What does Neurocutaneous mean?

Neurocutaneous syndrome is a broad term for a group of rare neurological (brain, spine, and peripheral nerve) disorders. These diseases are lifelong conditions that can cause tumors to grow inside the brain, spinal cord, organs, skin, and skeletal bones. The most common disorders found in children are skin lesions.

What are the Phakomatoses?

Phakomatoses, or phacomatosis pigmentovascularis (PPV), is the term used for a group of rare syndromes involving structures arising from the embryonic ectoderm. These are characterised by vascular and pigmentary birthmarks or skin lesions, and often involving multiple organ systems in the body.

Is tuberous sclerosis the same as neurofibromatosis?

Neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC) represent two neurocutaneous disorders in which affected individuals develop tumors at an increased frequency.

What causes Neurocutaneous syndrome?

The 3 most common types of neurocutaneous syndromes are tuberous sclerosis (TS), neurofibromatosis (NF), and Sturge-Weber disease. These diseases are all present at birth (congenital). They are caused by gene changes. A neurocutaneous syndrome is a lifelong condition that has no cure.

What are Neurocutaneous markers?

Neurocutaneous syndromes are disorders that affect the brain, spinal cord, organs, skin, and bones. The diseases are lifelong conditions that can cause tumors to grow in these areas. They can also cause other problems such as hearing loss, seizures, and developmental problems.

What is Sturge Weber Syndrome?

Sturge-Weber syndrome is a condition that affects the development of certain blood vessels, causing abnormalities in the brain, skin, and eyes from birth.

What are Neurocutaneous disorders?

What is Neurocutaneous Melanosis?

Neurocutaneous melanosis (NCM) is a rare disorder characterized by the presence of benign or malignant pigment cell tumors of the leptomeninges in association with large or multiple CMN (3).

What is an enterocutaneous fistula?

An enterocutaneous fistula (ECF) is an abnormal connection that develops between the intestinal tract or stomach and the skin. As a result, contents of the stomach or intestines leak through to the skin. Most ECFs occur after bowel surgery.

How is spontaneous nephrocutaneous fistula associated with complete staghorn calculus diagnosed?

The author presents a case of spontaneous nephrocutaneous fistula associated with a complete staghorn calculus in a nonfunctioning kidney. A renal scan, an intravenous pyelogram, and a right retrograde pyelogram confirmed the need for a nephrectomy. The procedure and results are described here.

What is neurocutaneous melanosis?

This site is in-development and may not reflect the final version. Neurocutaneous melanosis (NCM) is a rare, non- inherited condition of the central nervous system. It is characterized by melanocytic nevi in both the skin and the brain.

Can a fistula cause a barium swallow?

Enterocutaneous fistulas (ECFs) can cause contents of the intestines or stomach to leak through a wound or opening in the skin. It also can cause: Barium swallow, also called an esophagram. This test is a series of X-rays of the esophagus.