Angiolipoma of the right inferior colliculus: a rare central cause of hearing loss and limb ataxia

Angiolipoma of the right inferior colliculus: a rare central cause of hearing loss and limb ataxia

Case reports Angiolipoma of the right inferior colliculus: A rare central cause of hearing loss and limb ataxia Sanjay J. Pawar1 MD, Rewati Raman Sha...

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Case reports

Angiolipoma of the right inferior colliculus: A rare central cause of hearing loss and limb ataxia Sanjay J. Pawar1 MD, Rewati Raman Sharma1 MD, Anil P. Karapurkar2 MD, Manoj Kumar Tewari1 MD, Santosh D. Lad1 MD 1

Department of Neurosurgery, Khoula Hospital, Post Box-90, Postal code-116, Mina-Al-Fahal, Muscat, Oman, 2Department of Neurosurgery, Indraprastha Apollo Hospital, New Delhi, India

Summary Intracranial angiolipomas are rare entities and are infrequently symptomatic. These benign lesions are adherent to the surrounding neurovascular structures as they share the same blood supply, which limits their resectability, and impose a possibility of persistence of symptoms. However, due to recent advancements in the neuro-radiological fields and microneurosurgical techniques it has been possible to localise precisely and excise these lesions with low morbidity and mortality. The authors present an interesting rare case of intracranial angiolipoma of the right inferior colliculus situated in the quadrigeminal plate cistern. The patient had presented with ipsilateral hearing loss and upper limb ataxia. Interesting clinical findings, neuro-imaging studies and peroperative features are presented and discussed alongwith a brief review of the literature. ª 2003 Elsevier Science Ltd. All rights reserved.

CASE REPORT A 25-year old Omani gentleman was admitted with progressive impaired hearing on the right side for 1 year and occasional occipital headaches over the last 6 months. He complained of uncoordinated right upper limb movements. His general physical and systemic examinations were normal. Neurological examination revealed right sided sensory neural hearing loss, which was confirmed by audiometric assessment and the brain stem auditory evoked response showed a central cause for the hearing loss. The computed tomography (CT) scan showed a low attenuation mass in the right half of the quadrigeminal plate cistern between right inferior colliculus and cerebellar vermis. Magnetic resonance imaging (MRI) study confirmed an hyperintense lesion embedded in the region of the right inferior colliculus and was quite separate from the cerebellar vermis. As it was hyperintense on T1 weighted images and slightly hypointense on T2 weighted sequences of the MRI, thereby suggesting lipomatous pathology. The ventricular system was normal (see Figs. 1–3). His haematological and biochemical studies were normal. The patient underwent suboccipital craniectomy. At surgery the tumour was found to have a fibrous capsule. Dense surface adhesions to the right half of the cerebellar vermis posteriorly were then separated by sharp microsurgical dissection. It was arising from the right inferior colliculus of the midbrain. Numerous fine vessels and fibrous bands were seen passing through this mass. Due to these reasons and high vascularity, a partial excision was done. The post-operative period was, however, uneventful and the patient was discharged after 1 week. The histopathological ex-

Journal of Clinical Neuroscience (2003) 10(3), 346–348 ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00029-8

Keywords: inferior colliculus, quadrigeminal plate cistern, angiolipoma, hearing loss, ataxia, microsurgical approach Received 20 November 2001 Accepted 20 February 2002 Correspondence to: Rewati Raman Sharma, Senior Specialist Neurosurgeon, Post Box-397, Postal Code-118, Al-Harthy Complex, Muscat, Sultanate Of Oman. Tel.: +968-567339; Fax: +968-567339; E-mail: [email protected]

INTRODUCTION Central nevrous system (CNS) angiolipomas are rare neoplasms. They constitute less than 0.01% of intracranial tumours. With modern neuro-imaging facilities (CT & MRI scans) there has been an increase in the incidental detection of these lesions. They are mostly asymptomatic. Occassionaly they may present with symptoms and signs pertaining to their site of origin or their focal mass effect. These are histologically benign but their location makes total excision difficult as they share blood supply with the nearby neural pathways as well as because of adhesions to the surrounding neurovascular structures. Though total excision is the goal; partial excision sometimes gives good results in such circumstances. The authors report an interesting rare case of inferior colliculus angiolipoma presenting with ipsilateral hearing loss and hand ataxia. Interesting clinico-radiological & peroperative findings are presented and discussed in the light of the available literature. 346

Fig. 1 The CT Brain scan showing a low atteneuation lesion in the region of the right quadrigeminal plate cistern with distortion of the midbrain tectum on the right side.

