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This 1-year-old girl has a mass just inside the left lateral orbital rim.  This has been enlarging slowly over the last several months. She was treated for lacrimal adenitis but without any change in the clinical signs. Examination of the eyes was normal.  Further examination of the child revealed six tan colored flat pigmented “spots” on the trunk and the upper part of the arms. There was elicited no family history for the condition this child demonstrates.  A CT scan of the orbits was obtained.

1.	What is the most likely diagnosis?
	c -- neurofibromatosis The likely diagnosis in this case is neurofibromatosis I.  This a genetic disorder characterized by development of both benign and malignant tumors at a greater frequency than the general population.  In about half of the cases this condition is inherited as an autosomal dominant.  Neurofibromatosis II is characterized by acoustic neuroma, a first order relative affected, and sometimes a combined hamartoma of the retinal pigment.
2.	What is another thing you would look for in this child?
	b -- melanocytic hamartomas of the iris In neurofibromatous cases you are likely to see two or more melanocytic hamartomas of the iris.  These are called Lisch nodules, and are pathognomonic of this condition.
3.	How would you treat this patient?
	d -- watchful waiting and family counseling The best course is “watchful waiting” with computer tomography plus regular eye examinations, and treatment of complications of the disease, especially the occurrence and growth of plexiform neuromas.