Vascular tumors may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe cases, there may be disseminated intravascular coagulation. Other vascular tumors may be responsible, such as infantile hemangioma. The radiographic features are specific to each vascular tumor. They can be found in multiple locations, more frequently subcutaneous, musculoskeletal, splenic and hepatic in location.
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Kasabach-Merritt syndrome is characterised by the combination of rapidly growing vascular tumour, thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy.
The blood clotting disorder results from platelets and other clotting factors of the blood being used up within the tumor. We report a two- and- half month old Saudi female infant who presented with epistaxis, conjunctival haemorrage and bilateral periorbital ecchymosis. The association of hemangioma, thrombocytopenia, and hypofibrinogenemia was first described in by Kasabach and Merritt [ 1 ], who took care of an infant with a giant capillary hemangioma and thrombocytopenic purpura. Kasabach-Merritt syndrome KMS is a rare disorder that can affect infants from the time of birth, or may appear later in infancy as the vascular malformation grows.
Diagnosis of KMS is made based on the constellation of a vascular lesion, thrombocytopenia, consumptive coagulopathy, and microangiopathic hemolytic anemia. Unlike true capillary hemangiomas that regress in childhood and are a cosmetic nuisance, the lesions in KMS are distinctive vascular tumors that include tufted angiomas and kaposiform hemangioendotheliomas [ 2 ].
The pathophysiology is believed to be consumption of platelets and fibrinogen by intralesional thrombosis [ 3 ]. The lesions are typically superficial and solitary, but may involve internal structures such as the liver. Cardiac failure may result from high-volume arteriovenous shunting. A two- and-half month old Saudi female infant was admitted to Al —Taif Children Hospital, Kingdom of Saudi Arabia, with history of nasal blockage, mild respiratory distress and noisy breathing for 20 days, associated with bilateral periorbital ecchymosis and redness of both eyes for 15 days Figure 1 A.
There was no history of fever, cough, vomiting, trauma or foreign body inhalation or ingestion. She was delivered at term following normal spontaneous vaginal delivery to non consanguineous parents. No history of neonatal problems. No past or family history of significance.
A Extensive bilateral periorbital ecchymosis B Apparent bilateral subconjunctival haemorrhage. On routine physical examination, she was ill looking, pale, not cyanosed or jaundiced. She was keeping her neck in hyperextension posture, afebrile with mild respiratory distress and well hydrated. Extensive bilateral periorbital ecchymosis was documented with bilateral subconjunctival haemorrhage with no discharge Figure 1 B. She did not have purpuric rash, bruises or ecchymosis on her skin or mucus membranes.
Her vital signs and oxygen saturation were normal. Examination of the chest, cardiovascular, abdomen and central nervous systems swere normal. Soon after admission she developed epistaxis from both nostrils. Investigations revealed Hb She had normal chemistry, prothrombin time C — reactive protein was negative. Blood gas result was normal and there was no bacterial growth from blood and urine cultures.
Ultrasound and computed tomography CT of brain and abdomen were normal. X- ray neck showed enlarged adenoid glands. While the patient was in hospital she was seen again by the ENT surgeon who assured the parents. She was also seen by the ophthalmologist who confirmed presence of bilateral perioccular and subconjunctival hemorrhage with normal anterior chamber, pupils, with clear lens and normal fundi.
There were no vitreous or retinal hemorrhages, and on reevaluation after two days the presence of bilateral retinal hemorrhage was documented. The patient was stable while she was in hospital with no active bleeding from any site although her daily CBC showed decrease in Hb to 9.
Bone marrow, following aspiration, was normocellular with only megakaryocytic hyperplasia which suggested Idiopathic thrombocytopenic purpura. She was referred to a higher specialized centre for further evaluation by the hematologist who agreed with the plan of management.
