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Dr. Joseph A. Kuhn MD Lawrence D. Wagman MD John A. Lorant MD Fredrick W. Grannis MD Mordecai Dunst MD William R. Dougherty MD Daniel I. Jacobs MD 《Annals of surgical oncology》1994,1(4):353-359
Background: A radical forequarter amputation with partial chest wall resection (one to four ribs) has been reported for benign and malignant
lesions involving the shoulder and chest wall region. Concerns about reconstruction and postoperative pulmonary function have
previously limited more extensive chest wall resections. The current report describes the first case in which a complete unilateral
anterior and posterior chest wall resection and pneumonectomy (hemithoracectomy) accompany a forequarter amputation. A novel
reconstructive technique used the full circumference of the forearm tissue with an intact ulna as a free osseomyocutaneous
flap.
Methods: In this case, a 21-year-old patient presented with an extensive recurrent desmoid tumor that involved the shoulder, brachial
plexus, subclavian vein, and chest wall from the lateral sternal border to the midportion of the scapula and down to the eighth
rib. The operative technique involved removal of the entire right hemithorax from the midline sternum to the transverse process
posteriorly, down to the ninth rib inferiorly. Due to the absence of a rigid hemithorax, the uninvolved ipsilateral lung was
also removed. The forearm flap was prepared before final separation of the specimen and division of the subclavian vessels.
Results: Postoperatively, the patient maintained excellent oxygenation without atelectasis or fever and was extubated on the 15th
postoperative day. As expected after pneumonectomy, significant decreases from preoperative to immediate postoperative values
were noted for the vital capacity (VC) (from 4.87 L to 1.29 L), forced 1-s expiratory volume (FEV1) (from 3.77 L to 1.02 L),
and inspiratory capacity (IC) (3.33 1 to 0.99 1). Rehabilitation included a specially designed external prosthesis to provide
cosmesis and prevent scoliosis. By the 15th postoperative week the patient had returned to normal social and physical activities,
with a gradual improvement in all respiratory parameters: VC 1.52 L, FEV1 1.29 L, IC 1.04 L. There has been no evidence of
tumor recurrence at 1 year.
Conclusions: This report provides evidence that a complete hemithoracectomy, pneumonectomy, and forequarter amputation can be safely performed
for selective tumors involving the shoulder region with extensive chest wall invasion. Reconstruction may be achieved with
an extended forearm osseomyocutaneous free flap with an excellent functional outcome.
Presented at the 46th Annual Cancer Symposium of The Society of Surgical Oncology, Los Angeles, California, March 18–21, 1993. 相似文献
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Intestinal obstruction proximal to a transition zone without an interposed physical barrier usually indicates Hirschsprung disease. The authors report one case of focal small bowel muscular thinning just distal to a transition zone that produced clinical and radiographic findings that simulated long-segment Hirschsprung disease in a 2-day-old infant. 相似文献
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Mutations in the retinal guanylate cyclase (RETGC-1) gene in dominant cone-rod dystrophy 总被引:3,自引:0,他引:3
Kelsell RE; Gregory-Evans K; Payne AM; Perrault I; Kaplan J; Yang RB; Garbers DL; Bird AC; Moore AT; Hunt DM 《Human molecular genetics》1998,7(7):1179-1184
The dominant cone-rod dystrophy gene CORD6 has previously been mapped to
within an 8 cM interval on chromosome 17p12-p13. The retinal- specific
guanylate cyclase gene (RETGC-1), which maps to within this genetic
interval and previously was implicated in Leber's congenital amaurosis, was
screened for mutations within this family and in a panel of small families
and individuals with various cone and cone- rod dystrophy phenotypes. A
missense mutation (E837D) was identified in affected members of the CORD6
family, as well as a second missense mutation (R838C) in three other
families with dominant cone-rod dystrophy. RETGC-1 is only the fourth gene
to be implicated in cone-rod dystrophy and this is the first report of
dominant mutations in this gene.
相似文献
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Primary and revision total knee arthroplasty have become common orthopaedic procedures. The operating surgeon, at times, may be faced with a difficult surgical case due to soft tissue contractures or bone deformities. A review of multiple surgical techniques using soft tissue releases and osteotomies are presented including their potential complications. Although these techniques are aimed at the atypical operative case, the operating surgeon may utilize them for ‘routine’ exposures as well. Importance is focused on the functional integrity of the knee extensor mechanism. 相似文献