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     

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.     

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.     

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.     

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.     

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.     

Acute paediatric compartment syndrome of the hand caused by hereditary angiooedema

Compartment syndrome of the leg and forearm are well described in the literature. However, compartment syndrome of the hand is rare and in children it is even rarer. Atraumatic hand compartment syndrome has not to our knowledge been previously reported. We describe a case of an atraumatic compartment syndrome of the hand in a child who underwent an urgent fasciotomy. The child was diagnosed with hereditary angiooedema. We highlight a rare but serious complication of a hereditary disease not commonly seen by the surgical community. We hope that this report raises the awareness of this condition, thereby reducing delays in reaching a prompt diagnosis.     

Heparin-induced CVA

Surgeons commonly use heparin as prophylaxis against post-operative venous thromboembolism. Heparin-induced thrombocytopenia and thrombosis syndrome (HITTS) is a rare but potentially fatal complication of heparin therapy. We describe a case of HITTS in a 49-year-old woman, after elective cholecystectomy, which resulted in a CVA. The purpose of this case report is to increase the awareness of this phenomenon among health care professionals involved in day-care surgery and to discuss its management.     

Total knee arthroplasty in Meier-Gorlin syndrome

This report documents the case of severe knee arthritis in a 33-year-old man with a rare genetic disorder, Meier-Gorlin syndrome. The purpose of this report is to present the first case of which we are aware consisting of a patient with this syndrome requiring total knee arthroplasty, illustrating the technical difficulties associated with knee arthroplasty in this syndrome, as well as to increase awareness of the orthopedic manifestations of this uncommon genetic syndrome.     

Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report

OBJECTIVE AND IMPORTANCE: Cobb syndrome is a rare clinical entity characterized by the combination of a vascular skin nevus and an angioma in the spinal canal at the same metamere. We present a case report of an infant with Cobb syndrome. CLINICAL PRESENTATION: A 5-month-old girl presented with cutaneous hemangioma of the thoracolumbar region (T5-T12) and paraparesis. The infant was examined by magnetic resonance imaging and aortography and was diagnosed with Cobb syndrome. INTERVENTION: The patient received orally administered prednisolone therapy and underwent endovascular embolization of paravertebral and spinal angiomas with the use of n-butyl-2-cyanoacrylate. Her symptoms improved by combined treatment with liquid embolization and corticosteroid therapy. CONCLUSION: Although Cobb syndrome has been reported in older children, it is extremely rare in infants. To our knowledge, this is the first report of an infant with Cobb syndrome treated with endovascular embolization and corticosteroid therapy. The combined treatment with corticosteroid therapy and endovascular embolization of cutaneomeningospinal angioma in Cobb syndrome seems effective in alleviating symptoms and minimizing morbidity.     

Unrecognized acute exertional compartment syndrome of the leg and treatment

Acute-on-chronic exertional compartment syndrome is rare and may be easily missed without a high degree of awareness and clinical suspicion. We report a case of unrecognized acute-on-chronic exertional compartment syndrome in a recreational soccer player. The late sequela of this condition, foot drop, was successfully treated with transfer of the peroneus longus tendon.     

Acute compartment syndrome of the triceps and deltoid

Compartment syndrome is a condition in which the circulation and function of tissues within a closed space are compromised by increased pressure within that space. We report on the rare occurrence of compartment syndrome of the upper arm and deltoid in a professional power-lifter. This case stresses the importance of heightened awareness and expedient measures to prevent ischemic muscle necrosis and nerve injury.     

Surgical risk factors in Larsen's syndrome

The authors report on a child with typical Larsen's syndrome with some rare findings such as mixed-type hearing loss and with some potentially fatal operative risks including laryngomalacia and cervical instability. A few deaths with Larsen's syndrome have been reported associated with various fatal risks such as spinal instability. Therefore, laryngomalacia and several other potentially fatal risks are presented in this report as awareness may prove essential to orthopedic surgeons.     

Acute exertional compartment syndrome in an athlete.

Acute exertional compartment syndrome is a rare condition, associated with strenuous, unaccustomed exercise. This report describes its onset in a professional footballer during a regular training session. It is often diagnosed late due to lack of awareness and patient stoicism. We illustrate the consequences of delay and reinforce the need for prompt and decisive fasciotomy if complications are to be avoided.     

