Postgraduate Medicine
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Mid-Aortic Syndrome Presenting With Unusual Stenoses K.M. Burgazli MD & Sabine H. Daebritz MD To cite this article: K.M. Burgazli MD & Sabine H. Daebritz MD (2014) Mid-Aortic Syndrome Presenting With Unusual Stenoses, Postgraduate Medicine, 126:3, 274-276 To link to this article: http://dx.doi.org/10.3810/pgm.2014.05.2776
Published online: 13 Mar 2015.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [University of California, San Diego]
Date: 05 November 2015, At: 19:07
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C L I N I C A L F E AT U R E S
Mid-Aortic Syndrome Presenting With Unusual Stenoses
Downloaded by [University of California, San Diego] at 19:07 05 November 2015
DOI: 10.3810/pgm.2014.05.2776
K.M. Burgazli, MD 1 Sabine H. Daebritz, MD 2 1 Wuppertal Research and Medical Center, Department of Internal Medicine and Angiology, Wuppertal, Germany; 2Department of Cardiovascular Surgery, EJK, Duisburg, Germany
Abstract: We report on the case of a 13-year-old female presenting with dizziness and nausea related to high blood pressure. A complete medical evaluation revealed that the patient had middle-aortic syndrome (MAS), in which there was a severe stenosis of the abdominal aorta that affected her renal and visceral arteries. If left untreated, this syndrome may lead to serious complications such as renal insufficiency or congestive heart failure. Therefore, the decision was made to perform a thoracoabdominal surgery with surgical patch augmentation. The patient had successful postoperative recovery. We have reported this case to draw attention to the coarctation of abdominal aorta, a condition which should be considered in pediatric patients with hypertension, and to emphasize a successful treatment approach for MAS. Keywords: mid-aortic syndrome; aortic coarcation; hypertension; aorto-aortic bypass; children
Introduction
Mid-aortic syndrome (MAS), also known as coarctation of abdominal aorta, is a rare entity characterized by progressive segmental narrowing of the distal thoracic and/or abdominal aorta and its major branches. The syndrome is often observed in young adults and children,1 is frequently congenital, and may present clinically as uncontrolled hypertension or lower-limb claudication.2 Diagnostic imaging by angiography of the descending and abdominal aorta is the preferred evaluative modality. Diminished renal function and uncontrollable hypertension are 2 important indications for surgical intervention.3 Although a variety of therapeutic options exist for managing patients with MAS, several publications have showed surgical treatment to be curative and the treatment of choice.4 We present a case of a 13-year-old female admitted to the clinic with dizziness and nausea caused by high blood pressure. She was successfully treated with surgical patch augmentation.
Case Report Correspondence: K. M. Burgazli, MD, Department of Internal Medicine and Angiology, Wuppertal Research and Medical Center, Wichlinghauser Str. 110/112, 42277, Wuppertal, Germany. Tel: +49-202-641-877 Fax: +49-202-640-620 E-mail: [email protected]
274
A 13-year-old female adolescent was admitted to an outpatient clinic with dizziness and nausea, which turned out to be related to high blood pressure. Her medical history was unremarkable. She was hospitalized by the Cardiology Department for further investigation. Use of an intracardiac catheter did not show any evidence of aortic coarctation; however, angiography and computerized tomography depicted MAS. Severe stenosis of the abdominal aorta was diagnosed, involving the celiac trunk, the renal arteries on both sides, and the superior and inferior mesenteric arteries (Figure 1). Additionally,
© Postgraduate Medicine, Volume 126, Issue 3, May 2014, ISSN – 0032-5481, e-ISSN – 1941-9260 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected] Warning: No duplication rights exist for this journal. Only JTE Multimedia, LLC holds rights to this publication. Please contact the publisher directly with any queries.
