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 Contralateral  Difficult intubation from airway
Syndromes
hemianopia hemangiomas
Sturge-Weber disease/syndrome   Uncontrolled hemorrhage, from rupture
Headaches
 Developmental delay of hemangiomas
Sturge-Weber syndrome, also known as Krabbe-Weber-Dimitry
  Uncommon
Mental retardation
disease, is a neurocutaneous syndrome, which is probably more
 Glaucoma Subarachnoid/subdural hemorrhage
common than would be concluded from the relatively small num-
 Choroidal Heart failure from shunting
ber of cases recorded in the literature. The cardinal features of this
hemangioma Recurrent thrombotic episodes
disease are a localized atrophy and calcification of the cerebral
 Strokes
cortex with an associated intracranial venous malformation (angio-
 Bilateral cerebral
matosis) and an ipsilateral port-wine colored facial nevus, usually
involvement
located in the ophthalmic and maxillary distribution of the tri-
geminal nerve. Any portion of the cerebral cortex may be affected ICP ¼ intracranial pressure; IOP ¼ intraocular pressure
by the atrophic process, but the occipital and parietal regions
are most commonly involved. Vascular changes are found also
in the leptomeninges, pituitary, thymus, lung, spleen, and lymph
in the occipital and parietal lobes are usually more calcified
nodes.183,184,185 Altered blood vessel fibronectin expression in
than those in the frontal lobe. Minimal cerebral involvement
Sturge-Weber syndrome could contribute to abnormal vascular
is difficult to detect and can only be diagnosed by angiography
structure and function.186
or contrast-enhanced CT scan. It is recommended that any
Clinical manifestations may range from localized, superficial
parturient with a port-wine nevus in the ocular trigeminal
skin lesions to extensive systemic involvement. It is possible for
nerve distribution have an MRI with contrast to rule out an
the combination of a port-wine facial nevus and localized cortical
ipsilateral intracranial vascular malformation, especially if a
atrophy to exist without clinical symptoms, but in the majority of
seizure disorder is present.184,188
cases convulsions are present from infancy. Mental retardation,
Any patient who presents with complaints of seizure disorder,
contralateral hemiplegia, or hemianopia without cerebral infarc-
facial nevus, ocular manifestations, or an intracranial vascular
tion are present in a high percentage of cases.187 Ipsilateral
malformation should be evaluated early in pregnancy and man-
exophthalmia, glaucoma, buphthalmos and angiomas of the
aged by a multidisciplinary team. The treatment of patients with
retina, optic atrophy, and dilated vessels in the sclera may also
Sturge-Weber disease is essentially symptomatic. Control of sei-
be present. The combination of Sturge-Weber disease with other
zures follows similar principles as outlined earlier, and requires
phakomatoses has often been noted. In most cases, the angiomas
optimal control with anticonvulsive drugs. Subarachnoid and
and hypertrophy coincide in an arm or leg, as in Klippel-
subdural hemorrhage may occur but are uncommon. Heart fail-
Trenaunay syndrome and on one side of the face as in Sturge-
ure occurs rarely and is due to shunting through the intracranial
Weber syndrome, but exceptions and dissociated forms have
angiomas. Recurrent thrombotic episodes producing gradual loss
been noted.185 Klippel-Trenaunay syndrome (see Chapter 3) is a
of function may require use of antiplatelet agents.184,189,190 Many
rare congenital malformation that may include: port-wine stain
patients will have had AV malformations or other anomalies
usually on one limb, soft tissue and bony hypertrophy (excessive
treated surgically. Photodynamic therapy or external beam radia-
growth of the soft tissue and/or bones), venous malformations
tion therapy has been recommended, but there is no evidence
(varicose veins), and lymphatic abnormalities (lymphedema).
that they are of any benefit.191,192 Hemispherectomy may be of
Fused toes or fingers, or extra toes or fingers, may be present.
benefit to control seizures.193
Complications may include cellulitis, venous thrombosis, or pul-
monary embolism. Bleeding may occur, often as a result of a
Anesthetic management
rectal or vaginal capillary tumor. There is no associated CNS
The signs and symptoms (i.e. location of the nevus, symptoma-
atrophy. Anesthetic implications from peripheral venous malfor-
tology, and vascular anomaly) and careful evaluation for asso-
mations are similar to Sturge-Weber Syndrome without any CNS
ciated anomalies determine the choice of anesthetic technique.
signs and symptoms.
Anesthesia should be carefully planned to avoid any trauma to the
Sturge-Weber disease can be diagnosed without difficulty
hemangiomatous lesions and to prevent any rise in intraocular or
from the clinical syndrome. The presence of the cortical
intracranial pressure (see Table 9.13).185 Anesthetic concerns for
lesion can be demonstrated in the majority of cases by the
the epileptic parturient have been discussed earlier.
appearance of characteristic shadows in the x-ray. The lesions



