NEUROMA 
OF THE 
Right Upper Eyelid and Adjacent 
Temporal Region. 
By G. E. de SCHWEINITZ, M.D., 
OF PHILADELPHIA. 
\From Transactions of American Ophthalmological Society, 1891.] NEUROMA 
OF THE 
Right Upper Eyelid and Adjacent 
Temporal Region. 
By G. E. de SCHWEINITZ, M.D., 
OF PHILADELPHIA. 
[From Transactions of American Ophthalmological Society, 1891.] NEUROMA OF THE RIGHT UPPER EYELID AND 
ADJACENT TEMPORAL REGION. 
By G. E. de SCHWEINITZ, M. D., 
OF PHILADELPHIA. 
Strictly speaking, the term neuroma should be limited to 
tumors composed of new-formed nerve fibres, and, while in cer- 
tain instances true new formation of nerve tissue does occur, 
the majority of these growths are false neuromata, i. e., fibro- 
mata and myxomata of the connective tissue of the nerve, un- 
accompanied by multiplication of its nerve fibres. Indeed, the 
latter usually are compressed and atrophied by the surrounding 
tissue. Vernueil* gave the name "plexiform neuroma," and Paul 
Brunsf "Rankenneurom," or "Neuroma Cirsoideum" to a morbid 
process of this character when it appears in the form of a more 
or less convoluted mass, somewhat corded beneath the skin, and 
affecting an entire nerve territory. In a few instances this va- 
riety of the growth has been situated in the eyelid and surround- 
ing region, notably the temporal area. The researches of Von 
Recklinghausen have shown that multiple fibrous tumors of the 
skin often contain nerve fibres, or, to speak more accurately, are 
developed from the fibrous sheath of small cutaneous nerves, 
and thence involve the connective tissue structure of the ves- 
sels, sweat glands, and hair follicles, and are, in fact, neuro- 
fibromata. Tumors of this nature may invade the fibrous 
sheaths of the different tubular structures contained in the skin 
and sometimes reach a great size, until finally the appearance 
of elephantiasis is developed, which, to distinguish it from the 
acquired variety of this type of hypertrophy, is designated by 
some authors as "congenital elephantiasis." Under such cir- 
cumstances the tumors may not be circumscribed, but an 
overgrowth of the skin takes place, giving rise to an extensive 
hypertrophy. 
* Archives Ginerales de Medicine, 1886, Vol. II., V serie, T 18. 
t Virchow's Archiv., Vol. L, 1870, p. 80. Schweinitz : Neuroma of the Right Upper Eyelid. 
3 
To the cases of so-called plexiform neuroma of the eyelid 
already upon record I desire to add another case of congeni- 
tal tumor of this region, which presented itself in the form of 
considerable thickening of the eyelid, causing an extensive pto- 
sis and a morbid growth in the temporal region, clinically giv- 
ing rise to the appearance of a fibro-fatty tumor, or to that con- 
dition which has received the name elephantiasis, and which in 
rare instances has appeared as a congenital affection in the 
upper lid. Examination of the slides and of the photo-micro- 
graphs will, however, demonstrate that in addition to hypertro- 
phy of the glandular structures in the skin and of the connec- 
tive tissue, the most marked pathological lesions are large 
masses, which in cross section appear as concentric whorls of 
soft fibrous tissue, containing in their center more or less degen- 
erated nerve fibres. 
The history of the case is as follows: Edward Hawkins, 
aged 20, was admitted to the Philadelphia Hospital September 
2, 1890, and came under the care of my colleague, Dr. G. M. 
Gould, on account of the condition which is represented in the 
accompanying photograph, namely, a large, irregular tumor of 
the right temporal region, ptosis, and thickening of the upper 
lid and the tissue between it and the margin of the brow. When 
I took charge of the wards I obtained the following history: 
The tumor was present at birth, and, indeed, had not materially 
changed in its appearances except by its expansion with the 
corresponding growth of the tissues in which it was situated. 
The distance from the edge of the brow to the lower margin 
of the swollen eyelid was 7.5 cm.; the longest transverse di- 
ameter, i. e., from commissure to commissure, 9 cm., and the 
thickness varied from 1 to 1.5 cm. The temple tumor extended 
from the outer commissure to the ear, and from the margin of 
the hair to the zygoma. The skin over the area of the growth 
was slightly brown in color, a pigmentation which had devel- 
oped since the birth of the patient. To the palpitating fingers 
the underlying mass gave the impression of a somewhat tabula- 
ted, slightly corded growth. There was no pain and no incon- 
venience, save that caused by the ptosis. Both eyes were nor- 
mal in structure and vision, and there were no congenital faults 4 
Schweinitz : Neuroma of the Right Upper Eyelid. 
or tumors elsewhere in the body, neither had a similar growth 
been present in any member of the patient's family or ancestors. 