However, while the patient was there, she developed severe respiratory distress with inspiratory stridor and desaturation. An urgent direct laryngoscopy examination showed a moderate to large sized haemangioma at the larynx. A tracheostomy tube was inserted immediately to maintain an adequate airway and the diagnosis of Kasabach-Merritt syndrome was made and was referred to the ENT surgeon for further investigation and treatment of the hemangioma. The syndrome results in a consumptive coagulopathy [ 4 , 5 ] from platelet trapping and aggregation within a specific type of hemangioma, and can have a high mortality rate.
The hemangioma is often within the skin but can be present anywhere, including retroperitoneal organs, the mediastinum, the pelvis, visceral organs, or the mesentery. Kaposiform hemangioendotheliomas are typically solitary tumours which appear in the soft tissues of the limbs, head and neck or retroperitoneum. They are usually seen in infants less than 2 years of age, although cases have been reported in adults.
They do not spread metastasize but can cause serious problems because of local growth, cardiac failure or the associated Kasabach-Merritt phenomenon. There are few reports of kaposiform hemangioendotheliomas without Kasabach-Merritt syndrome. Kaposiform haemangioendotheliomas usually regress with time but do not completely disappear.
Tufted angiomas usually present before 5 years of age, although they can occur throughout life. They present as brown, red or purple areas of skin and are firm to touch. They are often painful. Spontaneous regression is unusual. Most tufted angiomas do not cause Kasabach-Merritt syndrome and metastasis is rare. Treatment aims to involute the tumour to prevent significant morbidity or mortality, or in response to a life-threatening event.
Surgical excision is curative but most lesions are not amenable to this option. Historically, the first-line of treatment has been high-dose systemic corticosteroids. However, up to two-thirds of lesions will not respond to corticosteroids, or will quickly relapse once treatment is discontinued [ 6 ].
Also, this treatment is not without its own troubling adverse effects. The most promising recent option available for treatment of infantile hemangiomas is propranolol [ 8 ]. When evaluating a patient with these types of malformations, one must also consider syndromes associated with vascular malformations, such as Klippel-Trenaunay-Weber syndrome and Sturge-Weber syndrome.
National Center for Biotechnology Information , U. Journal List Sudan J Paediatr v. Sudan J Paediatr. Nader M Osman. Author information Copyright and License information Disclaimer. Omdurman, Sudan. Corresponding author. Abstract Kasabach-Merritt syndrome is characterised by the combination of rapidly growing vascular tumour, thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy.
Introduction The association of hemangioma, thrombocytopenia, and hypofibrinogenemia was first described in by Kasabach and Merritt [ 1 ], who took care of an infant with a giant capillary hemangioma and thrombocytopenic purpura.
Case Report A two- and-half month old Saudi female infant was admitted to Al —Taif Children Hospital, Kingdom of Saudi Arabia, with history of nasal blockage, mild respiratory distress and noisy breathing for 20 days, associated with bilateral periorbital ecchymosis and redness of both eyes for 15 days Figure 1 A.
Open in a separate window. Figure 1. References 1. Capillary hemangioma with extensive purpura: report of a case. American Journal of Diseases of Children. Kasabach-Merritt syndrome: therapeutic considerations. Pediatrics ; 79 : — Hall GW. Kasabach-Merritt syndrome: Pathogenesis and management.
Clinical analysis of kasabach-merritt syndrome in 17 neonates
Metrics details. Kasabach-Merritt syndrome KMS is characterized by giant hemangiomas and severe thrombocytopenia, which may result in life-threatening multi-organ hemorrhage. This study evaluated the clinical characteristics, treatments, and outcomes in neonates with KMS, in order to find out the optimal therapy. Four patients had visceral hemangiomas and 13 had cutaneous hemangiomas.
DermNet provides Google Translate, a free machine translation service. Note that this may not provide an exact translation in all languages. The blood clotting disorder results from platelets and other clotting factors from the blood being used up consumed within the tumour. Kasabach-Merritt syndrome is caused by kaposiform haemangioendotheliomas, tufted angiomas and sometimes other vascular tumours. Previously Kasabach-Merritt syndrome was thought to be caused by large infantile haemangiomas also known as cavernous haemangiomas or capillary haemangiomas however this is not the case.