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Peripheral limb ischaemia and gangrene are devastating complications of pneumococcal sepsis. We report a 43-year-old professional pianist who presented with early sepsis and rapid development of this syndrome. No vasopressor medication was ever administered. We urgently reviewed the medical literature on a range of therapies recommended by consulting teams, to ensure he received optimal care. Based on our review and on feedback from the patient himself, we gained valuable insights into this illness and the merits of selected treatment options. His fingers ultimately recovered their function, intact, although several toes were later amputated. More recently published reviews postulate that imbalances in coagulation factors and natural anticoagulants occur as a result of disseminated intravascular coagulopathy and 'shock liver' in the sepsis syndrome, leading to microcirculatory thromboses. We submit this report as we believe it supports this hypothesis and adds further valuable information. We hope our observations will assist other critical care clinicians confronting this serious condition., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Hair tourniquet syndrome is an uncommon condition characterized by strangulation appendages by a hair or thread. The aim of this study was to evaluate the results of hair removal and antibiotic therapy in our patients with hair tourniquet syndrome., Methods: Between January 2012 and August 2018, 16 patients (8 boys, 8 girls; mean age: 118.5 [range: 20 to 380] days) were treated surgically for hair tourniquet syndrome. All patients were treated surgically under local or general anesthesia in the pediatric emergency department or in the operating theater using magnifying loupes. The age, gender, affected fingers or toes and the affected sides of the patients and the duration of symptoms until presentation were recorded., Results: A total of 24 toes and fingers were treated for hair tourniquet syndrome. The right side was affected in 12 patients, the left side was affected in three, and both sides in one. The second toe was affected in three patients, the third toe in eleven, the fourth in six, and the fifth in two patients. Both the thumb and the second finger were affected in one patient. The average duration of the symptoms (excessive crying, swelling, redness) was 1.5 (range: 1 to 2) days. All patients healed without any complications., Conclusion: Hair tourniquet syndrome should be kept in mind as an etiology in infants with toe and finger strangulation. These patients should be examined undressed. Immediate removal of hair is an effective treatment method to save appendage., Level of Evidence: Level IV, Therapeutic Study., (Copyright © 2019 Turkish Association of Orthopaedics and Traumatology. Production and hosting by Elsevier B.V. All rights reserved.)

Competing Interests: Competing interests: None declared.

This case illustrates a rare and unique case of a 73-year-old woman who presents with a rapidly developing digital ischaemia, superior mesenteric artery thrombus with positive-lupus anticoagulant. She then developed avascular necrosis of the femoral head. Discussion of the process of diagnosis and management of antiphospholipid syndrome and catastrophic antiphospholipid syndrome are reported.

Background: Critical limb ischaemia refers to an advanced form of peripheral vascular disease where severe arterial occlusion manifests as chronic ischaemic rest pain, nonhealing ulcers and gangrene. Depending on the severity of disease and level of occlusion, endovascular revascularisation and vascular surgical bypass are indicated to salvage the limb before the inevitable lifesaving choice limb amputation., Objective: This article illustrates a clinical scenario in which, without any intervention, the ischaemic anatomy may dry up and mummify. It is a remarkable reminder of the natural history of such events., Discussion: Medical management including analgesia, wound care, infection control and aggressive modification of atherosclerotic risks factors may contribute to a better prognosis. For inoperable cases, pneumatic compression and spinal cord stimulation can be considered to relieve symptoms and improve wound healing.

Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation., (Copyright © 2011 Elsevier España, S.L. All rights reserved.)

Background: Uterine leiomyomas are commonly reported to cause deep venous thrombosis and rarely arterial compression., Case: A 48-year-old woman was transferred to our institution with acute right lower limb ischemia and tissue loss. She underwent urgent iliac thrombectomy and was subsequently found to have right common iliac artery compression by a large uterine leiomyoma. She underwent successful resection of the tumor followed by endovascular iliac stent placement., Conclusion: This case emphasizes the importance of preoperative imaging when possible in the setting of acute arterial ischemia to evaluate for sources of extrinsic compression. Management requires correction of the etiology of extrinsic compression.

