Lymphangioleiomyomatosis: A Case Report and Review of Literature

Rhee, J.A.C.; Adial, Ajay; Gumpeni, Rammohan; Iftikhar, Asma

doi:10.7759/cureus.3938

Cited by 7 publications

(8 citation statements)

References 15 publications

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“…This patient met 7 Major criteria. Definitive diagnostic criteria for LAM is the presence of characteristic and compatible lung HRCT and lung biopsy or characteristic findings on HRCT chest with anyone either angiomyolipoma (kidney) or TSC [ 3 ]. However, in our case, as the patient did not give consent, lung biopsy for histopathological confirmation of LAM could not be done.…”

Section: Discussionmentioning

confidence: 99%

A rare pulmonary lymphangioleiomyomatosis disease in a male with tuberous sclerosis complex

Kabi

Panda

Sama

et al. 2020

Respiratory Medicine Case Reports

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Background Pulmonary Lymphangioleiomyomatosis (LAM) is an uncommon disease and may be associated with tuberous sclerosis complex (TSC). LAM is reported to occur exclusively in females of the premenopausal age group. Here we report a rare entity of lymphangioleiomyomatosis in a male patient of tuberous sclerosis, who developed pneumothorax following mechanical ventilation. Case summary A young adult presented to the emergency room with history of recurrent seizures since the 6th month of his age. He was intubated in the emergency room for protection of the airway and was initially maintained on manual ventilation using Bain's circuit. Neuroimaging revealed multiple calcified subcortical nodules and giant cell astrocytoma in left lateral ventricle. On the third day of hospitalization, he developed subcutaneous emphysema on his neck and anterior wall of chest. Contrast-enhanced CT chest revealed presence of subcentimetric thin walled cystic lesions in lungs, pneumomediastinum, right sided pneumothorax, and diffuse subcutaneous emphysema. Right sided pneumothorax was managed by intercostal chest tube drainage. CECT abdomen showed well defined heterogeneously enhancing lesions in right kidney suggestive of angiomyolipoma. A final diagnosis of Lymphangioleiomyomatosis (LAM) in tuberous sclerosis (TSC) was made. Considering the high recurrence of pneumothorax, pleurodesis was done and sirolimus (2 mg per oral OD) was initiated. Conclusion Cystic lung disease consistent with LAM is a rare entity in males with TSC, which can be missed easily in patients with extra-pulmonary manifestations. Treating clinician or intensivist should remain vigilant. Active follow-up, chest imaging and pulmonary function testing should be advised to screen the patients for coincidental finding of LAM.

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Section: Discussionmentioning

confidence: 99%

A rare pulmonary lymphangioleiomyomatosis disease in a male with tuberous sclerosis complex

Kabi

Panda

Sama

et al. 2020

Respiratory Medicine Case Reports

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Section: Discussionmentioning

confidence: 99%

“…The most common presenting symptom for LAM is pneumothorax, and this is typically associated with dyspnea and chylous pleural effusions [4,5]. Patients also have extrapulmonary manifestations of LAM, particularly angiomyolipomas [5]. The diagnostic guidelines for LAM, as described in Johnson SR et al, include a high-resolution CT scan for characteristic cystic lesions in the lung and detection of extrapulmonary manifestations of LAM [6].…”

Section: Discussionmentioning

confidence: 99%

Lymphangioleiomyomatosis Presenting as Recurrent Pneumothorax

Odak

Anandani

Rogers

2020

Cureus

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Lymphangioleiomyomatosis (LAM) is a disorder that causes cystic disease in the lungs. This condition is associated with renal angiomyolipomas and commonly occurs in individuals with tuberous sclerosis. Despite its frequent association with tuberous sclerosis, LAM is a rare condition and is often underdiagnosed. An identification of the array of signs for LAM is necessary to start the patient on appropriate long-term guideline-directed medical therapy. A 24-year-old female patient with a past medical history of tuberous sclerosis, gestational hypertension, stable renal angiomyolipoma, and recent pneumothorax presented to our emergency department complaining of four weeks of productive cough. On presentation, she was found to have stable vital signs, and on examination, she had absent breath sounds in the right basilar and anterior mid-lung field compared to the left. Subsequent imaging confirmed a recurrence of pneumothorax from her visit three months prior to her current presentation as well as progressive cystic lung disease and an unchanged angiomyolipoma, suggestive of LAM. We present this article with the hope of raising the index of suspicion for LAM in the setting of the particular signs and symptoms and to encourage prompt stabilization of the patient and initiation of guidelinedirected medical therapy and strict follow-up to provide the greatest possible improvement in the patient's quality of life.

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“…Lung cysts may be congenital or occur due to Langerhans histiocytosis, rare genetic disorders, such as the Birt-Hogg-Dubé syndrome or various interstitial lung diseases [ 10 ]. Moreover, lymphangioleiomyomatosis (LAM), a multisystemic disorder characterized by the proliferation of smooth muscle cells resulting in cystic lung disease [ 11 ], should be considered in this patient. This disease is a rare progressive disorder with a prevalence of about 2.6 patients per 1,000,000 population [ 12 ].…”

Section: Differential Diagnosismentioning

confidence: 99%

“…As the disease progresses it leads to narrowing and obstruction of the airways and presents similarly to obstructive lung disease. It results in alveolar damage and the development of cystic disease of the lungs and the lymphatic system [ 11 ]. LAM patients lose about 90 mL of their forced expiratory volume in 1 s (FEV1) annually.…”

Section: Differential Diagnosismentioning

confidence: 99%