Inferior colliculus angiolipoma and deafness

Fig. 2 Axial (A) and sagittal (B) T1 weighted MRI scans showing a hyperintense sharply localised and well circumscribed lesion involving the right inferior collicular region and brachium conjunctivum.

Fig. 3 Post-operative axial CT brain scan showing restoration of midbrain tectal anatomy.

amination showed features typical of an angiolipoma. He was seen in the outpatient clinic for regular follow-up. His right-sided hearing loss, and right upper limb ataxia showed significant improvement when seen at 1 year following surgery. DISCUSSION Lipomas are sharply circumscribed but tightly fastened to the adjacent nervous structures along the blood vessels by penetrating strands of connective tissue. Lipomas rich in blood vessels are termed angiolipomas.1 There may be an osseous nucleus with lipomatous tissue around the blood vessels.2 ª 2003 Elsevier Science Ltd. All rights reserved.

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Primary intracranial lipomas (ICL) are rare entities. They account for less than 0.5% of all primary intracranial tumours.3 With the advent of MRI more and more cases of ICLs are being detected.4–9 They are usually incidental findings, as the majority of ICLs are asymptomatic. Common sites for ICLs are corpus callosum (64%), quadrigeminal cistern or ambient cistern (13–26%), infundibular-chiasmatic region (13%), sylvian cistern (0.3%), and cerebellopontine angle (0.06%).3 More than 50% of the corpus callosal, 80% of cerebellopontine angle, 50% of sylvian fissure, and 20% of quadrigeminal plate cistern ICLs are asymptomatic. Infundibular-chiasmatic ICL are normally asymptomatic.3 In the past, it was not very clear whether the patients were symptomatic because of associated congenital malformations or due to the focal pressure effects. Evidence is now mounting to show that the pressure effect, which when relieved, makes many patients symptom-free. Though the majority of patients with dorsal brain stem lipomas (DBSLs) are asymptomatic, they can present with seizures with or without mental retardation,10 impairment of vertical gaze,6;11;12 trochelear nerve palsy,13 abducent nerve involvement, features of raised intracranial pressure because of obstruction to the CSF flow3 and sensory neural hearing loss. These symptoms are indicative of the area involved and act as guiding factors undertaking a surgical recourse.14 Thus DBSLs compressing brain stem or cerebellum can present as a focal mass lesion with ocular apraxia, diplopia, hemianaesthesia, ataxia, and headaches.3;6;12;15 Extension into the cerebellopontine angle area can manifest as sensory-neural hearing loss and ataxia because of pressure on the eighth cranial nerve and cerebellar peduncle. Inoue et al.16 have described a case of cerebellopontine angle lipoma that presented with hemifacial spasms and in that patient both the seventh and eighth nerves were involved by the lipomatous lesion. Seizure disorder and mental retardation may reflect towards co-existing congenital malformation.3;15 Shuangshoti et al.12 have reported an unusual case of angiolipoma of the left thalamic region. This patient presented with acute onset of symptoms (headache, hemiparesis, aphasia) suggestive of cerebrovascular stroke. Onset of sudden intracranial bleed due to an angiolipoma is a very unusual finding. The unique features of the case under discussion were right hearing deficits with right upper limb ataxia due to a pathological lesion arising from the right inferior colliculus (ipsilateral hearing deficits) and adjacent brachium conjunctivum (ipsilateral ataxia). These clinical features pointed to the site of the lesion, which was confirmed on the imaging studies as well as at the time of microsurgery. The histopathology showed features of angiolipoma. Such a presentation is instructive in clinical neurosurgical practice and adequately emphasizes the value of clinical history taking and performance of detailed neurological examination by the clinicians. The CT and MRI findings of ICLs are pathognomonic. On the CT scans, these lipomas are sharply demarcated hypoatteneuation lesions with CT values in the range of fatty tissues. They usually do not enhance on contrast administration except in a few cases of angiolipomas but occasional rim calcification may be present. The MRI scans show hyperintense lesions on the T1 weighted and slightly hypointense lesions on the T2 weighted images. They are homogenous in contrast to dermoids and epidermoids, which are heterogeneous. Uchino et al.17;18 after studying 23 intracranial lipomas using MRI have found that pericallosal and dorsal mesencephalic regions were the 2 principle sites for ICLs. They noted that dorsal mesencephalic lipomas are broad based, having irregular margins and cause deformities of the brain stem. Large dorsal brain stem lipomas (DBSL) may present with dilated ventricular system. Pericallosal ICLs are associated with other congenital anomalies Journal of Clinical Neuroscience (2003) 10(3)

