2008 ICD-9-CM Diagnosis Code 759.6 : Other congenital hamartoses not elsewhere classified

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2008 ICD-9-CM Diagnosis Code 759.6

Other congenital hamartoses not elsewhere classified

759.5

759.7

759.6 is a billable ICD-9-CM medical code that can be used to specify a diagnosis on a reimbursement claim.
Newer versions of 759.6: 2009 2010

Diagnosis Definition(s) Click to expand

A rare inherited disorder in which blood vessels grow abnormally in the eyes, brain, spinal cord, adrenal glands, or other parts of the body. People with von Hippel-Lindau syndrome have a higher risk of developing some types of cancer.
autosomal dominant disorder associated with cerebellar and retinal neoplasms; the most common manifestations are neurologic deficits associated with intracranial hemangioblastomas which may hemorrhage, causing ataxia, intracranial hypertension, and other signs of neurologic dysfunction.
An inherited familial cancer syndrome which is characterized by development of capillary hemangioblastomas of the central nervous system and retina; clear cell renal carcinoma; pheochromocytoma; pancreatic tumors; and inner ear tumors. The syndrome is associated with germline mutations of the VHL tumor suppressor gene, located on chromosome 3p25-26. Symptoms of VHL syndrome may not be apparent until the third decade of life. CNS hemangioblastoma is the most common cause of death, followed by clear cell renal cell carcinoma. --2004
An inherited condition characterized by generalized hamartomatous multiple polyposis of the intestinal tract. Transmitted in an autosomal dominant fashion, Peutz-Jeghers syndrome consistently involves the jejunum and is associated with melanin spots of the lips, buccal mucosa, and fingers. This syndrome is associated with abnormalities of chromosome 19. Also known as Jeghers-Peutz syndrome and Peutz's syndrome. --2004
A group of neurocutaneous disorders manifested by facial and leptomeningeal angiomas, ipsilateral gyriform calcifications of the cerebral cortex, seizures, development delay, hemiplegia, emotional and behavioral problems, and glaucoma and other ocular disorders. Nevus flammeus on the side of the face ipsilateral to angiomatosis sometimes extends to neck, chest, and back. Angiomatosis may occasionally involve the choroid plexus, thyroid, pituitary gland, lungs, gastrointestinal organs, pancreas, ovaries, and thymus. Correlation between the distribution of the nevus and the course of the trigeminal nerve is responsible for naming the syndrome "trigemino-encephalo-angiomatosis," but later findings found the relationship to be fortuitous. The syndrome frequently occurs in incomplete forms, presenting different combinations of symptoms.

Excludes

Also applicable to/known as Click to expand

ICD-9-CM Volume 2 Index Entries That Refer To 759.6 Click to expand

Amentia (see also Retardation, mental) 319

nevoid 759.6

Angiomatosis 757.32

cutaneocerebral 759.6
encephalocutaneous 759.6
encephalofacial 759.6
encephalotrigeminal 759.6
meningo-oculofacial 759.6
neuro-oculocutaneous 759.6
retina (Hippel's disease) 759.6
retinocerebellosa 759.6
retinocerebral 759.6

Dimitri-Sturge-Weber disease (encephalocutaneous angiomatosis)

759.6

Disease, diseased - see also Syndrome

Dimitri-Sturge-Weber (encephalocutaneous angiomatosis) 759.6
Hippel's (retinocerebral angiomatosis) 759.6
Kalischer's 759.6
Kraft-Weber-Dimitri 759.6
Lagleyze-von Hippel (retinocerebral angiomatosis) 759.6
Lindau's (retinocerebral angiomatosis) 759.6
Lindau-von Hippel (angiomatosis retinocerebellosa) 759.6
Peutz-Jeghers 759.6
Sturge (-Weber) (-Dimitri) (encephalocutaneous angiomatosis) 759.6
von Hippel's (retinocerebral angiomatosis) 759.6
von Hippel-Lindau (angiomatosis retinocerebellosa) 759.6

Dysplasia - see also Anomaly

neuroectodermal 759.6

Hamartoblastoma

759.6

Hamartoma

759.6

epithelial (gingival), odontogenic, central, or peripheral (M9321/0) 213.1
- upper jaw (bone) 213.0
vascular 757.32

Hamartosis, hamartoses NEC

759.6

Hemangioblastomatosis, cerebelloretinal

759.6

Hippel's disease (retinocerebral angiomatosis)

759.6

Jahnke's syndrome (encephalocutaneous angiomatosis)

759.6

Kalischer's syndrome (encephalocutaneous angiomatosis)

759.6

Krabbe's

syndrome
- cutaneocerebral angioma 759.6

Kraft-Weber-Dimitri disease

759.6

Lagleyze-von Hippel disease (retinocerebral angiomatosis)

759.6

Lawford's syndrome (encephalocutaneous angiomatosis)

759.6

Lindau's disease (retinocerebral angiomatosis)

759.6

Lindau (-von Hippel) disease (angiomatosis retinocerebellosa)

759.6

Milles' syndrome (encephalocutaneous angiomatosis)

759.6

Neuroangiomatosis, encephalofacial

759.6

Parkes Weber and Dimitri syndrome (encephalocutaneous angiomatosis)

759.6

Peutz-Jeghers disease or syndrome

759.6

Schirmer's syndrome (encephalocutaneous angiomatosis)

759.6

Sturge (-Weber) (-Dimitri) disease or syndrome (encephalocutaneous angiomatosis)

759.6

Sturge-Kalischer-Weber syndrome (encephalocutaneous angiomatosis)

759.6

Syndrome - see also Disease

Cowden 759.6
Jahnke's (encephalocutaneous angiomatosis) 759.6
Kalischer's (encephalocutaneous angiomatosis) 759.6
Krabbe's
- cutaneocerebral angioma 759.6
Lawford's (encephalocutaneous angiomatosis) 759.6
Milles' (encephalocutaneous angiomatosis) 759.6
neurocutaneous 759.6
Parkes Weber and Dimitri (encephalocutaneous angiomatosis) 759.6
Peutz-Jeghers 759.6
Schirmer's (encephalocutaneous angiomatosis) 759.6
Sturge-Kalischer-Weber (encephalotrigeminal angiomatosis) 759.6
Sturge-Weber (-Dimitri) (encephalocutaneous angiomatosis) 759.6
von Hippel-Lindau (angiomatosis retinocerebellosa) 759.6
Weber-Dimitri (encephalocutaneous angiomatosis) 759.6

Trigeminoencephaloangiomatosis

759.6

von Hippel (-Lindau) disease or syndrome (retinocerebral angiomatosis)

759.6

Weber-Dimitri syndrome

759.6

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