[Severe congenital skeletal hypoplasia and aplasia of the lower extremities in children. Definition].
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Biomedical subjects
Publications and source records attributed to P Rigault.
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There exists considerable confusion in the definition and classification of essential flat foot in the child. In an attempt to put an end to this confusion, the authors define the radiological characteristics and course of the abnormality. The latter is defined radiologically on lateral films of the foot whilst weight bearing by study of the angle of the calcaneum over the horizontal (Angle C) and by the measurement of the angle of talo-calcaneal divergence (Angle T.C.D.). Three groups may be distinguished on this basis: Group I - of minimal gravity; Group II - which may correct itself spontaneously and is, in general, well tolerated; Group III - which alone requires surgical correction. Treatment is only surgical in severe forms and is based upon the "Horseman" operation, orthopaedic treatment by special soles being rarely indicated.
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The authors have operated on 33 equinovarus feet, (14 patients) with various condition--myelomeningocele, arthrogryposis, club foot or dwarfism. The results were poor, only 11 being satisfactory. Some failures were due to incorrect tecunique. The authors describe the technique which they consider to be the best. This procedure appears to have its main indication in myelomeningocele and arthrogryposis when the feet are severely deformed and rigid.
The authors have treated 28 cases of traumatic separation of epiphysis of the lower end of the femur. They have noted the particular severity of the initial trauma. The results were less satisfactory when the child was younger and when the plate was crashed. However, 4 cases of fractures in breech delivery had rather good results. The authors think that a good reduction is essential, either close reduction fixed by Kirchner's wires or open reduction when the former is impossible.
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The authors have followed up 26 children suffering from severe scoliosis associated with congenital heart disease up to the end of puberty. The curve was usually a very severe idiopathic scoliosis developing early and requring surgical treatment. Two types may be distinguished: I. Scoliosis without excessive surgical risk, in patients in whom the heart disease is not associated with cyanosis or where the heart condition has already been treated surgically and in which there are no clinical, radiological or electrical signs of cardiac failure. 2. Scoliosis with considerable surgical risk because of heart disease with cyanosis not treated surgically, or with signs of heart failure or pulmonary hypertension. In such cases, the surgical treatment of the scoliosis is likely to endanger life.
Families of 241 patients among 913 cases of idiopathic scoliosis were surveyed. The data suggest two possible modes of inheritance: either an autosomal dominant inheritance with incomplete penetrance and more frequent manifestation in girls than boys, or a genetic heterogeneity with a mixture of dominant and multifactorial modes of inheritance.
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The authors have reviewed 32 cases of severe varus deformity following supra-condylar fractures in childhood. The sequellae were mainly cosmetic, the function being preserved. All cases were treated by supra-condylar osteotomy fixed by screwing. The technique is described. The results were satisfactory in 20 cases.