E23.0 - Hypopituitarism

Approximate Synonyms

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

• Acquired central hypothyroidism
• Acquired central hypothyroidism due to pituitary disorder
• Acquired central hypothyroidism due to Sheehan syndrome
• ACTH deficiency
• Adult growth hormone deficiency
• Adult growth hormone deficiency with onset in childhood
• Adult-onset growth hormone deficiency
• Alopecia and intellectual disability with hypergonadotropic hypogonadism syndrome
• Amenorrhea due to congenital gonadotrophin releasing hormone deficiency
• Amenorrhea due to hypothalamic disorder
• Anemia of endocrine disorder
• Anemia of pituitary deficiency
• Anterior pituitary hormone deficiency
• Asexual dwarfism
• Ateleiotic dwarfism
• Ateliotic dwarfism without insulinopenia
• Autoimmune hypopituitarism
• Autosomal dominant isolated somatotropin deficiency
• Autosomal recessive isolated somatotropin deficiency
• Axonal neuropathy
• Combined pituitary hormone deficiency genetic form
• Congenital disorder of facial nerve
• Congenital facial nerve palsy
• Congenital hypogonadotropic hypogonadism
• Congenital hypoplasia of nose
• Congenital malformation of anterior pituitary
• Congenital stenosis of carotid artery
• Deficiency in anterior pituitary function, variable immunodeficiency syndrome
• Dilated cardiomyopathy with hypergonadotropic hypogonadism syndrome
• Female hypogonadotropic hypogonadism
• Female infertility of pituitary - hypothalamic origin
• Follicle stimulating hormone deficiency
• Functional hypogonadotropic hypogonadism
• Gonadotropin releasing factor deficiency
• Growth hormone deficiency
• Growth hormone deficiency after bone marrow transplant
• Growth hormone neurosecretory dysfunction
• Hereditary growth hormone deficiency
• Hypergonadotropic hypogonadism with cataract syndrome
• Hypogonadism with anosmia
• Hypogonadotropic hypogonadism
• Hypogonadotropic hypogonadism due to follicle-stimulating hormone deficiency
• Hypogonadotropic hypogonadism due to isolated gonadotropin deficiency
• Hypogonadotropic hypogonadism due to luteinizing hormone deficiency
• Hypogonadotropic hypogonadism retinitis pigmentosa syndrome
• Hypogonadotropic hypogonadism with frontoparietal alopecia syndrome
• Hypogonadotropic hypogonadism, severe microcephaly, sensorineural hearing loss, dysmorphism syndrome
• Hypomyelination, hypogonadotropic hypogonadism, hypodontia syndrome
• Hypophysitis
• Hypopituitarism
• Hypopituitarism due to granulomatous disease
• Hypopituitarism due to metabolic disease
• Hypopituitarism due to pituitary tumor
• Hypopituitarism due to vascular disorder
• Hypopituitarism following procedure
• Hypoplasia of eye
• Hyposmia, nasal and ocular hypoplasia, hypogonadotropic hypogonadism syndrome
• Idiopathic growth hormone deficiency
• Idiopathic hypogonadotropic hypogonadism
• Idiopathic hypopituitarism
• Idiopathic panhypopituitarism
• Immunodeficiency associated with multiple organ system abnormalities
• Immunodeficiency with major anomalies
• Immunoglobulinemia with isolated somatotropin deficiency
• Internal carotid artery stenosis
• Isolated follicle stimulating hormone deficiency
• Isolated gonadotropin deficiency
• Isolated lutropin deficiency
• Isolated prolactin deficiency
• Isolated somatotropin deficiency
• Kallman syndrome with heart disease
• Late-onset isolated adrenocorticotropic hormone deficiency
• LH - luteinizing hormone deficiency
• Loss of sense of smell
• Lymphocytic hypopituitarism
• Male hypergonadotropic hypogonadism, intellectual disability, skeletal anomaly syndrome
• Martsolf syndrome
• Microcephalus, hypergonadotropic hypogonadism, short stature syndrome
• Moebius syndrome, axonal neuropathy, hypogonadotropic hypogonadism syndrome
• Morbid obesity
• Moyamoya angiopathy, short stature, facial dysmorphism, hypergonadotropic hypogonadism syndrome
• Moyamoya disease
• Myopathy in hypopituitarism
• Necrosis of pituitary
• Non-acquired combined pituitary hormone deficiency, sensorineural hearing loss, spine abnormalities syndrome
• Nonfamilial asexual dwarfism
• Nonfamilial hyperinsulinemic isolated somatotropin deficiency
• Panhypopituitarism
• Panhypopituitarism - anterior and posterior
• Partial growth hormone deficiency
• Partial hypopituitarism
• Partial loss of hair
• Peripheral axonal neuropathy
• Pituitary cachexia
• Pituitary deficiency due to empty sella turcica syndrome
• Pituitary dwarfism
• Pituitary dwarfism with large sella turcica
• Pituitary dwarfism with normal somatotropin level AND low somatomedin
• Pituitary dwarfism with small sella turcica
• Post-birth injury hypopituitarism
• Post-infarction hypopituitarism
• Post-infarction hypopituitarism
• Post-infarction panhypopituitarism
• Post-infective hypopituitarism
• Postpartum hypopituitarism
• Post-traumatic hypopituitarism
• Post-traumatic hypopituitarism
• Prepuberal panhypopituitarism
• Primary hypergonadotropic hypogonadism and partial alopecia syndrome
• Primary pituitary - hypothalamic infertility
• Primary testicular failure
• Proopiomelanocortin deficiency syndrome
• Psychosocial growth hormone deficiency
• RAB18 deficiency
• RHYNS syndrome
• Secondary hypopituitarism
• Secondary pituitary - hypothalamic infertility
• Sequelae of endocrine disorders
• Sequelae of endocrine disorders
• Sheehan's syndrome
• Short stature co-occurrent and due to endocrine disorder
• Transient somatotropin deficiency
• X-linked panhypopituitarism

