Angiodysplastische destruktive Arthropathie: Klinik, Behandlungsstrategien und Langzeitergebnisse eines seltenen Krankheitsbildes

Introduction: Vascular malformations (VM) represent a challenge for the treating physician in the diagnosis and treatment. If VMs are associated with decreased joint mobility and/or pain, this may be accompanied by destructive joint changes. This entity is called Hauert’s disease or angiodysplastic destructive arthropathy (ADA) and is classified into 3 stages ranging from merely synovial thickening in magnetic resonance imaging (MRI, stage 1), to decreased cartilage area in stage 2 up to bone destruction in stage 3. Methods: The files of patients treated for ADA in our institutions from 1983–2017 were collected in a retrospective study. All patients for whom follow-up examinations (2 months–46 years) were documented were included in this study. The long-term follow-up controls were done by clinical examinations (clinical course 2 months–46 years). The data were anonymized and analyzed according to stage, localization and treatment results. Results: Of the 87 patients treated for ADA during this time period, 50 patients could be included in this study (34 females, 16 males, average age 22 years, range 1–63 years). To evaluate the joint damage, native x‑rays, MRI and arthroscopy were performed. The disease was limited to 1 joint in 48 cases, of which the knee joint was affected most often (knee 41, hip 3, shoulder 2 and ankle 2). In 14 patients stage 1 was present, in 13 stage 2, in 21 stage 3 and in 2 patients both knee and ankle joints were affected. All 50 patients were treated invasively by vascular surgery with respect to the VM (average 4 surgeries, range 1–14). Regarding the ADA diagnostic arthroscopy was performed in 7 cases, arthroscopic debridement in 29 cases and joint replacement was required in 10 cases (all of which were stage 3). In four cases a conservative approach was adopted. All patients showed a postoperative improvement in joint mobility and pain. Conclusion: Angiodysplastic destructive arthritis is a very rare disease. It is not caused by the direct influence of the VM on the joint but describes the coherence of VM and progressive joint damage. An interdisciplinary approach with both invasive orthopedic treatment combined with vascular surgical resection of the malformation is required.