2020 ICD-10-CM Diagnosis Code Q85.8

Other phakomatoses, not elsewhere classified

    2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
  • Q85.8 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM Q85.8 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of Q85.8 - other international versions of ICD-10 Q85.8 may differ.
Applicable To
  • Peutz-Jeghers Syndrome
  • Sturge-Weber(-Dimitri) syndrome
  • von Hippel-Lindau syndrome
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as Q85.8. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • Meckel-Gruber syndrome (
    ICD-10-CM Diagnosis Code Q61.9

    Cystic kidney disease, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Meckel-Gruber syndrome
    Q61.9
    )
The following code(s) above Q85.8 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to Q85.8:
  • Q00-Q99
    2020 ICD-10-CM Range Q00-Q99

    Congenital malformations, deformations and chromosomal abnormalities

    Note
    • Codes from this chapter are not for use on maternal records
    Type 2 Excludes
    • inborn errors of metabolism (E70-E88)
    Congenital malformations, deformations and chromosomal abnormalities
  • Q85
    ICD-10-CM Diagnosis Code Q85

    Phakomatoses, not elsewhere classified

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • familial dysautonomia [Riley-Day] (G90.1)
    Phakomatoses, not elsewhere classified
Approximate Synonyms
  • Cowden syndrome
  • Peutz jeghers syndrome
  • Sturge weber sequence
  • Sturge-weber syndrome
  • Von hippel lindau syndrome
Clinical Information
  • A congenital disorder characterized by the presence of a port-wine nevus birthmark on one or both sides of the face. Additional clinical manifestations may include seizures, leptomenigeal angiomas, glaucoma, progressive hemiparesis and cognitive deficits.
  • A genetic disorder in which polyps form in the intestine and dark spots appear on the mouth and fingers. Having peutz-jeghers syndrome increases the risk of developing gastrointestinal and many other types of cancer.
  • 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 non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (port-wine stain), and capillary angiomatosis of intracranial membranes (meninges; choroid). Neurological features include epilepsy; cognitive deficits; glaucoma; and visual defects.
  • 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.
  • A rare, congenital disorder that affects the brain, skin, and eyes. Abnormal blood vessel growth occurs in the trigeminal nerve in the face and the meninges (covering) of the brain. This abnormal growth causes red or purple skin discoloration (sometimes called a port wine stain), usually on one side of the face, and can also cause seizures, learning disabilities, and glaucoma.
  • An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include hemangioblastoma in the retina; cerebellum; and spinal cord; pheochromocytoma; pancreatic tumors; and renal cell carcinoma (see carcinoma, renal cell). Common clinical signs include hypertension and neurological dysfunctions.
  • 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.
  • 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.
  • 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.
  • Von hippel-lindau disease (vhl) is a rare, genetic disease that causes tumors and cysts to grow in your body. The tumors can be either cancerous or benign. They can grow in your brain and spinal cord, kidneys, pancreas and, in men, their genital tract. Symptoms of vhl vary and depend on the size and location of the tumors. They may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems and high blood pressure.detecting and treating vhl early is important. Treatment usually involves surgery or sometimes radiation therapy. The goal is to treat growths while they are small and before they do permanent damage.
Present On Admission
POA Help
"Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are considered POA.
  • Q85.8 is considered exempt from POA reporting.
ICD-10-CM Q85.8 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 826 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedure with mcc
  • 827 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedure with cc
  • 828 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedure without cc/mcc
  • 829 Myeloproliferative disorders or poorly differentiated neoplasms with other procedure with cc/mcc
  • 830 Myeloproliferative disorders or poorly differentiated neoplasms with other procedure without cc/mcc
  • 843 Other myeloproliferative disorders or poorly differentiated neoplastic diagnoses with mcc
  • 844 Other myeloproliferative disorders or poorly differentiated neoplastic diagnoses with cc
  • 845 Other myeloproliferative disorders or poorly differentiated neoplastic diagnoses without cc/mcc

Convert Q85.8 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to Q85.8:
  • Type 1 Excludes: L81
    , Q82
    ICD-10-CM Diagnosis Code L81

    Other disorders of pigmentation

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • birthmark NOS (Q82.5)
    • Peutz-Jeghers syndrome (Q85.8)
    Type 2 Excludes
    • nevus - see Alphabetical Index
    ICD-10-CM Diagnosis Code Q82

    Other congenital malformations of skin

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • acrodermatitis enteropathica (E83.2)
    • congenital erythropoietic porphyria (E80.0)
    • pilonidal cyst or sinus (L05.-)
    • Sturge-Weber (-Dimitri) syndrome (Q85.8)

Diagnosis Index entries containing back-references to Q85.8:
  • Angiomatosis Q82.8
    ICD-10-CM Diagnosis Code Q82.8

    Other specified congenital malformations of skin

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Abnormal palmar creases
    • Accessory skin tags
    • Benign familial pemphigus [Hailey-Hailey]
    • Congenital poikiloderma
    • Cutis laxa (hyperelastica)
    • Dermatoglyphic anomalies
    • Inherited keratosis palmaris et plantaris
    • Keratosis follicularis [Darier-White]
    Type 1 Excludes
    • Ehlers-Danlos syndrome (Q79.6-)
    • encephalotrigeminal Q85.8
  • Dimitri-Sturge-Weber disease Q85.8
  • Hippel's disease Q85.8
  • Kraft-Weber-Dimitri disease Q85.8
  • Lindau Q85.8 (-von Hippel)
  • Peutz-Jeghers disease or syndrome Q85.8
  • Phakomatosis Q85.9
    - see also specific eponymous syndromes
    ICD-10-CM Diagnosis Code Q85.9

    Phakomatosis, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Hamartosis NOS
    • specified NEC Q85.8
  • Sturge Q85.8 (-Weber) (-Dimitri) (-Kalischer)
  • Syndrome - see also Disease
    • Cowden Q85.8
  • Von Hippel Q85.8 (-Lindau)

ICD-10-CM Codes Adjacent To Q85.8
Q84.8 Other specified congenital malformations of integument
Q84.9 Congenital malformation of integument, unspecified
Q85 Phakomatoses, not elsewhere classified
Q85.0 Neurofibromatosis (nonmalignant)
Q85.00 Neurofibromatosis, unspecified
Q85.01 Neurofibromatosis, type 1
Q85.02 Neurofibromatosis, type 2
Q85.03 Schwannomatosis
Q85.09 Other neurofibromatosis
Q85.1 Tuberous sclerosis
Q85.8 Other phakomatoses, not elsewhere classified
Q85.9 Phakomatosis, unspecified
Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
Q86.0 Fetal alcohol syndrome (dysmorphic)
Q86.1 Fetal hydantoin syndrome
Q86.2 Dysmorphism due to warfarin
Q86.8 Other congenital malformation syndromes due to known exogenous causes
Q87 Other specified congenital malformation syndromes affecting multiple systems
Q87.0 Congenital malformation syndromes predominantly affecting facial appearance
Q87.1 Congenital malformation syndromes predominantly associated with short stature
Q87.11 Prader-Willi syndrome

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.