Clinical Information

• Hypopituitarism-. diminution or cessation of secretion of one or more hormones from the anterior pituitary gland (including lh; follicle stimulating hormone; somatotropin; and corticotropin). this may result from surgical or radiation ablation, non-secretory pituitary neoplasms, metastatic tumors, infarction, pituitary apoplexy, infiltrative or granulomatous processes, and other conditions.
• Autoimmune Hypophysitis-. immune-mediated inflammation of the pituitary gland often associated with other autoimmune diseases (e.g., hashimoto disease; graves disease; and addison disease).
• Hypophysitis-. inflammation of the pituitary gland.
• Moyamoya Disease-. a noninflammatory, progressive occlusion of the intracranial carotid arteries and the formation of netlike collateral arteries arising from the circle of willis. cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. it is characterized by endothelial hyperplasia and fibrosis with thickening of arterial walls. this disease primarily affects children but can also occur in adults.
• Pituitary Gland-. a small, unpaired gland situated in the sella turcica. it is connected to the hypothalamus by a short stalk which is called the infundibulum.
• Congenital Facial Nerve Palsy-. partial or complete paralysis of the facial muscles of one side of an individual's face that is present at birth. it is caused by damage to the seventh cranial nerve.
• Growth Hormone Neurosecretory Dysfunction-. reduced spontaneous secretion of growth hormone with normal range response to growth hormone provocative stimuli.
• Autoimmune Hypophysitis|Lymphocytic Hypophysitis-. an autoimmune condition affecting the pituitary gland, characterized by lymphocytic infiltration, commonly presenting with pituitary hormone deficiencies.
• Grade 1 Hypophysitis, CTCAE|Grade 1 Hypophysitis-. asymptomatic or mild symptoms; clinical or diagnostic observations only; intervention not indicated
• Grade 2 Hypophysitis, CTCAE|Grade 2 Hypophysitis-. moderate; minimal, local or noninvasive intervention indicated; limiting age-appropriate instrumental adl
• Grade 3 Hypophysitis, CTCAE|Grade 3 Hypophysitis-. severe or medically significant but not immediately life-threatening; hospitalization or prolongation of existing hospitalization indicated; limiting self care adl
• Grade 4 Hypophysitis, CTCAE|Grade 4 Hypophysitis-. life-threatening consequences; urgent intervention indicated
• Grade 5 Hypophysitis, CTCAE|Grade 5 Hypophysitis-. death
• Hypophysitis-. an inflammatory process in the pituitary gland.
• Hypophysitis, CTCAE|Hypophysitis-. a disorder characterized by inflammation and cellular infiltration of the pituitary gland.
• Lymphocytic Neurohypophysitis-. an autoimmune condition affecting the posterior pituitary gland, which is characterized by lymphocytic infiltration, and which often presents as diabetes insipidus.
• 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.
• Pituitary Dwarfism-. proportionately decreased bodily growth due to failure of the pituitary gland to produce an adequate supply of growth hormone.
• Acute Motor and Sensory Axonal Neuropathy|Acute Motor And Sensory Axonal Neuropathy|Acute Motor-Sensory Axonal Neuropathy|Acute Motor-Sensory Axonal Neuropathy-. a subtype of guillain-barre syndrome that targets sensory motor axons, and is characterized by acute onset of quadriparesis, distal sensory loss, areflexia, and respiratory insufficiency.
