February 26, 2014
11:12:44am
WSPC/135-HS
FA1
1472007
Hand Surgery, Vol. 19, No. 1 (2014) 117–118 © World Scientific Publishing Company DOI: 10.1142/S0218810414720071
OSTEOLYSIS WITH SECONDARY ARTHRITIS OF THE SCAPHOTRAPEZIOTRAPEZOID JOINT IN HAJDU-CHENEY SYNDROME: A CASE REPORT Olivia Flannery and Karen Smith
Hand Surg. 2014.19:117-118. Downloaded from www.worldscientific.com by FLINDERS UNIVERSITY LIBRARY on 02/04/15. For personal use only.
Department of Plastics and Reconstructive Surgery Middlemore Hospital, Auckland, New Zealand Received 7 April 2013; Revised 23 July 2013; Accepted 23 July 2013 ABSTRACT We describe a case of Hajdu-Cheney syndrome affecting the scaphotrapeziotrapezoid joint presently being treated non-operatively. This syndrome poses the problem of non-union when surgical intervention is required. Keywords: Hajdu-Cheney; Syndrome; Acro-osteolysis.
INTRODUCTION
localized tenderness over the scaphotrapeziotrapezoid (STT) joint. She demonstrated a full range of movement of her wrist and thumb but pain at the extremes of movement. Plain radiographs revealed typical osteolysis of the distal phalanges and atypical osteolysis involving the distal pole of the scaphoid
Hajdu-Cheney syndrome is a rare autosomal dominant skeletal disorder, which is characterized by acro-osteolysis and osteoporosis. Any joint of the hand or wrist can be affected but typically there is bony resorption of the distal phalanges and narrowing of the metacarpophalangeal joint space. We present a case of osteolysis involving the scaphotrapeziotrapezoid joint with secondary osteoarthritic changes.
CASE REPORT A 22-year-old left hand dominant student, with a known diagnosis of Hajdu-Cheney syndrome, was referred to our hand clinic with a three-year history of worsening left-sided wrist pain. She complained of pain during her daily and sporting activities and had to modify her activities as a result. She had previously been treated with bisphosphonates but these have been discontinued. On examination she had the characteristic stubby appearance of her fingers and thumb (Fig. 1). She had
Fig. 1 Clinical photograph demonstrating the typical short fingers seen in Hajdu-Cheney syndrome.
Correspondence to: Dr. Olivia Flannery, 89 Hapua Street, Remuera, Auckland 1050, New Zealand. E-mail: omfl[email protected] 117
February 26, 2014
118
11:12:50am
WSPC/135-HS
O. Flannery & K. Smith
Fig. 2 Plain radiographs of the left writ and hand showing osteolysis of the distal phalanges and distal pole of scaphoid with widening of the STT joint. Hand Surg. 2014.19:117-118. Downloaded from www.worldscientific.com by FLINDERS UNIVERSITY LIBRARY on 02/04/15. For personal use only.
FA1
1472007
Fig. 3 CT images of the STT joint showed cortical irregularity of components with some erosive changes and small marginal osteophyte.
with widening of the STT joint (Fig. 2). A CT scan of the STT joint showed cortical irregularity, with some erosive changes and small marginal osteophytes (Fig. 3). To date her management has included splintage and analgesia. Her pain has decreased following modification her activities and thus she no longer requires any form of treatment.
DISCUSSION Hajdu-Cheney syndrome, which was first described by Hajdu and Kaunrze in 19481 and Cheney in 1965,2 is characterized by progressive focal bony destruction and generalized osteoporosis, and can have various musculoskeletal manifestations. There are approximately 50 cases reported in the literature, with the
majority describing osteolysis of the distal phalanges which result in the appearance of short stubby fingers.3 However, to our best knowledge there are no reports in the English literature on osteolysis of the scaphotrapeziotrapezoid joint and resultant osteoarthritis. The management plan will be similar to any STT arthritis, which includes modification of aggravating activities, splintage and analgesia. If non-operative management fails then a STT fusion would be indicated. However, fusion techniques in patients with Hajdu-Cheney syndrome need to be more aggressive in order to avoid fusion failure. Two cases are reported in the literature where spinal fusion failed in patients with Hajdu-Cheney syndrome. Herscovici et al.4 report on a seven year-old girl who required spinal fusion with bone graft from C1-C3, which was revised a year later due to resorption of the bone graft. Muthagh-Schaffer and Moquin5 report on a 42year-old who sustained fractures of C5-6 and C7-T1 with a kyphotic deformity of the C-spine. He underwent reconstruction and stabilization, supplemented with patella allograft and demineralized bone matrix. The fusion failed and he required revision surgery, where BMP with autograft/allograft, crushed cancellous bone mixed with demineralized bone matrix was used, as well as a bone stimulator post-operatively. Patients with Hajdu-Cheney syndrome requiring fusion need to be consented of the high possibility of fusion failure and the necessity for further surgeries. The combination of autograft, synthetic bone graft and bone stimulators are most likely required.
References 1. Hajdu N, Kauntze R, Cranio-skeletal dysplasia, Br Radiol 21:42, 1948. 2. Cheney WD, Acro-osteolysis, Am J Roentgenol 94:595–607, 1965. 3. O’Reilly MA, Shaw DG, Hajdu-Cheney syndrome. Ann Rheum Dis 53:276–279, 1994. 4. Herscovici D Jr, Bowen JR, Scott CI Jr, Cervical instability as an unusual manifestation of Hajdu-Cheney syndrome of acroosteolysis, Clin Orthop Relat Res 255:111–116, 1990. 5. Murtagh-Schaffer C, Moquin RR, Spinal reconstruction in Hajdu-Cheney syndrome, JAAPA 21:29, 31–33, 2008.
