Surgical Problems of Children with Physical Disabilities



Surgical Problems of Children with Physical Disabilities


Gordon Worley

Keith T. Oldham


Developmental Medicine and Pediatric Rehabilitation, Duke University Medical Center, Durham, North Carolina 27712.

Division of Pediatric Surgery, Medical College of Wisconsin, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin 53226.



Children with physical disabilities (cerebral palsy, spina bifida, spinal cord injury, and Down syndrome) benefit from a cooperative relationship between pediatricians and pediatric surgeons in managing their surgical needs. This benefit is manifested in the following ways:



  • By jointly managing problems, surgeons and pediatricians improve and simplify the care provided to children by parents, improve quality of life, and increase longevity.


  • The long-standing relationships between parents and pediatricians, and between pediatricians and pediatric surgeons, mean that decisions about surgical interventions are made in an atmosphere of trust and thoughtfulness.


  • The benefits and potential problems to patients of specific surgical interventions are well understood by all concerned, which is useful in helping families when adverse outcomes occur.


  • Good communication between pediatricians and surgeons results in appropriate selection of surgical candidates and expeditious evaluations of problems preoperatively, reducing lengths of hospitalization for surgery.

This chapter discusses surgical contributions to the management of four of the most serious common developmental disabilities: cerebral palsy, spina bifida, spinal cord injury, and Down syndrome. Although the term pediatrician is used to describe the kind of physician managing the medical problems of children with physical disabilities, such care is also provided by committed pediatric neurologists and pediatric physiatrists.

The importance of surgical solutions to some of the problems of children with physical disabilities is amply illustrated in Table 18-1. In one developmental pediatrician’s practice (GW), 55 patients had new problems diagnosed that required surgery in a 1-year period. This cooperative relationship works well because it respectfully recognizes both that pediatric surgeons can do the most good for these children based on specific surgical needs and that pediatricians contribute the necessary chronic care for their patients, managing most medical problems successfully and independently.


ETHICAL CONSIDERATIONS

Ethical theory and medical ethics are presented in detail in Chapter 19. The purpose of this section is to review aspects that require emphasis for the treatment of children with physical disabilities.

The principles on which decision making are based for children with disabilities are the same as for typical children: autonomy, beneficence, justice, nonmaleficence, veracity, and fidelity (1). Parents and legal guardians are recognized legally as substitute decision makers for neurologically impaired children.

It is important for pediatricians and pediatric surgeons to discuss openly and forthrightly their approaches toward the treatment of severely cognitively impaired children. Agreement on principles is essential to provide best care. When conflicts arise, helpful mediation by a single member of a hospital’s Ethics Committee can be useful, without being cumbersome and time consuming. If true conflicts exist over specific cases, then formal consideration by the hospital Ethics Committee can help with resolution (2), although this is rarely required.









TABLE 18-1 Patient Referrals for Surgery by One Developmental Pediatrician, January 1, 1994 to December 31, 1994.























































Procedure Patients Referred
Pediatric surgery 27
   Gastrostomy and fundoplication 10
   Gastrostomy alone 7
   Fundoplication alone 1
   Central line placement or removal 4
   Miscellaneous 5
Pediatric ophthalmology 11
   Strabismus
Pediatric neurosurgery 8
   Rhizotomy 3
   Central nervous system tumors (new diagnoses) 2
   Subdural hematoma 1
   Tethered cord 1
   Hydrocephalus 1
Pediatric orthopedics 4
Miscellaneous 5
Total 55


Advance Directives

In contrast to the situation for normal children, in which it is assumed that there is no limitation to the intensity of care that will be provided in an emergency (1), for children with profound mental retardation, who are hopelessly ill, or who have a progressive degenerative neurologic disease, it is important to determine in advance if parents want care to be limited. If parents request Do Not Resuscitate (DNR) status for their child, it is useful to have them sign a DNR statement in the medical record, both for clarity and for legal protection of physicians. In discussing limitations of care, a distinction should be made between initiation of life support and prolongation of life support. In our experience, many parents want the technique of resuscitation applied in the event their child is found dying but not dead, but want life support terminated if subsequently their child’s condition is found to be hopeless.

