ClOVes syndrome is a rare overgrowth disorder characterized by congenital lipomatous overgrowth, vascular malformation, epidermal nevi and spinal abnormalities. Clinical aspects described by Alomari Al and sapp JC [1,2] where mostly in patients over one-year-old, whereby, the aim of our report is to describe three fatal cases of newborns with ClOVes syndrome and their clinical features.The case report is on three patients who were pre-natally diagnosed with masses in the body. All of them were born of unremarkable gestations in which there were no maternal illnesses or teratogenic agents reported. The maternal ages ranged between 23 and 35 years, and all patients were Caucasian. CaSe SerieSCase-1 A 4,400 gm girl at birth presented with a giant mass on the right side of her body that extended from the axillary region to half of the trunk and back and was associated with an overgrowth of the lower extremity and absence of toes . The skin showed several vascular stains on the thigh and flank. Magnetic Resonance Imaging (MRI) showed a combined and extensive vascular malformation with adipose tissue around it, especially in the perineum. The retroperitoneum and mesentery were also involved. epidermal nevi and scoliosis were not present. The patient had elevated D-dimer and fibrinogen levels. The vascular malformation had two episodes of sudden growth due to internal bleeding observed on ultrasound, requiring several blood transfusions (7 units of red blood cells and 14 units of frozen plasma). liver, kidney and thyroid function tests were normal. The patient had progressive respiratory distress, requiring mechanical ventilation. The child died from severe systemic Inflammatory Response syndrome (sIRs) distress at 54 days of life. Case-2A full-term, 3,808 gm boy presented with overgrowth of the left lower limb and an abnormal foot with no toes (only small buds). On his right lower extremity, he presented with macrodactyly and an increased first web space . extensive vascular stains were identified on the left lower extremity and flanks, scrotum and right toes. Angio CT imaging showed extensive retro-peritoneal and subcutaneous lymphatic malformation compromising the abdominal wall, perineum and inguinal region with increased adipose tissue and a giant venous malformation in the thighs and legs [Table/ Fig-5]. There were no epidermal nevi and scoliosis present. The coagulation analysis and D-dimer level were normal. At 26 days of age, the infant presented with poor general condition, food refusal, fever, enlargement of both lower extremities, and erythema of the left groin. He required advanced resuscitation and mechanical ventilation due to septic shock of cutaneous origin, with spontaneous drainage of purulent fluid from the lower extremity. The shock was refractory to treatment, and the infant died at 29 days of life. The pathology analysis reported gas gangrene on the left leg and foot inside hamartomatous vascular tissue. Radiology Section Case-3This patient was a full-term, 4,415 gm Caucasian girl in whom multip...
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