Downloaded by [University of California, San Diego] at 19:07 05 November 2015
Mid-Aortic Syndrome Presenting With Unusual Stenoses
there were collateral arteries between the intercostal vessels and the superior mesenteric artery. Histologic evaluation confirmed inflammation-free tunica intima with moderate hyperplasia and somewhat increased mucoid degeneration. The observed stenosis pattern did not allow us to confirm the case as congenital. After treatment options were discussed with the patient’s family, patch dilatation surgery was chosen for treatment. Patch dilatation of the abdominal aorta and visceral arteries was performed 3 months later under general anesthesia. Surgical access was achieved longitudinally from the navel left to the costal arch with lateral right position. External and internal abdominal oblique muscles and aponeurosis of transversus abdominis muscle were cut. After passing the 12th rib and cutting the cartilage of 11th rib, the pleural space was opened. In order to access the abdominal aorta, the peritoneal bag and left kidney were displaced to the right side. Crossing of lymphatic vessels, lymph nodes, and collaterals made surgery quite difficult. During the intervention, systemic heparin was continuously infused and the patient’s body temperature was slightly lowered to 33°C. Augmentation of the visceral arteries began at the celiac trunk, over to the superior mesenteric artery using patches (Figure 2). Renal arteries, the inferior mesenteric artery, lumbar arteries, and collaterals were included into the augmentation. Additionally, a thickened and uneven tunica intima layer was found in the left renal artery. The surgical procedure was completed without any complications. Although a moderate amount of free fluid was found early on postoperatively in the area of Douglas, sonographic controls for visceral organs were normal with regular peristalsis. Urine analysis and renal clearance were within
normal ranges. Postoperative magnetic resonance imaging showed successful results. The patient did not require any external blood products. On postoperative day 3, the patient was transferred to the general ward. At 1-year follow-up, the patient maintained normal blood pressure levels with the use of anti-hypertensive medication and she was asymptomatic.
Discussion
Mid-aortic syndrome is an uncommon variety of aortic coarctation, characterized by segmental narrowing of the abdominal aorta and its major branches.5 That MAS mostly appears in children and young adults, premature infants, and newborns has been described.6 It is most often congenital in origin; nevertheless, acquired cases of MAS can result from aortitis, atherosclerosis, neurocutaneous syndrome, and Williams syndrome, and should be considered in the differential diagnosis.7 Patients with MAS usually present with refractory arterial hypertension, which is typically severe and difficult to manage.8 Other clinical manifestations of MAS may include systolic ejection murmur, widespread bruits, and weak femoral pulses.9 If MAS is left untreated, severe hypertension can result in serious complications, including lower-limb claudication, renal insufficiency, and congestive heart failure8,10; therefore, early detection and treatment are essential for prevention of irreversible complications of Figure 2. Intraoperative views before (A) and after (B) surgical patch augmentation.
Figure 1. Angiography showing severe stenosis (arrows) in the abdominal aorta and visceral arteries.
© Postgraduate Medicine, Volume 126, Issue 3, May 2014, ISSN – 0032-5481, e-ISSN – 1941-9260 275 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected] Warning: No duplication rights exist for this journal. Only JTE Multimedia, LLC holds rights to this publication. Please contact the publisher directly with any queries.
Downloaded by [University of California, San Diego] at 19:07 05 November 2015
K. M. Burgazli and Sabine H. Daebritz
MAS. Magnetic resonance angiography or multidetector computed tomography can be used for evaluation; however, angiography of the aorta is the gold standard diagnostic test to confirm the diagnosis of MAS.11 Therapeutic approaches include medical management, endovascular treatment, and surgical intervention. Followup after a variety of revascularization techniques has shown successful results in maintaining patient renal function and relief of cardiac insufficiency9; however, several publications have showed surgical treatment to be curative and the treatment of choice.4 The timing of surgery depends on the age of the patient and severity of hypertension, and management of MAS should be individualized.12
Conclusion
In conclusion, our case highlights that although MAS remains uncommon (0.5% to 2% of all coarcations), the syndrome should be considered in the differential diagnosis of secondary hypertension, especially in young patients. Our case further confirmed that surgery remains the treatment of choice in patients with MAS, even if it is technically challenging.
