Objective. To identify the clinical factors and/or test results necess
ary to determine the diagnosis and prognosis for a group of neonates w
ho presented with respiratory insufficiency and multiple contractures.
Methods. We performed a retrospective review of medical records and i
dentified 15 newborns over a 10-year period (1980 through 1990) who ha
d arthrogryposis multiplex congenita and required ventilator support a
t birth. We designed a 104 item data base to record clinical informati
on; this was subsequently analyzed using the Clinfo data base program
for statistical analysis. Results. Fourteen of the 15 patients died. O
f the fourteen, 13 were electively extubated after a variable time cou
rse (2 hours to 64 days). Autopsies performed on all 14 revealed an ap
proximately equal distribution of central nervous system (CNS) malform
ations, peripheral neuropathies, and peripheral myopathies as the etio
logy. The single survivor had myasthenia gravis. Conclusion. In neonat
es with arthrogryposis, ventilator dependence at birth carries a poor
prognosis. Prenatal factors that potentially predict respiratory insuf
ficiency for the fetus with arthrogryposis include decreased fetal mov
ements, polyhydramnios, micrognathia, and thin ribs. The combination o
f a thorough physical examination and radiographic and neuromuscular s
tudies may not result in a specific diagnosis, but it can indicate whe
ther the abnormality is in the CNS, peripheral nerves or muscles. Befo
re elective withdrawal of ventilatory support, an edrophonium chloride
challenge test should be performed to rule out myasthenia gravis.