J Korean Neurosurg Soc.  2014 Sep;56(3):237-242. 10.3340/jkns.2014.56.3.237.

Microsurgical Treatment and Outcome of Pediatric Supratentorial Cerebral Cavernous Malformation

• Noh JH
• Cho KR
• Yeon JY
• Seol HJ
• Shin HJ

• KMID: 2339963
• DOI: http://doi.org/10.3340/jkns.2014.56.3.237

Abstract

OBJECTIVE
The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system.
METHODS
Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed.
RESULTS
The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness.
CONCLUSION
With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary.

Keyword

Cavernous malformation; Hemosiderin deposit; Neuronavigation; Transparent tubular retractor

MeSH Terms

Central Nervous System
Child
Demography
Headache
Hemangioma, Cavernous, Central Nervous System*
Hemorrhage
Humans
Incidental Findings
Mortality
Neuronavigation
Seizures

Figure

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  Fig. 1 Thin section MRI was taken preoperatively and registered to the intraoperative neuronavigation system. With help of neuronavigation system, planning of the optimal trajectory to the target lesion could be easily achieved and margins of the lesion were assumed during surgery.

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  Fig. 2 Preoperative MRI of 15-year-old boy showed multiple dark signal structures including right parieto-occipital area (A). Transparent tubular retraction system (TTRS) was inserted via minimal corticotomy. Without wide cortical incision and excessive parenchymal retraction, competent surgical view and flexible working space through TTRS was achieved (B). MRI performed at 3 month after surgery did not show a residual lesion, but dark signal intensity was observed in T2WI, which indicates that the hemosiderin-stained area was left behind (C).

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  Fig. 3 Preoperative MRI of 17-year-old girl showed popcorn-like structure in the right precentral gyrus (A). One year after resection of the lesion in the precentral gyrus, there was no residual cavernous malformation, but there was hemosiderin deposition (B).

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