Does Gastrostomy Tube Prolong Life in Rett Syndrome?

A recent study (K Wong et al. J Pediatr 2018; 200: 188-95) examined a longitudinal cohort of 323 females in the Australian Rett Syndrome Study.

Key findings:

  • 30.3% of the cohort underwent gastrostomy placement
  • BMI was greater in individuals with gastrostomy placement
  • Median age of gastrostomy placement was 9 years
  • The all-cause mortality rate was greater in those who had gastrostomy placement compared with those who had not (hazard ratio 4.07, CI 1.96-8.45)
  • Survival: 66.1% of the entire cohort was alive at 20 years of age (median survival was 33 years). The survival was 87.3% in those without a gastrostomy.
  • Placement of a gastrostomy tube was not associated with fewer hospitalizations or improvement in parental physical or mental health

While the mortality was higher in those who received a gastrostomy tube, the study’s nonrandomized design does not allow definitive assessment of whether a gastrostomy tube is detrimental to long-term survival.  Children who received gastrostomy tube may have had additional comorbidities.

My take: In adult medicine, it is generally accepted that Gtube placement does not prolong life (Clin Gastro & Hep 2007; 5: 1372).  This study indicates that a Gtube may not improve longevity in many pediatric disorders as well.

Related blog posts:

Rett Reference

JPGN 2013; 57: 451-60.  “Assessment and Management of Nutrition and Growth in Rett Syndrome”

  • This references expert recommendations from an international multidisciplinary panel.  “The level of evidence for the statements was low.”
  • “A body mass index of approximately the 25% can be considered a reasonable target in clinical practice.  Gastrostomy is indicated for extremely poor growth, if there is risk of aspiratrion and if feeding times are prolonged.”
  • There was no consensus with regard to fundoplication.  It should be used with “caution in those with significant air swallowing.”

Related previous post:

GI & Nutrition Problems in Rett Syndrome | gutsandgrowth

GI & Nutrition Problems in Rett Syndrome

A nationwide survey of 983 patients with Rett syndrome identifies a high prevalence of GI and nutritional problems (Motil KJ et al. JPGN 2012; 55: 292-98).

Parents from 983 female patients with Rett syndrome responded to the study questionnaire; this was a 59% response rate from the 1666 families in the North American Rett Database.  Patients included those who fulfilled clinical criteria for diagnosis or who had MECP2 gene (methyl-CpG-binding-protein).

Prevalence of GI problems were listed in article’s Table 2 and included the following:

  • Gastrointestinal problems 92%
  • Feeding problems 81%
  • Constipation 80%
  • Poor weight gain 38%
  • Gastroesophageal reflux 39%

Z-scores for height-for-age, weight-for-age, and BMI are presented for ages 0-40 (see Table 1) -these are all significantly lower than age-matched healthy children.

Important findings:

  • Surgical interventions were common: 11% had fundoplication, 28% gastrostomy, 3% and cholecystectomy
  • Many gastrointestinal symptoms improved with age.  However, short stature, gastrostomy tube, and bone health issues were more common in older patients.
  • Bone fractures are 3- to 4-fold higher than in healthy children

Addtional references:

  • -J Pediatr 2010; 156: 135.  Longevity in Rett syndrome –about 1/2 survive to age 40.
  • -Ann Neurol 2010; 68: 944-50.  Rett diagnostic criteria
  • -JPGN 2007; 45: 582-90.  Growth/feeding issues in Rett syndrome