Children with severe neurologic impairment comprise a diverse group from an etiological perspective. Yet despite this diversity, there are predictable commonalities in many of the comorbidities and complications they experience. As an example, a child with a history of hypoxic ischemic encephalopathy and another with a genetic disorder associated with cerebral dysgenesis each have different reasons for their presentations but will likely have in common issues such as intellectual disability, risk for seizures, risk for oromotor dysfunction and aspiration, gastrointestinal dysmotility, osteopenia. and altered muscle tone. Please see Chapter 180 for a brief introduction to etiology and comorbidities as well as review of management of common acute care issue in this population. The focus of this chapter is on chronic comorbidities.

The hospitalist who appreciates common comorbid conditions in children with severe neurologic impairment will be prepared to more fully evaluate the child admitted with an acute illness for predisposing conditions which if treated may reduce their risk of recurrent illness and hospitalization. In addition, length of hospital stay is often longer in children with severe neurologic complexity. As a result, chronic issues which may not directly relate to the admission but require attention and management are likely to surface. The range of comorbidities in children with underlying neurologic impairments is quite extensive and can encompass virtually any organ system (Table 181-1). Data relating to prevalence of specific conditions are sparse, and when available usually relate to a specific underlying condition such cerebral palsy.¹ Data that delineate a broader range of associated medical conditions in children with disability do not relate specifically to children with neurologic complexity.² Despite the range of possible comorbidities and the lack of prevalence data, a systematic approach to assessment and management can enable the care provider to feel confident in monitoring for likely complications. What follows is a review of the most common comorbid conditions clinically encountered in children with severe neurologic impairment along with guidelines to their outpatient management.

NEUROLOGIC

Many children with severe neurologic impairment have an increased predisposition to seizure disorders. If concern for seizures is identified, an EEG is indicated along with referral to a neurologist for assistance in further evaluation and management. Many children with medical complexity who have with seizure disorders end up on multiple antiepileptic agents which in turn can be associated with detrimental side effects such as somnolence, behavioral change, hepatitis or pancreatitis, or kidney stones.³ For particularly refractory seizure disorders, more radical treatment options are considered. These include the ketogenic diet, vagal nerve stimulator, and on occasion, neurosurgical ablation of the epileptic focus.

Developmental delay is often identified in children with underlying complex neurologic disorders. There may be intellectual disability or learning disabilities that affect social and academic achievement. Obtaining accurate testing for cognitive ability is challenging in the child who may have motor coordination difficulties, dysarthria, or sensory impairments. Testing must be conducted by specialists trained to work with this population of children. Delays are often noted in all streams of development, including speech, gross and fine motor skills, and social/adaptive skills.

Behavioral dysregulation, including self-injurious behaviors, occurs with some frequency in the child with underlying neurologic impairment. Approaches to management include behavioral interventions, and at times, mood stabilizing medications.⁴ It is important to recognize when behaviors reflect frustration with communicative abilities and to work on augmenting options for the child as much as possible. Additionally, if the behavior is self-injurious it is important to rule out pain as an etiology. A thorough multisystem evaluation is warranted in these cases (see Chapter 180, Table 180-2 for a differential diagnosis).

Children may have hydrocephalus secondary to central nervous system (CNS) malformations, cysts, meningitis, tumors, or intraventricular bleeding. Ventriculoperitoneal or ventriculoatrial shunts are often placed in this condition. These devices are prone to dysfunction and may cause acute problems related to increased intracranial pressure or infection, as discussed in Chapter 180.

Many children with severe neurologic impairment demonstrate oromotor dysfunction, which is responsible for significant morbidity.⁵ Dysphagia can result in challenges in chewing and swallowing and increased risk of aspiration of foods and liquids. It can also result in diminished oral intake associated with poor weight gain and malnutrition. Decreased fluid intake can further exacerbate constipation. In addition, oromotor dysfunction can contribute to sialorrhea. The child who drools excessively is usually not producing an excess of saliva but rather has difficulty managing the secretions he or she does produce. Finally, from a developmental perspective, oromotor dysfunction can affect speech. It is extremely important when evaluating the nonverbal or dysarthric child to make no assumptions about underlying cognitive ability, as the two are not always correlated. Augmentative technology can play a critical role in facilitating a child’s communication.

SENSORY IMPAIRMENT

Children with underlying neurologic conditions often have associated sensory impairment. In addition to the routine ophthalmologic conditions of childhood, common concerns in this population include cortical visual impairment (inconsistent visual attentiveness), amblyopia, nystagmus, visual field cuts, refractory errors, and exposure keratosis. Hearing loss may take the form of sensorineural loss or may be conductive, perhaps on the basis of congenital malformation. Early identification of any degree of vision or hearing impairment is important in determining the best mechanisms of communication for a child as well as devising educational plans and interventions.⁶ Children with unidentified sensory impairments can appear to have lower cognitive abilities and decreased social awareness than may be actually true. Exploring their baseline level of cognitive functioning with parents and other caregivers is helpful.