• Acute Motor Axonal Neuropathy|AMAN-. a subtype of guillain-barre syndrome that targets motor axons, and is characterized by symmetric limb weakness, diffuse areflexia, facial and oropharyngeal muscle weakness, and respiratory insufficiency.
• Axonal Neuropathy-. any nerve disorder affecting the axon of a nerve.
• GAN wt Allele|GAN1|Giant Axonal Neuropathy (Gigaxonin) Gene|Gigaxonin wt Allele|KLHL16-. human gan wild-type allele is located in the vicinity of 16q24.1 and is approximately 65 kb in length. this allele, which encodes gigaxonin protein, is involved in both ubiquitination and neurofilament structure. mutation of the gene is associated with giant axonal neuropathy.
• Giant Axonal Neuropathy-. a rare inherited disorder affecting the neurofilaments. it is caused by mutations in the gan gene. it is characterized by the presence of abnormally large nerve cell axons. signs and symptoms include difficulty walking, sensory disturbances, lack of motor coordination and abnormal reflexes in the limbs.
• Spinocerebellar Ataxia, Autosomal Recessive, with Axonal Neuropathy 2|AOA2|Ataxia with Oculomotor Apraxia Type 2|SCAN2-. an autosomal recessive condition caused by mutation(s) in the setx gene, encoding probable helicase senataxin. it is characterized by juvenile onset progressive cerebellar ataxia, axonal sensorimotor peripheral neuropathy, and increased concentrations of serum alpha-fetoprotein. oculomotor apraxia is common, but is not always present.
• Moyamoya Disease-. a rare inherited vascular disorder characterized by constriction of arteries at the base of the brain, resulting in the formation of collateral circulation in order to compensate for the constriction. the name "moyamoya" in japanese means "puff of smoke" and derives from the characteristic radiographic appearance of the collateral vessels.
• Moyamoya Disease 2|MYMY2-. an autosomally inherited subtype of moyamoya disease often presenting in childhood caused by mutation(s) in the rnf213 gene, encoding e3 ubiquitin-protein ligase rnf213.
• RNF213 wt Allele|ALK Lymphoma Oligomerization Partner on Chromosome 17 Gene|ALO17|C17orf27|Chromosome 17 Open Reading Frame 27 Gene|DKFZp762N1115|FLJ13051|KIAA1554|KIAA1618|MGC46622|MGC9929|MYMY2|MYSTR|Moyamoya Disease 2 Gene|NET57|Ring Finger Protein 213 wt Allele|hCG_1812857-. human rnf213 wild-type allele is located in the vicinity of 17q25.3 and is approximately 135 kb in length. this allele, which encodes e3 ubiquitin-protein ligase rnf213 protein, may play a role in the regulation of protein ubiquitination. a chromosomal translocation t(2;17)(p23;q25) of this gene with the alk gene is associated with anaplastic large cell lymphoma.
• Acquired Central Hypothyroidism-. central hypothyroidism, the cause of which is not present at birth.
• Congenital Hypogonadotropic Hypogonadism-. insufficient production of estrogen or testosterone in the ovaries or testes due to decreased secretion of gonadotropins as a result of pituitary or hypothalamus gland dysfunction that is present at birth.
• Isolated Follicle Stimulating Hormone Deficiency-. subnormal concentration of follicle stimulating hormone (fsh), associated with mutations in the fshb gene, encoding follitropin subunit beta.

