ABSTRACT
The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder. The prevalence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand. The vascular EDS(vEDS) are rare among the subtypes but are the worst in prognosis. The article reports a case of vEDS admitted to the hospital. The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons. The diagnosis was made after the genetic testing. The patient suffered from vEDS. Then, the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient. The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging. This article hopes to report this case and to share the experiences to the better understanding of this disease.
ABSTRACT
Objective:To evaluate a surgical approach for partial resection of the tenth rib through a retroperitoneal approach for the exposure of Crawford type IV thoracoabdominal aortic aneurysm and complex abdominal aortic aneurysm from 2014 to 2019.Methods:A retrospective analysis was conducted on clinical data and follow-up results of 7 patients who underwent treatment for Crawford type IV thoracoabdominal aortic aneurysm and complex abdominal aortic aneurysm through partial resection of the tenth rib via a retroperitoneal approach.Results:One case (14.3%) had associated Marfan syndrome, and 5 cases (71.4%) underwent left renal artery reconstruction. None of the patients experienced severe complications such as cardiopulmonary complications or renal failure postoperatively, and there was no statistically significant difference in serum creatinine levels between preoperative and postoperative stages during hospitalization ( P=0.205). Follow-up examinations showed no long-term vascular stenosis. Conclusions:Partial resection of the tenth rib through a retroperitoneal approach can avoid incisions of the pleura and diaphragm. It allows for the exposure of the aorta below the diaphragm and has the ability to treat aortic diseases below the diaphragm with smaller incisions and lower complication risks.
ABSTRACT
Objective:To summarize the surgical treatment of malignant carotid body tumor (MCBT).Methods:A retrospective analysis of 14 MCBT patients admitted at our hospital from Mar 2005 to Nov 2019 was made, and the imaging data, surgical records, perioperative complications and follow-up data were collected.Results:There were 8 males and 6 females, with an average age of (40.8±11.3) years. 10 patients underwent surgical resection of CBT, with one case undergoing tumor enucleation only, nine cases underwent internal carotid artery reconstruction, and all patients underwent intraoperative lymph node biopsy.Tumors were completely removed in all 10 patients. No perioperative death or cerebral infarction occurred. The intraoperative blood loss was (955±658.5) ml. Four patients had permanent nerve injury after surgery. The follow-up time ranged 1-132 months. There were no cases of cerebral infarction or death, and the reconstructed graft remained patent. Apart from the 2 patients who developed tumor metastasis after surgery, other patients recovered uneventfully with no disease progression.Conclusions:Surgery is still the main treatment for MCBT, but MCBT is large and Shamblin class is more advanced. Therefore, complete tumor removal and reconstruction of the carotid arteries are difficult. Surgery should seek to completely remove the tumor and neck lymph node biopsy should be performed to determine the lymph node metastasis.
ABSTRACT
The inflammasome is a multiprotein complex localized in the cytoplasm.It can mediate the expressions of various inflammatory cytokines such as interleukin(IL)-1β and IL-18 and plays a key role in regulating inflammatory response.As sterile inflammation,abdominal aortic aneurysm currently can only be treated by surgery.This article reviews the research advances in the role of inflammasomes in abdominal aortic aneurysm.
Subject(s)
Humans , Aortic Aneurysm, Abdominal , Inflammasomes , Inflammation , Interleukin-1beta , NLR Family, Pyrin Domain-Containing 3 ProteinABSTRACT
Objective To explore the influence of family-integrated transition care on the daily living ability of discharged patients with stroke.Methods Seventy-eight patients with stroke who were admitted to Renmin Hospital of Wuhan University from May 2016 to October 2017 were selected by convenience sampling and were divided into a control group and a family-integrated transition care group (hereinafter referred to as transition care group).The patients in the control group received routine neurological health education,while those in the transition care group received a family-integrated transition care intervention in addition to routine neurological health education.The family-integrated transition care included team building,skills training for family members,family-integrated guidance for discharged patients,and regular visits.The scores of the modified Barthel index were compared between the two groups of patients at discharge,three months after intervention,and six months after intervention.Results Among the 71 patients that were finally included,35 cases were included in the control group,of which 17 cases were men (49％),18 cases were women (51％),and their mean age was (70.1±3.7) years;the transition care group comprised 36 cases,of which 18 cases were men (50％),18 cases were women (50％),and their mean age was (69.8±4.5) years.The baseline scores of the control group and transition care group on the day of discharge were (49.1 ± 7.5) and (49.7 ± 7.9),respectively,with no significant difference (P＞0.05).In terms of time effects,the scores of the patients in the two groups had statistically significantly improved at six months after discharge (P＜0.05).In the group comparison,the scores of the patients in the transition care group after the intervention were significantly higher compared to the scores of those in the control group (P＜0.05).In terms of time and inter-group effects,there was an interaction (P＜0.05),and therefore,the influence of time effects was excluded and the same timepoint was compared between the two groups.The scores at three months (63.9±8.8) and six months (76.9± 10.1) in the transition care group were higher than those in the control group (58.1 ±8.1 and 66.0 ±9.3,respectively).The difference was statistically significant (P ＜ 0.05).Conclusion Family-integrated transition care can effectively improve daily living ability and isworthy of promoting.