A distinction should also be drawn between DNR status and “Comfort Measures Only” status. DNR orders limit care only in circumstances that would otherwise be at the end of a patient’s life. The goal of treatment is still to prolong life. For patients with Comfort Measures Only status, it is recognized that the patient’s condition is hopeless (terminal), and the goal of treatment is no longer to prolong life but only to prevent suffering. For a patient to have a Comfort Measures Only status, a patient must also have a DNR status.


Exceptions to Do Not Resuscitate Orders

DNR orders do not apply to cardiopulmonary arrest resulting from an iatrogenic cause. Parents of a child with DNR status who is undergoing surgery should be informed that during the perioperative period, the DNR status may not apply because most causes of cardiopulmonary arrest occurring in this setting are iatrogenic (1).


Nonintervention in Children with Severe Disabilities

Advances in technology and in the application of technology have improved the longevity of children with even the most severe disabilities. For instance, one of us (GW) cares for a patient with Trisomy 18 who is minimally responsive and has restrictive lung disease, cardiomyopathy, and chronic congestive heart failure. Home ventilation, 20 hours per day nursing care, and frequent hospitalizations have resulted in her survival beyond 18 years of age.

Parent advocacy groups are quick to point out that denying care to a child because of the child’s disability is tantamount to discrimination against the child because of the disability and is, therefore, a denial of the civil liberties of the child. Quality of life considerations should not be taken into account, advocates believe, in making medical decisions about surgical interventions in children with severe disabilities.

However, physicians and surgeons do not have an ethical obligation to institute treatment to try to change the course of a condition in a child with severe disabilities and a hopeless immediate prognosis, if the treatment would be futile in prolonging life. Medical decision making for a child with severe disabilities should take into consideration the natural history of the underlying condition. Recently, life table analyses were published for patients with Trisomy 13 and Trisomy 18. Although the median survival was only 10 days, 5% to 10% of patients with these conditions survived beyond the first year of life (3). Presentation of data to parents about the natural history, the risks of surgery for mortality and morbidity, and the complications of surgery should be made empathetically, emphasizing to parents that the physician understands the magnitude of the tragedy that has befallen their child and them. If the surgeon considers a child not to be a surgical candidate because of the futility of surgery to prolong life in the surgeon’s experience, the surgeon does not have the obligation to perform surgery, even if parents request it. In this circumstance, it is important for the surgeon to explain to parents that the decision not to perform surgery is not due to the child’s disability, but rather to the futility of surgery for the purpose of prolonging life. For parents who continue to insist on surgery, referral to a more willing surgeon is an option.


CEREBRAL PALSY AND PROFOUND RETARDATION


Background

For purposes of discussion, cerebral palsy and severe and profound retardation without motor involvement are
linked because they share many of the same surgical problems. The focus of this discussion is on children with cerebral palsy because they are preponderantly the larger group needing surgery.

A consensus definition of cerebral palsy is that it is “an umbrella term covering a group of nonprogressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of its development” (4). Most cases of cerebral palsy and retardation are caused by abnormal brain development in the first trimester, not birth asphyxia or prematurity, as is commonly believed (5). Because it is not known how to prevent brain dysmorphogenesis, it is not surprising that the birth incidence of cerebral palsy, 1.5 to 2.5 per 1,000 live births, has increased only slightly in the last 40 years, despite the increased rate of cerebral palsy per 1,000 live births in the smallest surviving premature infants (6).

Cerebral palsy is classified according to the pattern of motor involvement of extremities (hemiplegia, one side; diplegia, lower extremities involved, upper extremities only mildly so; quadriplegia, all extremities involved) and by the type of neurologic dysfunction (spastic, hypotonic, dystonic, athetotic, or a combination) (6). Associated neurologic problems include seizure disorder, strabismus, visual field defects, sensorineural hearing loss, retardation, learning disabilities, and emotional problems. One-half of all children with cerebral palsy have normal intelligence, however, and some (especially among those with athetotic cerebral palsy from kernicterus) have superior intelligence.


