2027 ICD-10-CM Diagnosis Code Q87.19Other congenital malformation syndromes predominantly associated with short stature

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q87

ICD-10-CM Q87.19
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q87.19 is a billable ICD-10-CM diagnosis code for other congenital malformation syndromes predominantly associated with short stature. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 564 through 566. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 2 closely related codes. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q87.19
Billable Status
Yes — Valid for Submission
Code Describes
Other congenital malformation syndromes predominantly associated with short stature
Short Description
Other congen malform synd predom assoc with short stature
Parent Code
Q87.1
Congenital malformation syndromes predominantly associated with short stature

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ87Other specified congenital malformation syndromes affecting multiple systems
This CodeQ87.19Other congenital malformation syndromes predominantly associated with short stature

Present on Admission (POA)Billing

Q87.19 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations (Q80-Q89).

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 6q16 microdeletion syndrome
  • Aarskog syndrome
  • Absent thumb with short stature and immunodeficiency syndrome
  • Alopecia, contracture, dwarfism, intellectual disability syndrome
  • Anterior pituitary hormone deficiency
  • Aplasia of thumb
  • Ataxia, photosensitivity, short stature syndrome
  • Atkin Flaitz syndrome
  • Autosomal dominant Robinow syndrome
  • Autosomal recessive Robinow syndrome
  • Bent bone dysplasia group
  • BIDS brittle hair-impaired intellect-decreased fertility-short stature syndrome
  • Bird-headed dwarfism with progressive ataxia, insulin-resistant diabetes, goiter, and primary gonadal insufficiency
  • Blepharophimosis, ptosis, esotropia, syndactyly, short stature syndrome
  • Bowing deformity of lower leg
  • Brachydactyly, short stature, retinitis pigmentosa syndrome
  • Brachymorphism with onychodysplasia and dysphalangism syndrome
  • Chitty Hall Baraitser syndrome
  • Choanal atresia
  • Choanal atresia, athelia, hypothyroidism, delayed puberty, short stature syndrome
  • Cleft mandible
  • Cleft palate with short stature and vertebral anomaly syndrome
  • Cockayne syndrome
  • Cockayne syndrome type 1
  • Cockayne syndrome type 2
  • Cockayne syndrome type 3
  • Colobomatous microphthalmia
  • Congenital abnormal shape of frontal bone
  • Congenital abnormal shape of tibia
  • Congenital absence of thumb
  • Congenital bowing of tibia and/or fibula
  • Congenital dysplasia of nail unit
  • Congenital fusion of ribs
  • Congenital hypoplasia of anterior pituitary
  • Congenital malformation of angle of anterior chamber of eye
  • Congenital malformation of anterior pituitary
  • Congenital malformation syndromes associated with short stature
  • Congenital subaortic stenosis
  • Coxoauricular syndrome
  • CPE-related Prader-Willi-like syndrome
  • Cutaneous syndrome with ichthyosis
  • Dandy-Walker syndrome
  • De Lange syndrome
  • Defect of vertebral segmentation
  • Deletion of part of chromosome 6
  • Deletion of part of long arm of chromosome 6
  • Dentinogenesis imperfecta
  • Dentinogenesis imperfecta, short stature, hearing loss, intellectual disability syndrome
  • Developmental malformation of branchial arch
  • Dubowitz's syndrome
  • Dwarfism, alopecia, pseudoanodontia, cutis laxa
  • Dysmorphism, short stature, deafness, disorder of sex development syndrome
  • Dyssegmental dysplasia Silverman Handmaker type
  • Ear, face and neck congenital anomalies
  • Extrasystoles, short stature, hyperpigmentation, microcephaly syndrome
  • Finger joint locking
  • GEMSS syndrome
  • Genetic syndromic childhood obesity
  • GMS syndrome
  • Goniodysgenesis
  • Haspeslagh Fryns Muelenaere syndrome
  • Hennekam Beemer syndrome
  • Heritable disorder of neutrophil function
  • Hip pathological dislocation
  • Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome
  • Ichthyosis, short stature, brachydactyly, microspherophakia syndrome
  • KBG syndrome
  • Larsen-like osseous dysplasia, short stature syndrome
  • Lentiglobus
  • Loose anagen hair syndrome
  • Macrocephaly, short stature, paraplegia syndrome
  • MAGEL2-related Prader-Willi-like syndrome
  • Microphakia
  • Microspherophakia
  • Microtia
  • Morbid obesity
  • Mulibrey nanism syndrome
  • Multiple malformation syndrome with senile-like appearance
  • Multiple malformation syndrome, moderate short stature, facial
  • Multiple malformation syndrome, small stature, without skeletal dysplasia
  • Neck webbing
  • Noonan syndrome-like disorder with juvenile myelomonocytic leukemia
  • Noonan syndrome-like disorder with loose anagen hair
  • Noonan's syndrome
  • Oliver McFarlane syndrome
  • Osteodysplastic primordial dwarfism
  • Osteodysplastic primordial dwarfism, type 1
  • Prader-Willi-like syndrome
  • Robin sequence
  • Robinow syndrome
  • Rud's syndrome
  • Russell-Silver syndrome
  • Seckel syndrome
  • Severe lateral tibial bowing with short stature
  • Short stature Brussels type
  • Short stature locking fingers syndrome
  • Short stature with webbed neck and congenital heart disease syndrome
  • Short stature, Pierre Robin sequence, cleft mandible, hand anomalies, clubfoot syndrome
  • Short stature, pituitary and cerebellar defect and small sella turcica syndrome
  • Short stature, skeletal dysplasia, retinal degeneration, intellectual disability, sensorineural hearing loss syndrome
  • SHOX-related short stature
  • SIM1-related Prader-Willi-like syndrome
  • Sjögren-Larsson syndrome
  • Skeletal dysplasia with epilepsy and short stature syndrome
  • Spherophakia
  • Subaortic stenosis and short stature syndrome
  • Thong Douglas Ferrante syndrome
  • Trichothiodystrophy
  • Trigonocephaly
  • Trigonocephaly, short stature, developmental delay syndrome
  • Ullrich congenital muscular dystrophy
  • Urban Rogers Meyer syndrome
  • Warsaw breakage syndrome
  • Wiedemann Steiner syndrome
  • Xeroderma pigmentosum
  • Xeroderma pigmentosum and Cockayne syndrome complex
  • X-linked colobomatous microphthalmia, microcephaly, intellectual disability, short stature syndrome
  • X-linked intellectual disability, hypogonadism, ichthyosis, obesity, short stature syndrome