The safety of chemotherapy for patients with systemic sclerosis is unclear, and there are few published reports documenting the side effects of chemotherapy in patients with this condition. Here, we report the case of a patient with systemic sclerosis who developed severe digital ischemia during combination gemcitabine/S-1 chemotherapy for pancreatic cancer. In spite of aggressive treatment, the digital ischemia progressively worsened and gangrenous changes developed in multiple fingers and toes. In this patient, the systemic sclerosis had been well controlled, with no digital ischemic symptoms for the previous 6 years, so this progressive clinical course in spite of aggressive treatment strongly suggests that the chemotherapy triggered or aggravated the digital necrosis. To the best of our knowledge, this is only the third reported case of a patient with systemic sclerosis developing digital necrosis after gemcitabine-based chemotherapy. The incidence of digital necrosis during chemotherapy in patients with systemic sclerosis is unknown, and the mechanism by which it occurs is unclear, but the three reports published to date, including the present case, suggest that physicians should be very cautious about administering gemcitabine-based chemotherapy to patients with systemic sclerosis. Any resulting digital ischemia might be refractory to treatment and worsen progressively, even if chemotherapy is withdrawn in the early stages of digital ischemia.

Objective: The goals of this study were to determine if endothelial nitric oxide synthase (eNOS) affects both early and late collateral arterial adaptation and blood flow recovery after severe limb ischemia in a mouse model and to determine if eNOS-derived NO is necessary for recruitment of chemokine (C-X-C motif) receptor 4 (CXCR4)(+) vascular endothelial growth factor receptor-1 (VEGFR1)(+) hemangiocytes to the site of ischemia., Methods: Two studies were completed. In the first, hind limb ischemia was induced by unilateral femoral artery excision in three groups: C57Bl6 (wild-type), eNOS(-/-), and C57Bl/6 mice treated with N(G)-nitro-L-arginine methyl ester (L-NAME) from 1 day before excision through day 3 after excision (early L-NAME group). These groups were studied on day 3 after induction of ischemia. In the second study, hind limb ischemia was induced in C57Bl/6 mice (wild-type) and C57Bl/6 mice treated with L-NAME from days 3 through 28 after induction of ischemia. These groups were studied day 28 after ischemia induction. Dependent variables included hind limb perfusion, collateral artery diameter, and the number and location of hemangiocytes within the ischemic hind limb., Results: In the first study, toe gangrene developed in the eNOS(-/-) and early L-NAME treatment groups by day 2. These groups demonstrated less blood flow recovery and smaller collateral artery diameter than the wild-type group. Hemangiocytes were present within the adventitia of collateral arteries in the wild-type group but were only sparsely present, in a random pattern, in the eNOS(-/-) and early L-NAME treatment groups. In the second study, the late L-NAME group showed less blood flow recovery and smaller collateral artery diameter on day 28 of ischemia than the wild-type group. Hemangiocytes were present in a pericapillary distribution in the wild-type group, but were present only sparsely in the late L-NAME treatment group., Conclusion: Early (day 3) and late (day 28) adaptive responses to hind limb ischemia both require eNOS-derived NO. NO is necessary for normal hemangiocyte recruitment to the ischemic tissue., (Copyright 2010 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

A 39-year-old white man developed a severe left toe foot ischaemia and toe skin necrosis following his 12 courses of interleukin (IL)-2 (4.5 MIU twice a day, subcutaneously) for five days every two months. He had no known general risk factors for thrombosis other than HIV infection. An arterial Doppler ultrasound examination of the leg confirmed the permeability of the posterior tibial artery and its digital pulse. A diagnosis of foot ischaemia and toe skin necrosis was made. The suspected causative agent was IL-2 since this was the only drug that the patient was taking before the symptoms appeared. The patient was empirically treated with an aspirin and pentoxifylline in order to improve local microcirculation. We observed a satisfactory response with a quick resolve of skin lesions. The most possible cause of foot ischaemia and toe skin necrosis was considered to be IL-2 because of the temporal relationship between the exposure to the drug and onset of symptoms. Based on the Naranjo probability scale, IL-2 could be considered the probable cause of the foot ischaemia and toe skin necrosis. If clinical evaluation leads to the suspicion of ischaemic process, therapy with IL-2 should be discontinued immediately.

A 35-year-old patient with rest pain and peripheral gangrene was referred with a diagnosis of Takayasu's disease but had myeloproliferative disorder (primary thrombocythemia). The angiogram revealed surgically noncorrectable small vessel occlusive disease with no evidence of atheroma. Medical therapy with cytotoxic and antiplatelet agents achieved satisfactory results with excellent wound healing after surgical amputation of necrotic tissue. The need for early diagnosis of this condition is emphasized.