ABSTRACT
Objective To determine the incidence and clinical features of headache after carotid endarterectomy (CEA).Methods A retrospective analysis was made on the data of patients undergoing CEA in the Department of Vascular Surgery,Peking Union Medical College Hospital from Jan 2014 to Jan 2015.There were 119 males and 24 females,including 97 cases of symptomatic carotid stenosis and 110 cases of severe carotid stenosis.Results The incidence of newly emergent headache after surgery was 44％ (63/143).The earliest time of headache was 30 minutes after surgery and the latest was 6 days after surgery.95％ of headache occurred within 48 hours after surgery (60/63).79.4％ (50/63) of the headache lay ipsilaterally with CEA,and bilateral headache accounted for 20.6％ (13/63).Severe headache accounted for 4.8％ (3/63),all were ipsilaterally frontal and temporal headache,complicating central nervous system symptoms.Conclusions After CEA headache is a common clinical symptom.Most were mild to moderate and unilateral,which can be relieved spontaneously.Cerebral hemodynamics need to be further examined to differentiate the different pathological states of severe headache on the operative side after CEA because of the high risk of developing hyperperfusion syndrome or cerebral infarction.History of TIA or stroke is the risk factor of post-CEA headache.
ABSTRACT
Objective To investigated the changes of angiopoietin-like protein 2(Angptl2) in patients with arteriosclerotic occlusion (ASO). Methods A total of 140 subjects including 75 ASO patients (ASO group) and 65 healthy subjects (control group) were enrolled in this study. Angptl2 and adiponectin were evaluated by using enzyme-linked immunosorbent assay. Biochemical data and high sensitive C reactive protein were measured and recorded as well. Results Compared to the control group,the ASO group presented with significantly higher level of plasma Angptl2 [(13.55±9.17) μg/L vs. (9.04±4.79) μg/L,P=0.010]. Plasma Angptl2 level of critical limb ischemia subjects was significantly higher than that of intermittent claudication subjects [(17.01±10.20)μg/L vs. (10.53±6.97) μg/L,P=0.003]. The best diagnostic cutoff value of Angptl2 was 13.67 μg/L,with a sensitivity of 60.34% and a specificity of 81.25%. In addition,type 2 diabetes mellitus patients with ASO exhibited significantly higher serum Angptl2 levels [(18.67±9.84)μg/L] than those without ASO [(13.01±3.47) μg/L] (P=0.021). In ASO group,serum Angptl2 levels were negatively correlated with ankle brachial index (r=-0.244,P=0.035). Conclusion The plasma level of Angptl2 increases in ASO patients. Its level is remarkably increased when the disease progressions to critical limb ischemia. Angptl2 can be a potential biological marker of disease progression.