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q87.19: its own notes plus those printed at Q87.1, Q87, and Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • Aarskog syndrome
  • Cockayne syndrome
  • De Lange syndrome
  • Dubowitz syndrome
  • Noonan syndrome
  • Robinow-Silverman-Smith syndrome
  • Russell-Silver syndrome
  • Seckel syndrome

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Q87.1 Congenital malformation syndromes predominantly associated with short stature applies to 2 codes
  • Ellis-van Creveld syndrome (Q77.6)
  • Smith-Lemli-Opitz syndrome (E78.72)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

From Q87 Other specified congenital malformation syndromes affecting multiple systems applies to 21 codes
  • code(s) to identify all associated manifestations

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q87.19, its category, or a range that includes it.

Excludes1 9

These codes carry an Excludes1 note naming Q87.19: they are not reported together with it, unless the two conditions are unrelated.

  • E23.3 Hypothalamic dysfunction, not elsewhere classified
    Russell-Silver syndrome (Q87.19)
  • E34.3 Short stature due to endocrine disorder
    Russell-Silver syndrome (Q87.19)
  • E79 Disorders of purine and pyrimidine metabolism
    Ataxia-telangiectasia (Q87.19)
  • E79 Disorders of purine and pyrimidine metabolism
    Cockayne's syndrome (Q87.19)
  • Q81.0 Epidermolysis bullosa simplex
    Cockayne's syndrome (Q87.19)
  • Q96 Turner's syndrome
    Noonan syndrome (Q87.19)
  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q75 Other congenital malformations of skull and face bones
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q89.7 Multiple congenital malformations, not elsewhere classified
    congenital malformation syndromes affecting multiple systems (Q87.-) names Q87.-, which includes this code

Excludes2 1

These codes carry an Excludes2 note naming Q87.19: its condition is not part of those codes, and both may be reported when the patient has both.

