A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

Surgical management for mentosternal contractures by flap procedures

Mentosternal contractures still represent a surgical challenge due to their prominent position. They require early operative treatment for both function and aesthetic reasons. Careful clinical examination of scar position and traction forces, both at rest and when functioning, in addition to proper evaluation of the surrounding soft tissue are mandatory for precise preoperative planning of the required reconstruction. In general, the technically most feasible operation is favored if the functional and aesthetic results are good and postoperative risk for recurrence is low. Between 1987 and 1994, 21 patients with cervical, mentosternal, and mentothoracic contractures were operated upon. Eight patients were reconstructed with local flaps and 13 with microvascular free flaps.     

Enchondroma of the hand – clinical evaluation of diagnosis, surgical treatment, and outcome

Summary Enchondroma are benign cartilaginous tumors and are localized most often at the site of the phalanges. Between 1982 and 1993 73 patients with monostotic enchondroma and 5 patients with polyostotic enchondroma were operated at our clinic. Clinical signs of monostotic tumors were pathological fracture (38.4 %), pain or swelling. Eleven percent of cases were accidental findings. Surgical treatment was performed by complete removal of the tumors and filling the bone cavity with autologous spongiosa taken from the pelvic bones, the elbow, or the radius. Three patients (4.1 %) had to be operated a second time due to wound infections and hematoma. In one case Sudeck's dystrophy was diagnosed. One patient (1.4 %) developed a recurrent tumor. Our follow-up examination of 65 patients showed that 77 % of the patients with monostotic enchondroma achieve very good or good functional long-term results after this operation, but only 40 % of the patients with polyostotic enchondroma.      

Genetically modified fibroblasts induce angiogenesis in the rat epigastric island flap

Methods: Gene therapy was tested for inducing functional angiogenesis in the superficial rat epigastric island flap to allow earlier pedicle division. Autologous rat fibroblasts were grown, harvested, cultured and retrovirally transfected to produce platelet-derived growth factor AA (PDGF-AA), an angiogenetically active protein. Stable gene expression was monitored by PDGF-AA enzyme-linked immunosorbent assay (ELISA). One hundred and eighty animals were divided into three groups (I–III) and a bilateral flap created in each animal. In all experiments, the right-sided flap was subjected to experimental treatment and the left-sided flap served as control (1 ml saline 0.9%). During flap elevation, group I received 5×10⁶ GMFB (genetically modified fibroblasts) plus 1 ml Dul-becco's modified Eagle's medium. Group II was treated with 5×10⁶ NMFB (non-modified fibroblasts) plus 1 ml medium and group III received 1 ml medium only. The flaps were sutured back and the vascular pedicle was bilaterally ligated and divided in each of ten animals during the following 6 days. After 7 days, the flaps were harvested, the amount of necrosis measured and histologically examined. Results: The GMFB produced up to 560 times more PDGF-AA than the NMFB, measured by ELISA. The GMFB-treated flaps tolerated surgical division of the vascular pedicle significantly earlier than groups II and III. Histologically, fibroblasts persisted in all flaps of groups I and II, without major inflammatory reaction. In all GMFB-treated flaps, massive angiogenesis could be demonstrated. Conclusion: By means of retroviral gene transfer, autologous rat fibroblasts can be genetically modified for stable expression of the PDGF-A gene to produce high amounts of PDGF-AA, which is angiogenetically active. After injection into the panniculus carnosus, these cells induce functional angiogenesis to permit earlier division of the vascular pedicle in this flap model. Received: 5 January 1998 / Accepted: 17 June 1998