Nutrition

Many developmental disabilities share a risk of nutritional problems, including both undernutrition (generalized malnutrition and specific nutritional deficiencies) and overnutrition (overweight and obesity). Both may complicate orthopaedic care, presenting barriers to wound healing and increasing the risk of postoperative complications. For example, with further weight loss and elevated catabolism during the postoperative period, the underweight child with cerebral palsy is in danger of developing superior mesenteric artery syndrome. The resultant intractable emesis increases the probability of aspiration pneumonia. Beyond the surgical outcome, the child’s life may be in jeopardy.

Children may present to the orthopaedist with clinically significant nutritional problems not successfully addressed by other health care professionals or families. Primary physicians are too often distracted by the child’s other medical issues or mistakenly consider poor nutrition to be a “normal” part of the child’s disability. For families, clinically addressing nutritional issues may threaten the essential role of the parent in feeding the child.

If reliable measures of weight and height (or length) can be obtained, clinicians can plot these values on standard growth curves and determine the child’s body mass index. This value, which has replaced weight-to-height ratios, generally reflects nutritional status. The presence of contractures, limb disproportion because of genetic conditions or malformation, severe kyphoscoliosis, or similar body habitus differences renders typical growth chart plots suspect. In these instances, a dietitian obtains more reliable information using skinfold measurements that reflect fat stores and muscle mass. These measures can be supplemented, but not replaced, by standard measures such as absolute neutrophil/lymphocyte counts and serum albumin, iron, and zinc levels.

The poorly nourished child with a developmental disability requires a team approach to define and augment the nutritional status before surgery. The dietitian is a key member, working to identify more nutritional options within the range of foods and drinks palatable to the child, culturally acceptable to the family, and financially feasible. Rarely will the dietitian be successful without the physician’s support for and endorsement of making these important nutritional improvements as a condition for optimal surgery. A developmental pediatrician or other pediatric subspecialist, in concert with the child’s family physician, can assess the child for medical barriers to adequate intake, such as dysphagia, gastroesophageal reflux disease (GERD), or anorexia secondary to medications.

Occasionally, gastrostomy placement is required to effect nutritional improvement or ensure airway protection. Without adequate information and support from the team, this option is too often viewed as a traumatic step for families. When indicated, parents need preparation and supportive education about the procedure and the aftercare. With these accomplished, gastrostomy placement is usually associated with sustained improvements not only in growth, but in overall quality of life. These outcomes are commonly sustained long past recovery from the precipitating orthopaedic procedure.

The coordinated team approach with efforts to increase family participation is essential to addressing weight management in children with developmental disabilities. In preparing the overweight or obese child for orthopaedic surgery, the goal of significant weight loss is unlikely to be met without an extended period of time and an integrated program devoted to weight loss in children. Even with such a program in place, many children with obesity as a component of their developmental disability come to surgery obese. In this setting, the focus of the interdisciplinary team should be the identification of added risks associated with the obesity, such as restrictive lung disease, upper airway obstruction, diabetes, hypertension, and hypopnea. Development of an adequate plan for management requires input from anesthesiologists, pulmonologists, nursing, and respiratory therapists for preparatory and postoperative management.

Gastrointestinal Issues

Dysphagia, or abnormal coordination of swallow, commonly includes aspiration of foods, liquids, or saliva into the airway and lungs. In children with developmental disabilities, chronic pulmonary aspiration is usually “silent,” such that the child does not cough or choke at the time of swallowing. Rather, manifestations include indirect signs such as a wet voice quality, chronic congestion, or recurrent wheezing or pneumonia. Potential screening questions for dysphagia are listed in Box 9-2 .

Further complicating the situation for the orthopaedist, the child and familiar caregivers may compensate fairly well for the undiagnosed dysphagia. These compensations may be compromised after sedation, supine positioning in a spica cast, or other interventions that can worsen the dysphagia and result in a clinical aspiration event. Children with known dysphagia are best reevaluated by the physician managing their dysphagia before surgery so that updated recommendations can be communicated to the inpatient nursing staff. Those whose pulmonary history or symptom profile suggests aspiration benefit most from referral to an experienced pediatric dysphagia team for assessment of feeding and swallowing. A videofluoroscopic swallowing study can be structured to account for the child’s positioning, nutritional status, and medical problems, among other considerations.

