Sternal cleft associated with vascular anomalies and micrognathia

CASE REPORT PASIC ET AL STERNAL CLEFT AND VASCULAR ANOMALIES

Ann Thorac Surg 1993;56 165-8

c,

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References

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Fig 2. The double-armed 2-0 Ethibond mattress suture (M) is passed through a Starr-Edwards prosthesis saving ring, and the patch is sutured on the endocardia1 atrial surface. The atrioventricular continuity is restored.

Comment Left ventricular rupture is one of the most feared complications after mitral valve replacement and was first described in 1967 [9]. Several techniques have been proposed for repair, but mortality is still high [3, 7, 101, so the best approach is prevention [ll].Type I myocardial rupture (atrioventricular discontinuity) is related to excessive stretching or to a too radical excision of the posterior leaflet, particularly in a massively calcified leaflet. However, it is not always possible to avoid complete resection of the posterior leaflet; when it is extensively calcified (with calcification embedded in the posterior annulus) leaving intact the entire calcified mass or resecting too cautiously the leaflet and the subvalvular apparatus can result in prosthesis undersizing or in its early dehiscence. Once the atrioventricular discontinuity occurs, different reconstructive techniques can be used for repair [7]. We think that it is absolutely essential to rebuild firm atrioventricular continuity to counteract the dynamic forces of the left ventricle that tend to further disrupt the primary tear in the atrioventricular groove during systole and diastole. A straddling pericardial patch is characterized by a low compliance and actually counteracts ventricular dynamic forces that are equally distributed along the suture seam. Another advantage is that, like pledgets, it allows a better anchorage of sutures with lower risks of annular tear. In this report the straddling pericardial patch was used for preventing the myocardial rupture as soon as the surgeon realized that the atrioventricular continuity had been jeopardized. The operative and postoperative course was uneventful. In a previous experience [8] a similar technique had been successfully used in 2 patients with established type I rupture. Very few patients need to be treated with this technique, but we believe that it can be proposed as a technique of choice for treatment and prevention of myocardial rupture at the atrioventricular junction.

1. Zacharias A, Groves LK, Cheanvechai C, Effler DB. Rupture of the posterior wall of the left ventricle after mitral valve replacement. J Thorac Cardiovasc Surg 1975;69:259-64. 2. Stephenson LW, MacVaugh H, Edmunds LH. Surgery using cardiopulmonary bypass in the elderly. Circulation 1978;58: 250-61. 3. Karlson KJ, Ashraf MM, Berger RL. Rupture of the left ventricle following mitral valve replacement. Ann Thorac Surg 1988;46:590-7. 4. Treasure RL, Rainer WG, Streavey TE, Sadler TR. Intraoperative left ventricle rupture associated with mitral valve replacement. Chest 1974;66:511-9. 5. Miller DW, Johnson DD, Ivey TD. Does preservation of the posterior chordae tendinae enhance survival during mitral valve replacement? Ann Thorac Surg 1979;28:22-7. 6. Mills NL, McIntosh CL, Millo LJ. Techniques for management of the calcified mitral anulus. J Cardiac Surg 1986;l: 347-51. 7. Cohn LH. Management of complications related to mitral valve surgery. In: Waldhausen JA, Orringer ME, eds. Complications in cardiothoracic surgery. St. Louis: Mosby-Year Book, 1991:24%57. 8. Arena V, Alamanni F, Repossini A, et al. Riparazione della rottura ventricolare dopo sostituzione valvolare mitralica con patch atrio-ventricolare. Arch Chir Torac Cardiovasc 1992;14: 611. 9. Roberts WC, Morrow AG. Causes of early postoperative death following cardiac valve replacement: clinico-pathologic correlations in 64 patients studied at necropsy. J Thorac Cardiovasc Surg 1967;54:422-31. 10. Jacobowitz IJ, Sabado M, Zisbrod Z , et al. Repair of ventricular rupture following mitral valve replacement. J Cardiovasc Surg 1988;29:399405. 11. Danielson GK, Cooper E, Tweeddale DN. Circumflex coronary artery injury during mitral valve replacement. Ann Thorac Surg 1967;4:5%9.

