94 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH 



encephaloceles as well. In addition to this, in the adjoining areas there are hyper- 

 trichosis and thickened subcutaneous fat, varying from 5 to 25 mm. in thickness. 

 The wall of the large encephalocele varies from 3 to 9 mm. in thickness and is formed 

 by angiomatous tissue covered with a thin layer of epidermis penetrated by sweat- 

 glands. In two places near the tip of the sac nsevi are formed by the vascular 

 tissue extending to the surface. The walls of the small sacs vary from 3 to 20 mm. 

 and are formed chiefly by subcutaneous fat covered with scalp. On the left a pore 

 and canal pierce through to the subdural space. 



Spietscka in 1894 collected the various forms of skin changes associated with 

 spina bifida. Besides the varieties here found, he described pigment blotches and 

 such a marked increase of fatty tissue as to amount to lipomata. In an article on 

 skin anomalies by Bettmann, in Schwalbc's Morphologic der Missbildungen, naevi 

 are noted as among the most frequent anomalies. 



METHODS. 



A sagittal section was made of the specimen under discussion (see fig. 9). 

 The spinal column shows extreme lordosis, undeveloped arches throughout, and 

 shortening and fusion of the upper vertebrae. The central nervous system is very 

 much disturbed, a large part of it having slipped down below the cranium, through 

 a much enlarged foramen magnum. This portion lies on the thoracic and lumbar 

 vertebrae and protrudes into the sacs already described. The brain and cord were 

 removed, and a clay impression was made of the entire space occupied by the central 

 nervous system. This was then cast in wax and photographed, as shown in figures 

 4, 5, 6, and 7. By the help of this model the general shape taken by the central 

 nervous system was demonstrated and the study of its internal arrangement and 

 relation to other structures was facilitated. The consideration of these will be 

 taken up later in this paper. 



In the sagittal section, thick subcutaneous pads of fat are seen in the undif- 

 ferentiated region of the neck between chin and thorax, above the symphysis, and 

 over the sacral region. Dissection shows this subcutaneous fat to be likewise 

 particularly abundant over the back and shoulders. There is also found an extreme 

 grade of undeveloped or (split soft palate, associated with which is a bilateral 

 anlage of the uvula, that on the left side being shown in figure 9 a. Consideration 

 of the normal development of the soft palate will help to indicate how this defect 

 originated. It is generally agreed that at a very early date the tongue occupies 

 the area which is later occupied by the septum and palate. The normal rearrange- 

 ment of these parts to their final positions is accomplished by medial growth of the 

 palate and downward growth of the septum, associated with independent shifting 

 of the tongue. If for any reason the tongue can not withdraw, the palate remains 

 split to a greater or less degree. That such a cause was operative in this specimen 

 seems likely ; the distorted position of the cervical spine might easily have caused a 

 crowding in the adjoining pharyngeal region and so prevented the tongue from 

 receding. 