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC). applicable from 10/01/2022 through 09/30/2023.

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Patient Education

Pituitary Disorders

Your pituitary gland is a pea-sized gland at the base of your brain. The pituitary is the "master control gland" - it makes hormones that affect growth and the functions of other glands in the body.

With pituitary disorders, you often have too much or too little of one of your hormones. Injuries can cause pituitary disorders, but the most common cause is a pituitary tumor.

Combined pituitary hormone deficiency

Combined pituitary hormone deficiency is a condition that causes a shortage (deficiency) of several hormones produced by the pituitary gland, which is located at the base of the brain. A lack of these hormones may affect the development of many parts of the body. The first signs of this condition include a failure to grow at the expected rate and short stature that usually becomes apparent in early childhood.

People with combined pituitary hormone deficiency may have hypothyroidism, which is underactivity of the butterfly-shaped thyroid gland in the lower neck. Hypothyroidism can cause many symptoms, including weight gain and fatigue. Other features of combined pituitary hormone deficiency include delayed or absent puberty and lack the ability to have biological children (infertility). The condition can also be associated with a deficiency of the hormone cortisol. Cortisol deficiency can impair the body's immune system, causing individuals to be more susceptible to infection.

Rarely, people with combined pituitary hormone deficiency have intellectual disability; a short, stiff neck; or underdeveloped optic nerves, which carry visual information from the eyes to the brain.

Isolated growth hormone deficiency

Isolated growth hormone deficiency is a condition caused by a severe shortage or absence of growth hormone. Growth hormone is a protein that is necessary for the normal growth of the body's bones and tissues. Because they do not have enough of this hormone, people with isolated growth hormone deficiency commonly experience a failure to grow at the expected rate and have unusually short stature. This condition is usually apparent by early childhood.

There are four types of isolated growth hormone deficiency differentiated by the severity of the condition, the gene involved, and the inheritance pattern.

Isolated growth hormone deficiency type IA is caused by an absence of growth hormone and is the most severe of all the types. In people with type IA, growth failure is evident in infancy as affected babies are shorter than normal at birth.

People with isolated growth hormone deficiency type IB produce very low levels of growth hormone. As a result, type IB is characterized by short stature, but this growth failure is typically not as severe as in type IA. Growth failure in people with type IB is usually apparent in early to mid-childhood.

Individuals with isolated growth hormone deficiency type II have very low levels of growth hormone and short stature that varies in severity. Growth failure in these individuals is usually evident in early to mid-childhood. It is estimated that nearly half of the individuals with type II have underdevelopment of the pituitary gland (pituitary hypoplasia). The pituitary gland is located at the base of the brain and produces many hormones, including growth hormone.

Isolated growth hormone deficiency type III is similar to type II in that affected individuals have very low levels of growth hormone and short stature that varies in severity. Growth failure in type III is usually evident in early to mid-childhood. People with type III may also have a weakened immune system and are prone to frequent infections. They produce very few B cells, which are specialized white blood cells that help protect the body against infection (agammaglobulinemia).

Code History

• 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
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)