3. Masterson R, Scoble J, Taylor P, Cook G. Recovery of renal function following prolonged ischaemia in a patient with Mid-Aortic Syndrome. Nephrol Dial Transplant. 2000;15(9):1461–1463. 4. Levart TK, Klokocovnik T. Mid-aortic syndrome in a 3-year-old girl successfully treated by aorto-aortic grafting and renal artery implantation into the graft. Tex Heart Inst J. 2012;39(5):657–661. 5. West CA, Delis KT, Service GJ, Driscoll DJ, McPhail IR, Gloviczki P. Middle aortic syndrome: surgical treatment in a child with neurofibromatosis. J Vasc Surg. 2005;42(6):1236. 6. Kocova M, Kacarska R, Sukarova-Angelovska E, Kuzmanovska D. New vascular anomaly in a girl with Turner Syndrome: Mid-aortic narrowing. J Med Cases. 2012;3(4):229–233. 7. Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE. Idiopathic mid-aortic syndrome in children. Pediatr Nephrol. 2008;23(7):1135–1142. 8. Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC. Mid-aortic syndrome: a case report and review of the literature. Int J Cardiol. 2008;123(3):348–352. 9. Kumar S, Bury RW, Roberts DH. Unusual case of refractory hypertension: late presentation of the mid-aortic syndrome. Heart. 2002;87(2):E3–E3. 10. Rooke TW, Joyce JW. Uncommon arteriopathies. In: Rutherford RB, ed. Vascular Surgery, 5th edition. Philadelphia, PA: Saunders, 2000: 418–434. 11. Das BB, Recto M, Shoemaker L, Mitchell M, Austin EH. Midaortic syndrome presenting as neonatal hypertension. Pediatr Cardiol. 2008;29(5):1000–1001. 12. Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ. Middle aortic syndrome. Effectiveness and durability of complex arterial revascularization techniques. Ann Surg. 1986;204(3):331–339.
Conflict of Interest Statement
K. M. Burgazli, MD, and Sabine H. Daebritz, MD, disclose no conflicts of interest.
References 1. Liu LYM, Tsai CT, Lan LWR, Hou CJY. Severe hypertension in a young adult resulting from middle aortic syndrome. Acta Cardiologica Sinica. 2008;24:43–46. 2. Bhatti AM, Mansoor J, Younis U, Siddique K, Chatta S. Mid aortic syndrome: a rare vascular disorder. J Pak Med Assoc. 2011;61(10):1018–1020.
276
© Postgraduate Medicine, Volume 126, Issue 3, May 2014, ISSN – 0032-5481, e-ISSN – 1941-9260 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected] Warning: No duplication rights exist for this journal. Only JTE Multimedia, LLC holds rights to this publication. Please contact the publisher directly with any queries.
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Mid-Aortic Syndrome Presenting With Unusual Stenoses K.M. Burgazli MD & Sabine H. Daebritz MD To cite this article: K.M. Burgazli MD & Sabine H. Daebritz MD (2014) Mid-Aortic Syndrome Presenting With Unusual Stenoses, Postgraduate Medicine, 126:3, 274-276 To link to this article: http://dx.doi.org/10.3810/pgm.2014.05.2776
Published online: 13 Mar 2015.
Submit your article to this journal
Article views: 3
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [University of California, San Diego]
Date: 05 November 2015, At: 19:07
All rights reserved: reproduction in whole or part not permitted. All permission requests to reproduce or adapt published material must be directed to the journal office in Conshohocken, PA, no othe rpersons of offices are authorized to act on our behalf.
Reprints: [email protected] -- [email protected]
C L I N I C A L F E AT U R E S
Mid-Aortic Syndrome Presenting With Unusual Stenoses
Downloaded by [University of California, San Diego] at 19:07 05 November 2015
DOI: 10.3810/pgm.2014.05.2776
K.M. Burgazli, MD 1 Sabine H. Daebritz, MD 2 1 Wuppertal Research and Medical Center, Department of Internal Medicine and Angiology, Wuppertal, Germany; 2Department of Cardiovascular Surgery, EJK, Duisburg, Germany
Abstract: We report on the case of a 13-year-old female presenting with dizziness and nausea related to high blood pressure. A complete medical evaluation revealed that the patient had middle-aortic syndrome (MAS), in which there was a severe stenosis of the abdominal aorta that affected her renal and visceral arteries. If left untreated, this syndrome may lead to serious complications such as renal insufficiency or congestive heart failure. Therefore, the decision was made to perform a thoracoabdominal surgery with surgical patch augmentation. The patient had successful postoperative recovery. We have reported this case to draw attention to the coarctation of abdominal aorta, a condition which should be considered in pediatric patients with hypertension, and to emphasize a successful treatment approach for MAS. Keywords: mid-aortic syndrome; aortic coarcation; hypertension; aorto-aortic bypass; children
Introduction
Mid-aortic syndrome (MAS), also known as coarctation of abdominal aorta, is a rare entity characterized by progressive segmental narrowing of the distal thoracic and/or abdominal aorta and its major branches. The syndrome is often observed in young adults and children,1 is frequently congenital, and may present clinically as uncontrolled hypertension or lower-limb claudication.2 Diagnostic imaging by angiography of the descending and abdominal aorta is the preferred evaluative modality. Diminished renal function and uncontrollable hypertension are 2 important indications for surgical intervention.3 Although a variety of therapeutic options exist for managing patients with MAS, several publications have showed surgical treatment to be curative and the treatment of choice.4 We present a case of a 13-year-old female admitted to the clinic with dizziness and nausea caused by high blood pressure. She was successfully treated with surgical patch augmentation.
