Treatment Of Kasabach-Merritt Syndrome Research Articles

Vincristine combined with glucocorticoids for the treatment of 11 cases of infantile Kasabach-Merritt syndrome

Objective To explore effective therapy for Kasabach-Merritt syndrome (KMS) . Methods Clinical data were collected from 11 infants with KMS in the Department of Dermatology, Beijing Children′s Hospital affiliated to Capital Medical University between 2015 and 2017, and the efficacy of vincristine combined with glucocorticoids for the treatment of KMS was evaluated. Results Of the 11 patients, 4 were male and 7 were female. Their average age at the first clinic visit was 87.91 ± 72.01 days (range, 1-212 days) . Most of the hemangiomas manifested as hard violaceous plaques, and skin purpura occurred around the hemangiomas in 5 patients. The initial blood platelet counts of the 11 patients ranged from 4 × 109/L to 32 × 109/L. All the patients were treated with oral prednisone at doses of (2-5) mg·kg-1·d-1 combined with intravenous injection of vincristine at a dose of 0.05 mg/kg every week. Among 10 patients treated with glucocorticoids and vincristine, the blood platelet counts became normal after (1.8 ± 1.23) weeks of treatment, the fibrinogen levels returned to normal after (3.6 ± 1.26) weeks of treatment, and the hemangiomas started to become softer and smaller after (3.9 ± 0.74) weeks. The blood platelet count still did not return to normal in 1 patient after 5 weeks of vincristine treatment. After the treatment with venous embolization, the blood platelet count gradually became normal and remained stable. Conclusion Vincristine combined with glucocorticoids can control the development of hemangioma in infants with KMS, and improve the recovery of blood platelet count. Key words: Hemangioma; Thrombocytopenia; Vincristine; Glucocorticoids; Treatment outcome

Haemangioma, thrombocytopenia and coagulopathy- kasabach merritt syndrome: an appraisal for anaesthesiologists.

Sir, Haemangioma is a common type of vascular tumour in paediatric age group [1]. Most of them are benign and non-life threatening [1]. Kasabach Merritt syndrome (KMS) is a combination of giant haemangioma, thrombocytopenia and consumptive coagulopathy [1–5]. It presents with diverse clinical presentation and may be a part of other recognized syndrome [1,2]. KMS is a well-recognized but rare clinical entity that may be life threatening [1–4] and bears considerable anesthetic implications [3–5]. Here, we would like to report a case of an 11-year-old boy, after obtaining a written permission from parents, who came to us for preoperative evaluation with a haemangioma (15.7×13.3 cm in maximum dimension) involving the left forearm [Table/Fig-1]. It began to appear as a small lesion without causing any associated problem at the age of three years. Though the patient have attended various medical services, he was not provided with any conclusive diagnosis nor there any improvement in his symptomology. During the last eight years it enlarged many folds with destruction of radius, erosion & remodeling of ulna [Table/Fig-2]. As there was profound bonny destruction, an above elbow amputation was planned by his surgical team. His bed side physical examination did not reveal any other significant abnormality. All preoperative lab parameters were normal except for a platelet count of 72,000/μL. A subsequent coagulation profile revealed a prothombin time prolonged by five seconds and international normalized ratio (INR) of 1.52.Considering the possibility of KMS, he was referred to haematology service. He was diagnosed with KMS by the haematology service and no preoperative transfusion was advised. The possibility of massive blood loss and institution of massive blood transfusion protocol was discussed and planned accordingly. He was provided general anaesthesia with endotracheal intubation and mechanical ventilation during the surgery. Total anaesthesia time was 90 min and trachea was extubated at the end of surgery. We did not encounter any reduction of platelet count or deterioration of coagulation profile during the intraoperative period and there was no need of intraoperative blood transfusion. On the 1st postoperative day his platelet count raised to 1, 20,000 /μL and INR normalized to 1.3. Histology of the resected specimen revealed kaposiform haemangio endothelioma, which is the commonest histological type associated with KMS [1]. [Table/Fig-1]: Haemangioma involving the left forearm [Table/Fig-2]: X-ray of the left forearm showing a large expansile soft tissue density (arrow mark) lesion in the mid and distal forearm causing profound bone erosion and deformity, especially involving the radius and also the ulna A practice derived consensus statement discussing diagnostic workup, surgical and non-surgical treatment modalities of KMS has been published very recently [2]. Apart from the problems associated with anatomic locations of the tumour [1,3] and haematological abnormalities [1–5], cardiac derangement from high-volume arterio-venous shunting is also a concern [2–4]. The anaesthetic implications of pharmacological measures used during the treatment of KMS should also be addressed with assiduity [1–3]. Anaesthesia and surgery may possibly trigger disseminated intravascular coagulation and induce severe bleeding [3]. Although KMS is associated with mortality and morbidity [1,2], case reports of successful perioperative outcome is also available in literature [3,5]. Recently, survival benefit has been documented in patients with traumatic hemorrhage that were managed with hemostatic therapy guided by viscoelastic point-of-care coagulation devices (thromboelastography) [6]. Thromboelastography is also increasingly being used in hemorrhage of non-traumatic origin [7]. In a patient with KMS, FloTrac/Vigileo system was used successfully to guide appropriate resuscitative measures that ensured stable perioperative hemodynamics [5]. Thus, the authors believe that the impact of use of viscoelastic point-of-care coagulation and noninvasive cardiac output monitoring devices on mortality and morbidity of patients with KMS should be evaluated. Haemangioma is a common condition in pediatric age group [1,2], thus anaesthesiologists are expected to encounter patients with hemangioma frequently. KMS is a rare clinical manifestation in patients with haemangioma, moreover its clinical features are diverse [1–7]. Although this particular case did not pose much challenge in the perioperative period, this discussion should serve to remind anesthesiologists of the possible complications and management issues that may be encountered with KMS.