Aim of the Study: to assess the clinical course of minor amputations at the diabetic foot in relation to bone histopathology at the affected site, and at the osteotomy site., Patients and Methods: A total of 54 toes or metatarsal bones were amputated by general surgeons. Amputation level and mode of wound closure (primary wound closure) was left at the surgeons' discretion. The subjects were 45 diabetic patients, suffering from critical foot ischaemia (n = 27), polyneuropathy (n = 41) and chronic hemodialysis because of endstage renal failure (n = 4)., Results: Histopathology revealed osteomyelitis (n = 34), gangrene (n = 8), gout arthritis (n = 1), bone necrosis (n = 2), normal bone tissue or myelofibrosis (n = 9). The osteotomy site was located in healthy bone in 20 amputation specimens (Group A), and was affected by osteomyelitis in the remaining 34 specimens (Group B). Primary healing was observed in 11 wounds in Group A versus 3 wounds in Group B (p = 0.003). Further amputations were required in 3 cases in Group A (12 cases in Group B, n. s.). In 25 cases with critical foot ischaemia and osteomyelitis at the osteotomy site, primary healing was observed in only 1 (4 %) of the wounds, versus primary healing in 7 (77 %) of the 9 wounds without foot ischaemia and without osteomyelitis at the osteotomy site (p < 0.0001)., Conclusion: Under conditions of routine general surgery, osteomyelitic bone tissue will not always be fully resected by minor amputation procedures. Better pre-operative infection staging using MR imaging instead of radiography could prove advantageous.

A 61-year old man was admitted in our hospital for distal ischaemic lesions of the right foot with necrosis of the fifth toe. The lesions appeared a few days before admission. He was known for severe atheromatous disease and multiple aortic and femoro-popliteous aneurysms. He presented also numerous cardiovascular risk factors and a terminal chronic kidney disease was found 18 months earlier. The etiology of the kidney disease was not clear. The patient received hemodialysis since one month. We suspected a severe peripheral arterial disease but the investigations showed preserved distal arterial blood flow what made us think about a thromboembolic disease. We ruled out a potential embolic cardiopathy and, after finding eosinophilia and a previous consultation report of livedo, we suggested the diagnosis of cholesterol crystal embolisation. It was confirmed by the macroscopic examination of the necrotic toe. It's a rare and under-diagnosed pathology with a variable presentation. Though, it's important to detect this affection early as possible to prevent the frequent recurrences. The acute mortality is about 15%. There is no specific treatment, but an an aggressive control of the atheromatous disease is important. We must also avoid the known potential triggering factors: vascular surgery, angiography and anticoagulant or thrombolytic medications.

Strangulation of digits, the 'toe-tourniquet' syndrome needs prompt intervention as failure to recognise the condition can lead to ischaemia and loss of the appendage. It is a common condition though relatively under reported. Those who deal with children more frequently are aware of the condition but this is not the case for all medical practitioners and hence the diagnostic dilemma for accidental injury or child abuse arises. We report this case to increase physician awareness of the syndrome, to highlight the importance of early release of the tourniquet and to avoid misinterpretation of the condition as child abuse.

Necrosis of the digits is a rare complication of warfarin therapy of obscure pathogenesis. We report a 61-year-old woman with a 12-month history of Raynaud's phenomenon who developed multiple digital necrosis following aortic valve replacement with mechanical prosthesis for aortic insufficiency caused by nonbacterial thrombotic endocarditis. Exacerbation of Raynaud's phenomenon occurred during the postoperative period, with daily episodes of ischemia of the fingers and toes that improved with local warming. However, coincident with the occurrence of immune heparin-induced thrombocytopenia, and while undergoing routine warfarin anticoagulation because of the mechanical valve prosthesis, the patient abruptly developed progression of digital ischemia to multiple digital necrosis on postoperative day 8, at the time the international normalized ratio reached its peak value of 4.3. All limb pulses were readily palpable, and vascular imaging studies showed thrombosis only in the superficial femoral and popliteal veins of the right leg. Coagulation studies showed greatly elevated levels of thrombin-antithrombin complexes and prothrombin fragment F1.2 levels, consistent with uncontrolled thrombin generation. After vitamin K administration, no abnormalities of the protein C anticoagulant pathway were identified, consistent with previous studies of other patients with warfarin-induced necrosis complicating heparin-induced thrombocytopenia. Subsequently, the patient was shown to have metastatic breast adenocarcinoma, which explained the patient's initial presentation with nonbacterial thrombotic endocarditis. This patient case suggests that multiple digital gangrene can result from the interaction of various localizing and systemic factors, including compromised microvascular blood flow (Raynaud's phenomenon), increased thrombin generation (heparin-induced thrombocytopenia, adenocarcinoma), and warfarin-induced failure of the protein C natural anticoagulant pathway., (Copyright 2003 Wiley-Liss, Inc.)

