Congenital absent sternum is a rare birth defect that requires early intervention for optimal long-term outcomes. Descriptions of the repair of absent sternum are limited to case reports, and no preferred method for management has been described. Herein, we describe the use of porcine acellular dermal matrix to reconstruct the sternum of an infant with sternal infection following attempted repair using synthetic mesh. The patient was a full-term male with trisomy 21, agenesis of corpus callosum, ventricular septal defect, patent ductus arteriosus, right-sided aortic arch, and congenital absence of sternum with no sternal bars. Following removal of the infected synthetic mesh, negative pressure wound therapy with instillation was used to manage the open wound and provide direct antibiotic therapy. When blood C-reactive protein levels declined to ≤2 mg/L, the sternum was reconstructed using porcine acellular dermal matrix. At 21 months postoperative, the patient demonstrated no respiratory issues. Physical examination and computed tomography imaging identified good approximation of the clavicular heads and sternal cleft and forward curvature of the ribs. This case illustrates the benefits of negative pressure wound therapy and acellular dermal matrix for the reconstruction of absent sternum in the context of infected sternal surgical site previously repaired with synthetic mesh.
Acellular Dermis ; Agenesis of Corpus Callosum ; Aorta, Thoracic ; C-Reactive Protein ; Congenital Abnormalities ; Down Syndrome ; Ductus Arteriosus, Patent ; Early Intervention (Education) ; Head ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Male ; Methods ; Negative-Pressure Wound Therapy ; Physical Examination ; Ribs ; Sternum ; Surgical Mesh ; Thoracic Surgery ; Wounds and Injuries

The cisterna chyli, a dilated lymphatic sac in the retrocrural space, is usually located to the right of the aorta. We report a case of a left-sided cisterna chyli, which was incidentally detected on the radiologic examinations of a preoperative workup for cholangiocarcinoma. Computed tomography (CT) and magnetic resonance (MR) images revealed a cisterna chyli measuring 2.5 cm in length in the left retrocrural space. The dilated lumbar lymphatics joined with the cisterna chyli, which was continuous with the left-sided thoracic duct. To the best of our knowledge, this is the second antemortem case of a left-sided cisterna chyli in literature. The cisterna chyli can mimic retrocrural lymphadenopathy, solid tumor with cystic degeneration, abscess or hematoma. The left-sided cisterna chyli should be referred to as a structure so as to be cautious in surgical approach.
Abscess ; Aorta ; Cholangiocarcinoma ; Hematoma ; Lymphatic Abnormalities ; Lymphatic Diseases ; Thoracic Duct

Robotically assisted mitral valve repair has proven its efficacy during the last decade. The most suitable approach for patients with difficult anatomies, such as morbid obesity, sternal deformities, cardiac rotation, or vascular anomalies, represents a current challenge in cardiac surgery. Herein, we present the case of a 71-year-old patient affected by severe degenerative mitral valve regurgitation with pectus excavatum and a right aortic arch with an anomalous course of the left subclavian artery who was successfully treated using a Da Vinci–assisted approach.
Aged ; Aorta, Thoracic ; Congenital Abnormalities ; Funnel Chest ; Humans ; Mitral Valve Insufficiency ; Mitral Valve ; Obesity, Morbid ; Subclavian Artery ; Thoracic Surgery

OBJECTIVE: Duplication of the vertebral artery (VA) is a rare vascular variant. This paper describes the anatomy and embryological development of duplicated VAs and reviews the clinical significance.METHODS: Computed tomography (CT) angiography was performed in 3386 patients (1880 females, 1506 males) between March 2014 and November 2015. We defined duplication of the VA as a condition in which the VA has two origins that fused at different levels of the neck.RESULTS: Ten of the 3386 patients (0.295%) who received CT angiography had a dual origin of the VA; three on the left side, and seven on the right side. In all seven with right dual origin of the VA, both limbs of the VA origin originated from the right subclavian artery. In all three patients with left dual origin of the VA, both limbs of the VA originated from the left subclavian artery and aortic arch. In all 10 patients, the medial limb of the duplicated VA was located posteriorly and medially to the common carotid artery (CCA) and anteriorly and laterally to the vertebral transverse foramen. In two patients, the medial limb of the duplicated VA was located in close proximity to the CCA. In another two patients, the medial limb of the duplicated VA was located in close proximity to the CCA, carotid bifurcation, and proximal internal carotid artery.CONCLUSION: Although duplication of the VA is asymptomatic in most patients, clinicians should consider this anomaly during diagnosis and treatment.
Angiography ; Aorta, Thoracic ; Carotid Artery, Common ; Carotid Artery, Internal ; Congenital Abnormalities ; Diagnosis ; Embryonic Development ; Extremities ; Female ; Humans ; Neck ; Pregnancy ; Subclavian Artery ; Vertebral Artery

