- Email: [email protected]
Right aortic arch, right ligamentum, absent left pulmonary artery: a rare vascular ring
1472
CASE REPORT DODGE-KHATAMI ET AL RIGHT ARCH, RIGHT LIGAMENTUM, ABSENT LPA
determine that the increase in CO was attributed to a slight increase in SV and a large increase in HR, and that these increases were due to PLV alone without mitral valve repair or replacement. Acute changes in other cardiovascular parameters were observed, but the clinical meaning of and explanation for these changes are unclear. In particular, there was a large increase in LVedP that would be indicative of depressed function in a normal heart. Substantial increases in LVdE and LVesE were seen that would also suggest depressed function in a normal heart. However, some of the increases may be explained in part by the physical reduction in ventricular geometry. Reduced LVEDV will result in lower wall stress for a given internal pressure (LVedP). By extension, this would be interpreted as an increase in the elastance, meaning that more pressure is required to expand the ventricle during filling. We would have predicted that, by restoring the heart to “normal” geometry, the elastance would also increase to “normal” as defined by the Law of Laplace, however, this does not appear to be the case. The elastance after PLV was much greater than would be expected in a normal heart as evidenced by the elevated LVedP. This seems to suggest that factors, in addition to reduced geometry, may exist which contribute to the elevated elastance. We speculate that one possible explanation is that the mechanical properties of the myocardium have changed as a result of prolonged IDCM. In summary, based on the findings in this patient, we conclude that PLV without mitral valve repair or replacement can increase EF and CO attributed to increased SV and HR. There were other acute cardiovascular changes that occurred that are indicative of depressed heart function which are difficult to interpret. Most notably LVedP, LVesE, and LVdE were greatly increased above what would be expected in a normal heart. However, at 1 year follow-up, the patient remained clinically improved as evidenced by substantially improved NYHA and VO2 max while maintaining reduced LVEDV, increased LVEF, and trivial MR.
References 1. Sunagawa K, Yamada A, Senda Y, et al. Estimation of the hydromotive source pressure from ejecting beats of the left ventricle. IEEE Trans Biomed Eng 1980;27:299 –305. 2. Landes G. Einige untersuchungen an elektrischen analogieschaltungen zum kreitslaufsystem. Z Biol 1943;101:418–29. 3. Batista RJV, Verde J, Nery P, et al. Partial left ventriculectomy to treat end-stage heart disease. Ann Thorac Surg 1997;64: 634– 8. 4. Bolling SF, Pagani FD, Deeb GM, Bach DS. Intermediate-term outcome of mitral reconstruction in cardiomyopathy. J Thorac Cardiovasc Surg 1998;115:381– 88. 5. Gorcsan J III, Feldman AM, Kormos RL, Mandarino WA, Demetris AJ, Batista RJV. Heterogeneous immediate effects of partial left ventriculectomy on cardiac performance. Circulation 1998;97:839– 42. 6. Dickstein ML, Spotnitz HM, Rose EA, Burkhoff DB. Heart reduction surgery: an analysis of the impact on cardiac function. J Thorac Cardiovasc Surg 1997;113:1032–1040. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1472– 4
Right Aortic Arch, Right Ligamentum, Absent Left Pulmonary Artery: A Rare Vascular Ring Ali Dodge-Khatami, MD, Carl L. Backer, MD, Michael E. Dunham, MD, and Constantine Mavroudis, MD Divisions of Cardiovascular-Thoracic Surgery and Pediatric Otolaryngology, Department of Surgery, Northwestern University Medical School, The Children’s Memorial Hospital, Chicago, Illinois
A 5-month-old infant presented with respiratory failure secondary to severe right bronchial compression. Diagnostic imaging revealed a right aortic arch and absent left pulmonary artery. Surgical relief was obtained via median sternotomy by dividing a right ligamentum and pexing the enlarged right pulmonary artery to the ascending aorta. (Ann Thorac Surg 1999;67:1472– 4) © 1999 by The Society of Thoracic Surgeons
T
