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Congenital pseudarthrosis of the ulna treated by free vascularized fibular graft: A case report and review of methods of treatment
CONGENITAL PSEUDARTHROSIS OF TH FREE VASCULARIZED FIBULAR GRA AND REVIEW OF METHODS OF TREAT E. MASTERSON,
M. J. EARLEY
From the Children s Hospital,
and M. M. STEPHENS
Temple Street, Dublin, Eire
Congenital pseudarthrosis of the ulna is an extremely rare condition for which current surgical techniques have been unsatisfactory in restoring a normal two-bone forearm. We report a case which was treated by excision of the ulnar pseudarthrosis and interposition of a free ~asc~~ari~ed fibular graft with a skin island to monitor anastomotic patency. Forearm rotation has been restored and the potential for normal forearm growth has been preserved. Journal of Hand Surgery (British and European Volume, 1993) 18B: 285-288 strated complete resorption of the distal ulna (Fig 1). There was full flexion and extension of the wrist and elbow but pronation of the forearm caused subluxation of the radial head. Although the child’s mother had neurofibromatosis, she herself had no stigmata of the disease.
Congenital pseudarthrosis of the ulna in the presence of a normal radius is rare. Only 15 cases have been described in the English literature (Moore, 1949; Madsen, 19.56; Shertzer et al, 1969; Allieu et al, 1981; Ali and Hooper, 1982; Bayne, 1985; Ostrowski et al, 1985; Fabry et al, 1988; Bell, 1989). All but three cases were associated with neurofibromatosis. Untreated, this condition leads to progressive forearm deformity due to continued radial growth in the presence of an ulnar tether. The end result is a short, bowed forearm with a dislocated radial head. The multiplicity of treatment methods which have been described for this condition is a reflection of the difficulty in obtaining bony union. These have included cast immobilization, non-vascularized bone grafting with and without internal fixation, creation of a one-bone forearm, free vascularized fibular grafting and the Ilizarov compression-distraction technique. In the single case in which a free vascularized fibular graft was used, the distal end of the graft was keyed into the distal radius with a pin (Allieu et al, 1981). This must have severely impaired forearm rotation by effectively creating a one bone forearm. Furthermore, as no stabilization of the distal radio-ulnar joint was performed, wrist instability may have subsequently developed with future growth. We report a case in which a functioning two-bone forearm with the potential for normal growth has been preserved by excision of the ulnar pseudarthrosis and interposition of a free vascularized fibular graft with preservation of the distal ulnar epiphysis. CASE REPORT A l-year-old female infant injured her right arm in July 1987. Radiographs taken in the local casualty department confirmed a fracture of the distal ulnar shaft. The arm was treated in a cast for 4 weeks after which a radiograph was deemed satisfactory. In retrospect, this radiograph displayed typical features of an early pseudarthrosis. The child was discharged from further follow-up. In May 1990, the child reattended with a short, bowed forearm and a painful elbow. A radiograph demon-
Fig 1
285
Plain radiograph showing arthrosis of the right ulna.
established
congenital
pseud-
THE JOURNAL OF HAND SURGERY VOL. 18B No. 3 JUNE 1993
286
Wrist arthrography confirmed the presence of a distal ulnar epiphysis (Fig 2) and angiography of the right arm and leg demonstrated normal vascular anatomy. At 5 years and 3 months of age, the patient had resection of 7cm of the ulna in the area of the pseudarthrosis (later confirmed on histology) with preservation of the distal ulnar epiphysis. This was replaced by a similar length of the right fibula, including a lcm cuff of muscle, a vascular pedicle based on the peroneal artery and a skin island for monitoring as described by Yoshimura et al (1983). The graft was fixed proximally with a semitubular plate and distally with a Kirschner wire. A palmaris longus tendon graft was used to form a sling around the distal radius and the distal end of the bone graft in order to prevent displacement of the distal ulna. End-to-end vascular anastomoses were then formed between the peroneal and ulnar arteries and the peroneal and basilic veins. The wound was closed incorporating the skin island and a long arm cast applied. A bony synostosis was created between the
distal fibula and adjacent tibia to prevent the development of a valgus deformity at the ankle. Post-operatively, the graft viability was monitored by regular inspection of the skin island. The cast was removed after 6 weeks at which time a radiograph confirmed early bony union at the proximal end of the graft. The distal Kirschner wire was removed after 12 weeks. On review 9 months post-operatively, an excellent clinical result had been achieved. The elbow joint had a full range of movement and the radial head was stable. There was good restoration of forearm rotation with 90” of supination and 30” of pronation (Fig 3). The wrist joint had full flexion and 25” of extension. All arm and hand movements were pain-free. A plain radiograph showed that the vascularized graft had been solidly incorporated (Fig 4). The distal end of the graft did not show a definite continuation with the ulnar epiphysis and was therefore further evaluated by ultrasound examination. This showed that there was cartilaginous continuity between the graft and the distal ulnar epiphysis.
