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Systemic capillary leak syndrome (Clarkson's disease) during elective pylorus-preserving pancreaticoduodenectomy: case report.

Choi KM, Park CS, Kim MH - Korean J Hepatobiliary Pancreat Surg (2014)

Bottom Line: Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established.The mortality rate ranges from 30% to 76%.In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.

ABSTRACT
Systemic capillary leak syndrome (SCLS), also called Clarkson's disease is rare and life-threatening disorder of unknown etiology, which is a characteristic triad of hypovolemic shock, hemoconcentration, and hypoalbuminemia. Unexplained capillary leakage from the intravascular to the interstitial space, which has been estimated up to 70% of the intravascular volume, is the proposed mechanism. Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established. The mortality rate ranges from 30% to 76%. In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012. We describe a case of severe SCLS that suddenly occurred and rapidly progressed during pylorus preserving pancreaticoduodenectomy and review the literature.

No MeSH data available.


Related in: MedlinePlus

Episode of intraoperative hypotension (arrow).
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Figure 2: Episode of intraoperative hypotension (arrow).

Mentions: A 41-year-old, well-controlled type II diabetic woman (153 cm in height and 68 Kg in weight) was referred to the surgery department to have an elective pylorus-preserving pancreaticoduodenectomy (PPPD) for the distal bile duct cancer. Her preoperative blood pressure, pulse rate, and body temperature were 130/90 mmHg, 70/min, and 36.5℃, respectively. Laboratory studies showed leukocyte count 9,400 /ul, hemoglobin(Hb) 13.5 g/dl, hematocrit (Hct) 41.0%, C-reactive protein (CRP) 0.36 mg/dl, serum aspartate aminotransferase (AST) 124 IU/L, alanine aminotransferase (ALT) 179 IU/L, total bilirubin 3.8 mg/dl, alkaline phosphatase 1,313 IU/L, gamma-glutamyl transpeptidase 1,218 IU/L, cholesterol 328 mg/dl, and CA19-9 246 U/ml. Urine analysis and urine pregnant test were negative. Her initial total bilirubin (6.5 mg/dl) has been decreased to 3.8 mg/dl after the percutaneous transhepatic biliary drainage (PTBD) catheter was placed. A chest radiograph revealed no significant findings (Fig. 1A). An electrocardiogram and echocardiogram did not show any abnormal signs. Two days prior to surgery, the patient's body temperature had increased to 38.7℃, with normal range of white cell count and C-reactive protein, and the patient had no subjective symptoms. The body temperature was normalized soon without medication. On the operation day, pentothal sodium and vecuronium were administered to facilitate a tracheal intubation, and the anesthesia was maintained with nitrous oxide and desflurane in oxygen. At this point, the arterial blood pressure was 140/80 mmHg, heart rate, 80 bpm, central venous pressure (CVP) 10 mmHg and the oxygen saturation 100%, bispectral index (BIS) 45-50. About three hours forty minutes after the operation started, when the surgical specimen was removed out and the intestinal anastomosis was about to start, the end-tidal carbon dioxide (EtCO2) curve was abruptly reduced and then, disappeared. At the same time, her arterial blood pressure, CVP, and oxygen saturation abruptly decreased to 40/25 mmHg, 3 mmHg, and 88% respectively (Fig. 2) and BIS value was not checked without sudden blood loss or unusual conditions. There was no evidence of airway obstruction. Intravenous fluid (1,000 ml of crystalloid and 3,000 ml of colloid) were rapidly infused and cardiopulmonary resuscitation including cardiac massage, ephedrine, phenylephrine, epinephrine, and atropine were conducted for 20 minutes. After that, her vital signs were recovered to 130/80 mmHg but, BIS value was not. Intraoperative transesophageal echocardiogram (TEE) demonstrated normal heart function. Her vital signs were stable until the surgery finished.


Systemic capillary leak syndrome (Clarkson's disease) during elective pylorus-preserving pancreaticoduodenectomy: case report.

Choi KM, Park CS, Kim MH - Korean J Hepatobiliary Pancreat Surg (2014)

Episode of intraoperative hypotension (arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4492332&req=5

Figure 2: Episode of intraoperative hypotension (arrow).
Mentions: A 41-year-old, well-controlled type II diabetic woman (153 cm in height and 68 Kg in weight) was referred to the surgery department to have an elective pylorus-preserving pancreaticoduodenectomy (PPPD) for the distal bile duct cancer. Her preoperative blood pressure, pulse rate, and body temperature were 130/90 mmHg, 70/min, and 36.5℃, respectively. Laboratory studies showed leukocyte count 9,400 /ul, hemoglobin(Hb) 13.5 g/dl, hematocrit (Hct) 41.0%, C-reactive protein (CRP) 0.36 mg/dl, serum aspartate aminotransferase (AST) 124 IU/L, alanine aminotransferase (ALT) 179 IU/L, total bilirubin 3.8 mg/dl, alkaline phosphatase 1,313 IU/L, gamma-glutamyl transpeptidase 1,218 IU/L, cholesterol 328 mg/dl, and CA19-9 246 U/ml. Urine analysis and urine pregnant test were negative. Her initial total bilirubin (6.5 mg/dl) has been decreased to 3.8 mg/dl after the percutaneous transhepatic biliary drainage (PTBD) catheter was placed. A chest radiograph revealed no significant findings (Fig. 1A). An electrocardiogram and echocardiogram did not show any abnormal signs. Two days prior to surgery, the patient's body temperature had increased to 38.7℃, with normal range of white cell count and C-reactive protein, and the patient had no subjective symptoms. The body temperature was normalized soon without medication. On the operation day, pentothal sodium and vecuronium were administered to facilitate a tracheal intubation, and the anesthesia was maintained with nitrous oxide and desflurane in oxygen. At this point, the arterial blood pressure was 140/80 mmHg, heart rate, 80 bpm, central venous pressure (CVP) 10 mmHg and the oxygen saturation 100%, bispectral index (BIS) 45-50. About three hours forty minutes after the operation started, when the surgical specimen was removed out and the intestinal anastomosis was about to start, the end-tidal carbon dioxide (EtCO2) curve was abruptly reduced and then, disappeared. At the same time, her arterial blood pressure, CVP, and oxygen saturation abruptly decreased to 40/25 mmHg, 3 mmHg, and 88% respectively (Fig. 2) and BIS value was not checked without sudden blood loss or unusual conditions. There was no evidence of airway obstruction. Intravenous fluid (1,000 ml of crystalloid and 3,000 ml of colloid) were rapidly infused and cardiopulmonary resuscitation including cardiac massage, ephedrine, phenylephrine, epinephrine, and atropine were conducted for 20 minutes. After that, her vital signs were recovered to 130/80 mmHg but, BIS value was not. Intraoperative transesophageal echocardiogram (TEE) demonstrated normal heart function. Her vital signs were stable until the surgery finished.

Bottom Line: Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established.The mortality rate ranges from 30% to 76%.In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.

ABSTRACT
Systemic capillary leak syndrome (SCLS), also called Clarkson's disease is rare and life-threatening disorder of unknown etiology, which is a characteristic triad of hypovolemic shock, hemoconcentration, and hypoalbuminemia. Unexplained capillary leakage from the intravascular to the interstitial space, which has been estimated up to 70% of the intravascular volume, is the proposed mechanism. Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established. The mortality rate ranges from 30% to 76%. In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012. We describe a case of severe SCLS that suddenly occurred and rapidly progressed during pylorus preserving pancreaticoduodenectomy and review the literature.

No MeSH data available.


Related in: MedlinePlus