|Year : 2015 | Volume
| Issue : 1 | Page : 50-53
Congenital pseudoarthrosis of ulna
Sanjay Keshkar1, Nirmal Dey2, Deepak Dalmia3
1 Department of Orthopaedics, ESIC Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Orthopaedics, MGM Medical College, Kishanganj, Bihar, India
3 Department of Orthopaedics, Government Medical College, Kannauj, Uttar Pradesh, India
|Date of Web Publication||13-Jun-2016|
Professor and HOD, Department of Orthopaedics, ESIC Medical College and Hospital, Joka, Kolkata - 700 104, West Bengal
Source of Support: None, Conflict of Interest: None
Isolated congenital pseudoarthrosis of the ulna is a rare entity. We present such a case of a 12-year-old boy, who was referred to us for deformity of his right forearm (since birth), and pain on the right hand with exertion. The examination and investigation confirmed the diagnosis of congenital pseudoarthrosis of the ulna, which was associated with neurofibromatosis and was treated by the creation of one bone forearm as a salvage procedure.
Keywords: Congenital, neurofibromatosis, one bone forearm, pseudoarthrosis, ulna
|How to cite this article:|
Keshkar S, Dey N, Dalmia D. Congenital pseudoarthrosis of ulna. J Orthop Traumatol Rehabil 2015;8:50-3
| Introduction|| |
The problem of large defect or gap in one of the forearm bones is rare, but not uncommon, where most of the radius except the lower end may be missing, or most of the ulna except the upper end is absent or deformed. If a child of this defect remains uncorrected for a long period, secondary deformities will develop. Isolated congenital pseudoarthrosis of the ulna is a rare entity. We present such a case, which was associated with neurofibromatosis and was treated by the creation of one bone forearm as a salvage procedure.
| Case Report|| |
A 12-year-old boy was referred to us for deformity of his right forearm (since birth), and pain on the right hand with exertion. The child had café-au-lait spot typical of neurofibromatosis on his back with a family history of a similar spot on her mother. Varus deformity of the elbow was 30°; pronation was restricted, but supination was full. Shoulder, elbow, and wrist movements were good, and the hand function was normal [Figure 1]a.
|Figure 1: Preoperative clinical photograph (a) of patient showing deformity of his left forearm and radiograph (b) of left forearm (anteroposterior and lateral views) showing pseudoarthrosis of ulna associated with cubitus varus with radial bowing with proximal migration of radius|
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Radiological examination [Figure 1]b showed pseudoarthrosis of the ulna at its middle two-third. Proximal one-fourth of the ulna was present with tapering sclerotic end. However, distal one-fourth was just visible as a thin sclerotic line. Marked ulnar bowing of radius with proximal migration (dislocation) of the radial head is also noted on the radiograph.
Routine blood examination was normal. A thorough examination and related screening investigations are done to detect other obvious congenital anomaly of the skeletal or other system of the body, which was found to be negative. With an aim to correct deformity as a salvage procedure, creation of one bone forearm was planned in this case.
We used the posterior approach in this case. The incision starts on the radial side from the level of lateral epicondyle and curves toward the ulna. The soft tissues are erased from the ulna as well as the radius. Radius is osteotomized, a little longer than the actual length required to bring it into alignment with the upper end ulna. Proximal radius above the osteotomy was excised. The sclerotic end of proximal ulna along with tethered soft tissues was also excised. The bones are aligned and fixed internally by intramedullary K-wire [Figure 2]a. The wound was closed, and above elbow plaster slab was given.
