Practice parameter: Evaluation of the child with global developmental delay [RETIRED]
Report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society

The evaluation of a child with developmental delay is complex. There are, and should be, many different competing concerns in planning this evaluation. We find the recently published practice parameters for developmental delay a laudable exercise in evidence-based medicine, but find the recommendations insufficiently deferential to these concerns. [1]

There are many complexities requiring consideration:

1) The value of diagnosis is highly variable. Some diagnoses will initiate a successful therapy or prevent the initiation of inappropriate therapy, some will permit informed genetic counseling, and some will provide the solace of explanation to distressed family members. The value of a potential diagnosis must be balanced against the burdens of obtaining that diagnosis.

2) The certainty of specific diagnoses varies, and come findings (e.g. sulcal widening on MRI or diffuse slowing on EEG) have descriptive, but little etiologic, significance.

3) The costs associated with various tests, both in financial terms and the potential for untoward complications of studies under sedation or general anesthesia, must be part of the equation.

4) The population to be evaluated is poorly defined. Those with deficits in the development of motor ability, speech and language, cognitive, personal and social skills, or activities of daily living, either in isolation or in various combinations with one another, will have very different diagnostic yields on individual tests.

5) Local variation in the quality, cost, and logistic difficulties encountered in obtaining various tests are not accounted for, nor are variations in the level of expertise required to initiate a work up. The history, physical examination and opinion of subspecialists in neurology, neurodevelopmental disabilities, and genetics, as opposed to those of primary care providers, will likely introduce differences in the pretest probability of a positive result and alter the predictive value of individual diagnostic tests.

Practice parameters, as opposed to practice guidelines, have the potential for generating legal liability. They set a single standard against which the performance of pediatricians and pediatric subspecialists will be measured. We believe that the diagnostic evaluation of the child manifesting signs of abnormal development is sufficiently multidimensional that the available data, collected on widely varying populations by investigators with widely varying resources and expertise, do not permit establishment of such a standard. Nonetheless, we welcome and value the committee's considerable efforts as a first approximation for a proper diagnostic assessment.

Reference:

1) Shevell M, Ashwal, Donley D, et al. Practice Parameter: Evaluation of the child with global developmental delay. Report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology 2003;60:367- 380.

Practice Parameters are guidelines that through systematic review of the literature address the goal of achieving best practice. In the evaluation of the child with global developmental delay (GDD), developmental performance is targeted in follow-up programs addressing children at increased biological or social risk. Developmental concerns prompt early intervention programs and referral for further evaluation. [1, 2] The definition in the parameter [3] distinguishes the evaluation approach taken for this clinical problem from that which would be taken for a child with a delay restricted to a single domain (i.e. motor delay, specific language impairment). Cognitive delay invariably includes language and activity of daily living skills, and thus is included within our definition. The distinction between GDD and autistic spectrum disorders should not be troublesome as the latter requires observation of specific abnormalities in language and social skills beyond mere quantitative delay.

We appreciate that Crawford et al. consider the GDD practice parameter a "laudable exercise" the product of "considerable efforts" to yield a framework "for a proper diagnostic assessment". With regards to their specific points, the value of a diagnosis is indeed highly variable depending on the results obtained and its implications for child and family. However it cannot be known a priori before undertaking a search for causality. Perhaps, potential value can be judged by the overwhelming willingness of families to undergo the studies requested. Careful reading of the parameter reveals that the practitioner must consider and balance potential risks of various studies against their potential yield. Similarly, we acknowledge in the document that local limitations in access may modify the actual selection of tests. Additionally we emphasize the importance of the history and physical examination in the evaluation of the child with GDD. Clearly, one size or a single standard as suggested by Crawford et al. would not be suitable for all children. By emphasizing the importance of the history and physical examination throughout the document, we hope to capture the multidimensional nature of a diagnostic evaluation while at the same time providing a rational basis for selected testing.

