2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Disorders Involving The Immune Mechanism 279.* > 2006 ICD-9-CM Diagnosis 279.11 Digeorge's syndrome View the most recent version of ICD-9-CM 279.11 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. --2004 Congenital syndrome with the absence of the thymus and parathyroids causing impairment of cellular immunity. Immunoglobulin levels are normal. Branchial arch defects with craniofacial abnormalities, hearing loss, short stature, and learning difficulty. 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). 279.11 is a specific code that can be used to specify a diagnosis 279.11 contains 5 index entries View the ICD-9-CM Volume 1 279.* hierarchy Alternate Terminology Pharyngeal pouch syndrome Thymic hypoplasia  Index entries containing 279.11: Di George's syndrome (thymic hypoplasia) 279.11 Hypoplasia, hypoplasis 759.89 thymus (gland) 279.11 Pharyngeal - see also condition pouch syndrome 279.11 Syndrome - see also Disease DiGeorge's (thymic hypoplasia) 279.11 pharyngeal pouch 279.11