183
3 Nervous system disorders


Special attention needs to be paid to intubation and extubation.
Table 9.14 Classification and clinical manifestation of Arnold-
Difficulties with tracheal intubation may occur due to angiomas
Chiari malformation
of lip, oral cavity, tongue, larynx, and trachea.194 Uncontrolled
hemorrhage may result from perforation of vascular lesions. Classification Clinical manifestations
Tracheal intubation therefore should be performed as atraumatically
Chiari I:
as possible, with soft, nonstylleted, well-lubricated endotracheal
Herniation of medulla & High ICP from obstruction of CSF flow
tubes. Care during tracheobronchial suction is mandatory.
cerebellar tonsils 4th from 4th ventricle
Sudden increases in BP must be avoided during induction
ventricle in normal Occipital headache “ worse by
and intubation due to potential rupture of the malformations.
position coughing, strain, and head movement
Ocular manifestations require avoiding anesthetic agents that
Vomiting, difficulty swallowing, and
may increase intraocular pressure. Straining, bucking, and obstructed
hoarseness
airways during induction or emergence may increase intraocular, as
Arm and neck pain, visual
well as intracranial, pressure.185 An increase in ICP can result in
disturbances, intermittent vertigo,
intracranial hemorrhage from associated vascular malformations.
and ataxia
Regional anesthesia (spinal or epidural) is safe and logical in
the majority of cases, especially when localized superficial skin Chiari II:
lesions exist without clinical symptoms. Lumbar epidural analge- Herniation of medulla, Paralysis of the legs, muscle wasting,
sia with a low concentration of local anesthetic provides a con- tonsils, and vermis ataxia, areflexia, and mental
tinuous stable anesthetic level during first stage, which can be 4th ventricle at impairment
augmented for assisted vaginal delivery during second stage of foramen magnum High ICP and hydrocephalus symptoms
labor. With the more dilute local anesthetic solutions (e.g. bupi- Myelomeningocele
vacaine 0.0625% with fentanyl 2“2.5 ug/ml) there is minimal Aqueductal stenosis
motor block, hence pelvic muscle tone is maintained, possibly with hydrocephalus
decreasing the incidence of fetal malposition. If an epidural is not
Chiari III:
in place spinal anesthesia (saddle block) may be administered to
Further herniation Neuronal impairment of medulla can
facilitate an assisted vaginal delivery.
4th ventricle below cause breathing abnormality,
Two concerns with the use of epidural block are the potential
foramen magnum swallowing difficulty, sleep apnea,
for increased ICP caused by the injection of anesthetic drugs into
Encephalocele or and respiratory failure
the epidural space, and the catastrophic consequences of sudden
myelomeningocele Scoliosis
CSF leakage from an inadvertent dural puncture in the presence
Syrinx Progressive myelopathy
of an intracranial lesion. Slow continued leakage of CSF after the
needle has been withdrawn may be responsible for some cases of ICP ¼ intracranial pressure; CSF ¼ cerebrospinal fluid
delayed neurological deterioration. The presence of an epidural
catheter adjacent to the dural hole, as in the case in the combined
spinal“epidural (CSE) technique, may be associated with less CSF
leakage as reflected by an extraordinarily low incidence of post- the foramen magnum into the upper cervical spinal canal.
dural puncture headache.195 Classification and clinical manifestations of ACM are shown in
Preexisting neurological disease of the spinal cord or peripheral Table 9.14. Symptoms of ACMs are due to high ICP from
nerves is a relative contraindication to neuraxial block, but there obstruction of CSF flow from fourth ventricle or pressure effects
are circumstances when major conduction blocks are in the best of displacement, or entrapment of cranial nerves, resulting in
interest of the mother and her fetus. These patients might have occipital headache, shoulder and arm pain with cutaneous
a spinal AVM, which is unlikely to cause any problems during dysesthesia, visual disturbances, intermittent vertigo, and
the regional procedure itself providing it is performed below ataxia. Symptoms become worse with head movement and
the caudal end of spinal cord. Epidural or spinal anesthesia is coughing. Neuronal impairment of the medulla can cause
sleep apnea, respiratory failure, and death.196 Rarely, syncopal
preferred for C/S if time permits, and providing ICP is normal
and the patient agrees. General anesthesia is an option in cases episodes have been described and attributed to either com-
of extreme urgency, where there is increased ICP, where the pression of the midbrain ascending reticular system, or vas-
patient is uncooperative, or for obstetric considerations. Epidural cular compromise (vertebrobasilar artery compression,
hypotension).197
anesthesia may also be used in combination with GA for C/S in
order to minimize perioperative hypertension and improve post- Syringomyelia, a slow onset progressive myelopathy charac-
operative analgesia. terized by cystic degeneration within the spinal cord, is docu-
mented in 50% of ACM patients.196 Symptoms of syringomyelia
include skeletal muscle weakness, wasting, areflexia with
Arnold-Chiari malformation
or without thoracic scoliosis, and severe neurological deficits.
Adult onset Arnold-Chiari malformation (ACM) is primarily Women with Chiari I malformation with or without syringomye-
characterized by herniation of the cerebellar tonsils through lia are of particular concern because of the potential risk of



