Risk management protocol for gastrostomy and jejunostomy insertion in ventilator dependent infants
Introduction
Children with neuromuscular disorders, Trisomy 21 and chronic lung disease often fail to thrive. In children with neuromuscular disorders, this may be due to swallowing dysfunction as a result of bulbar muscle weakness, feeding difficulties or gastro-oesophageal reflux (GOR). Good nutrition has been shown to be important [1], [2], [3]. Observational studies in children with neuromuscular disorders showed improvements in weight and a decrease in chest infections with assisted enteral feeding [1], [2], [3]. In children with Trisomy 21 there also maybe swallowing and upper airway dysfunction due to hypotonic tongue and lips [4] and poor oromotor coordination [5]. Trisomy 21 children with cardiac abnormalities may also suffer from fatigue during feeding and aspiration events may lead to respiratory tract infections, which are is the commonest most common cause of mortality in this patient group [6], [7], [8]. The potential link with aspiration in this patient group warrants investigation and treatment [9], [10], [11]. In children with chronic lung disease, failure to thrive is most often due to fatigue during feeding along with the presence of GOR.
Enteral feeding via naso-gastric (NG) or naso-jejunal tubes (NJ) is commonly used to optimise nutrition in children with long term respiratory failure. NG and NJ tubes have risks which include discomfort, epistaxis and exacerbation of GOR, along with the potential of tube displacement. In addition supplementary feeding via a NG or NJ tube may result in a poor mask fit in children who require ventilatory support in the form of non-invasive ventilation (NIV). Other options to NG or NJ feeding are surgical insertion of a gastrostomy or jejunostomy. Gastrostomy and jejunostomy can both be performed percutaneously using endoscopic or laparoscopic surgical techniques. However, there are significant risks associated with this surgery in infants and children who have chronic ventilator dependency [12], [13]. These include: gastrointestinal bleeding; increase ventilatory dependence post operatively; acute sputum retention; atelectasis; aspiration and potential worsening of GOR with gastrostomy.
In children with GOR medical therapy may be ineffective. In some instances GOR has been associated with an increased risk of gastroenteritis and community-acquired pneumonia [14], [15]. If GOR persists despite maximal medical therapy, a surgical anti-reflux operation, Nissen’s fundoplication is usually indicated. In some centres a Nissen’s fundoplication would always be carried out in combination with gastrostomy insertion [13], [16]. Yuan and co-workers [16] reported that in the year following gastrostomy and Nissen’s fundoplication in a small group of spinal muscular atrophy Type I and II children there was a decrease in the frequency of pneumonia.
In addition to procedure risks as previously described, there is a high peri-operative risk associated with general anaesthesia. These children may experience failed extubations or an increase in ventilator dependence as a result of the paralysis and sedation required for the procedure. There may be a critical reduction in upper airway calibre with large tonsils and as a result of the hypotonia seen in Trisomy 21, and this may make extubation difficult. By definition, children requiring ventilatory support have a poor respiratory reserve; other issues include poor lung function and ineffective airway clearance. Decisions about the indications for and timing of surgery must therefore be balanced against the risk of peri- and post-operative respiratory and procedure complications.
There are no randomised controlled trials investigating the most appropriate surgical procedure for enteral feeding in this patient group, nor are there likely to be, given their rarity and diagnostic diversity. We therefore report our experience and outcomes with multidisciplinary management for children who undergo surgery at our centre. The aim of sharing this experience is to inform future multidisciplinary approaches for managing high risk ventilator dependent children who require abdominal surgical procedures.
Section snippets
Methods
We identified 22 “high risk” (increased operative and anaesthetic risk) children based on a diagnosis of respiratory insufficiency or sleep disordered breathing undergoing gastrostomy or jejunostomy between 2006 and 2011. Data were collected from medical notes and electronic patient tracking systems. Descriptive data including referral information, patient age, age at supplementary feeding and NIV use, were noted. All children were investigated pre-operatively using the following protocol (Fig.
Results
Twenty-two children operated on between 2006 and 2011 were identified from our database. Diagnoses and age at commencement of supplemental feeding are shown in Table 1. Twenty-four procedures were carried out at our centre. Referral pattern is shown in Fig. 2. The majority of patients operated on at our centre were under our long term care.
Pre-operative investigations are shown in Table 2 and individual patient procedures and results are shown in Table 3. Ten children had video fluoroscopy, of
Discussion
This is the largest sequential case series describing the peri-operative management of children with severe ventilatory insufficiency in the UK who underwent gastrointestinal procedures. We have shown that our multidisciplinary assessment and management protocols are associated with relatively minor post operative complications and technically successful outcomes. Our PICU LOS was shorter than previously reported in a similar population [16] (mean; range LOS 1.9; 1–8 days vs. 3.6; 1–9 days) which
Funding
This work was supported by the NIHR Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London.
Acknowledgements
The Authors would like to thank the paediatric multidisciplinary team at the Royal Brompton Hospital for their contributions to the development of clinical protocols and the management of these children.
References (25)
- et al.
Efficacy and tolerance of gastrostomy feeding in Duchenne muscular dystrophy
Clin Nutr
(2010) - et al.
Early laparoscopic fundoplication and gastrostomy in infants with spinal muscular atrophy type I
J Pediatr Surg
(2008) - et al.
Randomised trial of inpatient versus outpatient initiation of home mechanical ventilation in patients with nocturnal hypoventilation
Respir Med
(2008) - et al.
Extubation of patients with neuromuscular weakness
Chest
(2010) - et al.
Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy
Chest
(1997) - et al.
Spinal muscular atrophy type 1: a noninvasive respiratory management approach
Chest
(2000) - et al.
Enterostomy tube placement in children with spinal muscular atrophy type 1
J Pediatr
(2006) - et al.
Gastrostomy placement in paediatric patients with neuromuscular disorders: indications and outcome
Dev Med Child Neurol
(2007) - et al.
Efficacy and tolerance of gastrostomy feeding in pediatric forms of neuromuscular diseases
J Parenter Enteral Nutr
(2002) - et al.
Swallow function in children with Down syndrome: a retrospective study
Dev Med Child Neurol
(1996)
An exploration of feeding difficulties in children with Down syndrome
Dev Med Child Neurol
Patterns in mortality over 60 years among persons with mental retardation in a residential facility
Ment Retard
Cited by (16)
Physical Therapies in Pediatric Respiratory Disease
2019, Kendig's Disorders of the Respiratory Tract in Children37 - Respiratory Complications of Intensive Care
2019, Kendig's Disorders of the Respiratory Tract in ChildrenStandard of Care for Spinal Muscular Atrophy
2017, Spinal Muscular Atrophy: Disease Mechanisms and TherapyDescribing nutrition in spinal muscular atrophy: A systematic review
2016, Neuromuscular DisordersCitation Excerpt :Prevalence of nutrition support via feeding tube ranged from 0% to 98% [37,43]. 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].