Life Expectancy

The life expectancy of people with cerebral palsy and mental retardation is shorter than that of the general population. Those with mild disability can have a normal life span. However, the most severely involved patients have a much shorter than typical life span. Much effort has gone into development of life table analyses to predict survival of children with cerebral palsy, not for clinical use, but to determine “damages” in medical malpractice suits. For instance, children with cerebral palsy and fair motor control who can be fed by mouth have a relatively good prognosis, with a greater portion reaching adulthood than those without these skills. For children who are unable to lift their heads off a mat lying in the prone position and who are tube fed only, the median survival time was only an additional 7 years of life (7).


Surgical Considerations

Many children with cerebral palsy and other neurologic impairments have problems for which surgery is the only effective, long-term treatment. These include the following: malnutrition; neurologic dysphagia; aspiration; and delayed gastric emptying. The section on cerebral palsy in this chapter will cover: the indications for gastrostomy and/or fundoplication; complications of gastrostomy alone and of gastrostomy with fundoplication; outcomes of surgery; superior mesenteric artery syndrome; cryptorchism; and upper airway obstruction.


Malnutrition (Failure-to-Thrive)

The usual causes of undernutrition in children with cerebral palsy are inadequate caloric consumption due to poor oral motor function (neurologic dysphagia), increased metabolic demand due to hyperkinetic movement disorder (athetotic or dystonic cerebral palsy), food aversion as a learned response to the discomfort of eating caused by either gastroesophageal reflux or chronic aspiration (or both), and/or rarely parental neglect. Some patients have combinations of the above causes.

The general definition of undernutrition in children with cerebral palsy is the same as for typical children: a chronic nutritional state resulting from protein-calorie consumption that is inadequate to meet metabolic needs for health maintenance and growth over time. Undernutrition is associated with poor somatic growth, diminished bone mineral density, abnormal progression through puberty, limited societal participation, and poor health (8).

Growth charts for adequately nourished children with cerebral palsy are not available. Weight for height percentiles perform poorly to identify undernutrition in children with cerebral palsy. Adequate measurement of triceps skinfold thickness and mid-arm circumference and calculations from these measurements of mid-arm fat area requires expertise, expensive measuring tools, availability of tables of age-appropriate norms, and substantial time. Albumin and transthyretin concentrations are not valid indicators of undernutrition in this population and should not be used as screening tests. New techniques to measure total body fat are currently being developed and may have potential diagnostic usefulness. In the meantime, adequacy of nutritional status can be assessed by experienced physicians examining subcutaneous fat on the face, thorax, abdomen, buttocks, and extremities, and by following growth velocities over time.


Neurologic Dysphagia

The severity of neurologic dysphagia can be roughly judged by the time it takes parents to feed a child. Children who require more than 60 minutes to be fed a typical meal and who are fed four meals or more per day are rarely adequately nourished. Calorie counts determined by food diaries confirm this. A history of choking during meals, especially when drinking liquids, is suggestive of aspiration.

In children with cerebral palsy, the usual findings associated with neurologic dysphagia are hypotonic lips, poor lingual function, delayed swallow reflex, and poor esophageal peristalsis. These neurologic abnormalities
result in slow oral transit time and slow pharyngeal transit time (9). A gag reflex that is both difficult to elicit and has little palatal movement is associated with neurologic dysphagia and aspiration. Likewise, an extremely active gag reflex with repeated retching is also often associated with dysphagia and aspiration. Coughing during the clinical assessment, especially when drinking liquids, suggests aspiration. Neurologic dysphagia is often associated with gastroesophageal reflux.


Aspiration

The fundamental cause of aspiration occurring in children with cerebral palsy is inability to protect the airway. This is due to either glossopharyngeal dysfunction, immobility resulting in inability to roll from supine to side after vomiting, or to both. Aspiration in children with cerebral palsy is common, 27% in one series (10). Hypoxemia during feeding is a useful indicator of significant aspiration (11). Patients who are immobile and who are unable to roll to a side from the supine position are at high risk for aspiration, regardless of whether they have neurologic dysphagia. Pulmonary problems caused by aspiration are the same in children with cerebral palsy as in the general population (chronic asthma, bronchitis, recurrent pneumonia, atolectasis, bronchiectasis, and hoarseness, stridor, and apnea) (12). Aspiration can be documented by video fluoroscopic swallow study. “Silent aspiration” will eventually cause clinical problems in most patients in our experience and should not be considered to be benign.


