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Anticholinergic agents may be useful
in patients with suspected airway management problems. Restrictive lung
disease prolongs inhalational induction. Neuromuscular blockers should be
avoided until the airway has been secured. Subacute bacterial endocarditis
prophylaxis may be required. Kidney dysfunction may affect elimination of
(predominantly renally excreted) drugs.
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Other Conditions to Be Considered
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Jeune Syndrome: Very rare form of chondrodysplasia often leading
to death in infancy from respiratory insufficiency as a result of severe
thoracic restrictive deformation. Commonly associated with multiple skeletal
malformations and liver, renal, and retinal dysfunction.
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Saldino-Noonan Syndrome (Short-Rib Polysyndactyly Syndrome): Lethal, autosomal recessive inherited skeletal dysplasia characterized by a
hypoplastic thorax, short ribs and limbs, polydactyly, and visceral
abnormalities (e.g., transposition of the great arteries, polycystic
kidneys, atretic lesions of the gastrointestinal and genitourinary systems).
The pelvis resembles that in the Ellis-van Creveld syndrome and Jeune syndrome.
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Majewski Syndrome (Short-Rib Polysyndactyly Syndrome):
Autosomal recessive inherited disorder characterized by short stature with
extremely short limbs, preaxial and postaxial polysyndactyly of hands and
feet, short ribs, premature ossification of the proximal humerus and femur
epiphyses and the lateral cuboids, malformed ears, short and flat nose,
median cleft lip/palate, genital abnormalities (ambiguous genitalia),
anomalies of the epiglottis and larynx (malformed larynx with hypoplastic
epiglottis), pulmonary hypoplasia, and glomerular and renal tubular cysts
leading to renal failure. Sometimes associated with polyhydramnios and
hydrops fetalis.
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Verma-Naumoff Syndrome (Short-Rib Polysyndactyly Syndrome):
Autosomal recessive inherited disorder characterized by polydactyly, short
ribs, and narrow chest with neonatal asphyxia, often resulting in early
death. Craniofacial anomalies include frontal bossing, flat occiput, and
depressed nasal bridge. Genitourinary anomalies are common.
++
Beemer-Langer Syndrome: Genetic disorder characterized by
multiple skeletal (and other) anomalies and death either in utero or in the
early neonatal period as a result of respiratory failure.
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Weyers Syndrome II: Recognized phenotype of the Ellis-van Creveld
syndrome, also presenting with heart defects (atrial or ventricular septal
defects in >70% of patients) and postaxial polydactyly of the hands and
feet, hexadactyly, fusion of the fifth and sixth metatarsals and
metacarpals, and bony clefts of the mandibular symphysis. Other features may
include short stature, micrognathia, small mouth, orodental anomalies,
hypoplasia of the larynx, and hypoplastic and dysplastic nails.