Short rib syndrome

This code represents a group of rare genetic disorders characterized by abnormally short ribs, often leading to thoracic insufficiency and respiratory distress. These conditions are typically associated with skeletal dysplasias, affecting bone growth and development throughout the body.

Assign this code when documentation specifies a diagnosis of short rib syndrome, such as Jeune syndrome (asphyxiating thoracic dystrophy), Sensenbrenner syndrome, or other related short rib-polydactyly syndromes. This diagnosis is typically made based on radiographic findings and clinical presentation.

• Ensure the medical record clearly identifies the specific type of short rib syndrome if known, as some may have more specific codes.
• Code any associated congenital anomalies or respiratory complications separately.
• Do not confuse with other forms of skeletal dysplasia that may involve rib abnormalities but do not fit the specific criteria for short rib syndrome.