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November 1962, Vol 7, No. 5 >
Article | November 1962

Anencephalus, Hydrocephalus, and Spina Bifida: Title and subTitle BreakEpidemiology, with Special Reference to a Survey in Charleston, S.C.

MILTON ALTER, M.D.
[+] Author Affiliations

Submitted for publication March 9, 1962.


Arch Neurol. 1962;7(5):411-422. doi:10.1001/archneur.1962.04210050047005
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References
Comments

Anencephalus, hydrocephalus, and spina bifida constitute a significant proportion of the more serious congenital malformations. A better understanding of the epidemiology of these disorders may clarify the role of environmental and genetic factors in etiology and possibly lead to successful preventative measures. In this report, the results of a survey of anencephalus, hydrocephalus, and spina bifida in Charleston, S.C., are presented, and literature pertaining to the epidemiology of these disorders is reviewed.

Survey Method in Charleston, S.C.  Hospital and clinic records, autopsy reports, and mortality records of stillbirths and deaths were studied for the 10-year period January, 1946, through December, 1955. Also the records of the 2 neurosurgeons and the neurologist in the community as well as records of the Crippled Children Program of the State were made available to the investigator.All records with a diagnosis of anencephalus, hydrocephalus, spina bifida, and other myelodysplasias were noted. Not included in