here are very few reports of vascular rings that can not be addressed through a left thoracotomy [1, 2]. We report the rare combination of a right aortic arch, right ligamentum arteriosum, and absent left pulmonary artery in a 5-month-old infant that presented with acute respiratory failure. Through a median sternotomy, a tight right ligamentum arteriosum (acting like a “bow-string”) and an enlarged right pulmonary artery were found compressing and nearly occluding the right mainstem bronchus. Relief of the right bronchial compression was obtained by dividing the ligamentum and pexing the right pulmonary artery to the aorta. A 5-month-old girl presented with a 2-day history of clear rhinitis, coughing, wheezing, and tachypnea with fever. Chest roentgenogram showed a hyperinflated right lung and right upper and lower lobe atelectasis. Progressive respiratory failure despite bronchodilators led to endotracheal intubation and mechanical ventilation. Progressive respiratory acidosis motivated a transfer to our institution. Initial arterial blood gas (pH 7.17, pCO2 of 78 mm Hg, pO2 84 mm Hg) was on an FiO2 of 70%. Bronchoscopy demonstrated extrinsic anterior nonpulsatile compression of the right mainstem bronchus, causing nearly complete occlusion of this bronchus. The trachea and left main bronchus were normal. The bronchoscope did pass into the right main bronchus and there was purulent material suctioned from the distal bronchus. Computerized tomography scan of the chest revealed a right aortic arch, compression of the right main Accepted for publication Oct 10, 1998. Address reprint requests to Dr Backer, Division of CardiovascularThoracic Surgery, The Children’s Memorial Hospital, m/c 22, 2300 Children’s Plaza, Chicago, IL 60614; e-mail: [email protected].
0003-4975/99/$20.00 PII S0003-4975(99)00189-7
Ann Thorac Surg 1999;67:1472– 4
CASE REPORT DODGE-KHATAMI ET AL RIGHT ARCH, RIGHT LIGAMENTUM, ABSENT LPA
1473
Fig 1. Aortogram demonstrates a right aortic arch, mirror image branching of the brachiocephalic vessels, and a ductal “bump” that was consistent with the noted intraoperative right ligamentum arteriosum.
Fig 2. Contrast bronchogram with simultaneous levophase aortogram demonstrating a normal trachea and left main bronchus, with severe narrowing of the right mainstem bronchus at a site between the right descending aorta and right pulmonary artery.
bronchus, and the left pulmonary artery could not be identified. An echocardiogram was performed to rule out pulmonary artery sling [3]. This demonstrated normal intracardiac anatomy, but again no evidence of a left pulmonary artery. Cardiac catheterization with simultaneous contrast tracheo-bronchography demonstrated a large right pulmonary artery with compression of the right mainstem bronchus between the pulmonary artery anteriorly and the right-sided descending thoracic aorta posteriorly (Figs 1–3). A retrograde injection of the left pulmonary veins revealed a tiny intraparenchymal left pulmonary artery that did not communicate with the main pulmonary artery. Without a precise preoperative diagnosis, we elected to approach the right bronchial compression through a median sternotomy. The pericardium was opened and a right aortic arch noted. There was no evidence of a left-sided ligamentum arteriosum or intrapericardial left pulmonary artery. A ligamentum arteriosum was identified coming off the postero-superior aspect of the right pulmonary artery and inserting into the right descending thoracic aorta. The right ligamentum was doubly ligated and divided. The right pulmonary artery was fully mobilized to rule out a pulmonary artery sling. Intraoperative bronchoscopy was performed and visualized a now improved opening of the right main bronchus. This opening was further improved (to near normal) by suspending the right pulmonary artery to the posterior aspect of the ascending aorta. Peak airway pressures required for adequate ventilation dropped from 42 to 28 mm Hg. The patient was extubated on the first postoperative day. Bronchoscopic follow-up 2 weeks, 3 months, and 10 months postsurgery revealed a slightly “flattened” but patent right mainstem bronchus, and the child has con-
tinued to be symptom-free except for rare intermittent episodes of reactive airway disease.