Introduction: Localized large vessel thrombosis in acute leukaemia is rare, haemorrhagic complications being more common., Method: We present a patient with acute promyelocytic leukaemia (APL) presenting with an acutely ischaemic lower limb. Large vessel thrombosis is a rare presentation of APL. We reviewed the literature on the coagulopathy of APL and discuss the pathology and current treatment options., Discussion: Disordered haemostasis is typical of acute promyelocytic leukaemia (FAB M3) and relates to the intrinsic properties of the blast cells as well as thrombocytopenia from bone marrow involvement. Expression of procoagulants, stimulation of cytokines and alterations in endothelial cell anticoagulant properties initiate a disseminated intravascular coagulation (DIC) resulting in the typical clinical and laboratory findings in APL. The promyelocytes are characterized by the balanced reciprocal translocation between chromosomes 15 and 17. All-trans-retinoic acid (ATRA) induces differentiation in these cells, revolutionizing the treatment of APL., Conclusion: Unexpected limb ischaemia in a young, apparently healthy patient might be the presenting symptom of an underlying haematological disorder such as APL. A thorough haematological investigation should be performed prior to contemplating surgery. New treatment strategies based on knowledge of the molecular biology of APL has improved the prognosis of patients suffering from APL.

Fibrinogen plays a complex role in hemostasis, thrombosis, and vascular disease. Hyperfibrinogenemia is an independent vascular risk factor and dysfibrinogenemia can provoke thrombosis. Afibrinogenemia is usually responsible for hemorrhagic diathesis, and unexpected ischemic lesions are intriguing. We report the case of an afibrinogenemic patient, who at the age of 30 developed ischemic lesions of the feet related to severe stenosis of the iliac and hypogastric arteries. The biopsy of the iliac artery lesion showed an intense myointimal hyperplasia. We performed standard hemostatic analysis and analyzed the activation markers of platelets and coagulation factors and the kinetics of thrombin generation in the patient and in normal control plasmas treated or not with reptilase. Occlusive arterial lesions were attributed to a disruptive hematoma penetrating the vascular lumen. Thrombin concentration after calcium addition increase markedly in the afibrinogenemic patient and in defibrinated normal plasma, as compared to untreated normal plasma. Thrombin-antithrombin complexes (T-AT) were markedly enhanced while F1+2 prothrombin fragments stayed in the normal range. These results suggested activation of coagulation and in vivo circulating thrombin. Thrombin activates the platelets that secrete growth factors for smooth muscle cells and generate the intimal hyperplasia. Recurrent hemorrhage within the vessel wall might induce injury and local thrombin generation. Thrombin not trapped by the clot is available for platelet activation and smooth muscle cell migration and proliferation. The absence of a protective fibrin cap on the intima might account for intima vulnerability and embolization. Afibrinogenemia appears in this paradoxical situation as a vascular risk factor.

We report the case of a 53 year-old woman with paroxystic hypertension and digital ischemia. The only triggering factor was chronic intoxication by a nasal vasoconstrictor:fenoxazoline. The worsening of the lesions required amputation of the third phalanx of the 3rd and 5th finger and the pulp of the 4th finger of the left hand. The review of literature exhibits association between fenoxazoline intoxication and paroxystic hypertension, myocardial ischemia or stroke, but association between fenoxazoline intoxication and digital ischemia has never been reported. We emphasize the extreme caution necessary with the prescription and the chronic consumption of this kind of drug.

Severe hypofibrinogenemia was found in an Algerian woman who, since the age of 37 years, suffered three different episodes of ischemic necrosis of the toes and fingers leading to amputation of the toes and surgical removal of necrotic tissue (necretomy). No anti-fibrinogen antibody was present. The deficiency appeared to be due to severe congenital hypofibrinogenemia since the fibrinogen level remained at the same low level over a long period, without any abnormality of other coagulation proteins. The thrombotic events may be explained by the increased thrombin generation observed in the patient's plasma, due to the lack of thrombin adsorption onto a fibrin net.

