Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis

Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. This presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia often with an associated monoclonal gammopathy. We describe a patient with SCLS who required aggressive fluid resuscitation and emergency fasciotomies for compartment syndrome with rhabdomyolysis. At presentation the patient was considered to have severe erythrocytosis and was therefore initially referred to a haematologist, which appears to be a frequent sequence of presentation for patients with SCLS. This patient also highlights the importance of muscle compartment pressure monitoring during volume resuscitation in patients with SCLS.     

Secondary extremity compartment syndrome

BACKGROUND: Abdominal compartment syndrome has been reported to occur after fluid resuscitation in injured patients, even in the absence of intra-abdominal injuries. This report describes a set of patients who developed the secondary extremity compartment syndrome (SECS) in uninjured extremities after resuscitation for other injuries. METHODS: This study was a retrospective chart review of all trauma patients developing SECS at a Level I trauma center. Data are mean +/- SD. RESULTS: From 1996 to 2001, 10 patients (8 men, age 31 +/- 13 years, Injury Severity Score of 29 +/- 17, and 3 with penetrating trauma) from a series of 11,996 trauma admissions developed SECS after resuscitation for other injuries. The mean number of extremities developing the SECS per patient was 3.1. This included compartment syndromes in 10 upper extremities and in 12 lower extremities that did not have any apparent injuries (i.e., contusions, fractures, or vascular injuries). After evaluation by the trauma team, abdominal silos were needed in 7 of the 10 patients also, and the mortality in patients with the SECS was 70%. CONCLUSION: SECS is a rare complication of the postresuscitation systemic inflammatory response syndrome, is associated with significant morbidity, and may be a marker for mortality. SECS should be ruled out by measurement of compartment pressures in uninjured and injured extremities in patients with severe diffuse edema after resuscitation for injury.     

Compartment syndrome in multiple uninjured extremities: a case report

Compartment syndrome is a common problem in trauma patients. It can occur within any space bound by a dense fascial layer, such as the extremities or abdomen. It exists when increased tissue pressure within the limited anatomic space compromises perfusion. Failure to decompress the compartment leads to a self-perpetuating ischemia-edema process and resultant irreversible tissue damage. In the extremities, it typically arises from a vascular injury in that same extremity. Herein is reported a case of the unexpected development of compartment syndrome in multiple uninjured extremities in a trauma patient with hypotension requiring systemic vasopressors.     

Systemic capillary leak syndrome. Case report

We here report the case of a patient with systemic capillary leak syndrome (SCLS). This syndrome is a rare condition characterized by recurrent episodes of hypotension with hemoconcentration and hypoproteinemia. It is due to unexplained episodic capillary hyperpermeabilty that results in fluid and protein shift from the intravascular to the interstitial space: generalized edema, shock and renal failure follow. A 59 yo man was admitted to our intensive care unit because of unexplained shock with hemoconcentration, renal failure, and metabolic acidosis. Previous attemps to reverse shock in a medical ward with crystalloids and dopamine failed. An abdominal CT scan, a TEE, and chest X ray study were inconclusive. No sign or history of major infections or anaphylaxis were present. The patient was resuscitated with massive fluid infusions and norepinephrine on the guide of a Swan Ganz catheter. The diagnosis was made on the basis of a previous episode of severe shock complicated with renal failure and a compartment syndrome, the hemoconcentration, and the negative cardiopulmonary findings. A small amount of monoclonal immunoglobulin G, kappa chain, found in the serum confirmed the diagnosis. The SCLS should be considered in the differential diagnosis of idiopathic and anaphylactic shock. Patients may benefit from a prophylactic treatment with theophilline and terbutaline.     

Influenza A induced rhabdomyolysis resulting in extensive compartment syndrome

This is a case of influenza A induced rhabdomyolysis resulting in extensive compartment syndrome and acute renal failure in a 10-year-old child. The patient required fasciotomies in all four extremities. Even after fasciotomies were performed, the muscle tissue continued to swell, suggesting a primary myositis. This case emphasizes the importance of considering the diagnosis of compartment syndrome in patients with influenza infection and severe myalgia.     

The systemic capillary leak syndrome: a scarcely known nephrological entity

The idiopathic systemic capillary leak syndrome (SCLS) is a rare life-threatening disorder characterized by periodic episodes of hypovolemic shock, due to plasma leakage from the intravascular to the interstitial space, as reflected by accompanying hypoalbuminemia, hemoconcentration and edema. Here we report the case of a 65-year-old woman affected by SCLS who required aggressive resuscitation with norepinephrine, steroids, albumin and crystalloids. Then, a long-term prophylaxis with a ?(2)-adrenergic receptor agonist and theophylline was started. In conclusion, though SCLS is a rare entity, the associated morbidity and mortality require the physician's awareness to provide timely therapy. Underrecognition in the medical community and rarity of this syndrome have precluded analysis by rational clinical trial designs that are necessary to determine more targeted and adequate therapy. This report is meant to enhance awareness of SCLS in the nephrology community.     