Comment We recently reported our experience with nearly 300 patients with vascular rings over a 5-year time span [4]. In that series, this is the first child with a right aortic arch, right ligamentum, and absent left pulmonary artery. Before this, all of the patients with a vascular ring caused
Fig 3. Pulmonary angiogram illustrates a large right pulmonary artery, no left pulmonary artery, and no evidence for a pulmonary artery sling.
1474
CASE REPORT TAKATA ET AL NEURILEMOMA OF THE STERNUM
by a right aortic arch (79 patients) had the ring completed by a left ligamentum. In this patient, there was not a true “complete” ring, rather the right ligamentum acting like a “bow-string” along with the enlarged right pulmonary artery. The right pulmonary artery was enlarged because of the absent left pulmonary artery, with the entire cardiac output passing through the right pulmonary artery. This has been called a “hemodynamic vise.” We have categorized patients such as this with those with “complete” vascular rings because of the similarities in clinical presentation, diagnostic techniques, and surgical intervention. Robotin and colleagues from Paris reported an experience of more than 500 infants and children undergoing operation for symptomatic tracheo-bronchial compression [5]. They described a single patient with a right aortic arch and right descending aorta that had compression of the right main bronchus. They alleviated the bronchial compression by mobilizing the descending aorta through a right thoracotomy. That child did have a left pulmonary artery and had a patent ductus arteriosus, presumably on the left side (this was not stated). Do¨hlemann and colleagues reported 3 infants with hypoplasia or aplasia of the right lung, dextrocardia, and compression of the trachea by the ascending aorta as it traversed from right to left [6]. In a sense, these are “mirror images” of our patient, the difference being that despite having an absent left pulmonary artery, our patient had a normal-sized left lung and levocardia, hence, the great vessel orientation was essentially normal for a right arch. Despite a thorough preoperative work-up, failure to precisely determine the nature of the compression was understandable given the unusual pathology. We therefore elected a median sternotomy approach. This allowed direct visualization of any type of vascular ring and also permitted cardiopulmonary bypass, if necessary, for repair of pulmonary artery sling [7]. Intraoperative bronchoscopy is an invaluable diagnostic tool that allows direct assessment of the extrinsic compression of the airways, and documents the adequacy of repair. This illustrates how multiple diagnostic modalities may be necessary in the unusual patient with a vascular ring that does not fall into the “neat” diagnostic categories of double aortic arch, right aortic arch with left ligamentum, innominate artery compression syndrome, and pulmonary artery sling [8]. Although the usual tendency is to obtain too many studies that simply continue to verify the diagnosis, when the diagnostic studies are not typical, it is important to continue investigation as far as possible to insure appropriate surgical intervention. In this case, the final (surgical) diagnosis led to successful airway relief by combining division of the right ligamentum arteriosum and pexing of the right pulmonary artery to the aorta.
We thank Dr David Wax for providing the cardiac catheterization image.
© 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1474 – 6
References 1. McFaul R, Millard P, Norwicki E. Vascular rings necessitating right thoracotomy. J Thorac Cardiovasc Surg 1981;82:306–9. 2. Whitman G, Stephenson LW, Weinberg P. Vascular ring: left cervical aortic arch, right descending aorta, and right ligamentum arteriosum. J Thorac Cardiovasc Surg 1982;83:311–5. 3. Alboliras ET, Backer CL, Holinger LD, et al. Pulmonary artery sling: diagnostic and management strategy. Pediatrics 1996; 98:530(Abstr). 4. Backer CL, Mavroudis C. Surgical approach to vascular rings. In: Karp RB, Laks H, Wechsler AS, eds. Advances in cardiac surgery. Chicago: Mosby-Year Book, 1997:29– 64. 5. Robotin MC, Bruniaux J, Serraf A, et al. Unusual forms of tracheobronchial compression in infants with congenital heart disease. J Thorac Cardiovasc Surg 1996;112:415–23. 6. Do¨hlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy. J Pediatr Surg 1990;25:290–3. 7. Backer CL, Idriss FS, Holinger LD, et al. Pulmonary artery sling: results of surgical repair in infancy. J Thorac Cardiovasc Surg 1992;103:683–91. 8. Lowe GM, Donaldson JS, Backer CL. Vascular rings: 10-year review of imaging. Radiographics 1991;11:637– 46.
