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European Review for Medical and Pharmacological Sciences
2003; 7: 27-31
Pre-anesthesiological assessment in
paediatric cataract surgery
M. SAMMARTINO, G. MORELLI SBARRA, G. FERRO, F. CHIUSOLO, R. GARRA,
M. MARZOLA, F. PASCALE*
Department of Anesthesiology and Intensive Care, Catholic University - Rome (Italy)
*
Department of Anesthesia, Villa S. Pietro Hospital - Fatebenefratelli - Rome (Italy)
Abstract. – The authors underline the importance of accurate pre-anesthesiological assessment in children undergoing cataract.
The alteration is frequently related to many
genetic, metabolic and infectious pathologies
that could interfere in anesthesia management
whatever surgery is requested.
Some possibly responsible congenital syndromes and related alterations are mentioned,
particularly focusing on Down’s syndrome.
Key Words:
Paediatric cataract, Anaesthesia, Down syndrome.
Introduction
Cataract is defined as any opacity of the crystalline lens able to cause visual impairment.
Childhood cataracts were ascribed to undefined
genetic causes until 1941, when Sir Norman
McAlister Gregg1 introduced the concept of a
prenatal factor, first-trimester rubella, as a cause
of congenital cataracts. During the following
three decades, multiple causes of cataracts and
associated syndromes have been elucidated.
Recently, the Centers for Disease Control2 estimated the incidence of congenital cataracts at
approximately 1/10000 live borns, or 400 to 500
babies per year in the U.S. Seemingly additional 400 to 500 children per year develop cataracts
by the end of the first year of life.
Causes
There are many causes of congenital
cataracts, but specific etiology is often diffi-
cult to identify, particularly in those patients
with unilateral congenital cataracts. A large
group of patients with bilateral cataracts follow autosomal dominant pattern of inheritance without additional ocular or systemic
manifestations.
Before 1960s, maternal rubella infection
was the most frequent single cause. This is no
longer the case owing to widespread vaccination programs against rubella. Few cases are
still reported today which may reflect less effective immunological response to the vaccine. Herpes Simplex Virus, Cytomegalovirus
and Treponema Pallidum are other causative
agents3,4.
Higher survival rates in Low Birth
Neonates (LBN), due to better neonatal intensive care, have brought to an increase in
the incidence of prematurity-related cataract,
which often heals spontaneously5.
Several metabolic causes may underlie the
development of cataract in newborns. Among
them, galactosemia is especially important,
because its effects on the lens can be reversed
upon appropriate dietary restrictions.
Congenital cataracts are often unexplained; this is especially so if they are monolateral, present in the absence of a family history, known metabolic disease or prenatal
complications. Acquired cataracts are often
amenable to successful surgical treatment as
they are less likely to have lead to deprivation amblyopia. They are often the result of
penetrating and concussive ocular trauma
due to broken toys, sports, violence and accidents. Patients with penetrating ocular trauma present an additional risk of intraocular
infections6. Furthermore children with type I
diabetes under poor medical control, can develop cataract. In these latter category, gen27
M. Sammartino, G. Morelli Sbarra, G. Ferro, F. Chiusolo, R. Garra, M. Marzola, F. Pascale
der may play a role, because female adolescents seem to be more frequently affected7.
Hypocalcemia, hypoglycemia and galactokinase deficiency are other causes 8. The
main causes of congenital cataract are listed
in Table I, while the main causes of acquired
cataract are listed in Table II.
Furthermore, low birth weight, maternal
pre-eclampsia, neonatal distress syndrome
and a family history of precocious or senile
cataract may be causes of “idiopathic”
cataract, that is not so common in childhood.
However one of these factors could be a trigger in patients with a familiar predisposition9.
Table II. Acquired cataract.
• Trauma
• Diabetes
• Other metabolic disorders
Hypoglycemia
Hypocalcemia
Galactokinase defect
Galactosemia
• Systemic disorders
Asthma, Arthritis, Lupus,
Leukemia, Crohn disease
• Radiations
• Down’s syndrome
• Pierre-Robin’s syndrome
Pre-Operative Assessment
Cataracts may be associated with systemic
diseases or can be isolated disorders. An investigation of the causes of the disease is necessary for a correct pre-operative assessment
in the former and the anesthesiological evaluation will be more specific in relation to the
main disease. In the latter the pre-anesthesiological assessment will not differ from routine
pre-anesthesiological assessment for pediatric
ophthalmic surgery10,11. Concerning to anesthesiological techniques, other specific factors can be detected, such as the possible hypersensitivity to local anesthetics, considering
a regional anesthesia. Moreover in healthy
Table I. Congenital cataract.
