We report the case of a 12-year-old girl born with cerebral dysgenesis and congenital hydrocephalus first shunted shortly after birth. She had severe tissue-damaging self-injurious behavior, profound mental retardation, quadriparesis, as well as multiple cranial anomalies including turricephaly. After stage 1 cranial remodeling, a bone window was left pending second stage remodeling. Episodic changes in fluctuation of the scalp overlying the bone window were easily observed. During the course of a behavioral assessment for her self-injury, it was observed that the overall frequency of occurrence of self-injury increased significantly (P < .01) when the scalp was protruding and bulging compared with when the scalp was flush with the skull table. Periods of increased scalp protrusion were also associated with higher scores on a pain scale developed for children with communicative impairments associated with severe neurological impairment. After shunt replacement, there was remarkable improvement in functional status and decreased episodic self-injury.
- Functional analysis