Genetic neurodegenerative disorder characterized by
inflammation of the fingers and/or toes with paronychia and whitlows,
multiple ulcers (fingers, soles of the feet), and sensory loss in both arms
Giaccai (often misspelled Giacci) Familial Neurogenic
Acroosteolysis; Giaccai Syndrome; Hereditary Sensory Radicular Neuropathy,
Recessive form; Hereditary Sensory Neuropathy Type II (HSN II); Neurogenic
Very rare (fewer than 50 cases reported).
Peripheral nerve degeneration of unknown cause. A
high urinary excretion of sphingomyelin and lecithin in three sibs of a
Turkish family suggested that the pathogenetic mechanism was a disorder of
phospholipid metabolism. Several authors believe the disease is
Nerve biopsy shows wallerian degeneration of the axons,
with reduction of the number of myelinated fibers. Unmyelinated fibers also
were involved but to a lesser degree.
Age of onset usually is between 5 and 15 years,
which is earlier than the dominant type. There is a loss of all modalities
of cutaneous sensation in the lower extremities. It is associated with joint
and leg swelling that leads to cylinder-shaped limbs. Proximal sensation is
preserved and tendon reflexes are lost. There is no deafness or other
cranial nerve involvement. No nystagmus or ocular involvement. There is
generalized neurogenic osteoporosis, with acroosteolysis and collapse of the
distal phalanges, leading to digital clubbing.
Precautions before Anesthesia
Patients are often scheduled for
amputations secondary to osteomyelitis. Usually no specific cardiovascular
or respiratory involvement. No central nervous system problem. No airway
problems related to the syndrome.
Difficult venous access as a result of
pachydermoperiostosis and soft tissue swelling. Enhanced body temperature
loss caused by hyperhydrosis. Airways usually are not involved by soft
tissue swelling, but special attention must be given to this possibility.
Proper positioning on the operation table and in the
postoperative period to prevent pressure
sores. Use of regional anesthesia is controversial.
Effect of local anesthetics remains
unclear. Normal response to neuromuscular blockers.
Other Conditions to Be Considered
Hereditary Sensory and Autonomic Neuropathy Type I: Characterized
by a loss of sensation usually affecting the feet and legs more severely
than the hands and forearms. Other clinical features include open sores on
the feet and anomalies of pain and temperature sensations that are affected
more than touch-pressure sensation.
Charcot-Marie-Tooth Disease: Neurologic disorder characterized by
muscle atrophy and weakness most prominent in the legs and the small muscles
of the hands. A decrease in vibration, pain, and thermal sensation in the
hand, foot, and lower part of the leg may occur. Stretch reflexes are
Peripheral Neuropathy: Produced by disease of a single nerve
(mononeuropathy, mononeuritis), several nerves in asymmetrical areas of the
body (mononeuritis multiplex), or many nerves simultaneously
(polyneuropathy, polyneuritis, multiple peripheral neuritis). These symptoms
may involve sensory, motor, reflex, or blood vessel (vasomotor) function.
Syringomyelia: Caused by the development of a syrinx within the
spinal cord destroying the fascicle of Lissauer. Clinically, it is
characterized by loss of pain and temperature sensations first noticed in
the fingers, hands, arms, and upper chest. In the early stages, a sense of
touch is still present. As the disease evolves, a loss of feeling may spread
over the shoulders and back. Chronic progressive degeneration of the
stress-bearing part of a bone joint (Charcot joint) is another symptom.
Other clinical features include loss of tendon reflexes in the upper
extremities, spasticity, muscle weakness, muscular incoordination in the
lower extremities, and paralysis of the bladder.
Roussy-Levy Syndrome: Motor sensory disorder characterized by
a foot deformity in the shape of a claw (clawfoot), muscle weakness, atrophy
of the leg muscles, and tremor in the hands.