Novel Insights from Clinical Practice Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
Received: April 2, 2014 Accepted after revision: October 12, 2014 Published online: November 21, 2014
Tuberculoma of the Cavernous Sinus and Meckel’s Cave in a Child V.R. Roopesh Kumar a Venkatesh S. Madhugiri a Surendra Kumar Verma b S. Deepak Barathi c Awdhesh Kumar Yadav a Prasanna Bidkar d Departments of a Neurosurgery, b Pathology, c Radiodiagnosis and d Anaesthesiology and Critical Care, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India
Established Facts • Tuberculomas of the skull base involving the cavernous sinus area are very rare. • Pre-operative diagnosis is difficult to achieve, and thus most lesions are resected via extensive skull base approaches.
Novel Insights • Only 10 cases have been reported in adults; this is the first report of such a lesion in a child. • This is the first report of a skull base tuberculoma with extensive involvement of the cavernous sinus, Meckel’s cave and posterior fossa. • Despite the large size of the lesion and extensive bone and neural involvement, surgical debulking, histopathological diagnosis and antitubercular therapy result in cure in most instances.
Key Words Cavernous sinus · Meckel’s cave · Petrous apex · Otitis media · Meningitis · Tuberculoma · Antitubercular therapy
Abstract Tuberculous infection of the cavernous sinus and Meckel’s cave is extremely rare. In this report, we describe a patient with tuberculoma of the cavernous sinus and Meckel’s cave, extending to the petrous apex. The patient underwent microsurgical excision of the lesion and antitubercu-
© 2014 S. Karger AG, Basel 1016–2291/14/0496–0369$39.50/0 E-Mail [email protected] www.karger.com/pne
lar chemotherapy resulting in a good outcome. We describe the diagnostic difficulties and review the relevant literature. © 2014 S. Karger AG, Basel
Introduction
Tuberculosis (TB) remains a major public health concern in developing countries. The human immunodeficiency virus (HIV) pandemic and the increasing frequenV.R. Roopesh Kumar Departments of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) Pondicherry, 605006 (India) E-Mail roops1975 @ gmail.com
a
b
c
d
cy of global travel have together led to a resurgence of this disease in developed countries as well [1]. Central nervous system (CNS) TB has a wide spectrum of clinical manifestations and produces a variety of lesions in the neuraxis. The most commonly encountered form of CNS TB is meningitis [2]. When the disease involves atypical and unusual sites like the petrous apex or cavernous sinus, it can be very difficult to establish a pre-operative diagnosis, despite advances in neuro-imaging [3]. We report a very rare manifestation of CNS TB in a child. The infection took the form of a granulomatous lesion involving the cavernous sinus and Meckel’s cave. The patient underwent surgical extirpation of the mass lesion followed by antitubercular chemotherapy (ATT); this led to a complete cure of the infection and good neurological outcome. Cavernous sinus tuberculomas are extremely rare, and only 10 cases have been reported in the literature. In all reported instances, the patients were adults [4–13]. The occurrence of such a lesion in a child and the extensive involvement of the cavernous sinus, Meckel’s cave, petrous apex and petroclival region by the granulomatous lesion make this a unique case.
Case Report
e
f
Fig. 1. Pre-operative imaging. a Gd contrast-enhanced axial MRI image showing the florid granulation tissue in the cavernous sinus and petrous apex region, with a honeycomb pattern of peripheral enhancement. b Axial plain CT image. The lesion is hyperdense on CT images. c Heavily T2-weighted constructive interference steady-state 3-dimensional image, showing the lesion to be iso- to minimally hypo-intense. The extension into the posterior fossa is evident, as is the oedema in the right middle cerebellar peduncle. d Axial CT, bone window, showing irregularity and erosion of the petrous apex. e Sagittal Gd-enhanced MRI image, showing the right internal carotid artery to be patent and coursing through the lesion. f Coronal T2-weighted MRI image, showing the hypo-intense lesion in the sphenoid sinus.