Practice parameters involve multiple reviewers with many perspectives. All comments were carefully considered. Dr. Whelan was the sole reviewer during this lengthy process to raise concerns regarding our working definition. The editorial by Franklin and Zahn [4] cited by Dr. Whelan does not endorse her request for publication of a single 'dissenting opinion'. Dr. Whelan's comments regarding the process of how this practice parameter was developed and approved are inaccurate. The first draft of this document was prepared on August 12, 1999 and underwent multiple revisions before the Quality Standards Subcommittee (QSS) of the American Academy of Neurology (AAN) (August 11, 2001) first reviewed it. It was revised and then reviewed by members of the Child Neurology Society (CNS) Practice Committee and the AAN Reviewers Network. It was also reviewed by selected members of other organizations including the American Association of Mental Retardation, three sections from the American Academy of Pediatrics including the Section on Children with Disabilities, Section on Neurology and Section on Developmental and Behavioral Pediatrics as well as the Committee on Quality Improvement. An additional section with consensus-based recommendations for selective screening and an algorithm were also added. The document was again reviewed and approved by QSS on April 16, 2002. It then underwent further peer review by four individuals from Neurology and again by members of the CNS Practice Committee. This committee (June 30, 2002) voted to approve the document by email. Of 21 members who received the document, 14 responded-- 11 approved the document, two made comments, and one (Dr. Whelan) disapproved. The final draft was accepted by Neurology for publication on July 16, 2002 approved by QSS on August 1, 2002 and by the AAN Practice Committee on August 3, 2002. The Executive Boards of both organizations (CNS, August 15, 2002; AAN, October 19, 2002) then gave final approval. Overall nearly 100 individuals from multiple disciplines reviewed drafts of the document during this three-year period. All comments were addressed by a specific QSS developed process that explicitly documented the reviewer's concerns and how the authors addressed each issue. We feel that this process as it has done with previously published evidenced-based practice parameters sponsored by the AAN, results in documents of value to physicians and their patients.

References

1. Committee to Advise the Public Health Service on Clinical Practice Guidelines, Institute of Medicine. Field MJ, Lohr KN, eds. Clinical practice guidelines: directions of a new program. Washington, DC: National Academy Press, 1990.

2. American Academy of Pediatrics Committee on Children with Disabilities. Developmental surveillance and screening in infants and young children. Pediatrics 2001;108:192-196.

3. Shevell M, Ashwal S, Donley D, et al. Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology. 2003;60:367-80.

4. Franklin GM, Zahn CA. AAN clinical practice guidelines: Above the fray. Neurology 2002;59:975-976.

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The recently published Practice Parameter concerning the evaluation of the child with global developmental delay is unfortunately flawed by the authors' decision to reinterpret this perfectly clear term, which has always been understood to encompass cognitive delay, in such a fashion that a child with delays in only two of five domains would be thus classified [1]. Thus a child with cerebral palsy and consequent impairment in motor and ADL skills, but without cognitive, social, or language impairment, would meet the authors' definition. This stands in contradiction to the literature which they cite (authors' references 3-7) in support of their definition. It further contradicts the authors' statement elsewhere that global delay is to be distinguished from autistic spectrum disorder: on the basis of their definition, such a distinction would not be possible. It is also troubling to see an unmodified recommendation for routine MRI studies in the abstract: this was appropriately modified in the text. As presented, this parameter would appear to have been approved by the Practice Committee of the Child Neurology Society as well as the AAN QSS. As a member of the CNS Practice Committee, I would like to raise some procedural concerns. When polled at the last of the two Practice Committee meetings where this parameter was (briefly) presented, only one member expressed approval of the definition. However, abstaining votes from absent members were counted as approving (abstention from commentary has usually meant failure to read, an acknowledged problem in this committee) and request for simultaneous publication of a dissenting opinion was denied by the Co- Chair of the QSS, who has implicitly endorsed such a practice elsewhere [2,3]. Space does not permit further commentary, but both this parameter, and the process, which led to its apparent endorsement, is in need of revision.

References:

1. Shevell M, Ashwal S, Donley, D et al. Practice parameter: Evaluation of the child with global developmental delay. Neurology 2003;60:367-380.

2. Franklin GM, Zahn CA. AAN clinical practice guidelines: Above the fray. Neurology 2002;59:975-976.

3. Hart RG, Bailey RD. As assessment of guidelines for prevention of ischemic stroke. Neurology 2002;59:977-982.