Compartment syndrome in all four extremities: a rare case associated with systemic capillary leak syndrome

While isolated limb injuries often lead to suspicion for and treatment of compartment syndrome, multi-limb compartment syndrome induced by a systemic disease is a rare entity, in which lack of immediate diagnosis can have catastrophic consequences to the patient. We present a patient with idiopathic systemic capillary leak syndrome (SCLS) resulting in compartment syndrome in all four extremities. Treatment required bilateral hand, forearm, calf, and thigh fasciotomies. Presenting symptoms included pain in all four extremities, malaise, and confusion. Laboratory data included polycythemia, hypoalbuminemia, leukocytosis, and elevated creatinine, but not a monoclonal spike on serum electrophoresis as is common in SCLS. Thus, when faced with clinical evidence of multiple extremity compartment syndrome, the surgeon should consider systemic disorders, such as SCLS, and aggressively treat the compartment syndrome as well as the underlying disease.     

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     

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.     

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.     

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.     

Abdominal compartment syndrome complicating paediatric extracorporeal life support: diagnostic and therapeutic challenges

We report three paediatric cases, and summarise the reported experience in two others, with cardiorespiratory failure requiring extracorporeal life support for which supportive pump flows could not be maintained due to abdominal compartment syndrome. In two of our patients, the mechanism of abdominal compartment syndrome was massive intra-abdominal fluid extravasation secondary to sepsis, while in the third, the mechanism was post-traumatic intra-abdominal haemorrhage. Although all three children eventually died, decompressive laparotomy and arrest of haemorrhage in the trauma patient restored venous return and enabled technically adequate extracorporeal life support. In two previously reported cases of sepsis with massive fluid resuscitation resulting in abdominal compartment syndrome, one patient died without attempted decompression, while the other patient survived after peritoneal catheter placement restored venous return. Once correctable causes of inadequate venous cannula drainage have been excluded, abdominal compartment syndrome should be considered in any patient on extracorporeal life support with a taut abdomen and reduced venous return. If abdominal compartment syndrome can be proven or is strongly suspected, there may be a role for selective decompressive laparotomy.     

Secondary abdominal compartment syndrome causing failure to wean from cardiopulmonary bypass

The abdominal compartment syndrome has been associated with trauma or primary abdominal procedures. The secondary abdominal compartment syndrome which is not associated with a primary abdominal process is seen in burns and other clinical situations where aggressive fluid resuscitation is needed. This case report describes a secondary abdominal compartment syndrome that occurred during an elective coronary revascularization which resulted in an inability to wean from cardiopulmonary bypass (CPB). After a decompressive laparotomy was done, the patient was successfully weaned from bypass.     

Systemic capillary leak syndrome resulting in compartment syndrome and the requirement for a surgical airway

We report on a case of systemic capillary leak syndrome associated with a monoclonal band on plasma electrophoresis. In our patient hospital admission was precipitated by ischaemic pain in the left lower limb, associated with polycythaemia, renal failure and hypovolaemic shock. Fluid resuscitation, venesection and renal replacement therapy were instituted but a compartment syndrome developed necessitating surgery. Failure of tracheal intubation resulted in the requirement for a surgical airway. Despite surgical and resuscitative efforts the outcome was fatal from hypovolaemia and hyperkalaemia. We aim to highlight the difficulties in managing this condition and to remind healthcare workers to include it in the differential diagnoses for patients presenting with polycythaemia; in particular polycythaemic patients with a monoclonal band on plasma electrophoresis.     

Hypoperfusion, shock states, and abdominal compartment syndrome (ACS)

Cardiovascular dysfunction and failure are commonly encountered in patients with intra-abdominal hypertension or abdominal compartment syndrome. Accurate assessment and optimization of preload, afterload, and contractility are essential to restoring end-organ perfusion and maximizing patient survival. Application of a goal-directed resuscitation strategy, including abdominal decompression, when indicated, improves cardiac function, reverses end-organ failure, and minimizes intra-abdominal hypertension-related patient morbidity and mortality.     

Surgical decompression for abdominal compartment syndrome after emergency cardiac surgery

Abdominal compartment syndrome typically occurs in patients after abdominal surgical procedures or trauma. Abdominal compartment syndrome is also increasingly described in conditions not related to abdominal operations, such as fluid resuscitation or burns. We report two patients who required surgical abdominal decompression for abdominal compartment syndrome that developed early after emergency coronary artery bypass graft surgery. No intraabdominal abnormalities were found at operation. Both patients had a protracted clinical course, but they survived and were discharged from the hospital.     

