Letters to Editor
Yet another pulmonary manifestation of tuberous sclerosis Sir, We read a recent article titled “Sudden onset of dyspnea in a woman with skin lesions and lung cysts”[1] in pictorial quiz section of your esteemed journal with great interest. The authors have described lymphangioleiomyomatosis causing pneumothorax in a female with tuberous sclerosis. We would like to present yet another unusual pulmonary manifestation of tuberous sclerosis. A 52-year-old female patient presented to the casualty department with complaints of severe pain in the right lumbar region for the duration of two days. Imaging (ultrasound followed by computed tomography) demonstrated the presence of bilateral renal angiomyolipomas [Figure 1] with intratumoral bleed on the right side. General physical examination revealed the presence of adenoma sebaceum on the face [Figure 2]. Computed tomography of the brain showed multiple calcified foci at the gray-white matter junction and periventricular region.
The patient also complained of dry cough with breathlessness for a period of two years. The respiratory system examination demonstrated the presence of bilateral crepts. Radiograph of chest [Figure 3] and computed tomography [Figure 4] demonstrated the presence of multiple nodules in bilateral lung fields. The nodules were well defined, randomly distributed with peripheral and upper lobe predominance. Few small thin walled cysts were seen in the right upper lobe. Calcified nodules were seen in the bilateral upper lobes likely representing the sequela of the old infection. The presence of bilateral renal angiomyolipomas and adenoma sebaceum was diagnostic of tuberous sclerosis complex (TSC). The imaging findings of the lung in a patient with TSC were consistent with multifocal micronodular pneumocyte hyperplasia (MMPH). Until recently, lymphangioleiomyomatosis (LAM), a hamartomatous cystic lung disease, was the only reported pulmonary radiologic manifestation of TSC. This represents a hamartomatous cystic lung disease, observed
Figure 1: Coronal noncontrast computed tomography scan of abdomen: Bilateral kidneys (arrows) are replaced by multiple fat containing masses suggestive of angiomyolipomas
Figure 2: Adenoma sebaceum: Multiple erythematous papules on the nose and cheek representing angiofibromata
Figure 3: Radiograph of chest in anteroposterior projection: Multiple nodules are seen in bilateral lung fields. Ill-defined calcified opacities are seen in bilateral upper zones
Figure 4: Axial computed tomography image in the lung window at the level of carina: Multiple, small, randomly distributed, well defined nodules in bilateral lung fields. These nodules show peripheral and upper lobe predominance
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Lung India • Vol 31 • Issue 2 • Apr - Jun 2014
Letters to Editor
in 1-2.3% of TSC patients.[2] MMPH is an extremely rare pulmonary manifestation of tuberous sclerosis complex. It was first described by Popper et al. in 1991.[3] Less than 40 cases have been reported in the literature till date. This condition is seen in both males and females with equal frequency. It represents benign hamartomatous proliferations of type II pneumocytes along with alveolar septa and fibrous thickening, increased elastic fibres and aggregates of alveolar macrophages. The clinical manifestation of isolated MMPH includes dyspnoea, cough and mild to moderate hypoxemia. The clinical course in non progressive and treatment is not required. These are usually benign with no malignant potential.[4]
Satija Bhawna, Kumar Sanyal1 Department of Radiology, Delhi State Cancer Institutes, New Delhi,1Employees State Insurance Hospital and Post Graduate Institute of Medical Science and Research, Basaidarapur, New Delhi, India E-mail: [email protected]
REFERENCES 1. 2.
3.
MMPH occurs more frequently in patients with TSC than with LAM and usually not seen in absence of both. It manifests as multiple, diffuse pulmonary nodules on the chest roentgenogram. High resolution computed tomography (HRCT) demonstrates nodules 1-8 mm in diameter that are diffusely scattered. They show random distribution with peripheral and upper lobe predominance.[5] The radiological differentials include military granulomatous infections, langerhans cell histiocytosis and hematogenous metastasis. In summary, the present case is presented to familiarize the clinicians with a rather unusual pulmonary manifestation of tuberous sclerosis.
4. 5.
Singh A, Singh J. Sudden onset of dyspnea in a woman with skin lesions and lung cysts. Lung India 2013;30:164-5. Maruyama H, Ohbayashi C, Hino O, Tsutsumi M, Konishi Y. Pathogenesis ofmultifocal micronodularpneumocyte hyperplasia and lymphangioleiomyomatosisin tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2. PatholInt2001;51:585-94. Popper HH, Juettner-Smolle FM, Pongratz MG. Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis. Histopathology 1991;18:347-54. Muir TE, Leslie KO, Popper H, Kitaichi M, Gagné E,Emelin JK,et al. Micronodularpneumocyte hyperplasia. Am JSurgPathol 1998;22:465-72. Nagar AM, Teh HS, Khoo RN, Morani AC, Vrishni K, Raghuram J. Multifocal pneumocyte hyperplasia in tuberous sclerosis. Thorax 2008;63:186.
