A-068 Case Study: Neurodevelopment of Late Identification and Treatment of Nonsyndromic Multisutural Craniosynostosis

Abstract

Abstract Objective Craniosynostosis, the premature closure of cranial sutures, is frequently associated with intracranial hypertension, neurocognitive deficits, and neuromotor deficits. Younger age at treatment is associated with improved outcomes. This case study will expand upon the existing literature by characterizing the neurodevelopment of an individual whose nonsyndromic multisutural craniosynostosis was identified and treated late, when the patient was nearly three years old. Method A 7-year, 1-month old male with a history of bicoronal and sagittal craniosynostosis was seen for neuropsychological assessment. Diagnosis and cranial vault reconstruction occurred just before his third birthday. Evidence of increased intracranial pressure was found on presurgical CT scan, and by the appearance of his dura at surgery. His development after reconstruction was notable for articulation disorder, expressive language disorder, executive dysfunction, and adaptive skills deficits. Results Assessment revealed average non-verbal cognitive skills and low average verbal cognitive skills. Receptive language was below average. Exceptionally low scores were found on tasks of oromotor sequencing, verbal list learning, and immediate visual memory. Teacher ratings of executive functioning suggest below average skills. Measures of attention and impulsivity suggest mild weaknesses. Academically, he earned below average and exceptionally low scores on measures of reading and writing, with low average to average mathematics scores. Conclusions The neurodevelopment of children with late diagnosis/treatment of craniosynostosis is not well characterized and may confer increased risks. Late diagnosis is presumed to lead to more significant neurocognitive impairments due to the impact of prolonged intracranial pressure. Neuropsychological assessment and follow-up is critical for monitoring and intervention planning.