Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon.

A 3-y-3-mo old male child presented with massive hypertrophy and bluish-purple discoloration of the left upper limb and adjacent chest wall of 3 mo duration. There was no h/o fever, weight loss, painful large joint swelling, or any bleeding manifestations. He had spindle like nonprogressive, painless swelling of all fingers of the left hand since infancy. The child was moribund with microangiopathic hemolytic anemia, thrombocytopenia, and consumptive coagulopathy without sepsis. He received multiple transfusions of fresh frozen plasma (FFP), platelets, and packed RBC. Paradoxical worsening of symptoms with platelet transfusions and radiological evidences led to the diagnosis of a very rare congenital multifocal vascular tumor, kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP). The index case of KHE was multifocal with cutaneous lesions, osteolytic bony lesions of all phalanx and metacarpals of the left hand, and intrathoracic extension. It was successfully managed with a combination of steroid, vincristine and sirolimus.

Multifocal pure tubercular osteomyelitis: An unusual presentation in childhood.

Tuberculosis is a major health problem in the developing world. One-third of children infected with Mycobacterium tuberculosis have extra pulmonary involvement. Skeletal tuberculosis occurs in 1-6% of them with vertebra being the commonest site. Pure tubercular osteomyelitis without joint involvement occurs in only 2-3% cases of osteoarticular tuberculosis. Common sites are femur, tibia, and fibula. Disseminated skeletal involvement is very rare in children (7%) and calvarial osteomyelitis is even rarer (1%). Here, we report a unique case of disseminated skeletal TB. A 7-year-old tribal girl with no evidence of immunodeficiency presented with multiple lytic lesions involving skull, sternum, and hip bone surprisingly sparing the joints and appendicular skeleton. There was no pulmonary involvement either. FNAC from all three swellings showed presence of acid-fast bacillus. Bone biopsy followed by culture in BACTEC further confirmed the diagnosis. There was complete resolution of the swellings after one year of anti-tubercular drug therapy.