Access this article online Quick Response Code:
Website: www.lungindia.com DOI: 10.4103/0970-2113.129900
Recurrent subcutaneous emphysema in a treated pulmonary tuberculosis patient: Is there any association? Sir, A 55-year old nondiabetic, nonhypertensive, and nonsmoker female presented with dyspnoea for 1 month and swelling of face, neck, upper limb, and trunk for 2 weeks. Dyspnoea was gradual in onset, progressive, causing limitation of her daily activities. There was no history of palpitation, orthopnoea, paroxysmal nocturnal dyspnoea, and edema, or chest pain. The swelling started slowly, involving the neck and gradually involved face causing periorbital swelling and later involved upper part of trunk and upper limbs [Figure 1]. The patient had a past history of tuberculosis for which she was treated completely and declared cured 5 years ago. Examination revealed swelling of face, neck, upper limbs with crepitus all over. Trachea was shifted toward the right side. Chest examination revealed harsh vesicular breath sounds with scattered coarse crepitations. An urgent chest x-ray was done that showed subcutaneous emphysema without any evidence of pneumothorax. Areas of fibrosis were
noted in bilateral lung parenchyma. The patient was given high flow oxygen at the rate of 10 L/min, superficial surgical incisions were given at the level of thoracic inlet for the subcutaneous emphysema, but without of much benefit. CT scan of thorax showed gross bilateral subcutaneous emphysema, pneumomediastinum, minimal pneumothorax, and fibrotic changes in bilateral lung parenchyma [Figure 2]. In due course of time, other investigations were done that showed a negative mantoux test and her sputum for AFB was negative on two occasions. As initial management failed to improve patient condition, 20 F intercostal chest drainage tubes were put bilaterally under local anesthesia after consulting with chest medicine department and cardiothoracic surgery department. Two days after placement of intercostal chest drain the patient showed dramatic improvement in the form of decrease in the periorbital puffiness with generalized decrease in the amount of swelling. The chest drains were removed after one week. Consequently subcutaneous emphysema reappeared and gradually progressed in the same pattern
Lung India • Vol 31 • Issue 2 • Apr - Jun 2014 197
Copyright of Lung India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Yet another pulmonary manifestation of tuberous sclerosis Sir, We read a recent article titled “Sudden onset of dyspnea in a woman with skin lesions and lung cysts”[1] in pictorial quiz section of your esteemed journal with great interest. The authors have described lymphangioleiomyomatosis causing pneumothorax in a female with tuberous sclerosis. We would like to present yet another unusual pulmonary manifestation of tuberous sclerosis. A 52-year-old female patient presented to the casualty department with complaints of severe pain in the right lumbar region for the duration of two days. Imaging (ultrasound followed by computed tomography) demonstrated the presence of bilateral renal angiomyolipomas [Figure 1] with intratumoral bleed on the right side. General physical examination revealed the presence of adenoma sebaceum on the face [Figure 2]. Computed tomography of the brain showed multiple calcified foci at the gray-white matter junction and periventricular region.
The patient also complained of dry cough with breathlessness for a period of two years. The respiratory system examination demonstrated the presence of bilateral crepts. Radiograph of chest [Figure 3] and computed tomography [Figure 4] demonstrated the presence of multiple nodules in bilateral lung fields. The nodules were well defined, randomly distributed with peripheral and upper lobe predominance. Few small thin walled cysts were seen in the right upper lobe. Calcified nodules were seen in the bilateral upper lobes likely representing the sequela of the old infection. The presence of bilateral renal angiomyolipomas and adenoma sebaceum was diagnostic of tuberous sclerosis complex (TSC). The imaging findings of the lung in a patient with TSC were consistent with multifocal micronodular pneumocyte hyperplasia (MMPH). Until recently, lymphangioleiomyomatosis (LAM), a hamartomatous cystic lung disease, was the only reported pulmonary radiologic manifestation of TSC. This represents a hamartomatous cystic lung disease, observed
Figure 1: Coronal noncontrast computed tomography scan of abdomen: Bilateral kidneys (arrows) are replaced by multiple fat containing masses suggestive of angiomyolipomas
Figure 2: Adenoma sebaceum: Multiple erythematous papules on the nose and cheek representing angiofibromata
Figure 3: Radiograph of chest in anteroposterior projection: Multiple nodules are seen in bilateral lung fields. Ill-defined calcified opacities are seen in bilateral upper zones
Figure 4: Axial computed tomography image in the lung window at the level of carina: Multiple, small, randomly distributed, well defined nodules in bilateral lung fields. These nodules show peripheral and upper lobe predominance
196
Lung India • Vol 31 • Issue 2 • Apr - Jun 2014
Letters to Editor
in 1-2.3% of TSC patients.[2] MMPH is an extremely rare pulmonary manifestation of tuberous sclerosis complex. It was first described by Popper et al. in 1991.[3] Less than 40 cases have been reported in the literature till date. This condition is seen in both males and females with equal frequency. It represents benign hamartomatous proliferations of type II pneumocytes along with alveolar septa and fibrous thickening, increased elastic fibres and aggregates of alveolar macrophages. The clinical manifestation of isolated MMPH includes dyspnoea, cough and mild to moderate hypoxemia. The clinical course in non progressive and treatment is not required. These are usually benign with no malignant potential.[4]
Satija Bhawna, Kumar Sanyal1 Department of Radiology, Delhi State Cancer Institutes, New Delhi,1Employees State Insurance Hospital and Post Graduate Institute of Medical Science and Research, Basaidarapur, New Delhi, India E-mail: [email protected]
REFERENCES 1. 2.
