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Scimitar Syndrome: Another One for the Radiologists

Rowlens M. Melduni, MD^* and Farouk Mookadam, MBChB, FRCPC

^* Division of Cardiovascular Diseases and Department of Internal Medicine, Mayo Clinic, Rochester, Minn
Division of Cardiovascular Diseases and Department of Internal Medicine, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ 85259
e-mail: mookadam.farouk{at}mayo.edu

Editor:

We are writing to correct a letter to the editor (1) that appeared 6 years ago in your prestigious journal (in the January 1999 issue).

We discovered this letter during a recent literature search of MEDLINE while preparing an article on anomalous pulmonary venous drainage. During the MEDLINE search, we discovered the following information:

1. Anomalous pulmonary venous drainage was first described in 1836 by Cooper (2) and Chassinat (3). They individually described single cases of rare congenital malformations that included venous drainage below the diaphragm. The term "scimitar" did not appear in their reports.

2. The next description of this anomaly was in a report by Park (4) in 1912, in which the author described three cases of infants with anomalous pulmonary drainage at autopsy. Again, the word "scimitar" did not appear in this report.

3. In 1949, Dotter et al (5), who were also the first to use angiocardiography and cardiac catheterization to diagnose this anomaly in life, published a report where the scimitar sign was first described radiographically.

4. The first authors to use the term "scimitar" were Halasz et al (6) in 1956.

5. Finally, in 1960, Neill et al (7) were the first to describe the familial occurrence and clinical spectrum of the condition and name it the scimitar syndrome.

As can be seen from this chronology, Neill et al deserve credit for naming the scimitar syndrome, and Halasz et al should be credited for the first use of the eponym "scimitar," while the radiologists, as illustrated by Dotter et al, should be given credit for describing the scimitar sign.

	References

TOP References References

 

1. Mulligan ME. History of scimitar syndrome [letter]. Radiology 1999;210:288–290.[Free Full Text]
2. Cooper G. Case of malformation of the thoracic viscera: consisting of imperfect development of right lung and transposition of the heart. Lond Med Gaz 1836;18:600–601.
3. Chassinat R. Observation d'anomalies anatomiques remarquables de l'appareil circulatoire, avec hepatocele congeniale, n'ayant donne lieu pendant la vie a aucun symptom particulier. Arch Genet Med 1836;11:80–84.
4. Park EA. Defective development of the right lung, due to anomalous development of the right pulmonary artery and vein, accompanied by dislocation of the heart simulating dextrocardia. Proc N Y Pathol Soc 1912;12:88–93.
5. Dotter CT, Hardisty NM, Steinberg I. Anomalous right pulmonary vein entering the inferior vena cava: two cases diagnosed during life by angiocardiography and cardiac catheterization. Am J Med Sci 1949;218:31–36.[Medline]
6. Halasz NA, Halloran KH, Liebow AA. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 1956;14:826–846.[Medline]
7. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage "scimitar syndrome." Bull Johns Hopkins Hosp 1960;107:1–21.[Medline]

Response

Department of Diagnostic Radiology, University of Maryland Medical System, N2W78, 22 South Greene Street, Baltimore, MD 21201-1595
e-mail: mmulligan{at}umm.edu

I thank the authors for adding one more piece of information (point 3 in their letter) to the other facts regarding scimitar syndrome that I presented in my previous letter to the editor (1) and that are available in the book Classic Radiologic Signs (2).

As a radiologist, I would like to see Dr Dotter and colleagues claim "another one for the radiologists," but their wording is consistent throughout their report (3) and describes the pulmonary venous anomaly as a "crescent-like" shadow. Since they do not use the word "scimitar" in their report of two angiographic cases, I would think it inappropriate to say that they were the first to describe the scimitar sign. They certainly seem to have been the first ones to report the angiographic findings in live patients and deserve credit for that. Halasz et al (4), despite being nonradiologists, continue to get my vote as the first to use the word "scimitar" in print in reference to this condition.

	References

TOP References References

 

1. Mulligan ME. History of scimitar syndrome [letter]. Radiology 1999;210:288–290.[Free Full Text]
2. Mulligan ME. Classic radiologic signs: an atlas and history. New York, NY: Parthenon, 1996.
3. Dotter CT, Hardisty NM, Steinberg I. Anomalous right pulmonary vein entering the inferior vena cava: two cases diagnosed during life by angiocardiography and cardiac catheterization. Am J Med Sci 1949;218:31–36.
4. Halasz NA, Halloran KH, Liebow AA. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 1956;14:826–846.[Medline]

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