Life-saving therapy for complete traumatic hemipelvectomy: a case report.
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ABSTRACT:
SUBMITTER: Zheng JH
PROVIDER: S-EPMC8183846 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Fibrodysplasia ossificans progressiva (FOP) is a rare disease in which heterotopic ossification (HO) is formed in muscles, tendons and ligaments. Traumatic events, including surgery, are discouraged as this is known to trigger a flare-up with risk of subsequent HO. Anesthetic management for patients with FOP is challenging. Cervical spine fusion, ankylosis of the temporomandibular joints, thoracic insufficiency syndrome, restrictive chest wall disease, and sensitivity to oral trauma complicate airway management and anesthesia and pose life-threatening risks. We report a patient with FOP suffering from life-threatening antibiotic resistant bacterial infected ulcers of the right lower leg and foot. The anesthetic, surgical and postoperative challenges and considerations are discussed. In addition, the literature on limb surgeries of FOP patients is systemically reviewed. The 44 year-old female patient was scheduled for a through-knee amputation. Airway and pulmonary evaluation elicited severe abnormalities, rendering standard general anesthesia a rather complication-prone approach in this patient. Thus, regional anesthesia, supplemented with intravenous analgosedation and N2O-inhalation were performed in this case. The surgery itself was securely planned to avoid any unnecessary tissue damage. Postoperatively the patient was closely monitored for FOP activity by ultrasound and [18F]PET/CT-scan. One year after surgery, a non-significant amount of HO had formed at the operated site. The systematic review revealed seventeen articles in which thirty-two limb surgeries in FOP patients were described. HO reoccurrence was described in 90% of the cases. Clinical improvement due to improved mobility of the operated joint was noted in 16% of the cases. It should be noted, though, that follow-up time was limited and no or inadequate imaging modalities were used to follow-up in the majority of these cases. To conclude, if medically urgent, limb surgery in FOP is possible even when general anesthesia is not preferred. The procedure should be well-planned, alternative techniques or procedures should be tested prior to surgery and special attention should be paid to the correct positioning of the patient. According to the literature recurrent HO should be expected after surgery of a limb, even though it was limited in the case described.
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Project description:Introduction and importancePrimary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy of unknown etiology, characterized by impaired lymphatic vessels drainage. The pathological changes in PIL result in usually localized or diffuse dilatation of intestinal lacteals, leading to leakage of lymphatic fluid rich of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease. In some patients, though, the outcome may be poor or even life-threatening. This case report demonstrates the severity of protein malnutrition, in some cases, and the extent of GI tract affected, requiring to start PN early and the need for its continuation as home parenteral nutrition (HPN).Case presentationWe present a case of 39-year-old male with Factor V Leiden deficiency, who presented initially with symptoms of malnutrition and anasarca. The diagnosis was confirmed by histopathological findings pathognomonic for PIL from biopsies of the stomach, small intestine and colon.Clinical discussionThe patient was started on low fat, high protein parenteral nutrition from the beginning of the treatment and required a long-term HPN for 3 years, because trials of tapering off and discontinuation of PN led to worsening of the biochemical results and recurrence of symptoms. Patient gradually improved and stabilized with persistent nutritional support.ConclusionsThe presented case report shows the magnitude of nutritional support (HPN) needed for severe PIL patients. HPN offers PIL patients with poor outcome and life-threatening complications a chance to improve and lead a normal life.
