Bilateral ocular scrofuloderma with orbital tuberculosis
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《美国医学杂志》
1 Department of Pediatrics Pathology, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
2 Department of Dermatology, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
Abstract
Ocular scrofuloderma with orbital tuberculosis is a rarely described presentation of childhood tuberculosis. Bilateral involvement has not been reported earlier in the medical literature. Here is reported a 3-year-old boy who presented with bilateral infraorbital swellings of tubercular etiology. Computed tomography (CT) scan of the upper face revealed enhancing soft tissue lesions in both the lower lids of the eyes, with extraconal extension into the orbits and with erosion of the right zygomatic bone. Tubercular etiology was confirmed by the Ziehl Neelsen staining of the aspirate from the lesion, which was positive for acid-fast bacilli and growth of Mycobacterium tuberculosis in the aspirate culture. The patient showed marked improvement of his lesions on anti-tubercular treatment.
Keywords: Ocular scrofuloderma; Orbital tuberculosis
Tuberculosis, one of the oldest diseases known to mankind continues to be a significant problem even as we have entered the next millennium. Far from being eradicated, conclusive evidence exists to suggest the presence of a global pandemic.[1] Extrapulmonary tuberculosis constitutes around 10% of all cases of tuberculosis.[2] Ocular scrofuloderma with orbital involvement is a rarely described presentation of childhood tuberculosis.[3] Bilateral involvement is distinctly uncommon. We are unaware of any previously reported case and a computerized literature search using MEDLINE disclosed no other examples. This paper presents the case of a 3-year-old boy who presented with bilateral infraorbital swellings of tubercular etiology.
Case report
A 3-year-old unimmunized boy presented with a history of painless swellings beneath both the eyes (initially involving the left side and 3 months later, the right); noticed to be gradually increasing over the last 9 months. There was no history of fever, cough, redness of eyes, deterioration of vision or swellings elsewhere. There was no history of pallor, bleeding from any site, or presence of lump in the abdomen. The swelling on the right side had ulcerated at the time of presentation. The parents complained that the child had a reduced appetite and had not gained weight for the last 9 months. The child's aunt was diagnosed as a case of pulmonary tuberculosis 1 year back, and had received anti-tubercular treatment.
Examination revealed an afebrile playful child who weighed 10 Kg and had a height of 87 cm. The right infraorbital region had a nodulo-ulcerative lesion, 3 X 2 cm with central ulceration and crusting Figure1a. The lesion also had multiple discharging sinuses with sero-sanguineous discharge and bluish undermined edges. The left eye demonstrated a prominent infraorbital swelling (3 × 3 cm), with erythematous taut skin but no ulceration Figure1a. There was no significant lymphadenopathy. Ophthalmological examination revealed bilaterally normal visual acuity, anterior segment and fundus. Ocular movements were full. Systemic examination revealed no abnormalities.
Investigations revealed a normal hemogram, liver, and renal function tests. The ESR was raised (75 mm in the first hour). Chest X-ray and ultrasonography of the abdomen revealed no abnormalities. The tuberculin test with 5 TU was strongly reactive (20 mm). Computed tomography (CT) scan of the upper face revealed mild enhancing soft tissue swellings in infraorbital regions bilaterally, with extraconal extension into orbits Figure2a and erosion of the anterior part of the zygomatic area Figure2b. The fine needle aspiration cytology (FNAC) from the swelling on the left side revealed numerous epitheleioid cell granulomas, with mononuclear cell infiltrate and scattered giant cells. Ziehl-Neelsen staining of the aspirate revealed acid fast bacilli (AFB). Culture from the aspirate demonstrated growth of Mycobacterium tuberculosis after 8 weeks.
A diagnosis of bilateral ocular scrofuloderma with tubercular extraconal intraorbital involvement was made and the patient was stated on anti-tubercular treatment with an initial intensive phase with four drugs: isoniazid, rifampicin, ethambutol and pyrazinamide. Lesions on the face showed substantial clearing and the sinuses healed within a month of starting treatment Figure1b. The child also started gaining weight. After two months, the patient was shifted to continuation phase with two drugs: isoniazid and rifampicin, which were continued for 4 months.
