 23: 55-73, 1990.
4. Laties AM, Scheie HG: Adies Syndrome: duration of
methacoline sensitivity. Arch Ophthalmology 1965, 74:
458-459, 1995.
___________
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