370
Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
An 11-year-old girl was brought with complaints of right hemicranial headache, diplopia and numbness of the right side of her face for 2 months. Her medical history was significant for chronic discharge from the right ear and hearing loss for 2 years; she had not sought or received any medical treatment for this problem. On examination, she had right 6th-nerve paresis and hypo-aesthesia in the right V1 and V2 distribution. No facial paresis was noted. Active purulent discharge was noted in the right ear; cultures of this pus grew Acinetobacter baumannii. Magnetic resonance imaging (MRI) of the brain revealed a lesion involving the posterior part of the right cavernous sinus and Meckel’s cave, with erosion of the petrous apex and extension of the lesion into the posterior fossa. The lesion was hypo-intense on T1-weighted images and iso- to hypo-intense on T2-weighted images (fig. 1). There was a heterogeneous honeycomb-like pattern of enhancement on Gd contrast sequences (fig. 1a). The internal carotid artery was patent and appeared normal in calibre (fig. 1e). A cranial computerized tomogram (CT) revealed the extent of erosion of the petrous apex (fig. 1b, d). Considering the history of chronic ear discharge, a diagnosis of inflammatory granuloma of the cavernous sinus secondary to chronic otitis media was made. Hormone evaluation revealed tri-iodothyronine, thyroxine, thyroid-stimulating hormone and serum cortisol levels to be normal. Pure tone audiometry revealed moderate conductive hearing loss on the right side. Visual acuity and visual fields were normal. Serological tests for HIV-1 and -2 were negative. Soon after the MRI scan had been performed, the child developed high grade fever; clinical examination revealed the presence of neck rigidity and a positive Kernig’s sign. Lumbar puncture was performed; cerebrospinal fluid analysis was suggestive of menin-
Kumar/Madhugiri/Verma/Barathi/Yadav/ Bidkar
Color version available online
Lymphocytes
Caseous necrosis
Langhans giant cells Non-caseating epithelioid granuloma
a
Lymphocyte cuff
b
Fig. 2. Histopathology of the resected mass. Haematoxylin-eosin staining. a Both caseating and non-caseating granulomas are seen. Low power view. ×40. b The Langhans giant cells and the lymphocytic infiltrate are clearly
seen. High power view. ×100.
gitis (800 cells, predominantly polymorphs, protein 180 mg/dl and glucose 20 mg/dl). Cerebrospinal fluid cytology revealed no bacteria or abnormal tumour cells. She was administered antimeningitic doses of intravenous antibiotics (3rd-generation cephalosporin + aminoglycosides) as per the sensitivity pattern of the pus culture obtained from the ear discharge. Although her symptoms partially resolved after the institution of antibiotic therapy (reduction in ear discharge), she continued to have severe headache and her diplopia worsened. A fresh MRI of the brain (after 10 days of antibiotic therapy) revealed an increase in the size of the lesion. Surgical resection of the lesion was planned, with the dual intent of achieving a definitive diagnosis and reducing the patient’s symptoms (caused by raised intracranial pressure as well as direct involvement of neural structures by the lesion). A right frontotemporal craniotomy was made and an orbitozygomatic osteotomy was added. Via a temporopolar intradural approach, the outer wall of the cavernous sinus was opened. A fleshy, white, moderately vascular mass lesion with areas of pus collection within it was encountered. The 6th nerve was adherent to the lesion but could be preserved after careful dissection. Portions of the lesion densely adherent to the internal carotid artery were left behind. Postoperative recovery was uneventful, barring a mild worsening of 6th-nerve function. A postoperative contrast-enhanced CT scan of the brain revealed near total resection of the lesion. Histopathological examination of the resected lesion revealed caseating granulomas with Langhans’ giant cells and a chronic inflammatory infiltrate, diagnostic of a tubercular granuloma (fig. 2). ATT (with rifampicin, isoniazid, ethambutol and pyrazinamide) was instituted. Six weeks after surgery, MRI revealed a small residual lesion in the region of the petrous apex. Besides this residual lesion, several new lesions had appeared in the left cavernous sinus, right superior petrosal sinus and tentorial incisura (fig. 3). The patient, however, remained asymptomatic for these lesions and had developed no fresh neurological deficits. ATT was continued for the next 12 months and an MRI repeated at this time. The 12-month follow-up MRI demonstrated complete resolution of nearly all the lesions (fig. 4). As per our protocol, ATT will be administered for a total of 18 months.