BACKGROUND AND OBJECTIVES: Thoracic endovascular aortic repair exhibits limitations in cases where the aortic pathology involves the aortic arch. We had already developed a fenestrated aortic stent graft (FASG) with a preloaded catheter for aortic pathology involving the aortic arch. FASG was suitable for elective cases. MATERIALS AND METHODS: An aortic arch stent graft with a window-shaped fenestration (FASG-W) for supra-aortic arch vessels is suitable for emergent cases. This study aims to test a FASG-W for supra-aortic arch vessels and to perform a preclinical study in swine to evaluate the safety and efficacy of this device. Six FASG-Ws with 1 preloaded catheter were advanced through the iliac artery in 6 swine. The presence of endoleak and the patency and deformity of the grafts were examined with computed tomography (CT) at 4 weeks postoperatively. A postmortem examination was performed at 8 weeks. The mean procedure time for FASG-W was 27.15±4.02 minutes. The mean time for the selection of the right carotid artery was 5.72±0.72 minutes. RESULTS: Major adverse events were not observed in any of the 6 pigs who survived for 8 weeks. For the FASG-W, no endoleaks, no disconnection, and no occlusion of the stent grafts were observed in the CT findings or the postmortem gross findings. CONCLUSION: The procedure with the FASG-W was able to be performed safely in a relatively short procedure time and involved an easy technique. The FASG-W was found to be safe and convenient for use in this preclinical study of swine.
Animal Experimentation ; Aorta, Thoracic* ; Aortic Aneurysm, Thoracic ; Aortic Diseases ; Autopsy ; Blood Vessel Prosthesis* ; Carotid Arteries ; Catheters ; Congenital Abnormalities ; Endoleak ; Iliac Artery ; Pathology ; Stents* ; Swine* ; Transplants

This study evaluated the myocardial strain and aortic elasticity in patients with bicuspid aortic valve (BAV) and then investigated the relation between them. Thirty-nine patients (30 males; mean age 44±19 years; range 6 to 75 years) with BAV were recruited as BAV group, and 29 age- and sex-matched healthy controls (21 males; mean age 42±11 years; range 20 to 71 years) served as control group. Aortic strain, distensibility and stiffness index were derived using M-mode echocardiography. Left ventricular global myocardial strain was acquired with speckle-tracking echocardiography. Correlation between aortic elasticity and myocardial strain was also analyzed. The results showed that aortic stiffness was higher (17.5±14.0 vs. 5.3±2.7, P<0.001), and aortic strain (4.9±4.7 vs. 11.0±4.1, P<0.001) and distensibility (1.8±2.1 vs. 3.7±1.6, P<0.001) were lower significantly in BAV group than in control group. Global circumferential strain (-19.1±4.2 vs.-22.5±3.7, P<0.001), radial stain (29.8±14.9 vs. 38.0±8.8, P<0.001) and longitudinal stain (-18.4±3.4 vs.-20.8±3.5, P<0.001) were significantly lower in BAV group than in control group. There was weak association between aortic elasticity and myocardial strain. These findings indicated BAV patients manifest reduced myocardial strain which had weak relationship with aortic elastic lesion.
Adolescent ; Adult ; Aged ; Aorta ; pathology ; Aortic Valve ; abnormalities ; pathology ; Child ; Echocardiography ; Elasticity ; Female ; Heart Valve Diseases ; pathology ; Humans ; Male ; Middle Aged ; Myocardium ; pathology ; Sprains and Strains ; pathology ; Vascular Stiffness ; physiology

INTRODUCTIONBicuspid aortic valve (BAV) is the most common form of adult congenital heart disease. When compared to patients with a normal trileaflet aortic valve, dilatation of the aortic root and the ascending aorta (Asc Ao) are the common findings in patients with BAV, with consequent higher risk of developing aortic aneurysm, aortic dissection and rupture. We aim to determine the site of the Asc Ao where maximum dilatation occurs in Asian adult patients with BAV.

MATERIALS AND METHODSAll subjects underwent full echocardiography examination. The diameter of the Asc Ao was measured at 3 cm, 4 cm, 5 cm, 6 cm and 7 cm from the level of aortic annulus to the Asc Ao in 2D from the parasternal long-axis view.

RESULTSA total of 80 patients (male/female: 45/35; mean age: 45.3 ± 16.2 years) with congenital BAV and 30 normal control group (male/female: 16/14; mean age: 45.9 ± 15.1 years) were enrolled. The indexed diameters of the Asc Ao were significantly larger than the control group. In patients with BAV, maximum dilatation of Asc Ao occurred around 6 cm distal to the aortic annulus.

CONCLUSIONIn patients with BAV, dilatation of Asc Ao is maximal at the mid Asc Ao region around 6 cm distal to the aorta annulus.

