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Tuberous sclerosis w/ pulmonary lymphangioleiomyomatosis (LAM)

Patterson RAP - MedPix

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Tuberous sclerosis w/ pulmonary lymphangioleiomyomatosis (LAM)

History: 36 y/o female w/ a history of complex partial seizures since childhood and two episodes of spontaneous pneumothorax in the last year treated w/ drainage pleurodesis/ pleurectomy reports continued dyspnea on exertion. She currently takes no medications and has no family history of any problems.

Findings: CXR: Left and probable R lung surgery w/ hyperinflation and scattered bilateral increased lung markings c/w lymphangioleiomyomatosis; no definite evidence of pneumothorax. CT (Chest): severe cystic changes in lungs w/ no evidence of PTX or effusion. Prior CT (Abd, pelvis): numerous bilateral fatty lesions w/in kidneys c/w angiomyolipoma; multiple nonfatty angiomyolipomas bilaterally; enlarged lymph node in L iliac chain; cystic structure adjacent to the uterus on L, which may represent a retroperitoneal lymphangioma vs an enlarged L ovary w/ cyst; inhomogenous enhancement of the uterus which is also enlarged c/w fibroids; multiple osteoblastic foci w/in the vertebral bodies – study unavailable Prior MRI: periventricular calcifications – study unavailable

Ddx: Pulmonary lymphangioleiomyomatosis (LAM); phakomatoses (neurocutaneous syndromes) including tuberous sclerosis, neurofibromatosis, Sturge Weber disease, von Hippel-Lindau syndrome, and ataxia telangiectasia; emphysema, Langerhans’ cell histiocytosis (eosinophilic granuloma); lymphangioleiomyomatosis; interstitial pulmonary fibrosis; sarcoidosis; pulmonary lymphangiectasis; Diffuse pulmonary lymphangiomatosis, lymphangiomas; uterine leiomyosarcoma w/ lung mets; benign metastasizing uterine leiomyomata; mesenchymal cystic hamartoma of the lung; multiple pulmonary fibroleiomyomatosis hamartomas (very rare)

Dxhow: Biopsy

Exam: Vitals: Pulse ox w/ desats to mid-80s w/ exertion; HEENT: Multiple facial angiofibromas, gingival fibromas, multiple hypomelanotic macules, no LAD; CV: RRR; Lungs: CTAB but diminished breath sounds bilaterally; Skin: Confetti lesions x4 extremities; Shagreen’s patch on back, subungual fibromas

No MeSH data available.


CT (Chest): severe cystic changes in lungs w/ no evidence of PTX or effusion.
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MPX1015_synpic24327: CT (Chest): severe cystic changes in lungs w/ no evidence of PTX or effusion.


Tuberous sclerosis w/ pulmonary lymphangioleiomyomatosis (LAM)

Patterson RAP - MedPix

CT (Chest): severe cystic changes in lungs w/ no evidence of PTX or effusion.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX1015&req=5

MPX1015_synpic24327: CT (Chest): severe cystic changes in lungs w/ no evidence of PTX or effusion.

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Tuberous sclerosis w/ pulmonary lymphangioleiomyomatosis (LAM)

History: 36 y/o female w/ a history of complex partial seizures since childhood and two episodes of spontaneous pneumothorax in the last year treated w/ drainage pleurodesis/ pleurectomy reports continued dyspnea on exertion. She currently takes no medications and has no family history of any problems.

Findings: CXR: Left and probable R lung surgery w/ hyperinflation and scattered bilateral increased lung markings c/w lymphangioleiomyomatosis; no definite evidence of pneumothorax. CT (Chest): severe cystic changes in lungs w/ no evidence of PTX or effusion. Prior CT (Abd, pelvis): numerous bilateral fatty lesions w/in kidneys c/w angiomyolipoma; multiple nonfatty angiomyolipomas bilaterally; enlarged lymph node in L iliac chain; cystic structure adjacent to the uterus on L, which may represent a retroperitoneal lymphangioma vs an enlarged L ovary w/ cyst; inhomogenous enhancement of the uterus which is also enlarged c/w fibroids; multiple osteoblastic foci w/in the vertebral bodies – study unavailable Prior MRI: periventricular calcifications – study unavailable

Ddx: Pulmonary lymphangioleiomyomatosis (LAM); phakomatoses (neurocutaneous syndromes) including tuberous sclerosis, neurofibromatosis, Sturge Weber disease, von Hippel-Lindau syndrome, and ataxia telangiectasia; emphysema, Langerhans’ cell histiocytosis (eosinophilic granuloma); lymphangioleiomyomatosis; interstitial pulmonary fibrosis; sarcoidosis; pulmonary lymphangiectasis; Diffuse pulmonary lymphangiomatosis, lymphangiomas; uterine leiomyosarcoma w/ lung mets; benign metastasizing uterine leiomyomata; mesenchymal cystic hamartoma of the lung; multiple pulmonary fibroleiomyomatosis hamartomas (very rare)

Dxhow: Biopsy

Exam: Vitals: Pulse ox w/ desats to mid-80s w/ exertion; HEENT: Multiple facial angiofibromas, gingival fibromas, multiple hypomelanotic macules, no LAD; CV: RRR; Lungs: CTAB but diminished breath sounds bilaterally; Skin: Confetti lesions x4 extremities; Shagreen’s patch on back, subungual fibromas

No MeSH data available.