Lhermitte-Duclos Hastalığının Subtotal Rezeksiyondan Sonra İyileşmesi; Vaka Raporu

Abstract

Lhermitte-Duclos Hastalığı (LDH) genç erişkinlikte, genellikle 3-4. dekatta çok nadir görülen tipik olarak serebellumu tek taraflı tutan, serebellar korteksin moleküler ve granüler hücre katmanlarında kalınlaşma gösteren iyi huylu hamartomatöz bir hastalıktır. LDH yavaş ilerleyen bir hastalık olduğundan kitleye ait bulgu yoksa konservatif olarak tedavi edilebilirken, kitle etkisi olan hastalarda total eksizyon önerilmesine rağmen olgumuzda ameliyat sırasında bu ayrım net olarak yapılamadığı için subtotal rezeksiyon yapıldı. Postoperatif kontrolünde kraniyal manyetik rezonans (MRG) incelemesinde lezyonun gerilediği görüldü. Hastanın postoperatif 7. ay kontrollerinde nüks veya rezidü izlenmedi. Hastanın 7 aylık takibinde yeni gelişen nörolojik semptom veya defisit yoktu. Ancak uzun dönem takiplerde nüks açısından hastalar yakın takip edilmelidir.

Keywords

• Lhermitte Duclos
• Serebellum
• Subtotal Rezeksiyon

Lhermitte-Duclos Disease Recovery After Subtotal Resection; Case Report

Abstract

Lhermitte-Duclos Disease (LDD) is a rare benign hamartomatous disorder that typically manifests unilaterally in the cerebellum, leading to thickening of the molecular and granular cell layers of the cerebellar cortex. It most commonly occurs in young adulthood, typically in the third or fourth decade of life. Since LDD is a slowly progressive disease, conservative management is an option in the absence of mass effect, whereas total excision is recommended in symptomatic patients. In our case, subtotal resection was performed due to indistinct surgical margins observed intraoperatively. Postoperative cranial magnetic resonance imaging (MRI) demonstrated regression of the lesion. No recurrence or residual tumor was detected at the 7-month follow-up. During this period, the patient remained neurologically stable without newly developed symptoms or deficits. However, long-term follow-up is essential to monitor for potential recurrence.

Keywords

• Cerebellum
• Lhermitte Duclos
• Subtotal Resection

References

1. Afshar-Oromieh, A., Linhart, H., Podlesek, D., Schrempf, W., Schackert, G., & Krex, D. (2010). Postoperative cerebellar mutism in adult patients with Lhermitte-Duclos disease. Neurosurgical Review, 33, 401–408.
2. Akiyama, Y., Ikeda, J., Ibayashi, Y., Nonaka, T., Asayi, Y., & Houkin, K. (2006). Lhermitte-Duclos disease with cervical paraspinal arteriovenous fistula: Case report. Neurologia Medico-Chirurgica (Tokyo), 46, 446–449.
3. Banerjee, A. K., & Gleadhill, C. A. (1979). Lhermitte-Duclos disease: (Diffuse cerebellar hypertrophy) prolonged post-operative survival. Irish Journal of Medical Science, 148(1), 97.
4. Berkman, M. Z., Uluer, Ş., & Derinkök, T. (1998). Lhermitte-Duclos disease. Turkish Neurosurgery, 8, 96–100.
5. Borni, M., Kammoun, B., Kolsi, F., Abdelmouleh, S., & Boudawara, M. Z. (2019). The Lhermitte-Duclos disease: A rare bilateral cerebellar location of a rare pathology. Pan African Medical Journal, 33, 118.
6. Carter, J. E., Merren, M. D., & Swann, K. W. (1989). Preoperative diagnosis of Lhermitte-Duclos disease by magnetic resonance imaging: Case report. Journal of Neurosurgery, 70, 135–137.
7. Chen, K. S., Hung, P. C., Wang, H. S., Jung, S. M., & Ng, S. H. (2002). Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease). Pediatric Neurology, 27, 404–406.
8. Cheng, C. S., Ou, C. H., Chen, J. S., Lui, C. C., & Yeh, L. R. (2019). Lhermitte-Duclos disease: A case report with radiologic-pathologic correlation. Radiology Case Reports, 14(6), 734–739.

9. Fernandes-Cabral, D. T., Zenonos, G. A., Hamilton, R. L., Panesar, S. S., & Fernandez-Miranda, J. C. (2016). High-definition fiber tractography in the evaluation and surgical planning of Lhermitte-Duclos disease: A case report. World Neurosurgery, 92, 587.e9–587.e13.
10. Haris, A. A., Chandra, S. R., & Peethambaran, B. (2008). Lhermitte-Duclos disease. Neurology India, 56, 495–496.
11. Khandpur, U., Huntoon, K., Cohn, S. M., Shaw, A., & Elder, J. B. (2019). Bilateral recurrent dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) in Cowden syndrome: A case report and literature review. World Neurosurgery, 127, 319–325.
12. Klisch, J., Juengling, F., Spreer, J., et al. (2001). Lhermitte-Duclos disease: Assessment with MR imaging, positron emission tomography, single-photon emission CT, and MR spectroscopy. AJNR American Journal of Neuroradiology, 22, 824–830.
13. Lhermitte, J., & Duclos, P. (1920). Sur un ganglioneurome diffuse du cortex du cervelet. Bulletin de l’Association Française pour l’Etude du Cancer Paris, 9, 99–107.
14. Nowak, D. A., Trost, H. A., Porr, A., Sto, A., & Lumenta, C. B. (2001). Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Neurosurgery, 103, 105–110.
15. Xu, L., Gao, P. Y., Lin, Y., Tian, T. D., Lei, J., & Ma, L. (2005). Magnetic resonance imaging findings in Lhermitte-Duclos disease: Reports of three cases. Chinese Medical Journal, 118, 1933–1936.
16. Yesildağ, A., Baykal, B., Ayata, A., et al. (2005). Lhermitte-Duclos disease associated with neurofibromatosis type-1 and nonossifying fibroma. Acta Radiologica, 46, 97–100.