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The Central nervous system can be affected in extrapulmonary tuberculosis. The intracranial tuberculoma occurrence is a rare entity occurrence; most often occur in the form of discrete small multiple lesions. Rarely a large sized tuberculoma is observed with mass effects and usually managed surgically to provide rapid relief of mass effect, histopathological confirmation of diagnosis, reduction of pathological mass thereby increasing efficacy of medical therapy. Author reports an extremely rare case of giant tuberculoma occurring in a pediatric patient, which was managed with antituberculous medication along with cerebral decongestant. The patient was also advised surgical therapy in view of giant size of tuberculoma associated with significant mass effect; however, the parents were unwilling for any form of surgical intervention and finally choose to continue medical treatment alone. After 6 months of antitubercular medical therapy, magnetic resonance imaging brain showed completely vanishing of lesion and also amelioration of mass effect with marked subsidence of perilesional edema correlated very well with marked improvement in the clinical status. He received antituberculous therapy for 24 months. To the best knowledge of authors, the current case represents first case of its kind in the pediatric age group with giant intracerebral tuberculoma responding favorably with medication. Management of such rare case and pertinent literature is reviewed briefly.
Tuberculous involvement of the central nervous system constitutes a major health issue in the developing countries including India. However, with surge of HIV epidemic, further rise in the tuberculosis incidence is on the rising horizon across the globe including the developed world. Intracranial tuberculous affection can present as meningitis, tuberculous abscess, encephalitis, obstructive hydrocephalus secondary to meningitis, and rarely intraparenchymal cerebral tuberculomas. The central nervous system tuberculoma usually reported to take the form of discrete multiple smaller lesions, rarely multiple tuberculomas may coalesces together to form giant size mass and producing severe mass effect and focal neurological deficits. However, only a few cases of large sized tuberculomas are reported in literature mostly are in the form of isolated case reports. Authors could find only a few cases of large tuberculoma in detailed PubMed and Medline search.[1,2] Giant tuberculoma is usually mistaken for other intracranial mass lesion including the malignant pathologies and taken up for surgery to find out to confirm the histopathological diagnosis as well to reduce the mass effect and reduce raised intracranial pressure. However, management of giant tuberculoma is still debated,[3,4] and few authors advocate surgical resection of giant tuberculoma.
An 11-year-old boy presented with complaints of repeated episodes of focal seizures involving the face for the last 1 month. He also complained of progressive headache associated with repeated episodes of vomiting. On examination, he was alert, fundi revealed bilateral presence of papilledema, with left sided upper motor neuron seventh cranial nerve paresis associated with left sided spastic hemiparesis.
Magnetic resonance imaging (MRI) brain revealed the presence of an extra-giant mass lesion, measuring of 9 cm × 8.6 cm in the right frontotemporal parietal region with significant mass effect, center of lesion showed hypointense core on T2-weighted image, showing multiple conglomerated lesions with central hypodensity with peripheral edema. On gadolinium contrast administration, showing heterogeneously enhancement [Figures [Figures11–3] and but the radiological features were still doubtful [Figure 4] and intracranial tumor was suspected with one of the differentials was tubercular lesion. He was started on antituberculous medication, dexamethasone and cerebral decongestant along with antiepileptic medication. The parents were advised urgent surgical intervention in view of the presence of sever mass effect, however, his parents flatly refused any kind of surgical intervention. Fortunately, child showed clinical improvement in headache and reduced the frequency of seizure with antituberculous medication, and during follow-up at 6 months of therapy; MRI brain showed complete disappearance of tuberculoma with marked reduction in perilesional edema, [Figures [Figures55–7] and received antitubercular medication for 24 months. At last follow-up at 3 years, he was doing well with recovery of seventh nerve paresis and hemiparesis.
Tuberculosis remains a major health burden worldwide, and India has been classified as Group IV country with highest annual risk of tuberculosis infection. In India, estimated 2.3 million cases of tuberculosis occurred in the year 2011.[6,7] However, the global incidence of estimated to be about 9 million. Involvement of central nervous system takes place in approximately 10% of all cases suffering with tuberculosis. And tends to involve primarily at the younger age group.
Intracranial tuberculosis has a spectrum of presentations including meningitis, miliary tuberculosis associated with tuberculous meningitis, encephalopathy, tuberculous arteriopathy, and other rarer forms includes space-occupying mass lesions, i.e., isolated or multiple tuberculoma, discrete multiple small tuberculoma, miliary tuberculosis, and tuberculous abscess.