Fig 2
Fig 3
Arthrogram of the right wrist showing ulnar epiphysis.
the presence
of a distal
Clinical review 9 months post-operatively supination and 30” (b) of pronation.
showing
90” (a) of
ULNAR
287
PSEUDARTHROSIS
results. Six cases were treated by non-vascu.larized bone grafting with or without internal fixation (Ah and Hooper, 1982; Bayne, 1985; Bell, 1989). Four of these required two or more procedures, but despite this only three united. A further three cases were treated by excision of the distal ulna and proximal radius and creation of a single bone forearm (0strowsk:i et al, 1985; Bell, 1989). Finally a free vascularized fibular graft and the Ilizarov technique were used in one case each (Allieu et al, 1981; Fabry et al, 1988). The poor results of conservative treatment and nonvascularized bone grafting in this condition have prompted the use of other procedures, each with its own particular associated problems. Creation of a single bone forearm greatly reduces forearm rotation. The Ilizarov technique was successfully employed in a case with a short bony defect but it is difficult to envisage its successful application in a case such as ours where the greater part of the ulna is absent. Free vascularized fibular grafting was first described in 1975 for reconstruction of complex lower limb injuries (Taylor et al, 1975). The Chinese subsequently reported its use in the treatment of tibia1 pseudarthrosis (Chen et al, 1979). It has been used only five times in the treatment of forearm pseudarthrosis; twice in pseudarthrosis of both radius and ulna (Allieu et al, 1981; Sellers et al, 1988), twice in isolated radial pseudarthrosis (Bell, 1989; Yoshimura et al, 1983) and oace in isolated ulnar pseudarthrosis (Allieu et al, 198 1). Unfortunately, interpretation of the latter case is difficult because of the short follow-up (3 months) and a lack of clinical post-operative assessment. We have demonstrated the preservation of a functioning two-bone forearm following use of a free vascularized fibula. We stress attention to details such as the palmaris longus sling to prevent instability and the fixation of the distal segment of the donor fibula to its neighbouring tibia. References G. (1982). Congenital pseudarthrosis of the ulna due to neurofibromatosis. Journal of Bone and Joint Surgery, 64B: 5: 600-602. ALLIEU, Y., GOMIS, R., YOSHIMURA, M., DIMEGLIO, A. and BONNEL, F. (1981). Congenital pseudarthrosis of the forearn-Two cases ALI, M. S. and HOOPER,
Fig 4
Plain radiograph tory incorporation
9 months post-operatively showing of the vascularized fibular graft.
