Elsevier

The Journal of Pediatrics

Volume 149, Issue 6, December 2006, Pages 837-839
The Journal of Pediatrics

Original article
Enterostomy tube placement in children with spinal muscular atrophy type 1

https://doi.org/10.1016/j.jpeds.2006.08.048Get rights and content

Objective

To determine the major complication rate in the first 30 days after enterostomy tube insertion in infants with spinal muscular atrophy (SMA) type 1.

Study design

A retrospective case review of all children with SMA type 1 who had a gastrostomy or gastrojejunostomy tube placed by the image-guided technique at the Hospital for Sick Children from 1994–2004. Major complications were classified as peritonitis, aspiration pneumonia, respiratory failure, nonelective admission to the pediatric intensive care unit, and death.

Results

Twelve children were identified as having SMA type 1 with an enterostomy tube insertion. The median age at tube insertion was 6.1 months (range 2.2 to 15.8 months). Major complications in the first 30 days after the procedure included aspiration pneumonia (5/12 patients [41.6%]), respiratory failure requiring admission to the pediatric intensive care unit (4/12 [33%]), and death (2/12 [16.7%]). Children with development of aspiration pneumonia were significantly older at time of tube insertion (P < .05) than those with no aspiration.

Conclusions

Major complications including death are seen in children with SMA type 1 in the first 30 days after enterostomy tube insertion.

Section snippets

Methods

This study was conducted at the Hospital for Sick Children (SickKids), a tertiary care pediatric hospital in Toronto, Ontario, Canada. All patients who were SMA type 1 and who had enterostomy tubes placed at the SickKids from January 1994 to July 2004 were included in the study. Patients were identified through the Esh IGT database (Esh, Thornhill, Ontario, Canada). The study was approved by the SickKids Research Ethics Board.

Patients were classified as having SMA type 1 if they presented with

Results

Fourteen patients were identified through the Image Guided Therapy database as having SMA type 1 and enterostomy tube insertion. Two patients were excluded because their genetic testing did not show a deletion of the SMN gene, and muscle biopsy specimens were inconclusive. The median age at time of tube placement was 6.1 months (range 2.2 to 15.8 months), with a mean weight of 6.9 kg (range 2.7 to 14.6 kg) for the 12 patients. The primary indication for tube insertion in all patients was oral

Discussion

This study suggests that although the insertion of an enterostomy tube with the image-guided technique is technically successful with minimal anesthesia in children with SMA type 1, it may be associated with a high rate of early major respiratory complications, as well as death. Older age at time of tube insertion was associated with aspiration pneumonia, and this association approached significance with regard to development of respiratory failure. Older age, which may be a reflection of

References (13)

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Cited by (12)

  • Describing nutrition in spinal muscular atrophy: A systematic review

    2016, Neuromuscular Disorders
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    Four studies reported pre- and/or post-operative outcomes in patients undergoing enterostomy tube placement [18,25,42,50]. Prevalence of post-operative complications ranged from 42% to 64% [18,42]. Positive outcomes of gastrostomy placement included an increase in mean weight [25], decrease in gastro-oesophageal reflux [25], decrease in episodes of aspiration pneumonia and hospitalisations [50], and a significant reduction in the risk of death [35].

  • Risk management protocol for gastrostomy and jejunostomy insertion in ventilator dependent infants

    2013, Neuromuscular Disorders
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    We found that we were able to successfully operate on children with SMA type I some months after diagnosis allowing families to plan for surgery and also allowing medical teams to operate in periods of stability. Sy and co-workers [25] identified 12 children with SMA type I who had percutaneous gastrostomy or gastrojejunostomy insertion – 4 under local anaesthetic, 6 with sedation, and only two under GA, these last two being already intubated. We carried out all our procedures under general anaesthetic to ensure that the airway is secure.

  • Observational study of caloric and nutrient intake, bone density, and body composition in infants and children with spinal muscular atrophy type I

    2012, Neuromuscular Disorders
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    In addition, children with SMA have demonstrated metabolic abnormalities consistent with a generalized defect in fatty acid metabolism, which make them more vulnerable to acidosis in this setting [9]. Finally, gastroesophageal reflux and impaired gastrointestinal motility can make provision of optimal nutritional intake challenging, even when deficits are recognized [7,10]. These challenges include limited intake volume and fat intolerance.

  • Early laparoscopic fundoplication and gastrostomy in infants with spinal muscular atrophy type I

    2008, Journal of Pediatric Surgery
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    Silent and overt GER is common in all children with NMD and is a frequent cause for referral to pediatric surgeons, particularly when the need arises for enteral nutrition access. Several studies have evaluated the outcomes after enterostomy tube placement in children with NMD and found higher rates of perioperative morbidity (42%), need for reoperation, and a 15% 30-day mortality rate [10,11]. Our study included only children with SMA type I, the most severe phenotype, who are theoretically subject to the highest perioperative complication rate.

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