Acute abdominal compartment syndrome with pulseless electrical activity during colonoscopy with conscious sedation

We report an episode of acute abdominal compartment syndrome, with pulseless electrical activity, in a patient undergoing colonoscopic examination of a recently constructed mucus fistula.Associated clinical features of this acute abdominal compartment syndrome (tension pneumoperitoneum) were abdominal distention, which was very impressive, cardiopulmonary arrest, severe cyanosis, and progressive bradycardia.In general, increased intraabdominal pressure can have numerous adverse physiologic effects, which may include decreased cardiac output, altered ventilation-perfusion relationships, and decreased venous return. The magnitude of each effect likely depends on the magnitude of the increased intraabdominal pressure.Success with initial resuscitation efforts should not diminish further vigilance with these patients, as other problems may be discovered.     

Abdominal perfusion pressure: a superior parameter in the assessment of intra-abdominal hypertension

OBJECTIVE: To assess the clinical utility of abdominal perfusion pressure (mean arterial pressure minus intra-abdominal pressure) as both a resuscitative endpoint and predictor of survival in patients with intra-abdominal hypertension. METHODS: 144 surgical patients treated for intra-abdominal hypertension between May 1997 and June 1999 were retrospectively reviewed. Multivariate logistic regression and receiver operating characteristic curve analysis of common physiologic variables and resuscitation endpoints were performed to determine the decision thresholds for each variable that predict patient survival. RESULTS: Abdominal perfusion pressure was statistically superior to both mean arterial pressure and intravesicular pressure in predicting patient survival from intra-abdominal hypertension and abdominal compartment syndrome. Multiple regression analysis demonstrated that abdominal perfusion pressure was also superior to other common resuscitation endpoints, including arterial pH, base deficit, arterial lactate, and hourly urinary output. CONCLUSION: Abdominal perfusion pressure appears to be a clinically useful resuscitation endpoint and predictor of patient survival during treatment for intra-abdominal hypertension and abdominal compartment syndrome.     

Lumbar paraspinal compartment syndrome

The purpose of this study was to systematically review the available evidence on lumbar paraspinal compartment syndrome with specific reference to patient demographics, aetiology, types, diagnosis, clinical features, and treatment. This was an Institutional Review Board-exempt study performed at a Level 1 trauma center. A PubMed search was conducted with the title query: lumbar paraspinal compartment syndrome. Eleven articles met our search criteria. Three of the patients with acute paraspinal compartmental syndrome treated with fasciotomy had a full recovery and were able to resume skiing after four months. The aetiology of the onset of lumbar paraspinal compartment syndrome is broadly divided into acute and chronic. Lumbar paraspinal compartment syndrome is one of the causes of back pain with diagnostic clinical features which should be considered in the differential diagnosis of a patient with low back pain. Prospective multicentre trials may provide the surgeon with more insight into the diagnosis and management of lumbar paraspinal compartment syndrome.     

Secondary abdominal compartment syndrome: a potential threat for all trauma clinicians

Post-injury abdominal compartment syndrome (ACS) is an increasingly recognised phenomenon in critical care. During the last decade, ACS had also been characterised in patients without abdominal injuries, referred to as secondary ACS. Recent investigation has described this elusive syndrome better, with up to 70% mortality. Regardless of the cause of the syndrome and the nature of any extra-abdominal injuries, secondary ACS is invariably associated with massive fluid resuscitation. With a reliable, predictive model and new monitoring techniques, trauma clinicians should be able to identify the high-risk patient and attenuate the impact of this syndrome.     

