Whether cranial expansion is indicated in selected cases with microcephaly?

PURPOSE: Some children with microcephaly experienced severe social emotional deficits during their 1-2 years of age. Cranial expansion is generally not used in these cases. Our aim is to assess whether such operation is effective to improve their social emotional status, and thereby boosting their functional development in selected cases with such condition.

METHODS

A retrospective cohort review in microcephalic cases who had undergone cranial expansion in Shanghai Children's Hospital since Jun. 2016 to Jun. 2017 with at least 12 months follow-up was conducted. Inclusion and exclusion criteria were set for the selection of the target patients in the current study. Our study focused on the changes of social emotional status (evaluated by The Infant-Toddler Social and Emotional Assessment, ITSEA) and functional development (applying neurodevelopmental questionnaire) pre- and 12 months post-op in these cases.

RESULTS

A total of 14 cases were included in the current study. On the basis of a 2-cm enlargement during surgery(1 cm advancement of fronto-orbital rim on each side), HC continued to increase at an average of 0.5 cm in one year after the procedure in these cases, making its corresponding standard deviation improve from -4.5 to -3.4. Among those 14 cases, 11 (78.6%) showed improvement in all of these four domains in ITSEA at 12 months post-op. Scores were significantly better at 12 months post-op with p < 0.01 compared with those in domains of internalizing behavior and dysregulation behavior pre-operatively. Improvement was observed with p < 0.05 in domains of externalizing behavior and social-emotional competence as well. With regard to functional development, 8 cases (57.1%) were revealed improvement in all of these three sections of motor, speech and cognitive function in the questionnaire, 5 (35.7%) concluded partially upgraded in 1 or 2 sections. Only one (7.1%) showed no change during the follow-up. No surgery-related complications were recorded in the current study.

CONCLUSION

When progressive copper-beaten sign is revealed in their serial skull X-rays, microcephalic cases with social emotional deficits in their 1-2 years of age could benefit from cranial expansion surgery with regard to improving their social emotional status and functional development.