A family with autosomal dominant distal arthrogryposis multiplex congenita and Brown syndrome

Arthrogryposis multiplex congenita is a heterogeneous condition found in a number of different disorders and characterized by congenital joint contractures. We describe typical signs of congenital Brown syndrome (inability to elevate the affected eye actively or passively in full adduction) in three relatives with distal arthrogryposis multiplex congenita. We found a thickening of the superior oblique muscles in these patients with pain and increased intraocular pressure in upgaze. The pathogenesis of clinical and morphological findings is discussed. The association of Brown syndrome with distal arthrogryposis multiplex congenita has not been previously reported and provides us with an important point of reference in the understanding of both syndromes.     

Desmoplastic fibroblastoma of the thigh. A case report

Desmoplastic fibroblastoma (DF) is an extremely rare benign soft tissue tumor, prevalent in adult men, mostly arising in deep regions of extremities. The tumor presents with a slowly growing and no recurrence or metastases after surgical excision. Histologically, DF is characterized by a collagenous stroma that contains spindle- and stellated-shaped fibroblastic cells positive for vimentin. Differential diagnosis with locally aggressive soft tissue tumors could be difficult. This case report deals with the clinical pathological and immunoistochemical features of a DF of the left thigh in a 63-years old man.     

Superficial inferior epigastric artery (SIEA) free flap using perforator vessels as a recipient site: clinical implications in autologous breast reconstruction

BackgroundBreast reconstruction with autogenous tissue is a well-established technique, but there are some limitations related to donor-site morbidity. Among available techniques, the superficial inferior epigastric artery (SIEA) flap is the least invasive procedure because it does not require harvesting or incision of the rectus muscle or the abdominal fascia. Besides adequate flap choice, the proper selection of the recipient vessels is an important factor. Thus, the internal mammary perforator branches (IMPBs) have been an attractive option regarding recipient pedicle morbidity.MethodsThe investigators reported the use of the free SIEA flap with the IMPBs as a recipient site to reconstruct radical mastectomy.ResultsFive patients with invasive ductal carcinoma underwent modified radical mastectomy and reconstruction. The IMPBs were selected as the recipient site and a free SIEA flap was used. The donor defect was closed directly without synthetic mesh. Satisfactory breast shape was achieved, and no complications were observed.ConclusionsFor selected patients, the SIEA flap and IMPBs may constitute a new alternative for immediate breast reconstruction, because of the possibility of large tissue transfer with minimal donor and recipient area morbidity. The SIEA pedicle size and the quality of IMPB vessels should be carefully evaluated.     

Prospective Evaluation of 2,549 Morse Taper Connection Implants: 1‐ to 6‐Year Data

Background: The aim of this study is to evaluate the implant survival, the implant–crown success, and the prosthetic complications of 2,549 Morse taper interference–fit connection implants. Methods: A total of 2,549 Morse taper connection implants were inserted in 893 patients from January 2003 until December 2008. At each annual recall, clinical, radiographic, and prosthetic parameters were assessed. The implant–crown success criteria included the absence of pain, suppuration, and clinical mobility; an average distance between the implant shoulder and the first visible bone contact <2 mm from initial surgery; and the absence of prosthetic complications at the implant–abutment interface. Prosthetic restorations were fixed partial prostheses (462 units); fixed full‐arch prostheses (60 units); single crowns (531 units); and overdentures (93 units). Results: The cumulative implant survival rate was 98.23% (97.25% maxilla, 99.05% mandible). The implant–crown success was 92.49%. A few prosthetic complications at implant–abutment interface were reported (0.37%). After 6 years, distance between the implant shoulder and the first visible bone contact was 1.10 mm (± 0.30 mm). Conclusion: The use of Morse taper connection implants represents a successful procedure for the rehabilitation of partially and completely edentulous arches.