Discussion
Scrofuloderma, a form of reactivation tuberculosis, results from tubercular process in the lymph nodes, bones, joints and/or the subcutaneous tissues, with formation of cold abscesses and a secondary breakdown of the overlying skin.[4] Periocular tuberculosis is rare, and scrofuloderma involving the region is even more uncommon.[5],[6],[7] Sardana et al have reported one case of ocular scrofuloderma with orbital tuberculosis presenting as proptosis[3], but no case of bilateral involvement has been reported so far.
Orbital involvement in tuberculosis is a rare manifestation. Less than 40 cases have been described so far. Tubercular involvement of the eye in India, where the disease is endemic, is common; but orbital lesions are extremely rare. Agrawal et al have reported 14 cases of orbital involvement in tuberculosis from India,[8] however none of the cases had scrofuloderma, bone erosion or orbital involvement combined in a single case.
Orbital tuberculosis occurs by hematogenous spread or by extension from adjacent structures.[9] In this case, in view of the bilateral presentation, the likely route was hematogenous. However, despite investigation, we could not find a primary focus of tuberculosis. The disease is usually slow-growing, chronic and unilateral.[10] It is more commonly seen in children, girls being more susceptible than boys.[11] The response to anti-tubercular treatment alone as a primary therapy is usually good, but some authors have recommended wide surgical removal of all diseased tissue, combined with anti-tubercular treatment.[11],[12],[13]
The clinical differential diagnosis of bilateral infra-orbital swellings in pediatric age-group includes secondaries from neuroblastoma, nephroblastoma, Ewing's sarcoma and leukemic infiltrates. Radiologically the presence of enhancing soft tissue masses in both the orbits also led to the consideration of secondaries from a malignancy as the most likely diagnosis. In this case, tuberculosis was confirmed by the presence of acid fast bacilli on direct smear of the aspirate from the lesion, and a positive culture for Mycobacterium tuberculosis . Supportive evidence included a strongly reactive tuberculin test, presence of epithelioid granulomas on the aspirate, and the family history of tuberculosis. The diagnosis was re-affirmed by the dramatic response to anti-tubercular treatment. This case underscores the role of FNAC in such cases where there is a clinical and radiological diagnostic dilemma. FNAC not only suggested the diagnosis in this case but also provided material for culture.
This case emphasizes the importance of active vigilance of unusual associations and presentations of tuberculosis, especially in developing countries, where this disease is still rampant.
References
1. Bannon MJ. BCG and tuberculosis. Arch Dis Child 1999; 80: 80-83.
2. Grosset JH. Present status of chemotherapy for tuberculosis. Rev Infect Dis 1989; 11: 347-352.
3. Sardana K, Koranne RV, Langan U et al. J Dermatol 2002; 29: 232-234.
4. Sehgal VN, Wagh SA. Cutaneous tuberculosis: current concepts. Int J Dermatol 1990; 29: 237-252.
5. Sehgal VN, Shrivastav G, Khurana VK et al. An appraisal of epidemiological, clinical, bacteriological, histopathologic, and immunologic parameters in cutaneous tuberculosis. Int J Dermatol 1987; 26: 521-526.
6. Kakakhel KU, Fritsch P. Cutaneous tuberculosis. Int J Dermatol 1989; 28: 355-362.
7. Tur E, Brenner S, Meiron Y. Scrofuloderma (tuberculosis colliquativa cutis). Br J Dermatol 1996; 134: 350-352.
8. Agrawal PK, Nath J, Jain BS. Orbital involvement in tuberculosis. Ind J Ophthalmol 1977; 25: 12-16.
9. Patkar S, Singhania BK, Agrawal A. Intraorbital extraocular tuberculosis: a report of three cases. Surg Neurol 1994; 42: 320-321.
10. Helm CJ, Holland GN. Ocular tuberculosis. Surv Ophthalmol 1993; 38: 229-256.
11. Sen DK. Tuberculosis of the orbit and lacrimal gland: a clinical study of 14 cases. J Pediatr Ophthalmol Strabismus 1980; 17 : 232-238.