3.
MMPH occurs more frequently in patients with TSC than with LAM and usually not seen in absence of both. It manifests as multiple, diffuse pulmonary nodules on the chest roentgenogram. High resolution computed tomography (HRCT) demonstrates nodules 1-8 mm in diameter that are diffusely scattered. They show random distribution with peripheral and upper lobe predominance.[5] The radiological differentials include military granulomatous infections, langerhans cell histiocytosis and hematogenous metastasis. In summary, the present case is presented to familiarize the clinicians with a rather unusual pulmonary manifestation of tuberous sclerosis.
4. 5.
Singh A, Singh J. Sudden onset of dyspnea in a woman with skin lesions and lung cysts. Lung India 2013;30:164-5. Maruyama H, Ohbayashi C, Hino O, Tsutsumi M, Konishi Y. Pathogenesis ofmultifocal micronodularpneumocyte hyperplasia and lymphangioleiomyomatosisin tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2. PatholInt2001;51:585-94. Popper HH, Juettner-Smolle FM, Pongratz MG. Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis. Histopathology 1991;18:347-54. Muir TE, Leslie KO, Popper H, Kitaichi M, Gagné E,Emelin JK,et al. Micronodularpneumocyte hyperplasia. Am JSurgPathol 1998;22:465-72. Nagar AM, Teh HS, Khoo RN, Morani AC, Vrishni K, Raghuram J. Multifocal pneumocyte hyperplasia in tuberous sclerosis. Thorax 2008;63:186.
Access this article online Quick Response Code:
Website: www.lungindia.com DOI: 10.4103/0970-2113.129900
Recurrent subcutaneous emphysema in a treated pulmonary tuberculosis patient: Is there any association? Sir, A 55-year old nondiabetic, nonhypertensive, and nonsmoker female presented with dyspnoea for 1 month and swelling of face, neck, upper limb, and trunk for 2 weeks. Dyspnoea was gradual in onset, progressive, causing limitation of her daily activities. There was no history of palpitation, orthopnoea, paroxysmal nocturnal dyspnoea, and edema, or chest pain. The swelling started slowly, involving the neck and gradually involved face causing periorbital swelling and later involved upper part of trunk and upper limbs [Figure 1]. The patient had a past history of tuberculosis for which she was treated completely and declared cured 5 years ago. Examination revealed swelling of face, neck, upper limbs with crepitus all over. Trachea was shifted toward the right side. Chest examination revealed harsh vesicular breath sounds with scattered coarse crepitations. An urgent chest x-ray was done that showed subcutaneous emphysema without any evidence of pneumothorax. Areas of fibrosis were
noted in bilateral lung parenchyma. The patient was given high flow oxygen at the rate of 10 L/min, superficial surgical incisions were given at the level of thoracic inlet for the subcutaneous emphysema, but without of much benefit. CT scan of thorax showed gross bilateral subcutaneous emphysema, pneumomediastinum, minimal pneumothorax, and fibrotic changes in bilateral lung parenchyma [Figure 2]. In due course of time, other investigations were done that showed a negative mantoux test and her sputum for AFB was negative on two occasions. As initial management failed to improve patient condition, 20 F intercostal chest drainage tubes were put bilaterally under local anesthesia after consulting with chest medicine department and cardiothoracic surgery department. Two days after placement of intercostal chest drain the patient showed dramatic improvement in the form of decrease in the periorbital puffiness with generalized decrease in the amount of swelling. The chest drains were removed after one week. Consequently subcutaneous emphysema reappeared and gradually progressed in the same pattern
Lung India • Vol 31 • Issue 2 • Apr - Jun 2014 197
Copyright of Lung India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
