|Year : 2016 | Volume
| Issue : 1 | Page : 58-60
Kasabach Merritt syndrome: Is there a role of surgery?
Priya Dhruv Dhandore, Narendra Narayan Hombalkar, Chandrakant Kamlesh Pancholi
Department of General Surgery, Government Medical College Miraj and P. V. P. Government Hospital, Sangli, Maharashtra, India
|Date of Web Publication||6-Jan-2016|
Chandrakant Kamlesh Pancholi
Ex-surgery resident, Department of General Surgery, Government Medical College Miraj and P. V. P. Government Hospital, Sangli, Maharashtra
Source of Support: None, Conflict of Interest: None
Kasabach–Merritt syndrome (KMS) is a rare clinical presentation in patients with preexisting hemangioma. Abnormal trapping of platelets within proliferating blood vessels and subsequent premature activation of coagulation cascades lead to thrombocytopenia and coagulopathy. Management of KMS is often challenging particularly in neonates without any fixed protocols for treatment. Management strategies include use of corticosteroids, propranolol, compression, embolization, use of interferon, laser therapy, sclerotherapy, chemotherapy, radiation, and/or surgery. The treatment plan should be individualized depending on type of vascular anomalies, age at presentation and patient response. We hereby present a rare case of KMS in a neonate with giant congenital hemangioma successfully managed surgically at our institute.
Keywords: Congenital hemangioma, Kasabach–Merritt syndrome, thrombocytopenia
|How to cite this article:|
Dhandore PD, Hombalkar NN, Pancholi CK. Kasabach Merritt syndrome: Is there a role of surgery?. J Clin Neonatol 2016;5:58-60
| Introduction|| |
Kasabach–Merritt syndrome (KMS) is characterized by giant hemangiomas, severe thrombocytopenia and consumptive coagulopathy which may result in life-threatening multi-organ hemorrhage. KMS is usually associated with Kaposiform hemangioendothelioma (KHE), tufted angiomas and rarely with congenital hemangiomas (CHs). Almost 200 cases have been reported in the literature since Kasabach and Merritt described the first case in 1940. More than 80% of cases occur within the 1st year of life. The index case presented with large CH over right thigh since birth along with thrombocytopenia and coagulopathy. The patient was started on steroid and propranolol therapy but was ineffective. Surgical excision was performed to prevent further microangiopathic hemolytic anemia and life-threatening hemorrhage. In the current case report, we would like to present the challenges faced in the management of KMS along with a short review of the literature.
| Case Report|| |
A 24 h old full term male child, third by birth order, born of consanguineous marriage with vaginal delivery and 2.75 kg birth weight, was referred with giant hemangioma over right thigh since birth along with thrombocytopenia. No other external visible congenital anomalies were noted. On local examination, 10 cm ×6 cm ×4 cm nonpulsatile, non tender, warm and minimally-compressible swelling over medial aspect of right thigh was noted [Figure 1]. There was no thrill and no bruit was heard on auscultation. The systemic examination was within normal limits. Blood investigations revealed anemia, leukocytosis, and thrombocytopenia (hemoglobin 10 g%, total leukocyte count 15,500/cmm with 68% polymorphs and 32% lymphocytes, platelet count was 20,000/cmm, prothrombin time was 29 s with INR of 2.1. The bleeding time was 4 min). Ultrasound and color Doppler examination suggested it to be large hemangioma over right thigh with dilated femoral vessels feeding the hemangioma. There was an indistinct plane with underlying muscles [Figure 2]. Ultrasound of the abdomen was normal. The chest X-ray was within normal limits. The patient was treated conservatively with fluid resuscitation. The baby was put on steroid therapy. Prednisolone 2 mg/kg/day along with propranolol 2 mg/kg/day were started. Daily complete blood count with coagulation profile was monitored. On day 6 of life platelet count dropped to 7000/cubic mm and patient started oral bleed. There was evidence of hemolytic anemia [Figure 3] with total bilirubin levels rising up to 11 mg%. In view of continuing hemolysis, surgical intervention was planned to prevent further hemolysis and life-threatening multi-organ hemorrhage. Emergency excision of hemangioma was performed under general anesthesia. Primary closure of the defect could be obtained with skin flap mobilization [Figure 4]. The baby had stormy postoperative course and required ventilatory support. The baby was managed with fuid resuscitation, antibiotics, platelet transfusion and packed cell transfusion as per requirement. The baby was weaned off the ventilator support on the 3rd postoperative day. The steroid supplementation was continued in the postoperative period and then gradually tapered off. The biochemical parameters were monitored daily. Platelets count was also monitored daily which subsequently showed rising trends (from 15,000/cubic mm on day 2, to 80,000/cubic mm on day 8). The histopathological report of excised tissue specimen also confirmed the findings of congenital hemangioma with proliferating blood vessels [Figure 5]. The operative wound had healed well and the patient was discharged from neonatal intensive care on postoperative day 20.
