Lipomatosis of the Trigeminal Nerve Causing Trigeminal Neuralgia: Case Report and Literature Review

ABSTRACT

Cerebellopontine angle lipomas are rare and attempts at surgical excision are associated with significant morbidity. Lipomatosis of nerve, the fatty infiltration of nerves, is a distinct entity. We present a case of intractible trigeminal neuralgia caused by lipomatosis of the trigeminal nerve. Clinical case: A 25-year-old male presented with severe right-sided trigeminal neuralgia. Imaging showed a lesion involving the trigeminal nerve with signal characteristics of fat. At surgery the lesion was found to be a fatty infiltration of the nerve itself. Surgery was therefore limited to arachnoid adhesiolysis. The patient remains symptom-free and neurologically intact to date. Correctly identifying these lesions as lipomatosis of nerve rather than lipoma of the cerebellopontine angle make it clear that even partial surgical excision will inevitably result in neurological deficit and should not be attempted. However, in the case of intractable trigeminal neuralgia we demonstrate that surgery can still play a role.

KEYWORDS

Lipomatosis - trigeminal nerve - neuralgia - cerebellopontine angle - lipoma

REFERENCES

• 1 Nielsen G P. Adipocytic tumours. In: Fletcher CDM, Unni KK, Mertens F Pathology and Genetics of Tumors of Soft Tissue and Bone (The International Agency for Research on Cancer/WHO Classification of Tumours). Geneva; The World Health Organisation 2002: 24-25
• 2 Beck D W, Menezes A H. Lesions in Meckel’s cave: variable presentation and pathology.  J Neurosurg. 1987;  67 684-689
• 3 Berti E, Roncaroli F. Fibrolipomatous hamartoma of a cranial nerve.  Histopathology. 1994;  24 391-392
• 4 Bigelow D C, Eisen M D, Smith P G et al.. Lipomas of the internal auditory canal and cerebellopontine angle.  Laryngoscope. 1998;  108 1459-1469
• 5 Budka H. Intracranial lipomatous hamartomas (intracranial “lipomas”). A study of 13 cases including combinations with medulloblastoma, colloid and epidermoid cysts, angiomatosis and other malformations.  Acta Neuropathol. 1974;  28 205-222
• 6 Faerber E N, Wolpert S M. The value of computed tomography in the diagnosis of intracranial lipomata.  J Comput Assist Tomogr. 1978;  2 297-299
• 7 Kemmling A, Noelte I, Gerigk L, Singer S, Groden C, Scharf J. A diagnostic pitfall for intracranial aneurysms in time-of-flight MR angiography: small intracranial lipomas.  AJR Am J Roentgenol. 2008;  190 W62-67
• 8 Donati F, Vassella F, Kaiser G, Blumberg A. Intracranial lipomas.  Neuropediatrics. 1992;  23 32-38
• 9 Tankéré F, Vitte E, Martin-Duverneuil N, Soudant J. Cerebellopontine angle lipomas: report of four cases and review of the literature.  Neurosurgery. 2002;  50 626-631, discussion 631–632
• 10 Zimmermann M, Kellermann S, Gerlach R, Seifert V. Cerebellopontine angle lipoma: case report and review of the literature.  Acta Neurochir (Wien). 1999;  141 1347-1351
• 11 Schuhmann M U, Lüdemann W O, Schreiber H, Samii M. Cerebellopontine angle lipoma: a rare differential diagnosis.  Skull Base Surg. 1997;  7 199-205
• 12 Graves V B, Schemm G W. Clinical characteristics and CT findings in lipoma of the cerebellopontine angle. Case report.  J Neurosurg. 1982;  57 839-841
• 13 Rosenbloom S B, Carson B S, Wang H, Rosenbaum A E, Udvarhelyi G B. Cerebellopontine angle lipoma.  Surg Neurol. 1985;  23 134-138
• 14 Delgado Mije D, Moro Sánchez R M, Escribano Fernández M, Azuara Muslera M V. Neuralgia of the trigeminal secondary to lipoma of the pontocerebellar angle.  Med Clin (Barc). 1992;  99 556
• 15 Aihara N, Nagai H, Kamiya K, Matsumoto T, Yamashita N. Cerebellopontine angle lipoma—case report.  No To Shinkei. 1993;  45 559-562
• 16 Kato T, Sawamura Y, Abe H. Trigeminal neuralgia caused by a cerebellopontine-angle lipoma: case report.  Surg Neurol. 1995;  44 33-35
• 17 Behar P M, Dolan R, Dastur K, Marrangoni A G, Nayak N. Fibrovascular lipoma of the cerebellopontine angle mimicking trigeminal neuralgia.  Ear Nose Throat J. 1998;  77 58-60
• 18 Celik S E, Kocaeli H, Cordan T, Bekar A. Trigeminal neuralgia due to cerebellopontine angle lipoma. Case illustration.  J Neurosurg. 2000;  92 889
• 19 Alafaci C, Salpietro F M, Puglisi E et al.. Trigeminal pain caused by a cerebellopontine-angle lipoma. Case report and review of the literature.  J Neurosurg Sci. 2001;  45 110-113
• 20 Raieli V, Eliseo G, Manfrè L, Pandolfi E, Romano M, Eliseo M. Trigeminal neuralgia and cerebellopontine-angle lipoma in a child.  Headache. 2001;  41 720-722
• 21 Schlierter M, Schrey M, Schramm P. Lipoma in cerebellopontine angle.  Rofo. 2007;  179 1-3
• 22 Marton E, Basaldella L, Longatti P L. Minimal surgery for a cerebellopontine angle lipoma.  J Clin Neurosci. 2009;  16 129-132
• 23 Truwit C L, Barkovich A J. Pathogenesis of intracranial lipoma: an MR study in 42 patients.  AJR Am J Roentgenol. 1990;  155 855-864, discussion 865
• 24 Sunderland S. The adipose tissue of peripheral nerves.  Brain. 1945;  68 118-122
• 25 Marom E M, Helms C A. Fibrolipomatous hamartoma: pathognomonic on MR imaging.  Skeletal Radiol. 1999;  28 260-264
• 26 Lenthall R, McConachie N S, Jefferson D. Cerebellopontine angle lipoma with an incidental scalp lipoma in a patient with hemifacial spasm.  Eur Radiol. 2000;  10 195
• 27 Dalley R W, Robertson W D, Lapointe J S, Durity F A. Computed tomography of a cerebellopontine angle lipoma.  J Comput Assist Tomogr. 1986;  10 704-706
• 28 Ruggieri R M, Manfrè L, Calbucci F, Piccoli F. Therapeutic considerations in cerebellopontine angle lipomas inducing hemifacial spasm.  Neurol Sci. 2000;  21 329-331
• 29 Yildiz H, Hakyemez B, Koroglu M, Yesildag A, Baykal B. Intracranial lipomas: importance of localization.  Neuroradiology. 2006;  48 1-7
• 30 Rodríguez Prado N, Llorente Pendás J L, Gómez Martínez J R et al.. Cerebellopontine angle and internal auditory canal lipomas: report of four cases and review of the literature.  Acta Otorrinolaringol Esp. 2004;  55 126-130