Gastrostomy placement improves height and weight gain in girls with Rett syndrome.
BACKGROUND
Growth failure and undernutrition complicate the clinical course of girls with Rett syndrome (RTT). These abnormalities are, in part, the consequence of oral motor dysfunction and inadequate dietary intake.


OBJECTIVE
To determine whether gastrostomy placement for nutritional therapy alters the natural history of growth failure and undernutrition in RTT.


OBJECTIVE
We hypothesized that gastrostomy placement for nutritional therapy reverses the decline in height, weight, and body mass index (BMI) z scores in RTT.


METHODS
Standard stadiometric and anthropometric measures were obtained to derive height, weight, and BMI z scores and estimates of fat-free mass (FFM) and body fat in a cohort of girls (n = 92) with RTT before and after gastrostomy placement. Methyl-CpG-binding protein 2 (MECP2) mutations and the presence or absence of a fundoplication were recorded.


RESULTS
The differences in height (n = 73), weight (n = 81), and BMI (n = 81) z score slopes before and after gastrostomy placement were 1.31 + 2.06 (P < 0.001), 2.38 +/- 3.18 (P < 0.001), and 3.25 +/- 3.32 (P < 0.001), respectively. FFM and body fat (n = 43) increased after gastrostomy by 41 +/- 27 g/cm height (P < 0.001) and 7.5% +/- 5.7% body weight (P < 0.001), respectively. The differences in height, weight, and BMI z score slopes were similar regardless of the age at which the gastrostomy was placed. The differences in height, weight, and BMI z score slopes, as well as the change in FFM and body fat deposition after gastrostomy placement, did not differ between those who did or did not have a fundoplication and among the classes of MECP2 mutations.


CONCLUSIONS
Gastrostomy placement for aggressive nutritional therapy favorably altered the natural history of growth failure and undernutrition in RTT, but did not restore height and weight z scores to birth values, regardless of the age at which surgery occurred and in the presence or absence of a fundoplication.
Motil
Kathleen J
KJ
USDA/ARS Children's Nutrition Research Center, Baylor College of Medicine, Houston, TX 77030, USA. kmotil@bcm.edu
Morrissey
Matthew
M
Caeg
Erwin
E
Barrish
Judy O
JO
Glaze
Daniel G
DG
D000273:Adipose Tissue; D000293:Adolescent; D001825:Body Fluid Compartments; D001827:Body Height; D015992:Body Mass Index; D049628:Body Size; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D005260:Female; D018662:Fundoplication / Q000706:statistics & numerical data; D005774:Gastrostomy / Q000379:methods*; D006128:Growth; D006130:Growth Disorders / Q000209:etiology / Q000628:therapy*; D006801:Humans; D051783:Methyl-CpG-Binding Protein 2 / Q000235:genetics; D009154:Mutation; D018529:Nutritional Support / Q000379:methods*; D015518:Rett Syndrome / Q000150:complications / Q000628:therapy*
D051783:Methyl-CpG-Binding Protein 2