Gastroesophageal Reflux

Neurologically impaired children have a high prevalence of gastroesophageal reflux (GER), compared with typical children (13). The etiology of GER in this population may be due to brainstem dysfunction. Electrogastrophy demonstrated abnormalities in gastric contraction in these patients much more frequently than in neurologically typical patients with GER (14). Other factors that may contribute to the high prevalence of reflux include increased intraabdominal pressure due to abdominal muscle spasticity, constipation, and/or scoliosis.

It has been established that medical management of significant GER in children with cerebral palsy is of little or no long-term value (15,16). Neither vomiting nor aspiration of refluxed gastric contents is prevented. Even cisapride, no longer available, which was effective for the treatment of GER in typical children, was found not to work well in treating children with cerebral palsy (16). H2 antagonists and protein pump inhibitors alkalinize gastric acid, but alkaline GER still occurs and can cause esophagitis (17).

Persistent GER causes erosive esophagitis, which can result in pain, food refusal, hematemesis, esophageal stricture, and even Barrett’s esophageal dysplasia in children (18).

Table 18-2 presents a compilation of five reports addressing the issue of the development of GER-related symptoms subsequent to gastrostomy (19,20,21,22,23). Of 126 mentally retarded children with no reflux symptoms preoperatively who had a gastrostomy alone, 33 (25%) eventually required a fundoplication for symptoms. A larger number developed GER detected by pH probe study. Two children (2%) died from complications of reflux before a fundoplication was done. GER, or even vomiting in a child who aspirates, carries the risks of aspiration pneumonia and death if a child has had a gastrostomy without an accompanying fundoplication. Parents should be aware of this when they are deciding between a gastrostomy with or without a fundoplication.








TABLE 18-2 Outcome of Mentally Impaired Patients with No Gastroesophageal Reflux Who Underwent Gastrostomy without Fundoplication.















































Series Patients Patients Who Eventually Had Fundoplication Patients Who Died from Reflux Symptoms Before Fundoplication
Jolley et al. 1985a 9 3 (33%) 1 (1%)
Mollitt et al. 1985b 16 4 (25%) 0
Langer et al. 1988c 50 17 (34%) 1 (1%)
Grunow et al. 1989d 10 3 (30%) 0
Wheatley et al. 1991e 43 6 (14%) 0
Total 126 33 (25%) 2 (2%)
aFrom Jolley SG, Smith EI, Tunell WP. Protective antireflux operation with feeding gastrostomy: experience with children. Ann Surg 1985;201:736.
bFrom Mollitt DL, Golladay S, Seibert JJ. Symptomatic gastroesophageal reflux following gastrostomy in neurologically impaired patients. Pediatrics 1985;75:1124.
cFrom Langer JC, Wesson DE, Ein SH, et al. Feeding gastrostomy in neurologically impaired children: is an antireflux procedure necessary? J Pediatr Gastroenterol Nutr 1988;7:837.
dFrom Grunow JE, Al-Hafidh AS, Tunell WP. Gastroesophageal reflux following percutaneous endoscopic gastrostomy in children. J Pediatr Surg 1989;24:42.
eFrom Wheatley MJ, Coran AG, Wesley JR, et al. Redo fundoplication in infants and children with recurrent gastroesophageal reflux. J Pediatr Surg 1991;26:758.



Delayed Gastric Emptying

Delayed gastric emptying, defined as greater than 50% gastric retention of an isotope at 90 minutes (24), was found in 75% of neurologically impaired children who required a fundoplication, as compared with 25% of normal children undergoing fundoplication. Better surgical results after pyloroplasty accompanying fundoplication for patients with cerebral palsy and delayed gastric emptying have been reported by some authors (24,25), but not by others (26). Decreased retching and “gas-bloat” syndrome are reported if a pyloroplasty is done with a fundoplication in patients with neurologic impairment and preoperative delayed gastric emptying. Patients with gastric atony (gastroparesis) are unlikely to do well after gastrostomy and fundoplication, and should be considered for a surgically created jejunostomy instead.


