Ultrasound of Lipomatosis of Nerve Associated with Macrodactyly: ‘Spaghetti and Chocolate Cookie’ Appearance

We studied the US imaging findings of lipomatosis of nerve and macrodystrophia lipoma in three patients. Three patients were seen at three affiliated institutions with an enlarged digit. They all underwent US, subsequently followed by an MRI study. The nerves showed marked enlargement and extension over a length of 9-16 cm. Digital branches were always involved. The appearance on cross section was an enlarged hyperechoic endoneurium with inlying thickened and hypoechoic fascicles. On transverse images this resulted in a 'chocolate cookie' aspect and in the long axis a 'spaghetti-like' image. The US appearance of lipomatosis of nerve, not unlike MRI, is rather typical. With US, care should be taken in areas that are more difficult to assess as the sole of the foot, or where the 'chocolate cookie' appearance is not so obvious, such as the digits. Keywords: Lipomatosis of nerve Fibrolipomatous hamartoma, US Macrodystrophia lipomatosa Macrodactyly, US