348 Muzumdar et al.

of the corpus callosum. Antenatal ultrasound studies are also useful in picking up ICLs.3;19 The aim of surgery is to achieve the reversal of symptoms. Until recently only the procedures like ventriculo-peritoneal (VP) shunt were undertaken to relieve the symptoms of raised intracranial pressure.3 Of late many authors have reported good results with direct surgical attack on ICLs especially if the symptoms are ascribed to the local pressure exerted by ICLs. Though the goal of surgery is total removal using microsurgical techniques often a subtotal or partial removal is satisfying and the patient will improve symptomatically as happened in our case. It is not only the location and size of the tumour and its association with the superior cerebellar arteries and cranial nerves, which restricts the surgeon from going for total resection. Excision is extremely difficult because of the penetration of the adjacent vital central neural tissue by strands of connective tissues along the blood vessels. Therefore in some instances, the radical excision offers little additional benefit but greatly increases the risk of brain stem injury.3 Many authors feel that one should be conservative in approach if the DBSLs are asymptomatic. There is no agreement among various authors regarding direct surgical approach even in small symptomatic ICLs. Whereas some advocate an aggressive approach for symptomatic DBSL as they are known to grow in size with time.3;20 Other authors have their reservations over direct approach for small DBSLs and recommend CSF diversion procedures like VP shunt or third ventriculostomy.3;14;20;21 The prospect of direct judicious microsurgical approach have been able to show, though in small number of cases, including our case beyond doubt the usefulness of total or partial excision in relieving the clinical symptoms in patients with DBSLs.

10. Ono J, Ikeda T, Imai K, Mano T, Matsuoka T, Nagai T, Okada S. Intracranial lipoma of the quadrigeminal region associated with complex partial seizures. Pediatr Radiol 1998; 28(9): 729–731. 11. Ambrosetto P, Martinelli P, Bacci A, Daidone R. Lipoma of the quadrigeminal plate cistern. Ital J Neurol Sci 1985; 6(3): 347–349. 12. Shuangshoti S, Vajragupta L. Angiolipoma of thalamus presenting with abrupt onset suggestive of cerebrovascular disease. Clin Neuropathol 1995; 14(2): 82–85. 13. Hori A. Lipoma of the quadrigeminal region with evidence of congenital origin. Arch Pathol Lab Med 1986; 110(9): 850–851. 14. Nikaido Y, Imanishi M, Monobe T. Lipoma in the quadrigeminal cistern-case report. Neurol Med Chir (Tokyo) 1995; 35(3): 175–178. 15. Saatci I, Aslan C, Renda Y, Besim A. Parietal lipoma associated with cortical dysplasia and abnormal vasculature: case report and review of literature. Am J Neuroradiol 2000; 21(9): 1718–1721. 16. Inoue T, Maeyama R, Ogawa H. Hemifacial spasm resulting from cerebellopontine angle lipoma: case report. Neurosurgery 1995; 36(4): 846–850. 17. Uchino A, Hasuo K, Matsumoto S, Masuda K. MRI of dorsal mesencephalic lipomas. Clin Imaging 1993; 17(1): 12–16. 18. Uchino A, Maeoka N, Ohno M. Intracranial lipoma; MR imaging. Rinsho Hoshasen (Japanese) 1989; 34(13): 1591–1596. 19. Kim JY, Lee TJ, Chi JG. Congenital quadrigeminal lipoma with osteocartilagenous element. A case report. J Korean Med Sci 1996; 11(6): 537–539. 20. Kawamata T, Aoki N, Sakai T, Takakura K. Congenital triventricular hydrocephalus associated with a small lipoma in the quadrigeminal plate cistern. Childs Nerv Syst 1995; 11(2): 121–123. 21. Hayashi T, Shojima K, Yamamoto M, Hashimoto T, Fukuzumi A, Honda E. Intracranial lipomas-report of six cases (Japanese). No To Shinkei 1983; 35(3): 257–268.