|Figure 3: Peripheral smear showing dysplastic, nucleated and hypochromic red blood cells along with reduced platelets|
Click here to view
|Figure 5: Histopathological tissue section of excised hemangioma showing proliferating vascular channels|
Click here to view
| Discussion|| |
The main diagnostic features of KMS are giant hemangioma and decreased platelet count and consumptive coagulopathy. Giant cutaneous hemangiomas are easy to diagnose on physical examination. However visceral hemangiomas are easily missed, and patients may present with large ecchymosis with unexplained thrombocytopenia and coagulation disorders. A retrospective study conducted by Enjolras et al. revealed that KMS was more common due to KHE in up to 90% of cases and less common with infantile and CHs. The residual lesions of patients with cured KMS were predominantly tufted angiomas, whereas KHE was more common during the active phase of KMS. Thus, it is possible that KHE and tufted angiomas are the part of the same neoplastic spectrum and histologic continuum., The trigger factors for the development of KMS include surgical intervention, pregnancy, angiography, and needle aspiration of hemangioma. Platelet transfusions can also exacerbate bleeding. Platelet transfusions should therefore be used only when bleeding is clinically apparent as in our patient. The objectives of treatment of KMS are to prevent bleeding from thrombocytopenia and consumptive coagulopathy and to induce vascular tumor regression., The pathogenesis of KMS remains unestablished. However, platelet trapping by abnormally proliferating blood vessels leading to activation of both the platelets and coagulation cascades, eventually leads to consumption of various clotting factors. Different interventions are recommended including use of steroids, compression, embolization, use of interferon, laser therapy, sclerotherapy, chemotherapy, radiation, or surgery. In each case, the treating physician must decide the suitable treatment to achieve maximum involution of the lesion and preservation of organ function with the least toxicity. The index case did not respond to steroid and propranolol therapy, also clinical condition precluded prescription of vincristine and interferon. Hence, emergency surgical excision was done to prevent life-threatening multi-organ hemorrhage. Surgical excision for cutaneous lesions, splenectomy for multiple lesions in the spleen, wedge resection/hepatectomy for lesions in the liver have been reported as successful treatment modalities for many congenital vascular lesions. In a study conducted by Steven et al. on hemangiomas and vascular malformations of the limb in total 38 children up to 25 (66%) of the cases required surgical intervention, one with KMS also required amputation. Similarly in a series reported by Mac-Moune Lai et al. of total five patients of KHE, one with KMS required emergency amputation. Many other case reports also have reported emergency surgical excision of vascular lesion for KMS. However, in KMS the active ongoing coagulopathy and low platelets counts inadvertently affects the intra-operative course and postoperative recovery of neonate. In index case, KMS was the controlled successfully after surgical excision along with postoperative steroid supplementation. Hence, it can be used as one of the lifesaving treatment modality in patients with refractory KMS.
| Conclusions|| |
Vascular tumors in infancy may present with KMS and require aggressive treatment. Surgery can be offered as a treatment modality for refractory cases of ongoing KMS. Local excision or even the amputation can be a lifesaving intervention in selected cases. Clear guidelines for treatment of vascular tumors with KMS are still lacking and hence physician and surgeon should modify the treatment plan from patient to patient.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Arunachalam P, Kumar VR, Swathi D. Kasabach-Merritt syndrome with large cutaneous vascular tumors. J Indian Assoc Pediatr Surg 2012;17:33-6.
Wang P, Zhou W, Tao L, Zhao N, Chen XW. Clinical analysis of Kasabach-Merritt syndrome in 17 neonates. BMC Pediatr 2014;14:146.
Enjolras O, Mulliken JB, Wassef M, Frieden IJ, Rieu PN, Burrows PE, et al.
Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000;42(2 Pt 1):225-35.
Kim T, Roh MR, Cho S, Chung KY. Kasabach-merritt syndrome arising from tufted angioma successfully treated with systemic corticosteroid. Ann Dermatol 2010;22:426-30.
Phillips WG, Marsden JR. Kasabach-Merritt syndrome exacerbated by platelet transfusion. J R Soc Med 1993;86:231-2.
Acharya S, Pillai K, Francis A, Criton S, Parvathi VK. Kasabach merritt syndrome: Management with interferon. Indian J Dermatol 2010;55:281-3.
Kumar S, Taneja B, Saxena KN, Kalra N. Anaesthetic management of a neonate with Kasabach-Merritt syndrome. Indian J Anaesth 2013;57:292-4.
Hall GW. Kasabach-Merritt syndrome: Pathogenesis and management. Br J Haematol 2001;112:851-62.
Steven M, Kumaran N, Carachi R, Desai A, Bennet G. Haemangiomas and vascular malformations of the limb in children. Pediatr Surg Int 2007;23:565-9.
Mac-Moune Lai F, To KF, Choi PC, Leung PC, Kumta SM, Yuen PP, et al.
Kaposiform hemangioendothelioma: Five patients with cutaneous lesion and long follow-up. Mod Pathol 2001;14:1087-92.
Moura R, Sobreira ML, Bertanha M, Jaldin RG, Madalena SM, Lied P, et al
. Kasabach-Merritt syndrome: Clinical vs. surgical treatment. J Vasc Bras 2014;13:330-5. Available from: http://dx.doi.org/10.1590/1677-5449.0102
. [Last cited on 2015 Aug 02].
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]