Acute renal failure associated with systemic capillary leak syndrome

Systemic capillary leak syndrome (SCLS) is rare disorder characterized by unexplained episodic capillary hy-perpermeability, resulting in generalized edema and hypovolemic shock. During acute episodes, there is characteristic elevated hematocrit and reduced serum protein. We report a 55-year-old man who had two recurrent episodes of SCLS. Laboratory findings revealed severe hypoalbuminemia, sudden elevation of hematocrit, and paraprotein IgG κ chain in his serum. The patient had acute oliguric renal failure during the acute episode. Despite receiving a large amount of fluid resuscitation, his hematocrit rose to 71.4%. After combination therapy of venesection and fluid resuscitation, massive diuresis occurred, and the symptoms were alleviated. We found that venesection was effective in controlling the acute episode of SCLS, without troublesome adverse effects. Although prophylactic therapy with terbutaline and theophylline is effective for SCLS, treatment in the acute phase is still empirical. We conclude that fluid replacement therapy accompanied by venesection is an alternative regimen in patients with severe capillary leakage. Received: August 23, 1999 / Accepted: March 29, 2000     

Crush syndrome due to drug-induced compartment syndrome: A rare condition not to be overlooked

Purpose  The crush syndrome caused by drug-induced compartment syndrome (DCS) is a challenge for surgeons because it is regularly associated with potentially fatal complications. Drug-induced compartment syndrome can often be distinguished from other forms of compartment syndrome by the presence of severe rhabdomyolysis with kidney crush and severe postoperative complications such as local and generalized infection, persistent nerve damage, coagulopathy, and multiorgan failure. Methods  In the past 15 years, eight prospectively documented, operatively managed, DCS with subsequent crush syndrome cases were recorded. Results  All of the patients required renal replacement therapy. The creatine kinase (CK) values in the context of rhabdomyolysis reached an average of 86 (range 47–144) kU/l. The renal function recovered in all surviving patients. The analysis showed that the diagnosis of a DCS is usually made after an average of 13 h. It then took an average of an additional 7 h before a fasciotomy was performed. Six operational revisions were necessary. In three out of eight patients the extremities had to be amputated. Conclusions  In DCS the decision to open the compartment should be made immediately upon the clinical diagnosis. A protracted intensive phase is expected. The benefit to patients is closely associated with surgical wound debridement along with rigorous intensive therapy. M. Golling and H. Fonouni contributed equally to this work.     

汶川地震伤致挤压综合征合并肾功能衰竭患者的救治

目的 回顾性分析汶川地震挤压综合征(CS)的治疗结果,为指导类似灾难性事件中挤压伤、cs的救治提供理论依据和指导.方法 2008年5月12口汶川地震后所致49例合并急性肾脏功能衰竭(ARF)的cS患者,致伤原因均为地震中被重物砸伤;受挤压时间4～102 h,平均24.7 h.男27例,女22例;年龄6.8～76.0岁,平均31.8岁.平均累计肢体1.5肢.49例存在ARF,血Cr平均365.6μmol/L(169～842 μmol/L),CK平均64 022 U/L(34 571～76 836 U/L),均出现肌红蛋白尿,39例合并有高钾血症(血钾5.23～6.38 mmoL/L).所有患者均予持续肾脏替代疗法(CRRT)治疗,补液、利尿、碱化尿液和纠正电解质紊乱、输血、改善低蛋白血症、预防或治疗创面感染治,扩创换药、扩创缝合.结果 CRRT治疗49例,肾脏功能在11～37 d恢复.高钾血症均在入院后当天纠正.CK在人院后13～45 d恢复正常.无1例死亡.骨筋膜间室切开减压30例52肢,截肢35例45肢.5例因感染、肢体坏死行二次截肢手术.24例创而经换药、扩创或植皮愈合,8处创面(4例)因感染、组织坏死或创面过大等原因未愈合,均为长时间(>20 d)接受CRRT治疗患者,并合并有其他系统、器官损伤.结论 及时明确诊断、积极有效的早期局部和全身治疗,是成功治疗CS,降低并发症发生率和病死率的关键.CS时创面愈合与长时间CRRT治疗之间的协调,是目前部分尚存创而患者治疗的难点.     