satisfac-
DISCUSSION Of the 15 reported cases of isolated congenital ulnar pseudarthrosis, four were treated conservatively (Moore, 1949; Madsen, 1956; Ali and Hooper 1982; Bell, 1989). Ail four cases failed to unite. The remaining 11 cases were treated by a variety of procedures with mixed
treated by free vascularized fibular graft. Journal of Hand Surgery, 6A: 5: 415-48 I. BAYNE, L. G. (1985). Congenital pseudarthrosis of the forearm. Hand Clinics, 1: 3: 457-465. BELL, D. F. (1989). Congenital forearm pseudarthrosis: Report of six cases and review of the literature. Journal of Pediatric Orthopaedics, 9: 4: 438-443. CHEN, C. W., YU, Z. J. and WANG, Y. (1979). A new method of treatment of congenital tibia1 pseudarthrosis using free vascularized fibular graft. Annals of the Academy of Medicine of Singapore, 8: 4: 465-473. FABRY, G., LAMMENS, J., VAN MELKEBEEK, J. and STUYCK, J. (1988). Treatment of congenital pseudarthrosis with the Ilizarov technique. Journal of Pediatric Orthopaedics, 8: 1: 67-70. MADSEN, E. T. (1956). Congenital angulations and fractures of the extremities. Acta Orthopaedica Scandinavica, 25: 242-280. MOORE, J. R. (1949). Delayed autogenous bone graft in the treatment of congenital pseudarthrosis. Journal of Bone and Joint Surgery, 3lA: 1: 23-29. OSTROWSKI, D. M., EILERT, R. E. and WALDSTEIN, G. (1985). Congenital pseudarthrosis of the ulna: A report of two cases and a review of the literature. Journal of Pediatric Orthopaedics, 5: 4: 463-467. SELLERS, D. S., SOWA, D. T., MOORE, J. R. and WEILAND, A. J. (1988).
288 Congenital pseudarthrosis of the forearm. Journal of Hand Surgery, 13A: 1: 89-93. SHERTZER, .I. H., BICKEL, W. H. and STUBBINS, S. G. (1969). Congenital nseudarthrosis of the ulna: Reoort of two cases. Minnesota Medicine. 52: iO61-1066. TAYLOR, G. I., MILLER, G. D. H. and HAM, F. J. (1975). The free vascularked bone graft: a clinical extension of microvascular techniques. Plastic and Reconstructive Surgery, 55: 5: 533-544.
THE JOURNAL OF HAND SURGERY VOL. 18B No. 3 JUNE 1993 YOSHIMURA, M., SHIMAMURA, K., YOSHINOBU, I., YAMAUCHI, S. and UENO, T. (1983). Free vascularized fibular transplant. Journal of Bone and Joint Surgery, 65A: 9: 1295-1301.
Accepted: 10 Mt
Michael
December J. Earley,
1992 M Ch? FRCS @‘last.), Consultant 1, Eire.py 1993
Hospital, TempleStreet, Dubbn
Plastic Surgeon, The
Children’s
M. J. EARLEY
From the Children s Hospital,
and M. M. STEPHENS
Temple Street, Dublin, Eire
Congenital pseudarthrosis of the ulna is an extremely rare condition for which current surgical techniques have been unsatisfactory in restoring a normal two-bone forearm. We report a case which was treated by excision of the ulnar pseudarthrosis and interposition of a free ~asc~~ari~ed fibular graft with a skin island to monitor anastomotic patency. Forearm rotation has been restored and the potential for normal forearm growth has been preserved. Journal of Hand Surgery (British and European Volume, 1993) 18B: 285-288 strated complete resorption of the distal ulna (Fig 1). There was full flexion and extension of the wrist and elbow but pronation of the forearm caused subluxation of the radial head. Although the child’s mother had neurofibromatosis, she herself had no stigmata of the disease.
Congenital pseudarthrosis of the ulna in the presence of a normal radius is rare. Only 15 cases have been described in the English literature (Moore, 1949; Madsen, 19.56; Shertzer et al, 1969; Allieu et al, 1981; Ali and Hooper, 1982; Bayne, 1985; Ostrowski et al, 1985; Fabry et al, 1988; Bell, 1989). All but three cases were associated with neurofibromatosis. Untreated, this condition leads to progressive forearm deformity due to continued radial growth in the presence of an ulnar tether. The end result is a short, bowed forearm with a dislocated radial head. The multiplicity of treatment methods which have been described for this condition is a reflection of the difficulty in obtaining bony union. These have included cast immobilization, non-vascularized bone grafting with and without internal fixation, creation of a one-bone forearm, free vascularized fibular grafting and the Ilizarov compression-distraction technique. In the single case in which a free vascularized fibular graft was used, the distal end of the graft was keyed into the distal radius with a pin (Allieu et al, 1981). This must have severely impaired forearm rotation by effectively creating a one bone forearm. Furthermore, as no stabilization of the distal radio-ulnar joint was performed, wrist instability may have subsequently developed with future growth. We report a case in which a functioning two-bone forearm with the potential for normal growth has been preserved by excision of the ulnar pseudarthrosis and interposition of a free vascularized fibular graft with preservation of the distal ulnar epiphysis. CASE REPORT A l-year-old female infant injured her right arm in July 1987. Radiographs taken in the local casualty department confirmed a fracture of the distal ulnar shaft. The arm was treated in a cast for 4 weeks after which a radiograph was deemed satisfactory. In retrospect, this radiograph displayed typical features of an early pseudarthrosis. The child was discharged from further follow-up. In May 1990, the child reattended with a short, bowed forearm and a painful elbow. A radiograph demon-
Fig 1
285
Plain radiograph showing arthrosis of the right ulna.
