HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Louis P. Perrault Paul C. Cartier Jean Deslauriers Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Thibault, C. Articles by Deslauriers, J. Search for Related Content PubMed PubMed Citation Articles by Thibault, C. Articles by Deslauriers, J.

Ann Thorac Surg 1995;59:220-221
© 1995 The Society of Thoracic Surgeons

---

Case Report

Lobectomy in the Treatment of the Scimitar Syndrome

Division of Thoracic Surgery, Le Centre de Pneumologie de l'Hôpital Laval, Department of Cardiac Surgery, Institut de Cardiologie de l'Hôpital Laval, Department of Pediatric Cardiology, Le Centre Hospitalier de l'Université Laval, Sainte-Foy, Québec, Canada

Accepted for publication April 26, 1994.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
The scimitar syndrome is a congenital anomaly that consists mainly of total or partial anomalous venous drainage of the right lung to the inferior vena cava. We report the case of an 8-year-old girl diagnosed at birth as having a scimitar syndrome and who presented with recurrent right lower lung infections. She underwent resection of the lower half of her right lung without diversion of the anomalous venous drainage of the upper portion of the lung. She improved rapidly postoperatively and the shunt fraction diminished.

	Introduction

Top Footnotes Abstract Introduction Comment References

 
The scimitar syndrome consists of total or partial anomalous venous drainage of the right lung to the inferior vena cava. It often is associated with hypoplasia of the right lung and pulmonary artery, anomalous systemic vascular supply of the right lung from the aorta, dextrocardia, and bronchial anomalies [1]. The most effective surgical treatment of this syndrome is not well established, but may involve partial or total resection of the right lung or redirecting the abnormal vein into the left atrium.

An 8-year-old girl was first admitted at 14 days of age for persistent tachycardia and tachypnea. Initial chest roentgenogram showed hypoplasia of the right lung, mediastinal shift to the right, and the characteristic scimitar shadow (Fig 1). Heart catheterization not only confirmed total venous return of the right lung to the inferior vena cava, but also showed cardiac dextrorotation, hypoplasia of the right pulmonary artery, a patent foramen ovale, and blood supply to the lower lung originating from infradiaphragmatic branches of the aorta. Pulmonary artery pressure was 90/30 mm Hg.

View larger version (149K): [in this window] [in a new window]   Fig 1. . Characteristic right lung scimitar shadow with heart dextrorotation. Anomalous venous return of the right lung to the inferior vena cava (arrow). (SB = stomach bubble.)

Because the clinical presentation was mainly that of recurrent pulmonary infections, it was elected to proceed with right lower lobectomy without diversion of the abnormal vein. Surgical exploration revealed a ``single lobe'' right lung without an oblique scissure. Four large systemic arteries came through the diaphragm to supply the lower half of the lung, and these were ligated individually. The abnormal vein was dissected and the branch draining the inferior part of the lung was also ligated. The parenchyma was transected and the corresponding bronchus sutured with a bronchial TA stapler. Postoperative course was uneventful and 6 months after the procedure, the patient has better exercise tolerance without recurrence of pulmonary infections. Control chest roentgenogram shows good expansion of the right lung, and right heart catheterization shows a pulmonary artery pressure of 36/13 mm Hg with an improved pulmonary to systemic flow ratio of 1.6:1.

	Comment

Top Footnotes Abstract Introduction Comment References

 
In infants, the scimitar syndrome is usually asymptomatic. When symptoms are present, it is often attributable to a more severe form of disease than the one seen when symptoms appear later in life. In a review by Ponté and colleagues [2], 11 of 14 patients with symptomatic scimitar syndrome at birth died precociously. Two patients survived after drug treatment of their cardiac failure, and 1 after surgical ligature of the systemic arteries to the right lung. Most of the patients had severe pulmonary hypertension, and all 6 patients who were treated by a right pneumonectomy died.

Reimplantation of the anomalous vein done in infancy has been complicated by thrombosis and anastomotic stenosis [3, 4]. Isolated ligature of the anomalous vein has also been unsuccessful [3]. According to Blaysat and co-workers [5], division of the systemic arterial supply was successful in 6 of 7 patients with symptomatic scimitar syndrome associated with severe pulmonary hypertension. This operation, combined with medical therapy, seems to give the best results for the more severe infantile form of the scimitar syndrome.

When a patient becomes symptomatic later in life or when a symptomatic infant reaches adulthood, the treatment should be individualized and based on the clinical presentation. Some authors suggest pneumonectomy for recurrent respiratory infections or lobectomy when only one lobe drains anomalously into the inferior vena cava. Others argue that pulmonary resection may cause unnecessary sacrifice of functioning lung tissue except when the lobe is obviously severely diseased or is sequestrated. Finally, Honey [6] recommends reimplantation of the abnormal vein in the left atrium when the shunt fraction exceeds 50% (2:1).

