CONGENITAL PTOSIS WITH SYNCHRONOUS 
MOVEMENTS OF THE AFFECTED LID 
AND LOWER JAW. 
BY 
ALVIN A. HUBBELL, M.D., 
PROFESSOR OF OPHTHALMOLOGY IN THE MEDICAL DEPARTMENT OF NIAGARA UNIVERSITY, 
BUFFALO, N. Y. 
( With three \ illustrations.) 
Reprinted from the Archives of Ophthalmology, Vol. xxii.. No. i, 1593. CONGENITAL PTOSIS WITH SYNCHRONOUS 
MOVEMENTS OF THE AFFECTED LID 
AND LOWER JAW. 
ALYIN A. HUBBELL, M.D., 
PROFESSOR OF OPHTHALMOLOGY IN THE MEDICAL DEPARTMENT OF NIAGARA UNIVERSITY, 
BUFFALO, N. Y. 
(With three illustrations.) 
T. W. B. is a boy now seven years of age. He is one of a 
family of three children whose parents are healthy and intelligent 
Americans. No malformation is present in any of them, except 
himself, and none is known to have existed in either the paternal 
or maternal ancestors. This boy is well developed, healthy, and 
unusually intelligent. He has a symmetrical and well-shaped 
head and face, and were it not for the ptosis on the right side he 
would be quite a handsome lad. Soon after birth it was noticed 
that the right eyelids did not open with the left, but would always 
remain closed, excepting when the mouth was opened, and then 
they would separate and close again with the closure of the 
mouth. This condition has continued without any apparent 
change to the present time. On examination I find that when 
the boy’s mouth is closed the right eyelids are also closed, and 
there is not the slightest movement of the upper lid when the 
left eyelids are ordinarily opened, or the left eyeball is directed 
upwards, or when any other movement is made by which the eye 
or head is turned, or the face drawn, or the lower jaw carried to 
one side. Figure i shows the appearance of the boy’s eyes when 
he is looking forward, with his mouth closed. If the boy is asked 
to open both eyes as widely as he can, the right upper lid is raised 
a little, as shown in Figure 2. But this is done entirely by the 
action of the occipito-frontalis muscle and not in the least by the 
Reprinted from the Archives of Ophthalmology, Vol. xxii., No. 1,1893. 66 
Alvin A. Huhhell. 
levator palpebrse. When, however, the mouth is opened, the right 
upper lid is involuntarily and unconsciously raised at once. The 
greater the depression of the lower jaw the higher is the upper 
lid lifted. In other words, the contraction of the right levator 
muscle takes place involuntarily with the contraction of the 
depressor (digastricus) of the lower jaw, and exactly proportion- 
Figure i. 
ately to that orthe latter. This action, however, can be overcome 
by the orbicularis palpebrarum, whose function is normal on both 
sides, since the right eye can be voluntarily kept closed while the 
mouth is opened. When the right eyelids are separated, the eye 
is seen to be of the same size, and the iris of the same color as 
the left ; but there is marked divergent strabismus together with Congenital Ptosis. 
67 
some downward deviation. The eye cannot be turned upwards 
beyond the median plane, or inwards but little beyond the vertical 
plane ; but downwards and outwards its movements are of usual, 
or perhaps more than usual extent. The pupil and iris are nor- 
mal in appearance, size, and reflexes, and are like the left. The 
refraction of both eyes is, by the ophthalmoscope, emmetropic. 
Figure 2. 
The fundus of the right eye is, like the left, normal in appearance 
in every respect. But the vision of this eye is amblyopic, being 
able barely to count fingers at four feet, while that of the left is 
normal, reading number 6, Snellen, at six metres (f). Figure 3 
shows the appearance of both eyes when the mouth is widely- 
opened. 68 
Alvin A. Huhhell. 
Remarks.—So far as I know, fifteen cases of this anomaly 
have been reported. While they have differed somewhat in 
some particulars, they have all had ptosis on one side with 
rising of the drooping lid synchronously with some move- 
ment of the lower jaw, generally depression. In no case 
has a post-mortem and histological examination of the brain 
Figure 3. 
been made. Hence the anatomical basis of these anomalous 
movements is at present merely conjectural. But taking 
into consideration the fact that the innervation for the 
levator palpebrae and the digastricus or other muscle of the 
lower jaw is in these cases undoubtedly one and the same, 
as shown by the associated movements of these muscles, Congenital Ptosis. 
69 
and that a part of the nucleus of the descending motor root 
of the fifth and the nucleus of the root of the third nerve 
are in close proximity to each other in the neighborhood of 
the aqueduct of Sylvius, the eupposition becomes very 
probable that an abnormal connection is here established. 
This abnormal nuclear transposition may vary in different 
cases, but it appears always to be from some part of the 
third to some part of the descending root of the fifth nerve. 
A lack of proper development of the nucleus of origin of 
more or less fibres of the third nerve appears also to be a 
prominent characteristic in all cases. In my own case I 
believe that the origin of the fibres supplying the levator 
muscle has been transferred from the nucleus of the third 
nerve to that part of the nucleus of the fifth which sends 
fibres to the anterior belly of the digastric muscle. The 
nucleus of the third nerve seems to be undeveloped in that 
portion from which arise the fibres supplying the levator of 
the upper lid, the superior rectus, and the internal rectus 
muscles, while that part sending fibres to the inferior rectus 
(possibly the inferior oblique) and the iris is normally 
present. Thus do we get in this case the ptosis, the diver- 
gent strabismus, the downward deviation, and the associated 
movements of the upper lid with the lower jaw. "Cbc ■fenichcrhocbcr (press, Hew gorfc 
G. P. Putnam’s Sons