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Congenital Pulmonary Lymphangiectasis

Congenital Pulmonary Lymphangiectasis Abstract Three patients with pulmonary lymphangiectasis were studied by cardiac catheterization. All had evidence of alveolar hypoventilation and pulmonary hypertension. Postmortem injection studies of the abnormal lungs demonstrated greatly dilated and intracommunicating pleural, interlobular, and perivascular lymphatics. Two of the patients had obstructed total anomalous pulmonary venous return, while the third had a ventricular septal defect. In the latter, a premortem diagnosis of pulmonary lymphangiectasis was made at 4 months of age by lung biopsy. Forty five cases from the literature have been reviewed. From the review it is postulated that pulmonary lymphangiectasis can occur in the following three forms: (1) as part of generalized lymphangiectasis; (2) secondary to pulmonary venous obstruction; and (3) as a primary developmental defect of the lung. The clinical picture and prognosis varies with the type present. References 1. Virchow R: Gesammelte Abhandlungen zur Wissenschaftlichen Medicin. Frankfurt , Meidinger Sohn & Co, 1856, p 982. 2. Bredt H: Lymphangiectasia pulmonum congenita . Virchow Arch Path Anat 321:517-530, 1952. 3. Laurence KM: Congenital pulmonary cystic lymphangiectasis . J Path Bact 70:325-333, 1955.Crossref 4. Laurence KM: Congenital pulmonary lymphangiectasis . J Clin Path 12:62-69, 1959.Crossref 5. Giammalvo JT: Congenital lymphangiomatosis of the lung: A form of cystic disease: Report of a case with autopsy findings . Lab Invest 4:450-456,1955. 6. Maidman L, Barnett RN: Congenital dilatation of pulmonary lymphatics . Arch Path 64:104-106,1957. 7. Carter RW, Vaugh HM: Congenital Pulmonary lymphangiectasis: Report of a case with roentgen findings . Amer J Roentgen 86:576-578,1961. 8. Tucker AS: Lymphangiectasis: Benign and malignant . Amer J Roentgen 91:1104-1113, 1964. 9. Giedion A, Muller WA, Molz G. Angeborene: Lymphangiecktasic der Lungen . Helv Paediat Acta 22:170-180, 1967. 10. Fronstin MH, Hooper GS, Besse BE, etal: Congenital pulmonary cystic lymphangiectasis . Amer J Dis Child 114:330-335, 1967.Crossref 11. Theros EG: An Exercise in Radiologic-Pathologic Correlation . Radiology 89:524-531, 1967.Crossref 12. Arkoff RS: Congenital pulmonary lymphangiectasis . Calif Med 109:464-466, 1968. 13. Brown MD, Reidbord, HE: Congenital pulmonary lymphangiectasis . Amer J Dis Child 114:654-657, 1967. 14. Meadows JA: Two cases of rare causes of respiratory distress in the newborn . J Med Assoc Alabama 37:1039-1044, 1968. 15. Javett SN, Webster I, Braudo JL: Congenital dilatation of the pulmonary lymphatics . Pediatrics 31:416-425, 1963. 16. Trinkle JK, Danielson GK, Noonan JA, et al: Infradiaphragmatic total anomalous pulmonary venous return: Report of a new and correctable variant . Ann Thorac Surg 5:55-60,1968.Crossref 17. Harvey DF, Zimmerman HM: Studies of the development of the human lung . Anat Rec 61:203-229, 1935.Crossref 18. Simer PH: Drainage of pleural lymphatics . Anat Rec 113:269-283,1952.Crossref 19. Tobin CE: Human pulmonic lymphatics: An anatomic study . Anat Rec 127:611-633, 1957.Crossref 20. Frank J, Piper PG: Congenital pulmonary cystic lymphangiectasis . JAMA 171:1094-1098, 1959.Crossref 21. McKendry JBJ, Lindsay WK, Gersteen MC: Congenital defects of the lymphatics in infancy . Pediatrics 19:21-34,1957. 