Secondary abdominal compartment syndrome: risk factors and outcomes

Secondary abdominal compartment syndrome (ACS), defined as intra-abdominal hypertension with associated pulmonary, renal, or hemodynamic compromise in the absence of preceding abdominal operation or injury, can markedly increase surgical morbidity and mortality. We performed a retrospective chart review of the physiologic parameters and outcomes of 10 patients with secondary ACS. Ten patients developed secondary ACS after aggressive resuscitation, at an average of 20.2 hours. Four of the patients sustained burns greater than 40 per cent, three of the patients had penetrating extremity trauma, one patient had blunt abdominal trauma, one patient was struck by lightning, and one patient developed a retroperitoneal bleed while on heparin. The average bladder pressure was 40.6. The average volume given in the first 24 hours was 33,001 cc (range, 12,400 to 69,000). The average base deficit at admission was -12 (range, +1 to -25). Seven of the 10 patients had decreased urine output. Nine of the 10 patients had decreased tidal volumes on pressure control ventilation. All 10 patients were hypotensive, with 7 of the 10 requiring vasopressors. Overall mortality was 60 per cent, with 43 per cent mortality for those decompressed. Prompt recognition and treatment are mandatory for survival of ACS. We recommend routine bladder pressure monitoring for patients with ongoing resuscitation greater than 500 cc/hr.     

A case report of systemic capillary leak syndrome (Clarkson's disease)

Systemic capillary leak syndrome (SCLS) is a very rare disorder also known as Clarkson's disease. The condition is characterized by recurrent episodes of severe capillary hyperpermeability resulting in severe hemoconcentration, hypoalbuminemia, hypovolemia and shock. We describe a 41‐year‐old previously healthy man who was admitted to hospital on several occasions with rapidly developing hypovolemic shock accompanied by extreme hemoconcentration and hypoalbuminemia. Our case is similar to other reports describing patients with SCLS where the initial suspicions have been pointing towards septic shock. He received a combination of prophylactic treatment with theophylline, β‐agonists, immunoglobulins and statins but eventually died after a severe episode of SCLS that ended with recurrent cardiac arrest. Clinical autopsy revealed pulmonary edema and acute and chronical organic fluid overload. SCLS should be kept in mind when treating patients suffering from attacks of severe idiopathic edema and mimics recurrent septic shock where no pathogen is found. The pathogenesis is unknown and the attacks may be lethal.     

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.     

The Wittmann Patch s a temporary abdominal closure device after decompressive celiotomy for abdominal compartment syndrome following burn

BACKGROUND: Abdominal compartment syndrome is frequently the result of aggressive fluid resuscitation after burn. Management of the open abdomen following decompressive celiotomy is a major problem. METHODS: From 2004 to mid-2005, six patients required decompressive celiotomy after developing abdominal compartment syndrome as a result of burn. A Wittmann Patch as used to close the abdominal wound. Patients were re-explored when clinical parameters improved and the abdomen was closed, with long-term follow-up for the abdominal wound. RESULTS: Of the six patients, five had thermal injury and one had electrical injury. The mean total body surface area affected for thermal burn was 78% and for electrical burn was 37%. Diagnosis of abdominal compartment syndrome was based on elevated bladder pressure and organ dysfunction. The patients were treated with decompressive celiotomy and Wittmann Patch closure. Survivors subsequently underwent primary abdominal closure, with no evidence of ventral hernia at long-term follow-up. CONCLUSION: In burn cases with abdominal compartment syndrome, a Wittmann Patch ay prove a helpful method of temporary abdominal closure, followed by primary closure with no complications.     

Abdominal compartment syndrome as a consequence of rectus sheath hematoma

Abdominal compartment syndrome is a well-documented entity arising from multiple and various causes. The rise of intra-abdominal pressure by the increase in volume of the peritoneal and retroperitoneal contents has been shown in the resuscitation and evaluation of surgical patients. However, the incidence of constriction of the abdomen causing intra-abdominal hypertension is unknown. Previously limited to burn eschar and externally applied devices (such as MAST trousers), external compression leading to abdominal compartment syndrome has been a limited entity. We report the first documented case of an expansive abdominal wall mass, a rectus sheath hematoma, leading to impending abdominal compartment syndrome.     

Intra-abdominal hypertension and abdominal compartment syndrome in nontrauma surgical patients

Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are commonly encountered in nontrauma surgical patients. Depending on the etiology of the patient's surgical illness (ruptured abdominal aortic aneurysm, acute pancreatitis, burns, etc.), both the incidence and mortality of IAH/ACS may be quite high. Recent advances in both the diagnosis and resuscitation of these surgical patients have resulted in significantly improved survival over that seen in years past. Intra-abdominal pressure measurements should be performed in any surgical patient who demonstrates risk factors for IAH/ACS.