2026 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 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

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 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 564 through 566. 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 other POA exempt codes.

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
  • Chronic deafness
  • 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 bowing of tibia and/or fibula
  • Congenital dysplasia of nail unit
  • 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
  • Disturbance of hair cycle
  • 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

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Aarskog's syndrome
    • Amsterdam dwarfism
    • Bonnevie-Ullrich syndrome
    • Bruck-de Lange disease
    • Cockayne's syndrome
    • Cornelia de Lange syndrome
    • De Lange's syndrome
    • Dubowitz' syndrome
    • Noonan's syndrome
    • Prader-Willi-like syndrome
    • Robinow-Silverman-Smith syndrome
    • Russell-Silver syndrome
    • Seckel's syndrome
    • Silver's syndrome
    • Sjögren-Larsson syndrome
    • Syndrome
      • Prader-Willi-like
    • Syndrome
      • pseudo -Turner's
    • Turner-like syndrome
    • Ullrich(-Bonnevie)(-Turner) syndrome
    • Weil(l)-Marchesani syndrome

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

  • Dentinogenesis Imperfecta

    an autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. the dentin develops poorly with low mineral content while the pulp canal is obliterated.

  • Choanal Atresia

    a congenital abnormality that is characterized by a blocked choanae, the opening between the nose and the nasopharynx. blockage can be unilateral or bilateral; bony or membranous.

  • 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.

  • Loose Anagen Hair Syndrome

    benign childhood alopecia that improves spontaneously with aging. it is characterized by anagen hairs (misshapen hair bulbs and absent inner and outer root sheaths), thin, and sparse hairs that pulls out easily.

  • 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)

  • Xeroderma Pigmentosum

    a rare, pigmentary, and atrophic autosomal recessive disease. it is manifested as an extreme photosensitivity to ultraviolet rays as the result of a deficiency in the enzyme that permits excisional repair of ultraviolet-damaged dna.

  • Xeroderma Pigmentosum Group A Protein

    a zinc finger motif protein that recognizes and interacts with damaged dna. it is a dna-binding protein that plays an essential role in nucleotide excision repair. mutations in this protein are associated with the most severe form of xeroderma pigmentosum.

  • Xeroderma Pigmentosum Group D Protein

    a dna helicase that is a component of transcription factor tfiih. it plays an essential role in nucleotide excision repair, and mutations in this protein are associated with xeroderma pigmentosum.

  • Morbid Obesity

    an excess of body weight, normally defined as an individual with a body mass index greater than 35 or a body weight greater than one hundred percent of ideal body weight.

  • Spherophakia

    a congenital disorder of the eye where the lens is abnormally small and spherical.

  • Weill-Marchesani Syndrome 1|Congenital Mesodermal Dysmorphodystrophy|Spherophakia-Brachymorphia Syndrome|Spherophakia-brachymorphia syndrome|Weill-Marchesani, Autosomal Recessive

    an autosomal recessive subtype of weill-marchesani syndrome caused by mutations in the adamts10 gene, encoding a disintegrin and metalloproteinase with thrombospondin motifs 10.

  • Dentinogenesis Imperfecta

    a congenital tooth development disorder caused by mutations in the dspp gene. the teeth are weak, discolored, and translucent.

  • DDX11 wt Allele|CHL1|CHL1-Like Helicase Homolog (S. cerevisiae) Gene|CHL1-Like Helicase Homolog Gene|CHL1-Related Helicase Gene 1|CHLR1|ChlR1|DEAD/H (Asp-Glu-Ala-Asp/His) Box Helicase 11 Gene|DEAD/H (Asp-Glu-Ala-Asp/His) Box Polypeptide 11 (CHL1-Like Helicase Homolog, S. cerevisiae) Gene|DEAD/H (Asp-Glu-Ala-Asp/His) Box Polypeptide 11 (S.cerevisiae CHL1-Like Helicase) Gene|DEAD/H (Asp-Glu-Ala-Asp/His) Box Polypeptide 11 Gene|DEAD/H-Box 11 Gene|DEAD/H-Box Helicase 11 wt Allele|KRG-2|KRG2|Keratinocyte Growth Factor Regulated Gene 2|Keratinocyte Growth Factor-Regulated Gene 2|Probable ATP-Dependent DNA Helicase DDX11 Gene|Probable ATP-Dependent RNA Helicase DDX11 Gene|WABS|Warsaw Breakage Syndrome Gene

    human ddx11 wild-type allele is located in the vicinity of 12p11.21 and is approximately 31 kb in length. this allele, which encodes atp-dependent dna helicase ddx11 protein, is involved in dna replication, dna repair, heterochromatin organization and ribosomal rna synthesis. mutation of the gene is associated with warsaw breakage syndrome.

  • Warsaw Breakage Syndrome

    an autosomal recessive condition caused by mutation(s) in the ddx11 gene, encoding atp-dependent dna helicase ddx11. it is characterized by severe intellectual disability and variable dysmorphic features.

  • Robinow Syndrome

    a rare autosomal recessive or dominant inherited disorder. the autosomal recessive form is caused by mutations in the ror2 gene. there is no causative mutation identified for the autosomal dominant form. it is manifested with short limbs, abnormal facial features, underdeveloped genitalia, and wedge-shaped vertebrae.

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:

  • What are birth defects?
  • What causes birth defects?
  • Who is at risk of having a baby with birth defects?
  • How are birth defects diagnosed?
  • What are the treatments for birth defects?
  • Can birth defects be prevented?

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 2021–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

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, 2025 through September 30, 2026.

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, 565, 566, with relative weights from 0.7493 to 1.5436 depending on complications. Higher weights mean higher Medicare reimbursement.

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.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.