Successful surgical management of congenital Kasabach–Merritt syndrome

Pascal, Sébastien; Bettex, Quentin; André, Nicolas; Petit, Philippe; Casanova, Dominique; Degardin, N.

doi:10.1111/ped.13171

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…This therapy often gives good results, but it is burdened by significant side effects especially when used for long time. 21 , 24 , 31 , 49 Vincristine is an effective chemotherapeutic drug, administered once a week at a dosage of 0.05 mg/kg/dose. Vincristine has been used in 34% of patients especially in association with steroid therapy in patients with KMS.…”

Section: Discussionmentioning

confidence: 99%

Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature

Chinello

Carlo

Olivieri

et al. 2018

Mediterr J Hematol Infect Dis

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BackgroundKaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and the first decade of life. It is locally aggressive and potentially life threatening when associated with consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of prospective trials, and the different standard care suggestions are based on retrospective case series.Case reportWe report the case of a 9-month-old male with KHE and KMS in which the initial response, obtained with prednisone and vincristine, was subsequently consolidated and strengthened by long-term treatment with sirolimus, a mTOR inhibitor. A summary of the published data is presented as well.ConclusionsThe inhibition of mTOR pathway represents the most important therapeutic innovation introduced in the last few years for KHE. Our case shows the effectiveness and good tolerance of long-term therapy with sirolimus.

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Section: Discussionmentioning

confidence: 99%

Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature

Chinello

Carlo

Olivieri

et al. 2018

Mediterr J Hematol Infect Dis

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“…Despite its e cacy, the treatment may cause side-effects when used for a long time. Non-pharmacologic treatments such as embolization can be useful, but surgery is recommended as the last option for treating KMS patients 17 .…”

Section: Discussionmentioning

confidence: 99%

Treatment of Kasabach-Merritt syndrome in infants' maxillofacial regions with local, accurate administration of urea combined with methylprednisolone

Dong

Cheng

et al. 2023

Preprint

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Infantile hemangioma is a common vascular tumor of infants. Kasabach-Merritt syndrome (KMS) is a rear but dangerous type of hemangioma. The present study analyzed the therapeutic and side effects of local administration of urea combined with methylprednisolone for the treatment of KMS in the maxillofacial region of infants. A retrospective study was conducted on the KMS patients in the department from July 2012 to September 2020. A total of 14 cases of KMS in infants’ maxillofacial region were treated with local injection of 40% urea solution and methylprednisone after the external carotid artery ligation. After 6–36 months of follow-up, 13 out of 14 cases were cured and 1 case was improved. During treatment, 2 cases relapsed and were treated continuously with local injection of urea for 6–10 more days. High efficacy and satisfactory recovery were observed in all cases after 1–2 months of treatment. No death or serious side effect occurred in the 14 cases. External carotid artery catheterization with urea and methylprednisolone injection is effective for the treatment of maxillofacial Kasabach-Merritt syndrome in infants. Careful postoperative observation and management of tube placement are important for the success of catheterization treatment.

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“…Curative surgical excision is the definitive treatment for KHE, but it may not be possible depending on the location of the tu mor 9) . Cases in which the lesions were resectable and treated with surgery have been reported 9) . However, KHEs usually show an infiltrative nature and have no clear boundaries for resection 10) .…”

Section: Discussionmentioning

confidence: 99%

Successful Management of Visceral Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Corticosteroids and Vincristine

2020

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Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neopla sm that often develops a coagulopathy known as KasabachMerritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days' gestation, weighing 2,360 g. He was admi tted for the management of respiratory failure. Blood tests showed anemia and thrombo cytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen le vels of 72.4 mg/dL, and Ddimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imag ing scan showed an illmarginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhance ment on T1 and T2weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual impro vement after its initia tion. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treat ment for 8 weeks. He remained symptomfree at the 39month followup.

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Successful surgical management of congenital Kasabach–Merritt syndrome

Cited by 5 publications

References 11 publications

Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature

Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature

Treatment of Kasabach-Merritt syndrome in infants' maxillofacial regions with local, accurate administration of urea combined with methylprednisolone

Successful Management of Visceral Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Corticosteroids and Vincristine

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