Arterial injury associated with acute compartment syndrome of the thigh following blunt trauma

BACKGROUND: Acute compartment syndrome of the thigh is a rare condition, and the basic causes of high pressure within a muscle compartment have been considered to be intramuscular haematoma and soft-tissue oedema. However, the importance of arterial injury has not been well recognized. METHODS: Among 3658 blunt trauma patients admitted to our Level 1 Trauma Centre between 1994 and 2001, there were eight patients (nine thighs) who had undergone emergency fasciotomy and these were the subjects of the present study. Arteriography of the proximal lower limb had been performed after the fasciotomy in patients with prolonged hypotension and persistent bleeding from the fasciotomy wound. RESULTS: All the patients had sustained high-energy trauma, systemic hypotension and local trauma to the proximal lower limb. Among them, four (five thighs) had undergone arteriography and four (four thighs) were confirmed as having sustained arterial injuries. In those patients with definitive arterial injuries, the time from injury to the onset of the compartment syndrome was less than 5 h. CONCLUSIONS: Acute compartment syndrome of the thigh in blunt trauma patients may be the result of associated arterial injuries. It is suggested that patients with local trauma to the proximal lower limb who exhibit an acute compartment syndrome together with haemodynamic instability should undergo arteriography soon after fasciotomy.     

The ‘silent’ compartment syndrome

Intractable pain out of proportion to the injury sustained is considered to be the earliest and most reliable indicator of a developing compartment syndrome. We report 4 cases where competent sensate patients developed compartment syndromes without any significant pain. The first patient developed a painless compartment syndrome in the well leg following surgery for femoral fracture on the other side. The second patient developed the silent compartment syndrome post-operatively following a tibial nailing for a tibial fracture. The third patient presented with the painless compartment syndrome following a tibial plateau fracture. Our prevailing culture of a high-index of clinical suspicion and surveillance prompted us to perform compartment pressure measurements. The surgical findings at immediate fasciotomy confirmed the diagnoses.Our experience indicates that pain is not a reliable clinical indicator for underlying compartment syndrome, so in a competent sensate patient the absence of pain does not exclude compartment syndrome.We believe that a high index of clinical suspicion must prevail in association with either continuous compartment pressure monitoring or frequent repeated documented clinical examination with a low threshold for pressure measurement.     

Lower extremity anterior compartment syndrome complicating bilateral mastectomy and immediate breast reconstruction: A case report and literature review

‘Well leg compartment syndrome’ refers to compartment syndrome occurring in a nontraumatic setting. This occurs most commonly in the lower limb during surgery performed with the patient in an anatomically vulnerable position. While this complication is well documented in the setting of orthopedic, urological and gynecological surgeries, it is an exceptionally rare complication in plastic surgery; only seven cases have been published on compartment syndrome complicating an operation performed on a supine patient. A case involving a 56-year-old woman who developed an anterior compartment syndrome of her right lower leg following a bilateral mastectomy with immediate breast reconstruction is presented. A detailed literature review is also included.     

Systemic capillary leak syndrome causes a compartment syndrome of both lower legs and a forearm. A case report with a review of the literature

Systemic capillary leak syndrome is a rare disorder which causes recurrent episodes of hypovolemic shock due to a markedly increased plasma shift into the interstitium. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy are characteristic laboratory findings. A rare manifestation of this disease is a compartment syndrome with rhabdomyolysis and acute renal failure. We describe a patient who suffers from a compartment syndrome of both lower legs and the left forearm secondary to systemic capillary leak syndrome, and discuss the pathophysiological background.     

Autoerythrocyte sensitization (Gardner-Diamond) syndrome mimicking compartment syndrome

We report a case with an unusual manifestation of autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome). The patient had the clinical signs of compartment syndrome of the forearm requiring fasciotomy. Dermatological signs identical to Gardner-Diamond syndrome should alert the physician to avoid unnecessary fasciotomy, as these patients respond well to psychiatric treatment as in the present case.     

Acute lumbar paraspinal compartment syndrome: a systematic review

While still a rare entity, acute lumbar paraspinal compartment syndrome has an increasing incidence. Similar to other compartment syndromes, acute lumbar paraspinal compartment syndrome is defined by raised pressure within a closed fibro‐osseous space, limiting tissue perfusion within that space. The resultant tissue ischaemia presents as acute pain, and if left untreated, it may result in permanent tissue damage. A literature search of ‘paraspinal compartment syndrome’ revealed 21 articles. The details from a case encountered by the authors are also included. A common data set was extracted, focusing on demographics, aetiology, clinical features, management and outcomes. There are 23 reported cases of acute compartment syndrome. These are typically caused by weight‐lifting exercises, but may also result from other exercises, direct trauma or non‐spinal surgery. Pain, tenderness and paraspinal paraesthesia are key clinical findings. Serum creatine kinase, magnetic resonance imaging and intracompartment pressure measurement confirm the diagnosis. Half of the reported cases have been managed with surgical fasciotomy, and these patients have all had good outcomes relative to those managed with conservative measures with or without hyperbaric oxygen therapy. These good outcomes were despite significant delays to operative intervention. The diagnostic uncertainty and subsequent delay to fasciotomy result from the rarity of this disease entity, and a high level of suspicion is recommended in the appropriate setting. This is particularly true in light of the current popularity of extreme weight lifting in non‐professional athletes. Operative intervention is strongly recommended in all cases based on the available evidence.     