ABSTRACT
Objective To investigate the role of proteasome inhibitor bortezomib (BTZ) in inflammatory response in abdominal aortic aneurysm (AAA) formation induced by angiotensin Ⅱ (Ang Ⅱ). Methods Ang Ⅱ-induced ApoEmice AAA models were established. Forty male ApoEmice (8-10-week-old) were randomly and equally divided into four groups:Sham group,BTZ group,Ang Ⅱ group,and Ang Ⅱ+BTZ group.HE staining,immunohistochemical staining,and flow cytometry were used to analyze the inflammatory response. Real-time quantitative polymerase chain reaction (qPCR) was used to analyze the mRNA expression of intercellular cell adhesion molecule-1 (ICAM-1). Western blotting was used to analyze the activation of nuclear factor κB signaling (NF-κB). Results The mean maximum suprarenal aortic diameter (Dmax) of Sham group,BTZ group,Ang Ⅱ group,and Ang Ⅱ+BTZ group were (1.00±0.01),(0.99±0.01),(1.50±0.13),and (1.20±0.04)mm,respectively (F=8.959,P=0.000). The Dmax of Ang Ⅱ group was significantly larger than those of Sham group (P=0.000) and Ang Ⅱ+BTZ group (P=0.015). The incidence of AAA in Ang Ⅱ group,Ang Ⅱ+BTZ group,and Sham group were 60%,17%,and 0,respectively. HE staining revealed that the abdominal aortic wall thickening was more severe in Ang Ⅱ group than in Sham group and Ang Ⅱ+BTZ group,similar with the infiltration of inflammatory cells. Immunohistochemical staining demonstrated that the CD3T lymphocyte count was significantly higher in Ang Ⅱ group than in Sham group (107.9±15.9 vs. 0,P=0.000) and Ang Ⅱ+BTZ group (107.9±15.9 vs. 0.8±0.5,P=0.000). Flow cytometry also demonstrated that the proportion of the CD3T lymphocytes of the Ang Ⅱ group [(13.50±0.69)%] was significantly higher than that in the Ang Ⅱ+BTZ group [(10.40±0.78)%] at week 1 (t=3.009,P=0.040),and the proportion of the CD3T lymphocytes of the Ang Ⅱ group [(22.70±0.93)%] was significantly higher than that in the Ang Ⅱ+BTZ group [(15.10±0.97)%] at week 4 (t=5.654,P=0.005). The qPCR analysis showed that the mRNA expression of ICAM-1 was significantly up-regulated in Ang Ⅱ group than in Sham group (1.93±0.54 vs. 1.00±0.15,P=0.011) and Ang Ⅱ+BTZ group (1.93±0.54 vs. 0.83±0.08,P=0.009). Western blot analysis showed a lower phosphorylation level of inhibitor of NF-κB in the Ang Ⅱ group compared with the Sham group or Ang Ⅱ+BTZ group,accompanied with an increased phosphorylation level of p65. Conclusion Proteasome inhibitor BTZ can attenuate AAA formation partially by regulating T lymphocytes infiltration through regulating the mRNA expression of ICAM-1 regulated by the activation of NF-κB signaling pathway.
Subject(s)
Animals , Male , Mice , Angiotensin II , Aortic Aneurysm, Abdominal , Drug Therapy , Apolipoproteins E , Genetics , Bortezomib , Pharmacology , Intercellular Adhesion Molecule-1 , Metabolism , Mice, Inbred C57BL , Mice, Knockout , NF-kappa B , Metabolism , Phosphorylation , Proteasome Inhibitors , Pharmacology , Random Allocation , Signal Transduction , T-Lymphocytes , Cell BiologyABSTRACT
Objective To study the effect of CBT resection on blood pressure in essential hypertensive subjects.Methods Clinical data of 24 essential hypertensive patients (BP ＞ 140/90 mmHg or was using anti-hypertensive drugs) with CBT resection from May 2005 to March 2016 was analyzed,retrospectively.The heart rate,blood pressure were recorded continuously during the peri-operative and follow-up period.All of the patients were followed-up for at least 1 year.Results The systolic blood pressure(SBP) and pulse pressure (PP) decreased 1-3 months after the operation,and the difference was significant (P ＜ 0.001),while the diastolic blood pressure (DBP) and heart rate (HR) did not change significantly (P ＞ 0.05).Postoperative patients used less amount of antihypertensive drugs,but the difference was statistically not significant (P ＞ 0.05).Conclusions Unilateral CBT resection is associated with short-term to intermediate-term lowering of BP in hypertensive patients (P ＞ 0.05).
ABSTRACT
Objective To study the effect of CBT resection on blood pressure in essential hypertensive subjects.Methods Clinical data of 24 essential hypertensive patients (BP ＞ 140/90 mmHg or was using anti-hypertensive drugs) with CBT resection from May 2005 to March 2016 was analyzed,retrospectively.The heart rate,blood pressure were recorded continuously during the peri-operative and follow-up period.All of the patients were followed-up for at least 1 year.Results The systolic blood pressure(SBP) and pulse pressure (PP) decreased 1-3 months after the operation,and the difference was significant (P ＜ 0.001),while the diastolic blood pressure (DBP) and heart rate (HR) did not change significantly (P ＞ 0.05).Postoperative patients used less amount of antihypertensive drugs,but the difference was statistically not significant (P ＞ 0.05).Conclusions Unilateral CBT resection is associated with short-term to intermediate-term lowering of BP in hypertensive patients (P ＞ 0.05).