• Idiopathic
• Infections:
German measles,
Herpes Simplex
Cytomegalovirus
Treponema Pallidum
• Prematurity
• Rare metabolic disease:
Galactosemia
Fabry’s Disease
Refsum Hypoglycemia
• Non metabolic
Syndromes:
Diseases:
28
Wolf-Hirshhorn
Hallermann-Streiff
Marfan, Alport, Lowe
Conradi, Steinert
Patau, Edwards
Stickler, Zellweger
• Treacher-Collins syndrome
• Goldenar’s syndrome
• Turner’s syndrome
children a psychologic preparation together
with parents is mandatory. Physical examination will be important to put in evidence a
positive history for allergies, bronchial asthma, susceptibility to airway infections and
previous surgical interventions. Malignant
hypertermia can be suspected when familiar
anesthesiological accidents or peri-operative
fever, especially if related with strabismus or
ptosis, occurred. Current therapies of the patient that can interfere with anesthesia, such
as corticosteroids and anti-convulsivants,
must be considered.
Routine tests for pediatric cataract surgery
under general anesthesia includes: glycemia,
azotemia, transaminases, electrolytes,
cholinesterases, cell blood count, coagulation
tests, urinanalysis, ECG and chest X-ray.
In the last few years the usefulness of these
routine pre-operative tests has been put into
discussion. In fact many studies has shown
that is possible to abandon a systematic prescription of complementary tests in favor of
an increased selectivity, based on data obtained during clinical examination and the interview with the parents during the pre-operative anesthesiological assessment12-14. That
notwithstanding there is a strong resistance to
change current medical approach.
The upper airway status is important for
pediatric anesthesiologist, because it may
compromise a good outcome of inhalatory
anesthesia.
Pre-anesthesiological assessment in paediatric cataract surgery
In the presence of an upper airways infection, the child has a risk to develop complications from 2 to 7 times higher than a
non-infectious child. In case of oro-tracheal
intubation this risk is 11 times higher and
the incidence of bronchospasm is 10 times
higher, while the incidence of laryngospasm is 5 times higher 15,16. In case of an
acute infection with purulent secretions and
cough, the surgery needs to be performed
from 2 to 7 weeks after the cessation of the
symptoms.
Ex pre-term child presents a higher risk of
post-operative complications. In particular
more than 20 seconds apneas associated with
bradycardia and cyanosis may occur. These
babies have bronchodysplasia and they often
present difficulties for the immediate postoperative extubation17.
Syndromes and Diseases
A large number of syndromes, rare and not,
may show an ocular disorder such as cataract.
Interview with parents and clinical examination of the little patient has to be more specific. The consultation of a pediatrician may
help to put in evidence congenital or acquired abnormalities associated with the ocular pathology, that must be taken into consideration by the anesthesiologist.
The main diseases that has to be considered are:
Congenital Heart-Diseases
Involvement of the atrio-ventricular structures in the trisomy 21 (Down’s syndrome);
Atrio-ventricular septal defects, patent
ductus arteriosus, destrocardia, transposition
of the great vessels, Fallot tetralogy in the trisomies 13 (Patau syndrome) and 18 (Edwards
syndrome)18-21.
Aortic valve regurgitation, possibility to
develop aortic aneurysm and dissecation
and mitralic valve prolapse in Marfan syndrome.
Malignant hypertermia risk in WolfHirshhorn syndrome (4p deletion)22; in all
these situations it is necessary to perform an
accurate clinical examination, an ECG and
an echocardiogram to detect the cardiac abnormalities and to extimate valve flow
speed.
Myasthenia, Myopathy, Myotonia
Steinert syndrome (miotonic dystrophy):
this syndrome includes progressive muscular
weakness originating from facial and extraocular muscles with a progressive mental deterioration. In this case an assessment of muscular enzymes and an electromiography that
will show impairments of the potentials is
useful.
