Cases reported "Marfan Syndrome"

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1/64. Fragile lung in the marfan syndrome.

    Two cases of the marfan syndrome presented with spontaneous pneumothorax. Both had chest radiographs showing bilateral bullae in the upper lung zones and pulmonary function tests consistent with mild emphysema. There were dereases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. It is suggested that pneumothorax and bullous emphysema in this syndrome are caused by a weakness in the pulmonary connective tissue framework.
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ranking = 1
keywords = chest, upper
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2/64. Aneurysms and hypermobility in a 45-year-old woman.

    EDS type IV presents a diagnostic and therapeutic challenge to the primary care physician, surgeon, and rheumatologist. In patients for whom the diagnosis is known, avoidance of trauma, contact sports, or strenuous activities, joint bracing and protection, and counseling on contraception are helpful preventive strategies. In patients presenting with vascular, gastrointestinal, or obstetric complications, a history of hypermobility and skin fragility (easy bruising, abnormal scarring, poor wound healing) should lead to a suspicion of this diagnosis, and to caution in the use of certain invasive diagnostic and operative techniques. Efforts should be made to examine family members. Most importantly, when caring for such patients, the acute onset of headaches, chest pain, shortness of breath, and abdominal pain should arouse suspicion of a potentially catastrophic vascular or visceral event.
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ranking = 2.495823145771
keywords = headache, chest
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3/64. Surgical repair of type B aortic dissection complicated by early postoperative lung vein and artery thrombosis.

    A 24-year old man with marfan syndrome previously operated for abdominal aortic aneurysm and type A dissection sustained a type B dissection. He underwent graft replacement of the descending and upper abdominal aorta, complicated by infarction of the left upper lobe and lobectomy was carried out. The postoperative course was uneventful. The mechanism for this rare complication is discussed.
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ranking = 0.19623520106853
keywords = upper
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4/64. Simultaneous repair of cardiovascular disorders and pectus deformity in a patient with Sprintzen-Goldberg syndrome: A case report.

    We report a 12-year-old girl with Sprintzen-Goldberg syndrome (SGS) who was complicated with annuloaortic ectasia with aortic regurgitation, mitral valve prolapse with mitral regurgitation, and a severe pectus excavatum. In this patient, aortic root replacement, mitral valve replacement, and sternal elevation were simultaneously performed, and a version of Ravitch's procedure that was technically modified to support the sternum was used for sternal elevation. This modified sternal elevation technique gave excellent operative exposure, and maintained chest wall stability after the operation.
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ranking = 0.90188239946573
keywords = chest
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5/64. Suprachoroidal hemorrhage during silicone oil removal in marfan syndrome.

    A 30-year-old male with marfan syndrome underwent pars plana lensectomy and vitrectomy combined with silicone oil injection for the treatment of retinal detachment with proliferative vitreoretinopathy (PVR). Four months after vitrectomy, the silicone oil was removed using an anterior chamber maintainer (AC) and a self-sealing clear corneal incision at the upper temporal quadrant. Suprachoroidal hemorrhage (SCH) developed at the end of the operation. It was limited to the nasal side of the retina. Suprachoroidal hemorrhage remained to be limited and did not show progression.
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ranking = 0.098117600534266
keywords = upper
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6/64. Stress fracture of the hip and pubic rami after fusion to the sacrum in an adult with scoliosis: a case report.

    Correction of adult scoliosis frequently involves long segmental fusions, but controversy still exists whether these fusions should include the sacrum. It has been suggested that forces associated with activities of daily living transfer the stresses to the remaining levels of the spine and to the pelvis. The case described here was a 43-year-old woman with scoliosis and chronic back pain refractory to non-surgical modalities. Radiographically, the patient had a 110 degree lumbar curve. An anterior and posterior fusion with Luque-Galveston instrumentation was performed. Six months postoperatively the patient returned with a 2-week history of right hip pain with no history of trauma. There was radiographic evidence of a displaced femoral neck fracture and pubic rami fractures. The femoral neck fracture was treated with a total hip replacement. Further surgeries were required to correct a lumbar pseudoarthrosis and hardware failure. We believe that this case provides evidence that fusion into the lumbosacral junction may distribute forces through the pelvic bones and hip resulting in stress and potential hardware complications, especially in patients at risk due to osteopenic conditions.
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ranking = 3.1621625398933
keywords = back pain, back
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7/64. Surgical treatment for acute type A aortic dissection in pregnancy: a case of aortic root replacement just after cesarean section.