ABSTRACT
Objective To compare the clinical efficacy of Rivaroxaban and Warfarin in the treatment of lower extremity deep vein thrombosis.Methods From January to December 2015,51 patients of deep vein thrombosis of the lower limb divided into.Warfarin group (21 cases) and Rivaroxaban group (30 cases).The time of each therapy lasted for 3 months or longer.The characteristics and the change of lower limb venous patency rate in two groups of patients were analyzed to evaluate the curative effect.Results Rivaroxaban group had shorter therapy time than Warfarin group.The lower limb venous patency rate in Rivaroxaban group were higher than that in Warfarin group (85.7％ vs.60％,P ＜0.05).Ultrasonography showed partial patency in 5 mixed thrombus patients of Warfarin group,while complete patency in 2 and partial patency in 3 of Rivaroxaban group.Normalized rate in peripheral venous thrombosis patients of Rivaroxaban group were higher than Warfarin group (84％ vs.25 ％ P ＜ 0.001).Conclusions Rivaraxaban is superior to Warfarin in the complete recanalization of DVT,while safe and reliable.
ABSTRACT
Objective To discuss the strategies of prevention and treatment for complications of endovascular therapy in aortoiliac artery occlusive disease.Methods 220 cases who received endovascular interventions with aortoiliac artery occlusive disease were retrospectively reviewed from June 2012 to June 2014.Among these patients,189 cases were males.The age was between 46 to 85yrs and the average age was 64yrs.Results The overall technique success rate was 97.2％.Nine procedure-related major complications requiring additional endovascular or surgical treatment were encountered in 7 patients including 2 acute in-stent thrombosis,1 iliac artery rupture,1 distal embolism,and 5 puncture associated complications (2 acute artery thrombosis,1 arteriovenous fistula,1 hematoma,1 artery dissection).8 cases improved after second endovascular or remedial open surgery,among those 4 cases were managed by endovascular interventions including 2 catheter directed thrombolysis,1 cover-stent implantation and 1 balloon-based angioplasty,4 patients received open surgery including 3 thrombectomies,1 hematoma cleating.After 22 months follow-up,the primary patency,assistant-primary patency and secondary patency was respectively 90.8％,92.1％ and 99.2％.Conclusions More attentions should be paid to the complications of endovascular therapy in aortoiliac artery occlusive disease.Helpful to prevent these complications are a comprehensive evaluation of the lesions,and individualized surgical plan prior to the operations,and a careful intraoperative management.
ABSTRACT
Objective To evaluate the extra-anatomic bypass surgery for aorto-iliac occlusive disease in the elderly. Methods From January 2005 to December 2008,33 elderly patients/39 limbs (age ranged from 70 to 87 years, mean 76.0 ± 3.0 years) with aortoiliac occlusive disease were retrospectively analyzed. According to Fontaine classification, there were 5 phase Ⅱ b cases (7 limbs), 22 phase Ⅲ cases (26 limbs), and 6 phase Ⅳ cases (6 limbs), all cases were with TASC C, D lesions. Results These 33 cases/39 limbs were tr eated with a variety of bypass, including axillobifemoral bypass (6 cases/12 limbs) ,axillofemoral bypass ( 20 cases/20 limbs ), femorofemoral bypass ( 7 cases/7 limbs ). Intermittent claudication improved in 5 cases, rest pain disappeared in 22 cases, and ulcers healed in 6 cases after operations. The mean ABI increased from 0.29 ± 0.11 to 0.66 ± 0.13 ( t = 2.69, P ＜ 0.05 ). All limbs were salvaged and there was no perioperative mortality within 30 days after operations. Postoperative complication rate was 9.1%. Patients were followed up for a median of 12 ±5 months (range, 6 to 28 months), primary patency rate was 89.7% (35/39), secondary patency rate was 94.9% (37/39), limb salvage rate was 92.3% (36/39). After dischargement the mortality was 6.1% (2/33) with one dying of myocardial infarction and another one of malignant tumor (gastric cancer). Conclusions Extra-anatomic bypass for aorto-iliac occlusive disease in the elderly is feasible, safe and effective, and the cardiopulmonary dysfunctions are the major risk factors for perioperative complications.
