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Haak C, Chin E, A-254 A Pediatric Case Study of Mild Intellectual Disability in the Context of Complex Chiari Malformation, Hydrocephalus, Cleft Palette, and Complex Psychosocial Environment, Archives of Clinical Neuropsychology, Volume 35, Issue 6, September 2020, Page 1049, https://doi.org/10.1093/arclin/acaa068.254
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Abstract
Chiari malformation is a congenital condition involving structural skull defects, causing brain tissue herniation and likely resulting in hydrocephalus. People with these medical conditions are at risk for varied cognitive impairments. Recently, emerging evidence has suggested the co-occurrence of Chiari malformation and cleft palette contributing to increased cognitive challenges, highlighting the importance of introducing interventions in a timely manner.
This case study presents on the neuropsychological profile of an 11-year-old girl with a medical history of complex Chiari malformation with accompanying hydrocephalus and syringomyelia, and lymphangioma born with cleft palette. Relevant social history includes recent parent separation and unstable home life. Presenting concerns included intermittent hand weakness and headaches status post 5 surgeries related to Chiari malformation and hydrocephalus, inattention/impulsivity, learning difficulties, and increased emotional and behavioral dysregulation.
Neuropsychological testing revealed impairments in intellectual functioning (WISC-V FSIQ = 64), adaptive skills, attention/executive functioning, language, visuomotor skills, memory, and academic skills. Significant emotional and behavior adjustment problems were indicated, secondary to medical treatment, resulting physical limitations, and psychosocial stressors. Finally, staring spells episodes were observed that were concerning for possible seizure activity.
Recommendations were given to aid in treatment planning for her referring neurosurgeon alongside clinical interventions (e.g., psychotherapy, social work, speech/language, and occupational therapy). Recommendations related to continued intensive special education services were also provided. This case study demonstrates the need for further understanding cognitive impairment in the context of Chiari malformation with co-occurring hydrocephalus and cleft palette. Multidisciplinary collaboration is essential to facilitate the prompt introduction of appropriate clinical interventions.
