(1973). Archives It is concluded that the overall results of selective early operation compare favourably with those of purely conservative management on the one hand, and with routine early operation on the other. It is suggested that this policy offers the best prospects of independence for the less severely affected child, and the least distress and suffering for the grossly afflicted.Routine early closure of myelomeningocele was advocated in Sheffield a decade ago (Sharrard et al., 1963) and has since become standard practice in many centres in Great Britain and abroad. The only detailed report of the results of unselective early operation is that of Lorber (1971a). The end results of an unparalleled expenditure of resources on myelomeningocele were so disappointing that he went on to advocate a policy of selection based on certain major adverse criteria at birth, which would have excluded just over half from operation. As yet, however, there are no published reports on the actual effectiveness of such a policy.Since the selection criteria employed at the Royal Hospital for Sick Children, Edinburgh, where routine early operation has never been accepted, accord closely with those now defined by Lorber, it is of interest to compare our results with those of the nonselective policy employed in Sheffield.Received 12 February 1973. Patients and methods of study Between April 1965 and March 1971, 163 unselected infants with open myelomeningoceles were admitted to the spina bifida unit. More than 98% were under 24 hours of age on admission, the range being 1 to 64 hours. On admission every child was subjected to a detailed paediatric and neurological examination by one of us, as previously described (Stark, 1971), and detailed records kept as part of a prospective study. No predefined criteria for selection were employed, but each case was considered on its merits-social as well as medical and surgical factors being taken into consideration.Patients fell into two groups. In group A 78 infants (48%) were subjected to early operation, associated problems were dealt with systematically, and complications treated vigorously. The 85 patients (52%) in group B were not subjected to operation. They were retained in the unit where they received good nursing care, were fed normally, and kept comfortable. In the event of life-threatening situations, such as ventriculitis, antibiotics were withheld and therapy confined to analgesics, such as heroin. In infants in this group 676 on 12 May 2018 by guest. Protected by copyright.
Two cases of trisomy 12p due to a familial translocation t(12;21) (p11;p11) inherited through three generations are presented. The clinical features of both affected individuals are consistent with those previously reported. Study of the NORs by silver staining showed translocation of the NOR from chromosome 21 onto the der(12) and suggested that the activity of this site has been suppressed in some carriers.
IntroductioinIn his famous work De Sedibus et CausiJ Morborum, Morgagni (1761) first recorded the occurrence of lower-limb deformities in children suffering from spina bifida cystica. For almost two centuries these deformities were regarded as associated malformations, and the term 'talipes' is still used by some when describing them. Sharrard (1962Sharrard ( , 1963 has, however, repeatedly emphasised that, with very few exceptions, lower-limb deformities in myelomeningocele are due to muscle imbalance caused by the spinal cord lesion and are analagous I o the paralytic deformities in poliomyelitis. Nevertheless, the nature o f the basci spinal cord lesion in myelomeningocele is still not fully understood.It is commonly assumed that the fundamental defect is absence of anterior horn cells, not from acute loss as in poliomyelitis, but from failure of development or 'myelodysplasia'. Even in standard textbooks of neurology, myelomeningocele is quoted as a typical 'lower motor neurone' lesion. With increasing neurological study of the newborn infant, however, the weakness of this assumption has become morc and more obvious. The frequency of upper notor neurone lesions with preservation distally of functioning but isolated cord segments has been emphasised by Guthkelch (1964)) Brocklehurst et al. (1966) and Stark and Baker (1967).The purpose o f this study is to clarify the relationship between the myelomeningocele plaque andl the neurological disorder in the lower limbs. Patients anid Methods of StudySeventy-five infants suffering from open myelomeningoceles were studied during the period 1967-70. Tn 71 patients, the lesion was more or less flat and in 4 it was pedunculated.The level of the myelomeningocele in relation to vertebral segments was determined by radiological examination of the spine, with wire markers outlining the lesion (Fig. 1). The lesions were distributed as follows: Thoraco-lumbar or thoraco-lunibo-sacral : 50 (67 per cent) Lumbar: 9 (12 per cent) Lumbo-sacral : 16 (21 per cent) Because of certain changes in the method of investigation which took place in 1969, the infants have b-en divided into two groups (Series I and Series II), according to when they were examined. The different methods used for studying each group are described below.Series I ( 1 967-1969) closure of the lesion.The 50 infants in this group were all studied below the age of 36 hours and before surgical ~_ _ _ _ _ *The C a w y Holrev Memorial Prize Essuy.
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