Intraosseous Neurilemoma of the Sternum Kohei Takata, MD, Kosuke Okuda, MD, and Mitsuo Ochi, MD Department of Orthopaedics, Shimane Medical University, Izumo, Japan.
Sternal benign bone tumor is uncommon. We report a very rare case of intraosseous neurilemoma in the sternum. A girl, 10 years of age, was diagnosed as having a sternal tumor. Computed tomographic scan disclosed a mushroom-shape tumor in the region from inside of the sternum to the ventral soft tissues. Surgical resection was performed. Histopathologic findings showed a typical neurilemoma with Antoni type A and B patterns. No recurrence has been found. (Ann Thorac Surg 1999;67:1474 – 6) © 1999 by The Society of Thoracic Surgeons
P
rimary bone tumors of the chest wall are uncommon [1]. Among those tumors, sternal tumors are rare and usually malignant [2]. On the other hand, intraosseous neurilemoma is rare and accounts for less than 1% of primary bone tumors [3]. We report an extremely rare case of neurilemoma developed in the sternum. A 10-year-old girl was referred to our department in December 1992. A tumor of 5 3 4 cm was found at the sternal region. Plain radiogram showed a round translu-
Accepted for publication Oct 12, 1998. Address reprint requests to Dr Takata, Department of Orthopaedics, Shimane Medical University, 89-1 Enyacho, Izumoshi, Japan 693-8501.
0003-4975/99/$20.00 PII S0003-4975(99)00237-4
CASE REPORT DODGE-KHATAMI ET AL RIGHT ARCH, RIGHT LIGAMENTUM, ABSENT LPA
determine that the increase in CO was attributed to a slight increase in SV and a large increase in HR, and that these increases were due to PLV alone without mitral valve repair or replacement. Acute changes in other cardiovascular parameters were observed, but the clinical meaning of and explanation for these changes are unclear. In particular, there was a large increase in LVedP that would be indicative of depressed function in a normal heart. Substantial increases in LVdE and LVesE were seen that would also suggest depressed function in a normal heart. However, some of the increases may be explained in part by the physical reduction in ventricular geometry. Reduced LVEDV will result in lower wall stress for a given internal pressure (LVedP). By extension, this would be interpreted as an increase in the elastance, meaning that more pressure is required to expand the ventricle during filling. We would have predicted that, by restoring the heart to “normal” geometry, the elastance would also increase to “normal” as defined by the Law of Laplace, however, this does not appear to be the case. The elastance after PLV was much greater than would be expected in a normal heart as evidenced by the elevated LVedP. This seems to suggest that factors, in addition to reduced geometry, may exist which contribute to the elevated elastance. We speculate that one possible explanation is that the mechanical properties of the myocardium have changed as a result of prolonged IDCM. In summary, based on the findings in this patient, we conclude that PLV without mitral valve repair or replacement can increase EF and CO attributed to increased SV and HR. There were other acute cardiovascular changes that occurred that are indicative of depressed heart function which are difficult to interpret. Most notably LVedP, LVesE, and LVdE were greatly increased above what would be expected in a normal heart. However, at 1 year follow-up, the patient remained clinically improved as evidenced by substantially improved NYHA and VO2 max while maintaining reduced LVEDV, increased LVEF, and trivial MR.