Sternal Cleft Associated With Vascular Anomalies and Micrognathia Miralem Pasic, MD, ScD, Thierry Carrel, MD, Martin Tonz, MD, Urs Niederhauser, MD, Ludwig K. Von Segesser, MD, and Marko I. Turina, MD Clinic for Cardiovascular Surgery, University Hospital Zurich, Zurich, Switzerland

Sternal defects combined with craniofacial vascular defects are rare. We report on a 45-year-old woman with a sternal cleft associated with craniofacial and brain hemangiomata, an aneurysm of the aortic arch, anomalous origin of the coronary arteries, a left superior vena cava, micrognathia, supraumbilical midline raphe, and a cervical cyst. The surgical procedure consisted of the resection and replacement of the aortic arch and the innominate artery with reimplantation of the left carotid artery into the graft under circulatory arrest and deep hypotherAccepted for publication Sep 11, 1992 Address reprint requests to Dr Pasic, Clinic for Cardiovascular Surgery, University Hospital Zurich, Ramistrasse 100, CH-8091 Zurich, Switzerland.

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Ann Thorac Surg 1993;56:165-8

mia. The presence of sternal cleft is an indication for the search for other asymptomatic internal vascular anoma(Ann Thorac Surg 1993;56:165-8) lies.

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artial or complete sternal cleft (sternum bifidum) presents as an isolated anomaly [l], or can be combined with other lesions, such as facial angiomatosis [2], mentosternal cleft [3], a supraumbilical midline raphe and a chromosome abnormality [4], ectopia cordis, frontonasal dysgenesis and amniotic bands [5], or facial angiomatosis and aneurysm of the ascending aorta [6]. The pathogenesis of an unusual combination of sternal defect associated with superficial craniofacial vascular and internal vascular lesion is unknown [2]. We report a case of a sternal cleft combined with craniofacial and brain hemangiomata, an aneurysm of the aortic arch, anomalous origin of the coronary arteries, micrognathia, supraumbilical raphe, and a cervical cyst. A 45-year-old woman underwent operation for an asymp-

tomatic aneurysm of the proximal aortic arch involving the innominate artery and the left carotid artery. This finding was accidentally diagnosed during check-up for operation on a left-sided inguinal hernia 6 months earlier. The family history was unremarkable. She was born with hemangioma of the face and the neck and micrognathia after uncomplicated pregnancy. The hemangiomata were successfully treated in infancy by irradiation. Also, because of present micrognathia and probable mandibular

Fig 1 . Preoperative angiogram reveals a saccular aneurysm of the proximal part of the aortic arch involving the innominate artery and the left carotid artery.

Fig 2. Preoperative chest computed tomographic scan (demonstrates sternal cleft and the presence of a calcified atherosclerotic saccular aneurysm of the aortic arch beneath the skin (arrow). Physical examination revealed prominent aortic pulsations in the sternal notch, cleft of the superior half of the sternum, and systolic murmur over cleft.

defects she had undergone several mandibular surgical procedures with bone transplant grafting in childhood. On chest roentgenograms, superior mediastinal widening was noted. Echocardiography, computed tomographic scan, cardiac catheterization, and aortography showed marked saccular aneurysm of the aortic arch involving the proximal part of the innominate artery as well as the origin of the left carotid artery (Fig l),and a left superior vena cava. No other cardiovascular abnormalities were defined. Electrocardiographic and laboratory findings were unremarkable. Physical examination revealed prominent aortic pulsations in the sternal notch with cleft of the superior half of the sternum (Figs 2,3) and a systolic murmur over the cleft. Micrognathia with skin scars over the mandibular and both anterolateral sides of the neck were present. A supraumbilical raphe in a form of a very fine supraumbilical skin scar was noted, which appeared to be an old laparotomy scar, but no abdominal operation had been performed. The thorax was opened through a midline sternotomy, and only the middle third of the sternum was not cleft. A cervical cyst was found and excised. Cardiopulmonary bypass was instituted by cannulating the right common femoral artery and the right atrium, and myocardial protection was achieved by application of continuous retrograde cold blood cardioplegia into the coronary sinus. The operation was performed during circulatory arrest and deep hypothermia with a nasopharyngeal temperature of 18°C. The operative findings included a saccular aneurysm of the proximal aortic arch affecting the proximal part of the innominate artery, and atherosclerotic changes of the distal innominate artery and the part of the aortic arch between the left carotid and the left subclavian artery. The aneurysm of the proximal aortic arch and the proximal

