T. Lewis and D. Embleton 37 
with complete syndactyly of the remaining two groups of toes, and an irregular 
though often symmetrical deformity of the hands. Its marked tendency to become 
hereditary will be subsequently discussed. 
Dealing first with those cases which have been reported as individual members 
of deformed fiimiiies (see Bibliography, Section A), and including our own with 
them, the following conclusions may be drawn. All four extremities show 
deformity in the vast majority of cases. In a few one hand and both feet are 
affected'-* (V, 38)*, in a slightly greater number of instances the feet are alone 
abnormal ■'• (III, 7). The hands are never defective in the absence of foot 
deformity-f-. One foot is never malformed alone. The feet tend to illustrate 
one of three main types of malformation. Of these the commonest is that in 
which all metatarsals except the 1st and 5th are absent (Figs. 8, 9), or rather the 
1st and 5th metatarsals are alone definitely represented. In the second type the 
main deformity falls on the 2nd toe (Figs. 20, 21, 24, 25), in the third type 
on the 3rd toe (Figs. 15, 16). The last two types are of about equally common 
occurrence|. Althovigh these main types may be isolated intermediate conditions 
remain. For descriptive purposes it may be said that, starting with the 2nd or 
3rd toe as a basis, the lesion is extended§ to the adjacent toes in the order of their 
proximity. First the phalanges and then their metatarsals disappear. When one 
toe is completely absent in all its jjarts, which is the rule, the neighbouring 
phalanges are rarely if ever unaffected. Lastly this exception must be added, 
that the 1st toe tends in the vast majority of cases to escape. In a few instances 
this toe also is shortened||, and in some has been absent'*' " (V, 24 and 31). In 
Perthes' case there was, in addition, affection of the corresponding cuneiform, 
navicular bone and astragalus, with some loss from the lower end of the tibia, 
but his case stands alone in this respect. Absence of the 5th toe must be equally 
rare. It may be shortened by the loss of one or more phalanges. Also it has 
happened that one or more phalanges have appeared to be absent when the 4th 
toe seemed to have its full complement (IV, 23), but it is doubtful if in these 
cases the phalanges have not been displaced. It is an almost rigid rule that a 
toe has fewer bony representatives according as it approaches the centre of the 
cleft. When the two outer toes remain intact they may be syndactylised by 
bone, but in any case they are joined by skin. There are frequently remains 
* The small number refers to the bibliography, the bracketed figures to the individuals in Plate I, 
Fig. 1- 
t This statement is made somewhat reservedly. There is no reported case in which the hands are 
described and the feet are stated to he normal. If it does occur it must be quite exceptional. 
J In comparing the frequency of types, the tendency in some families for one type to predominate, 
and the number of deformed members in each family have been taken into account. The examples 
referred to (Figs. 15 and 16) are not so decisive as many which occur in other families. 
§ In speaking of the extension of the deformity it must be clearly understood that the word is used 
purely for descriptive purposes and is in no way intended to imply that such extension has actually 
taken place. 
II On the other hand it may be lengthened in exceptional cases by reason of the presence of three 
phalanges. 
