Tube neural defects and craniosynostosis. Would this association be only a coincidence?

Introduction: The association between craniosynostosis and neural tube defects (NTD) has been seen typically as just a coincidence. There are only six articles reporting this association with a total of twelve patients in which no intervention for NTD correction was performed before birth. The main factor that stimulates cranial growth is brain development. They hypothesized that untreated NTD could induce the premature closure of some cranial sutures due to decreased intracranial pressure caused by egress of cerebrospinal fluid and neural tissue inside the defect that result in a deficient cerebral impulse for cranial growth and may stimulate premature sutural fusion. The association between NTD and craniosynostoses may be due to intrauterine constriction of the fetal head. In fact, this could be an added causative factor, as patients with NTD can have diminished intrauterine movements, which in turn could help to maintain the head in a fixed position against the pelvic bones. In the case described below the patient was submitted to an intrauterine myelomeningocele correction but nevertheless the premature closure of unilateral coronal suture occurred.

Objective: Describe a case of craniosynostosis associated to neural tube defects that was submitted to a prenatal myelomeningocele correction.

Method: Case report and literature review performed in PubMed. Results: Female, 1Y/O with history of myelomeningocele diagnosed on prenatal ultrasound, submitted to a prenatal correction of myelomeningocele at the twenty-fourth week of gestational age and premature birth at twenty-sixth week of gestational age. At eigth months-old, her mother noticed disproportionate head growth with the right forehead being larger. In the physical examination was evidenced a slight delay of neuropsychomotor development without another neurological deficit. A head CT scan evidenced closure of left coronal suture. At eleven months-old, she was submitted to a fronto-orbital advancement for craniosynostosis correction. She was discharged in the fourth PO day.

Conclusion: Literature data suggest that the association between craniosynostosis and neural tube defects has a mechanical relationship. In the described case, the association may be explained by the theory of “pelvic constriction”. We believe that the association is not a coincidence so that other pathophysiological mechanisms that explain this association should be investigated as well.

No conflict of interest has been declared by the author(s).