2024 ICD-10-CM Diagnosis Code Q87.19

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• 6q16 microdeletion syndrome
• Aarskog syndrome
• Absent thumb with short stature and immunodeficiency syndrome
• Alopecia, contracture, dwarfism, intellectual disability syndrome
• Anterior pituitary hormone deficiency
• 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
• Chronic deafness
• Cleft mandible
• Cleft palate with short stature and vertebral anomaly syndrome
• Cockayne 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 blepharophimosis
• Congenital bowing of tibia and/or fibula
• Congenital hypoplasia of cerebrum
• Congenital leg bone bowing
• 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
• Cutaneous mastocytosis
• 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
• 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
• Extrasystoles, short stature, hyperpigmentation, microcephaly syndrome
• Finger joint locking
• GEMSS syndrome
• 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
• Lentiglobus
• Loose anagen hair syndrome
• Macrocephaly, short stature, paraplegia syndrome
• MAGEL2-related Prader-Willi-like syndrome
• Microphakia
• Microphakia
• Microspherophakia
• Microspherophakia
• Microtia
• Mulibrey nanism syndrome
• Multiple malformation syndrome with senile-like appearance
• Multiple malformation syndrome with senile-like appearance
• Multiple malformation syndrome with senile-like appearance
• Multiple malformation syndrome with senile-like appearance
• Multiple malformation syndrome with senile-like appearance
• Multiple malformation syndrome, moderate short stature, facial
• Multiple malformation syndrome, moderate short stature, facial
• Multiple malformation syndrome, moderate short stature, facial
• Multiple malformation syndrome, moderate short stature, facial
• 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
• 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
• SHOX-related short stature
• SIM1-related Prader-Willi-like syndrome
• Sjögren-Larsson syndrome
• Skeletal dysplasia with epilepsy and short stature syndrome
• Spherophakia
• 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

Clinical Information

• 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.

• 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.

• 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.

Q87.19 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Present on Admission (POA)

Q87.19 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

Replacement Code

Q8719 replaces the following previously assigned ICD-10-CM code(s):

• Q87.1 - Congenital malform syndromes predom assoc w short stature

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020