Rich and McCordock suggested tuberculosis of CNS progresses though two distinct phases occurs in two stages. The first stage being the smaller tubercular lesions also known as Rich's foci usually develop during primary bacteremia or shortly afterward and can be located in the meninges, subpial or subependymal surface of the brain or the spinal cord. Rich's foci may remain in the dormant stages for many years. The second stage is characterized by progressive growth, or rupture Rich's foci leads to progression of the disease and responsible for the final manifestation of the central nervous system tuberculosis.[12,13] Tuberculoma is formed as progressive enlargement of Rich foci within the intracerebral parenchyma.
The clinical feature of intracranial intracerebral tuberculoma depends on location, size, mass effect, degree of presence and extent of associated edema, hydrocephalus, infarct, and response to antituberculous therapy. Infratentorial tuberculoma presents with features of cerebellar dysfunction, multiple cranial nerve palsies or brain stem compression while the supratentorial counterpart present with vomiting, headache low-grade fever, and seizures or focal neurological deficit.[1,15,16]
The appearance of tuberculoma on MRI imaging depend on rim, and content, which can be classified into three groups, first being noncaseating lesion, others are caseating variety with central solid component and caseation with completely liquid content.[17,18] The noncaseating tuberculoma commonly demonstrates hypointense signal on T1-weighted images, becoming hyperintense on T2 images and characteristically shows homogenous nodular enhancement on contrast study. While, caseating granuloma with solid center shows hypointense to isointense on T1 image and typical hypointense on T2 images and only shows peripheral rim contrast enhancement, while final group with central liquid counterpart shown as hypointense on T1 image and hyperintense on T2-weighted image with peripheral hypointense rim, and rim gets enhanced on contrast study. A variable degree of vasogenic edema usually accompany the lesion and is relatively more prominent in the early stages.
Management of intracranial tuberculosis is challenging as there is absence of consensus regarding protocol of treatment. Although surgery is advocated as main modality and includes craniotomy and excision of tuberculoma.
In the published literature, most of the giant tuberculomas were managed surgically in children as well adults.[4,20,21,22,23,24] Raheja et al. reported surgically managed, a 7-month-old child with supratentorial intracerebral tuberculoma. Giese et al. also reported a case managed with surgical excised located in the frontal region and protruding transcalvarial.
Kumar and Prasad also managed a 4-year boy with 8 cm × 10 cm large tuberculoma, located in the right temporo-parieto-occipital lesion showed paradoxical response following initiation of medication and managed surgically. Akhaddar and Boucetta reported a 6-year boy with left frontotemporal parietal tuberculoma managed with surgical excision. Further, in elderly group, surgical management is still preferred for giant tuberculoma, Tena-Suck et al. managed a 66-year female, who underwent mastectomy for breast carcinoma about 10 years back developed occipital tuberculoma, underwent surgical excision Sumer et al. also managed giant tuberculoma developing in a 66-year female with surgical excision.
Authors advocated only medical treatment and also reported good outcome following medical therapy for tuberculoma, however, current case had extra-giant tuberculoma possessing a dilemma about selection of a best treatment modalities either surgical or medical, however, refusal of surgical treatment by parents left only medical treatment, so a selected case may be provided with opportunity of medical therapy. Although a possibility of surgical option should always be considered before deciding to medical therapy as a sole treatment modality, and even during the course of medical therapy, patients may need urgent surgical intervention as in cases with paradoxical response to antituberculous medication. So surgical management, also remains a viable alternative in cases of failure of medical therapy, development of multidrug resistance, poor drug compliance, progressively increasing size of tuberculoma as paradoxical response to medical therapy, increasing sizes of hydrocephalus, increasing mass effect due to progression of the perilesional edema or progressive worsening of neurological deficit in form of focal deficit or progressively rising intracranial pressure.
Our case was managed without any surgical intervention. However, a very few selected cases may be given a trial of medical treatment and keeping surgical option as standby procedure as and when required or warranted and may be required as the emergent basis also.
In the developing countries, tuberculosis is still endemic. Tuberculomas should be kept as differentials and selected cases may be given a trial of antituberculous therapy with close observation and some may respond very well. However, as a caution surgical management option should also be considered and made available on an emergent basis in the event of failure of medical therapy or development of multidrug resistance, compliance of drug is poor, progressively increasing size of tuberculoma as paradoxical response to medical therapy or developing hydrocephalus, or increasing mass effect due to progressive perilesional edema or progressive worsening of neurological deficit in form of focal deficit or progressively rising intracranial pressure.
There are no conflicts of interest.