腹腔间隔室综合征研究进展

随着基础医学和临床医学研究的进展,人们对腹腔高压危害性的认识逐渐加深.腹腔间隔室综合征在病因、诊断、治疗等方面有了较大进步.但目前研究认为,腹腔间隔室综合征病死率仍很高.因此,早期及时发现和正确治疗以取得最佳治疗效果,显得很重要,唯一有效的方法为开腹减压,本文对腹腔间隔室综合征的研究进展作一综述.     

Nontraumatic compartment syndrome of the extremities in children

Acute compartment syndrome of an extremity poses a threat to both life and limb. The usual cause in children is trauma, especially fractures and burns. Two recent cases of nontraumatic compartment syndrome of the extremities in children are presented, both owing to infection followed by myositis or fasciitis. Both cases required urgent decompression by fasciotomy, resulting in limb salvage and full recovery of neuromuscular function of the extremity. The value of prompt recognition and treatment of this condition is emphasized.     

早期诊断和治疗塌方所致骨筋膜室综合征20例

目的:总结20例煤矿事故中塌方所致骨筋膜室综合征患者早期诊断和治疗的方法,以提高救治骨筋膜室综合征的诊治水平,减少伤残。方法:自2006年9月至2010年4月采用早期切开减压、全身综合支持和处理治疗骨筋膜室综合征20例,均为男性,年龄23～54岁,平均42岁。所有术前患肢高张力肿胀、剧痛,被动牵拉痛阳性,5例肢端感觉减退或消失,远端血管搏动正常、减弱或消失,5例有肌红蛋白尿、高血钾、血清尿素氮和肌酐升高,1例少尿。对疗效予以统计并对患肢功能进行伤残等级评定。结果:20例患者,并发患肢感染3例,肾功能损害6例。治愈16例,基本治愈3例,截肢1例。20例患者均获随访,时间6～15个月,平均12个月。根据劳动能力鉴定——职工工伤与职业病致残等级分级(GB/T16180-2006)评定,5级伤残1例,8级伤残2例,10级伤残1例,无等级伤残16例。结论:骨筋膜室综合征的早期发现不能简单地以足背动脉搏动减弱或消失为依据,早期诊断、早期切开减压、全身综合支持和处理是减少伤残的关键。     

Noninvasive assessment of visco-elasticity in the presence of accumulated soft tissue fluid

BACKGROUND: The diagnosis of compartment syndrome is made by clinical examination, but direct compartmental measurements are important and serve an adjunctive role in establishing the diagnosis. This study examines a noninvasive screening method for differentiating compartmental syndrome from edema without elevated internal pressure. MATERIALS AND METHODS: The study groups consisted of 16 normals, 22 subjects with edema, and 2 subjects with compartmental syndrome. Force-displacement curves on the posterior and anterior surface of the extremity at mid-calf of each extremity were recorded using a noninvasive mechanical tester. A cyclic force peaking at 120 N was applied over a skin area of 1.5 cm(2). In a uniform applied force environment, the peak force would be comparable to an applied pressure of 60 mm Hg. Mechanical parameters associated with tissue softness (SOFT), degree of hysteresis, and departure from linear elastic behavior were calculated. In seven subjects, direct intracompartment pressure readings were obtained by the Stryker method. RESULTS: Posterior SOFT was significantly larger than anterior SOFT, as expected, for all study groups, except those with compartmental syndrome. SOFT for subjects with compartment syndrome fell below the 99% confidence interval of all other groups in the affected compartment(s). Departure from linear elastic behavior values were also depressed in the posterior compartment for subjects with compartment syndrome as compared with the other groups. Degree of hysteresis was significantly increased for pitting edema. Extremities with nonpitting edema were not distinguishable from normal extremities for the levels of applied pressure used in this study. CONCLUSION: Noninvasively measured mechanical properties were significantly different between normal tissues and tissues with pitting and nonpitting edema. The mechanical properties of the extremity with compartmental syndrome were different than those with edema as well as normal extremities. A noninvasive mechanical tester is seen as a possible clinical tool to diagnose and monitor compartmental syndrome.     

Refractory oedema: think about systemic capillary leak syndrome

Systemic Capillary Leak Syndrome (SCLS) is a rare and recently discovered disease. The diagnosis must be evoked in front of an hypovolaemic shock associated with a refractory anasarca. We report an observation of SCLS and then discuss the diagnostic and therapeutic difficulties. SCLS is diagnosed when a patient meet a compatible clinical situation with an hypoalbuminemia in spite of relative hemoconcentration. Evidence of a monoclonal gammapathy constitute an additional clue.