During childhood, clinically significant GERD may present with indirect signs, such as poor oral intake, irritability, sleep disruption, or recurrent episodes of bronchospasm or pneumonia rather than with emesis or “heartburn.” In children with developmental disabilities, children at either weight extreme (poorly nourished or obese) and those with cerebral palsy are at particular risk. The pH probe provides a valid quantitative measure of esophageal acid exposure but the severity of pathologic acid reflux may not correlate with symptom severity or complications. Furthermore, this can be a challenging and stressful test to perform in an uncooperative child, leaving endoscopic studies of esophagitis and empirical treatment as diagnostic options. Nuclear scintigraphy may be useful in children with suspected gastric retention. Management options include acid blockers (ranitidine, omeprazole), prokinetic agents (metoclopramide), and surgical gastric fundoplication. In the surgical setting, where aspiration of gastric acid is the greatest concern, an acid blocker from the proton pump inhibitor class is often chosen, because it strongly reduces gastric acid production and also has some prokinetic activity. Fundoplication, with potential complications of dumping syndrome or bloating, is usually reserved for situations in which gastrostomy supplementation is also required for nutritional rehabilitation.

Other motility disorders are endemic to children with developmental disabilities. Constipation, abnormal esophageal motility, and delayed gastric emptying can have a clinical impact. Optimal management of constipation before surgery can help avoid poor oral intake, pain, and fecal impaction after surgery when narcotics are prescribed. The constipation in children with developmental disabilities is much more likely to require stimulant medication than typical childhood functional constipation. The use of mineral oil is contraindicated if there is any possibility of dysphagia. A dietitian can often assist families in devising strategies to augment fluid intake, a common underlying issue in this population. Similarly, fiber intake and need for fiber supplementation can be determined. Finally, if significant constipation has already developed, a “cleanout” can be undertaken using both stimulant agents by mouth (e.g., milk of magnesia or magnesium citrate) and enemas per rectum. Care must be taken when using phosphate enemas in the presence of renal disease. For longstanding constipation, radiographic documentation ensures the efficacy of the presurgical cleanout efforts.

Pulmonary Issues

Children with developmental disabilities are at risk for airway and pulmonary problems, frequently interrelated with other medical issues. For example, aspiration, whether due to dysphagia, GERD, or both, can cause chronic reactive airway disease and acute pneumonia. Obesity can lead to both restrictive lung disease and upper airway obstruction with apnea.

Upper airway obstruction is a particularly common problem among children with cerebral palsy and trisomy 21, with the incidence in the latter population estimated to be as high as 75%. Frequently unrecognized in these populations, it can be unmasked in the perioperative period when sedatives or narcotics exert their effects.

Clinicians usually rely on identification of risk factors, prior history of airway problems, and symptoms such as loud snoring with pauses and unexplained daytime somnolence. Once suspected, polysomnography can define the extent of airway compromise and guide interventions, which could include initiation of nighttime bilevel or continuous positive airway pressure (such as a BiPAP or CPAP), or ear-nose-throat intervention for tonsillectomy. Upper airway fluoroscopy can show dynamic upper airway collapse. Laryngomalacia and tracheomalacia can be identified by flexible fiberoptic bronchoscopy. Otolaryngologic evaluation helps detect nasal and pharyngeal obstructions. Documentation of intervention effectiveness aids management. In children with any degree of upper airway obstruction, excessive administration of supplemental oxygen may hasten the onset of apnea without interventions to maintain an airway.

Reactive airway disease occurs more commonly among children with developmental disabilities for two main reasons: the frequency of premature births, and the tendency for gastroesophageal reflux and aspiration to trigger reactive airway symptoms. Thus addressing the underlying cause of the wheezing often allows optimal management. Controlling airway inflammation is critical in children with more than mild, intermittent symptoms. Inhaled steroids remain the mainstay of treatment for children with more than the mildest degree of asthma. Children whose parents report frequent treatment with bronchodilators but who are not on inhaled steroids or other antiinflammatory medications warrant reassessment before general anesthesia.

