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2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Congential Anomalies 740-759 > Other And Unspecified Congenital Anomalies 759.* > 2008 ICD-9-CM Diagnosis 759.6 Other congenital hamartoses not elsewhere classified A congenital syndrome characterized by a port-wine nevus covering portions of the face and cranium (in the distribution of the ophthalmic division of the TRIGEMINAL NERVE) and angiomas of the meninges and choroid. Clinical manifestations include the onset of focal SEIZURES, progressive hemiparesis, GLAUCOMA, hemianopsia, and cognitive deficits in the first decade of life. By age two years, skull radiographs reveal "tramline calcifications" of the margins of the occipital and parietal lobes. Pathologically cortical neurons are replaced by glial tissue that undergoes calcification. (From Adams et al., Principles of Neurology, 6th ed, pp1018-9) 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. A hereditary disease caused by autosomal dominant mutations involving CHROMOSOME 19. It is characterized by the presence of INTESTINAL POLYPS, consistently in the JEJUNUM, and mucocutaneous pigmentation with MELANIN spots of the lips, buccal MUCOSA, and digits. 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. An autosomal dominant disorder associated with various neoplasms including central nervous system (most often cerebellar) and retinal HEMANGIOBLASTOMA, endolymphatic sac tumors, renal cell carcinoma (see CARCINOMA, RENAL CELL), renal and pancreatic cysts, HEMANGIOMA of the spinal cord, and PHEOCHROMOCYTOMA. The most common presenting manifestations are neurologic deficits associated with intracranial hemangioblastomas which may HEMORRHAGE, causing ataxia, INTRACRANIAL HYPERTENSION, and other signs of neurologic dysfunction. (From Neurochirurgie 1998 Nov;44(4):258-66) 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 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 759.6 is a specific code that can be used to specify a diagnosis 759.6 contains 60 index entries View the ICD-9-CM Volume 1 759.* hierarchy 759.6 also known as: Syndrome: Peutz-Jeghers Sturge-Weber (-Dimitri) von Hippel-Lindau 759.6 excludes: neurofibromatosis (237.7) Index entries containing 759.6: 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