Cavernous Sinus Tuberculoma
Fig. 3. Postoperative follow-up imaging, 6 weeks after surgery (un-
der ATT). Gd contrast-enhanced axial MRI image 3 months after surgery showing new lesions in the right superior petrous region and left cavernous sinus area.
Discussion
CNS TB constitutes about 0.5–10% of all CNS infections and is most often encountered in immunodeficient patients. Two common pathological forms of CNS TB include basal meningitis and granulomatous lesions (tuberculoma) [2]. Tuberculomas account for as much as 10–30% of all intracranial mass lesions in developing countries. In adults, they usually occur in the supratentorial space, either in a Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
371
Fig. 4. Twelve-month follow-up MRI, axial Gd-enhanced images, showing near total resolution of all lesions. a There is a very small area of enhancement posterior to the right petrous bone. b There is also a small residual area of enhancement in the right cavernous sinus. All other lesions have resolved.
a
subcortical location or in the basal ganglia [3]. In children, cerebellar lesions are commonly encountered [2]. Lesions at unusual sites such as the brain stem, ventricles, pituitary gland and the cerebellopontine angle have also been reported in the literature; most of these occur in adults [14–16]. CNS TB is almost always secondary; the usual primary site of infection is a pulmonary focus. In the present case, we presume that the portal of entry of infection was the infected middle ear cavity. The organism probably first infected the mastoid air cells, with subsequent spread of infection to the petrous bone and the cavernous sinus, either by contiguous spread or via venous channels. However, Mycobacterium tuberculosis could not be isolated in culture from the ear discharge. M. tuberculosis is a slowly replicating organism and is difficult to grow in culture; the presence of a superadded bacterial infection would make isolation of the organism even more difficult. The characteristics of the lesion on MRI (enhancing lesion with hypo-intense signals on T2-weighted images) were suggestive of an inflammatory granuloma; however, a definitive diagnosis of tuberculosis could not be made due to the unusual location as well as the initial delay in response to ATT. When tubercular granulomas occur at unusual sites in the skull base, a conclusive pre-operative diagnosis is difficult to establish, despite advances in neuro-imaging techniques [3, 17, 18]. This is emphasized by the fact that in nearly all published clinical reports about cavernous sinus TB, patients underwent surgery – either to achieve a definite tissue diagnosis or with an erroneous diagnosis of meningioma [6, 7, 9, 10, 13]. Tubercular granulomas have a characteristic appearance on histopathological examination, and a definitive 372
Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
b
diagnosis is possible on the basis of microscopic morphology in most cases. ATT is usually initiated on the basis of histopathological findings. Bacterial culture of M. tuberculosis may take several weeks to yield definite results and the bacilli are isolated in only 35% of resected granulomas. Hence, a negative bacterial culture does not preclude a diagnosis of TB [17, 18]. ATT should ideally be administered for 12–18 months, even if radiological resolution of the lesions is seen earlier [17]. However, the radiological response usually lags behind the clinical response to treatment. The appearance of new lesions on the 3-month follow-up MRI in the present case is an example of this. The reason for the appearance of new lesions could either be intra-operative dissemination of the infection (via the intercavernous sinus) or a paradoxical increase in the size of pre-existing lesions (as may occur during the intensive phase of ATT) [19]. Systemic steroids are generally administered during the initial weeks of ATT (especially in patients with tubercular meningitis) to prevent paradoxical enlargement of lesions [19].