Prostate cancer following testosterone replacement in Klinefelter syndrome

Klinefelter syndrome is a common cause of hypogonadism. Testosterone replacement therapy has beneficial effects on bone, muscle and psychosexual function. However, it may remove the relative protection from adenocarcinoma of prostate, which is otherwise rare in uncomplicated Klinefelter syndrome. We report the case of a 55-year-old man with Klinefelter syndrome who developed prostate cancer after only 7 years of androgen supplementation. Androgen deprivation therapy was complicated by the presence of testosterone implants. The patient was treated with androgen blockade followed by radiation therapy. We recommend that serum prostate specific antigen (PSA) and digital rectal examinations be carried out during, as well as before androgen replacement.     

Spontaneous rupture of the liver associated with pregnancy. A report of 5 cases.

A resume of the aetiology, precipitating factors, pathology and management of spontaneous rupture of the liver in pregnancy, with a report of 5 cases, is presented. Misdiagnosis and late treatment contribute to the high mortality rate. Pre-operative diagnosis is very rare despite the fact that this is a diagnosable syndrome in pregnant multiparas with evidence of pre-eclampsia or eclampsia, and right-sided hypochondrial or epigastric pain which rapidly progresses to peripheral vascular collapse, often with signs of intraperitoneal haemorrhage. An awareness of this syndrome and early active surgical management constitute the best and only chance of survival for these patients.     

Scoliosis secondary to cerebrocosto-mandibular syndrome. A case report with surgical management.

STUDY DESIGN: A case report of scoliosis secondary to cerebrocosto-mandibular syndrome. OBJECTIVES: To bring about awareness of the scoliosis and respiratory problems associated with this rare syndrome. SUMMARY OF BACKGROUND DATA: Of the approximately 50 cases of this syndrome previously reported, only 3 involved scoliosis, and only 1 of these required surgery (but the patient expired). METHODS: A retrospective chart was made, and a radiologic review were done. RESULTS: A successful surgical outcome was achieved, despite severe preoperative curvature (112 degrees scoliosis) and diminished pulmonary function (21% vital capacity). CONCLUSION: This syndrome can be associated with progressive scoliosis but can be managed successfully with surgery.     

Predictable removal of anticardiolipin antibody by therapeutic plasma exchange (TPE) in catastrophic antiphospholipid antibody syndrome (CAPS)

Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare life-threatening variant of antiphospholipid antibody syndrome (APS), with an associated mortality rate of > 50%. Treatment recommendations are aggressive and consist of intravenous heparin, steroids, immunoglobulins and/or therapeutic plasma exchange (TPE). At present, insufficient data exist to make precise recommendations regarding the most effective therapy for CAPS. Accumulating evidence over recent years is encouraging and may lead to future guidelines. We report predictive and effective removal of pathological anticardiolipin antibody (aCL AB) in a patient with CAPS. The case report and discussion provide valuable insight into aCL AB production and its removal by first- order kinetics using TPE.     

"Syndrome in syndrome": Wernicke syndrome due to afferent loop syndrome. Case report and review of the literature

Wernicke syndrome is a rare neurological pathology due to a deficit in vitamin B1. The syndrome is common among alcohol abusers, patients with malignant tumor or gastrointestinal diseases, those who undergo hemodialysis or long-term peritoneal dialysis, pregnant women with hyperemesis, women who breast-feed, patients with hyperthyroidism or anorexia nervosa or gastric or jejunal-ileal bypass surgery for obesity, patients submitted to gastric surgery or prolonged total parenteral nutrition or prolonged intravenous therapy. We report a case of Wernicke syndrome due to afferent loop syndrome characterized by incoercible vomiting.     

Mitral valve replacement in idiopathic hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome is a rare systemic disease that can cause one form of endocardial fibrosis. Endocardial fibrous tissue with overlying thrombus formation in idiopathic hypereosinophilic syndrome leads to a restrictive cardiomyopathy that may produce mitral and/or tricuspid regurgitation. This report describes a patient with idiopathic hypereosinophilic syndrome who underwent mitral valve replacement and successful steroid therapy for an usually localized form of endocardial fibrosis.