대한흉부영상의학회 Korean Society of Thoracic Radiology GuerBet

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대한흉부영상의학회 Weekly Case 검색
대한흉부영상의학회 Weekly Case 검색
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Weekly Chest CasesArchive of Old Cases

Case No : 44 Date 1998-08-31

  • Courtesy of KUN-IL Kim, M.D. / Pusan National University Hospital
  • Age/Sex 6 / M
  • Chief Complaint
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Chest PA

Diagnosis With Brief Discussion

Diagnosis
Congenital Pulmonary Lymphangiectasia
Radiologic Findings
Brief Review
This six-year-old male patient had recurrent episode of respiratory infections after birth. Inspite of treatment, his chest radiographs have never showed normal on each episode. Presenting symptoms and signs were dyspnea, nonproductive coughing, fever, wheezing, and left hemihypertrophy. Left edematous extremities showed non-pitting, hard consistency.

Chest radiograph shows diffuse reticular opacities with lower zonal dominance on both lungs. Note enlarged left arm with mottled lucencies in subcutanous area (left hand and forearm show same finding) suggestive of lymphangiectasia. HRCT shows thickening of bronchovascular bundles, core structures, interlobular septa, and visceral pleura which were known to have abundant lymphatics. Focal areas of consolidation, which seemed to be due to co-existing bronchopneumonia or this disease itself, were shown. Evaluation for cardiac disease revealed no specific abnormality. Biopsy on small intestine showed intestinal lymphangiectasia.
MRI on left extremities revealed classic findings of lymphedema. Chest radiologic findings were compatible with congenital pulmonary lymphangiectasis, a manefestation of generalized lymphangiectasia.
References
1. Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymhangiectasis. Am J Dis Child 1970;120:314
2. Lloyd ES, Press HC Jr. Congenital pulmonary lymphangiectasis. South Med J 1979;72:1205-1206
3. Scott-Emuakpor AB, Warren ST, Kapur S, Quiachon EB, Higgins JV. Familial occurrence of congenital pulmonary lymphangiectasis. Genetic implications. Am J Dis Child 1981;135:532-534
Keywords
Lung, Congenital,

No. of Applicants : 29

▶ Correct Answer : 19/29,  65.5%
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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