Laparoscopic cholecystectomy for symptomatic cholecystolithiasis (CCL) in “Kasabach–Merritt syndrome” (KMS) (Kaposi-tumor like hemangioendothelioma with case-specific perioperative management)
Authors: Arndt Stephan , Wex Cora , Häusler-Pliske Inken , Jechorek Dörthe , Krause Hardy , Halloul Zuhir , Meyer Frank 
Published: August 2023

Objectives

The Kasabach–Merritt syndrome (KMS) is characterized by the occurrence of hemangioendothelioma (giant hemangioma with thrombosis leading to thrombocytopenia), which can be associated with disseminated intravasal coagulation. Specific aim: Based on (i) selective references from the current scientific literature and derived recommendations as well as (ii) own experiences obtained in the diagnostic and perioperative management of a representative case from daily practice in abdominal surgery, the specific case undergoing elective cholecystectomy (CCE) in KMS is to be described by means of scientific case report.

Case presentation

(Patient-, finding- and treatment-specific characteristics): – Medical history: 72-years old female patient with a known KMS of the left arm and upper thorax, recurrent thrombophlebitis of the left arm and thoracic veins, previous upper GI bleeding (Mallory-Weiss syndrome in 2006, chronic anemia in lack of vitamin B12, type-A gastritis, former bleeding complications after teeth extraction/open appendectomy 1962/Caesarean section 1968 with need of transfusion [60 red blood cell packages]), intraabdominal adhesions, hypothyreosis, initial liver cirrhosis. – Symptomatology: Characteristic for cholecystolithiasis (CCL). – Diagnostic: Abdominal ultrasound shows CCL, fibroscan does not confirm suspicious cirrhosis. Laboratory parameters showed: Activation of intravasal coagulation with elevated prothrombin fragments, D-dimers and reduced antiplasmin concentration. Accelerated fibrinolysis capacity; currently, no secondary thrombocytopenia or factor-13 decrease. In addition, fibrinogen concentration within normal range, no hint onto the manifestation of an aquired von-Willebrand’s syndrome. – Diagnosis: Chronic fibrosing cholecystitis in CCL after former acute cholecystitis (3 months ago) with indication for surgical intervention. – Therapy: Laparoscopic CCE including careful exploration of upper abdominal cavity for KMS manifestation (with no revision of bile duct) and peritoneal adhesiolysis (histological finding, chronic fibrosing cholecystitis with thickening of the wall of the gall bladder but no hint of malignancy) under perioperative prophylaxis with antibiotics and temporary cessation of platelet medication for 7 d preoperatively, “bridging” with low molecular weight heparin (Clexane, 1 × 40 mg s.c.; Sanofi-Aventis, Frankfurt/Main, Germany); 1 h preoperatively, 15–20 mg/kg body weight Cyclocapron i.v. (once again 6–8 h postoperatively; thereafter, 500 mg of Cyclocapron 4×/d until the 3rd postoperative day). – Intraoperatively: Congestion of veins but not at the immediate surgical field (gall bladder, hepatic bed of the gall bladder, Calot’s triangle). – Outcome: Uneventful, in particular, no (bleeding) complications.

Conclusions

If surgical approach is indicated, the intervention should be thoroughly planned (in particular, under elective circumstances) with regard to hemangioma site and extension as well as distance to the surgical field and possible surgical alternative options (surgical access site, open/laparoscopic approach etc.) to prevent – at the best possible rate – bleeding complications intra-/postoperatively and, thus, to provide adequate patient safety.