ABSTRACT
Objective To evaluate the efficacy and safety of perioperative anticoagulation and antiplatelet therapy of carotid endoarterectomy (CEA). Methods A retrospective study on 110 cases (122 CEAs) of carotid stenosis between Jan 2004 and Dec 2008 was undertaken. 122 cases were divided into anticoagulation group and antiplatelet group according to the perioperative medical treatment. Postoperative results of stroke/death and wound hemotoma were compared between the two groups and statistically analyzed. Results 45 CEAs were given perioperative combination of anticoagulation and antiplatelet treatment. This comprised the anticoagulation group. The antiplatelet group consisted of the other 77 CEAs which were treated with antiplatelet solely. Perioperative stroke/death rates were equivalent (2.2% anticoagulation vs. 2.6% antiplatelet, P =0.897). Wound hemotoma rates were found with statistical significant difference between the two groups (13.3% anticoagulation group vs. 1.3% antiplatelet group, P = 0. 006 ). Conclusion Our results suggest that perioperative antiplatelet therapy in perioperative carotid endoarterectomy does not increase perioperative stroke/death risk, while perioperative anticoagulation increases the risk of wound hematoma.
ABSTRACT
Objective To evaluate arterial reconstruction of traumatic iliac-femoral artery pseudoaneurysm in parenteral drug abusers.Methods Data of 21 consecutive patients who presented with iliac-femoral artery pseudoaneurysm with the diameter ranging from 3.0 cm～7.5 cm secondary to parenteral drug abuse from 2004 to 2007 were analyzed.Fifteen patients were male and the median age was 3 1.3 years.the aneurysm involved the common femoral artery and distal external iliac artery.Autologous greater saphenous vein was used as a graft for arterial reconstruction after iliac-femoral artery pseudoaneurysm excision through a single curved inguinal incision.Results The surgical procedure was successful in all 21 eases without intraoperative mortality and severe complications.All the 21 patients were free of postoperative claudication symptoms except 1 case with preoperative popliteal artery stenosis.Wound infection and tissue fistula developed in one ease.Ingunal incisional hematoma and cutaneous abnormal sensation developed in one each eases.Conclusions The use of autologous greater saphenous venous grafts for aaefial reconstruction after false aneurysm excision in drug abusers is safe and effective.The harvest of the greater saphenous vein is precondition of this precedure.These preliminary results indicate that the implementation of this technique offers advantages compared with the artery ligation alone without revascularization,that is frequently associated with later intermittent claudication.
ABSTRACT
Objective Repair of aortic arch aneurysm is technically demanding and usually requiring complex circulatory management. Operative morbidity and mortality may be prohibitive with traditional surgical intervention. We described our experience with 5 hybrid endovascular procedure for aorta repair with different kinds of bypass followed by concomitant placement of stent graft in the aorta. Methods We retrospectively reviewed the clinical data of 5 consecutive patients presenting with aortic aneurysm or dissection from 2007 to 2008 treated by the hybrid aorta repair. Complete surgical rerouting of the supra-aortic vessels was followed by endovascular repair of aortic arch aneurysm with a Zenith TX2 stent graft. Hybrid left carotid-subclavian bypass with Zenith stent graft deployment covering the ostium of the LSA was performed in a Debakey type Ⅲ aortic dissection case. Procedures were successfully completed with exclusion of the aortic aneurysm. All stent grafts were deployed retrograde from the femoral artery in these patients. Results Technical success with complete aneurysmal exclusion was achieved in all patients (100%). At a follow-up period of 2-10 months, there was no incidence of endoleak. Documented perioperative neurelogic events did not occurred in all patients. Postoperatively one patient suffered from ARDS and cardiac failure and recovered. One patient died of myocardial infarction. Conclusions Hybrid arch repair provides an alternative to patients otherwise considered prohibitively high risk for traditional open arch and thoracoabdominal aorta repair.
ABSTRACT
Objective To evaluate the result of revascularization for lower limb artery ischemia in elder patients. Method During Jan 2006 to Nov 2008, 262 elder patients (60 years old and up), underwent artery revascularization for lower limb arterial ischemia. Mortality, morbidity, primary patency, secondary patency and limb salvage were analyzed. Result There were a total of 323 ischemia limbs in those 262 elder patients undergoing revascalarization, among those 102 limbs underwent artery bypass, 98 limbs underwent endoluminal angioplasty with or without stenting, 67 limbs underwent embolectomy or endarterectomy, 56 limbs underwent open surgery combined with endoluminal treatment due to multiple segment lesions. Operation success rate was 94.7%. Two patients died within 30 days. Perioperative morbidity developed in 15 cases. 245 patients (93.5%) were followed-up from 1 month to 35 months. Mortality was 6.1% (15 cases), primary patency was 80.5%, secondary patency was 92.7% and limb salvage rate was 95.2%. Risk factors analysis showed that history of cardiac disease and elder ages were associated with higher mortality. Diabetes mellitus, critical ischemia and multiple segment lesions were associated with poor long term patency and limb salvage. Conclusion Lower limb ischemia is severe disease related to elder patients' death and amputation. Careful patient selection, detailed pre-operation work-up, meticulous operative technique play crucial roles for a successful arterial reconstruction.
