Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review

Kaposiform hemangioendothelioma (KHE) is a very rare, locally aggressive vascular neoplasm. It occurs mostly in children and is rarely observed in adults. It typically originates on the skin, later affecting the deep soft tissue of the extremities, head or neck, and retroperitoneum by infiltrative growth. It is locally aggressive, does not regress spontaneously, and tends to metastasize locally as well as to the regional lymph nodes. In this article, we report a case of adult-onset KHE with neurofibromatosis type 1. The patient presented to our department with a 2-month history of a painful ulceration in her left popliteal area. Since KHE had not previously been reported in patients with neurofibromatosis, the diagnosis was difficult due to the similarity of the skin manifestation to neurofibromatosis-associated lesions. We share our experience of diagnosing and treating this rare case of adult-onset KHE.

Bilateral Fasciocutaneous Sliding V-Y Advancement Flap for Meningomyelocele Defect

PURPOSE: Meningomyelocele is the most common type of neural tube defect disease. Early surgical treatment is recommended to prevent central nervous system infection. Several reconstruction methods were reported previously regarding surgical wound defect closure following meningomyelocele excision. In this article, we report two successful patients using the bilateral fasciocutaneous sliding V-Y advancement flap as a covering for meningomyelocele defects. METHODS: Two patients with meningomyelocele were evaluated. Both patients were male and received their operations on the 1st and 4th day of life. After neurosurgeons completed their part of the operation, the V-Y advancement flap was used to close the defect. Initially a bilateral V-shape incision design was made on the skin such that the base of the V-flap measures identical to the size of the wound defect. An incision was made down to the fascia in order to allow the V-flaps to slide into the defect. Subfascial dissection was performed up to 1/3 to 1/4 the length of the V-flap from the wound while minimizing injury to the perforating vessels. RESULTS: Both patients were treated successfully and there was no evidence of complication in 2 months follow up. CONCLUSION: Several reconstruction methods such as local flaps, skin graft and myocutaneous flaps were reported regarding meningomyelocele surgical wound defect closure. Bilateral fasciocutaneous sliding V-Y advancement flap is an easy method without involving the underlying muscles or a secondary skin graft in a short operation time. Therefore we recommend this treatment option for reconstruction of the wound defect following meningomyelocele excision.