177
Arthrogryposis Multiplex Congenita; Feeding, Language and Other Health Problems By R. O. Robinson Newcomen Centre, Guy's Hospital, St Thomas Street, London SEI 9RT, U. K.
A survey of the health problems of 87 individuals with arthrogryposis multiplex congenita revealed a high proportion (51) with major feeding difficulties in infancy. This was generaIly related to structural abnormalities of the jaw and tongue. Children thus involved were more prone to recurrent chest infections, constipation, and poor growth and weight gain. In addition, there was a strong association between initial feeding difficulties and subsequent language problems. Early identification of a sub-group of children with arthrogryposis multiplex congenita is therefore possible who may require continuing therapy from a number of disciplines.
Keywords Arthrogryposis multiplex congenita - Congenital contractures - Dysphagia - Recurrent chest infections
Introduction Arthrogryposis, or curved joints, is a characteristic of over 150 specific diagnoses (4, 6). Involvement of the tempero-mandibular joint, recognised in arecent review (2) is occasionally emphasised in specific descriptions (1, 3, 5). Much of our information about arthrogryposis multiplex congenita, focuses not unnaturally on its most obvious facet - the types of limb contractures 10gether with their management. Less weIl recognised are the profound clinical effects that tongue and jaw involvement commonly have on several areas. Contact with a number of these children led me 10 believe that several disabilities in arthrogryposis were interrelated as weIl as being associated with general health problems. At the instigation and with the assistance of The Arthrogryposis Group a survey of their members was undertaken to assess the validity of this clinical impression.
Method In 1987 The Arthrogryposis Group was circulated with a questionnaire concerning health problems. This suggested an over representation of feeding difficulties which appeared related to language delay and a number of other health problems. Accordingly in 1988 the Group received a secReceived October 27, 1989; accepted November 10, 1989 N europediatrics 21 (1990) 177-1 78 © Hippokrates Verlag Stuttgart
ond questionnaire asking the nature of the feeding and language difficulties in more detail. After one postal reminder a total of 87 people replied to both questionnaires from a total of 142 originally circulated. Data was transferred to punch cards and analysed manually. The probability of inequities of distribution occurring by chance were estimated using the chi-squared test.
Results Of the 87 in the sampIe, 51 reported feeding problems; 40 problems with chewing, 37 with sucking and 33 with swallowing. Problems with sucking and swaIlowing were frequently sufficiently severe as to prompt tube feeding for weeks or months. Chewing problems were often persisting throughout childhood. Feeding problems were closely interrelated with each other. Nearly all children had two types of feeding problems and 23 children had aIl three. Feeding difficulties appeared to be related to structural problems with the tongue or jaw. Thus of the 51 with feeding difficulties, only 14 had neither a small nor stiff jaw nor a small or immobile tongue. Jaw involvement was sometimes an isolated finding. Of 32 children with jaw involvement only 14 had a tongue involvement also. However, when the tongue was thought to be involved, the jaw usuaIly was as weIl. Of 18 children with tongue involvement, 14 had jaw involvement also. Feeding problems were closely related with jaw or tongue anomalies. Because of the close links between types of feeding difficulties on the one hand and jaw with tongue involvement on the other, types of feeding problems did not correlate with particular structural anomalies. Of the 51 children with feeding problems, 34 had subsequent language difficulties. In 26 there appeared to be a problem with articulation only but in 18 there appeared to be generallanguage delay. Ten children had both articulation and generallanguage difficulties. Structural jaw and tongue anomalies were reasonably predictive of subsequent language problems. Of the 34 children with language difficulties, only 7 had neither tongue nor jaw involvement. Conversely of the 36 with either tongue or jaw involvement all but 9 had language diffieulties. Again beeause of the elose assoeiation between types of feeding diffieulties on the one hand and types of language diffieulties on the other, types of feeding diffieulties did not predict a particular type of language diffieulty. Onee again about 2/3 of the ehildren with sueking or swallowing or ehewing diffieulties had either an artieulation or generallanguage disorder. However, the more severe the feeding problem the more likely the subsequent language difficulty. Thus of 23 ehildren with all three types of feeding diffieulties - only 3 had no subse-
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Abstract
178 Neuropediatrics 21 (1990)
R. O. Robinson
Table 1
severity of feeding diffieulties reported is eonsistent with the subsequent poor weight gain. However, growth, partieularly of the limbs, in arthrogryposis may be restrieted irrespeetive of nutritional status as shown by the lack of eorrelation between poor growth and poor weight gain seen in those ehildren without Paar weight gain 20 3
Arthrogryposis Multiplex Congenita; Feeding, Language and Other Health Problems By R. O. Robinson Newcomen Centre, Guy's Hospital, St Thomas Street, London SEI 9RT, U. K.
