Cases reported "Marfan Syndrome"

Filter by keywords:



Filtering documents. Please wait...

1/62. 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.
- - - - - - - - - -
ranking = 1
keywords = operative
(Clic here for more details about this article)

2/62. Anaesthetic management of two paediatric patients with Hecht-Beals syndrome.

    We undertook the anaesthetic management of two children with Hecht-Beals syndrome for orthopaedic surgery under general anaesthesia. Both patients had arachnodactyly, kyphoscoliosis, and multiple congenital joint contractures, but limited mandible excursion was not obvious preoperatively in either, although mental retardation made it difficult for them to cooperate with mouth examination. They had no apparent difficulties with their mouths in daily activities. The anaesthesia records of one patient showed that intubation had been difficult in an earlier procedure. The other patient also had a history of difficult intubation, with slight tearing of the corners of her mouth during an intubation procedure. During slow induction of general anaesthesia with sevoflurane, face mask ventilation was easily performed. We attempted to visualize the larynx under anaesthesia with muscle relaxation, but we were unsuccessful because of the limited mouth opening. After several trials, blind oral intubations were fortunately successful in both patients. There were no postoperative problems with the airway.
- - - - - - - - - -
ranking = 2
keywords = operative
(Clic here for more details about this article)

3/62. 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.
- - - - - - - - - -
ranking = 5
keywords = operative
(Clic here for more details about this article)

4/62. 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.
- - - - - - - - - -
ranking = 1
keywords = operative
(Clic here for more details about this article)

5/62. Atlantoaxial rotatory subluxation in patients with marfan syndrome. A report of three cases.

    STUDY DESIGN: This is a retrospective case series of three patients, ages 9 1/2, 13, and 20 years old, with marfan syndrome treated for atlantoaxial rotatory subluxation. In the first two cases, acute torticollis was noted postoperatively, following pectus excavatum repair. The diagnosis was made in the third patient after she presented to the emergency room with a week-long history of unresolved neck pain following minor trauma. OBJECTIVE: To report and discuss the courses and clinical sequelae of atlantoaxial subluxation in patients with marfan syndrome. SUMMARY OF BACKGROUND DATA: Radiographic analysis of patients with marfan syndrome has shown that increased atlantoaxial translation, larger odontoid height, and basilar impression are more prevalent in this population compared to age-matched controls. Despite these findings, there are sparse data on injuries secondary to cervical spine instability or abnormalities in this population. To the authors' best knowledge, no report of atlantoaxial rotatory subluxation in patients with marfan syndrome exists in the literature. methods: Case records of rotatory instability of the atlanto-axial level were reviewed and are presented in the following report. RESULTS: The first two patients described in this report were noted to have "cock robin" posturing of their necks following pectus excavatum repairs. The first patient's subluxation was partially reduced with halo traction, and he subsequently underwent posterior spinal fusion of C1-C2 with internal fixation. The patient was well aligned postoperatively, and had no neurologic deficits. The second patient's subluxation reduced after 20 days of halter and traction; he was immobilized in a collar following discharge and reduction was maintained. The third patient's subluxation failed to reduce with halo traction; further imaging studies revealed odontoid prominence in the foramen magnum. She underwent posterior spinal fusion, occiput to C3, with satisfactory result. CONCLUSIONS: The cervical bony and ligamentous abnormalities seen in patients with marfan syndrome may slightly increase their risk for atlantoaxial rotatory instability. Special attention to intubation and positioning, both intraoperatively and postoperatively, may be necessary in patients with marfan syndrome. Additionally, rotatory subluxation should be included in the differential diagnosis for Marfan patients with neck pain after injury.
- - - - - - - - - -
ranking = 4
keywords = operative
(Clic here for more details about this article)

6/62. Clear lens extraction and intraocular lens implantation in Marfan's syndrome.

    Intracapsular clear crystalline lens extraction and intraocular lens (IOL) implantation were performed in 4 highly myopic eyes of 2 patients with Marfan's syndrome. One eye of each patient received an anterior chamber IOL and the other, a scleral-fixated posterior chamber IOL. The preoperative spherical equivalent ranged between -14.50 and -28.00 diopters (D) and axial length range, between 25.32 and 36.02 mm. The SRK II formula was used. Mean uncorrected visual acuity improved from counting fingers to 20/80. Postoperative spherical equivalent correction ranged from -0.75 to 2.75 D. One eye had vitreous loss that was managed by anterior vitrectomy. Modern surgery for cataract and management of its complications suggest that clear crystalline lens extraction and IOL implantation can be attempted in selected cases with Marfan's syndrome.
- - - - - - - - - -
ranking = 2
keywords = operative
(Clic here for more details about this article)

7/62. Complications of endocapsular tension ring implantation in a child with Marfan's syndrome.

    A 4-year-old boy with Marfan's syndrome had severe visual impairment after subluxation of the crystalline lens with bisection of the pupil. In the first eye, a capsular tension ring and an intraocular lens (IOL) were uneventfully placed in the capsular bag after phacoemulsification. During implantation of the endocapsular tension ring in the second eye, an inadvertent tear of the anterior capsule occurred, and the posterior chamber IOL was placed in the sulcus. In both eyes, severe lens epithelial proliferation with secondary IOL decentration developed postoperatively. Several surgical revisions were necessary to keep the IOL within the optical axis. In the eye with the sulcus-implanted IOL, the endocapsular tension ring was markedly decentered.
- - - - - - - - - -
ranking = 1
keywords = operative
(Clic here for more details about this article)

8/62. 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.
- - - - - - - - - -
ranking = 1
keywords = operative
(Clic here for more details about this article)

9/62. 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.
- - - - - - - - - -
ranking = 2
keywords = operative
(Clic here for more details about this article)

10/62. aortic aneurysm rupture in infantile Marfan's syndrome.

    A 3-year-old boy with early rupture of an aortic aneurysm due to infantile Marfan's syndrome is presented. In an emergency operation we prepared a composite graft using a 17-mm St. Jude prosthesis with an 18-mm vascular conduit. The postoperative period was complicated by pneumothoraces, transient bilateral phrenic nerve paralysis, cerebral convulsion, and supraventricular tachycardia. Four months postop the composite graft was replaced with an aortic homograft due to severe stenosis. His condition after 12 months is excellent.
- - - - - - - - - -
ranking = 1
keywords = operative
(Clic here for more details about this article)
| Next ->


Leave a message about 'Marfan Syndrome'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.