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.     

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     

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.     

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.     

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.     

Distribution of Expiratory Gas and Rebreathing in T-piece Modifications Used in Weaning

The simple T-piece is frequently used as a weaning system during respirator treatment. It is modified with an expiratory non-compliant reservoir (EnCR), an inspiratory compliant reservoir (ICR) and/or an expiratory one-way valve. The distribution of expiratory gases and rebreathing were studied in a model set-up in the corresponding systems at different fresh gas flows (FGF) and tidal volumes (V_T). An EnCR produces no change, whereas an ICR causes the expiratory gas to flow into the inspiratory limb, an effect which is intensified by the presence of an expiratory valve. With a falling FGF and a rising V_T, increasing amounts of expiratory gas are found in the inspiratory limb in the modifications with an ICR. However, this only gives rise to rebreathing in the valve modification with a low FGF and high V_T. The modification of the T-piece with an ICR but without a one-way valve is advantageous, as this system combines only slight dilution via the expiratory limb and a minimal risk of rebreathing.     

A 12-year-old boy with an infected ectopic ureter presenting with acute appendicitis-like symptoms and acute scrotum

An ectopic ureter draining into the seminal vesicle or vas deferens in males is a very rare anomaly and is usually associated with renal dysplasia or agenesis. An ectopic ureter associated with a dysplastic kidney is not usually a suspected cause during clinical evaluation of children with abdominal pain. This report presents a rare case of an ectopic ureter associated with a dysplastic kidney with an acute infection in a previously healthy 12-year-old boy, demonstrated by magnetic resonance imaging. He presented with abdominal pain that mimicked acute appendicitis-like symptoms which was subsequently complicated by epididymitis manifesting as an acute scrotum. Clinicians should consider including an ectopic ureter in the differential diagnosis of children presenting with acute abdomen.     

Selective treatment of duodenal ulcer with perforation.

Selective treatment of duodenal ulcer with perforation has been based on several premises: 1) The natural history of the ulcer following closure of a perforation is generally favorable with an acute and unfavorable with a chronic ulcer. 2) An upper gastrointestinal series with water soluble contrast media can reliably document a spontaneously sealed perforation. 3) With a spontaneous seal, nonsurgical therapy is an acceptable option and is preferable for an acute ulcer or a chronic ulcer with poor surgical risk. 4) The treatment of choice for an unsealed perforation of an acute ulcer is simple surgical closure. 5) The treatment of choice of perforation of a chronic ulcer with acceptable surgical risk is an ulcer definitive operation. Sixty cases of perforation of duodenal ulcer have been treated. Nonsurgical therapy was employed without complication in eight cases with radiologically documented spontaneous seal. Truncal vagotomy and pyloroplasty in 36 cases and truncal vagotomy and antrectomy in two cases were each without mortality. Four fatalities occurred among 13 cases of closure and omental patch, each a case with severe associated disease. The mortality was 6.7% among the 60 cases; 2.4% for chronic ulcer and 16% for acute ulcer.     

Post dural puncture headache in a pediatric patient with idiopathic intracranial hypertension

We describe the occurrence of postdural puncture headache (PPDH) in an adolescent with idiopathic intracranial hypertension (IIH) and its successful management with an epidural blood patch. PPDH is a very rare occurrence in patients with intracranial hypertension and is described as a paradoxical situation in the literature. There are only two previous case reports (in adults) of the possible association. A 15-year-old obese patient with a diagnosis of IIH had an uneventful diagnostic spinal tap using a 22G Quincke needle in the pediatric emergency department but returned 24 h later with PPDH. After a failed trial of conservative management, she had an uneventful but curative epidural blood patch with 15 ml of autologous venous blood and was able to return to school the day after the blood patch. Follow-up review by her neuro-ophthalmologist shows resolution of her headaches, considerable improvement in her visual field defect and resolution of papilledema. This is the first report of PPDH and its successful management with an epidural blood patch in a pediatric patient with IIH.     

Chronic suppurative cryptococcal extensor tenosynovitis in a patient with Castleman's disease: a case report

Cryptococcus neoformans is an ubiquitously occurring encapsulated fungus that is commonly found in the environment. It is also an opportunistic pathogen that has potential to cause systemic fungal infection, predominantly in the immunocompromised host with cell-mediated immunological defects. Cryptococcal tenosynovitis is an extremely rare condition, with only a few cases previously documented in the literature. We report a case of chronic suppurative cryptococcal extensor tenosynovitis in a patient with Castleman's disease who was successfully managed with surgical debridement and antifungal therapy.     

Does aspirin attenuate the effect of angiotensin-converting enzyme inhibitors in hypertension or heart failure?