Ann Thorac Surg 1993;56:165-8

part of the innominate artery was excised, and inspection of both the distal part of the innominate artery and the nondilated part of the aortic arch between the left carotid artery and the left subclavian artery showed stenotic atherosclerotic plaques; therefore, these atherosclerotic but not dilated parts were also removed. The left inferior thyroid artery anomalously arising from the innominate artery was dissected. A 20-mm Dacron graft was anastomosed distally, just proximal to the origin of the left subclavian artery, and then the left carotid artery was reimplanted into the graft. The resected part of the innominate artery was replaced with a 10-mm polytetrafluoroethylene graft and reimplanted into the replaced aortic arch. Proximal anastomosis was performed about 1 cm above the ostia of the coronary arteries, which both arose anomalously from the anomalously positioned sinus in the posterior location. Although the ascending aorta showed no macroscopic atherosclerotic changes, the ascending aortic wall was very thin, and therefore felt was used to support the proximal anastomosis of the graft on the ascending aorta. The circulatory deep hypothermic arrest lasted 28 minutes, and the total aortic clamping time was 46 minutes with a total extracorporeal circulation time of 83 minutes. The inferior part of the sternum was approximated with three sternal wires (Fig 4). The postoperative course was uneventful, and the patient was discharged from the hospital on the seventh postoperative day. A brain hemangioma was found as an accidental finding during postoperative intravenous digital subtraction angiography.

Comment Partial or complete sternal cleft is a rare congenital anomaly. Associated internal vascular lesions were frequently

Fig 3 . Preoperative computed tomographic scan of the chest showing sternal cleft (arrow) at the level of the bifurcation of the main pulmonary trunk.

CASE REPORT PASIC EI AL S7 hRNAL CLtFT AND VASCULAR ANOMALIES

167

Fig 4 . Postoperative lateral chest roentgenogram showing only three sternal wire sutures placed in the inferior part of the sternum, indicating partial sternal cleft.

identified in this rare condition with sternal defect and cutaneous craniofacial hemangiomata [2, 61. It appears to have different causes, such as acute amnion rupture in gestation with changes dependent on the timing of the amnion rupture [5]. Hersh and associates [2] emphasized that in most known cases with the sternal malformation/ vascular dysplasia association no teratogen has been identified as a cause for clinical manifestations. In a report on 2 patients with sternal cleft and cutaneous, craniofacial hemangiomata and internal vascular lesions involving in 1 case the upper respiratory tract and in the other the viscera, they presumed that an early disturbance affecting midline mesodermal structures leading to lack of complete fusion of lateral sternal bands and overlying cutaneous tissue, or deficient formation of a proposed medioventral unpaired structure, which may be involving in the formation of the sternum, and persistence and proliferation of midline angioblastic tissue may be a possible mechanism during the sixth to ninth gestational weeks 121. The cause of the aortic arch aneurysm of our patient is unclear. The finding of craniofacial and brain hemangiomata suggests vascular dysplasia as a cause of this aneurysm, but the role of irrddldtl~ntherapy performed 111 this patient at a yourig age for hemangiomatosis cannot be excluded. The specimen of the aorta showed two different types of changes: atherosclerotic plaques in the aneurysmatic sac and around the origins of the great vessels and thin aortic wall showing macroscopically no atherosclerotic changes out of the aneurysmal sac. Pathohistologic examination revealed two substrates of aortic wall pathology: gross calcified atherosclerotic process in the aortic arch and significant changes of the media with lack of the elastic lamellae of the resected part of the ascending aorta.

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CASE REPORT I N 0 ET AL BALLOON MITRAL COMMISSUROTOMY

Partial sternal cleft was an accidental finding in our middle-aged patient, and because of this age the sternum was not repaired. Direct approximation of the two sternal halves is possible in the neonatal period; an autogenous periostial graft should be used for older infants [7]. The result of the surgical treatment of sternal cleft is better and operation is easier when performed shortly after birth, while the bony thorax is still compliant [8]. In conclusion, patients with sternal malformation associated with hemangioma are exposed to enhanced risk of other potentially life-threatening internal vascular malformations. In an asymptomatic patient with sternal cleft, careful investigation is indicated to search for other coexisting vascular changes. Surgical therapy of aortic changes is the optimal treatment of this anomaly and should be undertaken as soon as the diagnosis is made.

