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Myelodysplasia and the Arnold-Chiari Malformation

BRUCE K. RUBIN, MD, FRCP(C); B. A. WHERRETT, MD, FRCP(C)
Am J Dis Child. 1986;140(10):971-972. doi:10.1001/archpedi.1986.02140240017010.
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Sir.—Oren et al1 reported a variety of respiratory disorders in four patients with myelodysplasia, and amelioration of these symptoms in two patients by atropine sulfate given by gastrostomy tube. Recently we have cared for two children with myelodysplasia and the Arnold-Chiari malformation (ACM) who had severe disorders of respiratory control. These reports illustrate the relationship of minor infections to respiratory decompensation and the use of atropine in one patient to relieve respiratory symptoms.

Patient Reports.—Patient 1.—A boy was born with a thoracolumbar myelomeningocele, paraplegia, ACM, and hydrocephalus. During his first year of life, the large spinal defect was eventually closed, and a ventriculoperitoneal shunt was inserted. The patient had frequent episodes of sleep apnea associated with oxygen desaturation and bradycardia that were unresponsive to theophylline. Symptoms of daytime irritability and poor feeding were alleviated somewhat by providing nasal oxygen during sleep. There were no episodes of

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