2020 ICD-10-CM Diagnosis Code Q87.3

Congenital malformation syndromes involving early overgrowth

    2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
  • Q87.3 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 Q87.3 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of Q87.3 - other international versions of ICD-10 Q87.3 may differ.
Applicable To
  • Beckwith-Wiedemann syndrome
  • Sotos syndrome
  • Weaver syndrome
The following code(s) above Q87.3 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 Q87.3:
  • 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
  • Q87
    ICD-10-CM Diagnosis Code Q87

    Other specified congenital malformation syndromes affecting multiple systems

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Use Additional
    • code(s) to identify all associated manifestations
    Other specified congenital malformation syndromes affecting multiple systems
Approximate Synonyms
  • Beckwith wiedemann syndrome
  • Proteus syndrome
Clinical Information
  • A genetic syndrome caused by abnormalities in chromosome 11. It is characterized by large birth weight, macroglossia, umbilical hernia, ear abnormalities, and hypoglycemia. Patients with this syndrome have an increased risk of developing embryonal tumors (gonadoblastoma, hepatoblastoma, wilms tumor, rhabdomyosarcoma) and adrenal cortex carcinomas.
  • A rare genetic syndrome caused by mutations in the nsd1 gene. It is characterized by excessive physical growth early in life, mild mental retardation, and delayed cognitive and social development. Children affected by this syndrome are tall with larger than normal heads.
  • A rare, overgrowth disorder in which babies are large at birth and may develop low blood sugar. Other common symptoms are a large tongue, large internal organs, and defects of the abdominal wall near the navel. Beckwith-wiedemann syndrome increases the risk of developing certain cancers, especially wilms tumor.
  • A syndrome of multiple defects characterized primarily by umbilical hernia (hernia, umbilical); macroglossia; and gigantism; and secondarily by visceromegaly; hypoglycemia; and ear abnormalities.
  • A syndrome of overgrowth of prenatal onset, advanced bone age, retarded psychomotor development, widened distal long bones, camptodactyly, and distinctive craniofacial appearance marked by large ears, broad forehead, hypertelorism, and long philtrum. Weaver syndrome is considered a variant of the marshall-smith syndrome but, according to some authors, these are separate entities with share some common features, including are abnormal bone maturation, accelerated growth, and delayed development but differ in their craniofacial dysmorphism. Widened middle and proximal phalanges, failure to thrive, craniofacial abnormalities (small face, prominent eyes, blue sclera, flat nose with anteverted nares, choanal atresia, and glossoptosis). Respiratory disorders, hypertrichosis, and early death uniquely characterize the marshall-smith syndrome. A syndrome marked by symptoms similar to those in the weaver syndrome with hyperprogesteronemia and maternal luteoma and one with cleft lip, accessory nipples, pectus excavatum, bifid xiphoid process, abnormal vertebral bodies, and inflexible right thumb are referred to as the weaver-like syndrome.
  • Congenital or postnatal overgrowth syndrome most often in height and occipitofrontal circumference with variable delayed motor and cognitive development. Other associated features include advanced bone age, seizures, neonatal jaundice; hypotonia; and scoliosis. It is also associated with increased risk of developing neoplasms in adulthood. Mutations in the nsd1 protein and its haploinsufficiency are associated with the syndrome.
  • Enlarged tongue, omphalocele and other umbilical abnormalities, cytomegaly of the adrenal cortex, hyperplasia of gonadal interstitial cells, renal hyperplasia with medullary dysplasia, pancreatic hyperplasia, visceromegaly, and hypoglycemia with various neoplastic diseases and other disorders.
  • Increased birth weight with excessive growth during the first four years of life, macrocephaly, characteristic facial features, nonprogressive cerebral disorder and mental retardation.
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.
  • Q87.3 is considered exempt from POA reporting.
ICD-10-CM Q87.3 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 564 Other musculoskeletal system and connective tissue diagnoses with mcc
  • 565 Other musculoskeletal system and connective tissue diagnoses with cc
  • 566 Other musculoskeletal system and connective tissue diagnoses without cc/mcc

Convert Q87.3 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

Diagnosis Index entries containing back-references to Q87.3:
  • Beckwith-Wiedemann syndrome Q87.3
  • Soto's syndrome Q87.3 (cerebral gigantism)
  • Syndrome - see also Disease
    • Soto's Q87.3
  • Weaver's syndrome Q87.3

ICD-10-CM Codes Adjacent To Q87.3
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
Q87.19 Other congenital malformation syndromes predominantly associated with short stature
Q87.2 Congenital malformation syndromes predominantly involving limbs
Q87.3 Congenital malformation syndromes involving early overgrowth
Q87.4 Marfan's syndrome
Q87.40 …… unspecified
Q87.41 Marfan's syndrome with cardiovascular manifestations
Q87.410 Marfan's syndrome with aortic dilation
Q87.418 Marfan's syndrome with other cardiovascular manifestations
Q87.42 …… with ocular manifestations
Q87.43 …… with skeletal manifestation
Q87.5 Other congenital malformation syndromes with other skeletal changes
Q87.8 Other specified congenital malformation syndromes, not elsewhere classified
Q87.81 Alport syndrome

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