22. Mann TP: Hemihypertrophy left side of body: congenital lymphatic edema of left arm: radiological enlargement of heart shadow . Proc Royal Soc Med 48:330, 1955. 23. Koblenzer PJ, Bukowski MJ: Angiomatosis (hamartomatous hemi-lymphangiomatosis): Report of case with diffuse involvement . Pediatrics 28:65-76, 1961. 24. Shortland-Webb, WR, Tozer RA, Cameron AH: Intra-uterine closure of the atrial septum . J Clin Path 19:549-554,1966.Crossref 25. Rywlin AM, Fojaca RM: Congenital pulmonary lymphangiectasis associated with a blind common pulmonary vein . Pediatrics 41:931-934,1968. 26. Tan-Vinh L, Cochard A, Dong VT: Lymphangiectasies pulmonaires congenitales et lymphangite pleuro-pulmonaire, concereuse metastatique de l'enfant . Arch Franc Pediat 21:165-182, 1964. 27. Sacrez R, et al: Lymphangiectasies pulmonaires congenitales avec fibroelastose de l'endocarde . Arch Franc Paediat 17:1344-1349, 1960. 28. Ekelund H, Palmstierna S, Ostberg C: Congenital pulmonary lymphangiectasis . Acta Paediat Scand 55:121-125,1966.Crossref 29. Heumann G, Korn R, Levy-Silogy J: Apropos d'un cas de laymphangiectasies Pulmonaires Congenitales . Arch Franc Paediat 17:1012-1023, 1960. 30. Moffat AD: Congenital cystic disease of the lungs and its classification . J Path Bact 70:361-372,1960.Crossref 31. Lauweryns JM, Boussauw L: The ultra-structure of pulmonary lymphatic capillaries of newborn rabbits and human infants . Lymphology 2:108-129, 1969. 32. Miller WS: The lymphatics of the lung . Anat Anz 12:110-114,1896. 33. Staub NC, Nagano H, Pearse ML: Pulmonary edema in dogs, especially the sequence of fluid accumulation in lungs . J Appl Physiol 22:227-240, 1967. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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References (38)

Publisher
American Medical Association
Copyright
Copyright © 1970 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1970.02100090088006
Publisher site
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Abstract

Abstract Three patients with pulmonary lymphangiectasis were studied by cardiac catheterization. All had evidence of alveolar hypoventilation and pulmonary hypertension. Postmortem injection studies of the abnormal lungs demonstrated greatly dilated and intracommunicating pleural, interlobular, and perivascular lymphatics. Two of the patients had obstructed total anomalous pulmonary venous return, while the third had a ventricular septal defect. In the latter, a premortem diagnosis of pulmonary lymphangiectasis was made at 4 months of age by lung biopsy. Forty five cases from the literature have been reviewed. From the review it is postulated that pulmonary lymphangiectasis can occur in the following three forms: (1) as part of generalized lymphangiectasis; (2) secondary to pulmonary venous obstruction; and (3) as a primary developmental defect of the lung. The clinical picture and prognosis varies with the type present. References 1. Virchow R: Gesammelte Abhandlungen zur Wissenschaftlichen Medicin. Frankfurt , Meidinger Sohn & Co, 1856, p 982. 2. Bredt H: Lymphangiectasia pulmonum congenita . Virchow Arch Path Anat 321:517-530, 1952. 3. Laurence KM: Congenital pulmonary cystic lymphangiectasis . J Path Bact 70:325-333, 1955.Crossref 4. Laurence KM: Congenital pulmonary lymphangiectasis . J Clin Path 12:62-69, 1959.Crossref 5. Giammalvo JT: Congenital lymphangiomatosis of the lung: A form of cystic disease: Report of a case with autopsy findings . Lab Invest 4:450-456,1955. 6. Maidman L, Barnett RN: Congenital dilatation of pulmonary lymphatics . Arch Path 64:104-106,1957. 