A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report

PURPOSE: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. METHODS: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. RESULTS: There was no evidence of recurrence of tumor for 19 months of follow-up examination. CONCLUSION: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.

Experience of Single Stage Treatment of Caniosynostosis, Hypertelorism, Exophthalmos Patient

The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

Rib/costal Cartilage Combination Graft in Rhinoplasty

Augmenatation rhinoplasty may be effectively accomplished with alloplastic materials. However, certain circumstances mandate the use of autologous grafts(e.g., dorsal augmentation that exceeds 8 mm and patient intolerance of alloplastic implants). Rib or costal cartilage graft have gained wide-spread use in rhinoplasty. Rib cartilages are pliable and soft but they easily undergo warpage, whereas solid bone graft frequently lead to an unnatural hardness of the nasal tip and dorsal resorption. The rib/costal cartilage combination graft(=autogenous chondro-osseous rib grafting) are excellent alternatives. In this study 12 patients received 14 rib grafts harvested in the 7th or 8th rib. Materials were used as cantilevered dorsal onlay grafts, columellar struts, and tip grafts. During 8 months of follow up periods, most patients have firm and stabilized nasal dorsum with soft and mobilized tip without prominent complications such as infection, protrusion, mobilization, etc. In conclusion, this autogenous chondro-osseous rib grafting technique can be an effective and safe method in the primary or secondary rhinoplasty.

Study of Loss of Free Flap and Safer Timing of the Operation in Electrical Injury

An electrical burn used to result in the damage of the skin and underlying deep soft tissue injury. Thus, in order to preserve devitalizing tissues and promote the structural survival free flaps with ample blood supply are frequently employed. However, early unpredictable vascular injury and progressive tissue necrosis may cause the free flaps full of hazards. We applied 50 free flaps upon 41 acute electrical burn cases between 1998 and 2004. Injured areas, timing of operation and causes of flap loss were studied. The victim's ages ranged from 13 to 60 years. (an average 37.8 years) Thirteen out of 50 free flaps were lost totally: three cases were due to arterial insufficiency and ten venous congestion. Total loss of flaps were observed in 5 of 12 cases in the postoperative 3 weeks, 6 of 20 cases between 3 and 6 weeks and 2 of 18 cases after 6 weeks. In three of 12 cases the free flap was lost partially in the postoperative 3 weeks, 4 of 20 cases between 3 and 6 weeks and 1 of 18 cases after 6 weeks. The result was statistically significant by a T-test (p<0.05). This study showed that timing of the operation is accountable for the loss of free flap. It is most important to conduct the free flap procedure on an electrical injury at the time when the recipient vessel is definitely discernible and intact so as to minimize the loss of flap and spare the structures.