Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy

Abstract

Background: Systemic drug therapy is generally recommended for infant huge vascular anomalies associated with thrombocytopenia and coagulopathy, but some patients are not suitable due to drug unresponsiveness or life threatening conditions before the drug works, who will need to receive surgical treatment. This study retrospectively analyzed the clinical features, imaging features, and surgical outcomes of these patients. Methods: The clinical data of 4 infants with huge vascular anomalies (2 vein malformations (VMs) and 2 kaposiform hemangioendothelioma (KHE)) associated with thrombocytopenia and coagulopathy treated from June 2016 to December 2017 were retrospectively analyzed. All patients received glucocorticoids, propranolol, vincristine or sirolimus treatment before admission, but the treatment was ineffective. Skin petechia, thrombocytopenia and coagulopathy were present at the time of admission. CT scanning was performed before operation. The patient's general clinical data, hematological examination results, operation time, surgical bleeding volume, blood transfusion volume and surgical complications were collected for analysis. The patients were followed up for 10-26 months. Results: CT scanning results of 2 patients showed special CT features without detectable enhancement within the lesion after CT enhanced scanning and multiple phleboliths formation. Four patients underwent surgical treatment successfully. Two patients underwent complete resection of the lesion, and 2 underwent cytoreductive surgery. Preoperative clinical symptoms such as skin petechia, thrombocytopenia and coagulopathy were normal at 1 week after surgery. Postoperative pathological results showed 2 cases of KHE and 2 cases of VMs. All patients were discharged from hospital without physical dysfunction, recurrence, or death.

Combination therapy for pediatric patients with Kasabach–Merritt phenomenon: A single-center retrospective study

“…Although the surgical approach is not considered as the first-line therapy for KMP patients, it is highly recommended when the effects of other drugs are not obvious, especially in cases when the lesion is extensive. [16] Embolization has been widely applied for achieving a complete occlusion in the vascular tumor, including in patients with KMP. [17] In our case, all of the 12 patients had received arterial embolization in combination with steroid therapy or sirolimus, and they were ultimately, free of disease.…”

Curative effect of surgical resection in the treatment of Kaposiform hemangioendothelioma

“…While prednisolone, vincristine and sirolimus showed some effectiveness in decreasing the tumor size 16,17 , can cause side effects such as abdominal pain, elevations of aspartate aminotransferase (AST) and alanine aminotransferase (ALT), opportunistic infection, loss of appetite, etc 18 . Moreover, there are case reports of visceral KHE not responding to sirolimus 19,20 . In addition, for those KHE patients unresponsive to glucocorticoids, propranolol, vincristine or sirolimus treatment, surgical therapy reduced skin petechia and coagulopathy to normal levels.…”