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Project description:Abstract Background Transcatheter aortic valve implantation (TAVI) has rapidly evolved and changed the field of structural cardiovascular intervention. Its advances lead to a marked reduction in the risk of complications and improved outcomes. However, TAVI is still associated with potential serious complications. Case summary A 73-year-old man with severe aortic stenosis underwent TAVI using a 34-mm self-expanding aortic bioprosthesis. After valve deployment, the patient rapidly progressed to cardiac arrest. Acute left main occlusion, due to high valve implantation, was promptly recognized and advanced life support immediately initiated. Concomitantly, the valve was successfully retrieved toward the ascending aorta using the snare technique, resulting in immediate restoration of flow and successful cardiopulmonary resuscitation. Subsequently, a 29-mm balloon-expandable aortic bioprosthesis was uneventfully implanted. After TAVI, the patient had a remarkable clinical evolution and was discharged home at hospitalization day five without relevant electrocardiographic nor echocardiographic disturbances. At six-month follow-up, the patient remains asymptomatic and transthoracic echocardiography revealed a normofunctional aortic bioprosthesis with preserved left ventricular ejection fraction. Discussion Acute coronary occlusion is a rare and life-threating complication of TAVI that may be prevented with accurate procedure planning. Pre-procedural computed tomography angiography is essential for a comprehensive patient evaluation, allowing appropriate valve selection, a key factor for successful management. Self-expandable valve retrieval with snare technique can be an appropriate strategy for the management of this complication. This case highlights the importance of performing these procedures in highly experienced centres and with fully equipped catheterization laboratories to allow timely interventions when facing unexpected events.
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Project description:Hip fractures with unstable pelvic ring have great morbidity and mortality rates. These fractures result from high energy trauma such as falls from heights, road accidents and collapsing structures or other similar mechanisms of action. We report the case of a 63 years old man, construction worker, who stood inside a ditch during a wall construction when he was surprised by this collapse, which resulted in direct trauma to the right thigh and pelvis. The autopsy revealed diaphysis fracture of the right femur with an open book pelvic fracture with severe hemorrhagic infiltration and hematoma of the pelvic muscles without arterial injury. Bone bleeding and the vascular damage associated with disruption of the sacroiliac ligaments promote a very significant bleeding. Simple maneuvers such as sheet circumferential compression to promote pelvic ring closure are effective on stabilizing and closure of the sacroiliac joint. Hip manipulation of the fracture was performed during the necropsy to demonstrate and prove how a simple sheet contention can promote stabilization of the pelvic ring by closing the sacroiliac joints in open book fractures.
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Project description:Introduction:Atlantoaxial rotatory subluxation (AARS) is not uncommon in paediatric emergencies, however, the complications might be fatal. Long onset before presentation is correlated with higher recurrence and persistent deformity. There is no consensus on the treatment of AARS yet. Selected patients may benefit from conservative approaches; however, retention might be difficult, and subluxation may recur. Presentation of case:A 6-year-old boy was admitted to our institution with AARS for three months before admission. Typical Cock-Robin position was observed. Computed tomography (CT) indicated AARS Fielding and Hawkins grade III. We treated the case conservatively by closed reduction and cervical traction using Gardner-Wells tongs. However, poor compliance resulted recurrence of subluxation, so we decided to fuse the atlantoaxial joint using transarticular screws, posterior wiring, and autologous bone grafting. Posterior fusion resulted in a satisfactory outcome, in which the wound healed accordingly. Six months of follow up examination revealed normal motoric and sensory function. The patient was able to perform daily activities with no significant issues. Discussion:Patients with fixed deformity of more than three weeks have a higher rate for recurrence or persistent deformity, as reduction is harder and difficult to maintain. The use of posterior wiring alone is limited in maintaining reduction, while using transarticular screws alone is considered better in maintaining reduction; however, not providing it. Conclusion:The use of posterior cervical fusion using C-wire, transarticular screws, and autologous bone grafting may be applied in recurrent case of AARS to ensure adequate reduction and fixation of the atlantoaxial joint.
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Project description:Renal leiomyosarcomas (LMS) are extremely rare neoplasms with aggressive behaviour and poor survival prognosis. The most frequent somatic events in leiomyosarcomas are mutations in TP53, RB1, ATRX and PTEN genes, chromosomal instability and chromoanagenesis. By using chromosomal microarray analysis we identified monosomy of chromosomes 3 and 11, gain of Xp (ATRX) arm and three chromoanasynthesis regions (6q21-q27, 7p22.3-p12.1 and 12q13.11-q21.2), with MDM2 and CDK4 oncogenes copy number gains, whereas no CNVs or tumor specific SNVs in TP53, RB1 and PTEN genes were observed.
2020-06-04 | GSE151766 | GEO
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