The palpebral conjunctiva of the affected lid presented the ap- 
pearances of a chronic blenorrhoea, and across the lid, on its 
conjunctival surface, there were three furrows, bounded by cor- 
responding ridges, and several transverse divisions, so that the 
whole presented a somewhat lobulated appearance. There was 
Fig. i. 
NEURO FIBROMA OF THE RIGHT UPPER EYE-LID AND ADJACENT TEMPORAL 
REGION. 
moderately free secretion of muco-pus. Some attempts to re- 
duce the size of the tumor by local blood-letting had met with 
indifferent success. The first operation was done January 6, 
1891, and consisted in removing a semi-lunar flap from the lid, 
dissecting out all of the affected tissue which it was possible, 
and afterwards approximating the edges by means of sutures. 
After the operation the long transverse diameter of the lid meas- 
ured 5 cm. The second operation was made January 20, 1891, 
and consisted in an attempt to overcome the ectropion which 
had resulted from the first operation. The outer commissure Schweinitz : Neuroma of the Right Upper Eyelid. 
5 
was slit for a distance of 5.5 cm., and from the end of the incis- 
ion a second one was carried forward and backward for 5 cm., a 
parallelogram of skin and subcutaneous tissue, together with the 
underlying tumorous mass, was dissected away from the temple, 
and the edges were approximated. Twenty millimeters of the 
thickened outer edge of the upper lid were cut off, the lower lid 
pared correspondingly, and the two united with sutures as in the 
Fig. 2. 
FROM A PHOTOGRAPH AFTER THE THIRD OPERATION. THE ARTIST IN RE 
DRAWING HAS SOMEWHAT EXAGGERATED THE EFFECT. 
operation for tarsorrhaphy. This operation relieved the ectro- 
pion, and when the patient looked directly forwards the lid fissure 
was separated about one centimetre. A third operation was done 
in April, 1890, which consisted in Panas's operation for ptosis 
slightly modified, owing to the thickened tissues.. This yielded 
a fair result, elevated the lid, and gave the boy a reasonable 
palpebral opening. 
The tumorous masses which had been dissected from the lid, 
and to less degree from the temple (the mother positively de- 
clined to allow the whole qaass from the temple to be removed) 6 
Schweinitz : Neuroma of the Right Upper Eyelid. 
were placed in Mueller's fluid, carefully hardened, and sectioned 
for the microscope, as were also the pieces of skin which had 
been removed from the temple and from the eyelid. The fol- 
lowing are the most important microscopic lesions: 
Integument of the Eyelid. The horny layer of the epidermis 
is thickened, uneven, its flat cells being somewhat discolored; 
both the granular layer and the rete mucosum are thicker and 
more prominent than is normal in the skin of the eyelid. As is 
natural to the part, the papillae of the pars papillaris are not 
well marked in this layer, and the pars reticularis of the corium 
is not readily separated; the entire corium appears to be 
composed of a loose fibrous connective tissue containing 
numerous wavy bundles, free corpuscles, and elastic fibres. 
Prominent features in this portion of the microscopic field 
are the hypertrophy of the sebiparous glands and the wide 
dilatation of the lymphatic spaces which sometimes assume 
large areas lined by distinct, flat, endothelial cells, in close rela- 
tion to wide-mouthed, thin-walled veins (Fig. 2, Pl. I). Here and 
there in some sections racemose and tubular glands with hyper- 
trophied walls are present. In the deeper portion of a section 
made directly through the integument, the first portions of the 
true tumor appear in the form of the strands presently to be de- 
scribed, which are the characteristic feature of the growth. 
Main Body of the Growth. This is composed of strands 
which pass through a framework of loose connective tissue con- 
taining fat cells and large blood-vessels, lymphatics, and muscle 
fibres. The strands themselves, when seen in cross section, 
are composed of concentric whorls of loose fibrous connective 
tissue containing many nuclei and in their center medulla- 
ted nerve fibres, sometimes intact and sometimes partially 
destroyed by fatty degeneration. Figure I, Pl. I represents the 
general outline of a cross section of several of the strands seen 
under a low magnifying power, while these other sections exhibit 
various appearances of the center of the whorls under a higher 
magnifying power. In order to positively demonstrate the pres- 
ence of medullated nerve fibres, which, however, are readily seen 
in many of the sections stained by a carmine nuclear dye, the 
method of Weigert was employed, and Figures 3 and 4, Pl. II Plate I. 
