✓Vertebral hemangiomas are common entities that rarely present with neurological deficits. The authors report the unusual case of a large L-3 vertebral hemangioma with epidural extension in a 27-year-old woman who presented with hip flexor and quadriceps weakness, foot drop, and leg pain. The characteristics of the mass on magnetic resonance imaging suggested an aggressive, hypervascular lesion. The patient underwent embolization of the lesion followed by direct intralesional injection of ethanol. Significant resolution of clinical symptoms was observed immediately after the procedure and at her follow-up visits. Follow-up imaging studies obtained 9 months after the procedure also documented a considerable reduction in the size of the hemangioma with minimal loss of vertebral height and a mild kyphosis at the affected level. On repeated imaging studies obtained 21 months postoperatively, the size of the hemangioma and the degree of vertebral body compression were stable. As demonstrated in this case, patients with vertebral hemangiomas can present with acute nerve root compression and signs and symptoms similar to those of disc herniation. Vertebral hemangiomas can be treated effectively with interventional techniques such as embolization and ethanol injection.
H. Isaac Chen, Gregory G. Heuer, Kareem Zaghloul, Scott L. Simon, John B. Weigele and M. Sean Grady
Gregory G. Heuer, Kareem A. Zaghloul, Richard Roberts, Michael F. Stiefel and Phillip B. Storm
✓ Coil migration is a rare but potentially serious complication of endovascular procedures. Occasionally coils can be retrieved via endovascular techniques. The authors describe the microsurgical management of a case in which endovascular techniques failed. A 2-year-old girl with pulmonary atresia and a Blalock–Taussig shunt underwent attempted endovascular closure of the shunt with Gianturco steel coils. During deployment, a coil was lost in the aorta and an angiogram showed that it had lodged in the proximal M1 segment of the middle cerebral artery. The coil could not be retrieved by endovascular techniques, and the patient was taken to the operating room to undergo a craniotomy. After the sylvian fissure was split, the coil was visible through the vessel wall. Temporary clips were placed on the proximal M1 and the proximal M2 segments, trapping the coil. A small arteriotomy was performed, the coil was removed, and the arteriotomy was closed. A cerebral angiogram showed excellent perfusion with no dissections. The patient’s motor examination demonstrated a mild hemiparesis on the left with no tremulousness. Coil migration can be treated by microsurgical techniques in pediatric patients with a good clinical outcome.
Kareem A. Zaghloul, Gregory G. Heuer, Marta D. Guttenberg, Eileen M. Shore, Frederick S. Kaplan and Phillip B. Storm
✓ Fibrodysplasia ossificans progressiva (FOP) is a rare, autosomal dominant disorder characterized by congenital malformation of the great toes and episodes of soft tissue swelling that lead to progressive heterotopic ossification. The genetic cause of FOP was recently discovered to be a recurrent missense activating mutation in the activin A type I receptor, a bone morphogenetic protein type I receptor in all classically affected individuals worldwide. The authors present a child with the classic features of previously undiagnosed FOP who developed a paraspinal soft-tissue mass after a lumbar puncture for a fever workup. Excision of the mass resulted in a massive inflammatory response leading to progression of heterotopic ossification. Awareness of the classic clinical features of FOP prior to the appearance of heterotopic ossification can prompt early clinical diagnosis and confirmation through genetic testing, thus avoiding interventions that lead to irreversible iatrogenic harm.
Gregory G. Heuer, Douglas A. Hardesty, Kareem A. Zaghloul, Erin M. Simon Schwartz, A. Reghan Foley and Phillip B. Storm
Schizencephaly is a rare congenital cortical brain malformation defined by unilateral or bilateral clefts of the cerebral hemispheres. These malformations are often associated with medically intractable epilepsy. Surgical solutions include lesionectomy, lobectomy, or hemispherectomy. The authors describe the case of an anatomic hemispherectomy for medically intractable epilepsy in an 8-year-old boy with a large schizencephalic cleft. Seven years prior to his epilepsy surgery, the patient underwent placement of a ventriculoperitoneal shunt for communicating hydrocephalus that resulted in severe left-to-right shift. Subsequently, medically refractory epilepsy developed and the patient underwent an anatomic hemispherectomy for seizure control. The preoperative brain shift remained after the surgery, although the patient tolerated the procedure well and was seizure free postoperatively. Anatomic hemispherectomy is a viable option for treating medically intractable epilepsy in a schizencephalic pediatric patient—even one with considerable brain shift.
Raymund L. Yong, Tianxia Wu, Nino Mihatov, Michael J. Shen, M. Anthony Brown, Kareem A. Zaghloul, Grace E. Park and John K. Park
Maximal safe tumor resection is part of the standard of care for patients with newly diagnosed glioblastoma. The role of reoperation in the care of patients with recurrent glioblastoma is less clear, and less than a quarter of patients undergo a second surgery. Previous studies have identified preoperative variables associated with the improved survival of patients following reoperation, and guidelines for the selection of patients for reoperation have been devised and validated. In this study, the authors analyzed the relative survival benefit of maximal safe tumor removal in a series of patients with recurrent glioblastoma who all underwent reoperation.
In this longitudinal study, the clinical and radiological data of 97 consecutive patients who underwent reoperation for recurrent glioblastoma were prospectively collected. Multiple regression analyses and Kaplan-Meier plotting were performed to identify pre- and postoperative clinical and radiological variables associated with increased survival following reoperation.
The median postoperative survival of all patients following reoperation was 12.4 months (95% confidence interval [CI] 9.0–15.6 months). Multiple Cox regression analysis revealed that patients with large (> 3 cm3) residual tumors following reoperation had significantly decreased survival relative to those with residual tumors that were small (> 0–3 cm3; hazard ratio [HR] = 3.10, 95% CI 1.69–5.70; p < 0.001) or radiologically absent (0 cm3; HR = 5.82, 95% CI 2.98–11.37; p < 0.001). Large residual tumors had faster rates of subsequent regrowth than small (odds ratio [OR] = 4.22, 95% CI 1.19–14.97; p = 0.026) or radiologically absent (OR = 11.00, 95% CI 2.79–43.43; p = 0.001) residual tumors, and a faster regrowth rate was significantly associated with decreased survival (HR = 4.01, 95% CI 2.26–7.14; p < 0.001).
The overall survival of patients with recurrent glioblastoma who underwent reoperations increased with decreasing postoperative residual tumor volumes. For patients meeting prognostic criteria for reoperation, the surgical goal should be to minimize residual tumor volume to maximize overall survival. Clinical trial registration no.: NCT00060541 (ClinicalTrials.gov).