  • G11.3 Cerebellar ataxia with defective DNA repair
    Cockayne's syndrome (Q87.19)

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Cockayne Syndrome

    a syndrome characterized by multiple system abnormalities including dwarfism; photosensitivity disorders; premature aging; and hearing loss. it is caused by mutations of a number of autosomal recessive genes encoding proteins that involve transcriptional-coupled dna repair processes. cockayne syndrome is classified by the severity and age of onset. type i (classical; csa) is early childhood onset in the second year of life; type ii (congenital; csb) is early onset at birth with severe symptoms; type iii (xeroderma pigmentosum; xp) is late childhood onset with mild symptoms.

  • De Lange Syndrome

    a syndrome characterized by growth retardation, severe mental retardation, short stature, a low-pitched growling cry, brachycephaly, low-set ears, webbed neck, carp mouth, depressed nasal bridge, bushy eyebrows meeting at the midline, hirsutism, and malformations of the hands. the condition may occur sporadically or be associated with an autosomal dominant pattern of inheritance or duplication of the long arm of chromosome 3. (menkes, textbook of child neurology, 5th ed, p231)

  • Noonan Syndrome

    a genetically heterogeneous, multifaceted disorder characterized by short stature, webbed neck, ptosis, skeletal malformations, hypertelorism, hormonal imbalance, cryptorchidism, multiple cardiac abnormalities (most commonly including pulmonary valve stenosis), and some degree of intellectual disability. the phenotype bears similarities to that of turner syndrome that occurs only in females and has its basis in a 45, x karyotype abnormality. noonan syndrome occurs in both males and females with a normal karyotype (46,xx and 46,xy). mutations in a several genes (ptpn11, kras, sos1, nf1 and raf1) have been associated the ns phenotype. mutations in ptpn11 are the most common. leopard syndrome, a disorder that has clinical features overlapping those of noonan syndrome, is also due to mutations in ptpn11. in addition, there is overlap with the syndrome called neurofibromatosis-noonan syndrome due to mutations in nf1.

  • Silver-Russell Syndrome

    genetically and clinically heterogeneous disorder characterized by low birth weight, postnatal growth retardation, facial dysmorphism, bilateral body asymmetry, and clinodactyly of the fifth fingers. alterations in genetic imprinting are involved. hypomethylation of igf2/h19 locus near an imprinting center region of chromosome 11p15 plays a role in a subset of silver-russell syndrome. hypermethylation of the same chromosomal region, on the other hand, can cause beckwith-wiedemann syndrome. maternal uniparental disomy for chromosome 7 is known to play a role in its etiology.

  • Sjogren-Larsson Syndrome

    an autosomal recessive neurocutaneous disorder characterized by severe ichthyosis mental retardation; spastic paraplegia; and congenital ichthyosis. it is caused by mutation of gene encoding microsomal fatty aldehyde dehydrogenase leading to defect in fatty alcohol metabolism.

Patient EducationClinical

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.

The full article covers:

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Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Code History & ChangesHistory

Replacement Q87.19 replaces the following previously assigned code(s):

  • Q87.1 - Congenital malform syndromes predom assoc w short stature
FY 2020AddedAdded to the ICD-10-CM code setEffective October 1, 2019.
FY 2021–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q87.19Overview

Is Q87.19 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other congenital malformation syndromes predominantly associated with short stature on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does Q87.19 group to?

When other congenital malformation syndromes predominantly associated with short stature is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q87.19 a CC or MCC?

CMS lists Q87.19 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 2 closely related codes in its exclusion list.

Is Q87.19 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for other congenital malformation syndromes predominantly associated with short stature on inpatient claims. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.

Can Q87.19 be reported with Q77.6?

Not as a rule. The Excludes1 note at Q87.1, which applies to Q87.19, lists "Ellis-van Creveld syndrome (Q77.6)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).