A survey of the health problems of 87 individuals with arthrogryposis multiplex congenita revealed a high proportion (51) with major feeding difficulties in infancy. This was generaIly related to structural abnormalities of the jaw and tongue. Children thus involved were more prone to recurrent chest infections, constipation, and poor growth and weight gain. In addition, there was a strong association between initial feeding difficulties and subsequent language problems. Early identification of a sub-group of children with arthrogryposis multiplex congenita is therefore possible who may require continuing therapy from a number of disciplines.
Keywords Arthrogryposis multiplex congenita - Congenital contractures - Dysphagia - Recurrent chest infections
Introduction Arthrogryposis, or curved joints, is a characteristic of over 150 specific diagnoses (4, 6). Involvement of the tempero-mandibular joint, recognised in arecent review (2) is occasionally emphasised in specific descriptions (1, 3, 5). Much of our information about arthrogryposis multiplex congenita, focuses not unnaturally on its most obvious facet - the types of limb contractures 10gether with their management. Less weIl recognised are the profound clinical effects that tongue and jaw involvement commonly have on several areas. Contact with a number of these children led me 10 believe that several disabilities in arthrogryposis were interrelated as weIl as being associated with general health problems. At the instigation and with the assistance of The Arthrogryposis Group a survey of their members was undertaken to assess the validity of this clinical impression.
Method In 1987 The Arthrogryposis Group was circulated with a questionnaire concerning health problems. This suggested an over representation of feeding difficulties which appeared related to language delay and a number of other health problems. Accordingly in 1988 the Group received a secReceived October 27, 1989; accepted November 10, 1989 N europediatrics 21 (1990) 177-1 78 © Hippokrates Verlag Stuttgart
ond questionnaire asking the nature of the feeding and language difficulties in more detail. After one postal reminder a total of 87 people replied to both questionnaires from a total of 142 originally circulated. Data was transferred to punch cards and analysed manually. The probability of inequities of distribution occurring by chance were estimated using the chi-squared test.
Results Of the 87 in the sampIe, 51 reported feeding problems; 40 problems with chewing, 37 with sucking and 33 with swallowing. Problems with sucking and swaIlowing were frequently sufficiently severe as to prompt tube feeding for weeks or months. Chewing problems were often persisting throughout childhood. Feeding problems were closely interrelated with each other. Nearly all children had two types of feeding problems and 23 children had aIl three. Feeding difficulties appeared to be related to structural problems with the tongue or jaw. Thus of the 51 with feeding difficulties, only 14 had neither a small nor stiff jaw nor a small or immobile tongue. Jaw involvement was sometimes an isolated finding. Of 32 children with jaw involvement only 14 had a tongue involvement also. However, when the tongue was thought to be involved, the jaw usuaIly was as weIl. Of 18 children with tongue involvement, 14 had jaw involvement also. Feeding problems were closely related with jaw or tongue anomalies. Because of the close links between types of feeding difficulties on the one hand and jaw with tongue involvement on the other, types of feeding problems did not correlate with particular structural anomalies. Of the 51 children with feeding problems, 34 had subsequent language difficulties. In 26 there appeared to be a problem with articulation only but in 18 there appeared to be generallanguage delay. Ten children had both articulation and generallanguage difficulties. Structural jaw and tongue anomalies were reasonably predictive of subsequent language problems. Of the 34 children with language difficulties, only 7 had neither tongue nor jaw involvement. Conversely of the 36 with either tongue or jaw involvement all but 9 had language diffieulties. Again beeause of the elose assoeiation between types of feeding diffieulties on the one hand and types of language diffieulties on the other, types of feeding diffieulties did not predict a particular type of language diffieulty. Onee again about 2/3 of the ehildren with sueking or swallowing or ehewing diffieulties had either an artieulation or generallanguage disorder. However, the more severe the feeding problem the more likely the subsequent language difficulty. Thus of 23 ehildren with all three types of feeding diffieulties - only 3 had no subse-
Downloaded by: NYU. Copyrighted material.
Abstract
178 Neuropediatrics 21 (1990)
R. O. Robinson
Table 1
severity of feeding diffieulties reported is eonsistent with the subsequent poor weight gain. However, growth, partieularly of the limbs, in arthrogryposis may be restrieted irrespeetive of nutritional status as shown by the lack of eorrelation between poor growth and poor weight gain seen in those ehildren without Paar weight gain 20 3
