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Genetics of anencephaly and spina bifida
172
INTERNATIONAL
A. Robert Beck, and Theodore _lewett, Jr. Arch. Surg. 97:194-197 (August) 1968. The experience gained in the management of 115 children with wringer injuries during a 2year-period forms the basis for this report. All patients with wringer injuries were admitted to the hospital during this study, and all children were treated with bulky compression dressings without elastic support, continuous elevation and careful observation. One hundred and twelve children have been functionally rehabilitated. Three children remain with permanent functional loss. The first was a 3%year-old boy with complete avulsion of the dorsum of the thumb, requiring pedicle grafting and tendon transplant. The second was a 3-year-old girl with avulsion of the median and ulnar nerves about the elbow, and the third was a 28-month-old boy with complete avulsion of the palm of his left hand requiring finger amputation. No patient in the entire series had a fracture. -A. M. Salsberg. GENXTICS OF ANENCEPHALY AND SPINA BIFIDA. S. Yen and B. MacMahon. Lancet 2~623 (September 14) 1968. Variations in the incidence of anencephaly and spina bifida over short periods of time and with socio-economic status leave little doubt that the malformalions have important environmental determinants. Some workers, however, consider that environmental factors merely influence the expression of an underlying genetic susceptibility. The authors present data on 1095 cases of anencephaly and spina bifida born in Rhode Island between 1936 and 1965. The risk of recurrence in siblings born after the first index case was 4.6 per cent. The next sibling born after an affected child showed no greater risk than subsequent siblings. In 7 families where the mother remarried, there were 23 maternal halfsibs of an affected case and 3 of these were affected. The risk of recurrence in affected sibships declined substantially during a period when the incidence of the anomalies was declining in the general population. The risk of recurrence is probably higher in sibships with 2 affected cases than in those with one only. In 8 consecutive series of cases there have been 108 instances of affected twins; in none of these was the co-twin affected. It is concluded that the recurrence of these anomalies in sibships is as likely to be due to persistence of recurrence of environmental factors as to a common genetic inheritance.-j. H. .lohnston.
ABSTRACTS
OF
PEDIATRIC
SURGERY
SOME ASPECTS OF CONGENITAL DYSPLASIA OF THE HIP. A. L. Eyre-Brook. Ann. Roy. Coll. Surg. Eng. 43:1-18 (July) 1968. The pathology found in 2 postmortem specimens of dislocated hips in premature infants is described. In both cases it is similar to that found at operation in cases at 2 years of age or even later. A third dislocated hip, that of an untreated child who died at 18 months, is described in which a very evident feature was a tight fold in the capsule inferiorly which, with the overlying psoas tendon, was one of the most effective bars to complete reduction. The results of treatment in a personal series of 53 hips which have largely matured to a true end result are described. The radiograph is the best guide to the state of the hip as it gives some idea of the prognosis. The length of follow up varied from 9 to 26 years. Fourteen hips were classified as belonging to grades IV, V, and VI (graded by Severin’s modification of Wiberg’s C.E. angle). These were the result of prolonged management by non-operative methods. Of the 53 hips acetabuloplasties were performed in 12. Only one of these is in grade IV. Eight fall within groups I and II, and 3 in group III. In a newer series of 27 hips 15 were reduced by open operation. Femoral osteotomy was performed in 26 and pelvic osteotomy in 1. Acetabuloplasty was performed in one case. The size of the labrum was reduced in only 3 cases. At followup 3 to I2 years later 18 of these hips were classified as group I, 4 as group II, 4 in group III, and 1 in group IV. The effects of an external rotation osteotomy seem to be entirely satisfactory. The author has come to the conclusion that he should use open reduction with acetabuloplasty much more often in the treatment of congenital dislocation of the hip. The operation of open reduction and acetabuloplasty are described.--J. J. Corkery. NEOPLASMS SINGLE VERSUS MULTIPLE APY IN WILMS’
TUMOR.
