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Pseudarthrosis of the ulna due to neurofibromatosis: a case report
Case reports
239
Pseudarthrosis of the ulna due to neurofibromatosis: a case report K. Rajaratnam, Jai Shanker and Samuel Varughese Department
of Orthopaedics
and Traumatology,
Victoria Hospital, Blackpool
Introduction Pseudarthrosis of long bones associated with neurofibromatosis is a well established complication (Aegerter, 1950; McCarroll, 1950). Though most commonly seen in the tibia (Hunt and Pugh, 1961), over the years the literature has gathered many cases of pseudarthrosis in other long bones (Moore, 1949 Manske, 1979). Nevertheless, reports of isolated ulnar pseudarthrosis still remain a rarity. A survey in the English literature revealed eleven cases. In the case reported here a fracture of the ulna proceeded to non-union and established the diagnosis of neurofibromatosis.
Case report A 6-year-old boy, apparently in good health, was referred to our department after closed fractures of the distal third of the radius and ulna. He had been manipulated in a neighbouring hospital and
a Figure I. Series of radiographs
b showing
0 Butter-worth & Co (Publishers) Ltd 0020-1383/89/040239-02 $03.00
C the development
placed in a long arm plaster. Radiographs after reduction showed a satisfactory position. The osseous architecture appeared normal (Fig. la). By the 6th week the radial fracture was well healed but the ulnar fracture showed signs of delayed union and subsequently progressed to non-union (Fig. lb, c). A systemic examination of the child revealed cafe au lait spots and his mother was found to have multiple cutaneous neurofibromatoses. The ulnar pseudarthrosis was bridged with an autogenous corticocancellous tibia1 bone graft and secured with a semi-tubular plate at 16 weeks. The result was disappointing. The graft became incorporated with the proximal end of the ulna but remained pseudarthrotic distally (Fig. 14. A technetium bone scan at this stage revealed a viable ulnar head. Hence the site was re-explored, the plate and screws removed, the gap bridged with a cancellous bone graft and the limb placed in an above-elbow plaster cast. This also failed to achieve union (Fig. le, f). Tissue from the non-union did not contain any evidence of neurofibromatosis histopathologically.
d of ulnar pseudarthrosis.
e
Injury: the British Journal of Accident Surgery (1989) Vol. 20/No.
240
Discussion Non-union of fractures in children is an unusual complication. If this occurs, consideration should be given to systemic disorders in addition to any local adverse factors. Many instances are recorded in the literature of normallooking fractures resulting in pseudarthroses in children (Cobb, 1968) where cutaneous manifestation of neurofibromatosis was detected later in the patient or in the parents or siblings (Cobb, 1968; Richin et al., 1976). Histopathology of the non-union need not always show evidence of neurofibromatosis (Boyd and Sage, 1958).
Acknowledgements The authors wish to thank Mr K. P. Boardman for kind permission to report on his case. They also thank Mrs Hilary Booth and Miss Arnanda Jeffs for their invaluable help in the preparation of this manuscript.
4
matosis, congenital pseudarthrosis and fibrous dysplasia. 1. Bone joint Surg %A, 618. Boyd H. B. and Sage F. P. (1958) Congenital pseudarthrosis of the tibia. 1. Bone Joint SME. 4oA, 1245. Cobb N. (1968) Neurofibromatosis and pseudarthrosis of the ulna. I BoneJoint Surg. SOB, 146. Hunt J. C. and Pugh D. G. (1961) Skeletal lesions in neurofibromatosis. Radiology, 76, 1. Manske Paul R. (1979) Forearm pseudarthrosis-neurofibromatosis. Ch. orfhop. 139, 125. McCarroll H. Relton (1950) Clinical manifestations of congenital neurofibromatosis. I. Bone Joint Surg. 32A, 601. Moore John R. (1949) Delayed autogenous bone graft in the treatment of congenital pseudarthrosis. 1. Bone joint Swg. 31A, 23. Richin P. F., Kranik A., Van Herpe L. et al. (1976) Congenital pseudarthrosis of both bones of the forearm. 1. Bone Joint Surg. 58A, 1032. Paper accepted
22 March 1989.
Requests for reprints shod
References Aegerter E. E. (1950) The possible relationship of neurofibro-
Brachial artery entrapment dislocation
be addressed to: Mr K. Rajaratnam, Department of Orthopaedics, Freeman Hospital, High Heaton, Newcastle-upon-Tyne NE7 7DN, UK.
in closed elbow
A. D. Wilmshurst, P. A. Millner and A. G. Batchelor St James’s University
Hospital, Leeds, West Yorkshire
Introduction Vascular injury complicating dislocation of the elbow is an unusual but well-recognized event. It rarely occurs, however, unless the dislocation is open or there is an associated fracture (Grimer and Brooks, 1985). Exploration of the vessels characteristically reveals contusion of the artery with an intimal tear and thrombosis, or frank arterial disruption (Louis et al., 1974). We report a case of displacement with obstruction of the intact bra&al artery in a closed elbow dislocation without fracture.