Conclusion
TB should be considered in the differential diagnosis of cavernous sinus lesions in developing countries. A strong clinical suspicion may restrict surgery to only a biopsy rather than performing an extensive skull base approach and radial resection. ATT remains the mainstay of treatment and, in the majority of cases, can result in a cure and good neurological outcome. Kumar/Madhugiri/Verma/Barathi/Yadav/ Bidkar
References 1 Solovic I, Jonsson J, Korzeniewska-Koseła M, Chiotan DI, Pace-Asciak A, Slump E, Rumetshofer R, Abubakar I, Kos S, Svetina-Sorli P, Haas W, Bauer T, Sandgren A, van der Werf MJ: Challenges in diagnosing extrapulmonary tuberculosis in the European Union, 2011. Euro Surveill 2013;18:pii:20432. 2 Arvind C, Korath PM, Raveendranadhan K, Jagadeesan K: A retrospective study of 1,247 cases of intracranial tuberculomata diagnosed by computerized tomography. J Assoc Phys Ind 1993;41:559–561. 3 Gupta RK, Jena A, Singh AK, Sharma A, Puri V, Gupta M: Role of magnetic resonance (MR) in the diagnosis and management of intracranial tuberculomas. Clin Radiol 1990;41: 120–127. 4 Al Soub H, Al Alousi FS, Al-Khal AL: Tuberculoma of the cavernous sinus. Scand J Infect Dis 2001;33:868–870. 5 Bafna S, Lee AG: Presumed tuberculosis presenting as a cavernous sinus syndrome. J Neuroophthalmol 1997;17:207–208. 6 Boutarbouch M, Arkha Y, Gana R, El Maquili MR, Bellakhdar F: Tuberculoma of the cavernous sinus mimicking a meningioma: case report and review of the literature. J Neurol Sci 2009;278:123–126.
Cavernous Sinus Tuberculoma
7 Grayeli AB, Redondo A, Salama J, Rey A: Tuberculoma of the cavernous sinus: case report. Neurosurgery 1998;42:179–181. 8 Hui AC, Wong WS, Wong KS: Cavernous sinus syndrome secondary to tuberculous meningitis. Eur Neurol 2002;47:125–126. 9 Jaimovich SG, Thea VC, Guevara M, Gardella JL: Cavernous sinus tuberculoma mimicking a neoplasm: case report, literature review, and diagnostic and treatment suggestions for tuberculomas in rare locations. Surg Neurol Int 2013;4:158. 10 Morris JT, Joyce MP: Central nervous system tuberculoma presenting as a cavernous sinus tumor. Clin Infect Dis 1992;15:181–182. 11 Phookan G, Towns GM: Tuberculoma of the cavernous sinus – a case report. Br J Neurosurg 1995;9:205–207. 12 Rebai R, Boudawara MZ, Bahloul K, Chabchoub I, Chaari S, Boudawara T, Ben Mansour H: Cavernous sinus tuberculoma: diagnostic difficulties in a personal case. Surg Neurol 2001;55:372–375. 13 Yanardag H, Uygun S, Yumuk V, Caner M, Canbaz B: Cerebral tuberculosis mimicking intracranial tumour. Singapore Med J 2005; 46:731–733.
14 Pereira J, Vaz R, Carvalho D, Cruz C: Thickening of the pituitary stalk: a finding suggestive of intrasellar tuberculoma? Case report. Neurosurgery 1995;36:1013–1016. 15 Povedano Rodriguez V, Mellado Rubio R, Jurado Ramos A, Seco Piñero I, López Villarejo P: Tuberculoma as expansive process of the cerebellopontine angle. Case report and bibliographical survey (in Spanish). Ann Otorrinolaringol Ibero Am 1993; 20: 55–60. 16 Vajramani GV, Devi BL, Hegde T, Santosh V, Khanna N, Vasudev MK: Intraventricular tuberculous abscess: a case report. Neurol India 1999;47:327–329. 17 Poonnoose SI, Rajshekhar V: Rate of resolution of histologically verified intracranial tuberculomas. Neurosurg 2003; 53: 873– 878. 18 Unal A, Sutlas PN: Clinical and radiological features of symptomatic central nervous system tuberculomas. Eur J Neurol 2005;12:797– 804. 19 Reiser M, Fätkenheuer G, Diehl V: Paradoxical expansion of intracranial tuberculomas during chemotherapy. J Infect 1997; 35: 88– 90.
Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
373
Copyright: S. Karger AG, Basel 2015. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Received: April 2, 2014 Accepted after revision: October 12, 2014 Published online: November 21, 2014
Tuberculoma of the Cavernous Sinus and Meckel’s Cave in a Child V.R. Roopesh Kumar a Venkatesh S. Madhugiri a Surendra Kumar Verma b S. Deepak Barathi c Awdhesh Kumar Yadav a Prasanna Bidkar d Departments of a Neurosurgery, b Pathology, c Radiodiagnosis and d Anaesthesiology and Critical Care, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India
Established Facts • Tuberculomas of the skull base involving the cavernous sinus area are very rare. • Pre-operative diagnosis is difficult to achieve, and thus most lesions are resected via extensive skull base approaches.
Novel Insights • Only 10 cases have been reported in adults; this is the first report of such a lesion in a child. • This is the first report of a skull base tuberculoma with extensive involvement of the cavernous sinus, Meckel’s cave and posterior fossa. • Despite the large size of the lesion and extensive bone and neural involvement, surgical debulking, histopathological diagnosis and antitubercular therapy result in cure in most instances.
Key Words Cavernous sinus · Meckel’s cave · Petrous apex · Otitis media · Meningitis · Tuberculoma · Antitubercular therapy
Abstract Tuberculous infection of the cavernous sinus and Meckel’s cave is extremely rare. In this report, we describe a patient with tuberculoma of the cavernous sinus and Meckel’s cave, extending to the petrous apex. The patient underwent microsurgical excision of the lesion and antitubercu-
© 2014 S. Karger AG, Basel 1016–2291/14/0496–0369$39.50/0 E-Mail [email protected] www.karger.com/pne
lar chemotherapy resulting in a good outcome. We describe the diagnostic difficulties and review the relevant literature. © 2014 S. Karger AG, Basel
Introduction
Tuberculosis (TB) remains a major public health concern in developing countries. The human immunodeficiency virus (HIV) pandemic and the increasing frequenV.R. Roopesh Kumar Departments of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) Pondicherry, 605006 (India) E-Mail roops1975 @ gmail.com
a
b
c
d
cy of global travel have together led to a resurgence of this disease in developed countries as well [1]. Central nervous system (CNS) TB has a wide spectrum of clinical manifestations and produces a variety of lesions in the neuraxis. The most commonly encountered form of CNS TB is meningitis [2]. When the disease involves atypical and unusual sites like the petrous apex or cavernous sinus, it can be very difficult to establish a pre-operative diagnosis, despite advances in neuro-imaging [3]. We report a very rare manifestation of CNS TB in a child. The infection took the form of a granulomatous lesion involving the cavernous sinus and Meckel’s cave. The patient underwent surgical extirpation of the mass lesion followed by antitubercular chemotherapy (ATT); this led to a complete cure of the infection and good neurological outcome. Cavernous sinus tuberculomas are extremely rare, and only 10 cases have been reported in the literature. In all reported instances, the patients were adults [4–13]. The occurrence of such a lesion in a child and the extensive involvement of the cavernous sinus, Meckel’s cave, petrous apex and petroclival region by the granulomatous lesion make this a unique case.