Central Nervous System Impairments
In trisomies 13, 18, 21, in Conradi syndrome, in Lowe syndrome and in hypocalcemia due to hypoparathyroidism, the child
may show mental and psychomotor retardation, bird-head and corpus callosum agenesia.
Accurate neurologic examination, diagnostic
and instrumental tests to put in evidence
compression, dilation of cerebral ventricles
and cerebral edema have to be performed. It
has to be taken into consideration the possibility of electrolytes impairment due to a preexistant therapy with diuretics and corticosteroids.
Renal Impairment
Alport syndrome (nephritis) and Lowe
syndrome (renal tubular acidosis): it is necessary to evaluate renal functions (creatininemia, BUN, creatinine clearance).
Cranio-Facial Abnormalities
Hallerman-Streiff syndrome (cranial
anomalies, mandibular aplasia, dental malformations, curve nose bird prophile); the trisomy 13 and 18 (cleftlip and palate, micrognatia and limited neck extension); the trisomy 21 (laxitude of atlantis-occipitis joint).
Turner syndrome (pterigium colli, hypoplasic mandibula, palatal fixuration and vertebral malformations, that limit neck movements).
In all these pathologies a difficult intubation has to be considered and a fiber-optic
bronchoscopy device has to be ready to hand
in the operating theatre.
Down’s Syndrome (21 Trisomy)
It is the most common chromosomic impairment with an incidence of 6.3 per 10.000
live borns. Frequently these babies present a
congenital cataract23. This is the reason why,
it seems to be convenient, to dedicate to
Down’s syndrome a larger section of this re29
M. Sammartino, G. Morelli Sbarra, G. Ferro, F. Chiusolo, R. Garra, M. Marzola, F. Pascale
view. Life expectancy in these children is also
higher than in other chromosomic alterations,
in which the cataract’s incidence is also high,
as Patau syndrome (13 Trisomy) that has life
expectancy of six months. So it is more frequent in Down babies a surgical correction of
the cataract.
Down’s disease is characterised by different abnormalities with morphological characteristic aspect, mental retardation and peculiar anesthesiological problematics18. It is important to execute an accurate examination
of the cardio-vascular system, because of cardiac abnormalities of the atrio-ventricular
structures in these children.
This may mean right-left shunt pulmonary
overflow, responsible of pulmonary hypertension.
Concerning to the respiratory system, malformations of the palate, macroglossia, micrognatia and increased susceptibility to infections can be found.
Furthermore subglottis stenosis can be present and a smaller size endo-tracheal tube
can be necessary25-27. Concerning gastro-enteric system, dental abnormalities, duodenal
stenosis, gastro-esophageal reflux and
Hirschsprung disease are observed.
Muscular-skeletal system presents hypotonia,
flection hyperreflexia, pelvic dysplasia. Orotracheal intubation may be difficult because
of an instability of the atlantoccipital joint
due to a laxitude of the ligaments, that is present with a high frequency (12-32%). This
can lead to a sub-dislocation of the joint. In
particular head movements during surgical
procedures constitute an increased risk of dislocation. It is mandatory to perform these
movements with particular accuracy. In literature many cases of spinal cord damage due
to a sub-dislocation of this joint in Down patients during general anesthesia have been reported 28-30 . Concerning nervous system,
Down patients may present mental retardation and epilepsy.
They may show a predisposition to the infections, above all pulmonary and extra-thoracic airway infections. This may result from a
depression of the thymus-dependent immunity. Haematologic disorders include leukaemia
and polycythemia, with Hct = 70-80%.
One third of the patients have anti-thyroid
antibodies and their hypothyroidism has to
be treated for the important anesthesiological
30
implications. Furthermore these little patients present a low rate of circulating catecholamines.
A not universally recognized specificity is a
higher sensitivity to atropine, showed as an
exaggerated midriasis after local ocular instillation and a great increase of heart rate after
parenteral administration31.
In conclusion, we stress the importance of
an accurate pre-anesthesiological assessment
of children undergoing cataract extraction.
This has to be mandatory to reduce anesthesiological complications possibly related to
the above mentioned syndromes.
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