Radiological and clinical features of bone and joint disease in patients with congenital insensitivity to pain and anhidrosis
10.3760/cma.j.issn.1005-1201.2016.03.010
- VernacularTitle:先天性无痛无汗症骨关节病的临床与X线表现特征
- Author:
Ashan PAN
;
Qingdong CHEN
;
Huheng LIN
;
Qiande QIU
- Publication Type:Journal Article
- Keywords:
Hereditary sensory and autonomic neuropathies;
Radiography;
Signs and symptoms
- From:
Chinese Journal of Radiology
2016;50(3):205-208
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the radiological and clinical features of osteoarthrosis in congenital insensitivity to pain with anhidrosis (CIPA).Methods The clinical and radiological features in 8 cases of CIPA diseases were retrospectively analyzed. There were five males and three females,age ranged from 11 to 18 years with a median age of (14±3) years. X-ray plain film findings in eight cases were analyzed for the location, extent and contour of the lesions, surrounding bony sclerosis, as well as joint and soft tissue changes. Results The main clinical presentations were insensitivity to pain, anhydrosis, fever, mental retardation and infection. In these eight cases, a total of 166 pieces of bone were involved, including the plantar and phalanges (n=115),phalanx (n=29).The radiological findings include dissolution of the entire bone or most of the bone (n=113), dissolution of a few small areas of the bone (n=40), dissolution of the bone with only residual punctate areas (n=7), the“pushpin sign”and“pencil tip sign”and“flat cut sign”of the finger osteolysis (n=6), sclerosis of peripheral bone (n=12), pathological fractures (n=2). Joint dislocation (n=3),joint deformity (n=3), and Charcot joint (n=3) were observed.Necrosis of soft tissue(n=4), complete absence of soft tissue in the finger or toe (n=4), dot-like calcification of the soft tissue(n=3) were also observed. Conclusion Osteoarthrosis in patients with CIPA had certain X-ray and clinical characteristic findings, which could facilitate its diagnosis.