Indications for Enteral Feedings

Indications for gastrostomy in malnourished children with cerebral palsy are controversial. There is general agreement among physicians who care for these children that both aspiration and undernutrition associated with any adverse health consequence should be treated by gastrostomy feedings. There is also agreement that progressively worsening nutritional status in an already undernourished child is an indication for gastrostomy feedings. Feeding problems that are present early in life in children with cerebral palsy tend to persist (27). Feeding efficiency will not improve in these children in proportion to their increasing need for calories as they grow; therefore, undernourishment will only worsen with time. Finally, aspiration of oral feedings even in the absence of pulmonary disease can be an indication for enteral feedings because it places a patient at great risk. It should be recognized that percutaneous endoscopic gastrostomy (PEG), although often a “day procedure” done by a gastroenterologist, is not minor surgery and major complications are common, with 25% requiring a second operation (28). Pediatric surgery participation with gastroenterologists who perform the procedure has been recommended (28).


Indications for Fundoplication in Children with Cerebral Palsy

Table 18-3 presents indications for fundoplication with gastrostomy in children with cerebral palsy. The table reflects the opinions of the authors, and there is controversy about each indication. The principles on which our recommendations are based are:



  • Inability of a child with cerebral palsy to protect the airway is the most important factor in deciding who needs a fundoplication with a gastrostomy. Even intermittent GER or rare vomiting in a child with inability to protect the airway can result in aspiration pneumonia and death. Children who are completely immobile are at great risk for aspiration when they vomit, even without aspiration or GER, because they too cannot protect their airway.








    TABLE 18-3 Indications for Fundoplication with Gastrostomy in Neurologically Impaired Children.




    Inability to protect the airway (independent of gastroesophageal reflux):
       History of aspiration pneumonia after vomiting
       History of recurrent pneumonia
       History of recurrent “bronchitis”
       History of recurrent active airway disease
    A   spiration of oral feedings demonstrated by videofluoroscopic swallowing study, including “silent aspiration”
       Immobility associated only with profound or severe retardation, preventing the child from rolling from supine to side if vomiting occurs and from calling for help
    Gastroesophageal reflux in patients who can protect their airway:
       Symptomatic esophagitis (hematemesis, stricture, heartburn)
       Symptomatic reflux on maximum medical therapy (vomiting, regurgitation, pain)
       Persistent asymptomatic gastroesophageal reflux on maximum medical therapy (“full column” reflux on radiological evaluation; frequent episodes during a 2-hr period after radioisotope study; or more than 10% duration of a 24-hr pH probe study with pH ≤ 4.0)
       Vomiting during a trial of nasogastric tube feedings


  • Patients with cerebral palsy older than 3 years of age who have no symptoms of GER or of aspiration and who can protect their airway, but who have an abnormal pH probe study (pH ≤ 4.0 for 10% of a 24-hour study), should also have a fundoplication because it is unlikely that the GER will resolve (29).


  • Patients who develop symptomatic GER after percutaneous endoscopic gastrostomy are also surgical candidates for a fundoplication because the alternative is lifelong medical management and endoscopic surveillance (30).


  • Patients who need a gastrostomy for treatment of under nutrition, but who have no preoperative evidence for gastroesophageal reflux and no aspiration, may do well without a fundoplication. In patients who have no risk factors for aspiration, a trial of tube feedings can be helpful in selecting those who will tolerate bolus feedings without vomiting. Patients who aspirate and who have had a gastrostomy alone followed by development of GER, gastrojejunal feedings, which are advocated by some as an alternative to fundoplication, are associated with an unacceptably high rate of complications and are not preferable to doing an antireflux procedure (31,32).


  • Neurologically impaired patients with preoperative retching and vomiting may have abnormal activation
    of the emetic reflex rather than GER (33). Post operative retching occurs after a fundoplication in 75% of such patients. Most physicians consider this to be a relative, not absolute, contraindication to antireflux surgery only, however.