Case Report Correspondence: K. M. Burgazli, MD, Department of Internal Medicine and Angiology, Wuppertal Research and Medical Center, Wichlinghauser Str. 110/112, 42277, Wuppertal, Germany. Tel: +49-202-641-877 Fax: +49-202-640-620 E-mail: [email protected]
274
A 13-year-old female adolescent was admitted to an outpatient clinic with dizziness and nausea, which turned out to be related to high blood pressure. Her medical history was unremarkable. She was hospitalized by the Cardiology Department for further investigation. Use of an intracardiac catheter did not show any evidence of aortic coarctation; however, angiography and computerized tomography depicted MAS. Severe stenosis of the abdominal aorta was diagnosed, involving the celiac trunk, the renal arteries on both sides, and the superior and inferior mesenteric arteries (Figure 1). Additionally,
© Postgraduate Medicine, Volume 126, Issue 3, May 2014, ISSN – 0032-5481, e-ISSN – 1941-9260 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected] Warning: No duplication rights exist for this journal. Only JTE Multimedia, LLC holds rights to this publication. Please contact the publisher directly with any queries.
Downloaded by [University of California, San Diego] at 19:07 05 November 2015
Mid-Aortic Syndrome Presenting With Unusual Stenoses
there were collateral arteries between the intercostal vessels and the superior mesenteric artery. Histologic evaluation confirmed inflammation-free tunica intima with moderate hyperplasia and somewhat increased mucoid degeneration. The observed stenosis pattern did not allow us to confirm the case as congenital. After treatment options were discussed with the patient’s family, patch dilatation surgery was chosen for treatment. Patch dilatation of the abdominal aorta and visceral arteries was performed 3 months later under general anesthesia. Surgical access was achieved longitudinally from the navel left to the costal arch with lateral right position. External and internal abdominal oblique muscles and aponeurosis of transversus abdominis muscle were cut. After passing the 12th rib and cutting the cartilage of 11th rib, the pleural space was opened. In order to access the abdominal aorta, the peritoneal bag and left kidney were displaced to the right side. Crossing of lymphatic vessels, lymph nodes, and collaterals made surgery quite difficult. During the intervention, systemic heparin was continuously infused and the patient’s body temperature was slightly lowered to 33°C. Augmentation of the visceral arteries began at the celiac trunk, over to the superior mesenteric artery using patches (Figure 2). Renal arteries, the inferior mesenteric artery, lumbar arteries, and collaterals were included into the augmentation. Additionally, a thickened and uneven tunica intima layer was found in the left renal artery. The surgical procedure was completed without any complications. Although a moderate amount of free fluid was found early on postoperatively in the area of Douglas, sonographic controls for visceral organs were normal with regular peristalsis. Urine analysis and renal clearance were within
normal ranges. Postoperative magnetic resonance imaging showed successful results. The patient did not require any external blood products. On postoperative day 3, the patient was transferred to the general ward. At 1-year follow-up, the patient maintained normal blood pressure levels with the use of anti-hypertensive medication and she was asymptomatic.
Discussion
Mid-aortic syndrome is an uncommon variety of aortic coarctation, characterized by segmental narrowing of the abdominal aorta and its major branches.5 That MAS mostly appears in children and young adults, premature infants, and newborns has been described.6 It is most often congenital in origin; nevertheless, acquired cases of MAS can result from aortitis, atherosclerosis, neurocutaneous syndrome, and Williams syndrome, and should be considered in the differential diagnosis.7 Patients with MAS usually present with refractory arterial hypertension, which is typically severe and difficult to manage.8 Other clinical manifestations of MAS may include systolic ejection murmur, widespread bruits, and weak femoral pulses.9 If MAS is left untreated, severe hypertension can result in serious complications, including lower-limb claudication, renal insufficiency, and congestive heart failure8,10; therefore, early detection and treatment are essential for prevention of irreversible complications of Figure 2. Intraoperative views before (A) and after (B) surgical patch augmentation.