Case Report
e
f
Fig. 1. Pre-operative imaging. a Gd contrast-enhanced axial MRI image showing the florid granulation tissue in the cavernous sinus and petrous apex region, with a honeycomb pattern of peripheral enhancement. b Axial plain CT image. The lesion is hyperdense on CT images. c Heavily T2-weighted constructive interference steady-state 3-dimensional image, showing the lesion to be iso- to minimally hypo-intense. The extension into the posterior fossa is evident, as is the oedema in the right middle cerebellar peduncle. d Axial CT, bone window, showing irregularity and erosion of the petrous apex. e Sagittal Gd-enhanced MRI image, showing the right internal carotid artery to be patent and coursing through the lesion. f Coronal T2-weighted MRI image, showing the hypo-intense lesion in the sphenoid sinus.
370
Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
An 11-year-old girl was brought with complaints of right hemicranial headache, diplopia and numbness of the right side of her face for 2 months. Her medical history was significant for chronic discharge from the right ear and hearing loss for 2 years; she had not sought or received any medical treatment for this problem. On examination, she had right 6th-nerve paresis and hypo-aesthesia in the right V1 and V2 distribution. No facial paresis was noted. Active purulent discharge was noted in the right ear; cultures of this pus grew Acinetobacter baumannii. Magnetic resonance imaging (MRI) of the brain revealed a lesion involving the posterior part of the right cavernous sinus and Meckel’s cave, with erosion of the petrous apex and extension of the lesion into the posterior fossa. The lesion was hypo-intense on T1-weighted images and iso- to hypo-intense on T2-weighted images (fig. 1). There was a heterogeneous honeycomb-like pattern of enhancement on Gd contrast sequences (fig. 1a). The internal carotid artery was patent and appeared normal in calibre (fig. 1e). A cranial computerized tomogram (CT) revealed the extent of erosion of the petrous apex (fig. 1b, d). Considering the history of chronic ear discharge, a diagnosis of inflammatory granuloma of the cavernous sinus secondary to chronic otitis media was made. Hormone evaluation revealed tri-iodothyronine, thyroxine, thyroid-stimulating hormone and serum cortisol levels to be normal. Pure tone audiometry revealed moderate conductive hearing loss on the right side. Visual acuity and visual fields were normal. Serological tests for HIV-1 and -2 were negative. Soon after the MRI scan had been performed, the child developed high grade fever; clinical examination revealed the presence of neck rigidity and a positive Kernig’s sign. Lumbar puncture was performed; cerebrospinal fluid analysis was suggestive of menin-
Kumar/Madhugiri/Verma/Barathi/Yadav/ Bidkar
Color version available online
Lymphocytes
Caseous necrosis
Langhans giant cells Non-caseating epithelioid granuloma
a
Lymphocyte cuff
b
Fig. 2. Histopathology of the resected mass. Haematoxylin-eosin staining. a Both caseating and non-caseating granulomas are seen. Low power view. ×40. b The Langhans giant cells and the lymphocytic infiltrate are clearly
seen. High power view. ×100.