Evaluation of Undernutrition, Reflux, and Aspiration in Children with Cerebral Palsy

Table 18-4 presents a plan for evaluating children with cerebral palsy or other neurologic impairment and undernutrition, GER, and/or aspiration.

Videofluoroscopic investigation is helpful in both defining problems more precisely and in developing therapeutic approaches to increasing caloric consumption safely. Under fluoroscopic observation, children are fed four textures of food (thin liquids, thickened liquids, pureed foods, and chopped foods), all mixed with barium. Phases of swallowing are observed and abnormalities noted. The therapist assisting the radiologist tries to determine optimal positioning for safe swallowing for feeding the child and whether the temperature of food affects swallowing ability. Using this information, a feeding program is devised for parents to follow at home. If weight gain is not achieved in a reasonable amount of time, then children should be considered candidates for enteral feeding. Parents accept the need for enteral feedings better if they have tried to improve the situation at home, under guidance, first.

An upper gastrointestinal (GI) series should be obtained along with the videofluoroscopic swallowing study. It is important to ensure surgical candidates do not have an esophageal stricture, hiatal hernia, malrotation, or other GI anomaly that would affect surgery.








TABLE 18-4 Suggested Evaluation of Neurologically Impaired Children with Failure-to-Thrive.




Observation by therapists of a parent feeding the child:
   Time required for the feeding encounter
   Severity of neurologic dysphagia
   Pulmonary symptoms during feeding
   Pulse oximetry desaturations during feeding
Videofluoroscopic swallowing study, to assess for aspiration (necessary preoperatively)
Upper gastrointestinal series to assess malrotation (necessary preoperatively)
Radioisotope liquid-phase gastric emptying study with esophageal views over 2 hrs to determine gastric emptying time and frequency and severity of reflux during the study (necessary preoperatively)
pH probe study (selected patients)
Esophagoscopy (selected patients)
Trial of nasogastric tube feedings to assess for reflux symptoms (necessary before doing a PEG alone in patients who can protect their airway and who have no evidence of reflux clinically or radiologically)

A liquid-phase radionucleide gastric emptying study with additional esophageal views is useful to both measure gastric emptying time and to assess for GER. If significant GER is found, then it is likely, in our experience, that a 24-hour pH probe monitoring study will also reveal significant reflux (pH ≤ 4 for more than 10% of the 24-hour study).

Although a 24-hour pH probe study is considered to be the “gold standard” for diagnosis of GER, Heine, et al. found that the sensitivity of an abnormal pH study as a predictor of esophagitis was only 38.5% and the specificity was only 71.4% (34). Esophagoscopy can be useful in evaluating children for erosive esophagitis and its consequences. For patients with long-standing symptoms of esophagitis, esophageal biopsy for Barrett’s esophageal dysplasia should be done. This can be accomplished just prior to the fundoplication, under the same general anesthesia.


Complications of Gastrostomy and Fundoplication

The complication rate of gastrostomy and fundoplication in children with cerebral palsy is higher than in typical children (35,36). Preoperative pulmonary disease is associated with wrap disruption. Table 18-5 presents early and late
complications in approximate order of occurrence, complied from several case series. Several complications deserve specific comment. Retching can be caused by either delayed or overly rapid gastric emptying (37). A radionuclide gastric emptying study can be helpful in diagnosis. Delayed gastric emptying after surgery is often a transient problem. Continuous infusion of formula and treatment with metoclopramide can improve feeding tolerance until gastric emptying increases. Some children with normal gastric emptying preoperatively, however, develop delayed gastric emptying after gastrostomy and fundoplication and require a subsequent pyloroplasty. The dumping syndrome can occur after fundoplication. Patients with the dumping syndrome have retching associated with diarrhea and symptoms of hypoglycemia (pallor, sweating, and tachycardia). Their symptoms can often be controlled satisfactorily by feeding with an elemental formula by continuous infusion. Adding cornstarch to their formula is often useful. A “button” feeding device for a gastrostomy eliminates the complication of gastrostomy tube migration into the duodenum and associated small bowel obstruction.

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Aug 25, 2016 | Posted by in PEDIATRICS | Comments Off on Surgical Problems of Children with Physical Disabilities

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