ABSTRACT
<p><b>OBJECTIVE</b>To analyze the functions of differentially expressed genes between abdominal aortic aneurysm and normal aortic tissue by cDNA microarray.</p><p><b>METHODS</b>Total RNAs were respectively isolated from the normal aorta and aortic aneurysm, purified into mRNAs by oligotex. Subsequently they were reverse-transcribed into cDNAs incorporated with fluorescent dUTP to make hybridization probes, which were hybridized as the cDNA microarray for scanning of fluorescent signals and differentially expressed genes between the normal aortic and aortic aneurysm by using GenePix Pro 3.0 software.</p><p><b>RESULTS</b>A total of 18 differentially expressed genes were detected, accounting for 0.44% of total genes. Among these genes, 11 were related to cell cycle and the remaining 7 to cell apoptosis. The number of upregulated genes in the aortic aneurysm was 9 (mean ratio: 3.860) and that of the downregulated 9 (mean Ratio: 0.294). Bio-informative analysis showed that these 18 genes might influence the growth and apoptosis of smooth muscle cells in abdominal aortic aneurysms.</p><p><b>CONCLUSIONS</b>During the development of abdominal aortic aneurysms, modulations of multi-gene expression would undergo various changes. Cell cycle and apoptosis-related genes were related to the growth and apoptosis of smooth muscle cells in abdominal aortic aneurysms. Further research into these genes will clarify the mechanisms of abdominal aortic aneurysms.</p>
Subject(s)
Humans , Aortic Aneurysm, Abdominal , Genetics , Pathology , Apoptosis , Physiology , Cell Cycle , Genetics , Cells, Cultured , Gene Expression , Gene Expression Profiling , Myocytes, Smooth Muscle , Metabolism , Pathology , Oligonucleotide Array Sequence AnalysisABSTRACT
<p><b>OBJECTIVE</b>To report a case and a firstly described pedigree with Ehlers-Danlos syndrome in China.</p><p><b>METHODS</b>Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members with 4 generations were analyzed. Dilated internal jugular vein in the proband was removed operatively. The diagnosis, surgical treatment, and postoperative complications were retrospectively summarized. Etiology, clinical characteristics and classification of Ehlers-Danlos syndrome were also analysed.</p><p><b>RESULTS</b>Vessels of the proband in the pedigree were crisp and easily lacinated during the precedure for removal of his internal jugular vein. Repeat postoperative hemotomas were found though complete stanching was made during operation. The patient successfully recovered after prompt debridement in operative theatre and needle sucking. The other 5 members of the pedigrees all had the triads of Ehlers-Danlos syndrome, but no combined vascular diseases were found in them.</p><p><b>CONCLUSIONS</b>Though the morbidity rate was extremely low, green attention should be paid to the high mortality rate and complication of Ehlers-Danlos syndrome during surgical management of in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoprative vascular lacination and incontrollable hamorrhage.</p>
Subject(s)
Humans , China , Ehlers-Danlos Syndrome , Pedigree , Retrospective StudiesABSTRACT
<p><b>OBJECTIVE</b>To describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China.</p><p><b>METHODS</b>Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members within 4 generations were analyzed. Dilated internal jugular vein in the proband was removed by operation. The diagnosis, surgical treatment, and postoperative complications were retrospectively reviewed.</p><p><b>RESULTS</b>Vessels of the proband in the pedigree were crisp and easily lacinated during the procedure of removing his internal jugular vein. Repeating postoperative hematomas were found though complete stanching was achieved during the operation. The patient was successfully recovered by promptly debridgement and needle sucking. The other 5 members of the pedigree all had the triads of Ehlers-Danlos syndrome.</p><p><b>CONCLUSIONS</b>Though it was of extremely low morbidity rate, the high mortality rate and complication of Ehlers-Danlos syndrome deserve great attention during surgical management, especially in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoperative vascular lascination and incontrollable hemorrhage.</p>