Intramedullary cavernoma presenting with hematomyelia: report of two girls

Abstract

Introduction: Less than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit.

Case material: A 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8-T11), intramedullary edema (T6-L1), and an ImC at T9-T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4-C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma.

Conclusion: With adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.

Fig. 1

a1–a3 Preoperative sagittal T1, T2, and T2 FFE (fast field echo) MR images revealing a markedly swollen cord, including intramedullary edema (T6–L1), hematomyelia (T8–T11), and an ImC at T9–T10; b1–b3 3 months postoperative sagittal T1, T2, and axial T2 MR images confirming complete resection; c1–c3 2 years postoperative sagittal T1, T2, and axial T2 MR images demonstrating a thin spinal cord without evidence of local recurrence; d1, d2 2 years postoperative anteroposterior and lateral spinal X-rays to monitor kyphoscoliosis

Fig. 2

a1–a3 Preoperative sagittal T1, T2, and axial T2 MR images revealing a markedly swollen cord, including hematomyelia at C4–C6 and a left anterolateral ImC at C5; b1, b2 3 months postoperative sagittal T2 and axial T2 FFE (fast field echo) MR images, note multiple small cerebral cavernomas; c1, c2 2.5 years postoperative sagittal and axial T2 MR images, the cord still appears swollen; d1, d2 3.5 years postoperative sagittal and axial T2 MR images, the cord now appears less swollen and the child is asymptomatic

Fig. 3

a Macroscopic intraoperative image at C4–C5 (left dorsolateral view, one denticulate ligament sectioned) showing subtle subpial yellow-blue discoloration at the lateral surface of the spinal cord. b Ultrasonic intraoperative image at C3–C6 (sagittal view) specifying the exact location of the cavernoma in the swollen cord segment