Congenital central alveolar hypoventilation syndrome

This code represents a rare, life-threatening neurological disorder characterized by a failure of automatic control of breathing, particularly during sleep. Individuals with this syndrome experience inadequate ventilation, leading to hypoxemia and hypercapnia, without an apparent primary lung or heart disease. It is present from birth, often requiring lifelong ventilatory support.

Use this code for patients diagnosed with congenital central alveolar hypoventilation syndrome, also known as Ondine's Curse. Documentation should clearly state the congenital nature of the central hypoventilation and rule out other causes of respiratory failure. This code is appropriate when the condition is the primary reason for the encounter or a significant co-morbidity.

• Ensure documentation specifies the "congenital" aspect to differentiate from acquired central hypoventilation.
• Do not confuse with other forms of sleep apnea or respiratory insufficiency unless the underlying etiology is explicitly identified as congenital central alveolar hypoventilation.
• Consider sequencing this code as the primary diagnosis when it is the focus of treatment, especially for ventilator management or related complications.