92 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH 



extreme dorsal flexion and shortening of the trunk. The head is drawn back close 

 to the sacral region. The chest and abdomen are unusually prominent. The arms 

 and legs are symmetrical and well developed, but the shoulders are hunched up and 

 lie far forward, close to the cheeks. The face is directed upward, which throws the 

 top of the head back so that the vertex lies level with the raised shoulders. The 

 neck is obliterated and the chin and chest lie in one plane. The features are well 

 formed with the exception of lack of prominence of the chin and deformity of the 

 ears. Figures 1 and 8 show the right ear. The deformity of the left ear is similar. 

 The anthelix is pushed outward so as to be unusually prominent; the tragus is 

 shifted medially and upward, so that it lies opposite the concha; the antitragus 

 lies below it, pressed against the cheek. Darwin's tubercle is present. The exter- 

 nal auditory meatus is patent and the parts of the middle and inner ear prove on 

 dissection to be well developed. The whole external ear is considerably narrowed, 

 as is indicated by as low a physiognomical index as 48.5. The average physiog- 

 nomical index of the right ear of 14 white infants under 3 weeks of age, in the 

 obstetrical ward of the Johns Hopkins Hospital, was found to be 69.1, varying 

 between 62.5 and 78.7. Measurements made by Dr. A. H. Schultz of 4 dead 

 white infants not older than 1 month showed the physiognomical index of the 

 right ear to be 65.0, with a variation between 60.0 and 73.1. Though the physi- 

 ognomical index shows a rather wide variation due to the great flexibility of the 

 ear cartilage in infants, nowhere in the small group of available normal cases is 

 it nearly so low as in his specimen. The ear deformity is apparently caused by 

 pressure upon and twisting of the external parts of the ear during their develop- 

 ment by the backward-bent head and the shoulders which lie close on either side. 

 Marx describes a deformed ear which he designates as "Wildermiithsche Ohr, " in 

 which the anthelix is very prominent. From the base of each ear a crease in the 

 skin extends for 3 cm. medially under the chin to within 2 cm. of the rnidline, as 

 is seen in figure 2. 



INTEGUMENT. 



The dome of the head is narrowed and flattened, and is covered with light- 

 brown hair, 1 cm. in length. Just above and behind the ears the hair is 2 cm. long 

 and is quite thick. Across the middle of the forehead at the hair margin is a narrow 

 raised ridge of puckered skin, 2 cm. long. A section through this area shows the 

 structures of the skin to be well developed and similar to the adjoining normal 

 skin, except that where the surface is raised the papillae are somewhat flattened 

 underneath. The ridge is probably the result of rough handling before fixation. 



From the back of the head protrude three encephaloceles in a horizontal line, 

 extending from a point 6 cm. behind the tip of the left ear to a point 2 cm. 

 behind the tip of the right ear. These are best displayed in figures 1 and 3. They 

 measure 13 cm. horizontally along their superior margin, and the vertical diameter 

 is 4.3 cm. The midline of the specimen comes between the left and middle enceph- 

 aloceles. In the midline their vertical diameter extends from a point 3.6 cm. below 

 the vertex to within 6 cm. of the anus. The middle swelling is the largest of the 