DACTINOMYCIN A
CONTROLLED
THEHCOOP-
ERATrvE STUDY CONDUCTED BY THE CHILDREN’S CANCER
STUDY
GROUP
A
(FORMERLY
ACUTE
GROUP A). Prepared by Writing Committee: J. A. Wolff, (Chairman), W. Kriuit, W. A. Newton, Jr., and G. J. D’ Angio. New Eng. J. Med. 279:290-294 (August) 1968. LEUKEMIA
The
value
COOPERATIVE CHEMOTHERAPY
of a single
course
of Dactinomycin
INTERNATIONAL
A. Robert Beck, and Theodore _lewett, Jr. Arch. Surg. 97:194-197 (August) 1968. The experience gained in the management of 115 children with wringer injuries during a 2year-period forms the basis for this report. All patients with wringer injuries were admitted to the hospital during this study, and all children were treated with bulky compression dressings without elastic support, continuous elevation and careful observation. One hundred and twelve children have been functionally rehabilitated. Three children remain with permanent functional loss. The first was a 3%year-old boy with complete avulsion of the dorsum of the thumb, requiring pedicle grafting and tendon transplant. The second was a 3-year-old girl with avulsion of the median and ulnar nerves about the elbow, and the third was a 28-month-old boy with complete avulsion of the palm of his left hand requiring finger amputation. No patient in the entire series had a fracture. -A. M. Salsberg. GENXTICS OF ANENCEPHALY AND SPINA BIFIDA. S. Yen and B. MacMahon. Lancet 2~623 (September 14) 1968. Variations in the incidence of anencephaly and spina bifida over short periods of time and with socio-economic status leave little doubt that the malformalions have important environmental determinants. Some workers, however, consider that environmental factors merely influence the expression of an underlying genetic susceptibility. The authors present data on 1095 cases of anencephaly and spina bifida born in Rhode Island between 1936 and 1965. The risk of recurrence in siblings born after the first index case was 4.6 per cent. The next sibling born after an affected child showed no greater risk than subsequent siblings. In 7 families where the mother remarried, there were 23 maternal halfsibs of an affected case and 3 of these were affected. The risk of recurrence in affected sibships declined substantially during a period when the incidence of the anomalies was declining in the general population. The risk of recurrence is probably higher in sibships with 2 affected cases than in those with one only. In 8 consecutive series of cases there have been 108 instances of affected twins; in none of these was the co-twin affected. It is concluded that the recurrence of these anomalies in sibships is as likely to be due to persistence of recurrence of environmental factors as to a common genetic inheritance.-j. H. .lohnston.
ABSTRACTS
OF
PEDIATRIC
SURGERY
SOME ASPECTS OF CONGENITAL DYSPLASIA OF THE HIP. A. L. Eyre-Brook. Ann. Roy. Coll. Surg. Eng. 43:1-18 (July) 1968. The pathology found in 2 postmortem specimens of dislocated hips in premature infants is described. In both cases it is similar to that found at operation in cases at 2 years of age or even later. A third dislocated hip, that of an untreated child who died at 18 months, is described in which a very evident feature was a tight fold in the capsule inferiorly which, with the overlying psoas tendon, was one of the most effective bars to complete reduction. The results of treatment in a personal series of 53 hips which have largely matured to a true end result are described. The radiograph is the best guide to the state of the hip as it gives some idea of the prognosis. The length of follow up varied from 9 to 26 years. Fourteen hips were classified as belonging to grades IV, V, and VI (graded by Severin’s modification of Wiberg’s C.E. angle). These were the result of prolonged management by non-operative methods. Of the 53 hips acetabuloplasties were performed in 12. Only one of these is in grade IV. Eight fall within groups I and II, and 3 in group III. In a newer series of 27 hips 15 were reduced by open operation. Femoral osteotomy was performed in 26 and pelvic osteotomy in 1. Acetabuloplasty was performed in one case. The size of the labrum was reduced in only 3 cases. At followup 3 to I2 years later 18 of these hips were classified as group I, 4 as group II, 4 in group III, and 1 in group IV. The effects of an external rotation osteotomy seem to be entirely satisfactory. The author has come to the conclusion that he should use open reduction with acetabuloplasty much more often in the treatment of congenital dislocation of the hip. The operation of open reduction and acetabuloplasty are described.--J. J. Corkery. NEOPLASMS SINGLE VERSUS MULTIPLE APY IN WILMS’
TUMOR.
DACTINOMYCIN A
CONTROLLED
THEHCOOP-
ERATrvE STUDY CONDUCTED BY THE CHILDREN’S CANCER
STUDY
GROUP
A
(FORMERLY
ACUTE
GROUP A). Prepared by Writing Committee: J. A. Wolff, (Chairman), W. Kriuit, W. A. Newton, Jr., and G. J. D’ Angio. New Eng. J. Med. 279:290-294 (August) 1968. LEUKEMIA
The
value
COOPERATIVE CHEMOTHERAPY
of a single
course
of Dactinomycin