2013 ICD-9-CM Diagnosis Code 277.86
Peroxisomal disorders
- autosomal recessive peroxisomal disorder, also known as Zellweger syndrome, that typically presents in the neonatal period and is usually fatal; clinical features include hypotonia, dysmorphic skull and facial bones, visual compromise, multifocal seizures, hepatomegaly, biliary dysgenesis, and swallowing difficulties; pathologically, there are migration deficits of the neocortex and degeneration of white matter tracts; Zellweger-like syndrome refers to conditions that phenotypically resemble neonatal Zellweger syndrome, but occur in childhood or adulthood.
- childhood genetic disease, transmitted as an X-linked recessive trait, characterized by diffuse abnormality of cerebral white matter and adrenal atrophy; mental deterioration progresses to dementia, aphasia, apraxia, dysarthria, and loss of vision.
- A syndrome combining the characteristics of adrenocortical insufficiency (Addison disease) with those of cerebral sclerosis (Schilder disease). Skin bronzing and sclerosis of the brain and demyelination are the principal manifestations.
- ICD-9-CM 277.86 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Applies To
Convert 277.86 to ICD-10-CM 
ICD-9-CM Volume 2 Index entries containing back-references to 277.86:
glutaric
type I 
