What is Thymic Hypoplasia?

Thymic Hypoplasia
Image of a persistent large velopharyngeal gap causing velopharyngeal insufficiency in a patient with 22q11.2 deletion syndrome undergoing video nasopharyngeal endoscopy (PPW = posterior pharyngeal wall, VPG = velopharyngeal gap, SP = soft palate). Obstructive sleep apnea syndrome in children with 22q11.2 deletion syndrome after operative intervention for velopharyngeal insufficiency. Crockett DJ, Goudy SL, Chinnadurai S, Wootten CT - Frontiers in pediatrics (2014). Not Altered. CC.

Thymic hypoplasia is a condition in which the thymus is underdeveloped or involuted.

What is the Pathology of Thymic Hypoplasia?

The pathology of thymic hypoplasia is:

-Etiology: The cause of thymic hypoplasia may be due to genetic disorders or birth defects. 

-Genes: PAX1, TBX1, TBX2

-Pathogenesis: Lack of development and maturation of thymic tissue. This results in immature T cells from the bone marrow not being able to properly mature, which may lead to susceptibility to infections. 

-Histology: Unremarkable thymic tissue. 

How does Thymic Hypoplasia Present?

Patients with thymic hypoplasia typically present in immature infants. Underdevelopment of the thymus results in an increased infection risk. 

How is Thymic Hypoplasia Diagnosed?

Thymic hypoplasia is diagnosed by physical exam and ultrasound. 

How is Thymic Hypoplasia Treated?

Thymic hypoplasia is treated by immuno-precautions. 

What is the Prognosis of Thymic Hypoplasia?

The prognosis of thymic hypoplasia is good.

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Last updated on May 16, 2022