REFERENCES

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Record, R. G., and McKeown, T.:  Congenital Malformations of the Central System: I. A Survey of 930 Cases , Brit. J. Prev. Soc. Med. 3:183, 1949;.
2 +
McIntosh, R.; Merritt, K. K.; Richards, M. R.; Samuels, M. H., and Bellows, M. T.:  The Incidence of Congenital Malformations: A Study of 5,964 Pregnancies , Pediatrics 14:505, 1954;.
3 +
Edwards, J. H.:  Congenital Malformations of the Central Nervous System in Scotland , Brit. J. Prev. Soc. Med. 12:115, 1958;.
4 +
Landauer, W.:  Sex and Season in Relation to Malformations in Chicken Embryos , Anat. Rec. 86:365, 1943;.
5 +
MacMahon, B., and Gordon, j. E.:  Epidemiologic Inferences Derived from Maternal Age , Amer. J. Med. Sci. 226:326, 1953;.
6 +
Schwidde, J. T.:  Spina Bifida: A Survey of 225 Encephaloceles, Meningoceles, and Myelomeningoceles , A.M.A. Amer. J. Dis. Child. 84:35, 1952;.
7 +
Polman, A.:  Anencephaly, Spina Bifida, and Hydrocephaly , Genetica 25:29, 1951;.
8 +
Böök, J. A., and Rayner, S.:  A Clinical and Genetical Study of Anencephaly , Amer. J. Hum. Genet. 2:61, 1950;.
9 +
Hegnauer, H.:  Misbildungshäufigkeit und Gebaralter , Geburts. u Frauenheilkl. 11:777, 1951;.
10 +
Landtman, B.:  On the Relationship Between Maternal Conditions During Pregnancy and Congenital Malformations , Arch. Dis. Child. 23:237, 1948;.
11 +
MacMahon, B.; Hertig, A., and Ingalls, T.:  Association Between Maternal Age and Pathological Diagnosis in Abortion , Obst. Gyn. 4:477, 1954;.
12 +
Malpas, P.:  The Incidence of Human Malformations and the Significance of Changes of the Maternal Environment in their Causation , J. Obstet. Gynaec. Brit. Emp. 44:434, 1937;.
13 +
Coffey, V. P., and Jessop, W. J. E.:  A study of 137 Cases of Anencephaly , Brit. J. Prev. Soc. Med. 11:174, 1957;.
14 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central Nervous System: II. Maternal Reproductive History and Familial Incidence , Brit. J. Prev. Soc. Med. 4:26, 1950;.
15 +
Carter, C. O.:  Maternal States in Relation to Congenital Malformations , J. Obstet. Gynaec. Brit. Emp. 57:897, 1950;.
16 +
Ingalls, T. E.; Pugh, T. J., and MacMahon, B.:  Incidence of Anencephalus, Spina Bifida, and Hydrocephalus Related to Birth Rank and Maternal Age , Brit. J. Prev. Soc. Med. 8:17, 1954;.
17 +
Murphy, D. P.: Congenital Malformations: A Study of Parental Characteristics with Special Reference to the Reproductive Process , Philadelphia, J. B. Lippincott Company, 1947;.
18 +
McDonald, A.:  Maternal Health and Congenital Defects: A Prospective Investigation , New Engl. J. Med. 258:767, 1958;.
19 +
Coffey, V. P., and Jessop, W. J. E.:  Maternal Influenza and Congenital Deformities: A Prospective Study , Lancet 2:935, 1959;.
20 +
Bradford-Hill, A.; Doll, R.; Galloway, T. McL., and Hughes, J. P. W.:  Virus Diseases in Pregnancy and Congenital Defects , Brit. J. Prev. Soc. Med. 12:1, 1958;.
21 +
Fraser, F. C.:  Causes of Congenital Malformations in Human Beings , J. Chron. Dis. 10:97, 1959;.
22 +
Prindle, R. A.; Ingalls, T. H., and Kirkwood, S. B.:  Maternal Hydramnios and Congenital Malformations of the Central Nervous System , New Engl. J. Med. 25:555, 1955;.
23 +
Pleydell, M. J.:  Anencephaly and Other Congential Abnormalities: An Epidemiologic Study in Northhamptonshire , Brit. Med. J. 1:309, 1960;.
24 +
Feeney, J. K., and Barry, A. P.:  Hydrocephaly as a Cause of Maternal Mortality and Morbidity: A Clinical Study of 304 Cases , J. Obstet. Gynaec. Brit. Emp. 61:652, 1954;.
25 +
Baird, D.:  Social Class and Fetal Mortality , Lancet 2:531, 1947;.
26 +
Bickers, D. S., and Adams, R. D.:  Hereditary Stenosis of the Aqueduct of Sylvius as a Cause of Congenital Hydrocephalus , Brain 72:246, 1949;.
27 +
Penrose, L. S.:  Familial Data on 144 Cases of Anencephaly, Spina Bifida, and Congenital Hydrocephaly , Ann. Eugen. (Lond.) 13:73, 1946;.
28 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central Nervous System: III. Risk of Malformation in Sibs of Malformed Individuals , Brit. J. Prev. Soc. Med. 4:217, 1950;.
29 +
Litt, S., and Strauss, H. A.:  Monoamniotic Twins, One Normal, the Other Anencephalic: Multiple True Knots in the Cords , Amer. J. Obstet. Gynaec. 30:728, 1935;.
30 +
Mudaliar, A. L.:  Double Monsters: A Study of Their Circulatory System and Some Other Anatomical Abnormalities and the Complications in Labour , J. Obstet. Gynaec. Brit. Emp. 37:753, 1930;.
31 +
Tabcr, K. W., and Elwell, W. J., Jr.:  Monozygotic Anencephalic Twins , Maryland Med. J. 9:14, 1960;.