Figure 1. Angiography showing severe stenosis (arrows) in the abdominal aorta and visceral arteries.
© Postgraduate Medicine, Volume 126, Issue 3, May 2014, ISSN – 0032-5481, e-ISSN – 1941-9260 275 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected] Warning: No duplication rights exist for this journal. Only JTE Multimedia, LLC holds rights to this publication. Please contact the publisher directly with any queries.
Downloaded by [University of California, San Diego] at 19:07 05 November 2015
K. M. Burgazli and Sabine H. Daebritz
MAS. Magnetic resonance angiography or multidetector computed tomography can be used for evaluation; however, angiography of the aorta is the gold standard diagnostic test to confirm the diagnosis of MAS.11 Therapeutic approaches include medical management, endovascular treatment, and surgical intervention. Followup after a variety of revascularization techniques has shown successful results in maintaining patient renal function and relief of cardiac insufficiency9; however, several publications have showed surgical treatment to be curative and the treatment of choice.4 The timing of surgery depends on the age of the patient and severity of hypertension, and management of MAS should be individualized.12
Conclusion
In conclusion, our case highlights that although MAS remains uncommon (0.5% to 2% of all coarcations), the syndrome should be considered in the differential diagnosis of secondary hypertension, especially in young patients. Our case further confirmed that surgery remains the treatment of choice in patients with MAS, even if it is technically challenging.
3. Masterson R, Scoble J, Taylor P, Cook G. Recovery of renal function following prolonged ischaemia in a patient with Mid-Aortic Syndrome. Nephrol Dial Transplant. 2000;15(9):1461–1463. 4. Levart TK, Klokocovnik T. Mid-aortic syndrome in a 3-year-old girl successfully treated by aorto-aortic grafting and renal artery implantation into the graft. Tex Heart Inst J. 2012;39(5):657–661. 5. West CA, Delis KT, Service GJ, Driscoll DJ, McPhail IR, Gloviczki P. Middle aortic syndrome: surgical treatment in a child with neurofibromatosis. J Vasc Surg. 2005;42(6):1236. 6. Kocova M, Kacarska R, Sukarova-Angelovska E, Kuzmanovska D. New vascular anomaly in a girl with Turner Syndrome: Mid-aortic narrowing. J Med Cases. 2012;3(4):229–233. 7. Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE. Idiopathic mid-aortic syndrome in children. Pediatr Nephrol. 2008;23(7):1135–1142. 8. Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC. Mid-aortic syndrome: a case report and review of the literature. Int J Cardiol. 2008;123(3):348–352. 9. Kumar S, Bury RW, Roberts DH. Unusual case of refractory hypertension: late presentation of the mid-aortic syndrome. Heart. 2002;87(2):E3–E3. 10. Rooke TW, Joyce JW. Uncommon arteriopathies. In: Rutherford RB, ed. Vascular Surgery, 5th edition. Philadelphia, PA: Saunders, 2000: 418–434. 11. Das BB, Recto M, Shoemaker L, Mitchell M, Austin EH. Midaortic syndrome presenting as neonatal hypertension. Pediatr Cardiol. 2008;29(5):1000–1001. 12. Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ. Middle aortic syndrome. Effectiveness and durability of complex arterial revascularization techniques. Ann Surg. 1986;204(3):331–339.
Conflict of Interest Statement
K. M. Burgazli, MD, and Sabine H. Daebritz, MD, disclose no conflicts of interest.
References 1. Liu LYM, Tsai CT, Lan LWR, Hou CJY. Severe hypertension in a young adult resulting from middle aortic syndrome. Acta Cardiologica Sinica. 2008;24:43–46. 2. Bhatti AM, Mansoor J, Younis U, Siddique K, Chatta S. Mid aortic syndrome: a rare vascular disorder. J Pak Med Assoc. 2011;61(10):1018–1020.
276
© Postgraduate Medicine, Volume 126, Issue 3, May 2014, ISSN – 0032-5481, e-ISSN – 1941-9260 ResearchSHARE®: www.research-share.com • Permissions: [email protected] • Reprints: [email protected] Warning: No duplication rights exist for this journal. Only JTE Multimedia, LLC holds rights to this publication. Please contact the publisher directly with any queries.