Treatment of Kasabach-Merritt syndrome in three infants by percutaneous selective digital subtraction angiography combined with transarterial hardened embolization

Objective To develop a new treament strategy for Kasabach-Merritt syndrome.Methods Three infants who were diagnosed with Kasabach-Merritt syndrome and suffered from thrombocytopenia as well as bleeding and clotting disorders were treated with percutaneous selective digital subtraction angiography combined with transarterial hardened embolization under general anesthesia.Sclerosing agents included bleomycin A5 (4.0 mg),iodized oil (1.5 ml),dexamethasone (2.5 mg) and iopamidol (3 ml).Polyvinyl alcohol mixed with iopamidol (at a volume fraction of 0.5) served as the embolic material.Results All the three patients were successfully treated by the minimally invasive surgery.The amount of blood platelet returned to normal within 24 hours after the operation.On the fourth day,all the patients were discharged from hospital with the restoration of coagulation function.Revisits at one month and three months after the operation showed that hemangiomas markedly shrank and even subsided,and blood platelet count was maintained within normal range.Conclusions Percutaneous selective digital subtraction angiography combined with transarterial hardened embolization can result in a recovery of blood platelet count and shrinkage of hemangioma,and may serve as a minimally invasive treatment option for Kasabach-Merritt syndrome.

Klippel-Trenaunay syndrome: clinical features, complications and management in children.

Clinical features and management of 47 children with Klippel-Trenaunay syndrome treated since 1970 were reviewed. Haemangiomas and soft tissue and/or skeletal hypertrophy were present in all 47 patients; venous varicosities developed in 37 (79 per cent). There was no clinical evidence of macrofistulous arteriovenous communications in any patient. Thromboembolic episodes occurred in five children (11 per cent) and 25 (53 per cent) experienced thrombophlebitis. The Kasabach-Merritt syndrome was observed in 21 (45 per cent) and six (13 per cent) presented with high-output cardiac failure. Other manifestations included haematuria in five (11 per cent), rectal or colonic haemorrhage in six (13 per cent), and vaginal, vulval or penile bleeding in six (13 per cent) children with visceral and pelvic haemangiomas. In 26 patients (55 per cent) symptomatic treatment only was required. Surgery was undertaken in selected cases for complications of the haemangioma, for cosmetic reasons and for chronic venous insufficiency. Only one of four children who underwent resection of varicose veins improved. There was no death, but significant morbidity was associated with the treatment of Kasabach-Merritt syndrome and high-output cardiac failure.

Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome.

The purpose of the present study was to evalutate the various treatment modalities for Kasabach-Merritt syndrome (KMS) and to identify the most reliable treatment modalities. A retrospective analysis was performed on 37 KMS patients who were admitted to Seoul National University Hospital between January 1979 and June 1999. Age, sex, locations of the hemangiomas, clinical symptoms and hematologic data were analyzed by multivariate logistical regression analysis to determine the response to various treatment modalities. Twenty-four of 37 patients (20 boys and 17 girls) were diagnosed during infancy. The locations of hemangioma were superficial skin in 31 patients, combined skin and visceral organs in four patients and visceral organs only in two patients. Tumor size was more than 5 cm in diameter in all cases, except for one. The treatment principle of KMS in our center involves a stepwise multimodal approach. Of the 37 cases, surgical resection was performed in two. Steroids were tried initially in 35 patients over a 1 week period, with an initial response rate of 11.4% (4/35). Combined steroid/radiation treatment was given in 28 cases, with a response rate of 75.0% (21/28). Four of five patients with additional interferon (IFN)-alpha therapy improved. No significant correlations were found between the prognostic factors, such as age, sex, size and locations of the hemangioma, hematologic data and the treatment modalities. Based on the experiences in a single center over a period of 20 years, we recommend a stepwise multimodal approach for the treatment of KMS; for example, steroid, radiation therapy and IFN-alpha in this order. However, surgical treatment is helpful if total resection is possible.

Treatment of Kasabach-Merritt syndrome

Treatment of Kasabach-Merritt syndrome

Use of 111In platelet scans in the detection and treatment of Kasabach-Merritt syndrome

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