various operations for its relief. While no complete statistics are given, the impression is conveyed that recovery is fairly frequent. It must be that these operations have been performed upon a few simple cases gleaned by survival from the very large number that succumb in the early weeks of life. Of 649 children that died of spina bifida in England prior to 1882, 612 died within the first year1. A very large majority, with or without operation (temporarily successful), die from the various complications about to be noted. Bayer had a mortality of 59 per cent in 17 operated cases. refused to operate except: 1. when there is no paralysis; 2. when there is no decided hydrocephalus; 3. when clinically no complications are to be expected in the sac (i.e.: only operating on simple meningoceles). Sachtleben in 1902 reported 30 cases from Mikulicz's clinic. The five permanently cured were simple meningoceles. Geo. Woolsey states that the prognosis in general is unfavorable. Most cases fortunately die early. Rarely have cases been reported in which paresis of the sphincters or lower limbs have been cured or improved by the operation. Hence, with a few exceptions, about all that can be expected from the operation is the relief of an annoying and unsightly tumor and the prevention of meningitis from rupture or ulceration. Among the records of 91,600 confinements in the Lying-in Hospital at or near term, spina bifida occurred 59 times. Nine more were sent in for operation from various outside sources. For completeness, the description and results of the entire series of 68 cases are herewith presented. Explanation of the origin of the condition is impossible or at least. obscure. It can readily be seen that there is a defect of development combined in some rare varieties with an irregular excess of development, but beyond this we know little. A study of the specimens at our command has led us no further into the teratogenesis. Clinically it is notorious that after surgical closure of the defect in the tissues, either a hydrocephalus is very apt to develop, or one already present is pretty certain to grow progressively worse. This occurs also with simple meningoceles where there is no apparent structural change in the spinal cord itself. It would appear possible that the mechanism of the outgo of the spinal fluid was in some way interferred with by the defect at the lower end of the spine. On the other hand, N. Sharpes by experimental studies on the lower animals is led to believe, "that the majority, if not all of spina bifidae are caused by the pressure exerted by an excessive secretion of spinal fluid." We must remember, however, in this connection that there are many instances of extreme hydrocephalus, occurring in the fetus the pressure of which can be relieved by spinal puncture and yet are quite unaccompanied by any evidence of spina bifida. Whether the excessive secretion or increased pressure of the spinal fluid is the cause or the effect of the spinal deformity, it undoubtedly is the most unavoidable complication interfering with the surgical cure of these cases. It is customary to divide spina bifida into different varieties according to structure. The following classification of J. W. Ballantyne is concise and commendable. I. Holo-Rhachischisis or Total Spina Bifida: the whole canal is wide and open and there is no sac. II. Hemi-Rhachischisis, Mero-Rhachischisis, or Partial Spina Bifida. A. Spina Bifida Occulta or Crypto-Mero-Rhachischisis. B. Spina Bifida without Spinal Hernia: the canal is widely open at one or more places, but there is no sac. C. Spina Bifida with Rachocele (common variety). 1. Meningo-myelocele (common sub-variety). 2. Meningocele. 3. Myelo-cystocele, Syringo-myelocele, or Myelocele. Under this classification, the hospital series presents three cases of holo-rhachischisis, forty-two cases of meningo-myelocele, and eight of simple meningocele. Spina bifida occulta is very apt to escape unrecognized at birth, to be called to attention later in life by the development of a heavy growth of hair at its site and by the occurrence of paraphlegias and trophic ulcers of the feet, It will be noticed that 79 per cent were of the meningo-myelocele variety and 15 per cent of the simple meningocele variety. The other forms are rare. In fifteen of the series, the variety of the spina bifida is not described in the histories. Eliminating all early abortions, ectopics and hydatidiform moles, there were 59 spina bifidae born in the hospital service in a series of 91,600 confinements at or near term, an incidence of one in 1,552. Obstetrically considered, several points of interest are to be noted. First, in 58 observed presentations, 13 were breech, or 22 per cent. Second, of the entire series of 68, 35 were at full term, 17 in the ninth lunar month of development and 12 were distinctly premature. Third, the condition of excessive amniotic fluid, or hydramnios, is frequently associated with monsters whose cerebro-spinal canals are defective; as in anencephalus and spina bifida. The fetus is usually premature when the spinal defect is above the lumbar region, and especially when complicated with other deformities of defect, such as gastroschisis, iniencephaly, etc. The cases happen to be just equally divided as to sex, 34 males and 34 females. All the iniencephalics with complete spina bifida both in the hospital series and in two additional museum specimens, not delivered in the hospital, were females. In 48 cases, the location of the tumor is described as follows: 26 were lumbo-sacral, 10 were sacral, 7 were lumbar, 3 were dorso-lumbar, and 3 were total. It is evident that the dorsal and cervical location is quite rare, except as part of a total spina bifida. The ages and parity of the mothers apparently have no bearing on the condition, though it might be of interest to observe that 37.5 per cent of the cases were born of primiparae. The average age of these primiparae was 24 years. The other congenital abnormalities which so frequently accompany that of spina bifida seriously complicate the prognosis. These are chiefly hydrocephalus, both spastic and complete flaccid paralysis of the legs, talipes equinus, paralysis of the anal sphincter, imperforate anus, congenital luxation of the hip, iniencephaly, gastroschisis and anencephaly. There were 23 instances of primary recognizable hydrocephalus at birth and ten more that developed after operation, being unobserved Fig. 1. Case No. 6, Lumbosacral meningocele, hydrocephalus, spastic paralysis of legs, died 54th day of increasing hydrocephalus. Photo before operation. previous to operation. Paralysis of the legs, more or less complete occurred in 20 cases; there were 12 with talipes equinus. There were 4 infants with paralysis of the anal sphincter, one accompanied with distinct eversion of the rectal mucosa; two had imperforate anus. One infant had one hip dislocated and one had both hips in that condition. Six were monsters with deformities incompatible with extrauterine existence three being iniencephalics, one anencephalic, and two with total gastrochisis. The general results in these 68 cases, so far as it is possible to trace them, are as follows: Seven were still-born, including the six monsters and one hydrocephalic requiring craniotomy to effect delivery. Thirty were treated without operation; all but one died within a few weeks. The exception, a simple meningocele, was discharged on the 49th day in good condition. It has not been possible to trace this case further. None were treated by injection of Morton's fluid, etc. Thirty-one were operated upon for radical cure of the rachocele. Of these, eighteen died within from one to twenty-two days, of meningitis, encephalitis and general infection. In thirteen, the operation was Fig. 2.-Case No. 21, sacral meningo-myelocele, spastic paralysis of legs and sphincter ani. Photo two months after operation. successful in closing the rachocele and healed with a solid scar. This gives us an immediate operative mortality of but 58 per cent and would be most misleading did we not trace the cases after discharge from the hospital. Nine of these thirteen died later as follows: One on the 12th day of increasing hydrocephalus and malnutrition. Of the remaining four, one was discharged on the 21st day with increasing hydrocephalus, and one on the 87th day with increasing hydrocephalus and paralyzed legs. It is doubtful if either of these is still living. The third is now two and one-half years old, a helpless hydrocephalic idiot with paralyzed legs. The fourth, three years old, is perfectly well. It was a simple meningocele operated upon at four weeks. Four other meningoceles, however, after a clean healing of the operative wound, died later of hydrocephalus with increasing pressure symptoms. |