References 1. Sunagawa K, Yamada A, Senda Y, et al. Estimation of the hydromotive source pressure from ejecting beats of the left ventricle. IEEE Trans Biomed Eng 1980;27:299 –305. 2. Landes G. Einige untersuchungen an elektrischen analogieschaltungen zum kreitslaufsystem. Z Biol 1943;101:418–29. 3. Batista RJV, Verde J, Nery P, et al. Partial left ventriculectomy to treat end-stage heart disease. Ann Thorac Surg 1997;64: 634– 8. 4. Bolling SF, Pagani FD, Deeb GM, Bach DS. Intermediate-term outcome of mitral reconstruction in cardiomyopathy. J Thorac Cardiovasc Surg 1998;115:381– 88. 5. Gorcsan J III, Feldman AM, Kormos RL, Mandarino WA, Demetris AJ, Batista RJV. Heterogeneous immediate effects of partial left ventriculectomy on cardiac performance. Circulation 1998;97:839– 42. 6. Dickstein ML, Spotnitz HM, Rose EA, Burkhoff DB. Heart reduction surgery: an analysis of the impact on cardiac function. J Thorac Cardiovasc Surg 1997;113:1032–1040. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1472– 4
Right Aortic Arch, Right Ligamentum, Absent Left Pulmonary Artery: A Rare Vascular Ring Ali Dodge-Khatami, MD, Carl L. Backer, MD, Michael E. Dunham, MD, and Constantine Mavroudis, MD Divisions of Cardiovascular-Thoracic Surgery and Pediatric Otolaryngology, Department of Surgery, Northwestern University Medical School, The Children’s Memorial Hospital, Chicago, Illinois
A 5-month-old infant presented with respiratory failure secondary to severe right bronchial compression. Diagnostic imaging revealed a right aortic arch and absent left pulmonary artery. Surgical relief was obtained via median sternotomy by dividing a right ligamentum and pexing the enlarged right pulmonary artery to the ascending aorta. (Ann Thorac Surg 1999;67:1472– 4) © 1999 by The Society of Thoracic Surgeons
T
here are very few reports of vascular rings that can not be addressed through a left thoracotomy [1, 2]. We report the rare combination of a right aortic arch, right ligamentum arteriosum, and absent left pulmonary artery in a 5-month-old infant that presented with acute respiratory failure. Through a median sternotomy, a tight right ligamentum arteriosum (acting like a “bow-string”) and an enlarged right pulmonary artery were found compressing and nearly occluding the right mainstem bronchus. Relief of the right bronchial compression was obtained by dividing the ligamentum and pexing the right pulmonary artery to the aorta. A 5-month-old girl presented with a 2-day history of clear rhinitis, coughing, wheezing, and tachypnea with fever. Chest roentgenogram showed a hyperinflated right lung and right upper and lower lobe atelectasis. Progressive respiratory failure despite bronchodilators led to endotracheal intubation and mechanical ventilation. Progressive respiratory acidosis motivated a transfer to our institution. Initial arterial blood gas (pH 7.17, pCO2 of 78 mm Hg, pO2 84 mm Hg) was on an FiO2 of 70%. Bronchoscopy demonstrated extrinsic anterior nonpulsatile compression of the right mainstem bronchus, causing nearly complete occlusion of this bronchus. The trachea and left main bronchus were normal. The bronchoscope did pass into the right main bronchus and there was purulent material suctioned from the distal bronchus. Computerized tomography scan of the chest revealed a right aortic arch, compression of the right main Accepted for publication Oct 10, 1998. Address reprint requests to Dr Backer, Division of CardiovascularThoracic Surgery, The Children’s Memorial Hospital, m/c 22, 2300 Children’s Plaza, Chicago, IL 60614; e-mail: [email protected].
0003-4975/99/$20.00 PII S0003-4975(99)00189-7
Ann Thorac Surg 1999;67:1472– 4
CASE REPORT DODGE-KHATAMI ET AL RIGHT ARCH, RIGHT LIGAMENTUM, ABSENT LPA
1473
Fig 1. Aortogram demonstrates a right aortic arch, mirror image branching of the brachiocephalic vessels, and a ductal “bump” that was consistent with the noted intraoperative right ligamentum arteriosum.