Figure i. Section of the tumor, showing the concentric whorls. 
[X 125-] 
Figure 2. Vertical section of the skin of the eyelid, showing 
hypertrophied gland and dilated lymph spaces. Plate II 
Figure 3. An oval whorl, showing a band of medullated nerve fibres 
passing through its centre. [X 250, Weigert's stain.] 
Figure 4. Small, oval whorl, showing in its centre a patch of 
nredullated nerve fibres. [X 125, Weigert's stain.] Schweinitz : Neuroma of the Right Upper Eyelid. 
7 
represent the various appearances elicited by this method 
of staining. In Figure 3 the oval whorl is seen to contain di- 
rectly in its center a band of medullated nerve fibres, while on 
either side the remains of the loose connective tissue, nuclei, 
and here and there some myxomatous change are visible. 
Additional sections represent, on the one hand, an oval whorl, 
and on the other an irregular-shaped whorl with nerve fibres in 
their centers, while in Figure 4 a small round whorl is depicted 
with the nerve fibres cut transversely and placed somewhat later- 
ally. All of the photo-micrographs were taken with apochromatic 
objectives, 16 mm., 8 mm., and 4 mm. of Zeiss, on orthochromatic 
gelatine dry plates, by sunlight illumination rendered monochro- 
matic. The photo-micrographic illustrations and the micro- 
scopic slides were prepared by Dr. William M. Gray of the 
Army Medical Museum. 
The literature of plexiform neuroma is not an extensive one. 
Marchand* has collected twelve cases, and Cartazf six cases, and 
in five, perhaps six, of these eighteen cases the growth has been 
situated in the region of the upper eyelid and temporal area. 
Since the paper of Marchand and Cartaz no very elaborate 
work on the subject has been done, although here and there 
scattered reports appear, not, however, with reference to a situ- 
ation in the eyelid. Interesting observations will be found in 
the papers of Christol,$ Garel,§ and especially in the research of 
Lacroix and Bonnaud,|| the latter observers having made a care- 
ful histological examination of a plexiform neuroma situated in 
the back in the neighborhood of the scapula, and presenting the 
rare characteristic of being largely composed of amyelinic nerve 
fibres. The development of plexiform neuroma belongs in the 
majority of instances to foetal life. Some hereditary tendency 
has been shown, but not with great definiteness. When this 
has existed it seems to have concerned that form of the tumor 
which partakes more of the nature of an elephantiasis than of a 
neuroma. When these tumors occur in the region with which 
we are at present interested, they appear in the form of a some- 
* Virchow's Archiv., Bd. LXX, page 36. 
+ Archives Generales de Medicine, August, 1876. 
t Gaz. Hebdomadaire, 1870. 
§ Lyon Medical, 1887. 
!l Archives de Medicine Expirimentale et d'Anatomie Pathologique, II., 1890, p. 411 8 
Schweinitz : Neuroma of the Right Upper Eyelid. 
what lobulated tumor, which gives the impression to the palpa- 
ting finger that the tumor is composed of fat and connective 
tissue, with perhaps occasionally an ill-defined sense of fluctua- 
tion. It may and has been mistaken for congenital lipoma, en- 
cephalocele, hydromeningocele, cystic tumor, and cavernous an- 
gioma. In fact, it would be practically impossible in any of the 
cases to have stated with certainty that the tumor was composed 
either in part or entirely of nerve fibres. Pain, as is usual with 
neuromata, was not a marked feature in any case, although ten- 
der spots have sometimes been found. As Lacroix and Bon- 
naud very properly insist, the diagnosis of a growth of this char- 
acter can rest alone upon a careful histological study. In a num- 
ber of such careful examinations it has been shown that the in- 
terstitial tissue between the cords or strands which form the 
characteristic gross appearance of the tumor, is composed of a 
fasciculated connective tissue of loose construction. The fasci- 
culi often arrange themselves in the neighborhood of the cords 
in a concentric manner, and around these, and separated from 
the cords themselves, are well-defined lymph spaces in which an 
endothelial lining could be detected. In one case the tissue of 
the tumor had a myxomatous character, and one observer has 
seen in the tumor a new connective tissue. Often in the inter- 
stitial substance well-formed sections of fat cells are found. Ves- 
sels are frequent. The cords, which are the essential part of the 
tumor, for the most part exhibit a clear peripheral zone which is 
composed of concentrically-arranged fibrillar connective tissue. 