    A 25-year-old woman with marfan syndrome in the 37th week of pregnancy was referred for acute chest pain and dyspnea. An emergency cesarean section was performed because of fetal distress. Intraoperative echocardiography at the end of the cesarean section showed dilatation of the aortic root and dissection of the ascending aorta. The patient underwent replacement of the aortic root and the ascending aorta on the following day because of uterine bleeding. The postoperative course was uneventful for the mother and her baby.
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ranking = 0.90188239946573
keywords = chest
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8/64. Clinical considerations in the chiropractic management of the patient with marfan syndrome.

    OBJECTIVE: To describe the chiropractic management of a patient with whiplash-associated disorder and a covert, concomitant dissecting aneurysm of the thoracic aorta caused by marfan syndrome or a related variant. CLINICAL FEATURES: A 25-year-old man was referred by his family physician for chiropractic assessment and treatment of neck injuries received in a motor vehicle accident. After history, physical examination, and plain film radiographic investigation, a diagnosis of whiplash-associated disorder grade I was generated. INTERVENTION AND OUTCOME: The whiplash-associated disorder grade I was treated conservatively. Therapeutic management involved soft-tissue therapy to the suspensory and paraspinal musculature of the upper back and neck. Rotary, manual-style manipulative therapy of the cervical and compressive manipulative therapy of the thoracic spinal column were implemented to maintain range of motion and decrease pain. The patient achieved full recovery within a 3-week treatment period and was discharged from care. One week after discharge, he underwent a routine evaluation by his family physician, where an aortic murmur was identified. Diagnostic ultrasound revealed a dissecting aneurysm measuring 78 mm at the aortic root. Immediate surgical correction was initiated with a polyethylene terephthalate fiber graft. The pathologic report indicated that aortic features were consistent with an old (healed) aortic dissection. There was no evidence of acute dissection. Six month follow-up revealed that surgical repair was successful in arresting further aortic dissection. CONCLUSION: The patient had an old aortic dissection that pre-dated the chiropractic treatment (which included manipulative therapy) for the whiplash-associated disorder. Manipulative therapy, long considered an absolute contraindication for abdominal and aortic aneurysms, did not provoke the progression of the aortic dissection or other negative sequelae. The cause, histology, clinical features, and management considerations in the treatment of this patient's condition(s) are discussed.
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ranking = 0.38756480798414
keywords = back, upper
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9/64. Postoperative necrotizing fasciitis of the thorax in cardiac surgery.

    Necrotizing fasciitis is a rare soft tissue infection and a life-threatening emergency, often fatal. Its incidence and management are described plentifully in the medical literature regarding the most common anatomical sites involved like the abdomen, lower and upper limbs, and perineum. However, available data and case reports of chest wall necrotizing fasciitis after thoracic procedures are scarce, mainly after major cardiac operations. We report and discuss a case of necrotizing fasciitis of the chest wall occurring in the immediate postoperative period of a cardiac procedure, and include a brief review of the concepts, pathophysiology, and treatment reported in the medical literature. We emphasize the need for early diagnosis and urgent and effective surgical debridement. Of importance is the fact that we have not found any references in the literature to cases similar or equal to the one we describe here, which occurred in the postoperative period of a cardiac procedure.
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ranking = 1.9018823994657
keywords = chest, upper
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10/64. Coexistent Marfan's syndrome and ankylosing spondylitis: a case report.

    We report on a 46-year-old man with a 4-year history of predominantly nocturnal pain at the thoracic and lumbar spine as well as accompanying morning stiffness and episodes of alternating buttock pain. At physical examination the patient presented with the typical traits for Marfan's syndrome (MFS), along with limitation of both chest expansion and movement in all planes of the lumbar spine. Pelvic and lumbar spine radiographs showed findings consistent with ankylosing spondylitis (AS). Laboratory tests were consistent with an inflammatory state and HLA typing was positive for the B27 antigen. Transthoracic echocardiography showed prolapse of the posterior mitral leaflet and mild aortic insufficiency. We diagnosed co-existent MFS and AS. The association of these two pathologies is particularly interesting, owing to the co-existence of hypermobility of peripheral joints due to MFS ligamentous hyperlaxity, and the reduction of both axial skeleton motility and chest expansion related to AS. As both of these diseases may damage the cardiovascular system over time, follow-up with echocardiography monitoring is indispensable.
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ranking = 1.8037647989315
keywords = chest
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