As we enter 2004, we enter the centennial year of one of the landmark
papers in the history of von Hippel-Lindau disease. The first step
in solving a problem is to have a good description of the problem.
Eugen von Hippel wrote in 1904 “about a very rare disease
of the retina” and in 1911 added “the anatomical basis”
of that disease, which he named angiomatosis retinae. He studied
one extended family, with several generations exhibiting numerous
lesions typical of those of von Hippel-Lindau Disease (VHL).
Dr. von Hippel was honored in his lifetime as a physician, clinician,
teacher and researcher. He spent his entire life in service to others.
Eugen von Hippel was the son of another exceptional ophthalmologist,
Arthur von Hippel (1841-1917), professor of ophthalmology, founder and
head of the University Eye Clinic in Göttingen, later succeeded in
that post by his son Eugen. Eugen studied at the best schools —
Giessen, Freiburg, Berlin, Heidelberg, and Göttingen — and
received his doctorate in medicine in 1890.
As a young physician he first worked in pathology under Dr. Arnold at
Heidelberg. During this time he studied cadavers with severe infections,
which influenced the course of his later research. In 1892 he began to
specialize in ophthalmology and became assistant to Dr. Leber. He studied
and lectured in ophthalmology and won a prize for his research.
He became fascinated with disorders in formation of the eye, including
hydrophthalmus (a form of glaucoma), corectopia (abnormal situation of
the pupil), corneal defects, congenital cataracts of various types, and
angiomatosis of the retina, which has become known as von Hippel’s
disease. He not only took great pains to describe these malformations
in exquisite detail, but also sought to determine just how they came about,
and how they might be tied to damage during embryonic development. His
paper on angiomatosis retinae was published in 1895.
From 1909 to 1914 he taught at Halle and published his research in a
series of publications dealing with the diagnostic signs and clinical
results of these disorders. Later he taught at Göttingen, and produced
a series of studies on sympathetic ophthalmia, tuberculosis of the eye,
and chorioid membrane sarcoma, and on diseases of the optic nerve. He
contributed to a number of textbooks, notably on diseases of the optic
nerve, pathological anatomy of the cornea, and malformations of the eye.
In his tribute to Dr. von Hippel, Dr. Baurmann of Karlsruhe admires
him for his astounding breadth and richness of creativity. He took an
active role in the work of his students, helping them think and talk through
the problems they were tackling. He demanded the best efforts of everyone,
physicians, nurses, and all other employees of the clinic. He instilled
in everyone the spirit of respect for accomplishment in everything they
did. This resulted in a very unusual harmony and positive mutual cooperation
which benefited the work of the clinic in very favorable ways. His students
went out into practice “equipped not only with a broad knowledge
of clinical medicine, but above all with the ability to think critically
and scientifically, and all had learned in that clinic how to gain self-confidence.”
Prepared with the kind assistance of Dr. Hartmut P. H.
Neumann.