E71.440

Approximate Synonyms

• Ruvalcaba myhre smith syndrome

• Ruvalcaba-myhre syndrome

Clinical Information

• A genetic syndrome caused by mutations in the pten gene. It is characterized by macrocephaly and the presence of hamartomas.

• A syndrome originally reported ruvalcaba) as an association of macrocephaly, intestinal polyposis, and pigmentation of the penis, bannayan later described it as a separate entity of macrocephaly, multiple lipomas, and hemangiomas, and riley and smith observed a concurrence of macrocephaly, pseudoepithelioma, and multiple hemangioma. The three conditions were recognized by dvir as different expressions of the same syndrome. The symptoms usually first become apparent in infancy but they may also appear later in life.

Details

• E71.440 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E71.440 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E71.440 – other international versions of ICD-10 E71.440 may differ.