A case of multiple neuro-fibromata of the ulnar nerve / by W.W. Keen and William G. Spiller.

  • Keen, William W. (William Williams), 1837-1932.
Date:
1900
Catalogue details

Licence: In copyright

Credit: A case of multiple neuro-fibromata of the ulnar nerve / by W.W. Keen and William G. Spiller. Source: Wellcome Collection.

Provider: This material has been provided by The Royal College of Surgeons of England. The original may be consulted at The Royal College of Surgeons of England.

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    muscles. Fibromata inollusca were found in both cases. In one patient the right lower limb had a circumference of 75 cm. and the left 22 cm., and the right lower limb was greatly deformed. Jordan was unable to classify these cases under any of the three forms of elephantiasis (elephantiasis fibromatosa, teleangiectodes, neuromatodes) known to him. Microscopically the condition was one of elephantiasis fibromatosa with multiple fibromata mollusca, multiple neuromata, and thickening of the large vessels ; microscopically the vessels were found to be the structures iu which the hyperplasia began, so that in both cases there was a com- bination of different forms of congenital elephantiasis. Jordan doubts whether cases of congenital elephantiasis in which only the skin and subcutaneous tissues are affected exist—-i. e., cases of solitary pachyder- matocele. Herczel1 was able to observe that iu a pachydermatocele the prolifera- tion of the connective tissue originated in the thickened fibrous nerve bands; that it was a true elephantiasis neuromatodes. The frequency of pigment nsevi in generalized fibromatosis has been noted by many writers, and in a careful microscopical study Soldan2 has recently shown that these pigment nsevi (Pigmentmaler) are in the majority of cases the first recognizable signs of a fibromatous process of the connective tissue of the nerves. The different forms of neuro- fibromatosis are conditioned by the localization, anatomical relations, and energy of growth. This brief summary of some of the most important writings on gen- eralized neuro-fibromatosis is sufficient to show that we are justified in classing under one head the neuro-fibromata of the nerves, the cutaneous fibromata, the plexiform neuromata, certain forms of elephantiasis, and certain pigment mevi. It does not follow, however, that all cutaneous fibromata arise in the nerves. The causes of generalized neuro-fibromatosis are unknown. Refer- ence has been made to the fact that heredity plays a role in some fami- lies, and iu addition to the cases cited I may mention that Menkes observed neuromata in members of three generations—grandmother, mother, and son—and he says a heredity through three generations ex- isted only in the cases cited by Herczel, Bruns, and Czerny. Most writers agree that the condition is not usually an acquired one. The tendency exists from the birth of the person, although the proliferation of connective tissue may not be observed until comparatively late in life. Trauma may in some cases cause the manifestation of a latent tendency in nerves. Another important question that demands attention is in relation to 1 Herczel. Ziegler's Beitriige, vol. viii. - Soldan. Arcliiv ftir kiln. Chirurgie, vol. lix., No. 2, p. 261. •'< Meuke. Berliner klin. Wochenschrift, October 31, 1898, No. 44, p. 974.