GROWTH, NUTRITION, AND GASTROENTEROLOGY

The enteric nervous system (ENS) governs the function of the gastrointestinal system. Even though the ENS can operate autonomously, it is modulated by extensive input from the central nervous system (CNS). As a result, children who have underlying disorders affecting the CNS end up with dysfunction of the gastrointestinal system.⁷ Dysmotility is thus quite common. Poor nutrition is a complex issue which reflects not only complications of dysmotility but an interplay between neurologic, endocrine, and environmental systems as well.⁸

Disorders of foregut dysmotility are seen in the form of esophageal dysmotility, changes in upper and lower esophageal sphincter function, and gastric dysmotility. This clinically manifests as retching, vomiting, and gastroesophageal reflux disease (GERD). Other factors contribute to GERD in this population as well, including hiatal hernia, prolonged periods of lying supine, and increased intra-abdominal pressure secondary to spasticity. GERD is a clinical diagnosis; however, it is important to establish objective findings in some cases with impedance probe studies. Treatment of GERD should be addressed with a proton pump inhibitor alone or in combination with a histamine type 2 receptor antagonist. There is growing evidence that baclofen can also be used in cases of severe GERD.⁹ If delayed gastric emptying is present, erythromycin 30 minutes prior to meals can help with gastric motility.

Persistent or refractory GERD can lead to pulmonary complications of recurrent pneumonia and progressive lung injury. If medical management does not adequately control symptoms, surgical intervention should be considered. A Nissen fundoplication involves wrapping the fundus of the stomach around the gastroesophageal junction. Fundoplication corrects a hiatal hernia (if present), lengthens the intra-abdominal portion of the esophagus, and increases the pressure of the lower esophageal sphincter, thereby helping to prevent GERD. This procedure is not without complications, and retching, dumping syndrome, and bloating occur with some frequency.¹⁰ A nonsurgical option for children who have a gastrostomy is feeding directly into the jejunum via a gastrojejunal (GJ) tube. GJ tubes are associated with risk of intussusception and dumping. More frequently encountered problems include dislodgement and recurrent clogging of the tube necessitating trips to interventional radiology for replacement. The decision to place a GJ tube versus performing a Nissen fundoplication is still a matter of ongoing research. At this time, the decision should be made on an individual basis, balancing the potential risks and benefits of each procedure and local practice patterns.¹¹

Chronic constipation is a common problem in this population, with an estimated prevalence of 26% to 50%.⁷ Poor colonic motility, physical inactivity, medications, and spasticity are some of the risk factors. Constipation is associated with abdominal pain, discomfort, urinary retention, recurrent urinary tract infections, and may be a cause of autonomic storming (paroxysmal sympathetic hyperactivity). Regular bowel movements are the goal, promoting efficient emptying, minimizing complications, and maximizing comfort. Change in dietary formulation with addition of fiber may be helpful. Polyethylene glycol 3350 powder is a mainstay of treatment and may be dissolved in any noncarbonated liquid. Although it is tasteless, it does alter the viscosity of the fluid in which it is dissolved. Some children resist it for this reason when it is taken by mouth, but it is generally well tolerated. It may be supplemented with senna, bisacodyl suppositories, or other stimulants. Suppositories may help parents regulate the time of bowel movements. Sometimes rectal irrigations or enemas are required. Keep in mind that a stimulant laxative may cause cramping if the patient is impacted. Do not use mineral oil in this patient population because of the risk of aspiration. At times, severe constipation can be refractory to medical management and surgical intervention may be considered. The antegrade colonic enema (ACE) procedure involves using the appendix as a stoma, which can then be used for intermittent colonic irrigation with normal saline or polyethylene glycol, facilitating evacuation.⁷

In addition to primary problems of dysmotility, poor nutrition is another common gastrointestinal disorder in children with neurologic conditions. Established growth curves have not been created for many of these conditions, some of them quite rare or unique, further complicating the creation of nutrition and growth goals. When poor nutrition is present or suspected, the reasons can be far-ranging, from oromotor dysfunction with dysphagia, resulting in diminished intake to dysmotility, resulting in diminished appetite. In the case of dysphagia, feeding therapy to address oromotor coordination and proper seating can be useful. The most concerning complication of dysphagia is the risk for aspiration. Thin liquids are the texture most likely to be aspirated because of the velocity at which they traverse the oral cavity. However, solids that require significant chewing may be swallowed whole and pose significant aspiration risk as well. Careful history must be taken to uncover possible aspiration. While coughing or choking during meals is a helpful sign when present, aspiration can be silent. History should be obtained relating to color change during meals, watery eyes, development of a “wet” voice, or need for frequent throat clearing. If any of these signs are identified, a modified barium swallow study should be obtained. If aspiration is present, frank discussion with the family is necessary to determine alternative feeding options. A child who aspirates thin liquids but not thicker ones may be able to consume an oral diet containing thickened liquids. If all consistencies are aspirated or if a child simply cannot take in enough fluid via a thickened liquid diet, a gastrostomy tube to allow direct enteral access may be indicated.

At times, discomfort secondary to GERD or constipation may lead to diminished appetite, which then leads to poor weight gain or weight loss. In these cases, treating the underlying cause may result in enhanced appetite. Cyproheptadine can occasionally be a useful agent to increase appetite.

There are several other gastrointestinal problems found commonly in this population. Pancreatitis can be a complication of medications. Hepatitis is another common effect of medications, particularly anticonvulsants and baclofen. Finally, gallstones are found on occasion, particularly in the child who has a history of parenteral nutrition in the past. In the child presenting with unexplained irritability, screening labs should include liver function tests, amylase, and lipase. An abdominal ultrasound is also a reasonable screen. If gallstones are identified, elective cholecystectomy is often indicated—particularly in the nonverbal child who is unable to communicate his or her discomfort—in an effort to avoid future emergent surgery.

AIRWAY CONCERNS

Along with complications of the gastrointestinal tract, complications of the airway are among the most prevalent comorbid conditions in children with underlying neurologic impairment. They are also among the conditions most likely to pose life-threatening events and/or impact life expectancy. Respiratory concerns may involve the upper or lower airway.