Inward from this follow loose connective tissue strands accom- 
panied by many nuclei. Usually in the center, but sometimes 
irregularly-placed, medullated nerve fibres may be found in all 
stages of degeneration, but sometimes well preserved. Non- 
medullated nerve fibres have also been demonstrated, and, as has 
just been quoted, in one instance the growth was largely com- 
posed of them. Through the cords more or less rich vessel for- 
mation is present, and in the largest cords the nerve fibres may 
be poorly developed, or fail altogether. The skin which covers 
the tumor generally is in a condition of distinct hypertrophy, 
which especially concerns the connective tissue of the cutis. In 
some cases hair follicles and sweat glands are hypertrophied, 
and the epidermis often shows pigmentation in the deeper layers Schweinitz : Neuroma of the Right Upper Eyelid. 
9 
of the rete Malpighii. Various opinions in regard to other 
fine details have been recorded by different authors. Czerny 
and Winiwarter picture an increase of the cells of the vessel 
walls and the capillaries, arteries, and veins, which is also pres- 
ent in the subcutaneous tissue and the skin, and according to 
the latter observer, help in the new formation of the connective 
tissue. A similar development has been supposed to be present 
in the vessels of the muscles. Cell proliferation of the sheath 
of the nerve fibres and of the sarcolemma of the muscle fibres 
is present, and also contributes to the connective tissue forma- 
tion. Czerny and Winiwarter promulgate the hypothesis that 
the whole affection is a disease of the trophic nerves. Whether 
there is really a development of new-formed nerve fibres is a 
question. 
This resume of the microscopic lesions found in so-called 
plexiform neur'oma has been quoted in large part from March- 
and's monograph, and it will be seen that it accords with the 
histological characters of the tumor to which I call your atten- 
tion to-day, and which hence must be recognized as belonging 
to this group of cases. The association of the disease with 
fibroma and elephantiasis congenita has been referred to, and 
Bruns calls attention to the commingling of circumscribed ele- 
phantiasis mollis congenita and neuroma, in their relation to an 
elephantiasis proper, which consists of an alteration of the subcu- 
taneous connective tissue, or one in which there is enormous 
development of the vessels (elephantiasis teleangiectodes). 
Therefore, a congenital elephantiasis with excessive develop- 
ment of the nerve tissues may be classified as an elephantiasis 
neuromatodes ; and Bruns further recites from an observation of 
two clinical cases, that almost imperceptible transitions occur 
from simple elephantiasis to elephantiasis neuromatodes, or neu- 
roma elephantiasticum. A brief description of the most impor- 
tant cases of so-called plexiform neuroma of the eyelid and ad- 
jacent temporal region which have been recorded is here ap- 
pended : 
Case I. Boy, aged 6; tumor of the right upper eyelid 
reaching to the temple, composed of cords in the center of 
which was a nerve which had partly undergone fatty degenera- 
tion. (Billroth, Archiv. f. klin. Chirurg., 1863, IV., s. 547.) 10 
Schweinitz : Neuroma of the Right Upper Eyelid. 
Case II. Boy, aged 18 ; plexiform tumor of the upper eye- 
lid and the neighboring temporal region. When freed from fat 
it was seen to be composed of grayish-white, smooth cords of 
plexiform arrangement sometimes ending in fine nerves. The 
center of the cords was composed of atrophic and fatty degene- 
rated nerves. (Billroth, Archiv. f. klin. Chirurg., Bd. XI., 169, 
s. 232.) 
Case III. Man, aged 28 ; part of the tumor, which occurred 
in the temporal region, reaching to and affecting the outer pal- 
pebral commissure. (Bruns, loc. cit.) 
Case IV. Man, aged 33 ; tumor of the left upper eyelid 
and temporal region, and at the same time general neuromato- 
sis and multiple neuromas of both vagi. (Bruns, loc. cit.) 
Case V. A brother of the preceding case ; neuroma of the 
left upper eyelid and corresponding temporal region. (Bruns, 
loc. cit. 
Case VI. Boy, aged 13 ; tumor of the left upper eyelid and 
corresponding temporal region. (Marchand, loc. cit.) |