There is a wealth of data that suggests an important interaction between aspirin and angiotensin-converting enzyme inhibitors in patients with chronic stable cardiovascular disease. The interaction is less obvious in the postinfarction setting, possibly reflecting the fact that many patients stop their aspirin therapy within a few months of such an event. An interaction is biologically plausible, because there is considerable evidence that angiotensin-converting enzyme inhibitors exert important effects through increasing the production of vasodilator prostaglandins, whereas aspirin blocks their production through inhibition of cyclooxygenase, even at low doses. There is some evidence that low-dose aspirin may raise systolic and diastolic blood pressure. There is also considerable evidence that aspirin may entirely neutralize the clinical benefits of angiotensin-converting enzyme inhibitors in patients with heart failure. In addition, aspirin may have an adverse effect on outcome in patients with heart failure that is independent of any interaction with angiotensin-converting enzyme inhibitors, possibly by blocking endogenous vasodilator prostaglandin production and enhancing the vasoconstrictor potential of endothelin. The evidence is not sufficient to justify advising long-term aspirin therapy for patients with cardiovascular disease in general, and for those with heart failure in particular. Thus, the lack of evidence of benefit with aspirin in patients with heart failure and coronary disease, along with growing evidence that aspirin is directly harmful in patients with heart failure and that aspirin may negate the benefits of angiotensin-converting enzyme inhibitors suggest that, unless there is an opportunity to randomize the patient into a study of antithrombotic strategies, then aspirin should be withdrawn or possibly substituted with an anticoagulant or an antiplatelet agent that does not block cyclooxygenase. In contrast, there is fairly robust evidence for a benefit of both aspirin and angiotensin-converting enzyme inhibitors during the first 5 weeks after a myocardial infarction, with little evidence of an interaction. The combination of aspirin and angiotensin-converting enzyme inhibitors is warranted during this period, after which discontinuation or substitution of aspirin with another agent should be considered.     

Anterior urethral valve in the fossa navicularis presenting as a split urinary stream in a child

An anterior urethral valve is uncommon compared to a posterior urethral valve as a cause of lower obstructive uropathy. Furthermore, an anterior urethral valve in the fossa navicularis is extremely rare. We describe the case in a 6-year-old boy who presented with a split urinary stream. Endoscopy revealed an anterior urethral valve in the fossa navicularis, and we successfully incised the valve with a hook knife. We should consider the possibility of an anterior urethral valve in any child with an abnormal urinary stream.     

Acute abdominal pain: the importance of genital examination

Torsion of an intra-abdominal testis is a rare cause of acute abdominal pain. The purpose of this report is to highlight a case of torsion of an abdominal testis in a patient with an unusual clinical presentation, with the fortuitous diagnosis of a "carcinoma in situ" and to emphasize the role of routine genital examination when investigating patients with abdominal pain.     

Diverticular abscess presenting as an incarcerated inguinal hernia

Acute diverticulitis may present with an abscess that is usually pericolonic or pelvic and can be treated with urgent surgery or percutaneous drainage. We present a case of a diverticular abscess presenting as a left inguinal hernia. This is analogous to an Amyand's hernia in which an inflamed appendix is found in a right inguinal hernia. The patient presented was managed with open drainage of the hernia and subsequent laparoscopic sigmoid resection in the same hospitalization.     

Obstructive nephropathy as a result of retroperitoneal fibrosis: a review of its pathogenesis and associations.

Retroperitoneal fibrosis is a rare disease, typically with an insidious clinical course. It is thought that this disease process is perhaps an exaggerated reaction to an inciting inflammatory event. In this study, a case of retroperitoneal fibrosis is reported, in which the patient presented with typical symptoms of retroperitoneal fibrosis, along with some atypical vasculitic symptomatology. Retroperitoneal fibrosis is a disease process with an unknown etiology, which has been observed to be associated with a number of different possible inciting factors. Two factors that have been documented in the literature as being associated with retroperitoneal fibrosis include the use of beta-blocking agents, and connective tissue disease processes such as systemic lupus erythematosus. The patient discussed was using beta-blocker medication and also had signs and symptoms suggestive of a lupus syndrome. There are no reported cases of the combined association of beta-blocker usage, lupus, and retroperitoneal fibrosis.     

Le Problème des Fins de Vie aux Soins Intensifs et la Nouvelle Loi sur l’Euthanasie

Torsion of an intra-abdominal testis is a rare cause of acute abdominal pain. The purpose of this report is to highlight a case of torsion of an abdominal testis in a patient with an unusual clinical presentation, with the fortuitous diagnosis of a “carcinoma in situ” and to emphasize the role of routine genital examination when investigating patients with abdominal pain.     

FATAL EPISTAXIS FROM AN ANEURYSM OF THE INTRACRANIAL INTERNAL CAROTID ARTERY

An instance of fatal epistaxis is reported in a patient with an unsuspected aneurysm of the infraclinoid portion of the internal carotid artery. There was no known history of trauma. The aneurysm was subsequently detected on an old X-ray film. Epistaxis from an aneurysm at this site is nearly always preceded by significant head trauma and is associated with cranial nerve palsies, a syndrome with a high mortality. Epistaxis from rupture of a non-traumatic aneurysm is very rare.     

Slipped upper femoral epiphysis

Slipped upper femoral epiphysis (SUFE) is a common hip disorder of adolescence, with unknown aetiology. The typical patient presenting with SUFE is an overweight adolescent boy, who reports pain in the groin, thigh or knee, with an associated limp. SUFE is defined as stable when the patient can walk, with or without support. The current treatment of choice for stable SUFE is by fixation in situ with a single screw. This method has a high probability of success, with minimal risk of osteonecrosis or chondrolysis. In those patients presenting with an unstable SUFE, immediate closed reduction and fixation with a single screw provides the best chance of a satisfactory result.     

Abdominoscrotal hydrocele: an insight into its origin

Abdominoscrotal hydrocele is an hourglass hydrocele with both an inguinoscrotal and abdominal component. It is a rare surgical entity with few cases reported in the literature. We report a case of abdominoscrotal hydrocele that gives an insight into its pathogenesis.