RESUMO

Proximal interphalangeal (PIP) joint contracture is a common, difficult clinical problem that can arise from minor trauma. Management is difficult because outcomes are unpredictable and often poor, due to residual flexion deformities postoperatively. The dorsal approach for flexion contracture of the PIP joint is not discussed in present literature. In this technique guide, we wish to describe and explain the rationale for a dorsal approach. In our experience, a dorsal approach allows for ease of access to all pathologic structures, with simple positioning of the digit to allow access to volar structures, as well as when addressing more than one digits with a PIP contracture. Finally, similar to the midaxial approach, the dorsal approach also eliminates any volar soft tissue concerns and need for supplemental coverage.

RESUMO

Abstract Background: Chronic graft versus host disease (cGVHD) simulating eosinophilic fasciitis (EF) is an underdiagnosed and challenging complication due to the lack of knowledge about its pathogenesis, refractoriness to traditional immunosuppressive agents and their negative impact on the physical function and quality of life. The aim of this study is to describe the clinical-biological characteristics and response to treatment of a case series and to provide a comprehensive literature review on cGVHD related EF involvement. Methods: Prospective observational study to describe the clinical and diagnostic evaluation characteristics of patients with EF-like follow-up as part of our multidisciplinary cGVHD consultations. In addition, the literature on joint and/or fascial musculoskeletal manifestations due to cGVHD was comprehensively reviewed. Results: 118 patients were evaluated in multidisciplinary cGVHD consultations, 39 of whom (33%) developed fasciitis. Notably, 11 patients had isolated joint contractures without sclerotic skin. After a median of three lines of treatment, the vast majority of patients achieved some degree of response. 94 potentially eligible articles were identified by the search strategy, with 17 of them, the majority isolated case reports, making the final selection. The validated staging scales used for the assessment were the Joint and Fascial Score and the Photographic Range of Motion. Conclusion: Fascial/articular involvement needs to be recognized and evaluated early. To our knowledge, our cohort is the second largest series to have been reported. Literature addressing fascial/joints complications related to cGVHD is scarce. The search for new biomarkers, the use of advanced imaging techniques and multidisciplinary approach may help improve the prognosis of patients with cGVHD.

Assuntos

RESUMO

El síndrome de fibromatosis hialina es una enfermedad autosómica recesiva rara, que se caracteriza por la presencia de contractura y dolor articular, placas y nódulos hiperpigmentados e hipertrofia gingival, producto de la acumulación de un material amorfo hialino similar al colágeno tipo VI en diferentes tejidos. Esta enfermedad incluye el síndrome de hialinosis sistémica y la fibromatosis hialina juvenil, dos entidades que, durante años, fueron consideradas de manera separada; sin embargo, las características clínicas y la edad de presentación se superponen. Además, ha sido documentado que la causa de ambas entidades se localiza en un mismo gen. Se presentan dos casos de hermanas de una misma familia colombiana afectadas por la enfermedad.

Hyaline fibromatosis syndrome is a rare autosomal recessive disease characterized by the presence of contracture and joint pain, hyperpigmented plaques and nodules and gingival hypertrophy. These findings are the result of the accumulation of a hyaline amorphous material similar to collagen type VI in different tissues. This syndrome includes systemic hyalinosis and juvenile hyaline fibromatosis, two entities that for years were considered separately. However, it has been documented that the cause of both entities is located in the same gene and the clinical features and age of presentation are overlapped. In this study two cases of sisters from a same colombian family affected by the disease are presented.

Assuntos

RESUMO

El síndrome de fibromatosis hialina es una enfermedad autosómica recesiva rara, que se caracteriza por la presencia de contractura y dolor articular, placas y nódulos hiperpigmentados e hipertrofia gingival, producto de la acumulación de un material amorfo hialino similar al colágeno tipo VI en diferentes tejidos. Esta enfermedad incluye el síndrome de hialinosis sistémica y la fibromatosis hialina juvenil, dos entidades que, durante años, fueron consideradas de manera separada; sin embargo, las características clínicas y la edad de presentación se superponen. Además, ha sido documentado que la causa de ambas entidades se localiza en un mismo gen. Se presentan dos casos de hermanas de una misma familia colombiana afectadas por la enfermedad.(AU)

Hyaline fibromatosis syndrome is a rare autosomal recessive disease characterized by the presence of contracture and joint pain, hyperpigmented plaques and nodules and gingival hypertrophy. These findings are the result of the accumulation of a hyaline amorphous material similar to collagen type VI in different tissues. This syndrome includes systemic hyalinosis and juvenile hyaline fibromatosis, two entities that for years were considered separately. However, it has been documented that the cause of both entities is located in the same gene and the clinical features and age of presentation are overlapped. In this study two cases of sisters from a same colombian family affected by the disease are presented.(AU)

RESUMO

A long metatarsal and/or metatarsophalangeal joint dislocation associated with a digital contracture is a surgical challenge. Without appropriate surgical correction, the patient will be predisposed to numerous complications, including persistent subluxation or dislocation, recurrent metatarsalgia, dorsiflexory contracture of the digit, transfer lesions, and inadequate pain relief. The results of the present surgical treatment options have varied, with the most common complication being a floating toe. The purpose of our study was to introduce a decompression, shortening, lesser metatarsal osteotomy with a modified fixation technique using a T-plate and to report our results. Additionally, we have discussed trigonometric analysis of metatarsal declination and shortening. We retrospectively reviewed the outcomes of 30 consecutive patients with 33 osteotomies who had been treated surgically for pathologic features associated with a long metatarsal and varying biomechanical abnormalities. Before surgery, all the patients had been treated conservatively for a minimum of 3 months. The surgical procedure included a dorsal to plantar V-shaped shortening osteotomy of a lesser metatarsal that was fixated with a T plate. The patients were assessed radiographically and using the American Orthopaedic Foot and Ankle Society Lesser Metatarsophalangeal-Interphalangeal Scale and visual analog scale. The mean age at surgery was 53 (range 37 to 75) years, with a mean follow-up period of 9.1 (range 6 to 15.4) months. The average shortening of the metatarsal was 2.7 mm. One patient (3%) had had asymptomatic delayed union and 2 patients (6%) hypertrophic nonunion. No incidence of malunion or avascular necrosis was identified. Five cases (15.2%) of hardware failure occurred. The mean American Orthopaedic Foot and Ankle Society score was 76.7 postoperatively. The visual analog scale score had improved from 6.7 to 1.7. Of the 30 patients, 72% rated the overall surgical experience as excellent or good. In conclusion, the modified fixation technique for decompression, shortening metatarsal osteotomy using a T plate is a viable option when choosing a procedure to address a long, prominent metatarsal and/or digital contracture at the metatarsophalangeal joint and results in a low incidence of floating toe complications.

Assuntos