Giant cerebral cavernous haemangioma: a case report and review of literature

CONCLUSIONS Clinical features of ipsilateral hearing loss and upper limb ataxia may sharply localise the inferior collicular lesions such as an angiolipomas. The value of the CT and MRI studies can not be over emphasised in confirming such clinical entities. Microsurgical excision is indicated in symptomatic patients when the symptoms are ascribed to the local pressure effects. It is now possible to excise these tumours completely using microneurosurgical techniques but if the lesions are embedded in the brain stem or enveloping other important neurovascualr structures then it is advisable to excise the tumour partially without adding morbidity to the patient as was done in the case under discussion.

D.P. Muzumdar

MCH,

M.G. Bhatjiwale

MCH,

Atul Goel

MCH

Department of Neurosurgery, Seth G.S. Medical College and King Edward Memorial Hospital, Parel, Mumbai 400012, India

Summary An 18-year-old male presented with uncontrolled left focal seizures with secondary generalisation for the past 10 years. Investigations revealed a large lobulated mass in the right frontal brain. Surgical excision of a giant cavernous haemangioma was performed. The patient is seizure-free following the surgery. The case and relevant literature on the rare entity of giant intracranial cavernous haemangiomas is discussed. ª 2003 Elsevier Science Ltd. All rights reserved.

REFERENCES 1. Prabhu SS, O’Donovan DG, Gurusinghe NT. Intracranial angiolipoma: report of two cases. Br J Neurosurg 1995; 9(6): 793–797. 2. Zulch KJ. In: Brain Tumours: Their Biology and Pathology. third edn. Springer, Berlin 1986; 441–445. 3. Baeesa SS, Higgins MJ, Ventureyra EC. Dorsal brain stem lipomas: case report. Neurosurgery 1996; 38(5): 1031–1035. 4. Bakshi R, Shaikh ZA, Kamran S, Kinkel PR. MRI findings in 32 consecutive lipomas using conventional and advanced sequences. J Neuroimaging 1999; 9(3): 134–140. 5. Ichikawa T, Kumazaki T, Mizumura S, Kijima T, Motohashi S, Gocho G. Intracranial lipomas: demonstration by computed tomography and magnetic resonance imaging. J Nippon Med Sch 2000; 67(5): 388–391. 6. Shuangshoti S, Wangsuphachart S. Angiolipoma of the suprasellar region. J Med Assoc Thai 1995; 78(11): 631–634. 7. Andaluz N, Balko G, Bui H, Zuccarello M. Angiolipomas of the central nervous system. J Neurooncol 2000; 49(3): 219–230. 8. Heiss E, Guhl L, Mironov A. A lipoma of the cerebellopontine angle German Neurochirurgia (Stuttg) 1988; 31(3): 104–106. 9. Weill A, Melancon D, Del Carpio R, Tampieri D, Ethier R. Angiolipoma of the central nervous system French Rev Neurol (Paris) 1991; 147(4): 285–292.

Journal of Clinical Neuroscience (2003) 10(3)

Journal of Clinical Neuroscience (2003) 10(3), 348–351 ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00012-2

Keywords: cavernous haemangioma, intracranial, magnetic resonance imaging Received 28 December 2001 Accepted 14 April 2002 Correspondence to: Prof. Atul Goel, Department of Neurosurgery, King Edward Memorial Hospital and Seth G.S. Medical College, Parel, Mumbai 400012, India. Tel.: +91-22-4129884; Fax: +91-22-4143435; E-mail: [email protected]

INTRODUCTION Cavernous haemangioma are hamartomatous hemorrhagic lesions. They are congenital in origin and are differentiated clearly from ª 2003 Elsevier Science Ltd. All rights reserved.