Best Practices In The Emergency Department Management Of Children With Special Needs

Table of Contents

 

1. Abstract
2. Case Presentations
3. Introduction
4. Critical Appraisal of the Literature
5. Etiology And Pathophysiology
   1. Gastrostomy Tube Types And Indications
   2. Tracheostomy Tube Types And Indications
   3. Ventricular Shunt Types And Indications
   4. Children With Developmental And Behavioral Disabilities
6. Differential Diagnosis
   1. Gastrostomy And Gastrojejunal Tubes
   2. Tracheostomy Tubes
   3. Ventricular Shunts
   4. Children With Developmental And Behavioral Disabilities
7. Prehospital Care
8. Emergency Department Evaluation
   1. General Approach To The Initial Evaluation
9. Diagnostic Studies
   1. Gastrostomy And Gastrojejunal Tubes
   2. Tracheostomy Tubes
   3. Ventricular Shunts
      1. Computed Tomography And Magnetic Resonance Imaging
      2. Plain Radiography
      3. Tapping The Shunt
      4. Shunt Flow Studies
      5. Other Diagnostic Studies
10. Treatment
    1. Gastrostomy And Gastrojejunal Tubes
       1. Gastrostomy Tube Replacement
       2. Gastrojejunal Tube Replacement
       3. Removal Of Gastrojejunal Tube Obstruction
       4. Correcting Leakage At The Stoma Site
       5. Infection
    2. Tracheostomy Tubes
    3. Ventricular Shunts
       1. Shunt Obstruction
       2. Shunt Infection
       3. Abdominal Pseudocyst
       4. Children With Developmental And Behavioral Disabilities
       5. Nonpharmacologic Measures
       6. Sedative/Analgesia Considerations
       7. Painful Procedures
          • Topical Anesthetics
          • Opioids And Benzodiazepines
          • Ketamine
       8. Nonpainful Procedures
          • Benzodiazepines
          • Barbiturates
          • Propofol
          • Dexmedetomidine
          • Etomidate
          • Chloral Hydrate
          • Nitrous Oxide
       9. Alternate Routes Of Sedation/Analgesia
11. Special Circumstances
12. Controversies And Cutting Edge
    1. Ventricular Shunt Flow
13. Disposition
    1. Gastrostomy And Gastrojejunal Tubes
14. Summary
15. Risk Management Pitfalls In The Management Of Children With Special Needs
16. Time- And Cost-Effective Strategies
17. Case Conclusions
18. Clinical Pathways
    1. Clinical Pathway For Management Of Pediatric Patients With Tracheostomy Malfunction (Hypoxia, Respiratory Distress, Cyanosis)
    2. Clinical Pathway For Management Of Pediatric Patients With Suspected Cerebroventricular Shunt Malfunction (Headache, Vomiting, Altered Behavior)
    3. Clinical Pathway For Management Of Pediatric Patients With Developmental Disorders Who Require A Procedure
19. Tables
    1. Table 1. Differential Diagnosis Of Gastrostomy Tube Complications
    2. Table 2. Differential Diagnosis Of Tracheostomy-Related Complications
    3. Table 3. Differential Diagnosis Of Ventricular Shunt Complications
    4. Table 4. Nonpharmacological Measures To Reduce Anxiety In Children With Developmental Delay Or Autism Spectrum Disorder
20. References

Abstract

Children with special needs have a wide variety of physical and developmental challenges. These children often have medical devices, subtle presentations, and behavioral or psychiatric issues that demand consideration when delivering emergency care. Some of the more common issues include the evaluation and management of complications in devices such a gastrostomy/jejunostomy feeding tubes, cerebroventricular shunts, and tracheostomy tubes, as well as impediments to the performance of common emergency procedures (eg, laceration repair, sedation for imaging) on children with developmental or behavioral disorders. This review will examine each of these circumstances and provide guidance on the best approaches to managing these patients.