12. Pillai S, Malone TJ, Abad JC. Orbital tuberculosis. Ophthal Plast Reconstr Surg 1995; 11: 27-31.
13. Aggarwal D, Suri A, Mahapatra AK. Orbital tuberculosis with abscess. J Neuro-Ophthalmol 2002; 22: 208-210.(Sharma Suvasini, Juneja M)
2 Department of Dermatology, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
Abstract
Ocular scrofuloderma with orbital tuberculosis is a rarely described presentation of childhood tuberculosis. Bilateral involvement has not been reported earlier in the medical literature. Here is reported a 3-year-old boy who presented with bilateral infraorbital swellings of tubercular etiology. Computed tomography (CT) scan of the upper face revealed enhancing soft tissue lesions in both the lower lids of the eyes, with extraconal extension into the orbits and with erosion of the right zygomatic bone. Tubercular etiology was confirmed by the Ziehl Neelsen staining of the aspirate from the lesion, which was positive for acid-fast bacilli and growth of Mycobacterium tuberculosis in the aspirate culture. The patient showed marked improvement of his lesions on anti-tubercular treatment.
Keywords: Ocular scrofuloderma; Orbital tuberculosis
Tuberculosis, one of the oldest diseases known to mankind continues to be a significant problem even as we have entered the next millennium. Far from being eradicated, conclusive evidence exists to suggest the presence of a global pandemic.[1] Extrapulmonary tuberculosis constitutes around 10% of all cases of tuberculosis.[2] Ocular scrofuloderma with orbital involvement is a rarely described presentation of childhood tuberculosis.[3] Bilateral involvement is distinctly uncommon. We are unaware of any previously reported case and a computerized literature search using MEDLINE disclosed no other examples. This paper presents the case of a 3-year-old boy who presented with bilateral infraorbital swellings of tubercular etiology.
Case report
A 3-year-old unimmunized boy presented with a history of painless swellings beneath both the eyes (initially involving the left side and 3 months later, the right); noticed to be gradually increasing over the last 9 months. There was no history of fever, cough, redness of eyes, deterioration of vision or swellings elsewhere. There was no history of pallor, bleeding from any site, or presence of lump in the abdomen. The swelling on the right side had ulcerated at the time of presentation. The parents complained that the child had a reduced appetite and had not gained weight for the last 9 months. The child's aunt was diagnosed as a case of pulmonary tuberculosis 1 year back, and had received anti-tubercular treatment.
Examination revealed an afebrile playful child who weighed 10 Kg and had a height of 87 cm. The right infraorbital region had a nodulo-ulcerative lesion, 3 X 2 cm with central ulceration and crusting Figure1a. The lesion also had multiple discharging sinuses with sero-sanguineous discharge and bluish undermined edges. The left eye demonstrated a prominent infraorbital swelling (3 × 3 cm), with erythematous taut skin but no ulceration Figure1a. There was no significant lymphadenopathy. Ophthalmological examination revealed bilaterally normal visual acuity, anterior segment and fundus. Ocular movements were full. Systemic examination revealed no abnormalities.
Investigations revealed a normal hemogram, liver, and renal function tests. The ESR was raised (75 mm in the first hour). Chest X-ray and ultrasonography of the abdomen revealed no abnormalities. The tuberculin test with 5 TU was strongly reactive (20 mm). Computed tomography (CT) scan of the upper face revealed mild enhancing soft tissue swellings in infraorbital regions bilaterally, with extraconal extension into orbits Figure2a and erosion of the anterior part of the zygomatic area Figure2b. The fine needle aspiration cytology (FNAC) from the swelling on the left side revealed numerous epitheleioid cell granulomas, with mononuclear cell infiltrate and scattered giant cells. Ziehl-Neelsen staining of the aspirate revealed acid fast bacilli (AFB). Culture from the aspirate demonstrated growth of Mycobacterium tuberculosis after 8 weeks.
A diagnosis of bilateral ocular scrofuloderma with tubercular extraconal intraorbital involvement was made and the patient was stated on anti-tubercular treatment with an initial intensive phase with four drugs: isoniazid, rifampicin, ethambutol and pyrazinamide. Lesions on the face showed substantial clearing and the sinuses healed within a month of starting treatment Figure1b. The child also started gaining weight. After two months, the patient was shifted to continuation phase with two drugs: isoniazid and rifampicin, which were continued for 4 months.