gitis (800 cells, predominantly polymorphs, protein 180 mg/dl and glucose 20 mg/dl). Cerebrospinal fluid cytology revealed no bacteria or abnormal tumour cells. She was administered antimeningitic doses of intravenous antibiotics (3rd-generation cephalosporin + aminoglycosides) as per the sensitivity pattern of the pus culture obtained from the ear discharge. Although her symptoms partially resolved after the institution of antibiotic therapy (reduction in ear discharge), she continued to have severe headache and her diplopia worsened. A fresh MRI of the brain (after 10 days of antibiotic therapy) revealed an increase in the size of the lesion. Surgical resection of the lesion was planned, with the dual intent of achieving a definitive diagnosis and reducing the patient’s symptoms (caused by raised intracranial pressure as well as direct involvement of neural structures by the lesion). A right frontotemporal craniotomy was made and an orbitozygomatic osteotomy was added. Via a temporopolar intradural approach, the outer wall of the cavernous sinus was opened. A fleshy, white, moderately vascular mass lesion with areas of pus collection within it was encountered. The 6th nerve was adherent to the lesion but could be preserved after careful dissection. Portions of the lesion densely adherent to the internal carotid artery were left behind. Postoperative recovery was uneventful, barring a mild worsening of 6th-nerve function. A postoperative contrast-enhanced CT scan of the brain revealed near total resection of the lesion. Histopathological examination of the resected lesion revealed caseating granulomas with Langhans’ giant cells and a chronic inflammatory infiltrate, diagnostic of a tubercular granuloma (fig. 2). ATT (with rifampicin, isoniazid, ethambutol and pyrazinamide) was instituted. Six weeks after surgery, MRI revealed a small residual lesion in the region of the petrous apex. Besides this residual lesion, several new lesions had appeared in the left cavernous sinus, right superior petrosal sinus and tentorial incisura (fig. 3). The patient, however, remained asymptomatic for these lesions and had developed no fresh neurological deficits. ATT was continued for the next 12 months and an MRI repeated at this time. The 12-month follow-up MRI demonstrated complete resolution of nearly all the lesions (fig. 4). As per our protocol, ATT will be administered for a total of 18 months.
Cavernous Sinus Tuberculoma
Fig. 3. Postoperative follow-up imaging, 6 weeks after surgery (un-
der ATT). Gd contrast-enhanced axial MRI image 3 months after surgery showing new lesions in the right superior petrous region and left cavernous sinus area.
Discussion
CNS TB constitutes about 0.5–10% of all CNS infections and is most often encountered in immunodeficient patients. Two common pathological forms of CNS TB include basal meningitis and granulomatous lesions (tuberculoma) [2]. Tuberculomas account for as much as 10–30% of all intracranial mass lesions in developing countries. In adults, they usually occur in the supratentorial space, either in a Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
371
Fig. 4. Twelve-month follow-up MRI, axial Gd-enhanced images, showing near total resolution of all lesions. a There is a very small area of enhancement posterior to the right petrous bone. b There is also a small residual area of enhancement in the right cavernous sinus. All other lesions have resolved.
a
subcortical location or in the basal ganglia [3]. In children, cerebellar lesions are commonly encountered [2]. Lesions at unusual sites such as the brain stem, ventricles, pituitary gland and the cerebellopontine angle have also been reported in the literature; most of these occur in adults [14–16]. CNS TB is almost always secondary; the usual primary site of infection is a pulmonary focus. In the present case, we presume that the portal of entry of infection was the infected middle ear cavity. The organism probably first infected the mastoid air cells, with subsequent spread of infection to the petrous bone and the cavernous sinus, either by contiguous spread or via venous channels. However, Mycobacterium tuberculosis could not be isolated in culture from the ear discharge. M. tuberculosis is a slowly replicating organism and is difficult to grow in culture; the presence of a superadded bacterial infection would make isolation of the organism even more difficult. The characteristics of the lesion on MRI (enhancing lesion with hypo-intense signals on T2-weighted images) were suggestive of an inflammatory granuloma; however, a definitive diagnosis of tuberculosis could not be made due to the unusual location as well as the initial delay in response to ATT. When tubercular granulomas occur at unusual sites in the skull base, a conclusive pre-operative diagnosis is difficult to establish, despite advances in neuro-imaging techniques [3, 17, 18]. This is emphasized by the fact that in nearly all published clinical reports about cavernous sinus TB, patients underwent surgery – either to achieve a definite tissue diagnosis or with an erroneous diagnosis of meningioma [6, 7, 9, 10, 13]. Tubercular granulomas have a characteristic appearance on histopathological examination, and a definitive 372
Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
b
diagnosis is possible on the basis of microscopic morphology in most cases. ATT is usually initiated on the basis of histopathological findings. Bacterial culture of M. tuberculosis may take several weeks to yield definite results and the bacilli are isolated in only 35% of resected granulomas. Hence, a negative bacterial culture does not preclude a diagnosis of TB [17, 18]. ATT should ideally be administered for 12–18 months, even if radiological resolution of the lesions is seen earlier [17]. However, the radiological response usually lags behind the clinical response to treatment. The appearance of new lesions on the 3-month follow-up MRI in the present case is an example of this. The reason for the appearance of new lesions could either be intra-operative dissemination of the infection (via the intercavernous sinus) or a paradoxical increase in the size of pre-existing lesions (as may occur during the intensive phase of ATT) [19]. Systemic steroids are generally administered during the initial weeks of ATT (especially in patients with tubercular meningitis) to prevent paradoxical enlargement of lesions [19].