Case report A 6-year-old boy was admitted as an emergency having fallen from a fence onto his right hand. His right elbow showed the typical appearance of a posterior dislocation. Distally, the limb was cool and pale and wrist pulses were absent. There was no sensory or motor deficit in the hand. Radiography showed a posterolateral dislocation of the elbow joint without frac+ure. He was taken without delay to the operating theatre and the dislocation was reduced by closed manipulation under general anaesthesia. Signs of distal ischaemia persisted, however, and a 0 1989 Butterworth & Co (Publishers) Ltd 0020-1383/89/040240-02 $03.00
Doppler probe failed to identify a peripheral pulse in the limb. Radiographs demonstrated widening of the joint space suggestive of soft issue entrapment (Fig. I). Surgical exploration followed, revealing extensive damage to structures in front of the joint. Brachialis muscle, lacertus fibrosus and the anterior joint capsule were all disrupted. The median nerve was intact but contused. The bra&al artery was not immediately apparent. Further exposure proximally and distally revealed the artery stretched behind the medial epicondyle of the humerus and emerging anteriorly through the joint (Fig,2). Red&cation of the
elbow permitted easy retrieval of the artery and replacement in its correct position. Rapid spontaneous recovery of pulsation in the vessel ensued with return of good distal pulses and colour to the hand. Soft tissue repair was effected and the wound drained and closed. Further radiographs confirmed complete reduction. At 8 weeks follow-up the patient was using the limb normally. Extension and flexion each lacked 20” but rotation was full. There were no vascular or neurological
sequelae.
Discussion Reports in the literature of vascular injury in closed elbow dislocation are few, and usually describe complete disruption
of the brachial
artery
(Peabody,
1978; Hofammann
239
Pseudarthrosis of the ulna due to neurofibromatosis: a case report K. Rajaratnam, Jai Shanker and Samuel Varughese Department
of Orthopaedics
and Traumatology,
Victoria Hospital, Blackpool
Introduction Pseudarthrosis of long bones associated with neurofibromatosis is a well established complication (Aegerter, 1950; McCarroll, 1950). Though most commonly seen in the tibia (Hunt and Pugh, 1961), over the years the literature has gathered many cases of pseudarthrosis in other long bones (Moore, 1949 Manske, 1979). Nevertheless, reports of isolated ulnar pseudarthrosis still remain a rarity. A survey in the English literature revealed eleven cases. In the case reported here a fracture of the ulna proceeded to non-union and established the diagnosis of neurofibromatosis.
Case report A 6-year-old boy, apparently in good health, was referred to our department after closed fractures of the distal third of the radius and ulna. He had been manipulated in a neighbouring hospital and
a Figure I. Series of radiographs
b showing
0 Butter-worth & Co (Publishers) Ltd 0020-1383/89/040239-02 $03.00
C the development
placed in a long arm plaster. Radiographs after reduction showed a satisfactory position. The osseous architecture appeared normal (Fig. la). By the 6th week the radial fracture was well healed but the ulnar fracture showed signs of delayed union and subsequently progressed to non-union (Fig. lb, c). A systemic examination of the child revealed cafe au lait spots and his mother was found to have multiple cutaneous neurofibromatoses. The ulnar pseudarthrosis was bridged with an autogenous corticocancellous tibia1 bone graft and secured with a semi-tubular plate at 16 weeks. The result was disappointing. The graft became incorporated with the proximal end of the ulna but remained pseudarthrotic distally (Fig. 14. A technetium bone scan at this stage revealed a viable ulnar head. Hence the site was re-explored, the plate and screws removed, the gap bridged with a cancellous bone graft and the limb placed in an above-elbow plaster cast. This also failed to achieve union (Fig. le, f). Tissue from the non-union did not contain any evidence of neurofibromatosis histopathologically.
d of ulnar pseudarthrosis.
e
Injury: the British Journal of Accident Surgery (1989) Vol. 20/No.