184
Chapter 9


there were no surgical complications or worsening of neurologi-
Table 9.15 Anesthetic concerns in parturients with Arnold- cal symptoms.
Chiari malformation

" Intracranial pressure Worsening symptoms and possibly brain-
Spinocerebellar ataxia (Friedreich ataxia)
stem herniation
Spinocerebellar ataxia (SCA) is a collection of diseases in which
During pushing
slow CNS degenerative changes are chiefly localized in the
Inadvertent dural puncture
spinal cord and the cerebellum; all of them are clinically char-
General anesthesia: at induction,
acterized by progressive ataxia. Friedreich ataxia is a familial and
intubation, and extubation
hereditary disease with degenerative changes chiefly localized to
During epidural injection
the dorsal half of the spinal cord and the cerebellum. The impact of
Scoliosis/ Technical difficulty
Friedreich ataxia on pregnancy and the obstetric/anesthetic man-
syringomyelia Spread of local anesthetics (LA)
agement of women with this condition is detailed in Chapter 8.
Possibility of enhanced LA neurotoxicity
Damage to spinal cord: if higher space
chosen for regional anesthesia
Summary and future directions
Meningomyelocele Nerve/spinal cord damage
Space may be shallower Many of the lesions discussed in this chapter are uncommon, so it
Lower motor neuron Possibility of hyperkalemia with is important that there is prompt reporting of individual cases.
lesion succinylcholine Advances in neurosurgical, obstetric, and anesthetic manage-
Prolonged nondepolarizing ment have resulted in a recent trend to treat the parturient
neuromuscular block aggressively for her neurological condition, as the welfare of the
Autonomic Hypotension fetus ultimately depends on the health of the mother. Appropriate
dysfunction In hypovolemic patients with an monitoring, rational, preemptive control of physiological vari-
increase in intrathoracic pressure ables, communication, a coordinated team approach, and timely
Vasodilation caused by spinal or intervention based on predetermined triage priorities are essen-
epidural anesthesia tial to optimal management.
Shunts Leakage of drug into peritoneal cavity
Shorter action of LA
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