External-internal cranial expansion to treat patients with craniocerebral disproportion due to post-shunt craniosynostosis: a case series

Introduction Secondary craniosynostosis subsequent to shunting is one of the late complications of ventricular shunt placement in the early childhood. Several interventions have been used to treat high intracranial pressure associated with this condition. This study aimed to evaluate the patients' clinical symptoms and head circumference before and after a method of decompressive craniotomy, coined as external-internal cranial expansion (EICE). Methods A retrospective study was conducted, and the patients who had undergone EICE for the treatment of post-shunt craniosynostosis between 2010 and 2020 were enrolled. This approach was a combination of a hinge multiple-strut decompressive craniectomy and internal cranial flap thinning by drill. Data, extracted from medical records, were used to evaluate the patients' symptoms and head circumferences before and 12 months after surgery. Results A total of 16 patients were enrolled in the study, of which eight were females. Before the surgery, 9 patients (56.2%) suffered from visual impairment, and all had intractable headache. Papilledema was recorded in all, with 3 cases having optic disc paleness. After cranial expansion, only two patients had headaches, diagnosed as migraine-type and psychosomatic headaches, respectively. In two patients, progressive visual impairments got worsening after surgery, which would be due to severe preoperative optic nerve atrophy. Patients' head circumferences significantly increased after the surgery (mean of 48.97 +/- 4.28 cm vs. 45.78 +/- 4.31 cm; P value < 0.0001). Conclusion In lower resource countries, where newer technologies like distraction osteogenesis is not easily available, external-internal cranial expansion can be considered an effective alternative for patients with post-shunt craniosynostosis.