Not uncommonly, children with special needs present to the orthopaedist with a history of “chronic lung disease,” which often represents a poorly characterized combination of restrictive or obstructive lung disease, with some component of airway compromise or central apnea as well. For example, a child with a genetic syndrome may have some restrictive lung disease because of a small chest cavity, with some component of lung scarring because of chronic aspiration. Preoperative involvement from a pediatric pulmonologist and a respiratory therapist helps develop a preoperative and postoperative care plan to minimize airway complications.

Osteopenia

Children with cerebral palsy and spina bifida exhibit an elevated risk of fractures, and osteopenia is often a contributor. Osteopenia can negatively influence the surgical outcome in other diagnostic groups. Particularly at risk are children who are chronically underweight, have poor intake of dietary calcium, or receive a variety of medications, including certain anticonvulsants, steroids, and hormonal preparations such as depo-progesterone. While plain radiographs poorly define the presence or absence of osteopenia, it can be measured with bone densitometry. Care must be taken in the positioning of the patients to obtain accurate results, and to be meaningful, the results must be compared with appropriate controls. The predictive value of bone density results for assessing the risk of future fractures in children remains unproven. Ensuring adequate, but not excessive, calcium intake and optimal renal function before and after surgery is recommended.

Cerebral Palsy

The preceding discussion of preoperative issues mentions cerebral palsy because of its higher requirement for orthopaedic care and the frequency with which it affects systems beyond the neuromuscular. This is increasingly true as the severity of the child’s cerebral palsy worsens. However, even the child with milder motor deficits related to cerebral palsy may frequently present with associated complications, such as seizures, dysphagia, or reactive airway disease. Common associated problems and their estimated frequency are listed in Table 9-1 .

Direct communication with the child’s other pediatric specialists before surgery to develop a comprehensive preoperative and perioperative plan of care minimizes the chances of morbidity and prolonged hospitalization. An actively involved primary care physician or—when the child’s complexity warrants and where available—a neurodevelopmental pediatrician can coordinate this “big picture” plan for the child. Issues likely to pose postoperative complications include the child’s nutritional state, pulmonary and airway status, and presence of dysphasia, GERD, or constipation. At times, these require a lengthy period of focused intervention and significant interventions, such as gastrostomy placement, may be indicated. Often, the goal of a desired orthopaedic procedure motivates a family to make changes that not only facilitate the upcoming surgery but have a long-term positive impact on the child’s quality of life.

If the child is one of the 30% with cerebral palsy who also have epilepsy, he or she is likely to be observed by a pediatrician or neurologist. The practitioners’ advice is valuable regarding the status of the child’s seizure control and the impact of the child’s anticonvulsant medications on surgical issues (e.g., hemostasis, liver function).

Dental status is a potentially important component of care. Given the multitude of medical issues, the many demands of care, and the great difficulty families have in finding a dentist willing to treat a child with a neuromuscular disorder, dental disease is a potential surgical concern. Untreated dental decay can act as a source of bacterial seeding.

Finally, prolonged casting and school absence may present a myriad of additional care issues to already stressed families. The impact of these obstacles to care routines, parental employment, and other issues are best explored proactively to prevent unnecessary delays in discharge. The roles that various team members can play in this process are discussed later.

Spina Bifida

In contrast to the wide variation seen in children with cerebral palsy, the child with spina bifida has a more predictable constellation of problems. These issues vary greatly from one child to the next, relative to the specific nature of the individual’s neural tube defect and the associated neuromuscular variations. The categorical nature of the issues does not seem to make them any simpler to treat. The status of the interrelated problems (e.g., neurologic/muscular/urologic/behavioral/gastrointestinal) can frequently change, sometimes suddenly. The child with spina bifida can develop life-threatening symptoms of shunt malfunction overnight despite recent radiographic and clinical assessment showing good shunt function. Problems commonly seen in young people with spina bifida are listed in Box 9-3 .