240
Discussion Non-union of fractures in children is an unusual complication. If this occurs, consideration should be given to systemic disorders in addition to any local adverse factors. Many instances are recorded in the literature of normallooking fractures resulting in pseudarthroses in children (Cobb, 1968) where cutaneous manifestation of neurofibromatosis was detected later in the patient or in the parents or siblings (Cobb, 1968; Richin et al., 1976). Histopathology of the non-union need not always show evidence of neurofibromatosis (Boyd and Sage, 1958).
Acknowledgements The authors wish to thank Mr K. P. Boardman for kind permission to report on his case. They also thank Mrs Hilary Booth and Miss Arnanda Jeffs for their invaluable help in the preparation of this manuscript.
4
matosis, congenital pseudarthrosis and fibrous dysplasia. 1. Bone joint Surg %A, 618. Boyd H. B. and Sage F. P. (1958) Congenital pseudarthrosis of the tibia. 1. Bone Joint SME. 4oA, 1245. Cobb N. (1968) Neurofibromatosis and pseudarthrosis of the ulna. I BoneJoint Surg. SOB, 146. Hunt J. C. and Pugh D. G. (1961) Skeletal lesions in neurofibromatosis. Radiology, 76, 1. Manske Paul R. (1979) Forearm pseudarthrosis-neurofibromatosis. Ch. orfhop. 139, 125. McCarroll H. Relton (1950) Clinical manifestations of congenital neurofibromatosis. I. Bone Joint Surg. 32A, 601. Moore John R. (1949) Delayed autogenous bone graft in the treatment of congenital pseudarthrosis. 1. Bone joint Swg. 31A, 23. Richin P. F., Kranik A., Van Herpe L. et al. (1976) Congenital pseudarthrosis of both bones of the forearm. 1. Bone Joint Surg. 58A, 1032. Paper accepted
22 March 1989.
Requests for reprints shod
References Aegerter E. E. (1950) The possible relationship of neurofibro-
Brachial artery entrapment dislocation
be addressed to: Mr K. Rajaratnam, Department of Orthopaedics, Freeman Hospital, High Heaton, Newcastle-upon-Tyne NE7 7DN, UK.
in closed elbow
A. D. Wilmshurst, P. A. Millner and A. G. Batchelor St James’s University
Hospital, Leeds, West Yorkshire
Introduction Vascular injury complicating dislocation of the elbow is an unusual but well-recognized event. It rarely occurs, however, unless the dislocation is open or there is an associated fracture (Grimer and Brooks, 1985). Exploration of the vessels characteristically reveals contusion of the artery with an intimal tear and thrombosis, or frank arterial disruption (Louis et al., 1974). We report a case of displacement with obstruction of the intact bra&al artery in a closed elbow dislocation without fracture.
Case report A 6-year-old boy was admitted as an emergency having fallen from a fence onto his right hand. His right elbow showed the typical appearance of a posterior dislocation. Distally, the limb was cool and pale and wrist pulses were absent. There was no sensory or motor deficit in the hand. Radiography showed a posterolateral dislocation of the elbow joint without frac+ure. He was taken without delay to the operating theatre and the dislocation was reduced by closed manipulation under general anaesthesia. Signs of distal ischaemia persisted, however, and a 0 1989 Butterworth & Co (Publishers) Ltd 0020-1383/89/040240-02 $03.00
Doppler probe failed to identify a peripheral pulse in the limb. Radiographs demonstrated widening of the joint space suggestive of soft issue entrapment (Fig. I). Surgical exploration followed, revealing extensive damage to structures in front of the joint. Brachialis muscle, lacertus fibrosus and the anterior joint capsule were all disrupted. The median nerve was intact but contused. The bra&al artery was not immediately apparent. Further exposure proximally and distally revealed the artery stretched behind the medial epicondyle of the humerus and emerging anteriorly through the joint (Fig,2). Red&cation of the
elbow permitted easy retrieval of the artery and replacement in its correct position. Rapid spontaneous recovery of pulsation in the vessel ensued with return of good distal pulses and colour to the hand. Soft tissue repair was effected and the wound drained and closed. Further radiographs confirmed complete reduction. At 8 weeks follow-up the patient was using the limb normally. Extension and flexion each lacked 20” but rotation was full. There were no vascular or neurological
sequelae.
Discussion Reports in the literature of vascular injury in closed elbow dislocation are few, and usually describe complete disruption
of the brachial
artery
(Peabody,
1978; Hofammann