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syndrome: Chromosome 5-P abnormal. Abnormal
cry, microcephaly, micrognathia. Congenital heart disease. Anesthetic
implications: Airway problems - stridor, laryngomalacia. Possibly
difficult intubation. SBE prophylaxis where indicated.
Crouzon
disease: Craniosynostosis, hypertelorism,
parrot beak nose, hypoplastic maxilla. Anesthetic implications:
Possibly difficult intubation. Be prepared for severe blood loss
with cranial operation.
Cutis
laxa: Elastic fiber degeneration, pendulous
skin, frequent hernias. Emphysema and cor pulmonale. Arterial
fragility. Anesthetic implications: Pulmonary infection, emphysema
and cor pulmonale. Poor tissues - IV difficult to maintain. Excess
of soft tissues around larynx may lead to respiratory obstruction.
Dermatomyosists,
see Collagen disease.
DiGeorge
syndrome: 3rd and 4th brachial arch or pharyngeal pouch syndrome.
Aortic arch abnormalities. Thymus and parathyroids absent. Hypoparathyroidism,
hypocalcemia, tetany, stridor. Immune deficiency with susceptibility
to fungal and viral infections, recurrent chest infections. Treated
by thymic transplants. Anesthetic implications: Use strict asepsis.
Donor blood must be irradiated to prevent graft vs. host reaction.
Donohue syndrome, see
Leprechaunism.
Down’s syndrome (mongolism,
trisomy 21): Microcephaly. Small nasopharynx.
Hypotonia. 60% have congenital heart disease. Duodenal
atresia in some. Cervical spine abnormalities. Anesthetic implications:
Difficult airway -
large tongue, small mouth. Risk of laryngeal spasm especially
on extubation. Problems of cardiac anomalies. Use antibiotic prophylaxis
where indicated.
Duchenne
muscular dystrophy: Muscular dystrophy with
frequent cardiac muscle involvement. Usually die in second decade.
Amount of skeletal muscle involvement and cardiac involvement
unrelated. Anesthetic implications: As for myotonia congenita
plus cardiac involvement. SuccinyIchloline may cause
rhabdomyolysis, cardiac arrest due to hyperkalemia and possibly
malignant hyperpyrexia. Minimal
drug dosage. Avoid excessive use of respiratory depressants, muscle
relaxants. Postoperative ventilatory support may be required.
Edward’s
syndrome (trisomy 18(E)): Congenital heart
disease in 95%. Micrognathia in 80%. Renal malformations 50-80%.
Usually die in infancy. Anesthetic implications: Possible difficult
intubation. Care with renally excreted drugs. SBE prophylaxis
where indicated.
Ehlers-Danlos
syndrome: Collagen abnormality with hyperelasticity
and fragile tissues. Nine different
types. Type 1: mitral valve prolapse. Type 4: GI
perforation. Possible dissecting aneurysm of aorta.
Fragility of other blood vessels. Bleeding diathesis. Anesthetic
implications: CVS - spontaneous rupture of vessels. Angiogram
1% mortality. ECG conduction abnormalities. IV difficult to maintain
- hematoma. Poor tissues and clotting defects lead to hemorrhage
especially GI tract. Spontaneous pneumothorax. Need for SBE prophylaxis. |