In a recent review of 122 cases of the ``adult'' form of the scimitar syndrome [7], 37 patients underwent reimplantation of the anomalous vein, and the results were disappointing. There were four operative deaths and 21 major postoperative complications, 17 of which were thrombosis of the anastomosis between the abnormal right pulmonary vein and the left atrium. Overall, only 12 of these 37 patients improved. As stated by Sanger and colleagues [8], lung resection is simpler, is associated with less operative and postoperative morbidity, and does not impair the respiratory function measurably.

We believe that operation should be aimed primarily at pulmonary resection if the lung is abnormal and is the source of symptoms. This is recommended even if the shunt fraction is greater than 2:1. Great care should be taken to only remove the nonfunctionnal parenchyma even if this means leaving abnormal pulmonary venous drainage to the inferior vena cava. In our case, pulmonary resection reduced the shunt, cured the lung infection, and led to an early improvement in the patient's symptoms. Follow-up will be necessary to determine the long-term functional outcome and need for reoperation.

	Footnotes

Top Footnotes Abstract Introduction Comment References

 
Address reprint requests to Dr Deslauriers, Division of Thoracic Surgery, 2725 Chemin Sainte-Foy, Sainte-Foy, Québec, Canada, G1V 4G5.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurence of hypoplasic right lung with systemic arterial supply and venous drainage ``scimitar syndrome.'' Bull Johns Hopkins Hosp 1960;107:1–21.[Medline]
2. Ponté C, Dupuis C, Rémy J, et al. Syndrôme de Halasz à révélation néonatale par hypertension artérielle pulmonaire. Arch Franç Péd 1977;34:514–26.
3. Haworth SG, Sauer U, Buhlmeyer K. Pulmonary hypertension in scimitar syndrome in infancy. Br Heart J 1983;50:182–9.[Free Full Text]
4. Woody JN, Graham TP Jr, Bradford WD, Sabiston DC Jr, Canent RV Jr. Hypoplastic right lung with systemic blood supply and anomalous pulmonary venous drainage: reversal of pulmonary hypertension with surgical management in infancy. Am Heart J 1972;83:82–8.[Medline]
5. Blaysat G, Kachaner J, Villain E, et al. Le syndrome du cimeterre du nourrisson. Physiopathologie et déductions thérapeutiques dans 12 cas. Arch Fr Pediatr 1987;44:245–51.[Medline]
6. Honey M. Anomalous pulmonary venous drainage of right lung to inferior vena cava (``scimitar syndrome''): clinical spectrum in older patients and role of surgery. Q J Med 1977;184:463–83.
7. Dupuis C, Charaf LAC, Brevière GM, Abou P, Rémy-Jardin M, Helmius G. The ``adult'' form of the scimitar syndrome. Am J Cardiol 1992;70:502–7.[Medline]
8. Sanger PW, Taylor FH, Robicsek F. The ``scimitar syndrome.'' Diagnosis and treatment. Arch Surg 1963;86:580–7.[Medline]

This article has been cited by other articles:

				V. L. Vida, S. Speggiorin, M. A. Padalino, G. Crupi, C. Marcelletti, L. Zannini, A. Frigiola, A. Varrica, D. Di Carlo, R. Di Donato, et al. The scimitar syndrome: an Italian multicenter study. Ann. Thorac. Surg., August 1, 2009; 88(2): 440 - 444. [Abstract] [Full Text] [PDF]

				R. Baskar Karthekeyan, R. Saldanha, M R. Sahadevan, S. K. Rao, M. Vakamudi, and B. K Rajagopal Scimitar Syndrome: Experience With 6 Patients Asian Cardiovasc Thorac Ann, June 1, 2009; 17(3): 266 - 271. [Abstract] [Full Text] [PDF]

				J. P. Le Rochais, P. Icard, S. Davani, D. Abouz, and C. Evrard Scimitar syndrome with pulmonary arteriovenous fistulas Ann. Thorac. Surg., October 1, 1999; 68(4): 1416 - 1418. [Abstract] [Full Text] [PDF]

				H. K. Najm, W. G. Williams, J. G. Coles, I. M. Rebeyka, and R. M. Freedom SCIMITAR SYNDROME: TWENTY YEARS' EXPERIENCE AND RESULTS OF REPAIR J. Thorac. Cardiovasc. Surg., November 1, 1996; 112(5): 1161 - 1169. [Abstract] [Full Text]

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Louis P. Perrault Paul C. Cartier Jean Deslauriers Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Thibault, C. Articles by Deslauriers, J. Search for Related Content PubMed PubMed Citation Articles by Thibault, C. Articles by Deslauriers, J.