References 1. Petersen KK, Rasmussen OS, Jurik AG. Complete sternal cleft. Rontgenblatter 1989;42:5256. 2. Hersh JH, Waterfill D, Rutledge J, et al. Sternal malformation/ vascular dysplasia association. Am J Med Genet 1985;Zl: 177-86. 3. Breton P, Dodat H, Freidel M. Un syndrome rnalformatif cervical rare: la fissure mento-sternale. A propos de 4 observations. Chir Pediatr 1987;28:17&4. 4. Stoll C, Vivier M, Renaud R. A supraumbilical midline raphe with sternal cleft in a 47,XXX woman. Am J Med Genet 1987;27:229-31. 5. Van Allen MI, Myhre S. Ectopia cordis thoracalis with craniofacial defects resulting from early amnion rupture. Teratolgy 1985;32:19-24. 6. Schieken LS, Brenner JI, Baker KR, Ringel RE, Pacific0 A. Aneurysm of the ascending aorta associated with sternal cleft, cutaneous hemangioma, and occlusion of the right innominate artery in a neonate. Am Heart J 1987;113:2024. 7. Valla JS, Bechraoui T, Belghith M, Daoud N, Grinda A. Fissure sternale congenitale. Fermeture par greffon perioste. Chir Pediatr 1989;30:219-21. 8. Salley RK, Stewart S. Superior sternal cleft: repair in the newborn. Ann Thorac Surg 1985;39:582-3.

Successful Balloon Mitral Commissurotomy in a Small Child: Use of Small Inoue Balloon Catheter Toshihiro Ino, MD, Shinjiro Shimazaki, MD, Masazumi Iwahara, MD, Eiji Miyazaki, MD, Keijiro Yabuta, MD, and Kanji Inoue, MD Department of Pediatrics, Juntendo University School of Medicine, and Division of Cardiovascular Surgery, Takeda Hospital, Tokyo, Japan

A 15-month-old girl weighing 5.8 kg, in whom congestive heart failure developed due to congenital mitral Accepted for publication Sep 18, 1992 Address reprint requests to Dr Ino, Department of Pediatrics, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113, Japan.

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stenosis, was successfully treated by percutaneous transluminal mitral commissurotomy using a small Inoue balloon catheter. Percutaneous transluminal mitral commissurotomy using a small Inoue balloon catheter may be a first-choice treatment for small children with congenital mitral stenosis. (Ann Thoruc Surg 1993;56:268-70)

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ercutaneous transluminal mitral commissurotomy (PTMC) has recently become an accepted alternative to operation in adults with rheumatic mitral valve stenosis since Inoue and colleagues first reported the technique in 1984 [l]. However, there have been very few reports of PTMC in pediatric patients [24]. Moreover, there is some controversy among cardiologists in various countries as to whether a conventional balloon catheter or the Inoue balloon catheter should be selected in this setting. We report a small child weighing 5.8 kg with congestive heart failure due to severe congenital mitral stenosis who was treated successfully by PTMC employing a small Inoue balloon catheter. A 1-month-old female infant was referred to our hospital for evaluation of poor weight gain and heart murmur. She had been born after 42 weeks of gestation weighing 2,508 g. On admission, the patient was not cyanotic, but inactive and unresponsive. Minor anomalies such as a high-arched palate, micrognathia, low-set ears, and sacral dimple were also noted. Membranous atresia ani had been diagnosed at birth and subsequently treated by operation at the age of 3 months. On cardiovascular examination, there was a pansystolic murmur of grade 3/6 at the fourth intercostal space of the left sternal border and a high-pitched diastolic murmur of grade 216 in the Erb area. The opening snap of the mitral valve was not audible. An electrocardiogram revealed a left-axis deviation of -60 degrees and right ventricular hypertrophy (RV1 = 15 mV; SV6 = 13 mV), and a chest roentgenogram showed mild cardiomegaly with a cardiothoracic ratio of 0.60. Two-dimensional echocardiography revealed slightly restrictive motion of the mitral valve and a ventricular septa1 defect 6 to 7 mm in diameter at the membranous septum. The interventricular septum was shifted from right to left, suggesting right ventricular and pulmonary hypertension. Administration of digitalis and diuretic agents was started, and the cardiac failure was controlled. A chromosome examination showed a 46,XX, 6q+ karyotype. At the age of 15 months, the patient was readmitted for precise evaluation of cardiac anatomy and PTMC. Twodimensional and color Doppler echocardiography on admission showed progressive, restrictive motion of the mitral valves with a markedly enlarged left atrium. Both anterior and posterior leaflets of the mitral valves were thickened. There was a small membranous tissue just above the mitral valve, suggesting the presence of a supravalvar mitral ring. A short-axis view at the level of the mitral valve revealed a small orifice in the mitral valve with commissure fusion (Fig 1). The mitral ring size obtained from the long-axis view was 8 mm in diameter.