ICD-11 Codes
for 5A00.0Z - Congenital hypothyroidism, unspecified

Teodor Jurukovski January 1, 1970

Fact checked by: Teodor Jurukovski

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Synonyms and Alternative Terms for ICD-11 Code 5A00.0Z

The ICD-11 code 5A00.0Z represents Congenital hypothyroidism, unspecified. Common clinical synonyms and patient-friendly terms include unspecified congenital thyroid hormone deficiency and thyroid hormone deficiency present at birth, unspecified.

Understanding Congenital Hypothyroidism, Unspecified (ICD-11 Code 5A00.0Z)

Congenital hypothyroidism, unspecified, identified by the ICD-11 code 5A00.0Z, refers to a condition where an infant is born with an underactive thyroid gland, meaning it does not produce sufficient thyroid hormone. The 'unspecified' designation indicates that the specific cause or type of this congenital condition has not been documented or determined. This condition requires prompt identification and management to prevent potential long-term health and developmental issues.

Common Symptoms and Presentation of Congenital Hypothyroidism

Infants with congenital hypothyroidism may exhibit a range of symptoms, although some may appear healthy at birth. Common clinical features can include:

• Decreased activity levels and increased sleepiness
• Feeding difficulties and constipation
• Prolonged jaundice after birth
• Characteristic facial features (myxedematous facies)
• Large fontanels (soft spots on the baby's head)
• Enlarged tongue (macroglossia)
• A distended abdomen, often with an umbilical hernia
• Reduced muscle tone (hypotonia)

The presentation can vary significantly among affected infants.

How Congenital Hypothyroidism, Unspecified is Diagnosed

The diagnosis of congenital hypothyroidism, particularly the unspecified type (ICD-11 code 5A00.0Z), typically begins with newborn screening programs. These programs routinely test infants for thyroid hormone levels shortly after birth. If screening indicates a potential issue, further diagnostic tests, primarily blood tests measuring thyroid hormone levels (like TSH and T4), are conducted to confirm the diagnosis. The 'unspecified' nature means that while hypothyroidism is confirmed, the underlying reason, such as a developmental defect or genetic factor, may not be identified or recorded.

Treatment and Management Considerations for Unspecified Congenital Hypothyroidism

The management of congenital hypothyroidism, even when unspecified (ICD-11 code 5A00.0Z), focuses on restoring adequate thyroid hormone levels. Treatment typically involves lifelong thyroid hormone replacement therapy, most commonly with levothyroxine. The dosage is carefully monitored and adjusted based on the infant's growth and thyroid hormone levels. Early and consistent treatment is crucial for normal physical and intellectual development. The unspecified nature of the cause does not alter the fundamental treatment approach, which is hormone replacement.

Documentation and Coding Considerations for ICD-11 Code 5A00.0Z

When documenting and coding congenital hypothyroidism where the specific cause or subtype is not identified, the ICD-11 code 5A00.0Z, Congenital hypothyroidism, unspecified, should be used. This code falls under the broader category of Hypothyroidism (5A00) within the ICD-11 classification system. Accurate documentation is essential for proper billing, statistical tracking, and continuity of care. This code is appropriate when a diagnosis of congenital hypothyroidism is established, but further details regarding its etiology are unavailable or not recorded in the patient's record.