Studies show that persons with spina bifida who receive care through a coordinated interdisciplinary program have improved health outcomes. Interdisciplinary programs serve the pediatric orthopaedist well in ensuring that the child’s neurologic, nutritional, pulmonary, immune, endocrine, integumentary, urologic, and emotional systems are in an optimal state for surgery. In the absence of such a resource, the orthopaedic team should—at a minimum—ensure that the child is up to date with neurosurgical and urologic care, is meeting growth expectations, and has no symptoms of dysphagia, constipation, or sleep apnea.

During the hospitalization and recovery period, special care needs to be taken to prevent pressure- or shear-related ulcers, to which these children are predisposed given the variable presence of insensate skin. Incontinence, when present, may further contribute to the development of secondary wounds or negatively affect the primary surgical wound. Family-focused developmentally appropriate continence programs help address this. Pressure relief, especially during time in the operating room, is indicated. Interdisciplinary team members, especially occupational therapists, can be very helpful in anticipating the impact of the planned surgical procedure on the child’s self-care skills and, if a wheelchair is used, on his or her positioning and mobility.

Intellectual Disabilities

Hundreds of genetic syndromes have associated intellectual disabilities in common, but otherwise have distinctive features that may present different challenges to the orthopaedic team. Some of these are considered individually in Chapter 41 . Others, however, are commonly seen and worth specific mention. Children whose syndromes are associated with obesity and hypotonia, such as Prader-Willi syndrome (PWS) and trisomy 21, are at high risk of obstructive sleep apnea, which may predispose to postoperative airway compromise. Feeding difficulties appear to be more common as the degree of disability worsens, as does the tendency toward constipation. Increasing cognitive impairment also brings greater communication challenges. This can complicate pain management and cooperation with activity limitations, therapies, and treatment plans. Encouraging the family to bring in tools that work at home or school, such as communication devices and behavioral management plans, and sharing these with inpatient staff, improves care and decreases staff frustration.

Prader-Willi syndrome illustrates the complex issues that arise in children with syndromic intellectual disability. PWS is caused by a deletion of the short arm of the paternally derived chromosome 15, and prominent features of PWS include hyperphagia leading to obesity. Individually planned applied behavioral management programs can moderate the obesity (and other potentially problematic social issues). Growth hormone (GH) deficiency further contributes to the obesity by impairing deposition of lean body mass. Controversies exist over the use of GH in this syndrome, however. GH has been well documented to normalize body composition, improve strength, and enhance agility. Unfortunately, there have also been cases of death due to airway obstruction after initiation of GH therapy. To protect against this, current recommendations are for polysomnography before initiation of GH therapy. Sleep studies should be considered before surgery to better define not only airway compromise but degree of hypoventilation and restrictive lung disease that often accompany obesity.

Other features of PWS that may affect postoperative recovery include (1) decreased ability to vomit, leading to significant abdominal distention; (2) osteopenia, impairing bone healing; and (3) skin-picking behavior, complicating wound healing or leading to infections such as those caused by resistant strains of Staphylococcus aureus .

Autism Spectrum Disorders

Children with autism spectrum disorders also present challenges to orthopaedic care, given the varying degrees of impairment in communication, social, and behavioral domains. There may be associated intellectual disability, feeding problems, sensory disturbances, and disruptive behaviors. Features of autism may exist in children diagnosed with spina bifida, cerebral palsy, and intellectual disability syndromes, further complicating their surgical management.

The interpersonal/social impairments that are the hallmarks of these disorders may render it particularly difficult for the child to adapt to the transition from home to clinic or hospital. Unfamiliar providers and routines may overwhelm the child with marked social difficulties. Communication barriers may result in the child feeling bombarded by multistep instructions and procedures. Due to sensory issues, the child may have relatively extreme reactions, compared with children without autism, when faced with certain foods, sounds, smells, lights, textures, and types of touch. Blood draws, anesthesia, and radiologic studies may provoke severe temper tantrums and aggression.

Preparing the child and family adequately helps ease transitions and increases awareness of team members about what both triggers and soothes difficult behaviors in individual children. Using pictorial and gestural cues and eliminating extraneous stimuli aids in engaging the child in a calm environment.