Feeding problems in merosin deficient congenital muscular dystrophy
J Philpota, A Bagnallb, C Kingb, V Dubowitza, F Muntonia
a Neuromuscular Unit,
Department of Paediatrics and Neonatal Medicine, Imperial College
School of Medicine, Hammersmith Hospital, Du Cane Road, London W12 ONN, UK, b Speech Therapy Department, Hammersmith
Hospital
Correspondence to: Professor Muntoni. email: fmuntoni{at}rpms.ac.uk
Accepted 9 February
1999
Feeding difficulties were assessed in 14 children (age
range 2-14 years) with merosin deficient congenital muscular
dystrophy, a disease characterised by severe muscle weakness and
inability to achieve independent ambulation. Twelve of the 14 children
were below the 3rd centile for weight. On questioning, all parents thought their child had difficulty chewing, 12 families modified the
diet, and 13 children took at least 30 minutes to complete a meal. On
examination the mouth architecture was abnormal in 13 children. On
videofluoroscopy only the youngest child (2 years old), had a normal
study. The others all had an abnormal oral phase (breakdown and
manipulation of food and transfer to oropharynx). Nine had an abnormal
pharyngeal phase, with a delayed swallow reflex. Three of these also
showed pooling of food in the larynx and three showed frank aspiration.
These six cases all had a history of recurrent chest infections. Six of
eight children who had pH monitoring also had gastro-oesophageal
reflux. As a result of the study five children had a gastrostomy, which
stopped the chest infections and improved weight gain. This study shows
that children with merosin deficient congenital muscular dystrophy have
difficulties at all stages of feeding that progress with age.
Appropriate intervention can improve weight gain and reduce chest
infections. The severity of the problem has not been previously
appreciated in this disease, and the study shows the importance of
considering the nutritional status in any child with a primary muscle disorder.
Keywords: congenital muscular dystrophy; feeding difficulties; aspiration; videofluoroscopy
© 1999 by Archives of Disease in Childhood
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