|Figure 2: Postoperative radiograph of left forearm (anteroposterior and lateral views) showing one bone forearm fixed by intramedullary K-wire (a) which was removed after 1 year (b|
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Sutures were removed after 3 weeks, and long arm plaster cast given for 2 months. Then plaster was changed, and short arm plaster cast applied for another 2 months. Finally, plaster was removed; X-ray was taken which was showing good union [Figure 2]b. Routine physiotherapy of upper limb was advised while the limb was on plaster and continued after removal. K-wire was removed after 1 year of operation and then 6 monthly follow-up was done. At final follow-up after about 4 years of operation, there is solid union with the remodeling of bone occurred [Figure 3]. The elbow, wrist, and finger movements were normal; however, pronation/supination was restricted which was compensated by shoulder movement [Figure 4]a,[Figure 4]b,[Figure 4]c,[Figure 4]d. The deformity got corrected, but the shortening of forearm was obvious. Overall, the function of upper limb was very good causing a high level of satisfaction to the patient and his parents.
|Figure 3: Follow-up radiograph of left forearm (anteroposterior and lateral views) after 3 years of removal of K-wires showing excellent bony union and remodeling|
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|Figure 4: Follow-up photograph (after 4 years) of operation showing full flexion/extension of elbow with correction of deformity (a and b) and pronation/supination compensated by shoulder (c and d)|
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| Discussion|| |
Congenital pseudoarthrosis of the ulna in neurofibromatosis is extremely rare. Few cases have been reported in the literature. Most of them showed, sclerotic tapering of the ends of the bones characteristic of type 2 congenital pseudoarthrosis of the tibia. However, 1 or 2 cases of them occurred through lytic lesion similar to type 3 congenital pseudoarthrosis of the tibia. Irrespective of the type, the pseudoarthrosis of ulna produces angulation of radius, shortening of the forearm, and dislocation of the radial head.
Because of significant bowing of radius in congenital pseudoarthrosis of the ulna in a very young child, early surgery is indicated. In established pseudoarthrosis of the ulna with tapering bone ends, bone grafting to fill up the gap is not successful. In such cases, the distal ulna should be excised early to relieve its tethering effect on radius followed by a suitable corrective brace. If the radial head dislocates, it should be excised and a synostosis (one bone forearm) produced between radius and ulna. The functional results of one bone forearm after the loss of ulna have been good provided the forearm should be in neutral with no supination or pronation.,,, In our case, the radial head was dislocated with severe cubitus varus deformity. Hence, one bone forearm was created.
Making of one bone forearm is based on the principle that upper end of the ulna is responsible for the stability of elbow joint and the lower end of radius for the stability of wrist joint. The deforming growth of the remaining bone is immediately controlled. The deformity of forearm radial or ulnar deviation of hand and instability of the elbow and wrist are all corrected. The movements at both the joints are also restored. If the lower epiphysis of the radius is intact, the length of the forearm also starts developing. The function of the hand improves considerably and is restored to normal. The loss of pronation and supination is not obvious because of fixation in mid prone position and compensatory movements at the shoulder.,
We did end to end fusion, taking a little more length of the longer bone so as to provide a compression force, because side to side fusion as used by Lloyd-Roberts leaves a shearing stress, and there are chances of delayed union or nonunion, although he has supplemented all his cases with cancellous bone grafting to eliminate nonunion. The contention is that if an end to end fusion is done chances of nonunion are considerably less, and bone grafting as advocated by Moore  is rarely required as in our case.
On the basis of a review of the literature and their two additional cases of congenital pseudoarthrosis of ulna due to neurofibromatosis, they recommended that the main aim of treatment should be the maintenance of the normal relative lengths of radius, and ulna with early excision of the pseudoarthrosis to remove restraining effect of the abnormal ulna. This will allow the normal development of lower end of humerus and radius, and prevent dislocation of the radial head.
| Conclusion|| |
With our experience of this case and also after reviewing the literature, we conclude that congenital pseudoarthrosis of the ulna due to neurofibromatosis is a rare entity, which needs early surgical intervention. One bone forearm is reliable salvage procedure for such cases particularly in older children or in adolescents.
We are thankful for director and other helping staff of National Institute for the Orthopaedically Handicapped, Kolkata (where the study was done 7 years ago when the corresponding author was working as Assistant Professor in Orthopaedics).
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]