2021 Volume 30 Issue 7 Pages 539-544
Craniometaphyseal dysplasia is a hereditary osteosclerotic disease characterized by hyperostosis of the skull and enlargement of the metaphysis of the long canal bone. By 2017, 105 cases had been documented, but cases associated with Chiari malformation type Ⅰ are extremely rare. We report a case of Chiari malformation type Ⅰ complicated with craniometaphyseal dysplasia.
A 15-year-old boy with craniometaphyseal dysplasia and progressive scoliosis was referred to our department because of tonsillar herniation and syringomyelia. Thermal hypoalgesia in the extremities was the only neurological finding of this lesion. Foramen magnum decompression was performed via suboccipital craniotomy and C1 laminectomy without duroplasty. The postoperative course was complicated with epidural hematoma, which required reoperation within 3 days. Follow-up MRI showed good decompression at the craniocervical junction and a significant decrease in the size of the syrinx.
Foramen magnum decompression is an effective treatment for Chiari malformation type Ⅰ complicated with craniometaphyseal dysplasia. However, patients should be treated with strict management because the perioperative course is likely to be associated with a higher incidence of complications because of time-consuming procedures, large postoperative dead space, and craniofacial deformities.
