2021 ICD-10-CM Diagnosis Code D82.1

Di George's syndrome

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • D82.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM D82.1 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of D82.1 - other international versions of ICD-10 D82.1 may differ.
Applicable To
  • Pharyngeal pouch syndrome
  • Thymic alymphoplasia
  • Thymic aplasia or hypoplasia with immunodeficiency
The following code(s) above D82.1 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 D82.1:
  • D50-D89
    2021 ICD-10-CM Range D50-D89

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

    Type 2 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • certain conditions originating in the perinatal period (P00-P96)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • human immunodeficiency virus [HIV] disease (B20)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
  • D80-D89
    2021 ICD-10-CM Range D80-D89

    Certain disorders involving the immune mechanism

    Includes
    • defects in the complement system
    • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
    • sarcoidosis
    Type 1 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • functional disorders of polymorphonuclear neutrophils (D71)
    • human immunodeficiency virus [HIV] disease (B20)
    Certain disorders involving the immune mechanism
  • D82
    ICD-10-CM Diagnosis Code D82

    Immunodeficiency associated with other major defects

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    Immunodeficiency associated with other major defects
Approximate Synonyms
  • Digeorge sequence
  • Digeorge syndrome
  • Digeorges syndrome
Clinical Information
  • A congenital anomaly characterized by immunodeficiency, abnormal facies, congenital heart disease, hypocalcemia, and increased susceptibility to infections. Pathologic characteristics include conotruncal abnormalities and absence or hypoplasia of thymus and parathyroid glands. Digeorge syndrome is associated with abnormalities of chromosome 22. Also known as digeorge anomaly.
  • A developmental defect of derivatives of the third and fourth pharyngeal pouches, almost always associated with agenesis or hypoplasia of the thymus and parathyroid gland, characteristic facies with downslanting palpebral fissures and ocular and nasal anomalies, hypocalcemia, cardiovascular anomalies, immunodeficiency, and other variable abnormalities. Patients who survive infancy are usually mentally retarded. Digeorge syndrome is considered by some researchers as a developmental field defect consisting of several casually distinct disorders, rather than a distinct syndromic entity. Conditions associated with the development of digeorge syndrome include diabetic embryopathy, fetal alcohol syndrome, and zellweger syndrome. Major features of this syndrome have been designated by the newcastle upon tyne group catch 22 (cardiac, abnormal facies, thymic hypoplasia, cleft palate, and hypocalcemia), the number 22 indicating deletion of the long arm of chromosome 22 (22q11).
  • Branchial arch defects with craniofacial abnormalities, hearing loss, short stature, and learning difficulty.
  • Congenital syndrome characterized by a wide spectrum of characteristics including the absence of the thymus and parathyroid glands resulting in t-cell immunodeficiency, hypocalcemia, defects in the outflow tract of the heart, and craniofacial anomalies.
  • Congenital syndrome with the absence of the thymus and parathyroids causing impairment of cellular immunity; immunoglobulin levels are normal.
ICD-10-CM D82.1 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 808 Major hematological and immunological diagnoses except sickle cell crisis and coagulation disorders with mcc
  • 809 Major hematological and immunological diagnoses except sickle cell crisis and coagulation disorders with cc
  • 810 Major hematological and immunological diagnoses except sickle cell crisis and coagulation disorders without cc/mcc

Convert D82.1 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
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to D82.1:
  • Type 1 Excludes: Q38.7
    , E20
    , E32
    , E32.8
    ICD-10-CM Diagnosis Code Q38.7

    Congenital pharyngeal pouch

      2016 2017 2018 2019 2020 2021 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital diverticulum of pharynx
    Type 1 Excludes
    • pharyngeal pouch syndrome (D82.1)
    ICD-10-CM Diagnosis Code E20

    Hypoparathyroidism

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • Di George's syndrome (D82.1)
    • postprocedural hypoparathyroidism (E89.2)
    • tetany NOS (R29.0)
    • transitory neonatal hypoparathyroidism (P71.4)
    ICD-10-CM Diagnosis Code E32

    Diseases of thymus

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • aplasia or hypoplasia of thymus with immunodeficiency (D82.1)
    • myasthenia gravis (G70.0)
    ICD-10-CM Diagnosis Code E32.8

    Other diseases of thymus

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Type 1 Excludes
    • aplasia or hypoplasia with immunodeficiency (D82.1)
    • thymoma (D15.0)

Diagnosis Index entries containing back-references to D82.1:
  • Alymphocytosis D72.810
    ICD-10-CM Diagnosis Code D72.810

    Lymphocytopenia

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Decreased lymphocytes
    • thymic D82.1 (with immunodeficiency)
  • Alymphoplasia, thymic D82.1
  • Aplasia - see also Agenesis
    • parathyroid-thymic D82.1
    • thymic, with immunodeficiency D82.1
  • Di George's syndrome D82.1
  • DiGeorge's syndrome D82.1 (thymic hypoplasia)
  • Dysplasia - see also Anomaly
    • thymic, with immunodeficiency D82.1
  • Hypoplasia, hypoplastic
    • thymus (gland) Q89.2
      ICD-10-CM Diagnosis Code Q89.2

      Congenital malformations of other endocrine glands

        2016 2017 2018 2019 2020 2021 Billable/Specific Code POA Exempt
      Applicable To
      • Congenital malformation of parathyroid or thyroid gland
      • Persistent thyroglossal duct
      • Thyroglossal cyst
      Type 1 Excludes
      • congenital goiter (E03.0)
      • congenital hypothyroidism (E03.1)
      • with immunodeficiency D82.1
    • thymic, with immunodeficiency D82.1
  • Pharyngeal pouch syndrome D82.1
  • Syndrome - see also Disease
    • di George's D82.1
    • pharyngeal pouch D82.1

ICD-10-CM Codes Adjacent To D82.1
D81.7 Major histocompatibility complex class II deficiency
D81.8 Other combined immunodeficiencies
D81.81 Biotin-dependent carboxylase deficiency
D81.810 Biotinidase deficiency
D81.818 Other biotin-dependent carboxylase deficiency
D81.819 …… unspecified
D81.89 Other combined immunodeficiencies
D81.9 Combined immunodeficiency, unspecified
D82 Immunodeficiency associated with other major defects
D82.0 Wiskott-Aldrich syndrome
D82.1 Di George's syndrome
D82.2 Immunodeficiency with short-limbed stature
D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus
D82.4 Hyperimmunoglobulin E [IgE] syndrome
D82.8 Immunodeficiency associated with other specified major defects
D82.9 Immunodeficiency associated with major defect, unspecified
D83 Common variable immunodeficiency
D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
D83.1 Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells
D83.8 Other common variable immunodeficiencies

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