32 +
Josephson, J. E., and Waller, K. B.:  Anencephaly in Identical Twins , Canad. Med. Ass. J. 29:34, 1933;.
33 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central Nervous System: Data on 69 Pairs of Twins , Ann. Eugen. (Lond.) 15:285, 1951;.
34 +
Penrose, L. S.:  Genetics of Anencephaly , J. Ment. Defic. Res. 1:4, 1957;.
35 +
Searle, A. G.:  The Incidence of Anencephaly in a Polytypic Population , Ann. Hum. Genet. 23:279, 1959;.
36 +
Marin, S.: Congenital Malformations: Thesis for Doctorate in Medicine , Hadassah University Medical School, Jerusalem, Israel, 1959;.
37 +
Neel, J. V., and Schull, W. J.: Effect of Exposure to the Atomic Bomb on Pregnancy Termination in Hiroshima and Nagasaki , Nat. Acad. Science, Publ. Nat. Research Council, 1956;.
38 +
Gentry, J. T.; Parkhurst, E., and Bulin, G. V.:  An Epidemiologic Study of Congenital Malformations in New York State , Amer. J. Public Health 49:497, 1959;.
39 +
Perrin, E. V., and Benirschkc, K.:  Somatic Sex of Anencephalic Infants , J. Clin. Endocr. 18:327, 1958;.
40 +
Hiilesmaa, V.:  A Study of Anencephalus and Hydrocephalus Deliveries , Acta Obstet. Gynec. Scand. 25:27 (Supp. 2) , 1945;.
41 +
Böök, J. A., and Fraccarro, M.:  Research on Congenital Malformations , Biol. Neonat. 5:39, 1956;.
42 +
Böök, J. A.:  The Incidence of Congenital Diseases and Defects in a South Swedish Population , Acta Genet. 2:289, 1951;.
43 +
Banner, E. A.:  Hydrocephalus: A 20 Year Survey of Hydrocephalic Births , Lancet 76:1, 1956;.
44 +
Harris, L. E., and Steinberg, A. G.:  Abnormalities Observed During the First 6 Days of Life in 8,716 Live-born Infants , Pediatrics 14:314, 1954;.
45 +
Stevenson, A. C., and Warnock, H. A.:  Observations on the Results of Pregnancies in Women-Resident in Belfast: I. Data Relating to All Pregnancies Ending in 1957 , Ann. Hum. Genet. 23:382, 1959;.
46 +
Kurland, L. T.:  Descriptive Epidemiology of Selected Neurologic and Myopathic Disorders with Particular Reference to a Survey in Rochester, Minnesota , J. Chron. Dis. 8:378, 1958;.
47 +
Wallace, H. M.; Baumgartner, L., and Rich, H.:  Congenital Malformations and Birth Injuries in New York City , Pediatrics 12:525, 1953;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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1 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central System: I. A Survey of 930 Cases , Brit. J. Prev. Soc. Med. 3:183, 1949;.
2 +
McIntosh, R.; Merritt, K. K.; Richards, M. R.; Samuels, M. H., and Bellows, M. T.:  The Incidence of Congenital Malformations: A Study of 5,964 Pregnancies , Pediatrics 14:505, 1954;.
3 +
Edwards, J. H.:  Congenital Malformations of the Central Nervous System in Scotland , Brit. J. Prev. Soc. Med. 12:115, 1958;.
4 +
Landauer, W.:  Sex and Season in Relation to Malformations in Chicken Embryos , Anat. Rec. 86:365, 1943;.
5 +
MacMahon, B., and Gordon, j. E.:  Epidemiologic Inferences Derived from Maternal Age , Amer. J. Med. Sci. 226:326, 1953;.
6 +
Schwidde, J. T.:  Spina Bifida: A Survey of 225 Encephaloceles, Meningoceles, and Myelomeningoceles , A.M.A. Amer. J. Dis. Child. 84:35, 1952;.
7 +
Polman, A.:  Anencephaly, Spina Bifida, and Hydrocephaly , Genetica 25:29, 1951;.
8 +
Böök, J. A., and Rayner, S.:  A Clinical and Genetical Study of Anencephaly , Amer. J. Hum. Genet. 2:61, 1950;.
9 +
Hegnauer, H.:  Misbildungshäufigkeit und Gebaralter , Geburts. u Frauenheilkl. 11:777, 1951;.
10 +
Landtman, B.:  On the Relationship Between Maternal Conditions During Pregnancy and Congenital Malformations , Arch. Dis. Child. 23:237, 1948;.
11 +
MacMahon, B.; Hertig, A., and Ingalls, T.:  Association Between Maternal Age and Pathological Diagnosis in Abortion , Obst. Gyn. 4:477, 1954;.
12 +
Malpas, P.:  The Incidence of Human Malformations and the Significance of Changes of the Maternal Environment in their Causation , J. Obstet. Gynaec. Brit. Emp. 44:434, 1937;.
13 +
Coffey, V. P., and Jessop, W. J. E.:  A study of 137 Cases of Anencephaly , Brit. J. Prev. Soc. Med. 11:174, 1957;.
14 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central Nervous System: II. Maternal Reproductive History and Familial Incidence , Brit. J. Prev. Soc. Med. 4:26, 1950;.
15 +
Carter, C. O.:  Maternal States in Relation to Congenital Malformations , J. Obstet. Gynaec. Brit. Emp. 57:897, 1950;.
16 +
Ingalls, T. E.; Pugh, T. J., and MacMahon, B.:  Incidence of Anencephalus, Spina Bifida, and Hydrocephalus Related to Birth Rank and Maternal Age , Brit. J. Prev. Soc. Med. 8:17, 1954;.