established
congenital
pseud-
THE JOURNAL OF HAND SURGERY VOL. 18B No. 3 JUNE 1993
286
Wrist arthrography confirmed the presence of a distal ulnar epiphysis (Fig 2) and angiography of the right arm and leg demonstrated normal vascular anatomy. At 5 years and 3 months of age, the patient had resection of 7cm of the ulna in the area of the pseudarthrosis (later confirmed on histology) with preservation of the distal ulnar epiphysis. This was replaced by a similar length of the right fibula, including a lcm cuff of muscle, a vascular pedicle based on the peroneal artery and a skin island for monitoring as described by Yoshimura et al (1983). The graft was fixed proximally with a semitubular plate and distally with a Kirschner wire. A palmaris longus tendon graft was used to form a sling around the distal radius and the distal end of the bone graft in order to prevent displacement of the distal ulna. End-to-end vascular anastomoses were then formed between the peroneal and ulnar arteries and the peroneal and basilic veins. The wound was closed incorporating the skin island and a long arm cast applied. A bony synostosis was created between the
distal fibula and adjacent tibia to prevent the development of a valgus deformity at the ankle. Post-operatively, the graft viability was monitored by regular inspection of the skin island. The cast was removed after 6 weeks at which time a radiograph confirmed early bony union at the proximal end of the graft. The distal Kirschner wire was removed after 12 weeks. On review 9 months post-operatively, an excellent clinical result had been achieved. The elbow joint had a full range of movement and the radial head was stable. There was good restoration of forearm rotation with 90” of supination and 30” of pronation (Fig 3). The wrist joint had full flexion and 25” of extension. All arm and hand movements were pain-free. A plain radiograph showed that the vascularized graft had been solidly incorporated (Fig 4). The distal end of the graft did not show a definite continuation with the ulnar epiphysis and was therefore further evaluated by ultrasound examination. This showed that there was cartilaginous continuity between the graft and the distal ulnar epiphysis.
Fig 2
Fig 3
Arthrogram of the right wrist showing ulnar epiphysis.
the presence
of a distal
Clinical review 9 months post-operatively supination and 30” (b) of pronation.
showing
90” (a) of
ULNAR
287
PSEUDARTHROSIS
results. Six cases were treated by non-vascu.larized bone grafting with or without internal fixation (Ah and Hooper, 1982; Bayne, 1985; Bell, 1989). Four of these required two or more procedures, but despite this only three united. A further three cases were treated by excision of the distal ulna and proximal radius and creation of a single bone forearm (0strowsk:i et al, 1985; Bell, 1989). Finally a free vascularized fibular graft and the Ilizarov technique were used in one case each (Allieu et al, 1981; Fabry et al, 1988). The poor results of conservative treatment and nonvascularized bone grafting in this condition have prompted the use of other procedures, each with its own particular associated problems. Creation of a single bone forearm greatly reduces forearm rotation. The Ilizarov technique was successfully employed in a case with a short bony defect but it is difficult to envisage its successful application in a case such as ours where the greater part of the ulna is absent. Free vascularized fibular grafting was first described in 1975 for reconstruction of complex lower limb injuries (Taylor et al, 1975). The Chinese subsequently reported its use in the treatment of tibia1 pseudarthrosis (Chen et al, 1979). It has been used only five times in the treatment of forearm pseudarthrosis; twice in pseudarthrosis of both radius and ulna (Allieu et al, 1981; Sellers et al, 1988), twice in isolated radial pseudarthrosis (Bell, 1989; Yoshimura et al, 1983) and oace in isolated ulnar pseudarthrosis (Allieu et al, 198 1). Unfortunately, interpretation of the latter case is difficult because of the short follow-up (3 months) and a lack of clinical post-operative assessment. We have demonstrated the preservation of a functioning two-bone forearm following use of a free vascularized fibula. We stress attention to details such as the palmaris longus sling to prevent instability and the fixation of the distal segment of the donor fibula to its neighbouring tibia. References G. (1982). Congenital pseudarthrosis of the ulna due to neurofibromatosis. Journal of Bone and Joint Surgery, 64B: 5: 600-602. ALLIEU, Y., GOMIS, R., YOSHIMURA, M., DIMEGLIO, A. and BONNEL, F. (1981). Congenital pseudarthrosis of the forearn-Two cases ALI, M. S. and HOOPER,
Fig 4
Plain radiograph tory incorporation
9 months post-operatively showing of the vascularized fibular graft.