7. Carter RW, Vaugh HM: Congenital Pulmonary lymphangiectasis: Report of a case with roentgen findings . Amer J Roentgen 86:576-578,1961. 8. Tucker AS: Lymphangiectasis: Benign and malignant . Amer J Roentgen 91:1104-1113, 1964. 9. Giedion A, Muller WA, Molz G. Angeborene: Lymphangiecktasic der Lungen . Helv Paediat Acta 22:170-180, 1967. 10. Fronstin MH, Hooper GS, Besse BE, etal: Congenital pulmonary cystic lymphangiectasis . Amer J Dis Child 114:330-335, 1967.Crossref 11. Theros EG: An Exercise in Radiologic-Pathologic Correlation . Radiology 89:524-531, 1967.Crossref 12. Arkoff RS: Congenital pulmonary lymphangiectasis . Calif Med 109:464-466, 1968. 13. Brown MD, Reidbord, HE: Congenital pulmonary lymphangiectasis . Amer J Dis Child 114:654-657, 1967. 14. Meadows JA: Two cases of rare causes of respiratory distress in the newborn . J Med Assoc Alabama 37:1039-1044, 1968. 15. Javett SN, Webster I, Braudo JL: Congenital dilatation of the pulmonary lymphatics . Pediatrics 31:416-425, 1963. 16. Trinkle JK, Danielson GK, Noonan JA, et al: Infradiaphragmatic total anomalous pulmonary venous return: Report of a new and correctable variant . Ann Thorac Surg 5:55-60,1968.Crossref 17. Harvey DF, Zimmerman HM: Studies of the development of the human lung . Anat Rec 61:203-229, 1935.Crossref 18. Simer PH: Drainage of pleural lymphatics . Anat Rec 113:269-283,1952.Crossref 19. Tobin CE: Human pulmonic lymphatics: An anatomic study . Anat Rec 127:611-633, 1957.Crossref 20. Frank J, Piper PG: Congenital pulmonary cystic lymphangiectasis . JAMA 171:1094-1098, 1959.Crossref 21. McKendry JBJ, Lindsay WK, Gersteen MC: Congenital defects of the lymphatics in infancy . Pediatrics 19:21-34,1957. 22. Mann TP: Hemihypertrophy left side of body: congenital lymphatic edema of left arm: radiological enlargement of heart shadow . Proc Royal Soc Med 48:330, 1955. 23. Koblenzer PJ, Bukowski MJ: Angiomatosis (hamartomatous hemi-lymphangiomatosis): Report of case with diffuse involvement . Pediatrics 28:65-76, 1961. 24. Shortland-Webb, WR, Tozer RA, Cameron AH: Intra-uterine closure of the atrial septum . J Clin Path 19:549-554,1966.Crossref 25. Rywlin AM, Fojaca RM: Congenital pulmonary lymphangiectasis associated with a blind common pulmonary vein . Pediatrics 41:931-934,1968. 26. Tan-Vinh L, Cochard A, Dong VT: Lymphangiectasies pulmonaires congenitales et lymphangite pleuro-pulmonaire, concereuse metastatique de l'enfant . Arch Franc Pediat 21:165-182, 1964. 27. Sacrez R, et al: Lymphangiectasies pulmonaires congenitales avec fibroelastose de l'endocarde . Arch Franc Paediat 17:1344-1349, 1960. 28. Ekelund H, Palmstierna S, Ostberg C: Congenital pulmonary lymphangiectasis . Acta Paediat Scand 55:121-125,1966.Crossref 29. Heumann G, Korn R, Levy-Silogy J: Apropos d'un cas de laymphangiectasies Pulmonaires Congenitales . Arch Franc Paediat 17:1012-1023, 1960. 30. Moffat AD: Congenital cystic disease of the lungs and its classification . J Path Bact 70:361-372,1960.Crossref 31. Lauweryns JM, Boussauw L: The ultra-structure of pulmonary lymphatic capillaries of newborn rabbits and human infants . Lymphology 2:108-129, 1969. 32. Miller WS: The lymphatics of the lung . Anat Anz 12:110-114,1896. 33. Staub NC, Nagano H, Pearse ML: Pulmonary edema in dogs, especially the sequence of fluid accumulation in lungs . J Appl Physiol 22:227-240, 1967.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Oct 1, 1970

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