17 +
Murphy, D. P.: Congenital Malformations: A Study of Parental Characteristics with Special Reference to the Reproductive Process , Philadelphia, J. B. Lippincott Company, 1947;.
18 +
McDonald, A.:  Maternal Health and Congenital Defects: A Prospective Investigation , New Engl. J. Med. 258:767, 1958;.
19 +
Coffey, V. P., and Jessop, W. J. E.:  Maternal Influenza and Congenital Deformities: A Prospective Study , Lancet 2:935, 1959;.
20 +
Bradford-Hill, A.; Doll, R.; Galloway, T. McL., and Hughes, J. P. W.:  Virus Diseases in Pregnancy and Congenital Defects , Brit. J. Prev. Soc. Med. 12:1, 1958;.
21 +
Fraser, F. C.:  Causes of Congenital Malformations in Human Beings , J. Chron. Dis. 10:97, 1959;.
22 +
Prindle, R. A.; Ingalls, T. H., and Kirkwood, S. B.:  Maternal Hydramnios and Congenital Malformations of the Central Nervous System , New Engl. J. Med. 25:555, 1955;.
23 +
Pleydell, M. J.:  Anencephaly and Other Congential Abnormalities: An Epidemiologic Study in Northhamptonshire , Brit. Med. J. 1:309, 1960;.
24 +
Feeney, J. K., and Barry, A. P.:  Hydrocephaly as a Cause of Maternal Mortality and Morbidity: A Clinical Study of 304 Cases , J. Obstet. Gynaec. Brit. Emp. 61:652, 1954;.
25 +
Baird, D.:  Social Class and Fetal Mortality , Lancet 2:531, 1947;.
26 +
Bickers, D. S., and Adams, R. D.:  Hereditary Stenosis of the Aqueduct of Sylvius as a Cause of Congenital Hydrocephalus , Brain 72:246, 1949;.
27 +
Penrose, L. S.:  Familial Data on 144 Cases of Anencephaly, Spina Bifida, and Congenital Hydrocephaly , Ann. Eugen. (Lond.) 13:73, 1946;.
28 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central Nervous System: III. Risk of Malformation in Sibs of Malformed Individuals , Brit. J. Prev. Soc. Med. 4:217, 1950;.
29 +
Litt, S., and Strauss, H. A.:  Monoamniotic Twins, One Normal, the Other Anencephalic: Multiple True Knots in the Cords , Amer. J. Obstet. Gynaec. 30:728, 1935;.
30 +
Mudaliar, A. L.:  Double Monsters: A Study of Their Circulatory System and Some Other Anatomical Abnormalities and the Complications in Labour , J. Obstet. Gynaec. Brit. Emp. 37:753, 1930;.
31 +
Tabcr, K. W., and Elwell, W. J., Jr.:  Monozygotic Anencephalic Twins , Maryland Med. J. 9:14, 1960;.
32 +
Josephson, J. E., and Waller, K. B.:  Anencephaly in Identical Twins , Canad. Med. Ass. J. 29:34, 1933;.
33 +
Record, R. G., and McKeown, T.:  Congenital Malformations of the Central Nervous System: Data on 69 Pairs of Twins , Ann. Eugen. (Lond.) 15:285, 1951;.
34 +
Penrose, L. S.:  Genetics of Anencephaly , J. Ment. Defic. Res. 1:4, 1957;.
35 +
Searle, A. G.:  The Incidence of Anencephaly in a Polytypic Population , Ann. Hum. Genet. 23:279, 1959;.
36 +
Marin, S.: Congenital Malformations: Thesis for Doctorate in Medicine , Hadassah University Medical School, Jerusalem, Israel, 1959;.
37 +
Neel, J. V., and Schull, W. J.: Effect of Exposure to the Atomic Bomb on Pregnancy Termination in Hiroshima and Nagasaki , Nat. Acad. Science, Publ. Nat. Research Council, 1956;.
38 +
Gentry, J. T.; Parkhurst, E., and Bulin, G. V.:  An Epidemiologic Study of Congenital Malformations in New York State , Amer. J. Public Health 49:497, 1959;.
39 +
Perrin, E. V., and Benirschkc, K.:  Somatic Sex of Anencephalic Infants , J. Clin. Endocr. 18:327, 1958;.
40 +
Hiilesmaa, V.:  A Study of Anencephalus and Hydrocephalus Deliveries , Acta Obstet. Gynec. Scand. 25:27 (Supp. 2) , 1945;.
41 +
Böök, J. A., and Fraccarro, M.:  Research on Congenital Malformations , Biol. Neonat. 5:39, 1956;.
42 +
Böök, J. A.:  The Incidence of Congenital Diseases and Defects in a South Swedish Population , Acta Genet. 2:289, 1951;.
43 +
Banner, E. A.:  Hydrocephalus: A 20 Year Survey of Hydrocephalic Births , Lancet 76:1, 1956;.
44 +
Harris, L. E., and Steinberg, A. G.:  Abnormalities Observed During the First 6 Days of Life in 8,716 Live-born Infants , Pediatrics 14:314, 1954;.
45 +
Stevenson, A. C., and Warnock, H. A.:  Observations on the Results of Pregnancies in Women-Resident in Belfast: I. Data Relating to All Pregnancies Ending in 1957 , Ann. Hum. Genet. 23:382, 1959;.
46 +
Kurland, L. T.:  Descriptive Epidemiology of Selected Neurologic and Myopathic Disorders with Particular Reference to a Survey in Rochester, Minnesota , J. Chron. Dis. 8:378, 1958;.
47 +
Wallace, H. M.; Baumgartner, L., and Rich, H.:  Congenital Malformations and Birth Injuries in New York City , Pediatrics 12:525, 1953;.

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