Fig 2. Contrast bronchogram with simultaneous levophase aortogram demonstrating a normal trachea and left main bronchus, with severe narrowing of the right mainstem bronchus at a site between the right descending aorta and right pulmonary artery.
bronchus, and the left pulmonary artery could not be identified. An echocardiogram was performed to rule out pulmonary artery sling [3]. This demonstrated normal intracardiac anatomy, but again no evidence of a left pulmonary artery. Cardiac catheterization with simultaneous contrast tracheo-bronchography demonstrated a large right pulmonary artery with compression of the right mainstem bronchus between the pulmonary artery anteriorly and the right-sided descending thoracic aorta posteriorly (Figs 1–3). A retrograde injection of the left pulmonary veins revealed a tiny intraparenchymal left pulmonary artery that did not communicate with the main pulmonary artery. Without a precise preoperative diagnosis, we elected to approach the right bronchial compression through a median sternotomy. The pericardium was opened and a right aortic arch noted. There was no evidence of a left-sided ligamentum arteriosum or intrapericardial left pulmonary artery. A ligamentum arteriosum was identified coming off the postero-superior aspect of the right pulmonary artery and inserting into the right descending thoracic aorta. The right ligamentum was doubly ligated and divided. The right pulmonary artery was fully mobilized to rule out a pulmonary artery sling. Intraoperative bronchoscopy was performed and visualized a now improved opening of the right main bronchus. This opening was further improved (to near normal) by suspending the right pulmonary artery to the posterior aspect of the ascending aorta. Peak airway pressures required for adequate ventilation dropped from 42 to 28 mm Hg. The patient was extubated on the first postoperative day. Bronchoscopic follow-up 2 weeks, 3 months, and 10 months postsurgery revealed a slightly “flattened” but patent right mainstem bronchus, and the child has con-
tinued to be symptom-free except for rare intermittent episodes of reactive airway disease.
Comment We recently reported our experience with nearly 300 patients with vascular rings over a 5-year time span [4]. In that series, this is the first child with a right aortic arch, right ligamentum, and absent left pulmonary artery. Before this, all of the patients with a vascular ring caused
Fig 3. Pulmonary angiogram illustrates a large right pulmonary artery, no left pulmonary artery, and no evidence for a pulmonary artery sling.
1474
CASE REPORT TAKATA ET AL NEURILEMOMA OF THE STERNUM
by a right aortic arch (79 patients) had the ring completed by a left ligamentum. In this patient, there was not a true “complete” ring, rather the right ligamentum acting like a “bow-string” along with the enlarged right pulmonary artery. The right pulmonary artery was enlarged because of the absent left pulmonary artery, with the entire cardiac output passing through the right pulmonary artery. This has been called a “hemodynamic vise.” We have categorized patients such as this with those with “complete” vascular rings because of the similarities in clinical presentation, diagnostic techniques, and surgical intervention. Robotin and colleagues from Paris reported an experience of more than 500 infants and children undergoing operation for symptomatic tracheo-bronchial compression [5]. They described a single patient with a right aortic arch and right descending aorta that had compression of the right main bronchus. They alleviated the bronchial compression by mobilizing the descending aorta through a right thoracotomy. That child did have a left pulmonary artery and had a patent ductus arteriosus, presumably on the left side (this was not stated). Do¨hlemann and colleagues reported 3 infants with hypoplasia or aplasia of the right lung, dextrocardia, and compression of the trachea by the ascending aorta as it traversed from right to left [6]. In a sense, these are “mirror images” of our patient, the difference being that despite having an absent left pulmonary artery, our patient had a normal-sized left lung and levocardia, hence, the great vessel orientation was essentially normal for a right arch. Despite a thorough preoperative work-up, failure to precisely determine the nature of the compression was understandable given the unusual pathology. We therefore elected a median sternotomy approach. This allowed direct visualization of any type of vascular ring and also permitted cardiopulmonary bypass, if necessary, for repair of pulmonary artery sling [7]. Intraoperative bronchoscopy is an invaluable diagnostic tool that allows direct assessment of the extrinsic compression of the airways, and documents the adequacy of repair. This illustrates how multiple diagnostic modalities may be necessary in the unusual patient with a vascular ring that does not fall into the “neat” diagnostic categories of double aortic arch, right aortic arch with left ligamentum, innominate artery compression syndrome, and pulmonary artery sling [8]. Although the usual tendency is to obtain too many studies that simply continue to verify the diagnosis, when the diagnostic studies are not typical, it is important to continue investigation as far as possible to insure appropriate surgical intervention. In this case, the final (surgical) diagnosis led to successful airway relief by combining division of the right ligamentum arteriosum and pexing of the right pulmonary artery to the aorta.