11:12:44am
WSPC/135-HS
FA1
1472007
Hand Surgery, Vol. 19, No. 1 (2014) 117–118 © World Scientific Publishing Company DOI: 10.1142/S0218810414720071
OSTEOLYSIS WITH SECONDARY ARTHRITIS OF THE SCAPHOTRAPEZIOTRAPEZOID JOINT IN HAJDU-CHENEY SYNDROME: A CASE REPORT Olivia Flannery and Karen Smith
Hand Surg. 2014.19:117-118. Downloaded from www.worldscientific.com by FLINDERS UNIVERSITY LIBRARY on 02/04/15. For personal use only.
Department of Plastics and Reconstructive Surgery Middlemore Hospital, Auckland, New Zealand Received 7 April 2013; Revised 23 July 2013; Accepted 23 July 2013 ABSTRACT We describe a case of Hajdu-Cheney syndrome affecting the scaphotrapeziotrapezoid joint presently being treated non-operatively. This syndrome poses the problem of non-union when surgical intervention is required. Keywords: Hajdu-Cheney; Syndrome; Acro-osteolysis.
INTRODUCTION
localized tenderness over the scaphotrapeziotrapezoid (STT) joint. She demonstrated a full range of movement of her wrist and thumb but pain at the extremes of movement. Plain radiographs revealed typical osteolysis of the distal phalanges and atypical osteolysis involving the distal pole of the scaphoid
Hajdu-Cheney syndrome is a rare autosomal dominant skeletal disorder, which is characterized by acro-osteolysis and osteoporosis. Any joint of the hand or wrist can be affected but typically there is bony resorption of the distal phalanges and narrowing of the metacarpophalangeal joint space. We present a case of osteolysis involving the scaphotrapeziotrapezoid joint with secondary osteoarthritic changes.
CASE REPORT A 22-year-old left hand dominant student, with a known diagnosis of Hajdu-Cheney syndrome, was referred to our hand clinic with a three-year history of worsening left-sided wrist pain. She complained of pain during her daily and sporting activities and had to modify her activities as a result. She had previously been treated with bisphosphonates but these have been discontinued. On examination she had the characteristic stubby appearance of her fingers and thumb (Fig. 1). She had
Fig. 1 Clinical photograph demonstrating the typical short fingers seen in Hajdu-Cheney syndrome.
Correspondence to: Dr. Olivia Flannery, 89 Hapua Street, Remuera, Auckland 1050, New Zealand. E-mail: omfl[email protected] 117
February 26, 2014
118
11:12:50am
WSPC/135-HS
O. Flannery & K. Smith
Fig. 2 Plain radiographs of the left writ and hand showing osteolysis of the distal phalanges and distal pole of scaphoid with widening of the STT joint. Hand Surg. 2014.19:117-118. Downloaded from www.worldscientific.com by FLINDERS UNIVERSITY LIBRARY on 02/04/15. For personal use only.
FA1
1472007
Fig. 3 CT images of the STT joint showed cortical irregularity of components with some erosive changes and small marginal osteophyte.
with widening of the STT joint (Fig. 2). A CT scan of the STT joint showed cortical irregularity, with some erosive changes and small marginal osteophytes (Fig. 3). To date her management has included splintage and analgesia. Her pain has decreased following modification her activities and thus she no longer requires any form of treatment.
DISCUSSION Hajdu-Cheney syndrome, which was first described by Hajdu and Kaunrze in 19481 and Cheney in 1965,2 is characterized by progressive focal bony destruction and generalized osteoporosis, and can have various musculoskeletal manifestations. There are approximately 50 cases reported in the literature, with the
majority describing osteolysis of the distal phalanges which result in the appearance of short stubby fingers.3 However, to our best knowledge there are no reports in the English literature on osteolysis of the scaphotrapeziotrapezoid joint and resultant osteoarthritis. The management plan will be similar to any STT arthritis, which includes modification of aggravating activities, splintage and analgesia. If non-operative management fails then a STT fusion would be indicated. However, fusion techniques in patients with Hajdu-Cheney syndrome need to be more aggressive in order to avoid fusion failure. Two cases are reported in the literature where spinal fusion failed in patients with Hajdu-Cheney syndrome. Herscovici et al.4 report on a seven year-old girl who required spinal fusion with bone graft from C1-C3, which was revised a year later due to resorption of the bone graft. Muthagh-Schaffer and Moquin5 report on a 42year-old who sustained fractures of C5-6 and C7-T1 with a kyphotic deformity of the C-spine. He underwent reconstruction and stabilization, supplemented with patella allograft and demineralized bone matrix. The fusion failed and he required revision surgery, where BMP with autograft/allograft, crushed cancellous bone mixed with demineralized bone matrix was used, as well as a bone stimulator post-operatively. Patients with Hajdu-Cheney syndrome requiring fusion need to be consented of the high possibility of fusion failure and the necessity for further surgeries. The combination of autograft, synthetic bone graft and bone stimulators are most likely required.
References 1. Hajdu N, Kauntze R, Cranio-skeletal dysplasia, Br Radiol 21:42, 1948. 2. Cheney WD, Acro-osteolysis, Am J Roentgenol 94:595–607, 1965. 3. O’Reilly MA, Shaw DG, Hajdu-Cheney syndrome. Ann Rheum Dis 53:276–279, 1994. 4. Herscovici D Jr, Bowen JR, Scott CI Jr, Cervical instability as an unusual manifestation of Hajdu-Cheney syndrome of acroosteolysis, Clin Orthop Relat Res 255:111–116, 1990. 5. Murtagh-Schaffer C, Moquin RR, Spinal reconstruction in Hajdu-Cheney syndrome, JAAPA 21:29, 31–33, 2008.