satisfac-
DISCUSSION Of the 15 reported cases of isolated congenital ulnar pseudarthrosis, four were treated conservatively (Moore, 1949; Madsen, 1956; Ali and Hooper 1982; Bell, 1989). Ail four cases failed to unite. The remaining 11 cases were treated by a variety of procedures with mixed
treated by free vascularized fibular graft. Journal of Hand Surgery, 6A: 5: 415-48 I. BAYNE, L. G. (1985). Congenital pseudarthrosis of the forearm. Hand Clinics, 1: 3: 457-465. BELL, D. F. (1989). Congenital forearm pseudarthrosis: Report of six cases and review of the literature. Journal of Pediatric Orthopaedics, 9: 4: 438-443. CHEN, C. W., YU, Z. J. and WANG, Y. (1979). A new method of treatment of congenital tibia1 pseudarthrosis using free vascularized fibular graft. Annals of the Academy of Medicine of Singapore, 8: 4: 465-473. FABRY, G., LAMMENS, J., VAN MELKEBEEK, J. and STUYCK, J. (1988). Treatment of congenital pseudarthrosis with the Ilizarov technique. Journal of Pediatric Orthopaedics, 8: 1: 67-70. MADSEN, E. T. (1956). Congenital angulations and fractures of the extremities. Acta Orthopaedica Scandinavica, 25: 242-280. MOORE, J. R. (1949). Delayed autogenous bone graft in the treatment of congenital pseudarthrosis. Journal of Bone and Joint Surgery, 3lA: 1: 23-29. OSTROWSKI, D. M., EILERT, R. E. and WALDSTEIN, G. (1985). Congenital pseudarthrosis of the ulna: A report of two cases and a review of the literature. Journal of Pediatric Orthopaedics, 5: 4: 463-467. SELLERS, D. S., SOWA, D. T., MOORE, J. R. and WEILAND, A. J. (1988).
288 Congenital pseudarthrosis of the forearm. Journal of Hand Surgery, 13A: 1: 89-93. SHERTZER, .I. H., BICKEL, W. H. and STUBBINS, S. G. (1969). Congenital nseudarthrosis of the ulna: Reoort of two cases. Minnesota Medicine. 52: iO61-1066. TAYLOR, G. I., MILLER, G. D. H. and HAM, F. J. (1975). The free vascularked bone graft: a clinical extension of microvascular techniques. Plastic and Reconstructive Surgery, 55: 5: 533-544.
THE JOURNAL OF HAND SURGERY VOL. 18B No. 3 JUNE 1993 YOSHIMURA, M., SHIMAMURA, K., YOSHINOBU, I., YAMAUCHI, S. and UENO, T. (1983). Free vascularized fibular transplant. Journal of Bone and Joint Surgery, 65A: 9: 1295-1301.
Accepted: 10 Mt
Michael
December J. Earley,
1992 M Ch? FRCS @‘last.), Consultant 1, Eire.py 1993
Hospital, TempleStreet, Dubbn
Plastic Surgeon, The
Children’s