Case Presentations

A 7-year-old girl with an intellectual disability arrives to the ED with her parents, who state that the girl has been lethargic all day, is not tolerating her gastrostomy tube feeds, and is having some leakage and redness around the exit site of her tube. She has been vomiting nonbloody, nonbilious material with every feeding attempt. Her parents describe her breathing as “harder than usual,” with an increase in her baseline home oxygen requirement. In addition, they noticed some scant blood from her tracheostomy tube after suctioning thick whitish-yellow secretions from it. Her urine output has decreased from 4 times per day to 1 time per day. She has a low-grade fever to 38.2°C. Her past medical history includes premature birth at 25 weeks with a complicated NICU stay, including hypoxic-ischemic encephalopathy, grade IV intraventricular hemorrhage requiring placement of a ventriculoperitoneal shunt, gastrostomy tube placement, prolonged intubation with chronic lung disease, tracheostomy tube placement, a seizure disorder, and significant developmental delay (the child is nonverbal) with static encephalopathy. With her many problems, how do you evaluate the cause of this child’s vomiting, lethargy, and difficulty breathing?

A 3-year-old boy with Down syndrome is brought to the ED 1 hour after pulling out his percutaneous endoscopically placed gastrostomy tube, which was placed 2 years ago. The mother believes he broke the balloon when he yanked the tube out. His past medical history includes repair of a ventricular septal defect and failure to thrive. What are the best ways to manage the dislodged tube?

An 8-year-old boy with autism arrives to the ED with a 5-cm scalp laceration extending to the temporal area of the head that he sustained after falling from a 5-foot height. His parents report a loss of consciousness for 1 to 2 minutes as well as abnormal behavior. You decide he needs a CT scan to evaluate for intracranial injury, and the laceration will need to be repaired. How do you approach the sedation of this child so he can safely and effectively undergo the interventions he requires?

Introduction

Children with special healthcare needs are defined as children “who have or are at increased risk for a chronic physical, developmental, behavioral, or emotional condition and who also require health and related services of a type or amount beyond that required by children generally.”¹ An estimated 12% to 18% of children in the United States have special needs,^2-5 and they account for 5 times the number of doctor visits as children without special needs.

Children with pervasive neurodevelopment disorders, autistic spectrum disorder (ASD), and developmental disorders, and behaviorally complex children are frequent patients in the emergency department (ED). These children often have complex medical histories and various comorbid conditions that complicate their care. In addition, these children may be limited in their capacity for cooperation and communication, making diagnosis and treatment difficult. In a Canadian study of patients with ASD who presented to the ED at a tertiary care center, 33 of 160 patients (20%) were admitted, 71% had an emergency triage score of 1 to 2 (suggesting high-acuity problems), and 79% had > 1 chronic health problem. Furthermore, the study found that patients with ASD comprised 2% of all ED visits. Parents of children with developmental disorders may also be more likely to use the ED as their medical home, especially if they do not have a well-established relationship with a primary care provider. In a survey of ED usage by children in the United States with developmental disorders, children with ≥ 3 ED visits were found to report less access to primary care sources than patients with no ED visits, who often reported well-established primary care.⁷

The term “children with special needs” covers a broad array of children, including those born prematurely, children who suffered hypoxia in the perinatal period, children with developmental and behavioral conditions, and medically complex patients, such as those with congenital cardiac and brain disorders, genetic disorders, inborn errors of metabolism, and mitochondrial diseases. This review focuses on identification and management of common problems with enteral feeding tubes, tracheostomies, and ventricular shunts, and will include a discussion on performing emergency procedures in children with autism and other cognitive disabilities.

Critical Appraisal Of The Literature

An extensive literature search was conducted on PubMed using combinations of the following search terms: special needs, children, disabled, technology dependent, epidemiology, prevalence, emergency department, gastrostomy, percutaneous endoscopic, compli-cations, jejunostomy, migration, ventriculoperitoneal shunt, infection, cellulitis, malfunction, tracheostomy, tracheoinnominate fistula, tracheitis, mucociliary clearance, emergency procedure, bleeding, sedation, autism, developmental delay, virtual reality, child life specialist, autism, behavior, procedure, management, acute setting, imaging, MRI, agitation , and aggressive . Only articles whose subjects included children aged 0 to 18 years were identified. Abstracts were reviewed for relevance to the topic. Supporting articles were gathered from related articles and the reference lists of review articles. All relevant articles were selected, reviewed, and included in the bibliography. Overall, there is a dearth of literature in this patient population, and more studies are needed.

