Anomalies of the Spinal Canal and Contents

Anomalies and Curiosities of Medicine
by George M. Gould, M.D., Walter L. Pyle, M.D.

(Page 28 of 53)

When there is a default in the spinal column, the vice of conformation is called spina bifida. This is of two classes: first, a simple opening in the vertebral canal, and, second, a large cleft sufficient to allow the egress of spinal membranes and substance. Figure 130 represents a large congenital sacral tumor.

Achard speaks of partial duplication of the central canal of the spinal cord. De Cecco reports a singular case of duplication of the lumbar segment of the spinal cord. Wagner speaks of duplication of a portion of the spinal cord.

Foot records a case of amyelia, or absence of the spinal cord, in a fetus with hernia cerebri and complete fissure of the spinal column. Nicoll and Arnold describe an anencephalous fetus with absence of spinal marrow; and Smith also records the birth of an amyelitic fetus.

In some persons there are exaggerated curvatures of the spine. The first of these curvatures is called kyphosis, in which the curvature is posterior; second, lordosis, in which the curvature is anterior; third, scoliosis, in which it is lateral, to the right or left.

Kyphosis is the most common of the deviations in man and is most often found in the dorsal region, although it may be in the lumbar region. Congenital kyphosis is very rare in man, is generally seen in monsters, and when it does exist is usually accompanied by lordosis or spine bifida. We sometimes observe a condition of anterior curvature of the lumbar and sacral regions, which might be taken for a congenital lordosis, but this is really a deformity produced after birth by the physiologic weight of the body. Figure 131 represents a case of lordosis caused by paralysis of the spinal muscles.

Analogous to this is what the accoucheurs call spondylolisthesis. Scoliosis may be a cervicodorsal, dorsolumbar, or lumbosacral curve, and the inclination of the vertebral column may be to the right or left. The pathologists divide scoliosis into a myopathic variety, in which the trouble is a physiologic antagonism of the muscles; or osteopathic, ordinarily associated with rachitis, which latter variety is generally accountable for congenital scoliosis. In some cases the diameter of the chest is shortened to an almost incredible degree, but may yet be compatible with life. Glover speaks of an extraordinary deformity of the chest with lateral curvature of the spine, in which the diameter from the pit of the stomach to the spinal integument was only 5 1/2 inches.

Supernumerary ribs are not at all uncommon in man, nearly every medical museum having some examples. Cervical ribs are not rare. Gordon describes a young man of seventeen in whom there was a pair of supernumerary ribs attached to the cervical vertebrae. Bernhardt mentions an instance in which cervical ribs caused motor and sensory disturbances. Dumerin of Lyons showed an infant of eight days which had an arrested development of the 2d, 3d, 4th, and 5th ribs. Cases of deficient ribs are occasionally met. Wistar in 1818 gives an account of a person in whom one side of the thorax was at rest while the other performed the movements of breathing in the usual manner.

In some cases we see fissure of the sternum, caused either by deficient union or absence of one of its constituent parts. In the most exaggerated cases these fissures permit the exit of the heart, and as a general rule ectopies of the heart are thus caused. Pavy has given a most remarkable case of sternal fissure in a young man of twenty-five, a native of Hamburg. He exhibited himself in one medical clinic after another all over Europe, and was always viewed with the greatest interest. In the median line, corresponding to the absence of sternum, was a longitudinal groove bounded on either side by a continuous hard ridge which articulated with the costal cartilages. The skin passed naturally over the chest from one side to another, but was raised at one part of the groove by a pulsatile swelling which occupied the position of the right auricle. The clavicle and the two margins of the sternum had no connections whatever, and below the groove was a hard substance corresponding to the ensiform cartilage, which, however, was very elastic, and allowed the patient, under the influence of the pectoral muscles, when the upper extremity was fixed, to open the groove to nearly the extent of three inches, which was more than twice its natural width. By approximating his arms he made the ends of his clavicles overlap. When he coughed, the right lung suddenly protruded from the chest through the groove and ascended a considerable distance above the clavicle into the neck. Between the clavicles another pulsatile swelling was easily felt but hardly seen, which was doubtless the arch of the aorta, as by putting the fingers on it one could feel a double shock, synchronous with distention and recoil of a vessel or opening and closing of the semilunar valves.

Madden pictures (Figs. 134 and 135) a Swede of forty with congenital absence of osseous structure in the middle line of the sternum, leaving a fissure 5 3/8 X 1 3/16 X 2 inches, the longest diameter being vertical. Madden also mentions several analogous instances on record. Groux's case was in a person of forty-five, and the fissure had the vertical length of four inches. Hodgen of St. Louis reports a case in which there was exstrophy of the heart through the fissure. Slocum reports the occurrence of a sternal fissure 3 X 1 1/2 inches in an Irishman of twenty-five. Madden also cites the case of Abbott in an adult negress and a mother. Obermeier mentions several cases. Gibson and Malet describe a presternal fissure uncovering the base of the heart. Ziemssen, Wrany, and Williams also record congenital fissures of the sternum.

Thomson has collected 86 cases of thoracic defects and summarizes his paper by saying that the structures deficient are generally the hair in the mammary and axillary regions, the subcutaneous fat over the muscles, nipples, and breasts, the pectorals and adjacent muscles, the costal cartilages and anterior ends of ribs, the hand and forearm; he also adds that there may be a hernia of the lung, not hereditary, but probably due to the pressure of the arm against the chest. De Marque gives a curious instance in which the chin and chest were congenitally fastened together. Muirhead cites an instance in which a firm, broad strip of cartilage resembling sternomastoid extended from below the left ear to the left upper corner of the sternum, being entirely separate from the jaw.

Some preliminary knowledge of embryology is essential to understand the formation of branchial fissures, and we refer the reader to any of the standard works on embryology for this information. Dzondi was one of the first to recognize and classify congenital fistulas of the neck. The proper classification is into lateral and median fissures. In a case studied by Fevrier the exploration of a lateral pharyngeal fistula produced by the introduction of the sound violent reflex phenomena, such as pallor of the face and irregular, violent beating of the heart. The rarest of the lateral class is the preauricular fissure, which has been observed by Fevrier, Le Dentu, Marchand, Peyrot, and Routier.

The median congenital fissures of the neck are probably caused by defective union of the branchial arches, although Arndt thinks that he sees in these median fistulas a persistence of the hypobranchial furrow which exists normally in the amphioxus. They are less frequent than the preceding variety.

The most typical form of malformation of the esophagus is imperforation or obliteration. Van Cuyck of Brussels in 1824 delivered a child which died on the third day from malnutrition. Postmortem it was found that the inferior extremity of the esophagus to the extent of about two inches was converted into a ligamentous cord. Porro describes a case of congenital obliteration of the esophagus which ended in a cecal pouch about one inch below the inferior portion of the glottidean aperture and from this point to the stomach only measured an inch; there was also tracheal communication. The child was noticed to take to the breast with avidity, but after a little suckling it would cough, become livid, and reject most of the milk through the nose, in this way almost suffocating at each paroxysm; it died on the third day.

In some cases the esophagus is divided, one portion opening into the bronchial or other thoracic organs. Brentano describes an infant dying ten days after birth whose esophagus was divided into two portions, one terminating in a culdesac, the other opening into the bronchi; the left kidney was also displaced downward. Blasius describes an anomalous case of duplication of the esophagus. Grashuys, and subsequently Vicq d'Azir, saw a dilatation of the esophagus resembling the crop of a bird.