Conclusion
TB should be considered in the differential diagnosis of cavernous sinus lesions in developing countries. A strong clinical suspicion may restrict surgery to only a biopsy rather than performing an extensive skull base approach and radial resection. ATT remains the mainstay of treatment and, in the majority of cases, can result in a cure and good neurological outcome. Kumar/Madhugiri/Verma/Barathi/Yadav/ Bidkar
References 1 Solovic I, Jonsson J, Korzeniewska-Koseła M, Chiotan DI, Pace-Asciak A, Slump E, Rumetshofer R, Abubakar I, Kos S, Svetina-Sorli P, Haas W, Bauer T, Sandgren A, van der Werf MJ: Challenges in diagnosing extrapulmonary tuberculosis in the European Union, 2011. Euro Surveill 2013;18:pii:20432. 2 Arvind C, Korath PM, Raveendranadhan K, Jagadeesan K: A retrospective study of 1,247 cases of intracranial tuberculomata diagnosed by computerized tomography. J Assoc Phys Ind 1993;41:559–561. 3 Gupta RK, Jena A, Singh AK, Sharma A, Puri V, Gupta M: Role of magnetic resonance (MR) in the diagnosis and management of intracranial tuberculomas. Clin Radiol 1990;41: 120–127. 4 Al Soub H, Al Alousi FS, Al-Khal AL: Tuberculoma of the cavernous sinus. Scand J Infect Dis 2001;33:868–870. 5 Bafna S, Lee AG: Presumed tuberculosis presenting as a cavernous sinus syndrome. J Neuroophthalmol 1997;17:207–208. 6 Boutarbouch M, Arkha Y, Gana R, El Maquili MR, Bellakhdar F: Tuberculoma of the cavernous sinus mimicking a meningioma: case report and review of the literature. J Neurol Sci 2009;278:123–126.
Cavernous Sinus Tuberculoma
7 Grayeli AB, Redondo A, Salama J, Rey A: Tuberculoma of the cavernous sinus: case report. Neurosurgery 1998;42:179–181. 8 Hui AC, Wong WS, Wong KS: Cavernous sinus syndrome secondary to tuberculous meningitis. Eur Neurol 2002;47:125–126. 9 Jaimovich SG, Thea VC, Guevara M, Gardella JL: Cavernous sinus tuberculoma mimicking a neoplasm: case report, literature review, and diagnostic and treatment suggestions for tuberculomas in rare locations. Surg Neurol Int 2013;4:158. 10 Morris JT, Joyce MP: Central nervous system tuberculoma presenting as a cavernous sinus tumor. Clin Infect Dis 1992;15:181–182. 11 Phookan G, Towns GM: Tuberculoma of the cavernous sinus – a case report. Br J Neurosurg 1995;9:205–207. 12 Rebai R, Boudawara MZ, Bahloul K, Chabchoub I, Chaari S, Boudawara T, Ben Mansour H: Cavernous sinus tuberculoma: diagnostic difficulties in a personal case. Surg Neurol 2001;55:372–375. 13 Yanardag H, Uygun S, Yumuk V, Caner M, Canbaz B: Cerebral tuberculosis mimicking intracranial tumour. Singapore Med J 2005; 46:731–733.
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Pediatr Neurosurg 2013;49:369–373 DOI: 10.1159/000369030
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