Purpose: The goals of any treatment for ischemic vascular disease are to relieve pain, allow wound healing, and regain/maintain ambulatory abilities. To determine if endovascular therapy could accomplish these goals in patients with limbs at risk, we undertook a retrospective analysis of the results of percutaneous transluminal angioplasty (PTA) and thrombolysis in leg salvage., Methods: Over an 8-year period ending in 1993, 327 patients (209 males, 118 females) with limb-threatening ischemia (defined as one or more of the following: ischemic rest pain, ischemic ulceration, or gangrene) in 361 limbs were seen. These patients had an ankle-brachial index < or = 0.40 in nondiabetic patients and/or toe-brachial index < or = 0.30 in diabetic patients. The patients were treated with thrombolysis and/or PTA according to standard techniques; intravascular stenting was used occasionally as required to treat PTA deficiencies or inadequate recanalization., Results: Among the 327 patients, 805 arteries and 25 thrombosed arterial grafts were treated. The arteries treated were: 6 aorta (0.75%), 75 iliac (9.3%), 34 common femoral (4.2%), 3 deep femoral (0.4%), 235 superficial femoral (29.2%), 219 popliteal (27.2%), 193 infrapopliteal (24.0%), and 40 pedal (5.0%). The patients were treated primarily with PTA (n = 307); urokinase thrombolysis (n = 99) and stents (n = 12) were used less frequently. Procedural success was 92.5%. Patients were followed from 6 months to 8 years (mean 3 years). Eighty-three patients were retreated during this time. Of the 249 patients available for this follow-up, total salvage was achieved in 181 (72.7%). Nineteen patients (7.6%) underwent minor amputations (e.g., toes); 4 patients (1.6%) had transmetatarsal amputation. Fifteen patients (3.6%) had above-knee amputation despite successful recanalization. In all, 32 patients (8.8%) had the amputation level extended from above to below knee, bringing the total leg salvage rate to 82% (defined as patients able to ambulate without any prosthesis). Using life-table analysis, the 5 year survival rate was 71%., Conclusion: This study shows that endovascular therapy for leg salvage is as effective as reconstructive vascular surgery in achieving wound healing and maintaining ambulation and has a more favorable 5-year survival rate.

Background: Vascular accidents in the survivor from pregnancies with twin-twin transfusion syndrome have been attributed to the release of thromboplastin from the dead fetus., Case: In a woman presenting at 22 weeks' gestation with acute polyhydramnios due to twin-twin transfusion syndrome, endoscopic laser coagulation of the communicating vessels was performed. At endoscopy, the toes of the left foot of the recipient fetus were noted to be gangrenous. This fetus had only a left umbilical artery, which was markedly dilated, and Doppler studies demonstrated impaired perfusion of the left leg. In four control fetuses with a single umbilical artery, Doppler studies demonstrated normal waveforms in both legs., Conclusion: In twin-twin transfusion syndrome, fetal peripheral gangrene may occur before the death of one twin. The ischemic necrosis of the toes probably resulted from the combined effect of polycythemia and umbilical artery steal syndrome. Interruption of twin-twin transfusion syndrome caused resolution of the polycythemia and prevented further damage by removing one of the components of the peripheral ischemia.

Four uremic patients with advanced peripheral arterial occlusive disease (PAOD) of lower limbs causing rest pain and ischemic-necrotic lesions were treated with a four-hour intravenous infusion of iloprost at doses of 0.75-2.5 ng/kg/min for twenty-eight days. After a week of the therapy all patients experienced disappearance of rest pain and prolonged walking distance. At the end of the trial a diabetic patient showed a complete regression of the necrotic areas of two toes while the other patients still showed ischemic-necrotic foot lesions that were well demarcated. Iloprost therapy can be effective in uremic patients with severe PAOD.

Thromboangitis obliterans, or Buerger's disease, is a progressive vascular disorder that affects the distal extremities of young, otherwise healthy patients. The authors present two case histories of Buerger's disease with spontaneous digital ischemia and gangrene, leading ultimately to digital amputation. A review of the literature is also presented. The natural history of Buerger's disease, current diagnostic techniques, and management of these patients are discussed with emphasis on prevention of the acute attack and long-term sequelae.