[MIM 618 278]

Extremely rare. Acronym for FIbrosis, Neurodegeneration, Cerebral Angiomatosis

Autosomal recessive transmission of a mutation of the NHLRC2 gene (10q25.3).

Association of:

• pulmonary fibrosis of varying severity that can lead to death in the first months of life but is sometimes moderate (interstitial fibrosis with pseudogranulomas, sometimes alveolar proteinosis or cholesterol pneumonia)
• leptomeningeal angiomatosis with epilepsy, dystonia, developmental delay
• hepatomegaly (steatosis) with hepatic and renal dysfunction
• cardiomyopathy
• growth retardation
• hemolytic anemia
• frequent infections

Anesthetic implications:

check room air SpO2, chest XRay, hemoglobin and liver function; epilepsy

References : 

-        Horvath B, Kloesel B,
FINCA syndrome, a novel interstitial lung and multiorgan disease.
Pediatr Anesth 2022; 32: 976-7.

Updated: July 2022