Adult ; Aneurysm, Dissecting ; epidemiology ; Aorta ; diagnostic imaging ; Aortic Aneurysm ; epidemiology ; Aortic Diseases ; diagnostic imaging ; epidemiology ; Aortic Rupture ; epidemiology ; Aortic Valve ; abnormalities ; diagnostic imaging ; Case-Control Studies ; Comorbidity ; Dilatation, Pathologic ; diagnostic imaging ; epidemiology ; Echocardiography ; Female ; Heart Valve Diseases ; diagnostic imaging ; epidemiology ; Humans ; Male ; Middle Aged ; Risk Factors ; Singapore ; epidemiology

Anatomical variations of carotid arteries may be related to their development (agenesis, aplasia, hypoplasia) or course (coiling, kinking, tortuosity). Partial or total aberrancies in carotid vessel anatomy rarely occur. We describe the case of a 95-year-old woman presented with sudden onset of confusion and disorientation together with upper limb clonus. Computed tomography (CT)-scan revealed a left frontal brain injury with a not conclusive carotid doppler ultrasound. CT angiography reported a bovine aortic arch with bilateral retroesophageal course of both common carotid arteries and left severe (>70%) internal carotid artery stenosis. The knowledge of anatomical variations of the course of carotid arteries is relevant for possible surgical or endovascular repair or in case of otolaryngology or intubation procedures.
Angiography ; Aorta, Thoracic* ; Brain Injuries ; Cardiovascular Abnormalities ; Carotid Arteries ; Carotid Artery, Common* ; Carotid Stenosis ; Female ; Humans ; Intubation ; Otolaryngology ; Ultrasonography ; Upper Extremity

Klippel-Feil syndrome (KFS) is a congenital developmental disorder of cervical spine, showing short neck with restricted neck motion, low hairline, and high thoracic cage due to multilevel cervical fusion. Radiculopathy or myelopathy can be accompanied. There were 2 patients who were diagnosed as KFS with exhibited radiological and physical characteristics. Both patients had stenosis and cord compression at C1 level due to anterior displacement of C1 posterior arch secondary to kyphotic deformity of upper cervical spine, which has been usually indicative to craniocervical fixation. One patient was referred due to quadriparesis detected after surgery for aortic arch aneurysmal dilatation. The other patient was referred to us due to paraparesis and radiating pain in all extremities developed during gynecological examinations. Decompressive C1 laminectomy was done for one patient and additional suboccipital craniectomy for the other. No craniocervical fixation was done because there was no spinal instability. Motor power improved immediately after the operation in both patients. Motor functions and spinal stability were well preserved in both patients for 2 years. In KFS patients with myelopathy at the C1 level without C1-2 instability, a favorable outcome could be achieved by a simple decompression without spinal fixation.
Aneurysm ; Aorta, Thoracic ; Cervical Vertebrae ; Congenital Abnormalities ; Constriction, Pathologic ; Decompression* ; Dilatation ; Extremities ; Female ; Gynecological Examination ; Humans ; Klippel-Feil Syndrome* ; Laminectomy ; Neck ; Paraparesis ; Quadriplegia ; Radiculopathy ; Spinal Cord Compression ; Spinal Cord Diseases* ; Spine

OBJECTIVETo review the experience of left ventricular assistance device (LVAD) using for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in perioperative period.

METHODSThere were 29 patients with ALCAPA underwent surgical repair from May 2006 to May 2013. The mean age was 6.5 months (ranging from 3.3 to 12.1 months). The mean weight was 6.2 kg (ranging from 4.1 to 9.5 kg). Diagnosis was established by echocardiography in all patients. There were clinical symptomatic of the severe heart dysfunction and ejection fraction were 23% to 45%. Mitral insufficiency was moderate to severe in 8 patients and less than moderate in others. Surgical methods included the intrapulmonary tunnel (Takeuchi procedure) of 4 cases, direct reimplantation of the left coronary artery onto the aorta of 6 cases and reimplantation by pericardiac patch enlarge of 19 cases. Valvuloplasty were performed in 5 patients with mitral severe insufficiency. Twenty-two patients were treated only by medicine therapy. LVAD was used in 7 patients: there were 3 patients with low blood pressure at the end of surgical repair and 4 patients with low cardio output within 24 hours postoperatively.

RESULTSPostoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery is fluently but left ventricular is also largement. The hemodynamic of 18 patients was stable in medicine group but 3 patients were sudden died of low cardiao output and ventricular fibrillation respectively. One patient was died of diffuse intravascular coagulation at the time of 72 hours after operation. The hemodynamic was stable in 6 patients in LVAD group and the devices after using time from 72 to 108 hours was taken down except one patient died of multi-organ dysfunction. The hospital mortality was 5/29 (17.2%). Nineteen survival (19/24) was followed up of 3.5 years (ranging from 1 to 7 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated that the blood flow of the left coronary artery are fluently. Mitral insufficiency was moderate in 2 cases, mild to moderate in 9 cases and mild in 8 cases. The ejection fraction value were 43% to 55% and apparent arrhythmia didn't occur.

CONCLUSIONSAlthough late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, heart function evaluation and LVAD should be used as an effective cardiac support technique to prevent heart failure in time.

Aged ; Aorta ; Bland White Garland Syndrome ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Heart ; Heart Failure ; Hospital Mortality ; Humans ; Mitral Valve Insufficiency ; Perioperative Period ; Postoperative Care ; Prostheses and Implants ; Pulmonary Artery ; abnormalities ; Reoperation ; Treatment Outcome ; Ventricular Function, Left