Discussion
Scrofuloderma, a form of reactivation tuberculosis, results from tubercular process in the lymph nodes, bones, joints and/or the subcutaneous tissues, with formation of cold abscesses and a secondary breakdown of the overlying skin.[4] Periocular tuberculosis is rare, and scrofuloderma involving the region is even more uncommon.[5],[6],[7] Sardana et al have reported one case of ocular scrofuloderma with orbital tuberculosis presenting as proptosis[3], but no case of bilateral involvement has been reported so far.
Orbital involvement in tuberculosis is a rare manifestation. Less than 40 cases have been described so far. Tubercular involvement of the eye in India, where the disease is endemic, is common; but orbital lesions are extremely rare. Agrawal et al have reported 14 cases of orbital involvement in tuberculosis from India,[8] however none of the cases had scrofuloderma, bone erosion or orbital involvement combined in a single case.
Orbital tuberculosis occurs by hematogenous spread or by extension from adjacent structures.[9] In this case, in view of the bilateral presentation, the likely route was hematogenous. However, despite investigation, we could not find a primary focus of tuberculosis. The disease is usually slow-growing, chronic and unilateral.[10] It is more commonly seen in children, girls being more susceptible than boys.[11] The response to anti-tubercular treatment alone as a primary therapy is usually good, but some authors have recommended wide surgical removal of all diseased tissue, combined with anti-tubercular treatment.[11],[12],[13]
The clinical differential diagnosis of bilateral infra-orbital swellings in pediatric age-group includes secondaries from neuroblastoma, nephroblastoma, Ewing's sarcoma and leukemic infiltrates. Radiologically the presence of enhancing soft tissue masses in both the orbits also led to the consideration of secondaries from a malignancy as the most likely diagnosis. In this case, tuberculosis was confirmed by the presence of acid fast bacilli on direct smear of the aspirate from the lesion, and a positive culture for Mycobacterium tuberculosis . Supportive evidence included a strongly reactive tuberculin test, presence of epithelioid granulomas on the aspirate, and the family history of tuberculosis. The diagnosis was re-affirmed by the dramatic response to anti-tubercular treatment. This case underscores the role of FNAC in such cases where there is a clinical and radiological diagnostic dilemma. FNAC not only suggested the diagnosis in this case but also provided material for culture.
This case emphasizes the importance of active vigilance of unusual associations and presentations of tuberculosis, especially in developing countries, where this disease is still rampant.
References
1. Bannon MJ. BCG and tuberculosis. Arch Dis Child 1999; 80: 80-83.
2. Grosset JH. Present status of chemotherapy for tuberculosis. Rev Infect Dis 1989; 11: 347-352.
3. Sardana K, Koranne RV, Langan U et al. J Dermatol 2002; 29: 232-234.
4. Sehgal VN, Wagh SA. Cutaneous tuberculosis: current concepts. Int J Dermatol 1990; 29: 237-252.
5. Sehgal VN, Shrivastav G, Khurana VK et al. An appraisal of epidemiological, clinical, bacteriological, histopathologic, and immunologic parameters in cutaneous tuberculosis. Int J Dermatol 1987; 26: 521-526.
6. Kakakhel KU, Fritsch P. Cutaneous tuberculosis. Int J Dermatol 1989; 28: 355-362.
7. Tur E, Brenner S, Meiron Y. Scrofuloderma (tuberculosis colliquativa cutis). Br J Dermatol 1996; 134: 350-352.
8. Agrawal PK, Nath J, Jain BS. Orbital involvement in tuberculosis. Ind J Ophthalmol 1977; 25: 12-16.
9. Patkar S, Singhania BK, Agrawal A. Intraorbital extraocular tuberculosis: a report of three cases. Surg Neurol 1994; 42: 320-321.
10. Helm CJ, Holland GN. Ocular tuberculosis. Surv Ophthalmol 1993; 38: 229-256.
11. Sen DK. Tuberculosis of the orbit and lacrimal gland: a clinical study of 14 cases. J Pediatr Ophthalmol Strabismus 1980; 17 : 232-238.
12. Pillai S, Malone TJ, Abad JC. Orbital tuberculosis. Ophthal Plast Reconstr Surg 1995; 11: 27-31.
13. Aggarwal D, Suri A, Mahapatra AK. Orbital tuberculosis with abscess. J Neuro-Ophthalmol 2002; 22: 208-210.(Sharma Suvasini, Juneja M)