Risk Management Pitfalls In The Management Of Children With Special Needs

1. “The G tube is leaking around the exit site. I’ll just upsize it to close the hole.”
   Upsizing a leaking G tube runs the risk of expanding the size of the stoma, which may lead to further expansion of the hole. Instead, pull the tube toward you until the tube is flush against the anterior abdominal wall. Make sure the balloon is inflated and functioning. If these maneuvers fail, consult with the service (usually surgery) that placed the tube.


2. “The blood from the tracheostomy was flowing pretty heavily when he got here, but now it’s stopped, his hemoglobin is normal, and the dad told me he was suctioning him every hour before it started. I’m going to send him home. The ear, nose, and throat specialist doesn’t need to be called in.”
   Tracheoinnominate fistula is a life-threatening condition that can’t be missed. Often there will be a sentinel bleed prior to a clinically important larger bleed. An ear, nose, and throat specialist should evaluate the tracheostomy with bronchoscopy if the amount of blood is impressive, even if it has since stopped. Tracheoinnominate fistula, bleeding granulomatous tissue, and tracheitis are all possible diagnoses, or excessive bleeding could be caused by aggressive suctioning.


3. “The patient’s mom told me that she put the dislodged G tube back in, and she’s been feeding him through it, so I don’t need to check its placement.”
   At the very least, pull back stomach secretions from the tube, and check the pH to ensure that the tube is, in fact, in the stomach. Make sure that the tube is amenable to a parent’s replacing it, such as a G tube with balloon, and not a GJ tube or one without a balloon.


4. “He just started vomiting, so he’ll probably develop diarrhea soon. It’s probably just a virus.”
   Whether this patient has a shunt or feeding tube or a history of abdominal surgery, maintain a lower threshold to work up the child with special needs who is vomiting. This could represent shunt malfunction, tube obstruction, intestinal obstruction, medication side effects, nonaccidental head trauma, or decompensation of a metabolic problem.


5. “The head CT scan shows small ventricles, so the symptoms must not be related to the VP shunt.”
   A subset of patients with VP shunts has nochange in ventricle size on CT, despite having a malfunctioning shunt. If the patient demonstrates symptoms of shunt obstruction, consult with neurosurgery to evaluate the patient, regardless of results of brain CT.


6. “He has tachycardia and fever, but he is an older child with special needs, so we don’t have to worry about his having a serious bacterial infection.”
   Sepsis is more common in children with special needs than in the general pediatric population. Although sepsis is not a common diagnosis among children with special needs, abnormal vital signs should not be ignored in this population.


7. “This child with autism just needs a couple stitches in his lip. I don’t want to sedate him, be-cause it’s too risky, so I’ll just hold him down.”
   Children with autism respond to procedural sedation as well as children who do not have autism using similar amounts of sedation medication. Follow usual protocols, avoid polypharmacy, and be ready with backup airway maneuvers.


8. “The CT is normal, and the VP shunt flow was normal when the neurosurgeon tapped it, so it must not be the shunt causing the headaches, lethargy, and vomiting.”
   In a small prospective study, 64% of the children had symptoms of shunt malfunction with a normal CT and normal shunt tap.70 Do not ignore signs of elevated ICP.


9. “I can’t find a source of fever in this patient who has a VP shunt, but we don’t need to tap the shunt, since he had his last revision 1 month ago.”
   Ninety percent of VP shunt infections will occur within the first 6 months after placement or revision. Children who have fever within the first month are at highest risk for VP shunt infection, so the shunt should be tapped to investigate for infection if no other obvious source is present.


10. “I don’t want the family to have to watch this fragile child with special needs go through this code. This family has been through enough.”
    Children with special needs are no different from other children, and parental presence should be offered in these situations. Importantly, you should determine early in the resuscitation efforts what the end-of-life care preferences are, including Allow Natural Death, DNR, or variations of these plans.

Tables

References

Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.

To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study will be included in bold type following the references cited in this paper, as determined by the author, will be noted by an asterisk (*) next to the number of the reference.

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