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Sevgi Sarikaya-Seiwert, Kristin Gierga, Rüdiger Wessalowski, Hans-Jakob Steiger, and Daniel Hänggi

Spinal solitary epidural cavernous angiomas are rare benign vascular malformations, which occur even less frequently in children than in adults. It is uncommon to find such lesions without adjacent vertebral involvement. Occasionally, these lesions can lead to neurological symptoms through growth or due to intralesional hemorrhage. In this report the authors describe 2 children presenting with acute symptoms and neurological deficits caused by hemorrhage within solitary spinal epidural cavernous angiomas. A 13-year-old girl and a 9-year-old girl, previously healthy, were admitted to the authors' department due to acute radicular pain and neurological deficits. In both cases MR imaging revealed a solitary epidural mass with signs of bleeding and compression of the spinal cord. Complete resection of the lesion via a dorsal approach was performed in both patients. The histological examination of the lesions revealed the characteristic structures of a cavernous angioma with hemosiderin deposits and acute hemorrhage. Both patients recovered fully after surgical removal of the lesions. Review of the literature confirmed that spinal epidural cavernous angiomas are extremely rare in the pediatric patient population, described currently in only 2 instances, but without acute hemorrhage. These cases suggest that epidural cavernous angiomas also have to be considered in the pediatric patient population in the differential diagnosis of intraspinal lesions with acute or progressive neurological symptoms. Microsurgical resection of these cavernous malformations is an effective and curative treatment option.

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Sevgi Sarikaya-Seiwert, Bernd Turowski, Daniel Hänggi, Giesela Janssen, Hans-Jakob Steiger, and Walter Stummer

Pineal cysts are benign and often asymptomatic intracranial entities. Occasionally they can lead to neurological symptoms through growth or due to intracystic hemorrhage. The purpose of the current report is to describe their clinical characteristics and treatment options.

In the current study, the authors illustrate the course of disease in 3 patients who developed neurological symptoms due to hemorrhage into a pineal cyst. Two of their patients had additional cerebral disease, and regular MR imaging examinations were conducted. This circumstance allowed documentation of growth and intracystic hemorrhage. After the occurrence of new neurological symptoms with severe headache, MR images showed a fluid-fluid interface due to intracystic hemorrhage. The third patient presented with acute triventricular hydrocephalus and papilledema due to aqueductal stenosis caused by intracystic hemorrhage.

In all 3 cases, excision of the pineal cysts via an infratentorial/supracerebellar approach was performed. Histological examination revealed the characteristic structure of pineal cyst in all cases, with hemorrhagic residues in the form of hemosiderin deposits. All patients recovered fully after surgical removal of the cysts. Furthermore, resolution of occlusive hydrocephalus could be demonstrated in those cases with ventricular enlargement.

Pineal cysts without neurological symptoms are often discovered as incidental findings on cranial MR images. In contrast, neurological symptoms such as severe headache, diplopia, or Parinaud syndrome, may occur as a result of pineal apoplexy due to intracystic hemorrhage. The authors' cases confirm that MR imaging can identify intracystic hemorrhage by a characteristic fluid-fluid interface. Their experience suggests that microsurgical resection of cysts may be an effective and curative treatment option.

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Abdallah Salemdawod, Johannes Wach, Mohammed Banat, Valeri Borger, Motaz Hamed, Hannes Haberl, Robert Sassen, Alexander Radbruch, Albert J. Becker, Hartmut Vatter, Rainer Surges, and Sevgi Sarikaya-Seiwert

OBJECTIVE

Focal cortical dysplasia (FCD) is a common cause of early-onset intractable epilepsy, and resection is a highly sufficient treatment option. In this study, the authors aimed to provide a retrospective analysis of pre- and postoperative factors and their impact on postoperative long-term seizure outcome.

METHODS

The postoperative seizure outcomes of 50 patients with a mean age of 8 ± 4.49 years and histologically proven FCD type II were retrospectively analyzed. Furthermore, pre- and postoperative predictors of long-term seizure freedom were assessed. The seizure outcome was evaluated based on the International League Against Epilepsy (ILAE) classification.

RESULTS

Complete resection of FCD according to MRI criteria was achieved in 74% (n = 37) of patients. ILAE class 1 at the last follow-up was achieved in 76% (n = 38) of patients. A reduction of antiepileptic drugs (AEDs) to monotherapy or complete withdrawal was achieved in 60% (n = 30) of patients. Twelve patients (24%) had a late seizure recurrence, 50% (n = 6) of which occurred after reduction of AEDs. A lower number of AEDs prior to surgery significantly predicted a favorable seizure outcome (p = 0.013, HR 7.63). Furthermore, younger age at the time of surgery, shorter duration of epilepsy prior to surgery, and complete resection were positive predictors for long-term seizure freedom.

CONCLUSIONS

The duration of epilepsy, completeness of resection, number of AEDs prior to surgery, and younger age at the time of surgery served as predictors of postoperative long-term seizure outcome, and, as such, may improve clinical practice when selecting and counseling appropriate candidates for resective epilepsy surgery. The study results also underscored that epilepsy surgery should be considered early in the disease course of pediatric patients with FCD type II.