We thank Dr David Wax for providing the cardiac catheterization image.
© 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1474 – 6
References 1. McFaul R, Millard P, Norwicki E. Vascular rings necessitating right thoracotomy. J Thorac Cardiovasc Surg 1981;82:306–9. 2. Whitman G, Stephenson LW, Weinberg P. Vascular ring: left cervical aortic arch, right descending aorta, and right ligamentum arteriosum. J Thorac Cardiovasc Surg 1982;83:311–5. 3. Alboliras ET, Backer CL, Holinger LD, et al. Pulmonary artery sling: diagnostic and management strategy. Pediatrics 1996; 98:530(Abstr). 4. Backer CL, Mavroudis C. Surgical approach to vascular rings. In: Karp RB, Laks H, Wechsler AS, eds. Advances in cardiac surgery. Chicago: Mosby-Year Book, 1997:29– 64. 5. Robotin MC, Bruniaux J, Serraf A, et al. Unusual forms of tracheobronchial compression in infants with congenital heart disease. J Thorac Cardiovasc Surg 1996;112:415–23. 6. Do¨hlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy. J Pediatr Surg 1990;25:290–3. 7. Backer CL, Idriss FS, Holinger LD, et al. Pulmonary artery sling: results of surgical repair in infancy. J Thorac Cardiovasc Surg 1992;103:683–91. 8. Lowe GM, Donaldson JS, Backer CL. Vascular rings: 10-year review of imaging. Radiographics 1991;11:637– 46.
Intraosseous Neurilemoma of the Sternum Kohei Takata, MD, Kosuke Okuda, MD, and Mitsuo Ochi, MD Department of Orthopaedics, Shimane Medical University, Izumo, Japan.
Sternal benign bone tumor is uncommon. We report a very rare case of intraosseous neurilemoma in the sternum. A girl, 10 years of age, was diagnosed as having a sternal tumor. Computed tomographic scan disclosed a mushroom-shape tumor in the region from inside of the sternum to the ventral soft tissues. Surgical resection was performed. Histopathologic findings showed a typical neurilemoma with Antoni type A and B patterns. No recurrence has been found. (Ann Thorac Surg 1999;67:1474 – 6) © 1999 by The Society of Thoracic Surgeons
P
rimary bone tumors of the chest wall are uncommon [1]. Among those tumors, sternal tumors are rare and usually malignant [2]. On the other hand, intraosseous neurilemoma is rare and accounts for less than 1% of primary bone tumors [3]. We report an extremely rare case of neurilemoma developed in the sternum. A 10-year-old girl was referred to our department in December 1992. A tumor of 5 3 4 cm was found at the sternal region. Plain radiogram showed a round translu-
Accepted for publication Oct 12, 1998. Address reprint requests to Dr Takata, Department of Orthopaedics, Shimane Medical University, 89-1 Enyacho, Izumoshi, Japan 693-8501.
0003-4975/99/$20.00 PII S0003-4975(99)00237-4