ICD-10-CM Code Q87.1

Congenital malformation syndromes predominantly associated with short stature

Version 2020 Replaced Code Non-Billable Code

Not Valid for Submission

Q87.1 is a "header" nonspecific and non-billable code code, consider using a code with a higher level of specificity for a diagnosis of congenital malformation syndromes predominantly associated with short stature. The code is NOT valid for the year 2020 for the submission of HIPAA-covered transactions.

ICD-10:Q87.1
Short Description:Congenital malform syndromes predom assoc w short stature
Long Description:Congenital malformation syndromes predominantly associated with short stature

Consider the following ICD-10 codes with a higher level of specificity:

  • Q87.11 - Prader-Willi syndrome
  • Q87.19 - Other congenital malformation syndromes predominantly associated with short stature

Replaced Code

This code was replaced in the 2020 ICD-10 code set with the code(s) listed below. The National Center for Health Statistics (NCHS) has published an update to the ICD-10-CM diagnosis codes which became effective October 1, 2019. This code was replaced for the FY 2020 (October 1, 2019 - September 30, 2020).

  • Q87.11 - Prader-Willi syndrome
  • Q87.19 - Other congen malform synd predom assoc with short stature

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code Q87.1:

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • Ellis-van Creveld syndrome Q77.6
  • Smith-Lemli-Opitz syndrome E78.72

Convert Q87.1 to ICD-9

  • 759.81 - Prader-willi syndrome (Approximate Flag)

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • Other congenital malformations (Q80-Q89)
      • Oth congenital malform syndromes affecting multiple systems (Q87)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - Code Deleted, effective from 10/1/2019 through 9/30/2020

Information for Patients


Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

A birth defect may affect how the body looks, works or both. Some birth defects like cleft lip or neural tube defects are structural problems that can be easy to see. To find others, like heart defects, doctors use special tests. Birth defects can range from mild to severe. Causes can include

  • Genetics
  • Exposures to medicines or chemicals. For example, alcohol abuse can cause fetal alcohol syndrome.
  • Infections during pregnancy
  • Certain medicines. Before you get pregnant, talk to your health care provider about any medicines you take.
  • Not getting enough of certain nutrients. For example, not getting enough folic acid before and during pregnancy is a key factor in causing neural tube defects.

For most birth defects, the cause is unknown.

Health care providers can diagnose certain birth defects during pregnancy, with prenatal tests. That's why it important to get regular prenatal care. Other birth defects may not be found until after the baby is born. Sometimes the defect is obvious right away. Other times, the health care provider may not discover it until later in life.

Babies with birth defects often need special care and treatments. The treatments may include surgery, medicines, assistive devices, and therapies.

Centers for Disease Control and Prevention


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Noonan syndrome Noonan syndrome is a condition that affects many areas of the body. It is characterized by mildly unusual facial features, short stature, heart defects, bleeding problems, skeletal malformations, and many other signs and symptoms.People with Noonan syndrome have distinctive facial features such as a deep groove in the area between the nose and mouth (philtrum), widely spaced eyes that are usually pale blue or blue-green in color, and low-set ears that are rotated backward. Affected individuals may have a high arch in the roof of the mouth (high-arched palate), poor teeth alignment, and a small lower jaw (micrognathia). Many children with Noonan syndrome have a short neck, and both children and adults may have excess neck skin (also called webbing) and a low hairline at the back of the neck.Between 50 and 70 percent of individuals with Noonan syndrome have short stature. At birth, they are usually a normal length and weight, but growth slows over time. Abnormal levels of growth hormone, a protein that is necessary for the normal growth of the body's bones and tissues, may contribute to the slow growth.Individuals with Noonan syndrome often have either a sunken chest (pectus excavatum) or a protruding chest (pectus carinatum). Some affected people may also have an abnormal side-to-side curvature of the spine (scoliosis).Most people with Noonan syndrome have some form of critical congenital heart disease. The most common heart defect in these individuals is a narrowing of the valve that controls blood flow from the heart to the lungs (pulmonary valve stenosis). Some have hypertrophic cardiomyopathy, which enlarges and weakens the heart muscle.A variety of bleeding disorders have been associated with Noonan syndrome. Some affected individuals have excessive bruising, nosebleeds, or prolonged bleeding following injury or surgery. Rarely, women with Noonan syndrome who have a bleeding disorder have excessive bleeding during menstruation (menorrhagia) or childbirth.Adolescent males with Noonan syndrome typically experience delayed puberty. They go through puberty starting at age 13 or 14 and have a reduced pubertal growth spurt that results in shortened stature. Most males with Noonan syndrome have undescended testes (cryptorchidism), which may contribute to infertility (inability to father a child) later in life. Females with Noonan syndrome can experience delayed puberty but most have normal puberty and fertility.Noonan syndrome can cause a variety of other signs and symptoms. Most children diagnosed with Noonan syndrome have normal intelligence, but a few have special educational needs, and some have intellectual disability. Some affected individuals have vision or hearing problems. Affected infants may have feeding problems, which typically get better by age 1 or 2 years. Infants with Noonan syndrome may be born with puffy hands and feet caused by a buildup of fluid (lymphedema), which can go away on its own. Older individuals can also develop lymphedema, usually in the ankles and lower legs.Some people with Noonan syndrome develop cancer, particularly those involving the blood-forming cells (leukemia). It has been estimated that children with Noonan syndrome have an eightfold increased risk of developing leukemia or other cancers over age-matched peers.Noonan syndrome is one of a group of related conditions, collectively known as RASopathies. These conditions all have similar signs and symptoms and are caused by changes in the same cell signaling pathway. In addition to Noonan syndrome, the RASopathies include cardiofaciocutaneous syndrome, Costello syndrome, neurofibromatosis type 1, Legius syndrome, and Noonan syndrome with multiple lentigines.
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